ABERNETHY MALFORMATION : I would like to share a rare case of Abernethy malformation. 38 yr old patient G2 P1 L1 PR LSCS. Consanguinous marriage. patient is obese,hirsute ,a known case of PCOD and hypothyroidism. I :Was treated for infertility./LSCS /Al female child /6 years/normal development. II : Was treated for secondary infertility. Tiffa normal. THERE WAS SINGLE UMBILICAL ARTERY. LSCS /CIAB /later developed cyanosis and was in NICU for 1 month.She was diagnosed as PPHN (Persistent pulmonary hypertension ). Now the baby is 2 years.Baby is having recurrent URTI. On examination,it was found that baby had abernethy malformation. ABERNATHY MALFORMATION (AM ). Congenital extra hepatic portosystemic shunt is a rare congenital anomaly that was first described by JOHN ABERNATHY in 1793 at autopsy of a 10 month old infant who died of unknown cause. AM is defined as congenital diversion of portal blood away from the liver by either end-to-side or side-to-side shunt. CLASSIFICATION OF CONGENITAL EXTRA HEPATIC PORTOSYSTEMIC SHUNT. Classified into two types. Type I and II. TYPE I SHUNT :Characterized by the absence of the intrahepatic portal vein and complete end -to-side shunt. There are 2 sub-types . TYPE I A :Superior mesenteric and spleenic vein drain separately into inferior vena cava. TYPE II B :Superior mesenteric and spleenic vein form a common trunk before draining into the inferior vena cava. TYPE II :Marked by the presence of a patent intra hepatic portal vein and a partial side-to-side shunt. AM is associated with several congenital malformations like *Congenital heart disease. *Polyspleenia. *Biliary atresia. *Duodenal atresia. *Malrotation. *Annular pancreas. *Situs inversus. *Renal anomalies. *Skeletal anomalies. CLINICAL FEATURES : 1.Dilatation of intra pulmonary vessels and hepato pulmonary syndrome. 2.Diversion of gut derived toxins to the systemic circulation leading to hepatic encephalopathy or diversion of vaso active mediators into the systemic circulation. 3.Digital clubbing (ventilation perfusion mismatch) 4.Varying degrees of dyspnea on exertion (porto pulmonary hypertension or hepato pulmonary syndrome ). 5.Hepatic encephalopathy. 6.Hypoglycemia. DIAGNOSIS. *USG. *CT. *MRI. MANAGEMENT. In patients with type I malformation,occlusion of the shunt is not an option since it represents the only drainage route for the mesenteric venous blood. Hence,these patients require clinical, biochemical and imaging follow up. Those who develop severe hepatic encephalopathy pr malignant liver nodules,liver trans plantation is the only treatment option. For patients with type II malformation and serious symptoms such as hepatic encephalopathy ,shunt occlusion can be performed,eithr surgically or by percutaneous transcathetar coli placement. Our baby has high levels of ammonia and is advised to undergo placement of stent.

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Seen one grown uo girl who presented like a cyanotic heart disease with strucurally normal heart. She had pulmonary av malformation behaving like a rt to lt shunt.

Mam very interesting case

Very nice case...It's rare too...

Thanks for sharing such a rare case of Abernethy Malformation. Informative & Good post.

Very informative.

Interesting n informative case

Useful and informative

Nice share

Intresting case

Interesting case

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