Language：Japanese   Publisher：Pediatric Surgery International  

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

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PubMed

Language：Japanese   Publisher：Pediatric Surgery International  

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

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PubMed

Language：Japanese   Publisher：Pediatric Surgery International  

Purpose: Management of persistently patent ductus arteriosus (PDA) in extremely low-birth-weight infants (ELBWIs) requires attention due to the risk of tissue hypoperfusion. We investigated the association between PDA and gastrointestinal perforation. Methods: We performed a retrospective chart review from 2012 to 2021. Preterm (≤ 32 weeks) ELBWIs with PDA after birth who developed necrotizing enterocolitis (NEC), focal intestinal perforation (FIP), and idiopathic gastric perforation were included; ELBWIs with congenital heart disease were excluded. Data were analyzed using chi-squared tests with Yates; correction, and Student’s t test. Results: Five hundred thirty-five preterm ELBWIs were analyzed, including 20 with NEC, 22 with FIP, and 1 with gastric perforation. In NEC and FIP, the ductus arteriosus remained open in 40% (4/10) and 63.6% (14/22) of cases, respectively, and cyclo-oxygenase inhibitor treatment showed poor efficacy (p = 0.492 and 0.240). The incidence of perforation in NEC (4/9 vs. 6/11, p = 0.653), mortality in NEC (3/4 vs. 3/6, p = 0.895) and FIP (6/14 vs. 3/8, p = 0.838) did not differ according to whether the PDA persisted or resolved. Conclusion: The presentation of PDA did not affect the mortality or morbidity of ELBWIs. However, it is essential to consider the possibility of gastrointestinal perforation due to decreased organ blood flow caused by ductal steal.

DOI： 10.1007/s00383-023-05420-2

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PubMed

Language：Japanese   Publisher：Pediatric Surgery International  

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

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PubMed

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

Publisher：東京医学社  

DOI： 10.24479/ps.0000000550

Language：English   Publisher：John Wiley & Sons Australia, Ltd  

Language：Japanese   Publisher：Asian Journal of Endoscopic Surgery  

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

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PubMed

Publisher：東京医学社  

DOI： 10.24479/ps.0000000493

Language：Japanese   Publisher：World Journal of Pediatric Surgery  

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

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PubMed

Language：English  

DOI： 10.1186/s40792-023-01679-8

PubMed

Language：Japanese   Publisher：Journal of Dermatology  

Psoriasis affects approximately 0.3% of the Japanese population. Recently, various effective systemic drugs have become available, and the continuation of a given treatment has become critical because of the chronic nature of psoriasis. Factors affecting drug survival (the time until treatment discontinuation) in psoriasis treatment include efficacy, safety, ease of use, and patient preference. In the present study, the authors retrospectively surveyed a multifacility patient registry to determine the real-world evidence of the survival rate of systemic interventions for psoriasis treatment. Patients with psoriasis who visited 20 facilities in the Western Japan area between January 2019 and May 2020 and gave written consent were registered as study participants, and their medical history of systemic interventions for psoriasis (starting from 2010) was retrospectively collected and analyzed. The drugs investigated were adalimumab, infliximab, ustekinumab, secukinumab, ixekizumab, brodalumab, guselkumab, risankizumab, cyclosporine, and apremilast. When drugs were discontinued, the reasons were also recorded. A total of 1003 patients with psoriasis including 268 with psoriatic arthritis (PsA) were enrolled. In biologics, more recently released drugs such as interleukin 17 inhibitors showed a numerically higher survival rate in the overall (post-2010) analysis. However, in the subset of patients who began treatment after 2017, the difference in the survival rate among the drugs was smaller. The reasons for discontinuing drugs varied, but a loss of efficacy against dermatological or joint symptoms were relatively frequently seen with some biologics and cyclosporine. The stratification of drug survival rates based on patient characteristics such as bio-naive or experienced, normal weight or obese, and with or without PsA, revealed that bio-experienced, obese, and PsA groups had poorer survival rates for most drugs. No notable safety issues were identified in this study. Overall, the present study revealed that the biologics show differences in their tendency to develop a loss of efficacy, and the factors that negatively impact the survival rate of biologics include the previous use of biologics, obesity, and PsA.

DOI： 10.1111/1346-8138.16737

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PubMed

Language：Japanese   Publisher：Journal of Dermatology  

Previous studies on family history of psoriasis showed that patients with a family history have an earlier onset of the disease, but such studies in Japan are still limited. To elucidate the characteristics of patients with familial psoriasis, we studied the family history of patients with psoriasis using the West Japan Psoriasis Registry, a multi-institutional registry operated by 26 facilities in the western part of Japan, including university hospitals, community hospitals, and clinics. This study enrolled 1847 patients registered between September 2019 and December 2021, with 199 (10.8%) having a family history of psoriasis. Patients with a family history of psoriasis had significantly earlier onset of the disease than those without a family history. Furthermore, patients with a family history of psoriasis had significantly longer disease duration. Psoriatic arthritis (PsA) was significantly more common in patients with a family history (69/199, 34.7%) than in those without a family history (439/1648, 26.6%) (adjusted P = 0.023). A subanalysis of patients with PsA revealed a significant difference in the patient global assessment (PaGA) score in Fisher's exact test and adjusted test. The numbers of patients with PaGA 0/1 were 29 (43.3%) and 172 (39.9%) in patients with PsA with and without family history of psoriasis, respectively, whereas the numbers of patients with PaGA 3/4 were 13 (19.4%) and 145 (33.6%) in patients with PsA with and without family history of psoriasis, respectively. Other disease severity variables did not show a difference between the two groups. Our findings suggest that genetics play a larger role in the development of PsA than in the development of psoriasis vulgaris. Most cases of PsA occur in patients who already have psoriasis, therefore dermatologists should pay attention to joint symptoms, especially in patients with psoriasis who have a family history of psoriasis.

DOI： 10.1111/1346-8138.16733

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PubMed

Language：English   Publisher：Springer Berlin Heidelberg  

Language：English  

DOI： 10.4103/jmas.jmas_10_23

PubMed

Publisher：東京医学社  

DOI： 10.24479/ps.0000000445

Language：Japanese   Publisher：Journal of Laparoendoscopic and Advanced Surgical Techniques  

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

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PubMed

Language：Japanese   Publisher：Journal of Pediatric Surgery  

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

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PubMed

Publisher：東京医学社  

DOI： 10.24479/pm.0000000804

Language：Japanese   Publisher：Frontiers in Pediatrics  

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

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PubMed

Language：English   Publisher：John Wiley & Sons Australia, Ltd  

Language：Japanese   Publisher：Asian Journal of Endoscopic Surgery  

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

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PubMed

Language：Japanese   Publisher：International Journal of Computer Assisted Radiology and Surgery  

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

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PubMed

Language：Japanese   Publisher：Pediatrics International  

DOI： 10.1111/ped.15699

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PubMed

Language：Japanese   Publisher：Surgery Today  

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

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PubMed

Language：Japanese   Publisher：Frontiers in Pediatrics  

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

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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：Annals of Pediatric Surgery  

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

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Language：Japanese   Publisher：Pediatric Surgery International  

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

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PubMed

Publisher：東京医学社  

DOI： 10.24479/peri.0000000602

Language：Japanese   Publisher：Pediatric Surgery International  

Purpose: Mayer–Rokitansky–Küster–Häuser syndrome (MRKH) usually presents with primary amenorrhea after puberty. The present study clarified the clinical features of MRKH diagnosed at under 16 years old. Methods: We conducted a questionnaire survey to determine the status of MRKH in Japan. Twenty-one MRKH patients were enrolled, and the patient background and clinical features were analyzed. Results: The median age at the diagnosis was 2.6 [1.0–9.0] years. Three patients (14.3%) were diagnosed with type I MRKH, while 18 (85.7%) were diagnosed with type II. The associated anomalies included (some cases overlapped): anorectal malformation (ARM) (n = 13, 72.2%), renal malformation (n = 12, 66.7%), vertebral malformation (n = 11, 61.1%), esophageal atresia (n = 5, 27.8%), and cardiac malformation (n = 4, 22.2%). The comparison of patients with and without ARM revealed that patients with ARM were significantly younger than those without ARM at the time of the diagnosis (1.5 [1–2.6] years vs. 9.1 [8.2–11.7] years, p < 0.05). Associated ARM was the most frequent reason for the diagnosis of MRKH (n = 10, 47.6%). Conclusions: MRKH patients with some associated anomalies, especially ARM, were frequently diagnosed in preoperative imaging examinations or radical operation for ARM. Pediatric surgeons should pay close attention to female patients with ARM to prevent errors in the diagnosis of MRKH.

DOI： 10.1007/s00383-022-05201-3

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PubMed

Publisher：東京医学社  

DOI： 10.24479/ps.0000000249

Language：English   Publisher：John Wiley & Sons Australia, Ltd  

小児の腹腔鏡下虫垂切除術にみられる術後痛に対するアセトアミノフェン静注の予定投与とオンデマンド投与の有効性を比較検討した。腹腔鏡下虫垂切除術を施行された小児患者94例を静注アセトアミノフェン予定投与群(SA群)42例(平均9.31±2.31歳)とオンデマンド投与群(ODA群)52例(平均9.21±2.89歳)に分類し、両群の患者背景、手術成績、術後アウトカムを比較した。体重、白血球数、CRP、AST値、ALT値、虫垂サイズに関してSA群とODA群との間に有意差はなかった。手術時間、気腹時間、癒着の有無、病理学的診断、術後摂食開始までの期間、抗生剤投与、術後在院期間にも有意な群間差はなく、術後合併症の発症はみられなかった。術後痛に関して、術翌日、2日後ともに疼痛の訴えはSA群の方がODA群より有意に少なく、蜂巣炎性虫垂炎、壊疽性虫垂炎のいずれでも術翌日はSA群の方が疼痛を訴える患児は少なかった。このほか、静注アセトアミノフェンの投与量はSA群の方が有意に多く、救済鎮痛薬としてのペンタゾシンの投与回数に有意な群間差はなかった。小児の腹腔鏡下虫垂切除術後の疼痛に対して、静注アセトアミノフェンの予定投与は有用であることが示された。

Language：English   Publisher：John Wiley & Sons Australia, Ltd  

症例は13歳男児で、腹痛と触知可能な腫瘤を呈し、造影CTで上行結腸に浸潤する最大径13cmの巨大腹部腫瘍を認め、バリウム注腸にて上行結腸の重度狭窄がみられた。PET-CTで多発性腹部リンパ節転移が検出され、MRIでは腫瘍は大網に起源し、T1強調像にて軽度低信号、T2強調像にて軽度高信号を呈していた。大網に生じる線維形成性小円形細胞腫瘍が考えられ、鑑別疾患としてリンパ腫、結核性腹膜炎が挙げられた。腹腔鏡下手術を行うこととし、腹腔内に進入すると右側腹部に大網を覆う巨大腫瘍を確認し、漿液性腹水がみられたが肉眼的に腹膜播種は認められなかった。腹水を採取すると悪性細胞は検出されず、転移が疑われるリンパ節の腫大を同定、巨大腫瘍は炎症性変化をきたして腹膜に癒着しており、超音波デバイスを用いて慎重に切離した。続いてリンパ節4Dと6を大網と右胃大網動脈から切離し、リンパ節202は回結腸動静脈を結紮後に切除した。さらに、回腸末端近位部に沿って回腸を15cmにわたって切離し、巨大腫瘍と横行結腸に対する摘出術を施行した。手術時間は5時間47分、出血量は20mLであった。術後の病理所見、免疫組織化学染色の結果から11q異常を伴うBurkitt様リンパ腫と確定診断を下した。術後経過は良好で、4日目に経口摂取を開始し、腫瘍融解症候群の症状なく寛解が得られている。

Language：Japanese   Publisher：Asian Journal of Endoscopic Surgery  

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

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PubMed

Language：Japanese   Publisher：Asian Journal of Endoscopic Surgery  

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

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PubMed

Language：Japanese   Publisher：Asian Journal of Endoscopic Surgery  

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

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PubMed

Language：Japanese   Publisher：Asian Journal of Endoscopic Surgery  

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

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PubMed

Language：Japanese   Publisher：Asian Journal of Endoscopic Surgery  

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

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PubMed

Language：English  

DOI： 10.1186/s40792-022-01535-1

PubMed

Language：English   Publisher：Springer Berlin Heidelberg  

Language：Japanese   Publisher：Pediatric Surgery International  

Purpose: We compared cases of anemia in gastroschisis versus omphalocele and investigated this clinical question. Methods: A multicenter study of five pediatric surgery departments in southern Japan was planned. Sixty patients were collected between 2011 and 2020, with 33 (gastroschisis: n = 19, omphalocele: n = 14) who met the selection criteria ultimately being enrolled. Anemia was evaluated before discharge and at the first outpatient visit. Results: Despite gastroschisis cases showed more frequent iron administration during hospitalization than omphalocele (p = 0.015), gastroschisis cases tended to show lower hemoglobin values at the first outpatient visit than omphalocele cases (gastroschisis: 9.9 g/dL, omphalocele: 11.2 g/dL). Gastroschisis and the gestational age at birth were significant independent predictors of anemia at the first outpatient visit, (gastroschisis: adjusted odds ratio [OR] 19.00, p = 0.036; gestational age at birth: adjusted OR 0.341, p = 0.028). A subgroup analysis for gastroschisis showed that the ratio of anemia in the 35–36 weeks group (8/10, 80.0%) and the > 37 weeks group (6/6, 100%) was more than in the < 34 weeks group (0/3, 0.0%). Conclusions: Gastroschisis may carry an increased risk of developing anemia compared with omphalocele due to the difference of direct intestinal exposure of amnion fluid in utero.

DOI： 10.1007/s00383-022-05150-x

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PubMed

Language：Japanese   Publisher：Pediatric Surgery International  

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

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PubMed

Publisher：東京医学社  

DOI： 10.24479/ps.0000000183

Language：Japanese   Publisher：Journal of Pediatric Surgery  

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

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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。

Publisher：東京医学社  

DOI： 10.24479/ps.0000000160

Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p>We successfully treated a case of pancreatic pseudocyst caused by type IIIb pancreatic injury via laparoscopic external drainage. [Case] A 14-year-old boy presented with abdominal pain after having his upper abdomen bruised by contact with another player’s knee during a soccer match. Magnetic resonance cholangiopancreatography (MRCP) showed complete rupture of the pancreatic body, and fluid collection was detected around it. The patient was diagnosed as having type IIIb pancreatic injury based on the classification of the Japanese Society of Trauma. We selected a non-operative management approach because the patient was hemodynamically stable. However, after conservative treatment, a pancreatic pseudocyst of 3 cm diameter was found; thus, endoscopic retrograde pancreatography (ERP) was performed on the 11th day of illness. The pancreatic duct was transected at the body of the pancreas, and a pancreatic duct stent was placed into the cyst, but it fell off after three days. After that, the pancreatic pseudocyst tended to grow and increased in size from the subdiaphragm to the lower pole of the kidney. Therefore, we performed laparoscopic external drainage on the 45th day of hospitalization. After the operation, the patient showed no abdominal symptoms, and no recurrence of cysts was recognized. [Conclusion] Laparoscopic drainage of pancreatic pseudocysts can enable the observation of the entire abdominal cavity while also facilitating safe and reliable drainage. Therefore, depending on the patient condition, laparoscopic external drainage of pancreatic pseudocysts may be considered as a treatment option.</p>

DOI： 10.11164/jjsps.58.4_734

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

今回、IIIb型膵損傷後の膵仮性嚢胞に対し、腹腔鏡下嚢胞ドレナージが奏功した1例を経験した。【症例】症例は14歳男児。サッカーの試合中に他選手の膝で上腹部を打撲後に腹痛を訴え救急搬送された。Magnetic resonance cholangiopancreatography(MRCP)では日本外傷学会分類IIIb型の膵損傷と診断した。全身状態は安定しており保存的に加療する方針とした。しかしその後に、3cm大の膵仮性嚢胞を認め、内視鏡的逆行性膵管造影を11病日に施行した。主膵管は膵体部で断裂し、膵管ステントを嚢胞内へ留置したが数日で脱落した。その後、嚢胞は横隔膜下から腎下極に及ぶまで増大したため、入院45日目に腹腔鏡下嚢胞開窓ドレナージ術を行った。術後は腹部症状や嚢胞の再発は認めず経過している。【結語】膵仮性嚢胞に対する腹腔鏡下嚢胞ドレナージは、腹腔内の観察ができ、確実なドレナージが可能である。病態に応じて膵仮性嚢胞のドレナージ法の選択肢として検討されうると考えられた。(著者抄録)

Language：Japanese   Publisher：日本小児泌尿器科学会  

【目的】急性陰嚢症では精巣捻転症を見逃さないことが重要となるが、鑑別疾患が多く診断に苦慮することを経験する。TWISTスコアは精巣捻転症を示唆する身体所見から算出する臨床スコアとして非専門医でも用い易い。当院での小児急性陰嚢症に対するTWISTスコアについて臨床的検討を行った。【対象と方法】2015年1月から2019年12月までの5年間に当院で経験した16歳未満の急性陰嚢症94例のうち、TWISTスコアが算出され治療後の経過が確認できた68例を後方視的に検討した。【結果】疾患内訳は精巣付属小体捻転症が30例(44.1%)、精巣上体炎18例(26.5%)、精巣捻転症14例(20.6%)、急性精巣炎と精巣外傷が2例(2.9%)、陰嚢浮腫と精巣腫瘍が1例(1.5%)であった。TWISTスコアは、精巣捻転症が非精巣捻転症(54例)より高値で、手術群(51例が)非手術群(17例)より高値で、更に手術群の中でも精巣捻転症が非精巣捻転群(37例)より有意に高値であった(いずれもp値<0.01)。しかし精巣捻転症のうち精巣摘出群(9例)と温存群(5例)の間には有意差はなかった。【結論】精巣捻転症のTWISTスコアは他疾患より高値であり、TWISTスコア0点では精巣捻転症を否定することが出来た。しかしTWISTスコアのみで精巣捻転症の診断を行うことは難しく、従来通り総合的な判断が必要である。今後本邦の臨床に則したTWISTスコアを見出せれば、その有用性を示すことも可能であると考える。(著者抄録)

Language：Japanese   Publisher：Journal of Laparoendoscopic and Advanced Surgical Techniques  

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

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PubMed

Publisher：東京医学社  

DOI： 10.24479/ps.0000000100

Language：English   Publisher：John Wiley & Sons Australia, Ltd  

症例は1歳8ヵ月の男児で、生後一時的に胆汁嘔吐をきたしたが症状は自然消失し、発達障害はみられなかった。1歳4ヵ月時、間欠的な胆汁嘔吐を認め、精査目的に当院を受診した。上部消化管造影検査で十二指腸球部から下部にかけての拡張がみられ、下部から水平部にかけての造影剤のうっ滞が生じていた。造影CTでは上腸間膜動脈と上腸間膜静脈(SMV)の配置に異常はみられなかったが、末梢部にてコークスクリュー徴候を認め、SMVの著明な狭窄と側副循環が検出された。以上の所見から中腸軸捻を伴う腸回転異常と診断し、腹腔鏡下手術を計画した。中腸軸捻を回盲部からの小腸トレースにより逆行性に解除し腸間膜根部を確認、腸間膜に重度の癒着が生じていたため、インドシアニングリーンによる蛍光ナビゲーション下に組織をクランプして灌流を図った。これにより安全な組織の切離が可能となり、腸間膜根部の開大を確認できた。腸管を非回転位に収納し脾彎曲部の結腸はBill法を用いて固定した。術後2日目に飲水、3日目に摂食が可能となり、5日目に退院となった。その後4ヵ月、軸捻やイレウスの再発は認めていない。

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：Japanese   Publisher：Asian Journal of Endoscopic Surgery  

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

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PubMed

Language：English  

DOI： 10.1186/s40792-022-01409-6

PubMed

Publisher：東京医学社  

DOI： 10.24479/ps.0000000080

Language：English   Publisher：Springer Berlin Heidelberg  

症例は3歳男児で、突然の腹痛を主訴に近医を受診した。ウイルス性胃腸炎と診断され、保存的治療が行われたが症状は改善しなかった。X線検査で腸管拡張とイレウスを認め、当院へ搬送された。腹部膨隆と腹部全体に圧痛を認め、特に下腹部で強い筋性防御(腹膜刺激徴候)を認めた。臨床検査で白血球数は20800/μL、C反応性蛋白質は5.79mg/dLの高値を呈した。ASTは29U/L、ALTは11U/L、総ビリルビンは0.8mg/dL、尿素窒素は8.0mg/dL、クレアチニンは0.21mg/dLであった。造影CTで構造が消化管に似た嚢胞性病変と腹水を認めた。腸管拡張またはイレウスの所見は見られなかった。消化管への血流は保持されていたため、メッケル憩室捻転の疑いで診断的腹腔鏡検査を実施した。右下腹部に黒色の管状構造を形成する嚢胞性病変が720°茎捻転していた。捻転を整復したところ、メッケル憩室捻転ではなく、消化管重複症であった。重複腸管を切除した。病理組織学的検査で切除標本は回腸壁の構成要素を有していた。術後経過は良好で、14日後に退院となった。3年後の再診で合併症は見られなかった。

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：Japanese   Publisher：Journal of Pediatric Surgery  

Aim of the study: We conducted a nationwide survey of persistent cloaca (PC) to determine its current status in Japan. We focused on the relationship between fetal ultrasonographic findings and irreversible renal dysfunction in PC. Method: Patient information was obtained via questionnaire, and 466 PC patients were ultimately enrolled in this study. Fifteen patients (3.2%) who required dialysis or kidney transplantation were compared to those who were able to survive with their native kidney. Univariable and multivariable analyses were performed using a logistic regression analysis to clarify the relationship between irreversible renal dysfunction and fetal ultrasonographic findings. Results: A multivariable logistic analysis showed that fetal ultrasonic findings of oligohydramnios independently increased the risk of irreversible renal dysfunction (adjusted odds ratio [OR] 5.8, 95% confidence interval [CI] 1.7–20, p = 0.005). A regression analysis showed that fetal ultrasonographic findings of hydroureteronephrosis (crude OR 5.6, 95% CI 0.9–24, p = 0.03) tended to be associated with irreversible renal dysfunction. In the PC patients with oligohydramnios, however, the ultrasonographic findings and associated anomalies did not affect the renal prognosis. The 15 renal dysfunction patients were treated as follows: hemodialysis (n = 4), peritoneal dialysis (n = 3), living donor renal transplantation (n = 8), and cadaveric renal transplantation (n = 1). Conclusion: Fetal ultrasonographic findings of oligohydramnios increase the risk of irreversible renal dysfunction. Such findings suggest we consider the need for earlier therapeutic intervention, such as fetal and postnatal treatment, to prevent the progression of renal dysfunction. Level of evidence: III (Study of diagnostic test, study of nonconsecutive patients and/or without a universally applied “gold” standard).

DOI： 10.1016/j.jpedsurg.2021.10.038

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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   Publisher：シュプリンガー・ジャパン(株)  

単施設において、1984年4月～2017年12月に後腹膜奇形腫(RPT)の治療を行った小児患者15例(男児4例、女児11例)の治療経験を報告した。年齢は日齢6～12歳であり、15例中11例(73.3%)が1歳未満であった。すべての患者で外科的完全切除術が施行された。術中の腫瘍破裂を4例(26.7%)に、周術期の血管損傷を6例(40.0%)に認め、最終的には1例(6.7%)が腎摘出術を要した。また、手術合併症として、片側腎機能障害を3例(20.0%)に認めた。術後化学療法を施行した患者は1例のみであった。その後いずれの患者も治癒している。

Language：English   Publisher：シュプリンガー・ジャパン(株)  

Hirschsprung病(HD)の小児患者65例を対象に、長期的な腸機能を基に、根治手術の最適な施行時期について検討した。根治手術の術式は、Soave-Denda式(SD)が25例、経肛門直腸プルスルー式(TA)が40例であった。対象を生後6ヵ月未満で根治術施行例(低年群)と、生後6ヵ月～12ヵ月で施行した患者(高年群)に振り分け、5歳、7歳、9歳時の腸機能を評価した。SD施行例では、術後の腸機能に低年群と高年群との間で有意差は認めなかった。TA施行患者でも同様に、年齢別による全体の腸機能スコアに有意差は認めなかった。TA施行例では、7歳時における下着の汚染スコアが、低年群よりも、高年群で有意に低かった。これらの結果から最適な腸機能を得るためには、TAは生後6ヵ月までに施行するべきと示唆された。

Language：Japanese   Publisher：Surgery Today  

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

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PubMed

Language：Japanese   Publisher：Surgery Today  

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

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PubMed

Language：Japanese   Publisher：Journal of Dermatology  

Generalized pustular psoriasis (GPP) is a rare and severe subtype of psoriasis. Because of its rarity, GPP studies with a large sample size have been scarce. We studied the characteristics of GPP and pustular psoriasis using data from the West Japan Psoriasis Registry that had been registered until the end of December 2020. The dataset included 104 patients with pustular psoriasis and 1290 patients with other subtypes of psoriasis. Multivariate analysis revealed a significantly greater number of female patients, a significantly lower mean body mass index, and a significantly lower ratio of habitual drinkers in pustular psoriasis, compared to other subtypes of psoriasis. Of the 104 patients, 102 had GPP, including 88 von Zumbusch, 10 juvenile-onset, and four annular pustular psoriasis. Although the male : female ratio of GPP with psoriasis vulgaris (GPP+PsV) (47/20) was similar to that of psoriasis in Japan, the GPP without PsV (GPP−PsV) group highlighted a female predominance (13/22). The mean age at GPP onset was 45.3 years, and the mean interval from PsV onset to GPP onset was 12.5 years. Four of nine patients with GPP had an IL36RN gene mutation. Infection, medicine, and pregnancy were the precipitating factors for GPP. A family history of psoriasis was present in eight (7.8%) patients with GPP. Twenty-four patients with GPP had psoriatic arthritis. Biologics were used in 76.5% of patients with GPP, followed by etretinate (37.3%), cyclosporine (24.5%), methotrexate (13.7%), apremilast (8.8%), and granulocyte and monocyte adsorption apheresis (6.9%). Etretinate was used in 17 (51.5%) of 33 patients with GPP with less than 10-year history. Thus, etretinate remains a good treatment option for GPP even in the era of biologics. Hypertension was the most commonly identified comorbidity, followed by diabetes. We believe that the characteristics revealed in this study can further contribute to effective GPP management.

DOI： 10.1111/1346-8138.16217

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

DOI： 10.5759/jscas.24.191

Publisher：(株)東京医学社  

DOI： 10.24479/j00645.2022081276

Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p><i>Purpose</i>: The incidence of intussusception is low in older children for various reasons. Owing to the lack of a consensus concerning clinical management, the treatment approach differs among clinicians. To optimize the management of intussusception, we retrospectively analyzed cases in older children admitted to our hospital over 10 years.</p><p><i>Methods</i>: From 2007 to 2017, 10 children >5 years old were diagnosed with intussusception and admitted to our hospital. We retrospectively reviewed their clinical records.</p><p><i>Results</i>: The patients were six boys and four girls between 5 and 14 years old. The main symptoms were abdominal pain (10/10), vomiting (6/10), and hematochezia (2/10). Eight patients were successfully managed by enema, but four of them developed recurrence. Five patients, including three recurrent cases, underwent surgery. The pathological lead point (PLP) was found in three patients. On laparotomy, intestinal wall thickening was found in these three patients, leading to ileocecal resection or an intestinal biopsy. Pathologically, they all showed lymphoid hyperplasia or inflammatory changes. The type of intussusception was ileocolic in nine and enteroenteric in one. To explore the PLP, nine patients underwent computed tomography (CT), magnetic resonance imaging (MRI), Meckel’s scintigraphy, and colonoscopy depending on their condition.</p><p><i>Conclusions</i>: Patients presenting with recurrent intussusception were likely to undergo surgery because identifying the PLP by imaging is difficult. To avoid unnecessary surgery, it is important to repeat enema reduction, even for recurrent cases, while searching for PLP through various modalities, including colonoscopy.</p>

DOI： 10.11164/jjsps.57.7_1049

Language：Japanese   Publisher：Pediatric Surgery International  

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

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PubMed

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

【目的】年長児腸重積症(以下本症)の発生頻度は少ないが、病的先進部を有する割合が多いとされている。年長児の本症に対しては一定の診療指針がなく、症例毎に様々なアプローチがなされている。今回、年長児症例に対する適切な診断、治療法を確立すべく自験例を後方視的に検討した。【方法】2007年1月～2017年12月の間に、鹿児島市立病院小児外科に入院した5歳以上の腸重積症10例を対象とし、診療録を用いて後方視的に検討した。【結果】対象は男児6例、女児4例、年齢は5～14歳であった。主要症状は、腹痛10例、嘔吐6例、血便2例であった。8名に非観血的整復が行われ、全例整復に成功したが、その内4例が1～5回再発した。病的先進部は3例(30%)に認められた。重積のタイプは、9名が回結腸型、1名が小腸小腸型であった。手術適応を5例に認め、そのうち整復後に再発をきたした3例において、術中腸管壁肥厚を触知し、悪性疾患が否定できないため、回盲部切除または腸管壁生検を行ったが、病理診断は、リンパ濾胞過形成または炎症性変化であった。病的先進部の検索で、腹部CT検査が9例に行われ、その他症例に応じてメッケル憩室シンチグラム、MRI検査、大腸内視鏡検査が行われていた。【結論】画像検査による病的先進部の検索には限界があり、本症を繰り返す症例には、手術が選択されることが多かった。本症は再発例でも特発性が一定の割合で存在するため、その都度非観血的整復を繰り返し、大腸内視鏡検査も含めた病的先進部の検索を行うことが不必要な手術の減少につながると思われた。(著者抄録)

Language：English   Publisher：シュプリンガー・ジャパン(株)  

胃切除が施行された患者にみられる胃排出能遅延の差に繋がる因子を検討した。腹腔鏡下噴門形成術と胃切除術を受けた神経症状を有する患者32例を対象とし、術前の胃の配位によって非挙上(NESP)群21例(男性19例、女性2例、平均12.5±11.2歳)と挙上(ESP)群11例(男性8例、女性3例、平均26.5±17.6歳)に分類、各群の患者特性、治療成績、術後の胃排出能を比較した。検討の結果、体重はNESP群の方がESP群より有意に低値であったが、男女比、身長、BMIに群間差はみられなかった。手術時間、気腹時間、出血量、術後合併症の発症率に関して有意差はなく、胃ドレナージ減少までの期間はESP群の方が有意に長く、経腸栄養開始までの期間には有意な群間差はなかった。ただし、全経腸栄養達成までの期間はNESP群の方が有意に長く、胃排出能遅延率と胃運動促進薬の使用率に有意差はみられなかった。多変量解析では、年齢と体重を補正後にも全経腸栄養達成までの期間はNESP群の方がESP群より長い傾向にあった。胃配位の調整は胃排出能遅延に関与する可能性が示された。

Language：Japanese   Publisher：Journal of Dermatology  

The efficacy and safety of new psoriatic treatments are confirmed in clinical trials, but such clinical trial data are limited by the number and heterogeneity of patients. Furthermore, the prevalence and characteristics of psoriasis differ among racial groups. Therefore, it is important to obtain real-world evidence in specific regions. To identify the optimal systemic treatment for psoriatic patients in Western Japan, we established the Western Japan Psoriasis Registry (WJPR). This registry is led by a neutral physicians’ league associated with university hospitals, general hospitals, and clinics that specialize in treatment of psoriasis. Systemically treated psoriatic patients who provided written informed consent were enrolled, and data were collected on their background information, several patient-reported outcomes, dermatologists’ objective evaluations, and treatment regimens. Patient enrollment began in 2019, and 1394 patients had been recruited by the end of 2020. The prevalence of psoriatic arthritis was 27.2% and that of pustular psoriasis was 7.5%. The mean body mass index was 24.1 kg/m2, and 12% of patients had severe obesity (body mass index ≥30 kg/m2). Major comorbidities were hypertension (35.0%), diabetes (14.1%), and hyperlipidemia (12.2%). Serological data showed that hepatitis B virus surface antigen, anti-hepatitis B virus core antibody, anti-hepatitis C virus antibody, and human T-cell leukemia virus type 1 antibody were detected in 1.1%, 18.0%, 3.1%, and 3.7% of patients, respectively. The most frequently used small-molecule-systemic intervention was apremilast (18.0%), followed by methotrexate (7.7%), etretinate (4.2%), and cyclosporin (3.7%). The most frequently used biologics were interleukin (IL)-17 inhibitors (31.8%), followed by IL-23 inhibitors (including IL-12/23 inhibitors) (26.7%), and tumor necrosis factor inhibitors (11.1%). The WJPR is the first Japanese prospective observational cohort of psoriatic patients. Annual WJPR updates may provide the incidences of comorbidities such as cardiovascular events or onset of arthritis in systemically treated patients, identify rare complications, and identify the optimal treatment regimens for various psoriatic patients.

DOI： 10.1111/1346-8138.16092

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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><i>Purpose</i>: We reviewed the cases of patients who underwent umbilicoplasty (VY flap) with a reverse Y-shaped incision at our hospital and evaluated its therapeutic results and usefulness.</p><p><i>Methods</i>: We retrospectively evaluated sex, age at surgery, birth weight, gestational age, comorbidities, operation time, and postoperative complications in 191 patients who underwent umbilicoplasty during the 15-year period from April 2004 to November 2018. In addition, we conducted a questionnaire survey about patient satisfaction.</p><p><i>Results</i>: Umbilicoplasty was performed in 183 patients before they entered school. The most common complication was inguinal hernia (n = 18; 9.4%). Periumbilical dermatitis was the most common postoperative complication (n = 9; 13.8%). Only three patients (4.6%; two with exudate and one with dermatitis) required ointment treatment. Most of the patients with complications recovered without treatment. Among the 68 patients who completed the questionnaire survey, 48 described their umbilical appearance as “satisfactory”. In the comparison between the satisfied group and the unsatisfied group, there were significant differences in age at surgery and sex. The patients in the satisfied group were older and the group had a higher proportion of boys. Five children (average age, 7.6 years) responded that they themselves wanted to undergo the operation.</p><p><i>Conclusions</i>: Satisfaction with the surgical procedure at our hospital was fully evaluated. Currently, there are various procedures for umbilical hernia. However, it seems that the appropriate timing of operation may be different for each operative procedure.</p>

DOI： 10.11164/jjsps.57.6_938

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

【目的】当科で施行した逆Y字皮膚切開による臍形成術(VY皮弁)の治療成績及び術式の有用性に関する検討を行った。【方法】2004年4月から2018年11月までの15年間に当院で臍ヘルニアの手術を行った191例を対象に、性別、手術時年齢、出生体重、出生週数、合併疾患、手術時間、術後合併症に関して後方視的に検討した。加えて患者満足度調査として5項目からなるアンケートを実施し、結果について解析した。【結果】症例191例の内訳は男児94例、女児97例で性差は認めなかった。手術時年齢の中央値は2歳(0.16～15.50)で、2歳が85例(44.5%)と最多であり、就学時前が183例(95.8%)と大多数を占めた。合併疾患は鼠径ヘルニア類疾患が18例(9.4%)と最も多かった。術後合併症としては臍周囲皮膚炎が9例(13.8%)と最も多かった。アンケート回収率は50.0%であり、臍外観に70.6%が「大変満足」と回答した。理想の臍外観の項目は「へこみ」(23.3%)や「大きさ」(20.0%)よりも「全体」が最も多く約半分を占めていた。手術時年齢は満足群の方が有意に高年齢であった(p=0.032)。性別に関しては満足群に男児の割合が多い傾向にあった(p=0.007)。【結論】逆Y字皮膚切開による臍形成術は、比較的簡単で術後満足度の高い術式である。当術式の満足群は年齢が高い傾向にあり、安定した満足度を得るためには手術時期を2歳以降に設定する選択肢があると思われた。現在臍ヘルニアに対する術式は様々であるが、各々の術式により適切な手術時期が異なる可能性もあると考えられた。(著者抄録)

Language：English   Publisher：John Wiley & Sons Australia, Ltd  

症例は新生児女児で、在胎28週6日に胎便性腹膜炎が疑われた。在胎37週3日で経腟分娩にて出生し、直ちにNICUに入院となった。同日、試験開腹術を施行した。強固な腸管癒着を認め、緊急ドレナージのみを行った。生後4日目に右大陰唇に膨張および発赤を認めた。生後12日目に破裂し、瘻孔からの多量の膿性液の流出を認めた。生後16日目に2度目の手術を施行した。腸管癒着は容易に剥離され、回腸末端の穿孔は閉じられた。術後、瘻孔からの液体の流出が持続していたが、生後24日目にようやく止まった。生後26日目に原因不明の発熱を呈したため、造影CT検査を実施した。その結果、腎盂腎炎と同時に腹腔から右陰唇にかけて鞘状突起右に沿った石灰化が認められた。生後62日目に医療的サポートなしで退院となった。

Language：English   Publisher：John Wiley & Sons Australia, Ltd  

症例は10ヵ月女児で、4ヵ月前から水様便、成長障害、脱水および低カリウム血症をきたした。CTで右副腎腫瘍が認められ、4.2×4.2×3.0cm大であり、辺縁平滑でリンパ節浸潤や遠隔転移はみられなかった。腫瘍の均一性と石灰化からみて定型的浸潤性神経芽細胞腫とは異なるものであり、123I-MIBGシンチグラフィーでは腫瘍内に造影剤の取り込みは認められなかった。また、臨床検査でニューロン特異的エノラーゼ、尿中バニリルマンデル酸および尿中ホモバニリン酸の上昇を認め、副腎血管活性腸管ポリペプチド産生神経芽細胞腫(VIPNT)と診断した。オクトレオチド投与を開始するも下痢の改善がみられず、腹腔鏡下腫瘍切除術を行うこととした。12mmポート1本を臍部に挿入し、さらに剣状下、右体幹および右上腹部にそれぞれ5mmポートを挿入した。腫瘍は十二指腸尾側に位置しており、腫瘍破裂を避けるため鉗子で把持することなく慎重に摘出処理を行った。手術時間は149分、出血量は2mLであった。摘出標本に対する免疫染色の結果、VIP陽性でありVIPNTと診断した。術後経過は良好で下痢は術直後に改善し、1年後も再発は認めていない。

Language：English  

DOI： 10.1186/s40792-021-01231-6

PubMed

Language：English   Publisher：Springer Berlin Heidelberg  

3ヵ月前から心窩部痛と嘔吐が2週毎に発生していた5歳女児症例について検討した。腹部超音波検査では肝円索付近に非血管病変が判明した。造影CTでは肝円索の右側にhyperdense lineとdisproportionate fat strandingに囲まれたlow attenuation massを認めた。腹水や出血はなく、肝円索膿瘍が示唆された。経口抗生物質投与で症状は改善し、待機的探索的腹腔鏡検査および切除術を計画した。マルチチャネルポートデバイスから臍に二つの5mmトロカール、右脇腹に一つの3mmトロカールを挿入した。腹腔の観察では肝円索に網が癒着しており、肉眼的観察では明らかな腫瘤病変は認めなかった。肝円索からの網癒着剥離術を行い、癒着形成部位を切除し、切除検体を臍創から摘出した。術後の経過は良好であった。病理学的所見では壊死性肝組織が判明し、切除組織は虚血性変化を伴った副肝葉であった。

Publisher：(株)東京医学社  

DOI： 10.24479/j00645.2021229963

Publisher：(株)東京医学社  

DOI： 10.24479/j00645.2021229964

Language：Japanese   Publisher：Surgery Today  

Purpose: Despite improvements in neonatal intensive care, the outcomes of extremely-low-birth-weight infants (ELBWIs) with surgical diseases remain to be improved. We started administering enteral miconazole (MCZ) to ELBWIs from 2002 to prevent fungal infection. Since then, the incidence of intestinal perforation has significantly decreased. We investigated this prophylactic effect of MCZ against necrotizing enterocolitis (NEC) and focal intestinal perforation (FIP) and explored a new prophylactic concept against intestinal perforation. Methods: We designed a historical cohort study to evaluate the effect of MCZ for intestinal perforation in ELBWIs who underwent treatment in our neonatal intensive-care unit between January 1998 and December 2005. We divided these cases into two groups: the Pre-MCZ group and the Post-MCZ group. We compared the morbidity, clinical outcomes and pathological features of NEC and FIP. Results: The rate of intestinal perforation with NEC was significantly reduced after the introduction of MCZ (p = 0.007, odds ratio; 3.782, 95% confidence interval; 1.368–12.08). The pathological findings of NEC specimens showed that the accumulation of inflammatory cells was significantly reduced in the Post-MCZ group when compared with the Pre-MCZ group (p < 0.05). Conclusions: The efficacy of the enteral administration of MCZ on intestinal perforation with NEC highlights a new prophylactic concept in the clinical management of ELBWIs.

DOI： 10.1007/s00595-020-02125-0

Scopus

PubMed

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

新生児胃破裂は近年稀な疾患となったが、我々は1年間に4症例のべ5件を経験した。今後の新生児管理に生かすため、自験例の発生原因を文献的に考察検討した。全例が緊急帝王切開で出生した早期産未成熟児で、大彎側の破裂(3件)、体部前壁の破裂(2件)は生後早期にみられた。いずれも新生児の胃壁に特徴的な解剖学的脆弱部であった。自験例では、周産期のdiving reflexによる胃壁血流障害に加えて、胎便栓による通過障害、甲状腺機能低下による機能的胃腸蠕動不良、臓器軸性胃軸捻等により胃内圧が耐容上限を超えたことが要因として考えられた。我々は、経時的に注意深く腹部所見の観察を重ねること、適正に上部消化管減圧を維持することの意義を再確認した。これらはNICUでは当然の管理であるが、新生児胃破裂の発症予防策としても重要である。(著者抄録)

Language：English   Publisher：シュプリンガー・ジャパン(株)  

ミコナゾール(MCZ)腸内投与の壊死性腸炎(NEC)、局所性腸穿孔(FIP)に対する予防効果を検討した。NICUに収容された超低出生体重児400例を対象に後ろ向きコホート研究を実施し、MCZ導入前の172例とMCZ導入後の172例に分類、各群の死亡率、臨床アウトカム、病理像を比較検討した。その結果、腸穿孔をきたしたのはMCZ導入前群が20例(11.6%)、導入後群が10例(4.4%)で、発症率はMCZ導入後に有意に減少していた。NECを伴う腸穿孔の発症率もMCZ導入後に有意に減少していたが、FIPの発症はMCZ導入前後とも4例であり有意な群間差はみられなかった。NECを伴う腸穿孔を発症した患者データの比較では、性別、在胎週数、出生時体重、胎児成長遅滞、手術時日齢、生存率に関してMCZ導入前群と導入後群との間に有意差はなかった。また、FIP発症例についても各パラメータに有意な群間差はみられなかった。NEC病変の病理所見では、炎症細胞の集積はMCZ導入後群の方が導入前群と比較して有意に減少していた。MCZ腸内投与はNECを伴う腸穿孔の予防に有効であることが示された。

Publisher：(株)東京医学社  

DOI： 10.24479/j00645.2021163596

Language：Japanese   Publisher：Pediatric Surgery International  

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

Scopus

PubMed

Language：Japanese   Publisher：Pediatric Surgery International  

Purpose: Necrotizing enterocolitis (NEC), focal intestinal perforation (FIP), and meconium-related ileus (MRI) are major diseases that cause gastrointestinal disorders in extremely low-birth-weight infants (ELBWIs). We conducted a review to compare the postoperative outcomes of ELBWIs with these diseases in our neonatal intensive-care unit. Methods: A retrospective chart review of ELBWIs surgically treated for NEC (n = 31), FIP (n = 35), and MRI (n = 16) in 2001–2018 was undertaken. This period was divided into early (2001–2005), middle (2006–2010), and late (2011–2018) periods. Data were analyzed with the Cochran-Armitage test. Statistical significance was defined as p < 0.05. Results: The survival rates in ELBWIs with NEC (early/middle/late: 36.4%/42.9%/61.5%; p = 0.212) and FIP (20%/50%/70.6%; p = 0.012) improved over time; all patients with MRI survived. The neuropsychological development of 24 cases was assessed with the Kyoto Scale of Psychological Development in the Postural-Motor, Cognitive-Adaptative, and Language-Social domains. The mean developmental quotient of all domains was 68.4 (range 18–95) at corrected 1.5 years of age and 69.1 (range 25–108) at chronological 3 years of age, both were considered as poor development. There was no improvement over time (p = 0.899). Conclusion: Ideal neuropsychological development was not observed with the improvement of survival rate. Less-invasive surgical intervention and adequate postoperative care are required to encourage further development.

DOI： 10.1007/s00383-020-04825-7

Scopus

PubMed

Language：English   Publisher：SAGE Publications  

<jats:sec><jats:title>Purpose:</jats:title><jats:p> 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. </jats:p></jats:sec><jats:sec><jats:title>Methods:</jats:title><jats:p> 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. </jats:p></jats:sec><jats:sec><jats:title>Result:</jats:title><jats:p> 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. </jats:p></jats:sec><jats:sec><jats:title>Conclusion:</jats:title><jats:p> 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. </jats:p></jats:sec>

DOI： 10.1177/1129729820987362

PubMed

Language：English  

DOI： 10.1186/s40792-020-01099-y

PubMed

Language：Japanese   Publisher：Asian Journal of Endoscopic Surgery  

We herein report a 10-month-old female infant with a 4-month history of diarrhea with electrolyte abnormalities and growth impairment. A 4-cm right adrenal tumor was detected by computed tomography. No metastasis or accumulation on I123-metaiodobenzylguanidine scintigraphy was recognized in the tumor. A vasoactive intestinal peptide-secreting neuroblastic tumor was suspected, and octreotide was started, but the diarrhea persisted. Tumor extirpation was laparoscopically performed. After tumor removal, the symptoms improved immediately, and her growth caught up by 9 months after surgery. A minimally invasive approach for pediatric solid tumor is difficult, especially for neuroblastoma, but may be beneficial for the patient's recovery.

DOI： 10.1111/ases.12916

Scopus

PubMed

Language：English   Publisher：Springer Berlin Heidelberg  

出生体重2.2kgの正期産男児症例について検討した。出生時に肛門閉鎖、大動脈縮窄症、気管食道瘻/食道閉鎖(TEF/EA)、腎奇形、橈骨欠損、四肢異常を認め、VACTERL連合と診断した。右胸心を伴う右肺低形成も認めた。TEF/EAに対する2段階手術を計画し、まず新生児期に経腸栄養を行うために胃食道接合部での食道絞扼術と胃瘻造設術を行い、体重が増加し、心機能が安定した後に、TEF/EAに対する胸腔鏡下手術を施行した。食道吻合を行う際、十分な作業スペースを確保するため、3mmの補助ポートを挿入した。食道吻合は成功したが、術後の食道造影検査において下部食道と右肺下部の接合を確認した。IB型交通性気管支肺前腸奇形と確定診断し、経口摂取を確立するために胸腔鏡下手術を再施行した。気管支食道瘻を離断したところ、経口摂取が可能となり、肺炎や呼吸困難は認めなかった。

Language：Japanese   Publisher：Surgery Today  

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

Scopus

PubMed

Language：Japanese   Publisher：Journal of Pediatric Surgery  

Purpose:: We aimed to investigate potential predictors of focal intestinal perforation (FIP) in extremely low birth weight infants (ELBWIs) among coagulation and fibrinolysis markers at birth. Methods:: We reviewed the medical records of FIP patients and their coagulation and fibrinolysis markers at birth between 2010 and 2019, and matched patients according to gestational age. FIP was diagnosed based on macroscopic intestinal perforation with a punched-out lesion without necrosis. Patient characteristics and blood test results, including coagulation and fibrinolysis marker levels, were compared between the groups. Results:: Two hundred forty ELBWIs were enrolled in this study (FIP, n = 18; controls, n = 222). In the FIP group, the gestational age at birth was significantly younger (p = 0.023) and the birth weight was significantly lower (p = 0.007) in comparison to the control group. Furthermore, the FIP group showed significantly lower levels of fibrinogen (p = 0.027) and factor XIII (F-XIII) (p = 0.007). The receiver operating characteristics curves for fibrinogen and F-XIII revealed that the 95% confidence intervals of fibrinogen and F-XIII were 0.530–0.783 (p = 0.027), and 0.574–0.822 (p = 0.007), respectively. Conclusions:: This is the first report focusing on coagulation and fibrinolysis markers in FIP patients at birth. The fibrinogen and F-XIII values at birth are potential predictors of FIP in ELBWIs. Type of Study:: Study of Diagnostic Test (Case Control Study) Level of Evidence:: Level IV

DOI： 10.1016/j.jpedsurg.2021.03.043

Scopus

PubMed

Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p>Neonatal gastric rupture has become rare in recent years, but we encountered a total of five incidents in four cases during a single year. In this study, we reviewed each incident and considered the related pathogenetic factors reported in the literature in order to apply them to future neonatal management. All the patients were premature infants born by emergent Caesarean section. They developed gastric rupture at the greater curvature side (n = 3) or at the anterior wall of the gastric body (n = 2) early after birth. The ruptures occurred in anatomically vulnerable areas characteristic of the stomach wall in newborns. In our cases, the etiologies were considered as follows: in addition to an impaired blood flow in the gastric wall due to physiological diving reflex induced by perinatal hypoxia events, an increased intragastric pressure that exceeded the tolerable upper limit of the neonatal stomach due to obstruction of the gastrointestinal passage by a meconium plug; functional hypoperistalsis of the gastrointestinal tract induced by hypothyroidism; and an organo-axial gastric volvulus peculiar to newborns. We reconfirmed the significance of careful monitoring of the abdominal findings over time and maintaining an appropriate decompression of the upper gastrointestinal tract as needed. These managements are quite common in the neonatal intensive care unit, but are important as preventive measures for the onset of neonatal gastric rupture.</p>

DOI： 10.11164/jjsps.57.3_625

Publisher：(一社)日本小児外科学会  

当院NICUは年間700例以上を支えるhigh volume centerであるが、今回、非観血的整復を完遂した腸重積症例を初めて経験したので報告する。症例は、日齢3の満期産男児。日齢2の夜間、緑色便に一部血性粘液の付着がみられた。翌朝、中等量の赤色粘血便をみとめ紹介となった。腹部膨満はなかったが、右側腹部にソーセージ様腫瘤を触知した。腹部超音波検査でmultiple concentric ring signをみとめ、回腸結腸型腸重積症と診断した。発症から19時間で高圧浣腸を試み、非観血的整復を完遂した。その後、再発はみられなかった。新生児期の腸重積は稀であるが、超音波検査により本症例の診断は容易であった。全身状態が良好で、腸管壊死を示唆する所見がない満期産新生児の回腸結腸型腸重積症例に対しては、穿孔時の緊急開腹手術体制を整えたうえで、安全に非観血的整復を試みることも妥当であると思われた。(著者抄録)

Publisher：(株)東京医学社  

1985年4月〜2020年3月までの鹿児島県における腹壁異常の手術症例69例の治療成績について後方視的検討を行った。なお、1985年4月〜2004年3月(前期)までは全例が鹿児島大学病院、2004年4月〜2020年3月(後期)までは鹿児島大学病院と鹿児島市立病院で診療された。対象の内訳は臍帯ヘルニア36例(男児19例、女児17例)、腹壁破裂33例(男児16例、女児17例)で、帝王切開での分娩は臍帯ヘルニア21例(58.3%)、腹壁破裂23例(70.0%)であった。術式は一期閉鎖術31例、silo造設のみ1例、silo造設後の二期閉鎖術37例であった。分娩様式や術式別の予後に有意差は認めなかった。死亡例は臍帯ヘルニア5例(13.9%)、腹壁破裂1例(3.0%)で、臍帯ヘルニア5例は染色体異常または重症合併奇形を認めた。出生前診断率、生存率は後期で増加していたが有意差は認めなかった。

Publisher：(一社)日本小児外科学会  

【目的】先天性胆道拡張症(以下本症)の出生前診断症例は、閉塞性黄疸や消化管通過障害などの有症状症例には早期対応が求められる。しかし、早期対応の方法に関しては、一律の方針はない。今回、出生前診断され、閉塞性黄疸や消化管通過障害などの症状を認めた本症に対して、生後早期に拡張胆管空腸吻合術(以下本術式)を施行し良好な結果を得たので報告する。【方法】2014年1月から2019年1月までに出生前診断された本症5症例を対象とした。出生後、全症例に本術式後に、二期的根治術(拡張胆管切除、肝管空腸吻合術)を行った。患者背景・手術成績等に関して後方視的に検討し、本術式の妥当性を評価した。【結果】平均在胎週数25週時に胎児超音波検査で指摘された。出生時の平均在胎週数は、38週6日、平均出生体重3,380gであった。戸谷分類は、Iaが3症例でIV-Aが2症例であった。全症例で、出生後早期に本術式を施行した。術後合併症は認めず、症状の改善を得られた。二期的根治手術は4症例に対して平均月例11.5月、平均体重8.8kgで施行した。1症例は、根治手術待機中である。【結論】全症例で生後早期に閉塞性黄疸や消化管通過障害などの症状を認めたため、本術式を行った。術後に症状は改善を認め、4症例に対し二期的根治手術を施行し、良好な結果を得られている。本術式後に二期的根治術を行う方法は、手技が簡単であり、出生早期の児に対しても安全に行うことができ、出生前診断された閉塞性黄疸や消化管通過障害などの症状を認める本症に対する治療方針の1つとして考慮してもよいと思われる。(著者抄録)

Publisher：Journal of Dermatology  

DOI： 10.1111/1346-8138.15156

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Publisher：The Japanese Society of Pediatric Surgeons  

<p><i>Purpose</i>: Cases of prenatally diagnosed congenital biliary dilatation (CBD) with the symptoms of obstructive jaundice and gastrointestinal obstruction should be treated as soon as possible. However, there is no standard protocol for these symptoms. We report the effect and favorable results of choledocheal cyst enterostomy on prenatally diagnosed CBD in the early period after birth.</p><p><i>Methods</i>: CBD was prenatally diagnosed in five patients from January 2014 to January 2019. The symptoms were found in all the patients after birth; thus, two-stage radical surgery (choledocheal cyst excision and hepaticojejunostomy) was performed after choledocheal cyst enterostomy. We retrospectively reviewed and evaluated the patient background characteristics and surgical results.</p><p><i>Results</i>: All cases were detected by fetal ultrasonography at an average gestational age of 25 weeks. The average gestational age at birth was 38 weeks and 6 days, and the average birth weight was 3,380 g. On the basis of Todani's classification, three cases were of type Ia and two cases were of type IV-A. All the patients underwent choledocheal cyst enterostomy in the early period after birth. There were no postoperative complications and the patients' symptoms improved. Two-stage radical surgery was performed in four patients (average age, 11.5 months; average body weight, 8.8 kg). One patient at the time of this writing is waiting for radical surgery. </p><p><i>Conclusions</i>: All the patients showed symptoms in the early period after birth. Thus, we performed cyst enterostomy; after which, the symptoms improved. Two-stage radical surgery was safely performed in four patients and favorable results were obtained. Two-stage radical surgery after choledocheal cyst enterostomy may be considered a choice of treatment for symptomatic patients with prenatally diagnosed CBD.</p>

DOI： 10.11164/jjsps.56.2_188

Publisher：日本膵・胆管合流異常研究会  

DOI： 10.34410/jspbm.43.0_68

Publisher：The Japanese Society of Pediatric Surgeons  

<p>We herein report the first case of neonatal intussusception that we have experienced, which was treated by enema reduction, in our neonatal intensive care unit that supports over 700 cases per year. The patient was a three-day-old full-term male infant. On the second night of life, some red mucus was found mixed with his green feces. The next morning, he was referred to our hospital with a moderate amount of bloody mucous stool. There was no abdominal distention, but a sausage-like mass was palpable on the right side of his abdomen. Abdominal ultrasonography revealed multiple concentric ring signs, on the basis of which he was diagnosed as having ileo-colic intussusception. An enema reduction was attempted 19 h after the onset, and the patient was cured noninvasively. There was no subsequent recurrence. Although intussusception in the neonatal period is rare, the diagnosis of this case was easily made by ultrasonography. In cases of ileo-colic intussusception in full-term neonates with a good general condition and no findings suggesting intestinal necrosis, it seems reasonable to attempt enema reduction safely under preparation of an emergent laparotomy at the time of perforation.</p>

DOI： 10.11164/jjsps.56.6_1021

Publisher：The Japan Society of Computer Aided Surgery  

DOI： 10.5759/jscas.22.180

Publisher：日本外科代謝栄養学会  

&emsp;腸管リハビリテーションプログラムとは, 腸管不全患者の最終的な治療目標である経口あるいは経腸栄養の確立にむけてのさまざまな取り組みである. なかでも, 長期にわたる静脈栄養管理中に起こる腸管不全関連肝障害 (intestinal failure associated liver disease : IFALD) の予防, 治療が患者の生命予後に大きく影響する. FALDの原因としてさまざまな要因が考えられてきたが, 中でも大豆由来脂肪乳剤の影響について研究が行われ, IFALD治療には100％魚油由来脂肪乳剤の有効性が報告されてきた. また, 新規混合脂肪乳剤も欧米では認可され, 臨床経験も集積されてきた. これまでの研究結果から各脂肪乳剤の肝臓に及ぼす影響とその効果について解説する. 加えて, 自施設で行ってきた短腸症候群ラットモデルを用いた各種脂肪乳剤によるIFALDに関する研究では大豆由来脂肪乳剤によるhepatic steatosis, いわゆるNAFLDが起こり, 魚油由来脂肪乳剤ではhepatic steatosisが軽減し, T/T比は正常範囲内であることが明らかになった.

DOI： 10.11638/jssmn.54.6_241

Publisher：(一社)日本小児外科学会  

症例は日齢2、男児。在胎34週の母体超音波検査で左精巣に嚢胞性病変を指摘されていた。在胎37週2日、2,890g、帝王切開分娩で出生した。出生後のMRI検査では10mm大の嚢胞性病変を認め、明らかな充実成分・脂肪成分は指摘されず、単純嚢胞や類表皮嚢胞が疑われ、生後1ヵ月時に手術を施行した。手術所見は精巣実質と境界明瞭な単房性嚢胞であり、嚢胞のみを核出した。病理検査では嚢胞壁内に扁平上皮と一部脂肪を含む線維筋性組織、腸管組織を認め、成熟嚢胞奇形腫の診断であった。陰嚢内精巣腫瘍が出生前診断されることは非常に稀で、発生頻度的には圧倒的に胚細胞腫瘍が多いが、本症例では画像所見上、充実成分を全く認めず、精巣嚢胞が最も疑われた。新生児精巣腫瘍において嚢胞のみの病変であっても胚細胞腫瘍を念頭に診断・治療を行う必要がある。(著者抄録)

Publisher：(一社)日本小児外科学会  

【症例1】14歳男児。主訴は食欲不振・るい痩で、腹部造影CT検査で腹部腫瘤を指摘され紹介となった。下行結腸に完全閉塞を伴う全周性隆起性病変とS状結腸以下に多発polypを認めた。左半結腸切除、D2+傍大動脈リンパ節郭清を行い、stage IVの診断であった。術後化学療法を行ったが、診断から1年後に死亡した。【症例2】10歳男児。主訴は血便・腹痛、腹部造影CT検査で腸重積の診断となり紹介となった。年齢と部位より器質的疾患の存在を考え、緊急で審査腹腔鏡を施行した。横行結腸脾彎曲部の腸重積部分に腫瘤性病変を認め、悪性リンパ腫と判断し局所切除を行った。病理診断は粘液癌で、腹腔鏡下左半結腸切除とD3リンパ節郭清を追加した。Stage IIIbの診断で化学療法を施行し、術後2年無再発生存中である。【結語】小児大腸癌は極めて稀であるが、年長時の遷延する消化器症状では鑑別する必要がある。(著者抄録)

Publisher：World Journal of Pediatric Surgery  

DOI： 10.1136/wjps-2018-000009

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Publisher：Journal of Laparoendoscopic and Advanced Surgical Techniques  

DOI： 10.1089/lap.2018.0195

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Publisher：(一社)日本小児外科学会  

症例1は日齢0の男児。母体の羊水過多を認め、胎児超音波で胃の拡張と下部消化管の描出不良を認め、先天性幽門閉鎖症と診断した。出生時腹部単純X線写真でsingle bubble signを認め、上部消化管造影検査で十二指腸への造影剤通過を認めず、日齢1日目に手術を施行した。症例2は日齢0の女児。母体の羊水過多、胎児超音波で消化管のdouble bubble sign様の像を認めた。出生時腹部単純X線写真及び上部消化管造影検査でもdouble bubble sign様の像を呈し、先天性十二指腸閉鎖症の診断で日齢1日目に手術を施行した。術中所見で幽門部に膜様閉鎖を認め、先天性幽門閉鎖症の診断となった。先天性幽門閉鎖症は術前の画像検査でのsingle bubble signが特徴的だが、典型像を示さない症例もある。先天性十二指腸閉鎖症を疑った場合でも、本疾患を念頭に置き診察・加療を進める必要があると考えられた。(著者抄録)

Publisher：The Japanese Society of Pediatric Surgeons  

<p>We experienced treating two patients with colonic cancer diagnosed at school age and adolescence. Neither patient had a family history of cancer. [Case 1] A 14-year-old boy presented with the chief complaints of appetite loss and weight loss. He was referred to our hospital because of anemia and abdominal tumor noted on computed tomography (CT) images. Enhanced CT showed a circumferential elevated lesion in which the lumen was completely occluded along with multiple polyps. Left hemicolectomy and D2 + paraaortic lymph node dissection were performed. The resected tumor was diagnosed as adenocarcinoma, and the pathological stage was IV. Subsequently, chemotherapy was performed. However, he died one year after the operation owing to recurrence and metastasis. [Case 2] A 10-year-old boy presented with the chief complaints of bloody stool and abdominal pain. He was referred to our hospital owing to a diagnosis of intussusception based on contrast-enhanced CT findings. Emergency laparoscopic exploration was performed to assess the tumor. The tumor was suspected to be malignant lymphoma, and local excision was performed. The pathological diagnosis was mucinous carcinoma. Laparoscopic left hemicolectomy and lymph node dissection of D3 were performed similarly to the treatment of adult colon cancer. The final pathological diagnosis was stage IIIb, and chemotherapy was performed. The patient experienced no recurrence for two years after operation. [Conclusion] Colorectal cancer in children is extremely rare, but it is necessary to identify it correctly when gastrointestinal symptoms are prolonged.</p>

DOI： 10.11164/jjsps.55.5_968

Publisher：The Japanese Society of Pediatric Surgeons  

<p>We herein report two cases of congenital pyloric atresia (CPA). The first case involved a male infant born at 35 weeks of gestation, who had been prenatally diagnosed as having CPA by fetal ultrasonography when the mother underwent a check-up for polyhydramnios. The X-ray and upper gastrointestinal series, which were conducted on the first day after birth, also showed a single-bubble sign. An operation was performed on the second day after birth and membranous atresia was recognized at the pylorus. The second case involved a female infant who had been prenatally diagnosed as having congenital duodenal atresia (CDA) because fetal ultrasonography showed a double-bubble sign when the mother underwent a check-up for polyhydramnios. She was born at 39 weeks of gestation. The X-ray and upper gastrointestinal series, which were conducted on the first day after birth, also showed the double-bubble sign and intestinal malrotation. An operation was performed on the second day after birth. Membranous atresia was recognized at the pylorus, but not at the duodenum. A Ladd operation and membrane resection were performed. CPA is a rare congenital malformation of the gastrointestinal tract. Prenatal ultrasound, X-ray and an upper gastrointestinal series after birth are useful for the preoperative diagnosis. The single-bubble sign is a typical finding of CPA; however, the double-bubble sign is sometimes observed because of stomach contraction. Repeated prenatal ultrasound examinations are important for the exact diagnosis of CPA or CDA. When CDA is suspected, CPA should be considered as the differential diagnosis.</p>

DOI： 10.11164/jjsps.55.1_120

Publisher：The Japanese Society of Pediatric Surgeons  

<p>The patient in the present case was a 2-day-old boy with a cystic lesion in his left testis, which was detected during the prenatal period by a maternal ultrasound examination at 34 weeks of gestation. At 37 weeks of gestational age, the 2,890 g boy was delivered by caesarean section. A cystic lesion of 10 mm size was recognized in a postnatal imaging examination, but no apparent solid or fat component was noted. A simple cyst or an epidermoid cyst was suspected. Considering the possibility of testicular atrophy due to a cystic lesion, we performed an operation at 1 month of age, which revealed a monoclastic cyst with a clear edge, which only nucleated the cyst. Histopathological analysis revealed squamous epithelial cells on the cyst wall surface, fibromuscular tissue, and intestinal tissue. Mature cystic teratoma was the diagnosis based on the examination findings of extirpated tissue. As shown in this case, the prenatal diagnosis of testicular tumors in the scrotum is very rare. In this case, a simple testicular cyst was initially considered to be the most likely diagnosis because no solid component was recognized on imaging after birth. In neonatal patients with testicular tumors, even lesions that only contain a cyst require a careful diagnosis and treatment with the possibility of germ cell tumors kept in mind.</p>

DOI： 10.11164/jjsps.55.7_1187

Publisher：Pediatric Surgery International  

DOI： 10.1007/s00383-018-4326-1

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Publisher：Surgery Today  

DOI： 10.1007/s00595-018-1660-9

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PubMed

Publisher：シュプリンガー・ジャパン(株)  

1984年4月〜2016年12月に総胆管嚢胞(CC)に対する手術を受けた患者を後向きに検討した。手術の結果、早期および晩期の術後合併症、術後経過観察率を分析した。手術時の年齢中央値が4歳3ヵ月のCC患者(男性33名、女性77名)を対象とした。1例が肝管十二指腸吻合術または109例が肝管空腸吻合術を受けた。晩期合併症は、肝内胆管(IHBD)拡張、IHBD結石、癒着性イレウスなどであった。対象患者において胆道癌の発現はなかった。

Publisher：Journal of Pediatric Surgery  

DOI： 10.1016/j.jpedsurg.2017.08.036

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Publisher：Surgery Today  

DOI： 10.1007/s00595-017-1534-6

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PubMed

Publisher：シュプリンガー・ジャパン(株)  

生存および非経口栄養(PN)離脱の予測因子について検討した。治療を受けた短腸症候群(SBS)児(男児6名、女児10名、平均妊娠期間36.7週、平均出生体重2592g)を対象とした。予測因子の解析にはKaplan-Meier解析を使用した。平均残存小腸(RSBL)は34.9cm、死亡した患者6例、非経口栄養(PN)から離脱した患者は9例であった。生存予測因子として、胆汁うっ滞、腸瘻造設、絶対RSBLが30cm未満、予想RSBL率が10%未満に有意差がみられた。PN離脱の予測因子として、胆汁うっ滞、回盲弁(ICV)喪失、絶対RSBLが20cm未満、予想RSBL率が10%未満に有意差がみられた。

Publisher：Pediatric Surgery International  

DOI： 10.1007/s00383-017-4144-x

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PubMed

Language：English   Publisher：John Wiley & Sons Australia, Ltd  

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

Language：English   Publisher：John Wiley & Sons Australia, Ltd  

Language：English   Publisher：John Wiley & Sons Australia, Ltd  

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

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

Language：Japanese   Publisher：(株)北隆館  

小児外科疾患により大量小腸切除を余儀なくされた短腸症候群患児らは、長期絶食下での完全静脈栄養(Total Parenteral Nutrition:TPN)による術後管理が必要となる。TPN管理下におかれた患児では腸管不全関連肝障害(Intestinal Failure Associated Liver Disease:IFALD)を発症し、予後を大きく左右する。当研究グループでは、これまでにIFALDの予防・治療法について、短腸症候群モデルラットを用いて、様々な消化管ホルモンや漢方薬の予防・治療効果をみるために動物実験を行っている。当研究グループはグルカゴンライクペプチド-2(Glucagon-Like Peptide-2:GLP-2)の腸管順応・抗炎症作用についても報告している。近年では腸管粘膜のtight junction蛋白の発現促進に関与しているとの報告があるが、IFALDに対する効果は不透明である。本研究では、IFALDを発症する短腸症候群モデルラットを用いてGLP-2のIFALDに対する予防・治療効果を検討し、IFALDの新規予防・治療法を開発することを目的としている。(著者抄録)

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

Language：Japanese   Publisher：Pediatrics International  

DOI： 10.1111/ped.14518

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

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

Publisher：(株)東京医学社  

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)

Publisher：日本外科代謝栄養学会  

腸管リハビリテーションプログラムとは、腸管不全患者の最終的な治療目標である経口あるいは経腸栄養の確立にむけてのさまざまな取り組みである。なかでも、長期にわたる静脈栄養管理中に起こる腸管不全関連肝障害(intestinal failure associated liver disease:IFALD)の予防、治療が患者の生命予後に大きく影響する。IFALDの原因としてさまざまな要因が考えられてきたが、中でも大豆由来脂肪乳剤の影響について研究が行われ、IFALD治療には100%魚油由来脂肪乳剤の有効性が報告されてきた。また、新規混合脂肪乳剤も欧米では認可され、臨床経験も集積されてきた。これまでの研究結果から各脂肪乳剤の肝臓に及ぼす影響とその効果について解説する。加えて、自施設で行ってきた短腸症候群ラットモデルを用いた各種脂肪乳剤によるIFALDに関する研究では大豆由来脂肪乳剤によるhepatic steatosis、いわゆるNAFLDが起こり、魚油由来脂肪乳剤ではhepatic steatosisが軽減し、T/T比は正常範囲内であることが明らかになった。(著者抄録)

Publisher：(一社)日本コンピュータ外科学会  

Publisher：(株)ニュー・サイエンス社  

小児外科疾患により大量小腸切除を余儀なくされた短腸症候群患児らは、長期絶食下での完全静脈栄養(TPN)による術後管理が必要となる。TPN管理下におかれた患児では腸管不全関連肝障害(IFALD)を発症し、予後を大きく左右する。当研究グループでは、これまでにIFALDの予防・治療法について、短腸症候群モデルラットを用いて、様々な消化管ホルモンや漢方薬の予防・治療効果をみるために動物実験を行っている。当研究グループはグルカゴンライクペプチド-2(GLP-2)の腸管順応・抗炎症作用についても報告している。近年では腸管粘膜のtight junction蛋白の発現促進に関与しているとの報告があるが、IFALDに対する効果は不透明である。本研究では、IFALDを発症する短腸症候群モデルラットを用いてGLP-2のIFALDに対する予防・治療効果を検討し、IFALDの新規予防・治療法を開発することを目的としている。(著者抄録)

Publisher：(株)東京医学社  

Publisher：(株)東京医学社  

＜Key Points＞(1)女児の持続的あるいは間欠的な腹痛を診た場合は卵巣捻転を鑑別する。(2)卵巣捻転であっても消化器症状をメインに呈することもあるため注意を要する。(3)間欠的腹痛に白血球数と炎症反応の上昇を伴う場合は画像診断を考慮する。(4)画像上、卵巣の腫瘍性病変を伴う場合は早急な手術が必要となるため手術可能な施設に速やか紹介する。(著者抄録)

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