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Abernethy malformation: a case report.

Ashish Pathak¹, Nitin Agarwal, Jagdish Mandliya

¹Department of Pediatrics, R.D. Gardi Medical College, Ujjain, India. drashishp@rediffmail.com

|May 31, 2012

Summary

Abernethy malformation, a rare vascular disorder, can occur without other congenital anomalies. Early diagnosis and shunt closure are key for managing this condition and preventing complications.

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Area of Science:

Vascular Malformations
Pediatric Gastroenterology
Congenital Disorders

Background:

Abernethy malformation is a rare congenital vascular anomaly characterized by portal blood diversion from the liver.
It is often associated with multiple congenital anomalies.
This case highlights Abernethy malformation in an Indian child presenting without other anomalies.

Observation:

A 5-year-old female presented with jaundice, initially suspected as viral hepatitis.
Persistent jaundice prompted detailed investigation, revealing portal vein drainage into the inferior vena cava via ultrasound and Doppler.
CT angiography confirmed Type 1b Abernethy malformation.

Findings:

The case demonstrates Abernethy malformation (Type 1b) in a pediatric patient.

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Absence of associated major congenital anomalies was noted.

Diagnostic workup involved ultrasound, Doppler, and CT angiography.

Implications:

Prompt diagnosis and management of Abernethy malformation are crucial.
Surgical closure of the shunt is recommended for cure or complication prevention.
Liver transplantation may be considered for symptomatic Type I cases where shunt closure is not feasible.
Long-term follow-up is essential for all patients with congenital portosystemic shunts.