Vishesh Jain1, Tsering Sangdup2, Sandeep Agarwala3, Akshay Kumar Bishoi2, Sandeep Chauhan4, Anjan Dhua1, Manisha Jana5, Devasenathipathy Kandasamy5, Rohan Malik6, Shyam Sunder Kothari7, Ravi Patcharu1, Abhimanyu Varshney1, Veereshwar Bhatnagar1. 1. Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India 110029. 2. Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India 110029. 3. Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India 110029. Electronic address: sandpagr@hotmail.com. 4. Department of Cardiac-Anaesthesia, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India 110029. 5. Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India 110029. 6. Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India 110029. 7. Department of Cardiology, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India 110029.
Abstract
PURPOSE: To study the varied presentations and the outcomes in children with Type 2 Abernethy malformation following shunt ligation. MATERIAL AND METHODS: 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. RESULTS: 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. CONCLUSION: 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 OF EVIDENCE: Level IV/ Treatment study.
PURPOSE: To study the varied presentations and the outcomes in children with Type 2 Abernethy malformation following shunt ligation. MATERIAL AND METHODS: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. RESULTS: 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. CONCLUSION: 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 OF EVIDENCE: Level IV/ Treatment study.