Surgically Restoring Portal Blood Flow to the Liver in Children With Primary Extrahepatic Portal Vein Thrombosis Improves Fluid Neurocognitive Ability

Abstract

OBJECTIVES. Children with primary extrahepatic portal vein thrombosis (EHPVT) have portal-systemic shunting, which may lead to disturbed neurocognitive function similar to portal-systemic encephalopathy (PSE) seen with chronic liver disease and cirrhosis. The functions most affected are those involving fluid cognitive ability, which comprise neurocognitive domains such as attention, processing speed, and short-term memory, that are particularly vulnerable to systemic illness or diffuse neurologic insult. We determined the fluid cognitive ability of children with EHPVT and whether surgically restoring portal blood flow by mesenteric left portal vein bypass (MLPVB) improved it.DESIGN. Twelve children with EHPVT and no overt PSE underwent comprehensive neurocognitive testing before and 1 year after undergoing surgery with intent to perform MLPVB. The evaluations sampled 4 functional domains at both time points: (1) neurobehavioral (behavior, emotional, executive functioning); (2) broad cognitive (intelligence, achievement); (3) fluid ability (attention, mental speed, working memory, memory encoding); and (4) visual motor (drawing, fine motor). Tasks in the fluid-ability and visual-motor domains were expected to be especially sensitive to adverse effects of EHPVT and to be most likely to show improvement with MLPVB. The test group consisted of 8 subjects who underwent successful MLPVB, and the comparison group was composed of 3 patients who received distal splenorenal shunts and one whose MLPVB failed.RESULTS. Both groups demonstrated similar fluid cognitive ability at initial evaluation. Successful MLPVB resulted in significantly improved fluid cognitive function: in the fluid cognitive domain, significant improvements were seen for the hit reaction time variability in the Conner's Continuous Performance Test, the attention scale of the Cognitive Assessment System, and immediate verbal memory in the Children's Memory Scale. In the visual-motor domain, z scores on the Grooved Pegboard Test improved. No improvement was observed in the comparison group.www.pediatrics.org/cgi

Mesoportal bypass for extrahepatic portal vein obstruction in children: close to a cure for most!

“…Improved linear growth was shown by Stringer [16]. Mack et al [17] and Chiu [18] showed that successful MPB improves fluid cognitive ability and reversed encephalopathy related to portosystemic shunting. Fuchs et al [19] reported the successful management, by MPB, of a patient with hepatopulmonary syndrome related to EPVO, which we have confirmed in 1 further case.…”

Long-term follow-up of children with extrahepatic portal vein obstruction: impact of an endoscopic sclerotherapy program on bleeding episodes, hepatic function, hypersplenism, and mortality

“…In the medical literature, there are few reports that have examined large groups of children who have undergone MLPVB, and longterm outcomes have not yet been assessed. No mortality related to the surgery was reported, but some complications were described, including an inability to create the shunt because of obliteration of the intrahepatic left portal vein, shunt thrombosis, and rebleeding [17]. In some children, MLPVB cannot be successfully performed because of an absent or rudimentary left portal vein.…”

Previous portal hypertension surgery negatively affects results of mesenteric to left portal vein bypass

“…As importantly, it has been shown to normalize a number of aspects of disordered hepatic homeostasis. Restoration of portal vein flow results in normalization of coagulation parameters [18], causes liver growth [19], and improves neurocognitive testing by eliminating symptoms and signs of subtle hepatic encephalopathy in children [20]. It has also been shown to reverse hepatopulmonary syndrome [21].…”