CT FEATURES OF CONGENITAL PORTOSYSTEMIC SHUNTS AT CHILDREN'S HOSPITAL 2: A CASE SERIES REPORT
Main Article Content
Abstract
Introduction:
Congenital portosystemic shunts (CPSS) are rare vascular anomalies in which portal venous blood bypasses the liver and drains directly into the systemic circulation. These anomalies are often associated with chromosomal abnormalities and have a wide range of clinical manifestations. In some cases, CPSS may be asymptomatic, making diagnosis challenging. The condition can lead to serious complications affecting multiple organ systems, including the liver, central nervous system, and respiratory tract. Imaging plays a pivotal role in the suspicion and diagnosis of CPSS, assessing the extent of the shunt, identifying associated anomalies, and guiding appropriate management strategies.
Objective:
To describe the computed tomography (CT). Imaging features and associated clinical findings of congenital portosystemic shunts diagnosed at Children's Hospital 2.
Material and Methods: A retrospective report of 9 confirmed cases of CPSS diagnosed by CT.
Results:
From April 2022 to October 2022, a total of 9 cases of CPSS were confirmed at Children's Hospital 2. Of these, 7 patients (77.8%) had intrahepatic portosystemic shunts, including 4 cases of type II, 1 case of type III, and 2 cases of type V according to Park's classification. The remaining 2 patients (22.2%) had extrahepatic portosystemic shunts, both classified as type Ib according to Abernethy's classification. Intrahepatic type II shunts were the most common, accounting for 42.9%. The mean age at diagnosis was 19.6 ± 26.8 months, ranging from 5 days to 58 months of age. CPSS was often discovered incidentally during investigations for other congenital anomalies. Four patients (44.4%) had congenital anomalies, with congenital heart disease and hemangiomas being the most common, each observed in 22.2% of cases.
Conclusion:
Congenital portosystemic shunts have diverse clinical presentations, ranging from asymptomatic cases to manifestations resembling neonatal cholestasis or complex congenital malformation syndromes. Imaging evaluation is of paramount importance for diagnosis and treatment planning, helping to prevent severe complications associated with this vascular anomaly.
Keywords
congenital portosystemic shunt, vascular malformation, pediatric, computed tomography
Article Details
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