Patent ductus venosus with a hypoplastic intrahepatic portal system presenting intrapulmonary shunt: A case treated with banding of the ductus venosus

Clinical features of congenital portosystemic shunt in children

“…Although spontaneous closure of CPSS can be expected to occur until about the age of 2 years, close observation is necessary for the detection of neuropsychiatric symptoms and pulmonary hypertension. Closure of the shunt persisting after age 2 years should be considered because CPSS has risks of severe complications in children [6]. For symptomatic CPSS, surgical ligation or transcatheter embolization is a therapeutic option.…”

“…Complete occlusion of the shunt in patients with a severely hypoplastic portal vein could result in acute portal hypertension and may lead to edema or necrosis of the colon [17]. Balloon occlusion test is useful before the closure of the shunt by measuring portal pressure while transiently occluding the shunt [6]. Extrahepatic shunts arising from the main portal vein are short and wide in diameter, making endovascular closure unsuccessful and require staged surgical closure [9].…”

“…Extrahepatic shunts arising from the main portal vein are short and wide in diameter, making endovascular closure unsuccessful and require staged surgical closure [9]. Banding of the shunt might result in increased intrahepatic portal branches [6]. If the portal pressure is too high to allow closure in one step, temporary surgical banding of the shunt allows sufficient intrahepatic portal expansion to permit definite closure after verifying that intrahepatic portal flows have developed satisfactorily [4,6].…”

The clinical anatomy of congenital portosystemic venous shunts

“…There is one report of hyperinsulinism and hyperandrogenism secondary to a type 1 shunt (Satoh et al, 2001) and two reports of hepatopulmonary syndrome with a type 2 shunt (Kamata et al, 2000;Tercier et al, 2006); both of these complications are a consequence of portosystemic venous shunting. Either type 1 or type 2 portocaval shunts can be associated with neonatal jaundice (Arana et al, 1997;Howard and Davenport, 1997), as in Case 5 in this series.…”

Interventional stent implantation in a child with patent ductus venosus and pulmonary hypertension

“…Two years later, no¯ow could be traced in the stent using ultrasound. A comparable stepwise approach of PDV closure was used by Kamata et al [7] by banding the PDV in a 6-year-old boy with a hypoplastic intrahepatic portal system and consecutive hepatopulmonary syndrome. The PDV was ligated 10 months later without clinical deterioration.…”