Surgical intervention for patent ductus venosus

Abstract

Patent ductus venosus (PDV) is a rare condition, which usually presents secondary to hepatic atrophy and hepatic failure. We have treated eight cases of PDV, all with hypergalactosemia and hyperbilirubinemia. Ultrasonography and three-dimensional computed tomography demonstrated communication between the portal vein and the inferior vena cava. Of the eight PDV cases, three from the older age group (ages 9, 11, and 14 years) had high-density lesions in their brain nucleus, and one case (age 19 years) had undergone prior Kasai portoenterostomy for biliary atresia. Six PDV patients underwent ligation of PDV and the remaining two cases underwent partial banding of PDV with intraoperative monitoring to maintain portal vein pressure (PVP) under 30 cm H(2)O. Improvement of the intrahepatic portal vein flow was achieved by ligation or banding of PDV. Postoperatively, serum galactose and bilirubin fell to normal ranges, but portal thrombus occurred postoperatively in the first case. We subsequently administered postoperative anticoagulation in the remaining cases and experienced no major complications. These results suggest that PDV ligation and banding are effective surgical approaches for patients with PDV. Close postoperative monitoring to avoid portal thrombus is imperative in these cases.

Postprandial Hyperinsulinaemic Hypoglycaemia Secondary to a Congenital Portosystemic Shunt

“…However, it was confirmed that the visible intrahepatic portal venous system is actually also present in patients with PSS and surgical closure of PSS can be successfully done [16]. Surgical treatment options for PSS include ligation, banding, coiling and stenting [17,18]. …”

Congenital Portosystemic Shunts in Children: Recognition, Evaluation, and Management

“…Two studies use the results of neonatal screening for galactosemia after a few days of feeding to estimate the prevalence of congenital portosystemic shunts 144,145 : High concentrations of blood galactose, unexplained by an abnormal activity of the enzymes of galactose metabolism, can be found in neonates 11,16,24,25,27,29,38,41,44,51,53,55,59,64,68,70,76,84,94,96,101,105,106,[118][119][120][121]126 with CPSS because galactose from milk bypasses the liver. The overall prevalence is close to 1:30,000 births and the prevalence of permanent CPSS can be estimated at 1:50,000.…”

“…In all other cases, closure of the shunt should be considered early, to prevent complications from occurring, for four reasons: (1) hepatopulmonary syndrome and pulmonary hypertension may be present during the very first years of life; (2) the regression of pulmonary hypertension cannot be ascertained once irreversible lesions of the pulmonary arteries are present; (3) chronic hyperammonemia and high blood levels of manganese have adverse effects on the developing brain 160,161 ; and (4) the plasticity of the intrahepatic portal system may be better in younger children. 121 The plasticity of the intrahepatic portal system will allow revascularization of the liver even when the intrahepatic portal system is not detectable on imaging studies and when portal pressure is high during an occlusion test. The experience gained over the past 15 years indicates that there is very little place left, if any, for liver transplantation in the management of congenital portosystemic shunts in children.…”

Inherited liver shunts in dogs elucidate pathways regulating embryonic development and clinical disorders of the portal vein

“…From several literature reviews and case reports, the disease appears to be more prevalent in the Japanese population. In total, there have been 173 human cases of EHPSS reported (Caruso et al 2010; Franchi-Abella et al 2010; Kobayashi et al 2010; Konstas et al 2010; Lautz et al 2011; Newman et al 2010; Ohno et al 2008) and 89 human cases of IHPSS (Ferrero et al 2010; Franchi-Abella et al 2010; Kamimatsuse et al 2010; Konstas et al 2010; Ohno et al 2008; Schierz et al 2011; Stringer 2008; Tsai et al 2009; Uchino et al 1999; Yoshimoto et al 2004). In human EHPSS, a classification has been made based on the presence or absence of portal blood flow (Abernethy 1793).…”