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.

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.

A Case of Suspected Intrahepatic Bile Duct Perforation after Direct Peroral Cholangioscopy / 대한췌담도학회지

Direct peroral cholangioscopy (POC) which permits direct visualization of the biliary tree has recently gained widespread clinical use for diagnosis and treatment of various pancreatobiliary diseases. But, there is currently little reliable data on evaluating the complications of POC. POC is associated with complications such as pancreatitis, cholangitis, hemorrhage, rarely air embolism, and ductal perforation. The incidence of complication during POC is 2.9-12%. However, pneumoperitoneum due to intrahepatic bile duct perforation after POC has not yet been reported in Korea. We report a case of pneumoperitoneum after POC which has been successfully managed with endoscopic nasobiliary drainage and antibiotics.

A Case of Iatrogenic Bronchobiliary Fistula / 대한소화기내시경학회지

Bronchobiliary fistula is a rare condition. It is defined by the presence of a passage between the biliary tract and the bronchial tree. Many conditions can give rise to the developement of such a communication. The patient was a 71-year-old man who had obstructive jaundice due to liver mass. At first, we inserted an uncovered metallic stent for biliary drainage. However, the bile duct was perforated due to the trapping of a catheter in the distal end of the deployed stent. The operation was performed immediately, but only the sump draingage was placed in the retroperitoneum because the perforation site could not be found. After 20 days from the procedure, the patient complained of bilioptysis because of a bronchobiliary fistula. We inserted a covered stent into the previous uncovered metallic stent. Bilioptysis rapidly resolved after the successful procedure. We report a case of iatrogenic bronchobiliary fistula which was managed by endoscopic biliary stenting.

Spontaneous Perforation of the Bile Duct

Spontaneous perforation of the bile duct in children is a very rare disorder. We experienced a 6 year-old girl with spontaneous perforation of the right hepatic duct. The patient was initially misdiagnosed as hepatitis because of elevation of liver enzyme and then as appendicitis because of fluid collection in the pelvic cavity demonstrated by ultrasonogram. A laparoscopic exploration was done and no abnormal findings were detected except bile-stained ascites. Peritoneal drainage was performed and the patients seemed to improve clinically. Abdominal pain, distention and high fever developed after removal of the drains. DISIDA scan showed a possible of bile leak into the peritoneal cavity. ERCP demonstrated free spiil of dye from the right hepatic duct. At laparotomy, the leak was seen in the anterior wall of the right hepatic duct 2cm above the junction of the cystk duct and common hepatic duct. The perforation was linear in shape and 0.8cm in size. The patient underwent cholecystectomy, primary closure of the perforation and T-tube choedochostomy. We could not identify the cause of the perforation; however, the T-tube cholangiography taken on the 42nd postoperative day showed a little more dilatation of the proximal common bile duct compared with the cholangiography taken on the 14th day. Long-term follow-up of the patient will be necessary because of the possibility for further change of the duct.