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

Abstract

Congenital portosystemic shunts are present in one in 30,000 children. Among the associated risks of severe complications are neonatal cholestasis, benign and malignant liver tumors, hepatopulmonary syndrome, portopulmonary hypertension, and encephalopathy. They can be detected on prenatal ultrasonograms, during the investigation of a positive galactosemia screening test in neonates or of a complication, or be found fortuitously on an abdominal ultrasound. Small intrahepatic shunts may resolve spontaneously within one year of age, but other shunts such as extrahepatic, persistent ductus venosus or persisting intrahepatic shunts, must be closed in one or two steps, by interventional radiology techniques or surgically. The plasticity of the intrahepatic portal system allows revascularization of the liver after shunt closure, even when no intrahepatic portal structures can be detected on imaging studies. This leaves little or no place for liver transplantation in the management of these children.

Role of per-rectal portal scintigraphy in long-term follow-up of congenital portosystemic shunt

“…Various imaging modalities and blood tests indicated that the natural histories of intrahepatic and extrahepatic CPSSs differed. Intrahepatic CPSS without hepatic tumor has been reported to spontaneously close or regress, whereas extrahepatic CPSS does not spontaneously regress (3,7,8,(16)(17)(18). Ultrasonography and dynamic contrast-enhanced CT evaluation showed spontaneous reductions in the shunt size in all of our patients with intrahepatic CPSS but in none with extrahepatic CPSS, strengthening the previous findings.…”

“…Surgical repair or embolization may be recommended for extrahepatic CPSS without closure because of the high risk of hepatopulmonary syndrome, pulmonary hypertension, and hepatic encephalopathy (4,7,8,(13)(14)(15)(16). Stringer (8) described that CPSS-affected individuals are at risk of developing hepatic encephalopathy and/or an intrahepatic tumor depending largely on the volume and duration of the shunt.…”

“…c ongenital portosystemic shunt (CPSS), which is a major cause of neonatal hypergalactosemia without galactosemetabolizing-enzyme deficiency (1), causes brain manganese deposition, pulmonary hypertension, and hyperammonemia leading to hepatic encephalopathy (2)(3)(4)(5)(6)(7)(8). CPSS is generally suspected if serum total bile acid (TBA) and ammonia levels are elevated, and it is diagnosed by using color Doppler ultrasonography, dynamic contrast-enhanced computed tomography (CT), and per-rectal portal scintigraphy (PRPS) (7,(9)(10)(11)(12).…”

“…CPSS is generally suspected if serum total bile acid (TBA) and ammonia levels are elevated, and it is diagnosed by using color Doppler ultrasonography, dynamic contrast-enhanced computed tomography (CT), and per-rectal portal scintigraphy (PRPS) (7,(9)(10)(11)(12). Some shunts close spontaneously, whereas others need to be closed surgically or with embolization because of hyperammonemia leading to severe hepatic encephalopathy (7,8,(13)(14)(15). Therefore, it is important to follow up CPSS patients carefully with color Doppler ultrasonography, dynamic contrast-enhanced CT, and blood tests.…”

Congenital intrahepatic portosystemic shunt diagnosed during intrauterine life

“…- 8 , 11 It may also be associated with: membranoproliferative glomerulonephritis, hyperinsulinemia with hyper- and hypoglycemia, hypothyroxinemia, hyperandrogenism, pancreatitis, and heart failure 812 There are reports in the literature suggesting that IUGR, as seen in our patient, may also be shunt-related 89 , 13 The explanation for IUGR is the reduction of liver perfusion caused by the shunt.…”