Abernethy malformation is a congenital condition where portal blood is diverted away from the liver, classified into two main types: type I, with absent intrahepatic portal veins, and type II, with patent intrahepatic veins. Symptoms can include hepatic encephalopathy and pulmonary complications, with diagnostic options including ultrasound, CT, and MRI. Management differs between types, with type I requiring monitoring and potential liver transplantation and type II allowing for shunt occlusion in symptomatic cases.

1. Dr Ambati Karuna sagar

2. 
 Abernethy malformation is defined as congenital diversion
of portal blood away from the liver by either end-to-side
or side-to-side shunt
Definition

3. 
Congenital extrahepatic portosystemic shunt is a rare
congenital anomaly that was first described by John
Abernethy in 1793 at autopsy of a 10-month-old infant who
died of unknown cause

4. 
 Morgen and Superina classification of congenital
extra hepatic portosystemic shunt
 Type I Absence of intrahepatic portal veins
 Type I a Superior mesenteric and splenic vein drain
separately into inferior vena cava
 Type I b Superior mesenteric vein and splenic vein
form a common trunk before draining into the inferior
vena cava
 Type II Important collateral, patent intrahepatic veins
Classification

5. 
 Portosystemic shunt anomalies have been classified into
two types
 Type I
 Type II

6. 
 Characterized by the absence of the intrahepatic PV and
complete end-to-side shunt
 Two subtypes
 Type Ia
 separate drainage of the superior mesenteric and splenic
veins into the IVC, iliac veins, or renal veins
 Type Ib
 Superior mesenteric and splenic veins joining to form a
short extrahepatic PV which drains into the IVC
Type 1 shunts

7. 
 Marked by presence of a patent intrahepatic PV and a
partial side-to-side shunt
Type II

8.  Congenital heart disease,
 Polysplenia,
 Biliary atresia,
 Malrotation,
 Duodenal atresia,
 Annular pancreas,
 Situs inversus,
 Anomalies of the renal tract, and
 Skeletal anomalies
Associations

9. 
 Dilatation of intrapulmonary vessels and hepatopulmonary
syndrome
 Diversion of gut-derived toxins to the systemic circulation
leading to hepatic encephalopathy or diversion of vasoactive
mediators into the systemic circulation
 Digital clubbing (ventilation perfusion mismatch)
 Varying degree of dyspnoea on exertion (portopulmonary
hypertension or hepatopulmonary syndrome)
 Hepatic encephalopathy
 Hypoglycemia (combined effect of defective uptake of
glucose and hyperinsulinemia due to reduced hepatic
degradation of normal quantity of secreted insulin)
Clinical manifestations

10. 
 USG
 CT
 MRI
Diagnosis

11. 
 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 merit clinical, biochemical, and
imaging follow-up
 For those who develop severe hepatic encephalopathy or
malignant liver nodules, liver transplantation is the only
treatment option
Management

12. 
 For patients with type 2 malformations and serious
symptoms such as hepatic encephalopathy, shunt occlusion
can be performed, either surgically or by percutaneous
transcatheter coil placement

13. 
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