Congenital extrahepatic portosystemic shunt (Abernethy malformation) treated endovascularly with vascular plug shunt closure

Abstract

A 3-year-old boy, who presented with progressive cyanosis and hypoxia, was diagnosed with a large congenital extrahepatic portosystemic shunt, interrupted IVC with azygos continuation, and multiple congenital anomalies. Traditionally open and laparoscopic surgical techniques have been used to treat this malformation. Endovascular repair using a 16-mm Amplatzer vascular plug (AGA Medical Corporation, Golden Valley, Minnesota, USA) was used to occlude the shunt. Immediate post-placement venography demonstrated cessation of flow within the shunt and increased portal venous flow. The patient's hypoxia and cyanosis decreased significantly, and he was discharged on the 5th post-procedure day in stable clinical condition. Three months follow-up evaluation demonstrated the vascular plug in place, unchanged in position.

Catheter interventional treatment of congenital portosystemic venous shunts in childhood

“…Shunt closure may be obtained either by open or laparascopic surgical ligation or by endovascular shunt closure using coils or vascular plugs .…”

“… Associated with Abernethy malformation are CHD, duodenal and biliary atresia, malrotation, annular pancreas, polysplenia, situs inversus, hepatic tumors (focal nodular hyperplasia, hepatoblastoma) and anomalies of genitourinary tract and musculoskeletal systems .…”

“…Interventional catheter treatment for patients with complex type of CHD offers the opportunity to close abnormal vascular malformation such as veno‐venous collaterals or aortic‐pulmonary collaterals in case of severe cyanosis using vascular devices like coils, plugs or covered stents . Infants with CPSVS can also benefit from this treatment by partial or complete closure of CPSVS.…”

Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review

“…Among them, 66 were female and 35 were male, with ages ranging from fetus to 61 years at the time of diagnosis. In total, 70 patients (69.30%) were<18 years of age, and <10% had type II malformations (45,58,75–77). …”

The Abernethy Malformation: A Rare Cause of Congenital Extrahepatic Portacaval Shunt

“…Literatürde, Abernethy malformasyonu olup karaciğer kitlesi bulunan ve hepatopulmoner sendrom gelişimi sonrası karaciğer transplantasyonu uygulanmış olgular bulunmaktadır (11,12). Tip 2 malformasyonda hepatik ensefalopati gibi ciddi bir komplikasyon gelişirse cerrahi veya perkütan transkateter yolla şant kapatılabilir (7,13,14). Abernethy malformasyonunda özellikle medikal tedaviye yanıtsız hepatik ensefalopati gelişir, malign karaciğer kitlesi saptanır ve şant ligasyonu uygulanması mümkün olmaz ise geri dönüşümsüz …”