Enfermedad de Caroli: presentación de caso pediátrico

Abstract

Caroli’s disease is a congenital anomaly that affects the bile ducts within the liver. It is characterized by localized cystic dilatation of these ducts, which increases the risk of gallstones, cholangitis, liver abscess, absence of cirrhosis, and portal hypertension. It is often associated with renal tubular ectasia or similar renal cystic disease. The estimated prevalence of this condition is one case per million people.

OBJECTIVE: To present an unusual case of a patient with Caroli’s disease that developed at a young age, affecting a single lobe of the liver, and requiring complex surgical intervention.

METHODOLOGY: An observational-descriptive study will be carried out, including a detailed description of the clinical case with all the relevant characteristics and significant findings. Additionally, a critical analysis will be made by reviewing the existing literature on the subject. Information is acquired from scientific digital databases such as PubMed, Elsevier, and ResearchGate; selecting scientific articles related to the subject, published during the last 5 years, in English and Spanish. This paper presents the case of an 11-month-old female patient who was admitted with a 1-month history of abdominal distention and postprandial vomiting. After multidisciplinary management was diagnosed with Caroli's disease, and treated without the need for liver transplantation.

RESULTS: Based on the indexed bibliography, different diagnostic guidelines, therapeutic options, and the importance of a multidisciplinary approach are related.