Perfuração espontânea da via biliar principal na infância / Spontaneous perforation of the main biliary tract in infancy

The authors report a case of spontaneous perforation of the biliary tract (S.P.B.T) in a three-month-old infant. The diagnosis was suspected before the operation by clinical signs and diagnostic tests. The importance of paracentesis and scintigraphy is stressed. A surgical approach was chosen and drainage procedure of the area around the perforation and a cholecystostomy were done. S.P.B.T. is rare and its etiology is controversial, but cannot be forgotten in association between biliary ascites and cholestatic jaundice during the first months of life.

Aortopulmonary window--impact of associated lesions on surgical results.

OBJECTIVES: The aortopulmonary window (APW) is a communication between the pulmonary artery (PA) and the ascending aorta in the presence of two separate semilunar valves. This review describes the natural history of the APW and the impact of associated defects on surgical results in patients treated at our institution. METHODS: Retrospective longitudinal study, based on the review of medical files of patients diagnosed between 1995 and 2005. RESULTS: Of nine patients diagnosed as having APW, six had associated lesions. Seven patients were submitted to surgical treatment with two deaths. One patient was not submitted to surgery due to pulmonary hypertension and another one died before the surgery due to a respiratory infection complication. CONCLUSION: The surgical results are satisfactory when the APW presents as an isolated defect and when surgery is performed early, preventing the development of irreversible arterial pulmonary hypertension (APH). The presence of associated complex congenital heart disease is a bad prognostic factor in our series.

Janela aortopulmonar: impacto das lesões associadas no resultado cirúrgico / Aortopulmonary window: impact of associated lesions on surgical results

OBJETIVOS: A janela aortopulmonar (JAP) é uma comunicação entre a artéria pulmonar (AP) e a aorta ascendente na presença de duas valvas semilunares separadas. Nesta revisão, descrevemos nossa experiência na história natural da JAP e do impacto de lesões associadas nos resultados cirúrgicos de pacientes tratados em nosso serviço. MÉTODOS: Estudo longitudinal retrospectivo, com revisão dos prontuários dos pacientes diagnosticados entre 1995 e 2005. RESULTADOS: Dos 9 pacientes diagnosticados como portadores de JAP, 6 apresentavam cardiopatia associada. Sete pacientes foram submetidos a tratamento cirúrgico, ocorrendo dois óbitos. Um paciente teve a cirurgia contra-indicada pela presença de hipertensão pulmonar, e outro faleceu antes do procedimento cirúrgico por complicação infecciosa respiratória. CONCLUSÃO: Os resultados cirúrgicos são satisfatórios quando a JAP se apresenta como defeito isolado e quando a cirurgia é realizada precocemente, evitando-se o desenvolvimento de hipertensão arterial pulmonar (HAP) irreversível. A presença de cardiopatia congênita complexa associada é fator de pior prognóstico em nossa série de casos.

OBJECTIVES: The aortopulmonary window (APW) is a communication between the pulmonary artery (PA) and the ascending aorta in the presence of two separate semilunar valves. This review describes the natural history of the APW and the impact of associated defects on surgical results in patients treated at our institution. METHODS: Retrospective longitudinal study, based on the review of medical files of patients diagnosed between 1995 and 2005. RESULTS: Of nine patients diagnosed as having APW, six had associated lesions. Seven patients were submitted to surgical treatment with two deaths. One patient was not submitted to surgery due to pulmonary hypertension and another one died before the surgery due to a respiratory infection complication. CONCLUSION: The surgical results are satisfactory when the APW presents as an isolated defect and when surgery is performed early, preventing the development of irreversible arterial pulmonary hypertension (APH). The presence of associated complex congenital heart disease is a bad prognostic factor in our series.