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.

Acute abdomen with jaundice: A clue to extrahepatic biliary tract perforation.

INTRODUCTION: Among a plethora of causes of acute abdomen, spontaneous common bile duct perforation (SCBDP) resulting in biliary peritonitis is almost never envisaged. Since the term SCBDP is often misconstrued as absence of an identifiable cause of perforation, 'nontraumatic perforation of CBD' is also in parlance to exclude relatively common causes such as trauma and iatrogenic injuries. In adults, choledochal cyst, cholangitis, infection, pancreatitis, pancreatobiliary maljunction have been identified as causes of perforation, however, choledocholithiasis remains the most common cause associated with spontaneous perforation of extra hepatic bile duct. CASE PRESENTATION: Here we present three cases of a spontaneous common bile duct perforation that presented as acute abdomen with peritonitis. Clinical presentation, biochemical abnormalities, imaging details, treatment options, and outcome were studied. This study has been reported in line with the SCARE 2020 Guideline [1]. DISCUSSION AND CONCLUSION: Spontaneous perforation of the extrahepatic bile duct is a rare but important presentation of gallstones in adults and needs a high index of suspicion during day-to-day practice. Surgeons should seek out this uncommon diagnosis in the patient for early surgical intervention and appropriate drainage to ensure survival.

Tratamiento quirúrgico inmediato en niños con perforación espontánea de la vía biliar. Experiencias de 16 años

Introducción: La perforación espontánea de los conductos biliares es una rara enfermedad caracterizada por una disrupción no traumática de la vía biliar en pacientes aparentemente sanos. Se trata de una grave situación potencialmente letal, pero diagnosticada y tratada correctamente tiene un pronóstico excelente. Objetivo: Caracterizar los principales elementos clínico-quirúrgicos expresados en una serie de 5 pacientes operados en un servicio de referencia nacional. Presentación de casos: Se presenta la experiencia con una serie de casos en 16 años en una sola institución. La afección se observó en niñas recién nacidas y lactantes con una edad media de 4 meses, y se presentó desde la clínica como una colestasis acompañada de distensión abdominal, ascitis biliar, acolia, y signos de irritación peritoneal. El 80 % de los casos se intervinieron en el hospital "William Soler", y en un caso se ejecutó el procedimiento después de una laparotomía por una posible apendicitis aguda, en otro hospital. El diagnóstico se basó en el cuadro clínico descrito, la ecografía abdominal, la paracentesis con medición del índice bilirrubina líquido ascítico/bilirrubina sérica, y la colangiografía intraoperatoria. La cirugía definitiva se realizó inmediatamente, y consistió en: lavado peritoneal, colangiografía diagnóstica, reparación hepaticoyeyunostomía en Y de Roux y colocación de drenaje. Conclusiones: El tratamiento realizado resulta eficaz y seguro en todos los casos, con una excelente evolución, sin complicaciones importantes y con una total supervivencia posoperatoria. La colangiografía intraoperatoria permitió identificar el sitio de la perforación y diagnosticar malformaciones asociadas como dilataciones biliares congénitas y anomalías de la unión bilio-pancreática.

Introduction: Spontaneous bile duct perforation is a rare condition characterized by non-traumatic disruption of the bile duct in apparently healthy patients. It is a serious potentially lethal situation, but correctly diagnosed and treated its prognosis is excellent. Objetive: To characterize the main clinical-surgical elements expressed in a series of 5 patients operated in a national referral service. Case presentation: The experience with a series of cases during a period of 16 years in a single institution is presented. The condition was observed mainly in newborn girls and infants with an average age of 4 months, and presented clinically as cholestasis accompanied by abdominal distension, biliary ascites, acholia, and signs of peritoneal irritation. 80% of the cases were operated primarily in the "William Soler" hospital, and in one case the procedure was performed after a laparotomy for a possible acute appendicitis, in another hospital. The diagnosis was based on the clinical picture described, abdominal ultrasound, paracentesis with measurement of the ascitic liquid bilirubin/serum bilirubin index, and intraoperative cholangiography. Definitive surgery was performed immediately and consisted of: peritoneal lavage, diagnostic cholangiography, Roux-en-Y liver and jejunostomy repair and drainage placement. Conclusions: The treatment performed was effective and safe in all cases, with an excellent evolution, no major complications and total postoperative survival. Intraoperative cholangiography made it possible to identify the site of perforation and to diagnose associated malformations such as congenital biliary dilatations and anomalies of the biliary-pancreatic junction.

Spontaneous perforation of common bile duct in a young female: An intra-operative surprise.

INTRODUCTION: Spontaneous CBD perforation is one of the rare causes of acute abdomen in infants and extremely rare in adults. It is rarely suspected and correctly diagnosed preoperatively. PRESENTATION OF CASE: A 17year old female presented to Emergency Department with sudden onset of pain and distention of abdomen, associated with vomiting and non-passage of flatus and stool for 3days and features of generalized peritonitis. On exploration, a perforation of size 0.5cm in diameter was present on the antero-lateral surface of supraduodenal part of common bile duct (CBD) below the junction of cystic duct and common hepatic duct. Cholecystectomy done and the CBD repaired over a T-tube. DISCUSSION: Spontaneous perforation of bile duct should ideally manage with T-tube drainage of the CBD along with cholecystectomy. In case with distal obstruction of the CBD, a biliary enteric bypass should be done. CONCLUSION: Due to the paucity of cases, the index of suspicion for this diagnosis is low. But bilious peritoneal tap, features of generalized peritonitis and absence of free gas under diaphragm in abdominal x-ray may be considered as clues for suspicion. Accordingly, Surgery remains the mainstay of treatment.

Therapeutic experiences of 25 patients with pediatric spontaneous biliary duct perforation / 中华肝胆外科杂志

Objective To summarize our therapeutic experiences on patients with pediatric spontaneous biliary duct perforation.Methods We retrospectively analyzed the clinical data of patients diagnosed as spontaneous biliary duct perforation who were admitted into the Department of Pediatric General Surgery,the Beijing Children Hospital from January 2008 to December 2014,and summarized the therapeutic experiences.Results There were 7 boys and 18 girls,with a average age of 2.4 years (range 11 months to 10 years).Twenty-one patients (84.0％) were diagnosed by ultrasonography.Two patients were treated with conservative therapy and were discharged home.The remaining 23 patients were treated with emergent surgery.Of these patients,9 were treated with cholecystostomy and abdominal drainage (the cholecystostomy group),and the remaining 14 were treated with choledochal drainage and abdominal drainage (the choledochal drainage group).The mean hospitalization stay for the cholecystostomy group was 25.2 days,and 3 patients developed comphcations (33.3％).The mean hospitalization stay for thecholedochal drainage group was 16.1 day,and 2 patients developed complications (14.2％).Twenty-four patients were diagnosed to suffer from congenital choledochal cysts or pancreaticobiliary maljunction by imaging studies during or after surgery.Elective choledochal cystectomy with hepaticojejunostomy were performed on 23 stable patients who developed no severe complications.Conclusions Pediatric spontaneous bile duct perforation is closely related with congenital choledochal cysts,and the pathological basis in diagnosis is pancreaticobiliary maljunction.Bile duct elastic fiber hypogenesis and specific blood supply are important to the onset of perforation.Abdominal ultrasonography plays an important role in the diagnosis.Patients with peritoneal irritation and non-localized perforation should be operated in time,and choledochal drainage with abdominal drainage is a good treatment choice.All patients diagnosed as congenital choledochal cysts or pancreaticobiliary maljunction should undergo elective choledochal cystectomy with hepaticojejunostomy.