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Abernethy malformation type 2: varied presentation, management and outcome
Vishesh Jain1, Tsering Sangdup2, Sandeep Agarwala1
1Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India 110029.
Journal of Pediatric Surgery
|September 29, 2018
Summary
Shunt ligation in children with Abernethy malformation (Type 2) led to symptom improvement. This rare condition requires careful diagnosis and management for better outcomes.
Area of Science:
- Pediatric Surgery
- Vascular Malformations
- Hepatology
Background:
- Abernethy malformation is a rare congenital portosystemic shunt.
- Type 2 Abernethy malformation involves complete absence of the intrahepatic portal vein.
- Varied clinical presentations can delay diagnosis.
Purpose of the Study:
- To evaluate the clinical presentations and outcomes of Type 2 Abernethy malformation in children treated with shunt ligation.
- To assess the efficacy of surgical intervention in managing this rare condition.
Main Methods:
- Retrospective study of 5 children diagnosed with Type 2 Abernethy malformation between 2013-2017.
- Diagnosis confirmed via ultrasonography, CECT, or angiography.
- Surgical shunt ligation performed after assessing bowel congestion, with follow-up investigations.
Main Results:
- Median age of patients was 6 years.
- Common presentations included hepatopulmonary syndrome (4/5) and liver tumor (1/5).
- All patients showed improved symptoms and good intrahepatic portal flow post-ligation at 14-month median follow-up.
Conclusions:
- Type 2 Abernethy malformation presents diversely and may require advanced imaging for diagnosis.
- Shunt ligation is an effective treatment, leading to symptom resolution and improved portal flow in pediatric patients.
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