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
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India 110029.
Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India 110029.
J Pediatr Surg. 2019 Apr;54(4):760-765. doi: 10.1016/j.jpedsurg.2018.08.053. Epub 2018 Sep 5.
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.
研究2型阿伯内西畸形患儿在分流结扎术后的不同表现及预后。
纳入2013年至2017年间接受手术的2型阿伯内西畸形患儿。诊断通过超声、CT增强扫描（CECT）或血管造影得以证实。所有患者均接受剖腹手术。找到分流血管，予以夹闭并记录肠充血情况。若肠充血不严重或有所改善，则结扎分流血管。进行相关随访检查以记录症状的缓解或改善情况以及分流血管的通畅情况。
5例患者纳入研究，中位年龄6岁。4例患者表现为肝肺综合征，1例患者表现为肝肿瘤。4例患者通过超声和CECT诊断为2型畸形，而1例患者未见肝门静脉远端。3例患者术后出现并发症。在14个月的中位随访期，所有患者肝内门静脉血流良好。所有患者症状均有改善/缓解。
阿伯内西畸形较为罕见，表现多样。可能需要进一步检查以确诊2型阿伯内西畸形。大多数患者症状逐渐改善。
IV级/治疗研究。
