ABSTRACT
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.
ABSTRACT
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.