Doenças císticas das vias biliares / Cystic diseases of the bile ducts

JUSTIFICATIVA E OBJETIVOS: Os ductos biliares podemapresentar dilatações congênitas ou adquiridas. As lesões congênitaspodem ser hipoplásticas (atresias) e císticas. O primeiroestudo descrito na literatura foi realizado por Vater and Ezler in1723. Os cistos biliares são isolados ou múltiplos, e aparecemem qualquer idade, mas 75% deles são antes dos 20 anos. Elespodem ocorrer em toda árvore biliar, sendo, as intra-hepáticasrestritas, conhecida como doença de Caroli. O objetivo desseestudo foi fazer uma revisão sobre as Doenças císticas das viasbiliares. CONTEÚDO: As dilatações císticas extra-hepáticaatingem diversos tamanhos e são classificadas segundo sua formae localização. São elas: fusiforme, divertículos e dilataçõessaculares. Háuma incidência variada dessas lesões com câncer,entre 2,5% e 17,5%. O quadro clínico mais comum éa colestaseneonatal, correspondendo a 80% dos pacientes. Na idadeadulta, podem ser assintomáticos ou apresentar apenas sintomasinespecíficos como náuseas,vômitos. A dificuldade de drenagemda colestase obstrutiva pode lavar a cirrose biliar secundária.CONCLUSÃO: O ultrassom de abdome pode confirmar odiagnóstico. No tocante ao laboratório, observa-se uma hiperbilirrubinemiae elevação de enzimas canaliculares. A terapêuticaempregada na maioria dos casos écirúrgica, com excisão do cistoe reconstrução da drenagem biliar, devendo-se evitar a drenagemisolada, pois tais pacientes tendem a evoluir com complicaçõescomo fístulas, estenose, colangite e carcinoma.(AU)

BACKGROUND AND OBJECTIVES: The bile duct dilatationmay have congenital or acquired. Congenital lesions can behipoplásticas (atresia) and cystic. The first study described in theliterature was performed by Vater and Ezler in 1723. Biliary cystsare isolated or multiple and appear at any age, but 75% of themare before age 20. They can occur throughout the biliary tree,and the intrahepatic restricted, known as Caroli disease. Theaim of this study was to review about the cystic biliary diseases.CONTENTS: Dilated cystic extrahepatic reach various sizesand are classified according to their shape and location. Theyare: fusiform, saccular dilatation and diverticula. There is anincidence of these lesions with cancer varied between 2.5% and17.5%. The most common symptom is a neonatal cholestasis,corresponding to 80% of patients. In adulthood, may beasymptomatic or show only nonspecific symptoms such as nausea,vomiting. The difficulty of draining obstructive cholestasis canwash the secondary biliary cirrhosis. CONCLUSION: Theabdominal ultrasound can confirm the diagnosis. Regarding thelaboratory, there is a hyperbilirubinemia and elevated GGT. Thetherapy used in most cases is surgical excision of the cyst andreconstruction of biliary drainage, should be avoided drainagealone because these patients tend to develop complications suchas fistula, stricture, cholangitis and carcinoma.(AU)

Optimización en la estrategia diagnóstica y terapéutica en el quiste de colédoco / Optimization in diagnostic and therapeutic strategy of choledochal cyst

Objectives: The choledochal cyst has an incidence of 1 in 100.000-150.000. The purpose of this study was to analyze the variables introduced for the optimization in the diagnosis and treatment of choledochal cyst. Material and Methods: Retrospective study of patients treated in our center by hepatic-jejunostomy and Roux-Y, from September 1988 to November 2012. We analyzed 40 variables including age, symptoms, type of cysts, diagnostic tests, changes in surgical technique, complications and outcomes. Results: Eighteen patients (66.6 percent female) were grouped according to the age of presentation: Prenatal (< 1 month), Early (124), Delayed (> 24 months). The most common symptoms were jaundice and abdominal pain (for early-onset and late-onset respectively). The 83.3 percent presented choledochal cysts type I, the ultrasound was sufficient for diagnosis in 94.4 percent. Since 2004 we modified the surgical technique, performing laparoscopic dissection of the bile duct and cyst, adding a mini-laparotomy (3-5 cm) for hepatic-jejunostomy with 40 cm intestinal loop using polypropylene suture. One complication was observed since 2004, one case of partial dehiscence of the anastomosis resolved with conservative treatment and a cholangitis in 1 patient with hepatic and renal polycystic. In 2012, 83.3 percent are asymptomatic. Conclusions: The diagnostic have been simplified, in more than 90 percent of cases was done by ultrasound; advances in minimally invasive surgery and creation of descending loop of 40 cm, have helped to improve the prognosis of choledochal cyst.

Objetivos: El quiste de colédoco presenta una incidencia de 1 en 100.000-150.000. El propósito de este estudio fue analizar las variables introducidas para la optimización en el diagnóstico y tratamiento del quiste de colédoco. Material y Métodos: Estudio retrospectivo de pacientes intervenidos en nuestro centro de quiste de colédoco mediante hepático-yeyunostomía en Y de Roux, desde septiembre de 1988 a noviembre de 2012. Se analizan 40 variables incluyendo edad, sintomatología, tipo de quiste, pruebas diagnósticas, cambios en la técnica quirúrgica, complicaciones y evolución. Resultados: 18 pacientes (66,6 por ciento mujeres) fueron agrupados de acuerdo a la edad de presentación: Prenatal (< 1 mes); Precoz (1-24 meses); Tardía (> 24 meses). Los síntomas más frecuentes fueron ictericia y dolor abdominal (para los de inicio precoz y tardío respectivamente). El 83,3 por ciento presentaban quistes de colédoco tipo I; siendo suficiente la ecografía para el diagnóstico en el 94,4 por ciento. A partir de 2004 modificamos la técnica quirúrgica, realizando por vía laparoscópica la disección de la vía biliar y mediante laparotomía mínima (3-5 cm) hepático-yeyunostomía con asa descendente de 40 cm, empleando sutura de polipropileno, observando desde el 2004, sólo 1 dehiscencia parcial de la anastomosis, resuelta con tratamiento conservador y 1 colangitis en paciente con poliquistosis hepática y renal. En el año 2012 el 83,3 por ciento están asintomáticos. Conclusiones: El diagnóstico se ha simplificado, en más de 90 por ciento de los casos se realiza sólo con ecografía; los avances en cirugía mínimamente invasiva y la creación de asa descendente de 40 cm, han logrado optimizar el pronóstico del quiste de colédoco.