Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: Frequent post-operative cholangitis in biliary atresia (BA) affects the long-term native liver survival. This study assessed the characteristics of early cholangitis and their influence on the prognosis. METHODS: Forty-three patients with BA who underwent surgery between 2000 and 2020 were analyzed for routine inflammatory markers. Early cholangitis characteristics were compared between native liver survivor (NLS) and living donor liver transplant (LDLT) patients. RESULTS: Among the 43 patients, 30 (69.8%) experienced 130 episodes of cholangitis. In the area under the receiver operating characteristics curve (AUROC) analysis, the cutoff value of the total cholangitis episodes was 3, with an area under the AUROC curve of 0.695 (95% confidence interval 0.522-0.868). Before 3 years old, 113 episodes (86.9%) of cholangitis were observed. The white blood cell, C-reactive protein, and alanine aminotransferase values at cholangitis onset did not markedly differ between the LDLT and NLS groups. Conversely, the neutrophil-to-lymphocyte ratio in the NLS group was significantly lower than in the LDLT group (0.85 vs. 1.63, p < 0.001). CONCLUSIONS: Cholangitis in the NLS group was lymphocyte-dominant and atypical in its pathogenesis. Lymphocyte-dominant cholangitis is non-suppurative, and future research should clarify its pathogenesis to improve the treatment and prognosis of BA.

DOI： 10.1007/s00383-024-05774-1

PubMed

Language：English  

DOI： 10.1007/s00383-024-05776-z

PubMed

Language：English  

DOI： 10.1007/s00383-024-05763-4

PubMed

Publisher：東京医学社  

DOI： 10.24479/ps.0000000851

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Ovarian endometriomas (OEs) are rarely found in the pediatric population, especially before menstruation. We report a 6-year-old girl who was postoperatively diagnosed with OE before menstruation. CASE PRESENTATION: A 6-year-old girl presented to a local pediatrician with abdominal pain and vomiting. Abdominal ultrasonography revealed a multilocular cystic lesion to the left of the bladder. Magnetic resonance imaging (MRI) revealed similar findings, with the contents of the cyst showing a low signal on T1-weighted imaging and a high signal on T2-weighted imaging. The patient was referred to our institution for further examination. Enhanced computed tomography (CT) showed a multilocular cystic lesion sized 56 × 44 × 30 mm with partial calcification. The left ovarian vein was dilated, suggesting the origin of the tumor to be the left ovary. Extirpation of the lesion was performed under laparoscopic assistance. Pathological findings indicated an ovarian endometrioma. To our knowledge, this is the youngest report of an OE diagnosed in a patient prior to menstruation. CONCLUSIONS: OEs in children before menstruation are extremely rare; thus, the long-term prognosis is yet to be determined.

DOI： 10.1186/s40792-024-01951-5

PubMed

Publisher：東京医学社  

DOI： 10.24479/ps.0000000773

Language：English   Publishing type：Research paper (scientific journal)  

Background: Transumbilical laparoscopy-assisted appendectomy (TULAA) is the technique of choice for all types of appendicitis. However, the technique is challenging for trainees to learn in comparison with performing conventional three-port laparoscopic appendectomy (CTPLA) in children. We aimed to compare the surgical outcomes of children with appendicitis treated by TULAA versus CTPLA performed by pediatric surgeons in training (PSITs). Materials and Methods: This retrospective study analyzed pediatric patients with acute appendicitis treated with CTPLA or TULAA between April 2016 and December 2022. Operative time (OT: minutes), pneumoperitoneum time (PT: minutes), blood loss (milliliter), length of hospital stay (days), and surgical site infection rate were compared between the two groups. Operative outcomes were also analyzed according to type of appendicitis such as uncomplicated and complicated cases. Results: Two hundred twenty-five laparoscopic appendectomies were performed by CTPLA (n = 94) or TULAA (n = 131). All cases were performed by PSITs and there was no open conversion cases. TULAA had a shorter OT (67.0 ± 28.4 versus 78.3 ± 21.7; P < .01) and PT (26.1 ± 17.4 versus 52.5 ± 22.1 min; P < .01). The surgical site infection rate was slightly higher in the TULAA group, but the difference was not statistically significant. In uncomplicated appendicitis (n = 164), significant differences between the CTPLA and TULAA groups were observed in OT (CTPLA versus TULAA: 70.7 ± 14.9 versus 59.1 ± 21.6, P < .01) and PT (CTPLA versus TULAA: 43.6 ± 13.1 versus 20.4 ± 13.6, P < .01). With regard to postoperative complications, only surgical site infection was significantly different between the CTPLA and TULAA groups (CTPLA: 0.0% versus TULAA: 8.2%, P < .05). In complicated cases (n = 61), there were significant differences between the groups in PT (CTPLA versus TULAA: 73.4 ± 24.9 versus 42.3 ± 17.2, P < .01) and length of hospital stay (CTPLA versus TULAA: 7.0 ± 1.3 versus 8.9 ± 4.7, P < .05). Conclusions: TULAA had a shorter OT and PT than CTPLA. TULAA for PSITs shows similar safety and feasibility to CTPLA for not only uncomplicated cases but also complicated cases.

DOI： 10.1089/lap.2023.0275

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Few studies have analyzed the effect of the size of the working space in training on the acquisition of endoscopic skills. In this study, adult- and infant-sized dry boxes (DBs) were used to verify how the size of the working space in training affects forceps manipulation and learning curve. MATERIAL AND METHODS: Seventy-two medical students were enrolled. The task was peg transfer. The training environment was divided into adult- and infant-sized DBs. Skill evaluations were also divided into adult- and infant-sized DBs (four groups in total). The forceps manipulation characteristics and task completion time were compared before and after training. RESULTS: Regarding skill evaluations using adult-sized DBs, there were no significant differences between the infant- and adult-sized DB-trained groups. Regarding skill evaluations using infant-sized DBs, there were no significant differences between the groups before training. After training, there was no significant difference in the total path length or average acceleration of the forceps between the groups. However, the infant-sized DB-trained group had a significantly faster average forceps velocity and faster task completion time than the adult-sized DB-trained group. CONCLUSION: Training with a small DB is more efficient in acquiring smoother and faster forceps manipulation in a small working space.

DOI： 10.1080/13645706.2024.2321950

PubMed

Language：English  

DOI： 10.1055/s-0044-1779624

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: We evaluated the effect of recombinant human hepatocyte growth factor (rh-HGF) on intestinal adaptation in a rat model of short-bowel syndrome (SBS). METHODS: Sprague-Dawley rats underwent jugular vein catheterization for continuous total parenteral nutrition (TPN) and 90 % small bowel resection. The animals were divided into 3 groups: TPN/SBS (control group, n = 7), TPN/SBS/intravenous recombinant human hepatocyte growth factor (HGF) (0.3 mg/kg/day) (HGF group, n = 7), and TPN/SBS/intravenous c-Met inhibitor (0.3 mg/kg/day) (anti-HGF group, n = 5). On day 7, rats were euthanized and histologically evaluated. Serum diamine oxidase (S-DAO) levels were evaluated using an enzyme-linked immunosorbent assay. The nutrient transporter and glucagon-like peptide-2 (GLP-2) receptor expression were evaluated using real-time polymerase chain reaction. RESULTS: The jejunal and ileal villus heights were higher and the S-DAO concentrations significantly higher (p = 0.04) in the HGF group than in the control and anti-HGF groups. The sodium-dependent glucose transporter 1 expression in the HGF group was significantly higher than in the control group and significantly suppressed in the anti-HGF group (p < 0.01). The peptide transporter 1 expression in the jejunum was higher in the HGF group than in the other groups and significantly suppressed in the anti-HGF group (p < 0.01). The GLP-2 receptor expression in the jejunum was higher in the HGF group than the other groups, and it was significantly suppressed in the anti-HGF group (p < 0.01). These jejunal results regarding nutrient transporter an GLP-2 receptor were not found in the ileum. CONCLUSIONS: The administration of rh-HGF appears to be more effective in the jejunum than in the ileum. TYPE OF STUDY: Experimental Research. LEVEL OF EVIDENCE: N/A.

DOI： 10.1016/j.jpedsurg.2023.11.028

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Most cases of intestinal malrotation appear in neonates with bilious vomiting due to midgut volvulus, whereas in cases that develop beyond infancy, the initial symptoms vary. This study investigated the clinical features of these two populations and identified issues that should be considered in daily practice. METHODS: A retrospective chart review was conducted from January 1, 2010, to December 31, 2022. Data on patients with intestinal malrotation were collected in an anonymized fashion from five pediatric surgical hub facilities in the Southern Kyushu and Okinawa areas of Japan. RESULTS: Of the 80 subjects, 57 (71.3%) were neonates (Group N) and 23 (28.7%) were infants and schoolchildren (Group I). The frequencies of initial symptoms, such as abdominal distention (Group N: 19.3% vs. Group I: 13.0%), bilious vomiting (59.6% vs. 43.5%), and hematochezia (8.8% vs. 21.7%), were not skewed by the age of onset (p = 0.535, 0.087, and 0.141, respectively). Midgut volvulus was significantly more frequent in Group N (71.9% [41/57] vs. 34.8% [8/23]; p = 0.005), while the degree of torsion was greater in group I (median 360° [interquartile range: 180-360°] vs. 450° [360-540°]; p = 0.029). Although the bowel resection rate was equivalent (7.0% [4/57] vs. 4.3% [1/23]; p = 1.000), half of the patients in Group N presented with 180° torsion. The neonatal intestine has been highlighted as being more susceptible to ischemia than that in older children. CONCLUSIONS: The incidence of midgut volvulus is higher in neonates than in older children. Even relatively mild torsion can cause ischemic bowel changes during the neonatal period. LEVEL OF EVIDENCE: LEVEL III.

DOI： 10.1016/j.jpedsurg.2023.11.020

PubMed

Publisher：東京医学社  

DOI： 10.24479/ps.0000000641

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: The purpose of this study was to investigate the autophagy associated with apoptosis in hepatic damage in the short bowel syndrome rat model. METHODS: SD rats underwent jugular vein catheterization for continuous total parenteral nutrition (TPN) and 90% small bowel resection. Animals were divided into two groups: TPN plus SBS (Control group) or TPN plus SBS plus intravenous administration of HGF (HGF group). On day 7, the rats were harvested, and hepatocellular injury was evaluated. RESULTS: In an SBS rat model, hepatic steatosis and lobular inflammation were histologically suppressed in the HGF group (p < 0.01). The expression of tumor necrosis factor-α in the HGF group tend to be higher than that in the control group (p = 0.13). The gene expression of transforming Growth Factor-β in the HGF group was suppressed compared to the control group (p < 0.01). HGF treatment may have an antiapoptotic effect via the intrinsic pathway by caspase 9. Protein expressions of Rubicon (p = 0.03) and p62 (p < 0.01) in the HGF group were found to have increased compared to those in the control group. CONCLUSION: The inhibitory effect of HGF on hepatic steatosis remains unclear, and further studies focusing on the mechanisms of fat accumulation are needed.

DOI： 10.1007/s00383-023-05593-w

PubMed

Language：English  

DOI： 10.1007/s00595-023-02760-3

PubMed

Publisher：東京医学社  

DOI： 10.24479/pm.0000001392

Language：Japanese   Publisher：(株)東京医学社  

Other Link： https://search.jamas.or.jp/default/link?pub_year=2023&ichushi_jid=J00645&link_issn=&doc_id=20231115050021&doc_link_id=%2Faq9syoge%2F2023%2F005511%2F020%2F1228-1233%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Faq9syoge%2F2023%2F005511%2F020%2F1228-1233%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Publisher：東京医学社  

DOI： 10.24479/ps.0000000600

Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p>Fallopian tube torsion with an adolescent onset is rare, and the lack of specific findings hampers a preoperative diagnosis. We experienced treating a case of fallopian tube torsion at puberty whose definitive diagnosis took a long time. [Case] A 13-year-old girl presented to the emergency clinic with a chief complaint of left lower abdominal pain that had begun one week before this visit. She was diagnosed with acute enteritis and kept under observation. However, she continued to have abdominal pain, and her pediatrician suspected that she had appendicitis with an abscess. Despite treatment with antimicrobial agents, her symptoms did not improve, so she was referred to our department with a suspicion of acute abdomen. Enhanced computed tomography (CT) showed a 4-cm cystic lesion in the pelvic cavity. Diagnostic laparoscopy was performed under suspicion of ovarian torsion or adnexal torsion. A dark-red twisted left fallopian tube was seen on the dorsal side of the uterus. Untwisting the tube laparoscopically proved difficult, so laparotomy of the lower abdomen was performed. The fallopian tube had a torsion of 1,440°, and it remained dark red even after the torsion had been released. Her left ovary was normal. Preservation of the fallopian tubes was impossible, and tubectomy was performed. [Conclusion] Many cases of tubal torsion are difficult to diagnose and ultimately result in tubal resection. An early diagnosis is important for preserving the fallopian tubes.</p>

DOI： 10.11164/jjsps.59.6_1004

Language：Japanese   Publisher：(一社)日本小児外科学会  

思春期発症の卵管捻転は稀で,特異的な所見に乏しく診断に苦慮する.今回診断に時間を要した思春期発症の卵管捻転の症例を経験したので報告する.【症例】13歳の女児.当施設受診の1週間前からの左下腹部痛を主訴に近医を受診したが,急性腸炎の診断で経過観察となっていた.その後も腹痛が続き,近医小児科で膿瘍形成虫垂炎を疑われ抗菌薬加療が行われるも症状改善がなく,急性腹症が疑われ当施設紹介となった.造影CT検査では骨盤腔内に4cm大の嚢胞性病変を認め,付属器捻転の可能性を考え審査腹腔鏡を行った.子宮背側に暗赤色の捻転した左卵管を認め,腹腔鏡下操作での捻転解除は困難でPfannenstiel切開での開腹手術へと移行した.左卵巣は正常であったが左卵管は1,440°捻転し,捻転解除後も暗赤色調で温存は困難と判断し左卵管切除術を施行した.【結語】卵管捻転は診断に時間を要し最終的に卵管切除となる症例が多く温存には早期診断が重要であると考えられた.(著者抄録)

Language：English  

DOI： 10.1007/s00383-023-05550-7

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: Anovestibular fistula (AVF) is the most common type of ARM in female patients. The present study investigated changes over time in the postoperative defecation function of female patients with AVF. METHODS: Patient data were collected from 1984 to 2021. Eighty-eight female patients with AVF were enrolled. Patients' characteristics and the long-term outcome of defecation function were reviewed and analyzed retrospectively. The bowel function was evaluated according to the Japan Society of ARM Study Group evacuation score (ES). RESULTS: Thirty-eight patients underwent anal transposition (AT), and 8 underwent anterior sagittal anorectoplasty (ASARP). The total evacuation score (ES) in AVF patients reached "excellent" at nine years old, regardless of the operative procedure. The constipation scores with AT showed better improvement than those with ASARP, but soiling scores in the ASARP group showed better improvement than those in the AT group. The postoperative complications did not affect the postoperative bowel function in AVF patients. CONCLUSION: Most patients with AVF eventually achieved a satisfactory total ES. Given the difference in defecation score transition depending on the operative procedure or postoperative complications, it may be important to perform long-term defecation management via surgical procedures.

DOI： 10.1007/s00383-023-05554-3

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: We compared the clinical features of patients with biliary atresia (BA) associated with a bleeding tendency (BT) at the time of the diagnosis with those of patients without a bleeding tendency (NBT). METHODS: The patients' background characteristics, age in days at the first visit, Kasai portoenterostomy (KPE), and postoperative course were retrospectively analyzed. RESULTS: Nine of the 93 BA patients (9.7%) showed a BT, including 7 with intracranial hemorrhaging (ICH), 1 with gastrointestinal bleeding, and 1 with a prothrombin time (PT) of 0%. The age at the first visit was 62 ± 12 days old for BT patients and 53 ± 27 days old for NBT patients (p = 0.4); the age at KPE was 77 ± 9 days old for BT patients and 65 ± 24 days old for NBT patients (p = 0.2); the time from the first visit to surgery was 13 ± 7 days for BT patients and 11 ± 10 days for NBT patients (p = 0.5); and the native liver survival rate was 56% for BT patients and 58% for NBT patients (p = 1), with no significant difference in any of the parameters. The neurological outcomes of survivors of ICH were favorable. CONCLUSIONS: Appropriate BT correction allowed early KPE even after ICH, resulting in native liver survival rates comparable to those of NBT patients without significant neurological complications.

DOI： 10.1007/s00595-023-02744-3

PubMed

Publisher：東京医学社  

DOI： 10.24479/ps.0000000550

Language：English   Publishing type：Research paper (scientific journal)  

Background: Endoscopic surgery also has been becoming widespread in the field of pediatric surgery. However, most disease treated by pediatric surgery in a single institution are small number of cases. Besides, the variety of operative procedures that need to be performed in this field is quite wide. For these reasons, pediatric surgeons have limited opportunities to perform endoscopic surgery. Therefore, it is difficult to introduce advanced endoscopic surgery at a single local hospital. To educate pediatric surgeons in local hospitals, for widespread advanced pediatric endoscopic surgery safely, and to eliminate the need for patient centralization, we have introduced a proctoring system. We compared the surgical results of our institution, a center hospital, with other local institutions, to investigate the feasibility of our proctoring system. Methods: The experienced pediatric surgeon of our institution visits local hospitals to provide onsite coaching and supervises pediatric surgeons on the learning curve. All patients who underwent laparoscopic cyst excision and hepaticojejunostomy for choledochal cysts, one of the advanced pediatric endoscopic surgeries was retrospectively reviewed. Results: Thirty-four cases were evaluated (14 cases in our institution, 20 cases in 9 other institutions). The procedures of all 34 cases were performed by surgeons with 0-2 cases of experience in the procedure. There were no open conversion cases. There was no significant difference in the operative date. There was 1 case (6.7%) of postoperative complications during hospitalization at our institution and 3 cases (14.3%) at other institutions (P = .47). Two cases of late complications (13.3%) occurred at our institution, whereas 6 cases (28.6%) occurred at other institutions (P = .28). Conclusion: With the proctoring system, the performance and completion of advanced pediatric endoscopic surgery at local institutions was feasible. This has important implications given the ever-growing demand for pediatric endoscopic surgery and the increasing need for competent pediatric endoscopic surgeons.

DOI： 10.1089/lap.2023.0087

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: After conducting a nationwide survey of persistent cloaca (PC), we assessed whether or not the timing of definitive anorectoplasty affects the long-term bowel function of patients with PC. METHODS: Patient information was obtained via questionnaire, and a total of 169 PC patients who underwent posterior sagittal anorectourethrovaginoplasty (PSARUVP) were enrolled in this study. Patients were classified into 2 groups based on their operative period, which was analyzed by the area under the receiver operating characteristic curve: the early group (EG) underwent anorectoplasty at ≤ 18 months old (n = 106), and the late group (LG) underwent anorectoplasty at > 18 months old (n = 63). The bowel function was evaluated using the evacuation score of the Japan Society of Anorectal Malformation Study Group. We also examined the postoperative results of vaginoplasty. RESULTS: The total evacuation score was significantly higher in the EG than in the LG (5.2 ± 1.7 vs. 4.2 ± 1.8, p = 0.003). The frequency of bowel movement and the constipation scores were significantly higher in the EG than in the LG (1.4 ± 0.6 vs. 1.2 ± 0.7, p < 0.05, 2.4 ± 1.0 vs. 2.1 ± 1.0, p < 0.05, respectively). Postoperative vaginal stenosis was observed in 18 cases (10.7%), of which 16 could be reconstructed transperineally. CONCLUSION: PSARUVP should be performed in early infancy and facilitate vaginal reconstruction.

DOI： 10.1007/s00383-023-05526-7

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: Cloacal exstrophy (CE) patients may need bladder reconstruction after initially undergoing surgery to obtain continence and improve their quality of life. This study attempts to clarify the clinical features of CE patients who underwent bladder augmentation (BA) and their urinary functional outcomes based on a nationwide survey in Japan. METHODS: A questionnaire survey was conducted, and 150 CE patients were enrolled. Their clinical characteristics and urinary outcomes were reviewed. RESULTS: BA was performed in 52 patients (34.7%). Most cases underwent early bladder closure at initial surgery in neonate period. The age at the BA was performed 6.4 [6-9.0] years. Among them, the most used organ for BA was ileum (n = 30, 57.7%). Regarding the outcomes, the age when the renal function was evaluated was 14.0 [10.0-20.5] years and the serum creatinine level was 0.44 [0.36-0.60] (mg/dl). Clean intermittent catheterization was required in 37 (71.2%) patients. On the other hand, no dialysis or kidney transplantation was necessary in any of these patients. CONCLUSION: The renal function and conditions of patients who underwent BA were relatively well preserved. Individualized management with a stepwise surgical approach for CE patients should thus be considered in the future.

DOI： 10.1007/s00383-023-05512-z

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

INTRODUCTION: Surgical management of neuroblastoma (NB) has been performed by open procedures for decades. However, advances in surgical devices and technology have made minimally invasive surgery safe and reproducible. In this study, we compared open and laparoscopic surgery regarding biopsy success and curative resection to determine the safety and feasibility of laparoscopic surgery for pediatric patients with adrenal NB. METHODS: We reviewed the clinical data of 22 NB patients who underwent surgery from 2006 to 2021 in our institution. All patients with adrenal NB were diagnosed histologically, and we retrospectively analyzed the data. RESULTS: The male/female ratio was 16/6. The median age was 2.5 (interquartile range: 2-4) years old, and the laterality was on the right in 13 and on the left in 9. A total of 20 patients underwent a tumor biopsy: 14 via laparotomy, five laparoscopically, and one retroperitoneally. Four patients underwent laparoscopic resection and 11 patients underwent open resection after chemotherapy. Two patients with stage LI underwent primary tumor resection laparoscopically. For curative resection in image-defined risk factor (IDRF)-negative patients, laparoscopic surgery resulted in shorter operation time, with less bleeding and an earlier oral intake. The three IDRF-single-positive patients on the liver (laparoscopic surgery: one patient) had a shorter operation time and less bleeding than the IDRF-multiple positive patients. CONCLUSION: Laparoscopic surgery was performed in a limited number of adrenal NB cases. A laparoscopic biopsy for adrenal NB seems to be safe and feasible to perform. Laparoscopic surgery, in carefully selected cases, permits safe and efficient resection of adrenal NB in pediatric patients.

DOI： 10.1111/ases.13204

PubMed

Language：Japanese   Publisher：(公社)日本小児科学会  

症例は日齢4、男児で、出生時に左頸部嚢胞を指摘され、頸部超音波検査および頸部造影検査で新生児梨状窩瘻と診断し、呼吸困難はなく全身状態は良好であったが、早期から嚢胞ドレナージと抗菌薬治療を行い、手術による嚢胞摘出を行うことで、呼吸不全をきたすことなく良好な転帰(術後2年を経過し、再発なし)を得た。

Publisher：東京医学社  

DOI： 10.24479/ps.0000000493

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: The repair of large abdominal wall defects that cannot be closed primarily is quite challenging. The component separation technique (CST) is a surgical approach using autologous tissue to close large abdominal wall defects. The CST requires extensive dissection between the abdominal skin and the anterior sheath of the rectus abdominis muscle. Subsequently, incisions are made at both sides of the external oblique aponeurosis, releasing the external oblique muscle from the internal oblique muscle, and then the right and left rectus abdominis muscles are brought together in the midline for defect closure. However, impairment of blood flow in the abdominal wall skin and necrotic changes are recognized as potential complications. CASE PRESENTATION: The CST was performed in a 4-year-old boy with a large ventral hernia who had undergone skin closure with abdominal wall relaxing incisions for the primary treatment of giant omphalocele in the neonatal period. Given his history of incisions on the abdominal wall, he was speculated to be at high risk for postoperative skin ischemia. Dissection was therefore kept to a minimum to preserve the blood supply from the superior and inferior epigastric arteries and perforating branches of those arteries through the rectus abdominis muscle. In addition, care was taken to adjust the muscle relaxant dosage while monitoring the intravesical pressure, ensuring that it did not exceed 20 mmHg to avoid impaired circulation in the abdominal wall caused by abdominal compartment syndrome. He was discharged 23 days after the surgery without any complications, and neither recurrence of the ventral hernia nor bowel obstruction was observed in 4 years. CONCLUSIONS: A giant omphalocele with primary skin closure was treated by applying the CST. The procedure can be performed safely while preserving the blood flow to the abdominal wall, even in patients with a history of relaxing incisions on the abdominal skin. The CST is expected to be effective for repairing the large abdominal wall defects seen in giant omphalocele when primary closure is not possible.

DOI： 10.1186/s40792-023-01679-8

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

We, herein, report a surgical technique for laparoscopy-assisted jejunostomy tube placement in an infant using a loop needle device to fix the jejunum and abdominal wall. A 3-year-old boy with Down's syndrome underwent nutritional management by gastrostomy due to oral feeding difficulty after radical surgery for congenital duodenal stenosis and following bile duct stenosis. However, intractable gastrostomy site leakage emerged; hence, laparoscopy-assisted gastrostomy takedown and simultaneous laparoscopy-assisted jejunostomy tube placement were planned. After laparoscopy-assisted gastrostomy closure was performed, the jejunum was extracted through the umbilical trocar wound. A jejunostomy tube kit was inserted at the left side of the umbilicus. After tube insertion into the jejunum, the jejunostomy tube was wrapped with four interrupted sutures using the Witzel technique. Suture threads were extracted by percutaneous insertion of a loop needle device, and then, the jejunum was fixed to the abdominal wall. The post-operative course was uneventful. Percutaneous insertion of a loop needle device is useful for fixing a jejunostomy tube to the abdominal wall using the Witzel technique. Our technique is safe, secure and expected to reduce the risk of dislodgement of tube and peritonitis associated with jejunostomy tube placement.

DOI： 10.4103/jmas.jmas_10_23

PubMed

Publisher：東京医学社  

DOI： 10.24479/ps.0000000445

Language：English   Publishing type：Research paper (scientific journal)  

Purpose: Severely neurologically impaired patients sometimes require anti-reflux surgery with preceding gastrostomy. We apply a traction technique for laparoscopic fundoplication (LF) without gastrostomy takedown (GTD) in such cases. We conducted a multicenter review to assess the feasibility of our approach. Materials and Methods: In brief, the traction technique involves left-lateral-traction of the stomach body, right-lateral-traction of the round ligament of the liver, and elevation of the left liver lobe to create a sufficient field for manipulating the forceps. Patients who underwent LF with Nissen's procedures in 2010-2022 were retrospectively reviewed. Data were analyzed by a one-way analysis of variance. Results: The operative approaches included the traction technique (n = 16; Group 1), GTD and reconstruction (n = 5; Group 2), and LF followed by gastrostomy (n = 92; Group 3). In comparison with Group 1, significant differences were only found in pneumoperitoneum time (Group 1 versus Group 2 versus Group 3: 174.4 minutes versus 250.4 minutes versus 179.5 minutes; P = .0179). Operating time (222.7 minutes versus 303.0 minutes versus 239.7 minutes; P = .0743), duration to full-strength enteral nutrition (10.4 days versus 17.2 days versus 11.0 days; P = .0806), and length of hospital stay (17.2 days versus 31.0 days versus 18.5 days; P = .3247) were equivalent. No re-fundoplication was required in Group 1. Conclusion: The traction technique secures the operative quality and outcome of LF without GTD.

DOI： 10.1089/lap.2022.0576

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: The size of the display used for endoscopic surgery has been increasing recently, but the effect of the display size on procedure outcomes is unclear. The present study clarified the effect of display size on the outcomes of endoscopic surgical procedures. METHODS: Eight pediatric surgeons performed the laparoscopic fundoplication task using a disease-specific laparoscopic simulator with 3 different display sizes (43, 32, and 24 inches). The movement of the forceps during the task was measured using an electromagnetic spatial three-dimensional position-measuring instrument. The movements of the eyes and head during the task were also measured using glasses-style smart eyewear. We assessed the time required to complete the task, total path length of forceps, average velocity of forceps, number of blinks, number and strength of eye movements, and head tilt of participants during the task. RESULTS: There were no significant differences in the required time, total path length, average velocity, number of blinks, number and strength of eye movements, or head tilt among the three display sizes. After the task, participants were asked which display felt the easiest to use. Four participants (50%) answered that the 32-inch display was easiest to use. CONCLUSION: This study showed that display size differences did not influence endoscopic surgical procedures. However, 50% of participants felt that the task was easiest using the middle display size. There may be an optimal monitor size for a given procedure. LEVEL OF EVIDENCE: Level II.

DOI： 10.1016/j.jpedsurg.2022.12.023

PubMed

Language：English  

INTRODUCTION AND IMPORTANCE: Mucoepidermoid carcinoma presents as an exophytic endobronchial mass that induces obstructive symptoms often followed by distal collapse atelectasis of the lung parenchyma. CASE PRESENTATION: A six-year-old girl had recurrent bacterial pneumonia and atelectasis of the right upper lobe. Computed tomography revealed a 30-mm mass in the anterior segment of the right upper lobe with an obstructed trachea and peripheral atelectasis. A minor salivary gland tumor was suspected, so thoracoscopic right upper lobectomy (RUL) was performed. Intraoperative bronchoscopy showed no protrusion of the tumor into the tracheal lumen. We confirmed that there was no injury to the middle lobe branch and no residual tumor via bronchoscopy before transection of the trachel bronchus of the right upper lobe. The histological type was low-grade mucoepidermoid carcinoma. The postoperative course was uneventful, and no recurrence was evident after one year. CLINICAL DISCUSSION: Primary pulmonary cancers in children are extremely rare. Mucoepidermoid carcinoma is the most common disease in pediatric primary lung tumors but remains relatively rare. Mucoepidermoid carcinoma of the tracheobronchial tree sometimes requires sleeve resection. Intraoperative bronchoscopy helped determine the exact position of the tumor. The value of intraoperative bronchoscopy for sparing the lung parenchyma and preserving as much of the respiratory function as possible. Intraoperative bronchoscopy should be actively performed in cases of pediatric lobectomy, especially those involving tracheobronchial tumors. CONCLUSION: Intraoperative bronchoscopy allowed for complete RUL without residual tumor or injury of the middle lobe bronchus.

DOI： 10.1016/j.ijscr.2023.108119

PubMed

Publisher：東京医学社  

DOI： 10.24479/pm.0000000804

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: Midgut volvulus is an urgent disease often occurring in neonates. This study clarified the clinical features of midgut volvulus and evaluated predictors to avoid bowel resection. METHODS: This bi-center retrospective study enrolled 48 patients who underwent surgery for intestinal malrotation between 2010 and 2022. Patients' background characteristics and preoperative imaging findings were reviewed. RESULTS: Midgut volvulus was recognized in 32 patients (66.7%), and 6 (12.5%) underwent bowel resection. Based on a receiver operating curve analysis of bowel resection, the cut-off value of the body weight at birth and at operation were 1984 g [area under the curve (AUC) 0.75, 95% confidence interval (CI) 0.52-0.99] and 2418 g (AUC 0.70, 95% CI 0.46-0.94), respectively. The cut-off value of intestinal torsion was 540° (AUC 0.76, 95% CI 0.57-0.95), and that of the time from the onset to the diagnosis was 12 h (AUC 0.85, 95% CI 0.72-0.98). For midgut volvulus with an intestinal torsion > 540°, the most sensitive preoperative imaging test was ultrasonography (75%) Patients with bloody stool tended to undergo bowel resection. CONCLUSIONS: Patients with a low body weight and bloody stool should be confirmed to have whirlpool sign by ultrasonography and scheduled for surgery as soon as possible.

DOI： 10.1007/s00383-023-05406-0

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: This study was performed to clarify the clinical features of cloacal exstrophy (CE) patients who underwent anoplasty and their functional outcomes based on a nationwide survey in Japan. METHODS: A questionnaire survey was conducted and data were obtained from 229 CE patients. After the exclusion of non-surviving patients and unknown data, 195 patients were enrolled. We compared the patient characteristics of the no anoplasty group (NAP group) to the anoplasty group (AP group). The defecation function of patients who underwent anoplasty was evaluated. RESULTS: The AP group had significantly lower rates of omphalocele (p = 0.045) and spinal defects (p = 0.003) than the NAP group. Of the 195 patients enrolled in this study, only 17 (8.7%) underwent anoplasty. Of the 17 patients who underwent anoplasty, 7 (41.1%) eventually had a permanent stoma after anoplasty [after ileostomy: n = 3 (60.0%), after colostomy: n = 4 (44.4%)]. Regarding soiling, 4 patients (66.7%) showed the absence of soiling, 2 (33.3%) showed accidental soiling, and no patients showed frequent soiling. CONCLUSION: In our study, spinal defects may have affected the determination of anoplasty. Anoplasty may improve the quality of life, because defecation can be kept clean, but indications, including colon function, may be carefully considered.

DOI： 10.1007/s00383-023-05403-3

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: Our previous clinical pilot study reported that miconazole (MCZ) prevented morbidity from surgical necrotizing enterocolitis (NEC). The present study re-investigated this effect in a long-term cohort over 20 years. METHODS: We conducted a retrospective cohort study from April 1998 to March 2020. A total of 1169 extremely low-birth-weight infants (ELBWIs) admitted to our neonatal intensive care unit, including 45 with NEC (3.8%), underwent surgery. Since 2002, protocol MCZ administration for 3 weeks has been applied for neonates born before 26 weeks' gestation or weighing under 1000 g. We compared the background characteristics and clinical outcomes between patients with and without MCZ administration. RESULTS: The morbidity rate decreased after applying the MCZ protocol, but no improvement in mortality was seen. A propensity score-matched analysis indicated that treated patients by MCZ showed a delay in developing surgical NEC by 12 days. The MCZ protocol also helped increase body weight at surgery. Prophylactic MCZ administration did not improve the neurological development of the language-social and postural-motor domains in the surgical NEC patients. But cognitive-adaptive domain caught up by a chronological age of 3 years old. CONCLUSIONS: Revising the protocol to extend the dosing period may improve the outcomes of surgical NEC after the onset.

DOI： 10.1007/s00383-023-05390-5

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

AIM OF THE STUDY: We conducted a nationwide survey of persistent cloaca (PC) to determine its current status in Japan. This study clarifies the potential risk factors for defecation problems in patients with PC. METHODS: Patient information was obtained via questionnaire, and a total of 213 PC patients who responded to a questionnaire on defecation problems and their bowel functions were enrolled in this study. We evaluated the constipation, incontinence, and soiling as bowel functions. Univariate and multivariate analyses were performed using a logistic regression analysis to clarify the risk factors for defecation problems. RESULTS: Of 213 patients with PC, 55 (25.8%) had defecation problems. A multivariate logistic regression analysis showed that sacral agenesis, as an associated anomaly, was significantly associated with defecation problems (odds ratio [OR] 3.19, 95% confidence interval [CI] 1.11-9.16, p = 0.03). The other multivariate logistic regression analysis showed that the PC patients who underwent antegrade continence enema and regularly took laxatives after anorectoplasty had defecation problems (OR 12.4, 95% CI 2.35-65.6, p = 0.003, OR 2.84, 95% CI 1.24-6.55, p = 0.01). CONCLUSION: Sacral agenesis is the potential risk factor of defecation problems in the patients with PC who underwent anorectoplasty. Those patients require vigorous defecation management.

DOI： 10.1007/s00383-023-05385-2

PubMed

Language：English  

Intradiaphragmatic extralobar pulmonary sequestration (IDEPS) is considered to be very rare among extralobar pulmonary sequestration (EPS), and IDEPS combined with congenital diaphragmatic hernia (CDH) with sac is extremely rare. The patient was a neonatal girl. Left-sided CDH with a hernia sac was diagnosed based on computed tomography (CT). Thoracoscopic repair was planned for the 5th day after birth. Left CDH with sac was recognized on the posterolateral side and isolated EPS was recognized on the sac. The hernia sac, including the EPS, was resected, and the diaphragm defect was closed. The resected specimen consisted of fibrous connective tissue and pulmonary sequestration (PS) connected with the sac. It was diagnosed as the hybrid form, with features of both type 2 congenital pulmonary airway malformation (CPAM) and EPS. The postoperative course was uneventful. At 1 year and 9 mo after the operation, no recurrence of herniation or laterality of the diaphragm was recognized.

DOI： 10.1111/ases.13120

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1111/ped.15459

PubMed

Language：English  

DOI： 10.1111/ped.15579

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: The present study clarified the efficacy of repeating laparoscopic surgery training using a disease-specific simulator and investigated the clinical outcomes of laparoscopic surgery for congenital biliary dilatation (CBD) in pediatric patients after training. METHODS: A high-fidelity laparoscopic hepaticojejunostomy simulator was used. Four pediatric surgeons performed practice laparoscopic hepaticojejunostomy three times using the simulator. The details of forceps manipulation during the task were analyzed. The clinical outcomes of 13 CBD cases treated with laparoscopic surgery in our institution were also evaluated based on medical records. RESULTS: The time required to complete the task became significantly shorter each successive time (1st: 1062.18 ± 346.79 s vs. 3rd: 717.44 ± 260.80 s, p = 0.039). There were no significant differences in the total path length of the right forceps (1st: 55.56 ± 23.21 m vs. 3rd: 28.25 ± 17.01 m, p = 0.17), total path length of the left forceps (1st: 47.79 ± 20.79 m vs. 3rd: 31.83 ± 17.62 m, p = 0.17), average velocity of the right forceps (1st: 58.78 ± 21.29 mm/s vs.44.98 ± 10.25 mm/s, p = 0.47), or the average velocity of the left forceps (1st: 50.39 ± 19.25 mm/s vs. 52.26 ± 19.59 mm/s, p = 0.78). Regarding the clinical outcome, all CBD patients underwent laparoscopic surgery performed by practiced pediatric surgeons who had no experience. The operative time was 545.53 ± 91.01 min, and the blood loss was 24.2 ± 25.8 ml. There were no cases of open conversion, intraoperative adverse events, or anastomotic leakage. CONCLUSION: Disease-specific simulator training significantly decreased the task performance time by improving the forceps manipulation economy. In addition, simulator training may improve the operative safety and quality of laparoscopic hepaticojejunostomy in pediatric CBD patients.

DOI： 10.1007/s11548-022-02793-y

PubMed

Language：English  

DOI： 10.1111/ped.15699

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: The present study attempts to clarify the clinical features of pediatric intra-abdominal solid organ injury at two institutions. METHODS: The injured organ, patient age, sex, injury grade, imaging findings, intervention, length of hospital stay, and complications were retrospectively reviewed using medical records at two centers from 2007 to 2021. RESULTS: There were 25 cases of liver injury, 9 of splenic injury, 8 of pancreatic injury, and 5 of renal injury. The mean age of all patients was 8.6±3.8 years old, with no difference between organ injury types. Radiological intervention was performed in four cases of liver injury (16.0%) and one case of splenic injury (11.1%), and surgery was performed in two cases of liver injury (8.0%) and three cases of pancreatic injury (37.5%). All other cases were treated conservatively. Complications included adhesive ileus in one case of liver injury (4.0%), splenic atrophy in one case of splenic injury (11.1%), pseudocysts in three cases of pancreatic injury (37.5%), atrophy of the pancreatic parenchyma in one case of pancreatic injury (12.5%), and urinoma in one case of renal injury (20.0%). No mortalities were observed. CONCLUSION: Pediatric patients with blunt trauma had favorable outcomes at two pediatric trauma centers covering a broad medical area, including remote islands.

DOI： 10.1136/wjps-2023-000560

PubMed

Language：English  

Near-infrared (NIR) fluorescence imaging with indocyanine green (ICG) has gained popularity in pediatric surgery as it has in general surgery. In addition, a water-jet dissector (WJD) has been successfully introduced in adult hepatic surgery. Tissue structures are dissected selectively and gently by the WJD. However, there have been no reports of hepatic resection for pediatric patients using a WJD. We applied NIR fluorescence imaging with ICG to visualize the resection line of the liver and used a WJD for liver parenchyma dissection in pediatric hepatoblastoma. The patient was a 3-year-old girl with a large liver tumor. Enhanced computed tomography revealed a liver tumor (maximum diameter: 120 mm) in the right lobe and three small lung metastases. The liver tumor was diagnosed as hepatoblastoma (PRETEXT 2) based on an open biopsy. We performed right hepatectomy after neoadjuvant chemotherapy. The right lobe was mobilized from the diaphragm, and then intraoperative ultrasound was performed to detect the localization of the tumor and its proximity to the vascular structures. We detected the right hepatic artery (RHA), right portal vein (RPV), and right hepatic vein (RHV). The middle hepatic vein was not involved. After ligation of the RHA and RPV to selectively control the right lobe inflow, ICG was administered intravenously and observed by an NIR endoscope. The resection line was clearly visualized by overlaying images in comparison to conventional demarcation line detection. Then, we used a WJD to dissect the parenchyma. Small vessels were divided from parenchymal tissue and were clearly visible. We resected them after clamping with metal clips. Finally, the RHV was transected by a linear stapler, and right hepatectomy was completed with 25 ml of blood loss. There was no postoperative hemorrhage. We performed hepaticojejunostomy because of stricture of the common bile duct on postoperative day 302. The patient was discharged after adjuvant chemotherapy. NIR imaging clearly showed the resection line. The WJD automatically separated, and thus made visible, the more resistant duct and vessel structures from the parenchyma. The combined use of NIR imaging and WJD was useful for pediatric hepatectomy.

DOI： 10.3389/fped.2023.1221596

PubMed

Language：Japanese   Publisher：日本膵・胆管合流異常研究会  

DOI： 10.34410/jspbm.46.0_54

Language：Japanese   Publisher：一般社団法人 日本移植学会  

<p>【はじめに】自験腸管不全例から患者支援の課題を明らかにし、その対策について報告する。【対象と方法】３か月以上の中心静脈栄養(PN)を要した短腸症候群(SBS)、腸管蠕動障害(MD)症例の診療録を後方視検討した。【結果】SBS16例では、胆汁うっ滞性肝障害(直接ビリルビン＞2mg/dL)を来たした症例の生存率が有意に低く（2/6(33.3%) vs 8/10(80.0%)；p=0.03）、腸管連続性の確保(p=0.0047)とPN離脱(p=0.0067)が予後関連因子であった。MD14例では、50％に胆汁うっ滞性肝障害がみられ、生存時間解析では予後関連因子であった（p=0.005）。患者QOLを鑑みると１歳を目途に在宅移行が望ましく、外来では個々の病勢を把握の上、残存腸管を活用しながら肝を庇護し、家族背景や地域特性なども考慮に入れた継続的支援が求められている。管理栄養士、認定看護師、言語聴覚士、作業・理学療法士、訪問看護団、地域薬剤師、臨床心理士らと協調した多職種介入が不可欠で、シリコン製カテーテルの安定供給、在宅用シリンジポンプ、ω３系脂肪乳剤、鉄を含まない微量元素製剤などの製造販売承認が切望される。【まとめ】以上の経緯から鹿児島大学病院では今年5月に難治性腸疾患支援センターを発足させた。本邦で腸管リハビリテーションプログラムが広く導入され、非移植施設と移植施設の連携にも寄与することを期待する。</p>

DOI： 10.11386/jst.58.supplement_s163_1

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: Various prognostic predictors for biliary atresia (BA) have been identified. This study aimed to evaluate the serial changes in the preoperative and postoperative ubiquitous inflammatory biomarkers and their relationship with the outcomes in patients with BA. PATIENTS AND METHODS: Forty-three BA patients were retrospectively reviewed to investigate serial levels of ubiquitous inflammatory biomarkers, including C-reactive protein (CRP) and lymphocyte ratio, and outcomes. The patients with BA were divided based on their outcomes into two prognostic groups: the native liver survivor group (n = 30) and the survivors with living-donor liver transplant group (n = 13). RESULTS: The area under the receiver operating characteristic (ROC) curve analysis showed that a preoperative lymphocyte ratio of < 61% and CRP value > 0.1 mg/dl predicted a poor outcome. In the ROC curve analysis, the timing of reaching the cut-off value of CRP after Kasai portoenterostomy was postoperative day (POD) 57. The third postoperative week, which was the timing of the discontinuation of steroid therapy, was the branchpoint of inflammatory markers between the two prognostic groups. CONCLUSION: The POD 57 CRP level predicts the surgical outcome of Kasai portoenterostomy. The postoperative anti-inflammatory management of BA can be monitored by the ubiquitous inflammatory biomarkers CRP and the preoperative lymphocyte ratio.

DOI： 10.1007/s00383-022-05231-x

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: The intravenous administration of acetaminophen (IAA) has become standard postoperative analgesic management for pediatric surgery. However, the most effective methods of IAA for postoperative acute appendicitis are unclear. We evaluated the analgesic efficacy of scheduled IAA vs on-demand IAA for postoperative acute appendicitis. METHODS: Ninety-four patients who underwent laparoscopic appendectomy in our institution between January 2017 and December 2020 were enrolled. The patients were divided into two groups based on the postoperative pain control protocols. The scheduled IAA group (SA group, n = 42) was managed by scheduled IAA and additional on-demand use of pentazocine as rescue therapy. The on-demand IAA group (ODA group, n = 52) was managed by on-demand IAA as the first choice, with pentazocine as the second choice for pain control. The patients' background characteristics, operative results and postoperative outcomes were reviewed. RESULTS: The number of times pain complaints were made per patient per day (NPPD) on postoperative days (POD) 1 and 2 was significantly lower in the SA group than in the ODA group (POD 1; 1.12 ± 1.21 vs 2.62 ± 1.89, p < 0.01; POD 2; 0.45 ± 0.86 vs. 1.31 ± 1.69, p < 0.01). According to pathological findings, NPPD was lower in the SA group than in the ODA group for both phlegmonous appendicitis (0.71 ± 1.01 vs. 2.10 ± 2.13, P < .05) and gangrenous appendicitis (1.33 ± 1.50 vs 2.94 ± 1.68, P < .01). On POD 2, the incidence of gangrenous appendicitis was significantly lower in the SA group than in the ODA group (0.57 ± 0.93 vs 1.78 ± 1.86, P < .01). CONCLUSIONS: Scheduled IAA may have favorable efficacy for postoperative pain control after emergency surgery for acute appendicitis in pediatric patients.

DOI： 10.1111/ases.13068

PubMed

Language：English  

We herein report a 13-year-old boy with a chief complaint of abdominal pain and a palpable mass. Contrast-enhanced computed tomography (CT) scan showed an abdominal bulky tumor involving the ascending colon causing severe stenosis, with multiple abdominal lymph node metastases detected by positron emission tomography (PET)-CT. Laparoscopic radical resection with right hemicolectomy and lymph node dissection was planned. The bulky tumor was dissected from the retroperitoneum and resected en bloc with the right-side colon and omentum. The preoperatively detected metastatic lymph nodes were resected along with the tumor. A 6-cm longitudinal umbilical incision was made, and the huge tumor was removed, with functional end-to-end anastomosis performed for intestinal reconstruction. The pathological diagnosis was Burkitt-like lymphoma with 11q aberration. The postoperative course was uneventful. Laparoscopy-assisted extirpation is feasible for pediatric solid tumors involving other organs, but indications and procedures should be carefully determined based on preoperative imaging, intraoperative findings and surgeon's skills.

DOI： 10.1111/ases.13081

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

Laparoscopic choledochal cyst excision and hepaticojejunostomy is a representative surgical procedure requiring laparoscopic suturing including needle driving and knot tying. Laparoscopic needle driving and knot tying is a highly technically demanding skill, especially in small infants and children. We developed a laparoscopic hepaticojejunostomy simulator for training. This simulator was developed for a 1-year-old infant body size based on computed tomography data and reproduced a pneumoperitoneum condition based on 3D scanner data of the clinical situation. Surgeon in training (SITs) of our institution have to acquire practice of laparoscopic hepaticojejunostomy using a simulator before performing clinical cases. Thirteen cases of choledochal cyst have been managed at our institution since the introduction of practice using this simulator in 2016. Six SITs performed 13 cases of laparoscopic choledochal cyst excision and hepaticojejunostomy. There were no cases of anastomotic bile leakage. Laparoscopic disease-specific simulator practice is effective for the acquisition of secure hepaticojejunostomy skills for the treatment of choledochal cysts in children as off-the-job training.

DOI： 10.1111/ases.13083

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

INTRODUCTION: Management of neonates with long gap esophageal atresia (LGEA) is one of the most challenging situations facing pediatric surgeons. Delayed anastomosis after internal traction for esophageal lengthening was reported as a useful technique for long gap cases. Additionally, the use of near-infrared (NIR) fluorescence imaging with indocyanine green (ICG) has gained popularity in pediatric surgery, especially for blood perfusion validation. We report a novel technique for safe and secure anastomosis for LGEA in the neonatal period using internal traction and ICG-guided NIR fluorescence. PATIENT AND SURGICAL TECHNIQUE: A pregnant woman with polyhydramnios was admitted to the department of obstetrics in our hospital. At 29 weeks of gestation, ultrasound showed mild polyhydramnios and absence of the fetal stomach. A male neonate was born at 38 weeks of gestation with 21 trisomy. EA (Gross type A) was diagnosed based on an X-ray study that showed the absence of gastric bubble with a nasogastric tube showing the "coil-up" sign. Thoracoscopic internal traction and laparoscopic gastrostomy were performed on day 4 after birth. We confirmed the distance between the upper pouch and lower pouch on X-ray. On day 16 after birth, thoracoscopic anastomosis was performed. We successfully performed esophageal anastomosis without tearing the esophageal wall. Blood perfusion of the upper and lower pouch was validated after anastomosis using ICG-guided NIR fluorescence. CONCLUSION: Delayed anastomosis for LGEA in the neonatal period using internal traction and ICG-guided NIR fluorescence is safe and feasible.

DOI： 10.1111/ases.13098

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Reduction en masse (REM) is a rare condition following manual inguinal hernia (IH) reduction in which a hernia sac is reduced back into the preperitoneal space with a loop of the bowel incarcerated at the neck of the sac. It resembles successful manual reduction and may thus be overlooked easily. We herein report an infantile case of REM of an IH that was successfully treated laparoscopically. CASE PRESENTATION: A 10-month-old boy with a surgical history of bilateral open IH repair at 4 months old presented with a bulge in his left groin and vomiting. A left incarcerated recurrent IH was suspected, and manual reduction was performed. The hernia was apparently reduced successfully, but abdominal distention and vomiting persisted. He was admitted for further observation due to the symptoms. On day 2 after admission, abdominal X-ray showed extensive small bowel obstruction (SBO). Enhanced computed tomography (CT) revealed protrusion of the small bowel with a closed-loop in the left groin. A closed-loop SBO due to postoperative adhesion or an internal hernia was suspected. To assess the etiology of SBO, emergent laparoscopic exploration with hernia repair was planned. Laparoscopy revealed REM of the left incarcerated IH with a thickened peritoneum at the neck of the sac. Laparoscopic reduction was performed, and the incarcerated small bowel showed no signs of ischemia. The hernia sac was not associated with the previously ligated processes vaginalis, which had been closed by a previous Potts' procedure. It was located at the inside of the processes vaginalis. The sac was successfully closed by laparoscopic percutaneous extraperitoneal closure procedures, and iliopubic tract repair was also performed via the previous inguinal incision. The postoperative course was uneventful. CONCLUSION: Pediatric IH is due to the patent processes vaginalis, and REM is extremely rare. Laparoscopic surgery for REM is a relatively common and useful approach for the diagnosis and treatment of adults. In our infantile case, the laparoscopic approach was similarly effective for both investigating the cause of SBO and performing high ligation of the sac for this rare condition with IH.

DOI： 10.1186/s40792-022-01535-1

PubMed

Language：English  

DOI： 10.1136/archdischild-2021-321653

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: Representative neonatal surgical diseases are often complicated by congenital heart disease (CHD). We reviewed our decade of experience from the perspective of the prognosis and report on the management of infants with CHD. METHODS: Cases with and without CHD between 2011 and 2020 were retrospectively compared. Qualitative data were analyzed using a chi-square test with Yates' correction, and quantitative data were compared using Student's t-test. RESULTS: Of the 275 neonatal surgical cases, 36 had CHD (13.1%). Ventricular septal defect was the most common cardiac anomaly, followed by atrial septal defect. Esophageal atresia showed the highest complication rate of CHD (43.8%, 14/32) followed by duodenal atresia (38.5%, 10/26). The mortality rates of patients with and without CHD (22.2% [8/36] vs. 1.3% [3/239]) were significantly different (χ2 = 30.6, p < 0.0001). Of the eight deaths with CHD, six patients had cyanotic complex CHD. Notably, four of these patients died from progression of inappropriate hemodynamics in the remote period after definitive non-cardiac surgery. CONCLUSION: Considering its high-mortality, the presence of CHD, especially cyanotic heart disease, is an important issue to consider in the treatment of neonatal surgical diseases. Pediatric surgeons should be alert for changes in hemodynamics after surgery, as these may affect mortality.

DOI： 10.1007/s00383-022-05178-z

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: Short bowel syndrome (SBS) patients require total parenteral nutrition (TPN) following massive small bowel resection (SBR), which may cause intestinal failure-associated liver disease (IFALD), a life-threatening complication. Hepatocyte growth factor (HGF) acts as a potent hepatocyte mitogen with anti inflammatory and antioxidant actions. The present study evaluated the effect of recombinant human HGF (rh-HGF) on SBR and subsequent IFALD using a parentally fed rat model of SBS. METHODS: Rats underwent jugular vein catheterization for continuous TPN and 90% SBR. They were divided into 2 groups: TPN alone (SBS/TPN group: n = 7) or TPN plus the intravenous administration of rh-HGF (0.3 mg/kg/day) (SBS/TPN+HGF group: n = 7). On day 7, their tissues and stool were harvested to evaluate the effects of HGF. RESULTS: Regarding the histological findings, based on the nonalcoholic fatty liver disease (NAFLD) activity score, the SBS/TPN+HGF group showed significantly less hepatic steatosis and inflammatory cell infiltration than the SBS/TPN group (NAFLD activity score, 4.00 ± 1.83 vs. 1.00 ± 0.82; p < 0.01). The SBS/TPN+HGF group showed a higher expression of Farnesoid X receptor in the liver and lower expression of Toll-like receptor 4 in the ileum than the SBS/TPN group. Regarding the composition of the bacterial gut microbiota, Actinobacteria, Bacteroidetes and Proteobacteria were decreased in the SBS/TPN+HGF group compared with the SBS/TPN group. CONCLUSION: In our SBS with TPN rat model, rh-HGF administration had a preventive effect against hepatic steatosis and dysbiosis. rh-HGF may therefore be a potentially effective therapeutic agent for SBS and subsequent IFALD. TYPE OF STUDY: Experimental research.

DOI： 10.1016/j.jpedsurg.2022.02.030

PubMed

Language：Japanese   Publisher：(株)東京医学社  

対象は1984年～2015年に肛門形成術を施行した中間位および高位鎖肛の男児64例。仙骨会陰式鎖肛根治術(SP法)は2007年までの52例に、modified PSARP(m-PSARP)法は2007年以降の12例に施行された。肛門形成時月齢はSP法6.5ヵ月、m-PSARP法5.2ヵ月であった。術後排便機能臨床的評価基準の便意・便秘・失禁・汚染の合計点は、両群においてゆっくりと改善し10歳以降で6点以上となった。m-PSARP群は、便秘と失禁のスコアは7歳以降でSP群より高値を推移し、汚染スコアはSP群より低値で推移した。m-PSARP法が術後排便機能の観点では優位な結果であった。

Publishing type：Research paper (scientific journal)   Publisher：Egypts Presidential Specialized Council for Education and Scientific Research  

Abstract

Background

The mortality rate of esophageal atresia (EA) has significantly improved, but late complications remain problematic. We evaluated the physical growth, late complications, and social prognosis of postoperative patients with EA who have reached 15 years of age.

Methods

EA patients who were treated at our institution from 1984 to 2003 were enrolled. The follow-up, physical growth at the last visit, late complications and treatment, academic status, and employment situation were evaluated.

Results

Twenty-nine EA patients were registered, and the 23 surviving patients (79.3%) were followed. Anthropometry at the latest visit tended to be below the standard values. Fundoplication was performed in 6 (26.1%) of 9 (39.1%) patients with gastroesophageal reflux. Anastomotic stenosis was found in 12 patients (52.2%), and 2 (8.7%) were treated with re-anastomosis. Thirteen patients were attending a regular school, and one was attending a school for disabled children. Four had jobs from 18 years of age. Follow-up was aborted during early childhood in nine patients.

Conclusions

The physical size of EA was smaller than in the healthy population of the same age. Late complications had not affected the physical growth but were sometimes recognized in adolescence. The social prognosis of the patients was largely favorable.

DOI： 10.1186/s43159-022-00185-0

Other Link： https://link.springer.com/article/10.1186/s43159-022-00185-0/fulltext.html

Language：Japanese   Publisher：(株)東京医学社  

過去5年間に当施設で入院診療を行った重症心身障碍児82人(男性56人、女性26人、平均年齢19.8歳)を対象として、便秘や呑気などの臨床症状と、処方されていた薬を後方視的に分析した。手術は95件行われており、噴門形成術、胃瘻造設術、気管切開術などの予定手術が81件、イレウス解除術などの緊急手術が14件であった。術前の栄養形態は半消化態栄養剤が67人で最も多く、成分栄養剤7人、消化態栄養剤1人、ミルク6人で経口摂取は1人のみであった。術前薬については漢方製剤を20人、刺激性下剤を20人、整腸剤を19人、塩類下剤を15人、浸透圧下剤を1人が使用していた。浣腸のみを含めて無投薬は33人であった。術前の腹部単純X線で宿便を認めたのは5人のみであったが、広範な小腸ガス貯留など呑気による影響と考えられる所見は44人で認められた。呑気症を背景にイレウスを繰り返した2症例を提示した。

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Enteric duplication is a congenital disease that occurs throughout the entire gastrointestinal tract. Although it may sometimes cause intestinal volvulus, a few reports have described cases of enteric duplication twisted on itself. We experienced a rare pediatric case of long-segment tubular ileal duplication showing torsion. Torsion of enteric duplication is extremely rare. We herein report a pediatric case showing unusual torsion of ileal duplication requiring emergency surgery. CASE PRESENTATION: A 3-year-old boy presented with abdominal pain and vomiting. Contrast-enhanced computed tomography (CT) revealed a cystic luminal structure with a blind end and fluid collection in the pelvic cavity. CT also showed no findings of ileus or intestinal dilatation except for a cystic luminal structure. The preoperative diagnosis was torsion of Meckel's diverticulum. The patient underwent emergent explorative diagnostic laparoscopy. As a result, a necrotic luminal structure and bloody ascites were recognized, and small-scale laparotomy was performed. Long-segment ileal duplication was recognized. The long-segment tubular ileal duplication shared the anti-mesenteric side of the intestinal wall along one-third of its length. The residual two-thirds of its length was free from the ileum and its blind end was twisted in a manner that looked similar to Meckel's diverticulum. Normal ileum and the duplication, including the twisted necrotic portion, were resected, and ileal anastomosis was performed. The postoperative course was uneventful. A pathological examination confirmed the definitive diagnosis of enteric duplication. CONCLUSIONS: We reported the unusual presentation of intestinal duplication mimicking torsion of Meckel's diverticulum. Enteric duplication shows various clinical symptoms and presentations. We must understand that the classification of digestive enteric duplication is diverse with a variety of associated clinical symptoms.

DOI： 10.1186/s40792-022-01409-6

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: In this study, we used a near-infrared ray catheter (NIRC) to visualize the urethral line. We herein report our intraoperative visualization technique of the urethra using an illuminating catheter in laparoscopy-assisted anorectoplasty (LAARP) for imperforated anus. PATIENT AND SURGICAL TECHNIQUE: A 3.0-kg term male neonate with anorectal malformation was delivered. An invertogram revealed the type as intermediate. Transverse colostomy was performed at the left upper abdomen. A recto-bulbar urethral fistula (RBUF) was diagnosed via distal colostogram and voiding cystourethrogram. LAARP was planned at 6 months of age. We performed the operation with four trocars. A 45° 5-mm scope was used to clearly view the deep pelvic space. Before starting rectal dissection, a 6-Fr pig-tail-type NIRC was inserted through the external opening of the urethra to visualize the urethra during the laparoscopic procedure. The catheter tip was placed in the bladder, and excretion of urine was maintained through the NIRC during the procedures. While dissecting the deep pelvic space between the posterior wall of the urethra and anterior wall of the rectum, the exact line of the urethra was clearly confirmed by overlay images of the NIRC. The RBUF was dissected safely using this innovative image-guided technique. Anoplasty was performed between the rectal stump and perineal skin. The postoperative course was uneventful. Oral intake was started on postoperative day 1. Postoperative dynamic urography showed no complications. CONCLUSION: An NIRC is useful for detecting the urethra during LAARP.

DOI： 10.1111/ases.13061

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

Background and Aim: The distribution of board-certified pediatric surgeons (BCPSs) in Japan is highly biased. While Prefecture M has one of the smallest numbers of BCPSs per pediatric population, neighboring Prefecture K has one of the largest numbers of BCPSs per pediatric population. We examined the effect of BCPSs population on laparoscopic surgery and postoperative management and outcomes. Materials and Methods: We compared postoperative duration to full-dose enteral nutrition, postoperative hospital stay, and complications of neurologically impaired patients who underwent laparoscopic fundoplication in two prefectures from 2006 to 2019. Results: Laparoscopic fundoplication was performed in 17 patients in Prefecture M and 63 in K. The mean operative time was 248.8 ± 79.9 minutes in Prefecture M and 260.8 ± 94.8 in K (P = .64). The median number of days to full-dose enteral nutrition was 11.5 in Prefecture M and 10 in K (P = .29). The median postoperative hospital stay was 14 days in Prefecture M and 15 days in K (P = .38). Postoperative complications occurred in 7 cases in Prefecture M and in 10 in K. The incidence was significantly higher in Prefecture M than in K (P = .041). Conclusion: Areas with insufficient numbers of BCPSs have a higher risk of complications in laparoscopic surgery than areas with sufficient numbers.

DOI： 10.1089/lap.2021.0713

PubMed

Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p>We treated two cases of testicular yolk sac tumor (YST) in infants, who underwent inguinal high orchiectomy combined with a trans-scrotal approach. [Case 1] A 20-month-old boy presented with swelling of his left scrotum and an elevated serum α-fetoprotein (AFP) level of 1,593 ng/ml. Testicular tumor was suspected, and inguinal radical high orchidectomy combined with a trans-scrotal approach was performed. The removed testis was 26 × 23 mm in size. The normal testicular tissue had been replaced by a simple solid tumor. The pathological diagnosis was YST limited to the testis pathologically. [Case 2] A 20-month-old boy presented with a left scrotum mass and elevated serum AFP level of 668 ng/ml. Testicular tumor was suspected, and inguinal radical high orchidectomy combined with a trans-scrotal approach was performed. The removed testis was 35 × 25 mm in size. The tumor had a multinodular component, and normal testicular tissue was partially recognized. The pathological diagnosis was YST limited to the testis. [Conclusion] In both cases, the tumors were completely resected without rupture or tumor spillage, leading to a diagnosis of stage I germ cell tumor (GCT). Basically, stage I testicular GCT requires no additional treatment and shows a good prognosis, but operative procedures sometimes affect both the postoperative diagnosis and prognosis. Radical orchiectomy with a trans-scrotal approach may be effective to ensure a good patient outcome.</p>

DOI： 10.11164/jjsps.58.1_29

Language：English  

DOI： 10.1016/j.jpedsurg.2021.08.025

PubMed

Language：Japanese   Publisher：(一社)日本小児外科学会  

今回、鼠径部アプローチに経陰嚢操作を加えて高位精巣摘除術(本法)を行った精巣原発卵黄嚢腫瘍の2幼児例を経験した。【症例1】1歳8ヵ月男児。左陰嚢腫大とAFPの上昇(1,593ng/ml)を認め、精巣腫瘍を疑い、本法を行った。精巣は26×23mmと腫大し、内部は充実性で単一の腫瘍であった。病理では卵黄嚢腫瘍の診断で精巣内に限局しており、病期I期の診断となった。【症例2】1歳8ヵ月男児。左陰嚢腫大とAFPの上昇(668ng/ml)を認め、精巣腫瘍を疑い、本法を行った。精巣は35×25mmと腫大し、内部は多結節性で正常精巣組織を一部に認めた。病理では卵黄嚢腫瘍の診断で精巣内に限局しており、病期I期の診断となった。【結語】病期I期の卵黄嚢腫瘍は予後良好であるが、術中操作が術後診断や予後に影響を与える可能性がある。本法を行うことで、より適切な手術操作を行うことが可能であると考えられた。(著者抄録)

Language：English   Publishing type：Research paper (scientific journal)  

Severely neurologically impaired patients often require the creation of a gastrostomy when they develop difficulty with oral intake followed by antireflux surgery. We use a traction technique for laparoscopic fundoplication without removing the gastrostomy in such cases. Right lateral traction of the round ligament of the liver and left lateral traction of the stomach body are performed using a needle device. The left liver lobe is elevated with a liver retractor inserted via the epigastrium. This traction technique allows a sufficient surgical field for manipulating forceps to be created in cases with a gastrostomy, enabling fundoplication to be performed safely without removing the gastrostomy.

DOI： 10.1111/ases.13028

PubMed

Language：English  

We herein report a 20-month-old boy who underwent laparoscopic Ladd's surgery. He showed intermittent bilious vomiting after birth and received a definitive diagnosis of intestinal malrotation. Laparoscopic Ladd's procedure was performed with four trocars. Midgut volvulus (180° counterclockwise) was resolved by retrogradely tracing the small intestine from the ileocecal region. After dividing Ladd's ligament, fluorescent navigation with indocyanine green was used. We decided where to dissect the mesenteries by confirming the vascular perfusion through clamping the tissue. Fibrous adhesions were gradually dissected, and it was confirmed that the mesenteric root was sufficiently broadening. Surgeons hesitate to dissect the mesenteric root in laparoscopic procedures compared with open procedures because of the narrow operative view and the risk of vascular injury. Laparoscopic Ladd's procedure using indocyanine green to confirm intestinal blood flow is safe and useful for the small working space in pediatric patients with intestinal malrotation and midgut volvulus.

DOI： 10.1111/ases.13026

PubMed

Language：English  

The usage of near-infrared (NIR) fluorescence imaging with indocyanine green (ICG) has gained popularity in many procedures in pediatric surgery. ICG generates fluorescent light only when it combines with a protein. We herein report a novel technique for detecting pancreaticobiliary maljunction (PBMJ) with co-injection of bile and ICG in laparoscopic choledochal cyst resection and hepaticojejunostomy for a pediatric patient. A 4-year-old girl presented with abdominal pain and intermittent vomiting. Enhanced computed tomography and magnetic resonance cholangiopancreatography showed a 17-mm type Ia choledochal cyst. Definitive PBMJ was not detected preoperatively. Laparoscopic choledochal cyst resection and hepaticojejunostomy were performed using five ports. A percutaneous silicon catheter was inserted into the gallbladder, and bile juice was aspirated. The amylase level of the bile juice was over 3 × 105 IU/L. The aspirated bile juice and ICG were mixed and co-injected into the gallbladder through the catheter. ICG combined with protein in bile juice and generated fluorescent light. Dilated common bile duct and pancreas were detected by NIR fluorescence imaging. This imaging technique was helpful for detecting the dissection margin of the distal side of the choledochal cyst inside the pancreatic tissue and preventing injury of the pancreatic tissue. This is the first case of ICG application for laparoscopic choledochal cyst resection in a pediatric patient. After resection of the choledochal cyst, laparoscopic hepaticojejunostomy was completely performed. Our technique is a safe and low-invasive method of detecting and excising the distal side of the cyst without a risk of radiography and residual bile duct.

DOI： 10.1055/s-0042-1747913

PubMed

Language：English  

Laparoscopic surgery has been applied for Hirschsprung's disease (HD). We herein report our approach to mesenteric processing for laparoscopic-assisted transanal endorectal pull-through (L-TERPT). Following mucosectomy and entering the abdominal cavity, a vessel sealing system is transanally inserted into the abdominal cavity for mesenteric processing based on concept of Natural Orifice Translumenal Endoscopic Surgery. Since the transanal axis is parallel to the dissected mesentery, it makes easier to operate in comparison to when the procedure is performed through the abdominal working port and can reduce the additional abdominal trocar wound. We also use indocyanine green (ICG) fluorescence navigation. Fluorescing the vessels with ICG allows intraoperative visualization of the blood flow in the retrieved intestine. With these innovative combined techniques, L-TERPT for HD can be safely performed, even in infants with small intraabdominal cavities.

DOI： 10.1055/s-0042-1751051

PubMed

Language：English  

DOI： 10.1111/ped.15024

PubMed

Language：English  

INTRODUCTION: In some patients with congenital biliary dilation (CBD), biliary cirrhosis has been reported to rapidly progress from the neonatal period to the infantile period. We herein report an infantile case of CBD that showed severe biliary cirrhosis at the diagnosis, which was treated by laparoscopic surgery. CASE PRESENTATION: A 16-month-old girl underwent conservative therapy for liver dysfunction and cholangitis on a remote island of our prefecture. She was transferred to our hospital after the detection of a huge dilated common bile duct on imaging at the previous hospital. Contrast-enhanced computed tomography showed a dilated common bile duct (maximum diameter: 5 cm), thus suggesting CBD. However, her laboratory data on admission showed a poor nutritional status and severe liver dysfunction (Alb, 2.5 mg/dl; AST, 79 IU/L; ALT, 43 IU/L; γ-GTP, 491 mg/dl; D-bil, 0.3 mg/dl; CHE, 90 IU/L; NH3, 123 μg/dl). We initially performed laparoscopic exploration and bile drainage via the gallbladder, noting severe hepatic fibrosis resembling end-stage liver cirrhosis. After placing a drainage tube in the gallbladder, cholangiography was performed. Cholangiography showed Todani type IVa CBD with pancreaticobiliary maljunction. Contrast agent flowing into the duodenum could not be confirmed. The patient received liver-supporting therapy and nutritional support for 7 weeks before definitive surgery. Following the improvement of the hepatic synthetic capacity (Alb, 4.0 mg/dl; AST, 82 IU/L; ALT, 78 IU/L; γ-GTP, 157 mg/dl; D-bil, 0.2 mg/dl; CHE, 232 IU/L; NH3, 75 μg/dl), we performed extrahepatic bile duct excision and hepaticojejunostomy laparoscopically. Laparoscopic surgery was successfully performed along with liver biopsy. Histopathologically, the liver specimen showed chronic hepatitis and fibrosis (F3A2). Biliary scintigraphy showed good bile excretion at postoperative day 15. The postoperative course uneventful, and the patient was discharged on the 23rd day after surgery. A needle liver biopsy six months later showed mild improvement of chronic hepatitis and fibrosis (F2-3A1). The patient was regularly followed at the outpatient clinic. CONCLUSIONS: Severe liver fibrosis was suspected to be continuous cholestasis of CBD after birth. CBD with severe liver fibrosis may avoid liver transplantation by two-stage surgery with hepatoprotection therapy and immunonutritional support.

DOI： 10.3389/fped.2022.1101000

PubMed

Language：Japanese   Publisher：The Japan Society of Computer Aided Surgery  

DOI： 10.5759/jscas.24.191

Language：English   Publishing type：Research paper (scientific journal)  

Ieiri and colleagues report an ingenious technique for performing ambidextrous suturing in laparoscopic hepaticojejunostomy for cases with small hepatic ducts. Surgeons who devote professional attention to minimally invasive surgery should take care to practice extensively with their non-dominant as well as dominant hand to improve the quality of laparoscopic surgery.

DOI： 10.1002/jhbp.1100

PubMed

Language：Japanese   Publisher：(一社)日本小児救急医学会  

【はじめに】女児会陰部外傷は頻度が少ない。加えて児の羞恥心や恐怖心から診察に協力が得られず、鎮静下診察や外科処置の判断に迷うことも多い。今回、女児会陰部外傷の臨床的特徴の検討を行った。【対象と方法】当院における過去20年間の女児会陰部外傷症例を対象とし、診療録をもとに後方視的検討を行った。【結果】女児会陰部外傷35例のうち16例(46%)で外科処置を行っていた。そのうち12例(34%)は鎮静下に診察・処置を行われていた。外科処置群は有意に創のサイズが大きく(外科処置群vs.保存的加療群=3.1±1.1vs0.73±0.4cm、p<0.01)、前庭部・会陰の受傷割合が高かった。【考察】女児会陰外傷の半数近くが外科処置を行っていた。本症において外科処置が必要な割合は比較的多く、確実な診察・処置のためにはためらわずに鎮静下に診療を行うべきと考えられた。(著者抄録)

Language：Japanese   Publisher：(一社)日本小児救急医学会  

【目的】小児外傷性肝損傷(以下:本症)の適切なフォローアップ方法を明らかにする。【方法】当科の20年間の本症について後方視的に検討した。【結果】対象は17例で、年齢6.9±3.9歳、受傷機転は全例が鈍的外傷で、日本外傷外科学会の臓器損傷分類は、Grade I 9例、III8例であった。治療法は、保存的治療が最も多く13例であった。入院日数は13.1±5.6日、フォローアップ期間は233.9±123.5日であった。血液生化学検査正常化まで23.5±20.9日、画像所見正常化まで105.6±86.7日を要した。仮性動脈瘤や胆管狭窄など重篤な合併症は認めなかった。Grade IとIIIでは検討項目に有意差を認めなかった。【結論】本症に対しては慎重な経過観察が求められ、入院中に重篤な合併症を検索する造影CT検査は積極的に行うべきであるが、正常化確認のための頻回な検査は不要である。(著者抄録)

Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p>The subglottic cyst is relatively rare. A boy born at 28 weeks of gestation had a birth weight of 1,202 g and required five days of endotracheal intubation during the neonatal period. At one year and two months of age, he developed a severe respiratory obstruction resulting in cardiopulmonary arrest, which required resuscitation. Flexible bronchoscopy under general anesthesia with a laryngeal mask revealed a small cyst on the right side of the subglottic space, but it was not considered to have been responsible for the critical airway obstruction. He showed frequent episodes of croup-like symptoms and was referred to us again at three years of age. Enhanced computed tomography showed a 5-mm cystic mass close to or inside the subglottic portion of the trachea, indicative of a subglottic cyst inside the trachea or a bronchogenic cyst compressing from the outside. We intended to excise the cyst via a cervical incision. As the cyst was not located outside the trachea, we performed meticulous unroofing of the subglottic cyst via laryngofissure with distal tracheotomy. During the follow-up period of one year, there was no recurrence of the cyst. However, another small cyst was found in the left subglottic area, but it did not increase in size during the subsequent six months of follow-up. One year and five months after tracheotomy, we cauterized the cyst using a diode laser and successfully decannulated the tracheostomy. We herein report our experience with the diagnosis and treatment of this patient with a subglottic cyst who had a somewhat unusual presentation of airway obstruction.</p>

DOI： 10.11164/jjsps.57.6_976

Language：Japanese   Publisher：(一社)日本小児外科学会  

症例は在胎36週6日、3,000gで出生した男児。日齢2に空腸閉鎖症の診断で一期的吻合を行った。術後2日目より経腸栄養を開始し、日齢15に中心静脈栄養から離脱したが、日齢17頃から灰白色便と直接ビリルビン優位の黄疸を認めたため利胆薬を開始した。日齢22に退院としたが、黄疸が持続したため精査したところ胆道閉鎖症を否定できなかった。日齢59に試験開腹を行い胆道閉鎖症(III-b1-ν)と診断し、肝門部空腸吻合術を施行した。術後は速やかに減黄し1歳の現在まで黄疸はなく経過良好である。小腸閉鎖症術後の胆汁うっ滞はしばしば経験するが、胆道閉鎖症との鑑別は困難であり、精査・治療介入が遅くなることが指摘されている。今回小腸閉鎖症に合併した胆道閉鎖症の本邦報告例および当科における小腸閉鎖症症例を検討した結果、生後1ヵ月過ぎても遷延する直接ビリルビン優位の黄疸は胆道閉鎖症を念頭に置いた精査が必要であると考えられた。(著者抄録)

Language：Japanese   Publisher：(一社)日本小児外科学会  

声門下嚢胞は比較的稀な疾患である。症例は在胎28週、1,202gで出生した男児。出生後5日間挿管された。1歳2ヵ月時に上気道炎、クループ症状から呼吸状態が増悪し前医に救急搬送され、心肺停止の状態となったが蘇生された。軟性気管支鏡検査では声門下腔右壁の軽度膨隆のみで、患児が呈した重篤な気道閉塞症状とは乖離しており、経過観察とされた。その後クループ症状が頻回となり、3歳で当科紹介受診となった。造影CTで声門下に5mm大の嚢胞性病変を認め、気管内外の嚢胞性病変を想定して、切除手術を施行した。病変は気管外にはなく、気管切開の上、声門下を開けると、嚢胞を確認、鋭的に嚢胞開窓術を施行した。半年後の気管支鏡検査では左側に微小な嚢胞を認めた。その後1年間の経過観察で増大なく、新たな再発病変はないため同部位にレーザー焼灼を施行し、気管切開後1年5ヵ月で気管切開カニューレを抜去した。診療に難渋し示唆に富む声門下嚢胞を経験したため、文献を踏まえて報告する。(著者抄録)

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Image-guided surgery with an open magnetic resonance imaging (MRI) system is applied for brain tumors in the neurosurgery field, but has rarely been reported in pediatric surgery. We report our initial experience of intraoperative confirmation of precision rectal pull-through during laparoscopically assisted anorectoplasty (LAARP) in an open MRI operating theater for pediatric patients with anorectal malformation (ARM). CASE PRESENTATION: A 3.0 kg term male neonate was delivered with anorectal malformation. An invertogram revealed the intermediate type. Transverse colostomy was made on the left upper abdomen. The recto-bulbar urethral fistula (RBUF) was diagnosed by a distal colostogram and voiding cystourethrogram. LAARP was planned at 6 months of age. Because this was the first procedure in which the pediatric abdomen had been scanned in an open MRI operating theater in our institution, we scanned his pelvic floor under sedation 3 weeks before the operation using the open MRI system in our operation room. We performed the operation with 4 trocars. The peritoneal reflection was carefully incised and the rectum was dissected. The RBUF was resected. The center of the muscle complex was detected at the perineal skin with an electrical nerve stimulator, and a 7-mm longitudinal skin incision was made on the perineal lesion for anoplasty. The muscle complex and the pubo-rectal sling were confirmed laparoscopically using a 3.5-mm bipolar forceps connected to the electrical nerve stimulator. Anoplasty was performed between the rectal stump and perineal skin. After anoplasty, the patient was scanned with open MRI under general anesthesia. We attached the quadrature-detection (QD) head coil around the patient's pelvis and inserted him in the gantry. A 0.45-T open MRI clearly revealed that the pulled through rectum was located in the center of the muscle complex on T2-weighted images. The postoperative course was uneventful. Oral intake was started on post-operative day 1. Postoperative dynamic urography showed no complication (e.g., leakage or residual fistula). CONCLUSIONS: We successfully performed LAARP for ARM, with intraoperative confirmation of precision rectal pull-through in an open MRI operating theater. Further cases are required to evaluate the application of open MRI systems in pediatric surgery.

DOI： 10.1186/s40792-021-01298-1

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Perivascular epithelioid cell neoplasm (PEComa) in a child is very rare. We herein report the first malignant case of PEComa developing in the liver of a pediatric patient. CASE PRESENTATION: A 10-year-old boy visited a private clinic with prolonged fever of unknown etiology. Abdominal ultrasonography was performed to evaluate the fever's origin, revealing a large tumor in the liver. He was thus referred to a nearby hospital to investigate the tumor further. Enhanced computed tomography (CT) showed a 6.8 × 5.9 × 10.5-cm solid lesion on S4 and S5. On magnetic resonance imaging (MRI), the tumor had a low signal intensity on T1 imaging and high signal intensity on T2 imaging, with partial diffusion restriction. 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) showed a marked uptake in the mass lesion with no evidence of metastasis. The patient was negative for all tumor markers, including AFP, CEA and PIVKA-II. The results of a needle biopsy suggested hepatocellular carcinoma. The tumor's rapid growth suggested malignancy. Hepatic segmentectomy (S4 + S5 + S8) was performed. The tumor was resected en bloc with a margin. Microscopically, the tumor showed atypical spindle, polygonal or oval-shaped cells with a high nuclear grade, and vascular invasion. Immunohistochemistry was positive for alpha-smooth muscle antigen (α-SMA), human melanin black-45 (HMB-45) and melan A. The pathological diagnosis was malignant PEComa. In the 6 months after surgery, the patient complained of shoulder pain. MRI showed a dumbbell-shaped tumor at the 2nd thoracic vertebrae, which was confirmed to be bone metastasis of PEComa. After chemotherapy, including ifosfamide and doxorubicin, vertebrectomy was performed. Two years later, thoracoabdominal CT showed a 10-cm solid mass occupying the pelvis and a 15-mm nodule in the middle lobe of the right lung. Under a diagnosis of peritoneal and lung metastases, they were surgically removed and metastasis of PEComa was pathologically confirmed. Four months after the 2nd relapse, pelvic metastasis appeared again and mTOR (mammalian target of rapamycin) inhibitor was initiated. To our knowledge, this is the first report of malignant hepatic PEComa in a pediatric patient. CONCLUSION: Although extremely rare, malignant hepatic PEComa can develop in a child.

DOI： 10.1186/s40792-021-01300-w

PubMed

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PURPOSE: Total parental nutrition (TPN) causes gastrointestinal mucosal atrophy. The present study investigated the effects of hepatocyte growth factor (HGF) on the intestinal mucosal atrophy induced by TPN. METHODS: Rats underwent jugular vein catheterization and were divided into four groups: oral feeding (OF), TPN alone (TPN), TPN plus low-dose HGF (0.3 mg/kg/day; TPNLH), and TPN plus high-dose HGF (1.0 mg/kg/day; TPNHH). On day 7, rats were euthanized, and the small intestine was harvested and evaluated histologically. The expression of c-MET, a receptor of HGF, and nutrition transporter protein were evaluated using quantitative polymerase chain reaction. RESULTS: The jejunal villus height (VH) and absorptive mucosal surface area in the TPNHH group were significantly higher than in the TPN group (p < 0.05). The VH in the ileum showed the same trend only in the TPNHH group, albeit without statistical significance. The crypt cell proliferation rate (CCPR) of the jejunum in both HGF-treated groups was significantly higher than in the TPN group (p < 0.01). The expression of c-MET and transporter protein in all TPN-treated groups was decreased compared with that in the OF group. CONCLUSION: HGF attenuated TPN-associated intestinal mucosal atrophy by increasing the villus height, which was associated with an increase in CCPR.

DOI： 10.1007/s00383-021-05002-0

PubMed

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PURPOSE: Few studies have focused on the operative age for Hirschsprung's disease (HD). We evaluated the optimal timing of surgery in HD patients based on their long-term bowel function. METHODS: HD was diagnosed in 65 pediatric patients in our institute between 1992 and 2018. Twenty-five patients underwent the Soave-Denda procedure (SD) and 40 underwent transanal endorectal pull-through (TA). We divided these patients into two groups: those who underwent surgery at < 6 months of age (younger group) and those who underwent surgery at 6-12 months of age (older group). We assessed bowel function at 5, 7, and 9 years of age. RESULTS: The bowel function of the patients who underwent the SD did not differ significantly between the groups. Similarly, the total bowel-function scores of the patients who underwent TA did not differ between the groups at any age. However, the soiling score at 7 years of age in the older group of patients who underwent TA was significantly lower than that in the younger group (p = 0.02). CONCLUSIONS: Our data suggest that to achieve optimal bowel function, TA should be performed at < 6 months of age.

DOI： 10.1007/s00595-021-02356-9

PubMed

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DOI： 10.1186/s40792-021-01245-0

PubMed

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PURPOSE: Retroperitoneal teratomas (RPTs) are rare in infants. We report our experience of treating pediatric patients with RPTs over many years at a single institution, with the aim of developing a safe and secure operative strategy for RPTs in infants. METHODS: We reviewed the medical records of patients who underwent treatment for RPTs in our institution between April, 1984 and December, 2017, to analyze their background and clinical data. The diagnosis of RPT was confirmed histologically in all patients. RESULTS: The subjects of this retrospective analysis were 14 pediatric patients (female, n = 11; male, n = 4), ranging in age from 6 days to 12 years, 11 (73.3%) of whom were under 1 year of age. Complete surgical resection was performed in all patients. The tumor ruptured during surgery in four (26.7%) patients and perioperative vessel injuries occurred in six (40.0%) patients, resulting in nephrectomy in one (6.7%). Three (20.0%) patients suffered unilateral renal dysfunction as a surgical complication. Only one patient received postoperative chemotherapy. All patients were free of disease at the time of writing. CONCLUSION: Perioperative complications are not uncommon during surgery for RPTs, despite their benign nature. Preoperative imaging evaluation is important and operative management may be challenging. Because of the favorable prognosis and the frequency of adverse events in surgery, partial resection or split excision is sometimes unavoidable. Meticulous follow-up for recurrence is required for such patients.

DOI： 10.1007/s00595-021-02327-0

PubMed

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BACKGROUND: Accessory liver lobe (ALL) is a rare liver malformation. An ALL develops due to malformation of the endodermal caudal foregut and segmentation of the hepatic bud in the third week of gestation. Most ALLs are asymptomatic and are detected incidentally during abdominal surgery. The incidence of ALL is < 1% in patients who undergo abdominal surgery. However, some ALLs twist and cause acute abdomen. We experienced a pediatric case of ALL torsion in a patient who underwent elective laparoscopic surgery. CASE PRESENTATION: The 5-year-old girl had a 3-month history of epigastralgia and vomiting, which occurred every 2 weeks. Abdominal ultrasonography with color Doppler imaging revealed an 11.8 × 13.6 mm nonvascular lesion with mixed echogenicity near the round ligament of the liver. Enhanced computed tomography confirmed a 14 × 16 × 20 mm low-attenuation mass surrounded by a hyperdense line and disproportionate fat stranding on the right side of the round ligament of the liver. There was no ascites or hemorrhage. These findings suggested an abscess of the round ligament of the liver. Her symptoms improved with the administration of oral antibiotics; thus, we planned to perform elective exploratory laparoscopy and subsequent resection. Two trocars (5 mm) were inserted through a multichannel port device at the umbilicus and one trocar (3 mm) was inserted at the right lateral abdomen. Upon observation of the abdominal cavity, the omentum was observed adhering to the round ligament of the liver. Macroscopic observation revealed no apparent mass lesions. We performed adhesiolysis of the omentum from the round ligament of the liver using a vessel sealing system. We performed resection at the site at which adhesion had formed between the round ligament of the liver with the surrounding tissue using a vessel sealing system and the resected specimen was extracted through the umbilical wound. The postoperative course was uneventful. A pathological examination revealed necrotic liver tissue. The resected tissue was founded to be an ALL with ischemic change. CONCLUSIONS: The recurrent abdominal pain was induced by torsion of the ALL. Pediatric surgeons should consider ALL torsion as a differential diagnosis for epigastralgia of unknown etiology.

DOI： 10.1186/s40792-021-01231-6

PubMed

DOI： doi.org/10.1111/ped.14550

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PURPOSE: Tunneled central venous catheter (tCVC) placement plays an important role in the management of pediatric patients. We adopted a real-time ultrasound (US)-guided supraclavicular approach to brachiocephalic vein cannulation. We evaluated the outcomes of tCVC placement via a US-guided supraclavicular approach. METHODS: A retrospective study was performed for patients who underwent US-guided central venous catheterization of the internal jugular vein (IJV group) and brachiocephalic vein (BCV group) in our institution. The background information and outcomes were reviewed using medical records. RESULTS: We evaluated 85 tCVC placements (IJV group: n = 59, BCV group: n = 26). Postoperative complications were recognized in 19 patients in the IJV group (catheter-related bloodstream infection (CRBSI), n = 14 (1.53 per 1000 catheter days); occlusion, n = 1 (1.7%, 1.09 per 1000 catheter days); accidental removal, n = 3 (5.2%, 0.33 per 1000 catheter days); and other, n = 1 (1.7%, 1.09 per 1000 catheter days)) and five patients in the BCV group (CRBSI, n = 2 (0.33 per 1000 catheter days); catheter damage, n = 1 (3.8%, 1.67 per 1000 catheter days); and accidental removal, n = 2 (7.7%, 0.33 per 1000 catheter days)). In the BCV group, despite that, the incidence of postoperative complications was lower (p = 0.205) and the period of placement was significantly longer in comparison to the IJV group (p = 0.024). CONCLUSION: US-guided placement of tunneled CVC though the BCV results in a low rate of postoperative complications despite longer CVC indwelling times compared to IJV insertion. Our results suggest that BCV insertion of tunneled CVC in children may offer advantages in terms of device performance and patient safety.

DOI： 10.1177/11297298211008084

PubMed

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PURPOSE: In general, patients with anorectal malformation (ARM) undergo surgical treatment at around 6 months of age in Japan. We aimed to clarify whether the timing of a definitive operation affects the long-term bowel function. METHODS: Patient data were collected from 1984 to 2007. Fifty-two male patients with high- and intermediate-type ARM were enrolled. Patients were classified into two groups based on their operative period: the early group (EG) underwent anorectoplasty at < 5 months of age (n = 22); the late group (LG) underwent anorectoplasty at ≥ 5 months (n = 30). The bowel function was evaluated at 3, 5, 7, 9, and 11 years of age using the evacuation score (ES) of the Japan Society of Anorectal Malformation Study Group. RESULTS: The total score and four functional outcomes improved chronologically with age. The constipation score in the EG showed significantly better improvement in comparison to the LG. The total score and the incontinence score in the EG were significantly higher than those in the LG at 11 years of age. CONCLUSION: The long-term bowel function in the EG was better than that in the LG. Anorectoplasty at an early age was important for achieving a better postoperative bowel function in ARM patients.

DOI： 10.1007/s00383-020-04842-6

PubMed

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PURPOSE: We sometimes experienced prolonged delayed gastric emptying (DGE) in neurologically impaired patients undergoing laparoscopic fundoplication and gastrostomy. We explored the difference in the DGE rate according to the preoperative stomach position. METHODS: Thirty-two neurological impaired patients who underwent laparoscopic fundoplication and gastrostomy between 2015 and 2019 were classified into two groups based on stomach position: non-elevated stomach position (NESP) and elevated stomach position (ESP). Patients' characteristics, operative result and postoperative gastric emptying were reviewed. RESULTS: The 11 ESP patients were significantly older than the 21 NESP patients (p < 0.05). The ESP patients had a significantly heavier body weight (BW) than the NESP patients (p < 0.05). The time taken (days) to reduce gastric drainage (days, below BW × 10 ml) in the NESP (4.3 ± 3.6) was significantly longer than that of ESP (1.3 ± 1.6) (p < 0.05). The time taken (days) to achieve full enteral nutrition in the NESP (14.1 ± 8.9) was significantly longer than that of the ESP patients (8.8 ± 3.1) (p < 0.05). CONCLUSION: Elevated stomach position (ESP) patients archived full enteral nutrition earlier than NESP patients after laparoscopic fundoplication and gastrostomy. Stomach position correction might, therefore, be involved in the incidence of DGE.

DOI： 10.1007/s00595-021-02274-w

PubMed

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Laparoscopically assisted anorectoplasty (LAARP) for recto-bulbar urethral fistula (RBUF) is not now a standard operation due to urethra injury risk and incomplete removal of fistula. Our approach is a novel and secure technique of trans-perineal transection using a 5-mm stapler for RBUF. Before performing LAARP, the orifice of RBUF was confirmed under flexible cystoscope inspection. Before transection of RBUF, the center of the muscle complex was detected at perineal skin. The muscle complex and the pubo-rectal sling were then also confirmed with electrical nerve stimulator under laparoscopic approach. A 5-mm trocar was inserted to pass through the center of the muscle complex from perineal incision of the neo-anus. RBUF was stapled and transected using a 5-mm stapler inserted from the neo-anus. The operator successfully confirmed complete adequate closure of RBUF under flexible cystoscope inspection. A 5-mm stapler was effective and useful for the transection of RBUF.

DOI： 10.1111/ases.12934

PubMed

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PURPOSE: Total parenteral nutrition (TPN) sometimes induces parenteral nutrition-associated liver disease (PNALD). Hepatocyte growth factor (HGF) acts as a potent hepatocyte mitogen anti-inflammatory and antioxidant actions. We aimed to evaluate the effect of HGF on PNALD in a rat model of TPN. METHODS: A catheter was placed in the right jugular vein for 7-day continuous TPN. All rats were divided into three groups: TPN alone (TPN group), TPN plus intravenous HGF at 0.3 mg/kg/day [TPN + HGF (low) group], and TPN plus HGF at 1.0 mg/kg/day [TPN + HGF (high) group]. On day 7, livers were harvested and the histology, inflammatory cytokines and apoptosis were evaluated. RESULTS: Histologically, lipid droplets were apparent in the TPN group, but decreased in the TPN + HGF (low) and TPN + HGF (high) groups. The histological nonalcoholic fatty liver disease activity scores in the TPN + HGF (low) and TPN + HGF (high) groups were significantly lower than that in the TPN group (p < 0.01). There were no significant differences in the inflammatory cytokine levels of the three groups. The caspase-9 expression levels in the TPN + HGF (low) and TPN + HGF (high) groups were significantly decreased in comparison to that in the control group (p < 0.05). CONCLUSION: The intravenous administration of HGF attenuated hepatic steatosis induced by 7-day TPN dose dependently.

DOI： 10.1007/s00383-020-04823-9

PubMed

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Laparoscopic choledochal cyst excision and hepaticojejunostomy for choledochal in children is performed only at particular institutions because of the technical difficulty. Although choledochal cyst excision and hepaticojejunostomy are feasible, the small working space of infants makes intraabdominal Roux-Y reconstruction technically challenging. A 2-month-old girl diagnosed with a choledochal cyst underwent complete laparoscopic choledochal cyst excision and hepaticojejunostomy, including intraabdominal Roux-Y reconstruction with a 5-mm stapler. After cyst excision, Roux-Y reconstruction was performed in the intraabdominal cavity. Jejunojejunostomy was performed in a side-to-side fashion using a 5-mm stapler, and the entry hole was closed laparoscopically by hand-sewing, along with the mesentery defect. All procedures were performed by laparoscopic surgery. The postoperative course was uneventful, and the patient showed a quick recovery. Using small-diameter instruments, completely laparoscopic procedures for choledochal cyst are feasible.

DOI： 10.1111/ases.12928

PubMed

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PURPOSE: Real-time ultrasound (RTUS)-guided central venipuncture using the short-axis approach is complicated and likely to result in losing sight of the needle tip. Therefore, we focused on the eye gaze in our evaluation of the differences in eye gaze between medical students and experienced participants using an eye tracking system. METHODS: Ten medical students (MS group), five residents (R group) and six pediatric surgeon fellows (F group) performed short-axis RTUS-guided venipuncture simulation using a modified vessel training system. The eye gaze was captured by the tracking system (Tobii Eye Tacker 4C) and recorded. The evaluation endpoints were the task completion time, total time and number of occurrences of the eye tracking marker outside US monitor and success rate of venipuncture. RESULT: There were no significant differences in the task completion time and total time of the tracking marker outside the US monitor. The number of occurrences of the eye tracking marker outside US monitor in the MS group was significantly higher than in the F group (MS group: 9.5 ± 3.4, R group: 6.0 ± 2.9, F group: 5.2 ± 1.6; p = 0.04). The success rate of venipuncture in the R group tended to be better than in the F group. CONCLUSION: More experienced operators let their eye fall outside the US monitor fewer times than less experienced ones. The eye gaze was associated with the success rate of RTUS-guided venipuncture. Repeated training while considering the eye gaze seems to be pivotal for mastering RTUS-guided venipuncture.

DOI： 10.1177/1129729820987362

PubMed

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BACKGROUND: Anaplastic large cell lymphoma (ALCL) is a CD30-positive T-cell lymphoma, which is a rare type of non-Hodgkin lymphoma. ALCL rarely presents in the gastrointestinal tract, and the esophageal involvement in of ALCL is extremely rare. CASE PRESENTATION: An 11-year-old boy who complained of abdominal pain and cough was diagnosed with ALK-positive ALCL on the basis of systemic lymphadenopathy findings and immunohistochemistry results of pleural effusion. Although remission was observed after chemotherapy at 5 months after diagnosis, dysphagia persisted, and esophagoscopy revealed a severe stricture in the middle thoracic esophagus. At 9 months after diagnosis, allogeneic bone marrow transplantation was performed to ensure that complete remission was maintained; however, dysphagia and saliva retention did not improve. Approximately 10 months after diagnosis, esophagoscopy revealed a blind end in the middle thoracic esophagus, similar to that in congenital esophageal atresia. Subsequently, we performed minimally invasive subtotal esophagectomy under thoracoscopy and laparoscopy and gastric conduit reconstruction via the retrosternal route more than 2 years after allogeneic bone marrow transplantation. The final pathological diagnosis was esophageal atresia with esophagitis, with no malignancy. During postoperative evaluation, the patient required swallowing training for a few months, although no major complications were noted. Oral intake was possible, and complete remission was maintained at 14 month post-surgery. CONCLUSIONS: Oncologists must consider the possibility of acquired esophageal cicatricial atresia as a complication during chemotherapy for ALCL. If esophageal obstruction or esophageal atresia occur and if remission is maintained, esophagectomy and esophageal reconstruction are useful treatment options for maintaining oral intake.

DOI： 10.1186/s40792-021-01108-8

PubMed

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PURPOSE: Composite lipid emulsion (CLE) has been used for intestinal failure-associated liver disease (IFALD) to compensate for the disadvantages of soybean oil lipid emulsion (SOLE) or fish oil lipid emulsion (FOLE). However, the influence of its administration is unclear. We evaluated the effects of these emulsions on IFALD using a rat model of the short-bowel syndrome. METHODS: We performed jugular vein catheterization and 90% small bowel resection in Sprague-Dawley rats and divided them into four groups: control (C group), regular chow with intravenous administration of saline; and total parenteral nutrition co-infused with SOLE (SOLE group), CLE (CLE group) or FOLE (FOLE group). RESULTS: Histologically, obvious hepatic steatosis was observed in the SOLE and CLE groups but not the FOLE group. The liver injury grade of the steatosis and ballooning in the FOLE group was significantly better than in the SOLE group (p < 0.05). The TNF-α levels in the liver in the FOLE group were significantly lower than in the SOLE group (p < 0.05). Essential fatty acid deficiency (EFAD) was not observed in any group. CONCLUSION: Fish oil lipid emulsion attenuated hepatic steatosis without EFAD, while CLE induced moderate hepatic steatosis. The administration of CLE requires careful observation to prevent PN-induced hepatic steatosis.

DOI： 10.1007/s00383-020-04802-0

PubMed

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Short-bowel syndrome (SBS) is defined as a state of malabsorption after resection or loss of a major portion of the bowel due to congenital or acquired factors. This article presents an overview on the recent management of pediatric SBS. The pediatric SBS population is very heterogeneous. The incidence of SBS is estimated to be 24.5 per 100,000 live births. The nutritional, medical, and surgical therapies available require a comprehensive evaluation. Thus, multidisciplinary intestinal rehabilitation programs (IRPs) are necessary for the management of these complex patients. The key points of focus in IRP management are hepato-protective strategies to minimize intestinal failure-associated liver disease; the aggressive prevention of catheter-related bloodstream infections; strategic nutritional supply to optimize the absorption of enteral calories; and the management and prevention of small bowel bacterial overgrowth, nephrocalcinosis, and metabolic bone disease. As the survival rate of children with SBS currently exceeds 90%, the application of small bowel transplantation has been evolving. The introduction of innovative treatments, such as combined therapy of intestinotrophic hormones, including glucagon-like peptide-2, may lead to further improvements in patients' quality of life.

DOI： 10.1007/s00595-020-02207-z

PubMed

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BACKGROUND: Langerhans cell histiocytosis (LCH) is an abnormal accumulation of Langerhans cells in various organs that sometimes induces organ dysfunction. LCH can affect the liver, resulting in sclerosing cholangitis and biliary cirrhosis. However, liver and bile duct involvement is usually observed in the disseminated form of LCH. We herein report a rare case of LCH localized only in the extrahepatic bile duct that resulted in severe liver cirrhosis. CASE PRESENTATION: A 3-year-old boy with elevated liver enzymes, obstructive jaundice, and dilation of the common bile duct was referred to our institution. Contrast-enhanced computed tomography showed atrophy of the right hepatic lobe, relative hypertrophy of the left hepatic lobe, choledocholiths, and biliary debris extensively with biliary duct dilation. Magnetic resonance cholangiopancreatography revealed dilation of the intrahepatic and extrahepatic bile ducts and multiple choleliths in the gallbladder and common bile duct. Laparoscopic cholecystectomy, intraoperative cholangiography, liver biopsy, and gastrointestinal fiberscopy were performed. A liver specimen showed severe biliary cirrhosis due to sclerosing cholangitis. The patient then underwent living-donor liver transplantation because of severe liver cirrhosis 3 months after the first surgery. The common bile duct was not suitable for duct-to-duct anastomosis and was resected because of severe inflammation. Histologic sections of the common bile duct showed histiocytic cell proliferation. Immunohistochemistry revealed histiocytoses that were positive for Langerin, S-100 protein, and CD1a. However, no histiocytic cell proliferation was noted in the liver tissue. The definitive diagnosis was LCH localized to the extrahepatic bile duct. LCH in the extrahepatic bile duct seemed to cause sclerosing cholangitis. The patient was discharged uneventfully 2 months after living-donor liver transplantation. CONCLUSIONS: LCH localized to the extrahepatic bile duct is extremely rare; however, LCH can still affect the extrahepatic bile ducts on occasion. LCH should be considered as a differential diagnosis if pediatric patients show the presence of sclerosing cholangitis.

DOI： 10.1186/s40792-020-00899-6

PubMed

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AIM OF THE STUDY: Liver retraction during laparoscopic fundoplication is important for obtaining an optimal space. Several methods have been developed, but the risks and benefits are unclear. We compared three different approaches and evaluated their safety and utility. METHODS: Forty-three neurologically impaired patients who underwent laparoscopic fundoplication between 2005 and 2018 were classified into three groups: A, snake retractor method, n = 18; B, crural suture method, n = 13; C, needle grasper method, n = 12. Patients' characteristics and outcomes were reviewed. MAIN RESULTS: The liver retraction time was significantly shorter in group C than in A or B (p < 0.05). The operative times were shorter in groups B and C than in A. There were no significant differences in the liver enzyme levels. The liver enzyme levels increased temporarily but improved within a week. The C-reactive protein levels were significantly lower in group B than in A or C (p < 0.05). CONCLUSIONS: The most convenient method was the needle grasper method, as the other two approaches create conflict with the operator's forceps. The crural suture method damages the liver less, but requires higher surgical skill levels. It is important to select the appropriate method according to the operator's skill and the patient's size and deformity.

DOI： 10.1007/s00383-020-04646-8

PubMed

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PURPOSE: Short bowel syndrome is associated with intestinal mucosal inflammation and microbial dysbiosis, leading to intractable complications. Partially hydrolyzed guar gum (PHGG) has trophic and anti-inflammatory effects on the intestine. We investigated whether PHGG ameliorates small intestinal mucosal damage and alters the intestinal microbiota using a rat small bowel resection (SBR) model. METHODS: Sprague Dawley rats were divided into sham operation (Sham), Sham/PHGG, SBR, and SBR/PHGG groups. On day 21, all rats were euthanized. To assess small intestinal mucosal damage, the degeneration rate was morphometrically evaluated and immunohistochemically examined using anti-CD45 antibodies. Analyses of fecal microbiota using 16S rRNA and short-chain fatty acid production were also performed. RESULTS: The mucosal degeneration rate was significantly higher in the SBR group than in the Sham or SBR/PHGG groups. The number of CD45-positive cells was significantly higher in the SBR group than in the Sham, Sham/PHGG, or SBR/PHGG groups. The relative abundance of family Lachnospiraceae was significantly higher in the SBR/PHGG group than in the SBR group. CONCLUSIONS: PHGG administration alleviated small intestinal mucosal damage which could be associated with modulation of the intestinal microbiota.

DOI： 10.1016/j.jpedsurg.2019.08.048

PubMed

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AIM OF THE STUDY: We previously showed an increased number of smaller portal vein (PV) branches in the portal areas of liver biopsy specimens of biliary atresia (BA) patients. We evaluated the correlation between this histopathological feature and the prognosis. PATIENTS AND METHODS: Twenty-five consecutive patients with BA encountered between 2000 and 2012 were classified into three prognostic groups based on their postoperative outcomes: Excellent (n = 11) for native-liver survivors with a normal liver function, Good (n = 6) for native-liver survivors with liver dysfunction, and Poor (n = 8) for survivors after liver transplant or on a waiting list. Data from morphometrical analyses, including the fibrotic portal area, numbers of PVs, diameter and total area of PV branches, were statistically compared among the three groups. MAIN RESULTS: The number of PV branches per unit area of the whole-liver specimen in the poor prognostic group was significantly lower than that in the excellent group (3.1 ± 0.6 vs. 5.2 ± 2.0/mm2, p = 0.03). There were no significant differences in the other parameters. CONCLUSIONS: This is the first report on the relationships between morphometrically analyzed PV branches and the postoperative course in BA patients. The portal venous system is involved as the primary lesion in BA.

DOI： 10.1007/s00383-019-04579-x

PubMed

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PURPOSE: Short bowel syndrome (SBS) patients require long-term parenteral nutrition following massive bowel resection, which causes intestinal failure-associated liver disease (IFALD). Previous reports have shown that glucagon-like peptide-2 (GLP-2) resulted in the bowel adaptation for SBS. The aim of this study was to evaluate the effect of GLP-2 for IFALD in a parenterally fed rat model. METHODS: Using rat, a catheter was placed in the jugular vein, and 90% small bowel resection (SBR) was performed. Animals were divided into three groups: SBR and total parenteral nutrition (TPN) (SBS/TPN group), SBR and TPN plus GLP-2 at 1 µg/kg/h [SBS/TPN/GLP-2 (low) group], and SBR and TPN plus GLP-2 at 10 µg/kg/h [SBS/TPN/GLP-2 (high) group]. On day 13, the liver was harvested and analyzed by using nonalcoholic fatty liver disease (NAFLD) score. RESULTS: Histologically, hepatic steatosis in the SBS/TPN group and SBS/TPN/GLP-2 (high) group was observed. Both steatosis and lobular inflammation score in the SBS/TPN/GLP-2 (low) group were significantly lower compared with those in the other two groups (p < 0.05). Active NAFLD score in the SBS/TPN/GLP-2 (low) group was significantly lower compared with that in the SBS/TPN/GLP-2 (high) group (p < 0.01). CONCLUSION: Low-dose GLP-2 intravenous administration improves hepatic steatosis of IFALD following in an SBS parenterally fed rat model.

DOI： 10.1007/s00383-019-04560-8

PubMed

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Background and Aim:
Training programs for developing laparoscopic suturing skills range from dry-box training to virtual simulator training. There are advantages and disadvantages to each training method; however, which training is best for medical students and young surgeons is unclear. The aim of this study was to compare the proficiency of medical students in acquiring laparoscopic suturing skills after various routes of short-term training: via a video, an expert teacher, or a virtual simulator.
Materials and Methods:
Seventeen medical students were registered and divided randomly into three groups: group receiving personal training while watching a training video (video group), group receiving training under the guidance of an expert (teaching group), and group receiving personal training with a virtual simulator (virtual group). The students practiced laparoscopic suturing and tying skills for 1 hour. Following their training, they performed the evaluation task of three sutures and ties using a laparoscopic fundoplication simulator. We developed a 1-year-old infant body model (body weight 10 kg) based on computed tomography data and established a pneumoperitoneum body model based on a clinical situation.
Results:
The path length of the assistant forceps in the virtual group tended to be longer than in the other groups. The average acceleration of the assistant forceps in the virtual group was faster than in the other groups (P = .04). There were no significant differences in the other evaluation parameters.
Conclusion:
A long-term and combination training study should be performed to develop the best method for training medical students and inexperienced young surgeons.

DOI： 10.1089/lap.2019.0212

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: This study assessed the impact of 2D and 3D environments by comparing pediatric surgeons (PS) and gastrointestinal surgeons (GIS) using a laparoscopic hepaticojejunostomy simulator. METHODS: We developed a high-fidelity simulator of laparoscopic hepaticojejunostomy. Thirty-five participants (19 PS and 16 GIS) performed hepaticojejunostomy in both 2D and 3D environments. We evaluated the required time, total path length, and average velocities of bilateral forceps in both situations using the para-axial port layout. RESULTS: Regarding the participants' characteristics, the performance history of laparoscopic hepaticojejunostomy differed significantly between PS and GIS. In PS, the 3D environment did not markedly affect compared with 2D. In GIS, however, the 3D environment affected the time and movement of the right forceps. There were no significant differences in the time between PS and GIS in either environment. In both environments, the right-hand movement of the PS group was shorter and slower than that of the GIS group, but the left-hand movement was the opposite. CONCLUSION: There were significant differences in forceps movement characteristics between the PS and GIS. The effects of a 3D environment could not be clarified in this study, because it may depend on the port layout used and the operative procedures.

DOI： 10.1007/s00383-019-04538-6

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

Purpose:
Various studies have shown the benefit of three-dimensional (3D) systems over two-dimensional (2D) systems in endoscopic surgery. However, few studies have focused on pediatric endosurgery. The purpose of this study was to assess the impact of 2D and 3D environments on the time taken and forceps manipulation by comparing experts and trainees using a laparoscopic hepaticojejunostomy simulator.
Methods:
We have developed a simulator of laparoscopic hepaticojejunostomy for congenital biliary dilatation. Seventeen participants of pediatric surgeons (4 experts and 13 trainees) performed hepaticojejunostomy using our simulator in both 2D and 3D environment. We evaluated the required time, total path length, and average velocities of bilateral forceps in both situations.
Results:
Obtained results show the findings for the required time (seconds; 2D, experts: 810.43 ± 321.64 vs. trainees: 1136.02 ± 409.96, P = .17) (seconds; 3D, experts: 660.21 ± 256.48 vs. trainees: 1017 ± 280.93, P = .039), total path length of right forceps (mm; 2D, experts: 38838.23 ± 30686.63 vs. trainees: 52005.47 ± 31675.02, P = .48)(mm; 3D, experts: 24443.09 ± 12316.32 vs. trainees: 45508.09 ± 26926.27, P = .16), total path length of left forceps (mm; 2D, experts: 131635.54 ± 71669.94 vs. trainees: 245242.48 ± 130940.25, P = .48)(mm; 3D, experts: 88520.42 ± 56766.63 vs. trainees: 230789.75 ± 107315.85, P = .024), average velocities of right forceps (mm/second; 2D, experts: 44.80 ± 21.85 vs. trainees: 44.37 ± 18.92, P = .97) (mm/second; 3D, experts: 37.28 ± 16.49 vs. trainees: 42.58 ± 14.40, P = .54), average velocities of left forceps (mm/second; 2D, experts: 156.65 ± 38.69 vs. trainees: 202.58 ± 63.80, P = .20) (mm/second; 3D, experts: 125.64 ± 44.30 vs. trainees: 219.42 ± 43.82, P < .01).
Conclusion:
The experts performed more effectively when using the 3D system. Using 3D, the total path length of the left forceps of expert pediatric surgeons was significantly shorter than trainee pediatric surgeons, and the average velocities of the left forceps tip of expert pediatric surgeons was significantly slower than trainee pediatric surgeons. These results suggest that training of assisting hand is necessary for advanced pediatric endosurgery to avoid organ injury.

DOI： 10.1089/lap.2019.0211

PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：BMJ Publishing Group  

Purpose: An early diagnosis of ovarian torsion is sometimes difficult due to variable clinical symptoms and non-specific imaging findings. We retrospectively reviewed patients with pediatric ovarian masses manifesting torsion. Methods: Fifty-eight ovarian masses (55 episodes) in 49 non-neonatal patients treated from April 1984 to March 2017 were retrospectively analyzed. The Mann-Whitney U test and Fisher's exact test were used for the statistical analysis. Results: The median age of these 55 episodes was 10.5 years old (range 1.0-23.0). Thirty-three patients presented with abdominal pain. Forty-five tumors and 13 cystic masses were resected and diagnosed pathologically (50 benign and 8 malignant). Torsion was identified in 15 cases (25.9%) at operation. The torsion masses were all benign, and 8 ovaries (53.3%) were successfully preserved. Comparing the torsion cases with the nontorsion cases, only the white cell count was significantly higher in the torsion cases (p=0.0133) and in the patients presented with abdominal pain (p=0.0068). The duration of abdominal pain was significantly shorter in ovary preserved cases than in oophorectomy cases. Conclusion: The white blood cell may be a helpful indicator of the presence of torsion as well as the need for surgery.

DOI： 10.1136/wjps-2018-000009

Scopus

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: Portal hypertension in patients with biliary atresia (BA) is generally thought to result from portal vein (PV) narrowing secondary to hepatic fibrosis. To test the hypothesis, we morphometrically analyzed the PVs and hepatic arteries (HAs). METHODS: Morphometrical analyses of 25 BA and 26 non-BA liver biopsy specimens from patients treated from 2000 to 2014. The total specimen area, the fibrotic portal area, vessel diameter and medial thickness of the HAs were measured. RESULTS: The PV diameter in BA patients was significantly smaller than that in non-BA patients. In BA, the numbers of normal-sized PVs and capillaries were decreased and increased, respectively. The PV diameter was not significantly correlated with the degree of fibrosis. We newly found that medial hypertrophy and the HA diameter increased with the number of endothelial cells in BA. The PV diameter was not significantly correlated with the medial thickness and was positively correlated with the HA diameter in BA. CONCLUSIONS: The narrowing of the PV is unlikely to occur secondarily to liver fibrosis. The medial hypertrophy of the HA is not correlated with the decrease in the PV blood flow. These findings seem to be unique to the primary vascular lesions of BA.

DOI： 10.1007/s00383-019-04459-4

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Single-incision laparoscopic surgery has emerged; however, the procedures might be complicated for trainees. We compared the clinical outcomes of conventional three-port laparoscopic appendectomy (CLA) and single-incision and one-puncture laparoscopic appendectomy (SIOPLA) by attending pediatric surgeons (APSs) and surgeons in training (SITs). MATERIALS AND METHODS: We reviewed the clinical outcomes of 72 randomized laparoscopic appendectomies that were consecutively performed by SITs and APSs for a 2-year period. The cases were categorized according to type of surgeon. Finally, 10 CLA and 18 SIOPLA procedures were performed by SITs, and 24 CLA and 20 SIOPLA procedures were performed by APSs. The operative time, blood loss, analgesic use, complications, and hospital stay were analyzed. RESULTS: There were no significant differences in any of the evaluation points between CLA and SIOPLA. CONCLUSIONS: SIOPLA is not inferior operation to CLA, and the postoperative outcomes of SIOPLA were satisfactory. Thus, SIOPLA was safe and feasible for young surgeons to perform.

DOI： 10.1089/lap.2018.0195

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Epignathus is a rare congenital orofacial teratoma infrequently associated with intracranial extension. Intracranial extension of an epignathus indicates a poor prognosis; however, only a small number of such cases have been reported. While there have been some studies reporting cases of epignathus expanding directly into the cranium, others have reported no communication between an epignathus and an intracranial tumor. CASE PRESENTATION: A fetus at gestational week 27 was suspected of having an epignathus with intracranial tumor as shown by ultrasonographic and magnetic resonance imaging. The fetus was stillborn and an autopsy was performed. An epignathus measuring 12 × 6 × 6 cm and weighing 270 g protruded from the mouth, with its base on the soft palate. An intracranial tumor weighing 14 g was located at the middle intracranial fossa and connected to the epignathus through the right side of the sella turcica. The intracranial tumor was encapsulated, and there was no invasion into the brain. Histologically, both the epignathus and intracranial tumor were immature teratomas, with neural and pulmonary components that were especially immature as compared to those of the internal organs and brain tissues of the fetus. CONCLUSION: There have been several reports of epignathus and intracranial tumors that did not communicate; therefore, careful evaluation is needed when a fetus is suspected of having an epignathus extending into an intracranial lesion. Our case supports the findings that an epignathus can directly expand into the cranium. Moreover, this is a rare case of an epignathus in which the intracranial lesion was encapsulated and did not invade the brain. These rare but important findings will provide additional, potential therapeutic strategies for gynecologists, neurosurgeons, and pathologists.

DOI： 10.1186/s13000-018-0776-y

PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Springer Tokyo  

PURPOSE: The late postoperative complications of choledochal cyst (CC) surgery are serious and include intrahepatic stones and biliary carcinoma; therefore, long-term follow-up is crucial. METHODS: The subjects of this retrospective study were patients who underwent surgery for CC at Kagoshima University Hospital between April, 1984 and December, 2016. We analyzed the operative results, early and late postoperative complications, and postoperative follow-up rate. RESULTS: The study population comprised 110 CC patients (male/female: 33/77) with a median age at surgery of 4 years, 3 months (range 12 days-17 years). The patients underwent hepaticoduodenostomy (n = 1; 0.9%) or hepaticojejunostomy (n = 109; 99.1%). Late complications included intrahepatic bile duct (IHBD) dilatation (n = 1; 0.9%), IHBD stones (n = 3; 2.7%), and adhesive ileus (n = 4; 3.6%). There was no incidence of biliary carcinoma in this series. The rates of follow-up at our institute within 10 years of surgery and more than 20 years after surgery were 69.2% (18 of 26) and 14.5% (8 of 55), respectively. CONCLUSIONS: The follow-up rate after definitive surgery declined with time. Late complications were observed within 20 years, but biliary carcinoma was not observed. The follow-up rate should be increased to detect late complications. Moreover, patient education on long-term follow up is essential to prevent life-threatening events after definitive surgery for CC.

DOI： 10.1007/s00595-018-1660-9

Scopus

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Since short bowel syndrome (SBS) patients face life-threatening conditions, the development of therapeutic strategies to induce intestinal adaptation has been investigated. Ghrelin, a ligand of growth hormone (GH) secretagogue-receptor that stimulates the release of GH and insulin like growth factor-1 (IGF-1), has several pleiotropic effects. We investigated whether ghrelin induces intestinal adaptation in parenterally fed rats with SBS. METHODS: Sprague-Dawley rats underwent venous catheterization and were divided into 3 groups: those receiving 90% small bowel resection while leaving the proximal jejunum and distal ileum (90% SBR) with TPN (SBS/TPN group), those receiving 90% SBR with TPN + ghrelin (SBS/TPN/ghrelin group), and those receiving sham operation and fed chow (sham group). Ghrelin was administered intravenously at 10 μg/kg/day. On Day 13, the rats were euthanized and the small intestine harvested, and the histology and crypt cell proliferation rates (CCPR), apoptosis, and nutrient transporter protein levels were analyzed and the plasma hormones were measured. RESULTS: The villus height and crypt depth of the ileum in the SBS/TPN/ghrelin group were significantly higher than in the SBS/TPN group. The CCPR of the jejunum and the ileum significantly increased by the administration of ghrelin; however, the apoptosis rates did not significantly differ between the SBS/TPN and SBS/TPN/ghrelin groups. Significant differences did not exist in the plasma IGF-1 and nutrient transporter protein levels among three groups. CONCLUSIONS: The intravenous administration of ghrelin stimulated the morphological intestinal adaptation of the ileum to a greater degree than the jejunum due to the direct effect of ghrelin.

DOI： 10.1016/j.peptides.2018.06.009

PubMed

Language：Japanese   Publisher：(一社)日本外科学会  

Language：English   Publisher：ELSEVIER SCIENCE INC  

Fibromuscular dysplasia (FMD) is an idiopathic, segmental, nonatherosclerotic, non-inflammatory vascular disease, which is often complicated by the occurrence of dissection. Although it is known to occur in all arteries, aortic involvement is relatively rare. To date, 33 cases of aortic FMD have been reported in available English literature, among which only three cases have been complicated by the occurrence of dissection. We describe the case of a 40-year-old woman diagnosed with aortic FMD complicated by the occurrence of a type A aortic dissection. Non-invasive imaging revealed an ascending to descending thoracic aneurysm measuring 8 cm in diameter associated with dissection. Histopathologically, a segment of the wall of the aneurysm showed architectural disorganization of the aortic wall with loss of elastic fibers, collagen deposition, and irregular proliferation of smooth muscle cells in the intima and media-features suggesting FMD. No atheromatous plaque or medial cystic degeneration was observed in the aorta. Although aortic FMD is sometimes fatal, it is often very difficult to diagnose using imaging techniques. Therefore, performing a histopathological diagnosis is very important and should be emphasized. (C) 2017 Published by Elsevier Inc.

DOI： 10.1016/j.carpath.2017.07.007

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PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：SPRINGER  

PURPOSE: We compared the characteristics and precision of right and left needle driving for right-handed pediatric surgeons using a laparoscopic diaphragmatic repair model. METHODS: Eighteen right-handed pediatric surgeons performed three needle driving maneuvers using both hands. We evaluated the required time and conducted an image analysis. The total path length, velocity, and acceleration of the needle driving were also evaluated. RESULTS: Obtained results show the findings for the required time (s, Rt 310.78 ± 148.93 vs. Lt 308.61 ± 122.53, p = 0.93), sum of needle driving balances (mm, Rt 5.23 ± 2.44 vs. Lt 5.05 ± 3.17, p = 0.83), the gap of the needle driving interval (Rt 1.2 ± 0.93 vs. Lt 2.17 ± 1.67, p = 0.04), total path length (mm, Rt 594.03 ± 205.29 vs. Lt 1641.07 ± 670.68, p < 0.01), and average velocity (mm/s, Rt 1.92 ± 0.54 vs. Lt 5.3 ± 1.39, p < 0.01). CONCLUSION: For right-handed pediatric surgeons, left needle driving showed almost same quality of right needle driving as regarding the precision. But left needle driving also showed too fast but not economical movement unfortunately, implying rough and risky forceps manipulation. Non-dominant hand training is necessary to avoid organ injury.

DOI： 10.1007/s00383-017-4144-x

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: Hirschsprung's disease (HSCR) is a congenital disorder of the enteric nervous system characterized by the absence of ganglion cells in the Auerbach's and Meissner's plexuses. Although about 7% of cases are hereditary, the causal mutations have not been completely characterized. We encountered a novel family with inherited HSCR and screened them for causal mutations. METHODS: A Japanese family of five female patients and six unaffected individuals was subjected to a whole-exome analysis with a next-generation sequencer. RESULTS: After exome sequencing and the annotation of mutations, we identified co-segregated mutations with sequential filtering steps via a standard protocol. Eight mutations were identified: two on chromosome 10 and six on chromosome 11. We used pathogenicity prediction tools such as Genomic Evolutionary Rate Profiling, SIFT, and PolyPhen2 to predict the impact of mutations on the protein activity. S922Y, a novel mutation of RET, was identified as a likely causal mutation. In addition, a mutation of rs2435357T, known as enhancer of RET located in intron 1 of RET, was detected in this family. CONCLUSION: The coexistence of RET mutations in both the exon (S922Y) and intron1 (rs2435357T) indicated a risk of HSCR in this family.

DOI： 10.1007/s00383-017-4134-z

PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：SPRINGER  

PURPOSE: Congenital esophageal stenosis (CES) is rare, and the available clinical data are limited. We explored the current diagnosis, treatment and outcomes of CES. METHODS: A questionnaire survey was performed using medical records at pediatric surgical centers in the Kyushu area. RESULTS: Over 10 years, 40 patients (24 males) had CES. The incidence of associated anomalies was 52.5% (21/40), and that of esophageal atresia was 20.0% (8/40). The mean age at the diagnosis was 12.0 months (range, 1 day-8.8 years). Seven (17.5%) patients were diagnosed in the neonatal period. Ten (25.0%) developed CES due to tracheobronchial remnants, 27 (67.5%) due to fibromuscular stenosis (FMS) and 1 (2.5%) due to membranous stenosis + FMS. Thirty-six (90.0%) were treated by balloon dilatation (mean, 3 times; range, 1-20). Perforation at dilatation occurred in 7 (17.5%) patients, and all were diagnosed with FMS. Eighteen (45.0%) patients underwent radical operation (3 primary, 15 secondary to dilatation). CONCLUSIONS: Our study clarified the characteristics and outcomes of CES, including neonatal diagnoses. CES occurred in 1 in every 33,000 births in the Kyushu area. Careful attention should be paid, even in cases of dilatation for FMS. CES requires long-term follow-up for symptom persistence after adequate and repeated treatment.

DOI： 10.1007/s00383-017-4133-0

Web of Science

PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：W B SAUNDERS CO-ELSEVIER INC  

BACKGROUND: We compared the long-term outcomes of the bowel function (BF) in Hirschsprung's disease (HD) patients who underwent transanal endorectal pull-through (TA) with those who underwent the Soave-Denda (SD) procedure. METHODS: Patient data were collected from 1984 to 2015 from 110 HD patients who underwent definitive diagnosis and operation. The follow-up data were analyzed retrospectively. BF was evaluated according to the evacuation score (ES) of the Japan Society of Anorectal Malformation Study Group at 3, 5, 7, 9, and 11years of age. RESULTS: The operative procedures performed were as follows: SD: 71 (64.5%), TA: 38 (34.5%), and Duhamel: 1 (1.0%). We compared SD with TA. The ES improved chronologically after both procedures, and patients achieved satisfactory results at least 10years after operation. There was no significant difference in the total ES at all ages. The incontinence and frequency of bowel movement scores of TA patients were significantly lower than those of SD patients at several points. CONCLUSION: TA is simple, less invasive, and adaptive for neonates and small infants. However, regarding the bowel function, TA is not always superior to SD. The surgical technique of TA must be improved according to a detailed understanding of the anatomy. LEVEL OF EVIDENCE: Retrospective study - level III.

DOI： 10.1016/j.jpedsurg.2016.09.029

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PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：W B SAUNDERS CO-ELSEVIER INC  

BACKGROUND/PURPOSE: Total parenteral nutrition (TPN) has been reported to be associated with mucosal atrophy of the small intestine. Ghrelin has hormonal, orexigenic, and metabolic activities. We investigated whether ghrelin improved intestinal mucosal atrophy using a TPN-supported rat model. METHODS: Rats underwent jugular vein catheterization and were divided into four groups: TPN alone (TPN), TPN plus low-dose ghrelin (TPNLG), TPN plus high-dose ghrelin (TPNHG), and oral feeding with normal chow (OF). Ghrelin was administered continuously at dosages of 10 or 50 μg/kg/day. On day 6 rats were euthanized, and the small intestine was harvested and divided into the jejunum and ileum. Then the villus height (VH) and crypt depth (CD) were evaluated. RESULTS: The jejunal and ileal VH and CD in the TPN group were significantly decreased compared with those in the OF group. TPNHG improved only VH of the jejunum. TPNLG improved VH and CD of the jejunum and CD of the ileum. The improvement of TPNLG was significantly stronger than that in CD of the jejunum and ileum. CONCLUSIONS: TPN was more strongly associated with mucosal atrophy in the jejunum than in the ileum. Low-dose intravenous administration of ghrelin improved TPN-associated intestinal mucosal atrophy more effectively than high-dose administration.

DOI： 10.1016/j.jpedsurg.2016.09.035

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PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：SPRINGER  

PURPOSE: Treatment for a large abdominal wall defect remains challenging. The aim of this study was to optimize tissue engineering therapy of muscle constructs using a rat model. METHODS: Experimental abdominal wall defects were created in Wister rats. The animal model was divided into three groups: collagen sponge (CS), hybrid scaffold (HS) and hybrid scaffold containing bone marrow liquid (HSBM). Hybrid scaffolds comprised collagen sponge and poly L-lactide (PLLA) sheets. Abdominal wall defects were covered by three kinds of sheets. Thereafter, the bone marrow liquid was spread onto the sheets. Rats were killed at 4, 8, and 16 weeks. Pathological examinations were performed using hematoxylin-eosin and desmin antibody staining. RESULTS: The CS group showed abdominal hernia, whereas the HS and HSBM groups did not. Vascular formation was confirmed in all groups. Muscle tissue was recognized at the marginal area of the sheet only in the HSBM group. CONCLUSION: The HS and HSBM groups show a greater intensity than the CS group. Muscle tissue regeneration is solely recognized in the HSBM group. Our experimental data suggest that the triad of scaffold, cell, and growth factor is fundamental for ideal biomaterials. The HSBM may be useful for reconstruction of abdominal wall defects.

DOI： 10.1007/s00383-016-3949-3

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PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：SPRINGER  

PURPOSE: Children with intestinal failure (IF) requiring central venous catheters (CVCs) often experience frequent catheter-related bloodstream infections (CRBSIs), which is a serious and life-threatening complication. To reduce the incidence of CRBSI, prophylactic ethanol lock therapy (ELT) was initiated. METHODS: Patients with IF received home parenteral nutrition via a silicone tunneled CVC. All of them had received therapeutic ELT from January 2009 (first period) and prophylactic ELT from December 2012 (second period). Prophylactic ELT refers to ethanol lock for 2 h during the monthly hospital visit. We compared the CRBSI rate and number of CVC replacements between both periods. RESULTS: Four patients received 19 CVCs for a total of 5623 catheter days. In the first period, there were 12 CRBSIs in 1823 catheter days (rate 6.77 per 1000 catheter days). In the second period, there were 9 CRBSIs in 3800 catheter days (rate 3.13 per 1000 catheter days). Overall, the rate of CVC replacement decreased from 4.92 to 1.72 per 1000 catheter days (p = 0.04). No adverse reactions were experienced during ethanol instillation. CONCLUSION: Monthly prophylactic ELT for IF patients is considered to be a safe and effective modality for reducing the replacement of CVCs due to CRBSIs.

DOI： 10.1007/s00383-016-3933-y

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PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：BioMed Central Ltd.  

Background: Albumin is considered a negative acute-phase protein because its concentration decreases during injury and sepsis. Hypoalbuminemia is a risk factor for mortality, postoperative complications, and prolonged hospital stay. The magnitude of the systemic inflammatory response during the perioperative period, as indicated by the acute-phase proteins - C-reactive protein (CRP) in particular -, may help identify the risk of postoperative infectious complication. The correlation between serum albumin and CRP with gastrointestinal cancer has been reported. However, it is unclear whether antecedent CRP could be utilized to predict future hypoalbuminemia in the perioperative period in colorectal surgery. The primary endpoint of this study was to reveal that antecedent CRP could be utilized to predict future hypoalbuminemia in the perioperative period of colorectal surgery. Methods: Thirty-seven patients who underwent elective open colorectal surgery were included in this study. Correlations between preoperative CRP and serum albumin on postoperative day (POD) 3, between preoperative CRP and serum albumin on POD 7 and between CRP on POD 3 and serum albumin on POD 7 were examined. Relationships between preoperative CRP and hypoalbuminemia on POD 3, between preoperative CRP and hypoalbuminemia on POD 7 and between CRP on POD 3 and hypoalbuminemia on POD 7 were examined by receiver operating characteristic analysis. Results: Three-quarters of patients were older than 65 years of age. Significant correlations were observed between preoperative CRP and serum albumin on POD 3 (p = 0.023), between preoperative CRP and serum albumin on POD 7 (p = 0.023) and between CRP on POD 3 and serum albumin on POD 7 (p &lt
0.001). The area under the receiver operating characteristic curve of CRP on POD 3 to development of hypoalbuminemia on POD 7 was 0.833 (95 % CI 0.679-0.987) with an optimal threshold of 12.43 mg/dL, sensitivity 75 % and specificity 80 %. Conclusions: The present study revealed that antecedent CRP was associated with future serum albumin. Additionally, CRP on POD 3 could be useful in predicting the development of hypoalbuminemia on POD 7. This result suggests that CRP on POD 3 may be a valuable indicator for early nutritional intervention.

DOI： 10.1186/s13104-015-1632-8

Scopus

PubMed

Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：Japanese Journal of Cancer and Chemotherapy Publishers Inc.  

A 55-year-old man with a positive fecal occult blood test visited our department, and after a thorough medical evaluation, was diagnosed with Stage IV Rs rectal cancer with marked para-aortic lymph node metastasis. In December 2007, the patient underwent low anterior rectal resection with D3 lymph node dissection, but the para-aortic lymph nodes were left. The metastatic lymph nodes showed a complete response(CR)to post-operative chemotherapy with FOLFOX, FOLFIRI, IRIS, and irinotecan+cetuximab, and the complete response was sustained for 18 months after surgery. Later, he developed Virchow's lymph node metastasis, which was also resected. At present, 5 years after the first surgery, the patient, whose chemotherapy has been discontinued, is alive without recurrence. It appears that using key drugs, such as 5-fluorouracil, leukovorin, oxaliplatin, irinotecan, and cetuximab, and performing aggressive salvage surgery for Virchow's lymph node recurrence, led to long-term recurrence-free survival.

Scopus

PubMed

Language：Japanese   Publisher：一般社団法人日本外科学会  

Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

DOI： 10.11164/jjsps.48.3_486

Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2012&ichushi_jid=J01113&link_issn=&doc_id=20120426230181&doc_link_id=%2Fes5shoge%2F2012%2F004803%2F181%2F0486-0486%26dl%3D0&url=http%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fes5shoge%2F2012%2F004803%2F181%2F0486-0486%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Language：English   Publisher：The Japanese Society of Pediatric Surgeons  

DOI： 10.11164/jjsps.48.3_437

Language：Japanese   Publisher：特定非営利活動法人 日本小児外科学会  

DOI： 10.11164/jjsps.48.4_798_2

Language：Japanese   Publisher：特定非営利活動法人 日本小児外科学会  

DOI： 10.11164/jjsps.47.7_1080_2

Language：Japanese   Publisher：(株)東京医学社  

Language：Japanese   Publisher：(株)東京医学社  

Language：Japanese   Publisher：(株)東京医学社  

Language：Japanese   Publisher：(株)東京医学社  

＜Key Points＞(1)H病の術後排便機能は良好とされてきたが,詳細な検索では3割程度の患児に術後の便秘症状がみられる。(2)多くは食事療法,整腸剤,緩下剤,漢方薬,浣腸などでコントロールされるが,重度になると腹部膨満,腸閉塞,腸炎を併発する。(3)幼児期の慢性便秘のなかにもH病患児が隠れていることがあるため,薬剤治療に抵抗性でコントロール不良の症例は,小児外科に紹介のうえ精査を依頼することが望ましい。(著者抄録)

Language：Japanese   Publisher：(株)東京医学社  

Language：Japanese   Publisher：金原出版(株)  

＜文献概要＞当科では先天性胆道拡張症に対し,2016年4月の保険収載後,腹腔鏡手術による肝外胆管切除・肝管空腸吻合術を導入している。小児患者では成人に比べ胆管が細いため,安全・確実な吻合には工夫を要する。また,患児の年齢や体格によっては術前に胆管や血管を詳細に検索することが難しいため,術中にインドシアニングリーン(indocyanine green;ICG)蛍光ナビゲーションによる脈管の可視化が有用である。乳児から成人の体格までカバーすることに加え,小児特有の細径胆管や破格を伴う症例に対する吻合の工夫について解説する。さらに当科では疾患型シミュレータを作成し,執刀医のトレーニングに使用しており,その有用性について客観的評価を行っているため,あわせて報告する。

Language：Japanese   Publisher：(一社)日本コンピュータ外科学会  

Language：Japanese   Publisher：(株)東京医学社  

Language：Japanese   Publisher：(株)東京医学社  

Language：Japanese   Publisher：(株)東京医学社  

Language：Japanese   Publisher：(株)東京医学社  

Language：Japanese   Publisher：(株)東京医学社  

Language：Japanese   Publisher：(株)東京医学社  

Language：English   Publishing type：Internal/External technical report, pre-print, etc.   Publisher：WILEY  

DOI： 10.1111/ped.14550

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Language：Japanese   Publisher：(株)東京医学社  

Language：Japanese   Publisher：日本小児呼吸器学会  

小児先天性嚢胞性肺疾患は肺実質内に先天性に気道以外に恒常的に嚢胞が存在する状態をいう。近年は出生前に胎児肺の異常として診断される症例も多く、生直後に呼吸器症状がない場合でも、90%以上の症例で幼児期までに反復する肺感染などを発症するため、乳児期、遅くも幼児期早期までに手術的に病変を切除すべきとされている。治療としては病変部の外科的切除が原則となるが、従来はそのほとんどの症例に対して開胸手術による切除が行われてきた。近年では内視鏡外科手術、つまり胸腔鏡手術で施行される症例が増えている。これは乳幼児・小児の狭小な胸腔内であっても拡大視効果が得られ精緻な手術が可能であること、また従来は成人用デバイスを流用した手術であったが、小児用の細径デバイスが開発され、新生児期であっても技術的に可能となったことが大きい要因である。出生前診断例の手術時期も含めて術後機能を考慮した小児呼吸外科手術の現状を解説する。(著者抄録)

Language：English   Publisher：(一社)日本循環器学会  

Language：English   Publishing type：Research paper, summary (international conference)   Publisher：ELSEVIER SCIENCE INC  

DOI： 10.1016/j.jamcollsurg.2019.08.1193

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