RESUMO
We describe a case of Caroli syndrome (Caroli's disease and congenital hepatic fibrosis) in a 10-year-old boy with bilobar involvement and numerous renal cysts. Liver transplantation offers the only hope for such patients.
Assuntos
Doença de Caroli/diagnóstico , Doença de Caroli/terapia , Dor Abdominal/etiologia , Biópsia por Agulha , Doença de Caroli/sangue , Doença de Caroli/complicações , Doença de Caroli/genética , Criança , Colangiopancreatografia Retrógrada Endoscópica , Colangite/etiologia , Diagnóstico Diferencial , Drenagem , Febre/etiologia , Humanos , Transplante de Fígado , Masculino , Prognóstico , Esfinterotomia Endoscópica , Stents , Tomografia Computadorizada por Raios X , Vômito/etiologiaRESUMO
Intestinal volvulus causing obstruction is an unusual complication of ventriculoperitoneal shunts. There are no clear guidelines on the management of this problem. In our patient exteriorization of the shunt with subsequent reinsertion at a different site led to an uneventful recovery. The extreme rarity of this complication and its challenging management are discussed.
Assuntos
Obstrução Intestinal/etiologia , Derivação Ventriculoperitoneal/efeitos adversos , Humanos , Lactente , Obstrução Intestinal/diagnóstico por imagem , Masculino , RadiografiaRESUMO
Mesenteric and retroperitoneal cysts develop from ectopic lymphatic tissue. Most of them present as symptomless abdominal swellings or are found coincidentally during abdominal operations. One third of patients may present with acute abdominal pain, and few may present with chronic abdominal pain. However, mesenteric cyst presenting as inguinal hernia is extremely rare. Only four cases have been reported in the English-language literature. The present case is the fifth, and only the second from this subcontinent. The treatment of choice is complete surgical excision.