Case Series of Endoscopic Papillary Balloon Dilation for Children with Common Bile Duct Stones and a Review of the Literature.

Background: Endoscopic sphincterotomy (EST) has been employed for the endoscopic treatment of common bile duct stones (CBDSs) and has been reported to have a high success rate for stone removal. However, EST is associated with a risk of bleeding, perforation, and sphincter of Oddi function disruption. To avoid these risks, endoscopic papillary balloon dilation (EPBD) is an option for CBDS. Sphincter of Oddi function preservation decreases long-term biliary infection and gallstone recurrence. EPBD may have advantages in children who require a long follow up. However, there have been few reports on pediatric cases, particularly in infants. Methods: From September 2017 to December 2023, we performed EPBD for four pediatric CBDSs. The patients were aged from 5 months to 8 years, including two infants aged 5 and 6 months. Furthermore, we reviewed the stone removal rate and complications of 545 ESTs performed at high-volume centers and 13 EPBD-reported cases in children with CBDSs. Results: CBDSs of all patients who underwent EPBD in our institution were successfully removed. No bleeding or perforation was noted; pancreatitis was observed in three patients. In an analysis of 545 ESTs in children, the stone removal rate was high, ranging from 83% to 100% (mean 96%). The incidence of pancreatitis was 0-9.6% (mean 4.4%), and the grade of pancreatitis was almost mild. The bleeding frequency was 1.3-5.4% (mean 2.7%). With regards to the grade of bleeding, seven cases were mild (64%) and four were moderate (36%). Compared with adults who underwent EST, the frequencies of pancreatitis and bleeding were almost equal in children; however, in children, once bleeding occurs, it has a higher risk of leading to blood transfusion. Stone removal via EPBD in children has a 100% success rate. Pancreatitis was responsible for all complications were related; its frequency was 46% (6/13 patients, including five mild cases and one moderate case), which is higher than that of EST and adult cases who underwent EPBD. In most children with pancreatitis, pancreatic enzyme levels returned to normal within 2-3 days following EPBD, and no severe cases caused by EPBD were reported. Conclusions: CBDS removal via EPBD in children has a high success rate with very low risk of bleeding and perforation. Although pancreatitis frequently occurs, most cases are mild. Sphincter of Oddi function preservation via EPBD is expected to prevent long-term stone recurrence and biliary tract infection, and EPBD is considered to be an effective method for CBDS removal in children.

Surgical outcomes of very-early-onset ulcerative colitis: retrospective comparative study with older pediatric patients.

PURPOSE: The study compares the surgical outcomes of very-early-onset ulcerative colitis (VEO-UC), which is a rare disease diagnosed in pediatric patients < 6 years, with those of older pediatric patients with ulcerative colitis (UC). METHODS: A retrospective observational study of 57 pediatric patients with UC was conducted at a single center. The study compared surgical complications and postoperative growth between the two groups. RESULTS: Out of the 57 patients, 6 had VEO-UC, and 5 of them underwent total colectomy. Compared with the surgical cases of older patients with UC (n = 6), the rate of postoperative complications in patients with VEO-UC (n = 5) was not significantly different, except for high-output ileostomy (80% vs. 0% at 3 weeks postoperatively, p = 0.02). The rate of postoperative central venous catheter (CVC) placement at > 90 days was higher in patients with VEO-UC (100% vs. 17%, p = 0.02). The median change in the Z-score of height before and 2 years after colectomy was not significantly different between VEO-UC and older patients (1.1 vs. 0.3, p = 0.13). CONCLUSION: With regard to complications and outcomes, total colectomy for VEO-UC patients and that for older pediatric UC patients is comparable. However, high-output ileostomy and the long duration of CVC placement may pose management challenges.

Impaired Aortic Growth in Neuroblastoma Patients After Intensive Treatment.

PURPOSE: This study evaluated the abdominal aortic diameter in high-risk neuroblastoma (NB) patients and the risk of aortic narrowing following intensive treatment. METHODS: We measured the aortic diameter at four specific levels of the abdominal aorta (diaphragmatic crus, celiac axis, and the root of the superior (SMA) and inferior (IMA) mesenteric arteries) on contrast CT scans. The control group consisted of 56 children with non-oncologic disorders, while the NB group included 35 patients with high-risk abdominal NB. We used regression analysis of age and aortic diameter to determine the regression formula for each level in each group and performed intergroup comparisons using t-test. RESULTS: We evaluated a total of 160 contrast-enhanced CTs performed in the 35 eligible cases. The aortic diameter of pretreated NB patients was not significantly different from the controls. After receiving any treatment, the aortic diameter was significantly smaller in the NB group (p < 0.01 each). Patients who underwent radical surgery, particularly gross total resection (n = 26), had smaller aortic diameters at all levels compared to controls (p < 0.01 each). Patients treated with radiotherapy (RT) had smaller aortic diameters than controls. External beam radiotherapy (EBRT) patients (n = 24) had smaller aortic diameters at all levels except the celiac axis (crus, SMA, IMA; p < 0.01 each), and intraoperative radiotherapy (IORT) ± EBRT patients (n = 5) had smaller aortic diameters at all levels (p < 0.01 each). CONCLUSION: Patients with NB may experience impaired development of the abdominal aorta after multimodal therapy, particularly after RT. Close observation and long-term follow-up is essential to monitor for catastrophic vascular complications. LEVEL OF EVIDENCE: LEVEL III.

Establishment of a quantitative assessment model and web-based calculation tool for the skeletal muscle index in children.

BACKGROUND & AIMS: The skeletal muscle index (SMI) is widely used in adults. The reference values for SMI in children are inadequate and require validation in pediatric patients for clinical usefulness. Therefore, this study developed a quantitative assessment model for SMI in children using standard deviation (SD) curves and validated the model's utility and generalizability. METHODS: We examined three compartments of the abdominal skeletal muscle region. SMI was calculated as skeletal muscle area divided by height squared for each compartment (PMI, psoas muscle index; PSMI, paraspinal muscle index; TSMI, total skeletal muscle index). The optimal model was generated using random grouping methods (training and testing), polynomial regression analysis, and the mean squared error evaluation methods. The generated model was validated with previously published SMI data and clinical data of patients with inflammatory bowel disease. RESULTS: The data of 474 children were analyzed. The previously reported SMI reference values overlapped well with our model. In patients with inflammatory bowel disease, the mean (SD) Z-scores for SMI were low in boys (PMI, -1.15 [1.11]; PSMI, -1.31 [1.07]; TSMI, -0.84 [0.91]) and girls (PMI, -1.22 [1.08]; PSMI, -1.44 [1.19]; TSMI, -0.74 [1.16]). Furthermore, SMI was positively correlated with body weight, body mass index, and serum albumin level, a nutritional marker, and negatively correlated with erythrocyte sedimentation rate, an inflammatory marker. CONCLUSION: We established a quantitative assessment model for SMI and validated the model's generalizability and clinical usefulness. We generated an easy-to-use calculation tool for Z-scores from skeletal muscle area obtained from computed tomography images, age, and height information; it has been made publicly available (http://square.umin.ac.jp/ped-muscle-calc/index.html).

Outcomes of adult native liver survivors with biliary atresia: the current situation in Japan.

PURPOSE: This study aimed to elucidate the difficulties faced by adult native liver survivors with biliary atresia (BA) in Japan. METHODS: A single-center, retrospective, observational study of 57 adult patients with BA was conducted. The clinical course of BA was compared between native liver survivors and non-survivors who reached adulthood. Indications and outcomes of liver transplantation (LT) among non-survivors were assessed. RESULTS: A significantly larger portion of non-survivors (n = 10) met the criteria for LT (p < 0.001) and received treatment for portal hypertension after reaching 20 years of age (p < 0.01) compared with the survivors. Causes of death included liver cirrhosis (n = 8), graft failure of living donor liver transplantation (LDLT) (n = 1), and hepatocarcinoma (n = 1). Two of the non-survivors who died of liver cirrhosis had no indication for LT because of alcohol dependence and uncontrolled infection. An appropriate donor candidate could not be found for the five patients who opted for LDLT. All six patients waitlisted for deceased donor liver transplantation (DDLT) died after a median waiting period of 17 months. CONCLUSION: Adult BA patients in Japan have limited options for LT, mainly owing to low donor candidate availability for LDLT and a low prevalence of DDLT.

Frequency of infiltrating regulatory T-cells in the portal tract of biliary atresia.

PURPOSE: Immunological abnormalities have been hypothesized as a pathogenesis of biliary atresia (BA). We previously investigated the frequency and function of circulating regulatory T-cells (Tregs) and reported no differences compared to controls. However, the local Treg profile remains uncertain. We aimed to investigate the frequency of Tregs in BA liver tissues. METHODS: The number of lymphocytes, CD4+ cells, and CD4+FOXP3+ Tregs infiltrating the portal tract and the percentage of Tregs among CD4+ cells of BA and control patients were visually counted. The correlation between these data and clinical indicators was also examined. RESULTS: The number of lymphocytes, CD4+ cells, and CD4+FOXP3+ Tregs was higher in the BA group. However, the percentage of Tregs among CD4+ cells was similar in both groups. Each parameter was correlated with serum γ-GTP, but there was no clear association with liver fibrosis, jaundice clearance, and native liver survival. CONCLUSION: The number of Tregs infiltrating the portal tract was higher in BA patients. However, the infiltration of lymphocytes was also generally increased. Tregs appear to be unsuccessful in suppressing progressive inflammation in BA patients, despite recruitment to local sites. Investigation of Treg function in the local environment is warranted.

Gastric tumor mimicking bronchial tissue associated with a laryngotracheoesophageal cleft: a case report.

BACKGROUND: Laryngotracheoesophageal cleft (LTEC) is a rare disease in which the larynx and trachea communicate posteriorly to the esophagus. It is often associated with other congenital malformations, particularly gastrointestinal anomalies. Herein, we report a case of LTEC associated with a gastric polypoid lesion in bronchial tissue. CASE PRESENTATION: A gastric mass was detected in a male fetus since week 21 of gestation using fetal ultrasonography. Esophagogastroduodenoscopy performed after birth revealed a pedunculated polypoid lesion of the gastric fornix. The patient experienced frequent vomiting and aspiration pneumonia, which persisted after nasoduodenal tube feeding. Communication between the airway and esophagus was suspected. Laryngoscopy performed 30 days later revealed an LTEC (type III). Partial gastrectomy was performed when the patient was 93 days of age. Histopathological examination revealed tumor consisting of cartilage tissue covered with a layer of respiratory epithelium. CONCLUSION: The gastric tumor associated with LTEC exhibited structures mimicking bronchial tissue. LTEC occurs because of foregut maldevelopment, and the tumorous respiratory tissue in the stomach may have been formed from the same abnormal foregut development event as LTEC.

Establishment and validation of reference values for abdominal skeletal muscle compartments in children.

BACKGROUND & AIMS: Sarcopenia, resulting from skeletal muscle loss, is a serious concern in children, for whom nutrition and physical activity are essential for growth. In sarcopenia research, the skeletal muscle mass is often obtained from computed tomography (CT) cross-sectional images in both adults and children; however, the method of evaluating skeletal muscle using CT, especially the area range to be measured, has not yet been validated in children. The aim of this study is to establish reference values for three compartments of the abdominal skeletal muscle area (aSMA) obtained from cross-sectional CT images, and to validate the differences among them by assessing correlations with physical development. METHODS: We conducted a single-centre, retrospective, cross-sectional study and included patients aged 1-17 years who underwent abdominal CT at Chiba University Hospital from 2007 to 2020. Patients with chronic diseases such as malignant tumours and inflammatory bowel disease were excluded from the analysis. aSMA was obtained from CT cross-sections at the lumbar L3-L4 intervertebral disc level by dividing it into three compartments: the psoas muscle area (PMA), paraspinal muscle area (PSMA), and total skeletal muscle area (TSMA). Quartile regression curves by sex and muscle compartment were generated using non-crossover and nonparametric regression quantile estimation. The correlation between each compartment of aSMA with height and weight was assessed using Spearman's rank correlation coefficient. RESULTS: We analysed the abdominal CT images of 593 children (male: n = 335, female: n = 258) and generated curves predictive for the 5th, 25th, 50th, 75th, and 95th percentiles for each compartment of aSMA by sex. In patients aged 13 years and older, boys had significantly larger aSMA areas than girls in all three compartments. Among the three compartments, PSMA had the strongest correlation coefficient with height and weight for both boys and girls. CONCLUSIONS: We generated quantile regression curves for three aSMA compartments obtained from cross-sectional CT images and established reference values in children. PSMA values were most strongly correlated with height and weight among the three aSMA compartments.

Recurrent massive bleeding from a small intestinal arteriovenous malformation after surgery for biliary atresia in an infant: a case report and literature review.

BACKGROUND: Small intestinal arteriovenous malformation (AVM) can cause bleeding. Most small intestinal AVMs occur during adulthood, rarely in infancy. We report a case of an infant with hemorrhage due to small intestinal AVM early and recurrently after Kasai portoenterostomy (PE) for biliary atresia (BA). CASE PRESENTATION: A 51-day-old male infant was admitted to our institution for obstructive jaundice. Laparotomic cholangiography revealed BA (IIIb1µ), and Kasai PE was performed at 60 days of age. On postoperative day 17, he developed massive melena and severe anemia. Contrast-enhanced computed tomography (CT) revealed that the jejunum around the PE site was strongly enhanced with enhancing nodules in the arterial phase, and a wide area of the Roux limb wall was slightly enhanced in the venous phase. As melena continued, emergency laparotomy was performed. There were no abnormal macroscopic findings at the PE site except for a clot in the Roux limb 5 cm away from the PE site, and the Roux limb was resected 5 cm. On further investigation, a red spot was detected on the jejunal serosa 30 cm away from the Roux-en-Y anastomosis site. PE and wedge resection for the red spot were performed. Histopathologically, both specimens indicated AVM. He was jaundice-free 65 days after the first surgery. However, at 7 months of age, he developed massive melena again. Contrast-enhanced CT and upper gastrointestinal endoscopy revealed no bleeding lesions. Hemorrhagic scintigraphy showed a slight accumulation at the hepatic hilum prompting an emergency surgery. Intraoperative endoscopy detected a bleeding lesion at the PE site, and the Roux limb was resected (approximately 6 cm). Intraoperative frozen section analysis of the stump of the resected jejunum revealed no abnormal vessels. PE was performed, and permanent section analysis revealed an AVM in the resected jejunum. The postoperative course was uneventful without re-bleeding. CONCLUSIONS: We experienced a case of recurrent massive bleeding from small intestinal AVM in an infant after surgery for BA. Intraoperative endoscopy and frozen section analysis helped identify the bleeding lesion and perform a complete resection of the small intestinal AVM, even after surgery, in the infant.

Current status and future challenges in the endoscopic management of non-variceal upper gastrointestinal bleeding in children.

PURPOSE: This study aimed to elucidate the characteristics of non-variceal upper gastrointestinal bleeding (NVUGIB) in children and the outcomes of endoscopic hemostasis (EH) performed by pediatric surgeons. METHODS: We retrospectively reviewed the medical records of pediatric patients with NVUGIB who had undergone esophagogastroduodenoscopy (EGD) at our hospital, between December 2006 and March 2020. RESULTS: Thirty-six patients were included. The most common endoscopic diagnosis was gastritis (28%), followed by gastric ulcer (25%) and duodenal ulcer (25%). Thirty patients (83%) had underlying diseases, including a recent viral infection (39%), followed by Helicobacter pylori infection (14%). Fourteen patients (39%) underwent EH, exhibiting a high initial rate of hemostasis (100%). This was achieved in 12/14 patients by clipping. One patient was treated using a combination of a hypersaline and epinephrine (HSE) injection and coagulation therapy, as the ulcer exceeded the width of the hemoclips. Rebleeding occurred in one patient (7.1%) initially treated with pure ethanol injections. The partial view of the pediatric duodenum prevented the placement of hemoclips; hence, the patient was treated using a combination of HSE and coagulation therapy. CONCLUSION: Clipping, injection, and/or coagulation therapy can lead to high success rates for hemostasis, considering the anatomical characteristics in a pediatric population.

Sindbis viral structural protein cytotoxicity on human neuroblastoma cells.

PURPOSE: Oncolytic viral therapy for neuroblastoma (NB) cells with Sindbis virus (SINV) is a promising strategy for treating high-risk NB. Here, we evaluated the possibility of using SINV structural proteins as therapeutic agents for NB since UV-inactivated SINV could induce cytopathogenic effects. METHODS: The cytotoxicity of UV-inactivated SINV toward human NB cell lines NB69, NGP, GOTO, NLF, SK-N-SH, SH-SY5Y, CHP134, NB-1, IMR32, and RT-BM-1 were analyzed. Apoptosis was confirmed by TUNEL assays. To determine the components of SINV responsible for the cytotoxicity of UV-inactivated SINV, expression vectors encoding the structural proteins, namely capsid, E2, and E1, were transfected in NB cells. Cytotoxicity was evaluated by MTT assays. RESULTS: UV-inactivated SINV elicited more significant cytotoxicity in NB69, NGP, and RT-BM-1 than in normal human fibroblasts. Results of the transfection experiments showed that all NB cell lines susceptible to UV-inactivated SINV were highly susceptible to the E1 protein, whereas fibroblasts transfected with vectors harboring capsid, E1, or E2 were not. CONCLUSIONS: We demonstrated that the cytotoxicity of the UV-inactivated SINV is due to apoptosis induced by the E1 structural protein of SINV, which can be used selectively as a therapeutic agent for NB.

Risk factors for unsuccessful atropine therapy in hypertrophic pyloric stenosis.

BACKGROUND: I.v. atropine (IA) for infantile hypertrophic pyloric stenosis (IHPS) is a good alternative to pyloromyotomy but has not been broadly accepted. The lower success rate is one of the greatest disadvantages of IA. We investigated the risk factors for unsuccessful results following IA for IHPS. METHODS: Medical records of patients with IHPS who were admitted to Kimitsu Chuo Hospital between 2002 and 2016 and were initially given atropine sulfate were retrospectively reviewed. Atropine was given i.v. (0.1 mg/kg/day in eight divided doses). Oral feeding of milk was started with a small amount and increased in a stepwise fashion to full feed. IA therapy was defined as unsuccessful in the presence of projectile vomiting more than three times a day or intolerance to a predetermined amount of milk. RESULTS: Of the 48 patients with IHPS, 33 patients were successfully treated with IA and 15 patients needed surgical intervention. On univariate analysis the risk factors for unsuccessful IA therapy were younger age, lower bodyweight, and shorter duration of symptoms before diagnosis. On multivariate analysis age at diagnosis < 30 days was the only significant risk factor for unsuccessful IA therapy (OR, 5.7 l P = 0.03). CONCLUSIONS: Age at diagnosis < 30 days is a risk factor for unsuccessful IA therapy in IHPS. This might be considered when IA therapy is used for neonates with IHPS.

Safety and efficacy of mucous fistula refeeding in low-birth-weight infants with enterostomies.

PURPOSE: To investigate the safety and efficacy of mucous fistula refeeding (MFR) in low-birth-weight infants. METHODS: Between December 2006 and December 2018, medical records of low-birth-weight infants who underwent small bowel enterostomy formation in the neonatal period and subsequent stoma closure at our institution were retrospectively reviewed. Patients were assigned to "refeeding" (RF) and "non-refeeding" (NRF) groups, which were compared for patient characteristics and clinical outcomes. We also cultured the proximal stoma output over time in the RF group and reviewed changes in the flora to evaluate the safety of refeeding. RESULTS: In the RF group, compared with that before refeeding, there was significantly more rapid weight gain after refeeding (17.7 vs 10.6 g/day; P = 0.002). Median total time of parenteral nutrition (PN) was 25 and 87 days in the RF and NRF groups, respectively (P = 0.001). The number of patients who developed PN-associated liver disease (PNALD) was smaller in the RF group (P = 0.12). No complications of MFR were noted and no pathogenic bacteria were cultured. CONCLUSION: MFR was able to diminish the need for PN, which potentially decreased the incidence of PNALD, and was safe as there were no complications of the refeeding process.

Recurrent intestinal ischemia following surgery for gastric and duodenal perforations: a case report.

BACKGROUND: Extremely low birth weight (ELBW) is a risk factor for various gastrointestinal complications. In the recent decades, advances in medicine have increased the survival of ELBW infants with necrotizing enterocolitis (NEC). To our knowledge, there have been no reports of neonates or infants developing simultaneous gastric and duodenal perforations and later developing NEC. We report a case of an extremely low birth weight infant (ELBWI) who developed both gastric and duodenal perforations at the same time and developed NEC after operation for gastric and duodenal perforations. CASE PRESENTATION: The patient was a female infant with ELBW who developed both gastric and duodenal perforations at the same time and developed NEC after operation for gastric and duodenal perforations. After birth, endotracheal intubation was performed. However, barotrauma occurred during positive pressure ventilation, resulting in a large area of emphysema in the left lower lung field, leading to collapse of the left lung. This giant bulla may have compressed the pulmonary vein, possibly resulting in pulmonary venous thrombosis (PVT). This episode could have triggered simultaneous gastric and duodenal perforations. In addition, we hypothesized that PVT caused acute arterial ischemia, leading to the development of NEC. The infant was started on heparin for anticoagulation. Later, the infant developed non-immunoglobulin E (IgE)-mediated gastrointestinal food allergies (non-IgE-GI-FAs). The giant bulla associated with barotrauma might have caused PVT, resulting in arterial emboli and multiple simultaneous gastrointestinal perforations. CONCLUSIONS: Anticoagulation therapy with heparin for acute arterial thrombosis is effective for preventing the development of short bowel syndrome. Duodenal and intestinal surgery probably acted as risk factors for the subsequent development of non-IgE-GI-FAs. The infant had been stabilized at the time of writing this report.

Neonates undergoing gastrointestinal surgery have a higher incidence of non-IgE-mediated gastrointestinal food allergies.

PURPOSE: We aimed at investigating the incidence and risk factors of non-IgE-mediated gastrointestinal food allergies (non-IgE-GI-FAs) in neonates and infants. METHODS: A total of 126 infants who underwent neonatal gastrointestinal surgeries were grouped into those with (n = 13) and those without an onset of non-IgE-GI-FAs (n = 113). The characteristics of the two groups (e.g., birth weight, delivery type, small intestinal surgeries, and pre-/postoperative nutrition) were compared. Small intestinal surgeries were classified into those with and those without full-layer invasion of the small intestine. For the statistical analysis, postoperative nutrition was classified into breast milk only, formula milk, and elemental diet only. RESULTS: Except for full-layer surgical invasion of the small intestine and the period of parenteral nutrition, no significant differences were found between the two groups. Surgery with full-layer invasion was a risk factor of non-IgE-GI-FAs (odds ratio (OR) 10.70, 95% confidence interval (95% CI) 2.11-54.20; p = 0.004). Formula milk after surgery was a risk factor of non-IgE-GI-FAs when compared to breast milk (OR 5.65, 95% CI 1.33-24.00; p = 0.019). CONCLUSION: Neonates undergoing gastrointestinal surgery have a higher incidence of non-IgE mediated gastrointestinal food allergies. We recommend that formula milk should not be administered to newborns who underwent neonatal gastrointestinal surgeries with full-layer invasion of the small intestine.

Oncolytic viral therapy for neuroblastoma cells with Sindbis virus AR339 strain.

PURPOSE: With current treatment regimens, high-risk neuroblastoma (NB) remains largely incurable. Oncolytic viral therapy uses replication-competent viruses, like Sindbis virus (SINV), to kill cancers. The SINV AR339 strain is blood borne and relatively non-virulent. We evaluated the feasibility of SINV AR339 for treating human NB. METHODS: The cytotoxicity and viral growth of SINV AR339 were evaluated for five human NB cell lines, SK-N-SH, IMR-32, LAN-5, GOTO, and RT-BM-1. SINV-induced apoptosis was confirmed by TUNEL assays and PARP-1 cleavage. In vivo effects of SINV on neuroblastoma cell xenografts in nude mice were assessed by intratumoral or intravenous SINV inoculation. RESULTS: In five human NB cell lines, SINV infections induced remarkable cytotoxicity. The mRNA expressions of anti-apoptotic genes, Bcl-2 and Bcl-xL, in LAN-5 and RT-BM-1, which were less sensitive to SINV infection, increased in response to SINV infection, while the other NB cell lines sensitive to SINV infection failed to respond. In nude mice, intratumoral and intravenous SINV inoculations caused significant regression of NB xenograft tumors. CONCLUSION: Our results suggested that SINV AR339 was significantly oncolytic against human NB. Thus, SINV showed promise as a novel therapy for treating NB.

Etiology of neonatal gastric perforation: a review of 20 years' experience.

PURPOSE: Gastric perforation (GP) of the newborn is a rare, serious, and life-threatening problem, and its etiology remains unclear. Although historically GP has often been described as "spontaneous'', some cases are non-spontaneous. The aim of the present study was to review cases of GP and to discuss its etiology in a single prefecture in Japan over a period of 20 years. METHODS: Eleven cases with GP that underwent surgery in 4 institutions in the Chiba Prefecture from 1991 to 2010 were reviewed and divided into 2 groups: the early (1991-2000, n = 7) and late (2001-2010, n = 4) groups. RESULTS: No factors were observed that could have caused GP other than malformations associated with distal obstruction (3 midgut volvulus, 1 jejunal stenosis, 1 diaphragm eventration). Distal obstruction was present in 1 case in the early group and all 4 cases in the late group (p = 0.015). While the incidence of GP did not change over the 20-year period reviewed, the incidence of GP without distal obstruction significantly decreased in the late group. CONCLUSION: The proportion of patients with GP and distal obstruction increased and true "spontaneous" cases of GP decreased over time. The possible presence of distal obstruction should be evaluated during surgery for GP.

Use of preoperative, 3-dimensional magnetic resonance cholangiopancreatography in pediatric choledochal cysts.

BACKGROUND: Standard choledochal cyst (CC) operations involve dilated extrahepatic bile duct excision followed by biloenterostomy. However, biliary variants and associated intrahepatic bile duct (IHBD) stenoses or dilatations triggering postoperative sequelae require additional procedures. The usefulness of preoperative 3-dimensional magnetic resonance cholangiopancreatography (3D MRCP) and virtual cholangioscopy (VES) for observing biliary morphology and pancreaticobiliary maljunction (PBM) was evaluated. METHODS: In 16 pediatric CC patients (age range, 4 months to 9 years; median, 3 years), visualization of PBM and aberrant bile duct anatomy and IHBD morphology at the hepatic hilum (HH), umbilical portion (UP), and posterior branch (POST) were compared between 3D-MRCP and intraoperative cholangiography (IOC). VES and intraoperative cholangioscopy (IOS) findings were compared. RESULTS: HH, UP, and POST visualization rates were 100%, 94%, and 94%, respectively, by 3D-MRCP, and 100%, 69%, and 69%, respectively, by IOC. IHBD stenosis detection rates at each region were 38%, 13%, and 13%, respectively, by 3D-MRCP, and 25%, 0%, and 9%, respectively, by IOC. IHBD dilatation detection rates at each part were 75%, 47%, and 60%, respectively, by 3D-MRCP, and 88%, 82%, and 91%, respectively, by IOC. PBM was confirmed in 56% and 93% of cases on 3D-MRCP and IOC, respectively. Both 3D-MRCP and IOC showed biliary variants in 5 cases (31%). VES showed membranous strictures at HH, UP, and POST in 6, 2, and 2 cases, respectively, whereas IOS did so at HH in 4 cases and POST in 2. CONCLUSION: Preoperative 3D-MRCP and VES accurately depict biliary morphology, allowing concrete operative planning in pediatric CC patients, complementing IOC and IOS.