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Congenital hyperinsulinism is a rare disease, and the newly developed 18 fluoro-L-dihydroxyphenylalanine-positron emission tomography (18F-DOPA PET) examination can detect hyperplastic lesions. Our purpose was to report the results of a nationwide survey on surgical treatment of congenital hyperinsulinism in Japan. A questionnaire was sent to the 159 accredited and affiliated training institutes certified as pediatric surgical institutes by the Japanese Association of Pediatric Surgeons, asking if they had encountered patients who underwent surgical treatment for congenital hyperinsulinism after 18F-DOPA PET examination from 2000 to 2017. Six institutes answered that they had treated such cases, and the total number of cases was 14. 18F-DOPA PET examination detected the focal lesion in 12 of the 14 cases. 18F-DOPA PET examination could accurately determine the site of the hyperplastic lesion in the pancreas in 11 (91.7%) of the 12 cases. All cases underwent surgical resection of the hyperplastic lesion at under 2 years of age. Surgical resection of a focal hyperplastic lesion in the pancreas was a safe and effective treatment if the hyperplastic lesion was a focal lesion. However, it is necessary to check the exact distribution of the lesion by intraoperative pathologic examination of frozen sections.

Owing to several therapeutic advancements, more patients with biliary atresia now survive into adulthood while retaining their native liver. However, the optimal strategy for long-term management of such patients remains unclear. Aiming to establish the current management strategies, we reviewed previous reports of long-term outcome of BA who underwent surgery at our institution as well as the relevant literature, focusing particularly on the treatment of late complications. Approximately 30–40% of long-term survivors of biliary atresia who retain their native liver exhibit late sequelae such as cholangitis and portal hypertension. Early and appropriate intervention with Kasai portoenterostomy is essential for ensuring long-term survival with good quality of life. In our hospital, the current standard for Kasai portoenterostomy involves dissecting the fibrous remnants along the porta hepatis, just on the level of the liver capsule. Cholangitis is an important late complication in biliary atresia, and the possibility of mechanical obstruction of the biliary drainage route or deformity of the intrahepatic bile ducts with or without gallstones should be thoroughly evaluated in patients with intractable cholangitis. Regarding portal hypertension, appropriate interventions such as endoscopic variceal treatment and partial splenic embolization are considered to provide good quality of life when hepatic function is preserved. Appropriate therapeutic management is strongly recommended in selected patients with late complications.

Kasai portoenterostomy (KP) is the primary procedure for biliary atresia (BA). However, due to reports of poor outcomes of KP, primary liver transplantation (LT) is preferred in selected cases. In Japan, primary LT is limited with no study reports details, particularly regarding the indications. We conducted the first nationwide survey to assess the status of primary LT in Japan. Questionnaires were sent to institutions where procedures other than KP were performed as the initial procedure for BA and registered to the Japan Biliary Atresia Registry. Parameters related to procedure selection were analyzed. Of 2895 patients registered (1989–2013), primary LT (n = 15) or exploratory laparotomy (EL) followed by LT (n = 9) without KP was performed in 24 cases (0.8%). The main reason for primary LT was late diagnosis, and for EL followed by LT, lack of fibrous tissue at the porta hepatis (ο-type). The prognoses of LT without KP was good. Non-KP initial procedures were limited. Clear criteria regarding the timing of diagnosis or patient condition could not be determined. Reasons for not initially selecting KP varied, but late diagnosis was predominant. Further study is needed to create guidelines for the initial treatment of BA patients.

Some patients with cystic biliary atresia (BA) achieve exceptionally good postoperative courses. Early differentiation of pediatric patients with the favorable-type disease can lead to beneficial long-term postoperative management. We examined whether the hepatic hilum morphology affects long-term prognosis in type I/I cyst BA, atresia of the common bile duct with/without a cyst. Of 253 BA patients identified since 1972, 40 were classified as having type I/I cysts and were divided into two subtypes according to hepatic duct size (subtype α: n = 18; duct diameter, ≥1 mm and subtype β: n = 22; duct diameter

Long-term survivors of biliary atresia (BA) sometimes experience liver dysfunction. We evaluated the prognostic factors for long-term native liver survival (NLS) in BA patients after the Kasai procedure. This study included 67 patients with jaundice disappearance after the Kasai procedure performed between 1972 and 1995, and NLS for over 10 years. We retrospectively evaluated the clinical parameters, including the type of BA, age at the Kasai procedure, medical conditions, and treatments. The adjusted odds ratios (aOR) were obtained for 20-year NLS using logistic regression analysis. The median age of the patients at the Kasai procedure was 63 days. Of the 67 study patients, 62 patients (92.5 %) had jaundice-free NLS at the age of 20 years, 4 patients died before the age of 20 years from liver failure, and 1 patient underwent living related liver transplantation. The presence of gastro-esophageal varices requiring endoscopic injection sclerotherapy was a significant factor (aOR 33.8; p = 0.0033), while hypersplenism and cholangitis were not identified as significant factors. The existence of symptomatic portal hypertension would influence long-term NLS in BA patients after the Kasai procedure. In such patients, accurate evaluation of hepatic function and adequate treatment for sequelae are needed.

This study aimed to assess outcomes of liver transplantation (LTx) in patients with biliary atresia (BA). The Kasai procedure was performed for 358 patients at Tohoku University Hospital between January 1955 and December 2013; 64 (17.9 %) required LTx. These 64 patients were divided into 4 groups according to their age at the time of transplantation: Group 1, aged

The Kasai redo surgery is important for treating biliary atresia. In the era of liver transplantation (LTx), pediatric surgeons must accurately select patients for redo surgery and ensure that potential LTx can be performed later. Although optimal timing for redo varies among cases, appropriate timing is essential. We reviewed the significance, optimal timing, operative procedures, and indications of Kasai redo surgery. Between 1989 and 2011, 2,630 patients were registered in the Japanese Biliary Atresia Registry (JBAR), and the data collected from JBAR regarding Kasai redo surgery were analyzed. Patients were divided into two groups, Group 1 (1989–1999, n = 1,423) and Group 2 (2000–2011, n = 1,207). The redo incidence significantly reduced in Group 2. Although no significant difference was found in the native liver jaundice-free survival rates between the two groups, the overall survival rate at initial registry was significantly higher in Group 2. This may be because of the limited number of patients selected for redo and increased availability of early LTx. Patients who achieved sufficient bile drainage following the initial Kasai surgery but developed sudden bile flow cessation were the best candidates for Kasai redo surgery; it should be performed only once for this subset.

We elucidated the life-threatening risk factors for intestinal failure (IF) and characterized the role of intestinal transplantation (ITx) in affected patients.We conducted a retrospective review of 38 patients with short bowel (SB) and 19 with motility disorders (MD). The SB patients were divided into three categories according to the length of their residual small bowel and the presence of the ileocecal valve. The four disease subcategories were grouped into two categories: low-risk category (mild and moderated SB) and high-risk category (extensive SB and MD). The age at the introduction of parenteral nutrition (PN) was1 year in 50 patients (infant group, IG) and 1-15 years in 7 patients (pediatric group, PG).Enteral autonomy was rarely achieved in the high-risk category (p0.0001). IG was associated with a higher incidence of developing intestinal failure-associated liver disease (IFALD) (p = 0.004). Eight patients died, due to IFALD in four, sepsis in three and acute heart failure in one. Twenty-eight patients (49 %) are currently alive without PN, including four after ITx.The treatment of high-risk IF is still challenging. Inclusion of ITx in appropriate timing, along with aggressive medical, nutritional and surgical management, may reduce advanced morbidity and mortality of high-risk IF.