RESUMO
A 42-year-old patient, diagnosed with Alagille syndrome when he was 1 month old, was admitted to the general surgery department to undergo right hepatectomy for right lobe carcinoma. Alagille syndrome, also known as biliary duct hypoplasia, is an extremely rare genetic condition with multiorganic implications. Liver and heart dysfunction will play key roles in the course of the disease and its prognosis. An exhaustive preoperative assessment of liver and heart function, blood chemistry and the skeleton will be essential for managing these patients. This article reviews the main principles underlying the anesthetic management of patients with Alagille syndrome.
Assuntos
Síndrome de Alagille , Anestesia , Adulto , Anestesia/métodos , Feminino , Humanos , Fatores de RiscoRESUMO
Paciente de 42 años, diagnosticada de Síndrome de Alagille en el primer mes de vida. Ingresa en el Servicio de Cirugía General con diagnóstico de hepatocarcinoma de lóbulo derecho, para la realización de hepatectomía derecha. El Síndrome de Alagille, también conocido como Síndrome de escasez de conductos biliares, es una enfermedad genética extremadamente rara, de afectación multiorgánica. La disfunción hepática y cardíaca será fundamental para la evolución y pronóstico de la enfermedad. Un estudio preoperatorio exhaustivo de la función hepática, cardiológico, hematológica y ósea se antoja funda- mental para el manejo de estos pacientes. En el presente trabajo se revisan los principales pun- tos de la actuación anestésica en los pacientes portado- res de este síndrome
A 42-year-old patient, diagnosed with Alagille syndrome when he was 1 month old, was admitted to the general surgery department to undergo right hepatectomy for right lobe carcinoma. Alagille syndrome, also known as biliary duct hypoplasia, is an extremely rare genetic condition with multiorganic implications. Liver and heart dysfunction will play key roles in the course of the disease and its prognosis. An exhaustive preoperative assessment of liver and heart function, blood chemistry and the skeleton will be essential for managing these patients. This article reviews the main principles underlying the anesthetic management of patients with Alagille syndrome
