Abernethy malformation type 2: varied presentation, management and outcome

• Published in: Journal of pediatric surgery 2019-04, Vol.54 (4), p.760-765
• Main Authors: Jain, Vishesh, Sangdup, Tsering, Agarwala, Sandeep, Bishoi, Akshay Kumar, Chauhan, Sandeep, Dhua, Anjan, Jana, Manisha, Kandasamy, Devasenathipathy, Malik, Rohan, Kothari, Shyam Sunder, Patcharu, Ravi, Varshney, Abhimanyu, Bhatnagar, Veereshwar
• Format: Article
• Language: English
• Subjects: Abernethy malformation; Adolescent; Angiography; Child; Child, Preschool; Female; Follow-Up Studies; Hepatopulmonary syndrome; Hepatopulmonary Syndrome - etiology; Humans; Laparotomy - methods; Ligation - methods; Male; Portal Vein - abnormalities; Portal Vein - surgery; Portosystemic shunt; Retrospective Studies; Tomography, X-Ray Computed - methods; Ultrasonography - methods; Vascular Malformations - complications; Vascular Malformations - diagnosis; Vascular Malformations - surgery
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/j.jpedsurg.2018.08.053

To study the varied presentations and the outcomes in children with Type 2 Abernethy malformation following shunt ligation. Children with Type 2 Abernethy who had had been operated between 2013 and 2017 were included in the study. The diagnosis had been confirmed on ultrasonography, CECT or angiography. All patients underwent laparotomy. The shunt was identified, clamped and the bowel congestion was noted. The shunt was ligated if the bowel congestion was not significant or had improved. Relevant follow-up investigations were done to document the resolution or amelioration of symptoms and the patency of the shunt. Five patients were included in the study with a median age of 6 years. Hepatopulmonary syndrome was the presentation in 4 patients while one patient presented with liver tumor. Ultrasonography and CECT were able to diagnose Type 2 malformation in 4 patients whereas in 1 patient the distal portal vein was not seen. The postoperative period was complicated in 3 patients. At the median follow up at 14 months, good intrahepatic portal flow in all patients. All patients demonstrated improvement/ resolution of symptoms. Abernethy is rare malformation which can have a varied presentation. Additional investigations may be needed to confirm the diagnosis of Type 2 variety. Most patients have gradual improvement of symptoms. Level IV/ Treatment study.