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Background: Pancreas divisum (PD), the most common pancreatic anomaly, is caused by the failure of pancreatic bud fusion in the embryo. Although most cases are asymptomatic, it can cause pancreatitis or epigastric pain. We report an unusual case of PD in an infant. Case presentation: The patient was a 9-month-old girl with no pertinent medical history. She had suffered vomiting and diarrhea for 1 week before transfer to our hospital. Her general condition was poor, and abdominal distention was noted. Blood tests revealed microcytic anemia with normal chemical markers. The parents reported no episode of pancreatitis. Ultrasonography revealed massive ascites, which was later found to be bloody. Enhanced computed tomography and magnetic resonance imaging depicted a cystic lesion, approximately 2 cm in size, anterior to the second portion of the duodenum. During exploratory laparotomy, a pinhole was identified on the cyst wall, which was mistakenly identified as a duodenal perforation, and direct closure was performed. Postoperative levels of serum amylase and inflammation markers were elevated, and the amount of ascites increased, impairing oral feeding. The level of pancreatic enzymes in the ascites was high. Imaging studies were repeated, but the cause of pancreatic fistula was not identified. Conservative therapy, including administration of total parenteral nutrition, antibiotics, and octreotide, was initiated, but the situation did not improve. Three months after admission, endoscopic retrograde cholangiopancreatography showed a thick dorsal pancreatic duct communicating with a hypoplastic ventral duct, which was indicative of PD. Contrast medium leaking from the dorsal duct near the minor ampulla revealed the presence of a pseudocyst. Stenting via the minor papilla was impossible because the minor papilla was obstructed. Instead, a stent was inserted into the ventral pancreatic duct. Endoscopic transgastric drainage of the cyst was effective, and the patient was discharged, 7 months after admission. The patient is healthy, but the gastric stent needs to be replaced regularly. Conclusion: In children, PD can manifest with pancreatic pseudocyst that causes pancreatic ascites, even in the absence of pancreatitis. This may be a previously unrecognized manifestation of PD in children, and clinicians need to be aware of it. [ABSTRACT FROM AUTHOR]

Background: A few cases of small omphalocele with umbilical evagination of the bladder have been reported. However, its embryology is yet to be elucidated. Only a few reports have indicated the existence of urachal anomalies and umbilical cysts related to bladder evagination. The incidence of urachal anomalies at birth is reported to be 1 in 5000–8000 live birth, and urachal aplasia is rare. Herein, we report a rare, novel case of urachal aplasia. Case presentation: We encountered a small omphalocele with bladder evagination associated with urachal aplasia for which the neonate underwent surgery one day after birth. The patient was a one-day-old boy with a prenatally diagnosed omphalocele. A fetal magnetic resonance image (MRI) scan (25 weeks of gestation) revealed a 30 × 33 mm (approximately 1.3 in.) cystic lesion which was suspected to be an umbilical cyst. The baby was born vaginally at 38 weeks, weighing 2956 g. An omphalocele (hernial orifice diameter, 4 cm × 3 cm) with bladder prolapse was recognized. After sac excision, the prolapsed bladder was resected and closed with two-layer sutures. In order to secure sufficient bladder capacity, we estimated the minimum residual volume as 21 ml after bladder plasty. The remaining bladder capacity was confirmed to be 30 ml by injecting a contrast dye and saline into the bladder. The neonate had no associated cardiac urogenital or skeletal anomalies. Postoperative course was uneventful. The patient was regularly followed up for two years after surgery and underwent umbilicoplasty. He had no trouble with urinary function. Conclusion: In this case, we experienced extremely rare condition of a small omphalocele with bladder evagination associated with urachal aplasia and reviewed 7 case reports of anomalies similar to those in the present case. Umbilical cord cysts may be an informative indicator of these symptoms in utero. Therefore, ultrasonography scans should be conducted until delivery, despite the spontaneous disappearance of cord cysts. [ABSTRACT FROM AUTHOR]

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

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

Purpose: Late postoperative complications in choledochal cyst (CC) patients are severe problems that affect the quality of life (QOL). We evaluated the postoperative complications and health-related QOL (HRQOL) of CC patients ≥ 18 years of age.Methods: From April 1984 to January 2018, 114 CC patients underwent definitive surgery at our institution. Seventy-nine patients reached ≥ 18 years of age. The HRQOL was assessed using the Japanese version of the SF-36v2. Eight health domain (physical functioning, role-physical, body pain, general health, role-emotional, vitality, mental health, and social functioning) scores and three component summary [physical component summary, mental component summary (MCS), and role-social component summary] scores were compared between the patients with and without complications (C [+] vs. C [-]).Results: Thirty-five patients answered the questionnaires. Twelve patients had postoperative complications. Among the eight domains, the score of general health was significantly lower in C [+] patients than in C [-] patients (p = 0.0488). Among the three component scores, the MCS score in C [+] patients was lower than in C [-] patients without significance (p = 0.0953).Conclusions: The HRQOL of CC patients ≥ 18 years of age was mostly acceptable. However, postoperative complications can impair the sense of well-being and affect the mental health. [ABSTRACT FROM AUTHOR]