Analysis of the Clinical Characteristics of Spontaneous Bile Duct Perforation in Children

OBJECTIVE: This study aimed to explore the etiology, clinical features, diagnosis, and treatment of spontaneous bile duct perforation (SBDP) in children. METHODS: The clinical data of children with SBDP who were admitted to Wuhan Children's Hospital between January 2014 and January 2020 were retrospectively analyzed. RESULTS: In all, 28 cases of children with SBDP (male, 28.6%; female, 71.4%; male-to-female ratio, 1:2.5; average age, 2.15 years) were analyzed. The most common symptoms were fever (85.7%), nausea and vomiting (78.6%), and abdominal distension (67.9%). Among the 28 patients, 26 (92.9%) had elevated hypersensitive C-reactive protein, 24 (85.7%) had an increased neutrophil percentage, and 22 (78.6%) had raised peripheral blood leukocyte counts. Moreover, 19 patients (67.9%) showed increased serum total bilirubin levels, and 5 (17.9%) showed an elevated conjugated bilirubin level. Abdominal CT examination revealed that the gallbladder wall of patients was thickened with edema, accompanied by gallbladder stenosis and gallbladder mucosa enhancement; furthermore, ascites was found in the abdominal cavity and lesser omental bursa. Twenty-two patients underwent abdominal paracentesis, and 20 (90.9%) of them were exposed to bile-based ascites. Among the 28 patients, four recovered with conservative treatment, whereas the others (85.7%) were surgically treated. Of the twenty-four patients undergoing surgery, the perforation site was found at the union of the hepatic and cystic ducts in 12 patients (50%), no perforation site was observed in 9 patients (37.5%), and a common hepatic duct was observed in 3 patients (12.5%). All 24 patients underwent stage I surgery, and temporary biliary drainage was performed because of severe abdominal inflammation. Cholangiography and enhanced CT revealed an abnormal location of the pancreatic duct joining the bile duct in 64.3% patients. Following surgery, 15 patients underwent hepaticojejunostomy. Subsequently, 3-month to 6-year follow-up (median, 30 months) indicated that the patients recovered well with no serious complications. CONCLUSION: SBDP in children may be associated with pancreaticobiliary malunion (PBM) and congenital weakness of the bile duct wall. However, the clinical manifestations of this condition lack specificity; this limitation can be assisted through diagnosis via abdominal CT and by performing abdominal paracentesis. Once SBDP diagnosis is confirmed, the patient should follow the principles of individualized treatment.