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Case Report |
1 Department of Radiodiagnosis, Postgraduate Institute of Medical Education and
Research, Chandigarh 160012, India.
2 Department of Pediatric Gastroenterology, Postgraduate Institute of Medical
Education and Research, Chandigarh 160012, India.
Received December 15, 2003;
accepted after revision March 8, 2004.
Address correspondence to K. S. Sodhi.
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At physical examination, the infant was found to have dysmorphic facies, a preauricular tag on the right side, and generalized hypotonia. No evidence of hepatosplenomegaly or congestive hepatic failure was found. Laboratory studies showed a total bilirubin level of 6.3 mg/dL, conjugated bilirubin level of 3.5 mg/dL, serum alkaline phosphatase level of 30 U/L, serum aspartate transaminase level of 37 U/L, and serum alanine transaminase level of 33 U/L. Karyotypic evaluation showed trisomy of chromosome 21.
The findings on echocardiography were normal. Abdominal sonography and color Doppler sonography showed hepatomegaly (8.0 cm) with the liver exhibiting a normal outline and echotexture. No focal lesion was seen. The gallbladder was normal. No cord sign was noted. Intrahepatic biliary radicals were not dilated. The main portal vein and its right branch were normal in caliber and flow pattern.
The left portal vein was dilated and was communicating with a dilated left hepatic vein through a 0.3-cm aberrant venous channel (Fig. 1A). The left portal vein showed a continuous waveform signal on spectral Doppler sonography, whereas the left hepatic vein showed increased turbulent flow (Fig. 1B). The right and middle hepatic veins showed normal phasic flow. The superior mesenteric artery and veins were normal. There was no evidence of cirrhosis or portal hypertension and no history of percutaneous biopsy.
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The parents were advised that the child could develop congestive heart failure and hepatic encephalopathy and were offered the options of surgical intervention or coil embolization, both of which they refused. The child was discharged, with advice of undergoing regular sonographic follow-up to detect any increase or decrease in the shunt size.
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Congenital anomalies of the portal venous system result from abnormal coalescence of the vitello–umbilical venous plexus during embryogenesis. These rare anomalies include cavernous transformation of the portal vein, preduodenal portal vein or peribiliary portal vein; duplication of portal vein; and portal vein atresia [1]. A unique consequence of some of these anomalies is congenital portosystemic shunting of the splenic vein circulation [1].
Park et al. [3] classified four types of intrahepatic portosystemic venous shunts: Type I is a single large shunt with a constant diameter that connects the right portal vein to the inferior vena cava. This is the most common site. Type II is a localized peripheral shunt in which single or multiple communications are found between peripheral branches of the portal and hepatic veins in one hepatic segment. In type III, the peripheral portal and hepatic veins are connected through an aneurysm. In type IV, multiple communications between the peripheral portal and hepatic veins are seen diffusely in both the lobes. Our patient was considered to have a Park type II intrahepatic portosystemic venous shunt.
Color Doppler sonography combined with pulsed Doppler sonography is a highly efficient noninvasive procedure for diagnosis of intrahepatic portosystemic venous shunts that obviates angiography [2]. A diagnosis of congenital intrahepatic portosystemic venous shunt was made on color Doppler sonography in the index case.
Patients with congenital portosystemic shunts are usually asymptomatic in early life. They may present with features of symptomatic hyperammonemia and hepatic encephalopathy. In such cases, early and accurate diagnosis is important to prevent misdiagnosis as a psychiatric or neurologic disorder. Kitagawa et al. [1] reported that intrahepatic portosystemic venous shunts may be associated with congenital cardiac defects or abnormalities of the hepatobiliary system, including abnormal lobulation of the liver, hepatoblastoma, and extrahepatic biliary atresia.
The choice of treatment of intrahepatic portosystemic venous shunts is controversial. The vascular anomaly may regress spontaneously during infancy [4]. In cases of large shunts that cause encephalopathy, therapeutic intervention, such as surgical banding, should be considered. In surgical banding [5], only semiclosure of the shunt is performed because completely suturing the shunt might result in life-threatening complications related to severe portal hypertension if the intrahepatic portal venous system could not accommodate the sudden restoration of normal blood flow. Another option is angiographic intervention with coil embolization using stainless steel or platinum coils [5].
For maldevelopment of the intrahepatic portal veins, banding can be performed if the liver architecture is normal. However, coexisting malformation of hepatic veins and severe anomalies such as cardiac defects preclude the possibility of surgical intervention [5].
To summarize, our patient was a 1-month-old infant with trisomy 21 who had a single communication between the left portal and left hepatic veins (a type II intrahepatic portosystemic venous shunt) that probably developed from partial persistence of the left vitelline vein. We have come across only one previous case report in the literature of a patient with Down syndrome who had intrahepatic portosystemic venous shunt.
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