{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T17:52:45Z","timestamp":1790099565282,"version":"4.0.1"},"reference-count":45,"publisher":"American Academy of Pediatrics (AAP)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2006,3,1]]},"abstract":"<jats:p>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.<\/jats:p>\n                  <jats:p>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.<\/jats:p>\n                  <jats:p>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.<\/jats:p>\n                  <jats:p>DISCUSSION. The results show that surgically restoring portal flow to the liver in children with primary EHPVT results in improved fluid cognitive ability. Subjects showed some neurocognitive abnormalities involving mainly fluid cognitive ability consistent with minimal PSE seen in adults with chronic liver disease. Cognitive defects in patients with minimal PSE seem to relate primarily to attention and fine motor skill, and although affected patients can function in everyday life, they are at risk for performance deficits in educational and vocational situations requiring the ability to pay close attention and react quickly (eg, driving, employment in manufacturing). The tests we administered in these domains are pediatric equivalents to measures used to detect minimal PSE in adults and should detect abnormalities in the same functional domains. Our results suggest that a narrow battery of tests could be used to detect minimal PSE in children in a manner similar to the 5-test battery used in adults, eliminating the need for the comprehensive and broad testing we performed. Our findings suggest that shunting of portal blood from the liver in primary EHPVT can result in PSE and question whether it is as benign a disease as previously thought. The importance of our findings is twofold. For understanding the pathophysiology of PSE, we have shown that restoring blood flow to the liver improves cognitive function in children with EHPVT. For therapy for EHPVT, it becomes clear that MLPVB is an excellent treatment option. It is effective for treating the complications of portal hypertension and provides effective portal blood flow that other medical and surgical therapies do not. The findings provide additional evidence that primary EHPVT should be considered curable by MLPVB. However, comparison of overall risks and benefits of MLPVB with those of other therapeutic options and longer-term outcome studies must be completed before MLPVB can be fully endorsed as the best treatment for EHPVT in children.<\/jats:p>\n                  <jats:p>CONCLUSIONS. Surgical restoration of portal venous flow to the liver in children with primary EHPVT by MLPVB improves fluid cognitive ability. MLPVB should be considered in treating primary EHPVT, because it corrects portal blood flow and could optimize learning potential.<\/jats:p>","DOI":"10.1542\/peds.2005-1177","type":"journal-article","created":{"date-parts":[[2006,2,15]],"date-time":"2006-02-15T20:43:26Z","timestamp":1140036206000},"page":"e405-e412","source":"Crossref","is-referenced-by-count":99,"title":["Surgically Restoring Portal Blood Flow to the Liver in Children With Primary Extrahepatic Portal Vein Thrombosis Improves Fluid Neurocognitive Ability"],"prefix":"10.1542","volume":"117","author":[{"given":"Cara L.","family":"Mack","sequence":"first","affiliation":[{"name":"Pediatrics"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank A.","family":"Zelko","sequence":"additional","affiliation":[{"name":"Psychiatry"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joan","family":"Lokar","sequence":"additional","affiliation":[{"name":"Pediatrics"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Riccardo","family":"Superina","sequence":"additional","affiliation":[{"name":"Surgery"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estella M.","family":"Alonso","sequence":"additional","affiliation":[{"name":"Pediatrics"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andres T.","family":"Blei","sequence":"additional","affiliation":[{"name":"Medicine, Northwestern University Feinberg School of Medicine, Children's Memorial Hospital, Chicago, Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter F.","family":"Whitington","sequence":"additional","affiliation":[{"name":"Pediatrics"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[2006,3,1]]},"reference":[{"key":"2021120820132185600_R1","doi-asserted-by":"crossref","unstructured":"Alonso EM, Hackworth C, Whitington PF. Portal hypertension in children. Clin Liver Dis.\u20081997;1:201\u2013222","DOI":"10.1016\/S1089-3261(05)70264-6"},{"key":"2021120820132185600_R2","doi-asserted-by":"crossref","unstructured":"Alvarez F, Bernard O, Brunelle F, Hadchouel P, Odievre M, Alagille D. Portal obstruction in children. I. Clinical investigation and hemorrhage risk. J Pediatr.\u20081983;103:696\u2013702","DOI":"10.1016\/S0022-3476(83)80460-0"},{"key":"2021120820132185600_R3","doi-asserted-by":"crossref","unstructured":"Alvarez F, Bernard O, Brunelle F, Hadchouel P, Odievre M, Alagille D. Portal obstruction in children. II. Results of surgical portosystemic shunts. J Pediatr.\u20081983;103:703\u2013707","DOI":"10.1016\/S0022-3476(83)80461-2"},{"key":"2021120820132185600_R4","unstructured":"Weissenborn K. Clinical features of hepatic encephalopathy. In: Zakim D, Boyer TD, eds. Hepatology. 4th ed. Philadelphia, PA: WB Saunders; 2003:431\u2013444"},{"key":"2021120820132185600_R5","doi-asserted-by":"crossref","unstructured":"Ferenci P, Lockwood A, Mullen K, Tarter R, Weissenborn K, Blei AT. Hepatic encephalopathy: definition, nomenclature, diagnosis, and quantification\u2014final report of the working party at the 11th World Congresses of Gastroenterology, Vienna, 1998. Hepatology.\u20082002;35:716\u2013721","DOI":"10.1053\/jhep.2002.31250"},{"key":"2021120820132185600_R6","unstructured":"Dennis M. Childhood medical disorders and cognitive impairment: Biological risk, time, development, and reserve. In: Yeates KO, Ris MD, Taylor HG, eds. Pediatric Neuropsychology: Research, Theory, and Practice. New York, NY: Guilford Press; 2000:10\u201313"},{"key":"2021120820132185600_R7","doi-asserted-by":"crossref","unstructured":"Blei AT, Cordoba J. Subclinical encephalopathy. Dig Dis.\u20081996;14(suppl 1):2\u201311","DOI":"10.1159\/000171577"},{"key":"2021120820132185600_R8","doi-asserted-by":"crossref","unstructured":"Weissenborn K, Heidenreich S, Ennen J, Ruckert N, Hecker H. Attention deficits in minimal hepatic encephalopathy. Metab Brain Dis.\u20082001;16:13\u201319","DOI":"10.1023\/A:1011654210096"},{"key":"2021120820132185600_R9","doi-asserted-by":"crossref","unstructured":"Del Piccolo F, Sacerdoti D, Amodio P, et al. Central nervous system alterations in liver cirrhosis: the role of portal-systemic shunt and portal hypoperfusion. Metab Brain Dis.\u20082003;18:51\u201362","DOI":"10.1023\/A:1021930702815"},{"key":"2021120820132185600_R10","doi-asserted-by":"crossref","unstructured":"Boyer TD. Transjugular intrahepatic portosystemic shunt: current status. Gastroenterology.\u20082003;124:1700\u20131710","DOI":"10.1016\/S0016-5085(03)00377-9"},{"key":"2021120820132185600_R11","doi-asserted-by":"crossref","unstructured":"de Ville de Goyet J, Alberti D, Clapuyt P, et al. Direct bypassing of extrahepatic portal venous obstruction in children: a new technique for combined hepatic portal revascularization and treatment of extrahepatic portal hypertension. J Pediatr Surg.\u20081998;33:597\u2013601","DOI":"10.1016\/S0022-3468(98)90324-4"},{"key":"2021120820132185600_R12","doi-asserted-by":"crossref","unstructured":"Bambini DA, Superina R, Almond PS, Whitington PF, Alonso E. Experience with the Rex shunt (mesenterico-left portal bypass) in children with extrahepatic portal hypertension. J Pediatr Surg.\u20082000;35:13\u201318","DOI":"10.1016\/S0022-3468(00)80005-6"},{"key":"2021120820132185600_R13","doi-asserted-by":"crossref","unstructured":"Fuchs J, Warmann S, Kardorff R, et al. Mesenterico-left portal vein bypass in children with congenital extrahepatic portal vein thrombosis: a unique curative approach. J Pediatr Gastroenterol Nutr.\u20082003;36:213\u2013216","DOI":"10.1097\/00005176-200302000-00010"},{"key":"2021120820132185600_R14","doi-asserted-by":"crossref","unstructured":"Gehrke I, John P, Blundell J, Pearson L, Williams A, de Ville de Goyet J. Meso-portal bypass in children with portal vein thrombosis: rapid increase of the intrahepatic portal venous flow after direct portal hepatic reperfusion. J Pediatr Surg.\u20082003;38:1137\u20131140","DOI":"10.1016\/S0022-3468(03)00257-4"},{"key":"2021120820132185600_R15","doi-asserted-by":"crossref","unstructured":"de Ville de Goyet J, Gibbs P, Clapuyt P, Reding R, Sokal EM, Otte JB. Original extrahilar approach for hepatic portal revascularization and relief of extrahepatic portal hypertension related to later portal vein thrombosis after pediatric liver transplantation. Long term results. Transplantation.\u20081996;62:71\u201375","DOI":"10.1097\/00007890-199607150-00015"},{"key":"2021120820132185600_R16","doi-asserted-by":"crossref","unstructured":"Superina R, Bambini DA, Lokar J, Rigsby C, Mack CL, Whitington PF. Correction of extrahepatic portal vein thrombosis by the mesenteric to left portal vein bypass. Ann Surg.\u20082005; In press","DOI":"10.1097\/01.sla.0000205827.73706.97"},{"key":"2021120820132185600_R17","doi-asserted-by":"crossref","unstructured":"Mack CL, Superina RA, Whitington PF. Surgical restoration of portal flow corrects procoagulant and anticoagulant deficiencies associated with extrahepatic portal vein thrombosis. J Pediatr.\u20082003;142:197\u2013199","DOI":"10.1067\/mpd.2003.93"},{"key":"2021120820132185600_R18","unstructured":"Achenbach TM. Manual for the Child Behavior Checklist 4-18 and the 1991 Profile. Burlington, VT: University of Vermont Department of Psychiatry; 1991"},{"key":"2021120820132185600_R19","doi-asserted-by":"crossref","unstructured":"Gioia GA, Isquith P, Guy S, Kenworthy L. Behavior Rating Inventory of Executive Function (BRIEF): Professional Manual. Odessa, FL: Psychological Assessment Resources; 2000","DOI":"10.1076\/chin.6.3.235.3152"},{"key":"2021120820132185600_R20","unstructured":"Kovacs M. The Children's Depression Inventory (Manual). North Tonawanda, NY: Multi-Health Systems, Inc; 1992"},{"key":"2021120820132185600_R21","unstructured":"Wechsler D. Manual for the Wechsler Intelligence Scale for Children. 3rd ed. San Antonio, TX: The Psychological Corporation; 1991"},{"key":"2021120820132185600_R22","doi-asserted-by":"crossref","unstructured":"Donders J. A short form of the WISC-III for clinical use. Psychol Assess.\u20081997;9:15\u201320","DOI":"10.1037\/1040-3590.9.1.15"},{"key":"2021120820132185600_R23","unstructured":"Wilkinson G. Wide Range Achievement Test. 3rd ed. Wilmington, DE: Wide Range, Inc; 1993"},{"key":"2021120820132185600_R24","unstructured":"Conners C. Conners' Continuous Performance Test. Toronto, ON, Canada: Multi-Health Systems, Inc; 1992"},{"key":"2021120820132185600_R25","unstructured":"Naglieri JA, Das JP. Cognitive Assessment System. Itasca, IL: Riverside; 1997"},{"key":"2021120820132185600_R26","unstructured":"Cohen M. Children's Memory Scale (Manual). San Antonio, TX: The Psychological Corporation; 1997"},{"key":"2021120820132185600_R27","unstructured":"Schretlen D. Brief Test of Attention (Professional Manual). Odessa, FL: Psychological Assessment Resources; 1997"},{"key":"2021120820132185600_R28","unstructured":"Beery KE. The Beery-Buktenica Developmental Test of Visual Motor Integration: With Supplemental Developmental Tests of Visual Perception and Motor Coordination (Administration, Scoring, and Teaching Manual). 4th ed. Revised ed. Parsippany, NJ: Modern Curriculum Press; 1997"},{"key":"2021120820132185600_R29","unstructured":"Matthews CG, Klove H. Instruction Manual for the Adult Neuropsychology Test Battery. Madison, WI: University of Wisconsin Medical School; 1964"},{"key":"2021120820132185600_R30","doi-asserted-by":"crossref","unstructured":"Berk PD, Martin JF, Blaschke TF, Scharschmidt BF, Plotz PH. Unconjugated hyperbilirubinemia: physiologic evaluation and experimental approaches to therapy. Ann Intern Med.\u20081975;82:552\u2013570","DOI":"10.7326\/0003-4819-82-4-552"},{"key":"2021120820132185600_R31","doi-asserted-by":"crossref","unstructured":"Howard ER. Encephalopathy without cirrhosis: hunt the shunt. Gut.\u20081999;45:329\u2013330","DOI":"10.1136\/gut.45.3.329"},{"key":"2021120820132185600_R32","doi-asserted-by":"crossref","unstructured":"Ishii Y, Inagaki Y, Hirai K, Aoki T. Hepatic encephalopathy caused by congenital extrahepatic portosystemic venous shunt. J Hepatobiliary Pancreat Surg.\u20082000;7:524\u2013528","DOI":"10.1007\/s005340070026"},{"key":"2021120820132185600_R33","doi-asserted-by":"crossref","unstructured":"Kerlan RK Jr, Sollenberger RD, Palubinskas AJ, Raskin NH, Callen PW, Ehrenfeld WK. Portal-systemic encephalopathy due to a congenital portocaval shunt. AJR Am J Roentgenol.\u20081982;139:1013\u20131015","DOI":"10.2214\/ajr.139.5.1013"},{"key":"2021120820132185600_R34","doi-asserted-by":"crossref","unstructured":"Raskin NH, Price JB, Fishman RA. Portal-systemic encephalopathy due to congenital intrahepatic shunts. N Engl J Med.\u20081964;270:225\u2013229","DOI":"10.1056\/NEJM196401302700503"},{"key":"2021120820132185600_R35","doi-asserted-by":"crossref","unstructured":"Raskin NH, Bredesen D, Ehrenfeld WK, Kerlan RK. Periodic confusion caused by congenital extrahepatic portacaval shunt. Neurology.\u20081984;34:666\u2013669","DOI":"10.1212\/WNL.34.5.666"},{"key":"2021120820132185600_R36","doi-asserted-by":"crossref","unstructured":"Uchino T, Matsuda I, Endo F. The long-term prognosis of congenital portosystemic venous shunt. J Pediatr.\u20081999;135:254\u2013256","DOI":"10.1016\/S0022-3476(99)70031-4"},{"key":"2021120820132185600_R37","doi-asserted-by":"crossref","unstructured":"Wakamoto H, Manabe K, Kobayashi H, Hayashi M. Subclinical portal-systemic encephalopathy in a child with congenital absence of the portal vein. Brain Dev.\u20081999;21:425\u2013428","DOI":"10.1016\/S0387-7604(99)00049-2"},{"key":"2021120820132185600_R38","doi-asserted-by":"crossref","unstructured":"Watanabe A. Portal-systemic encephalopathy in non-cirrhotic patients: classification of clinical types, diagnosis and treatment. J Gastroenterol Hepatol.\u20082000;15:969\u2013979","DOI":"10.1046\/j.1440-1746.2000.02283.x"},{"key":"2021120820132185600_R39","doi-asserted-by":"crossref","unstructured":"Crespin J, Nemcek A, Rehkemper G, Blei AT. Intrahepatic portal-hepatic venous anastomosis: a portal-systemic shunt with neurological repercussions. Am J Gastroenterol.\u20082000;95:1568\u20131571","DOI":"10.1111\/j.1572-0241.2000.02096.x"},{"key":"2021120820132185600_R40","doi-asserted-by":"crossref","unstructured":"Eroglu Y, Donaldson J, Sorensen LG, et al. Improved neurocognitive function after radiologic closure of congenital portosystemic shunts. J Pediatr Gastroenterol Nutr.\u20082004;39:410\u2013417","DOI":"10.1097\/00005176-200410000-00019"},{"key":"2021120820132185600_R41","doi-asserted-by":"crossref","unstructured":"de Ville de Goyet J, Alberti D, Falchetti D, et al. Treatment of extrahepatic portal hypertension in children by mesenteric-to-left portal vein bypass: a new physiological procedure. Eur J Surg.\u20081999;165:777\u2013781","DOI":"10.1080\/11024159950189573"},{"key":"2021120820132185600_R42","unstructured":"Conners CK, Staff M. Conners' Continuous Performance Test-II: Technical Guide and Software Manual. Toronto, ON, Canada: Multi-Health Systems, Inc; 2000"},{"key":"2021120820132185600_R43","doi-asserted-by":"crossref","unstructured":"Wiltfang J, Nolte W, Otto M, et al. Elevated serum levels of astroglial S100beta in patients with liver cirrhosis indicate early and subclinical portal-systemic encephalopathy. Metab Brain Dis.\u20081999;14:239\u2013251","DOI":"10.1023\/A:1020785009005"},{"key":"2021120820132185600_R44","doi-asserted-by":"crossref","unstructured":"Plauth M, Roske AE, Romaniuk P, Roth E, Ziebig R, Lochs H. Post-feeding hyperammonaemia in patients with transjugular intrahepatic portosystemic shunt and liver cirrhosis: role of small intestinal ammonia release and route of nutrient administration. Gut.\u20082000;46:849\u2013855","DOI":"10.1136\/gut.46.6.849"},{"key":"2021120820132185600_R45","doi-asserted-by":"crossref","unstructured":"Kramer L, Bauer E, Gendo A, et al. Neurophysiological evidence of cognitive impairment in patients without hepatic encephalopathy after transjugular intrahepatic portosystemic shunts. Am J Gastroenterol.\u20082002;97:162\u2013166","DOI":"10.1111\/j.1572-0241.2002.05441.x"}],"container-title":["Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/117\/3\/e405\/1070577\/zpe0030600e405.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/117\/3\/e405\/1070577\/zpe0030600e405.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,8]],"date-time":"2021-12-08T18:52:11Z","timestamp":1638989531000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.aap.org\/pediatrics\/article\/117\/3\/e405\/68623\/Surgically-Restoring-Portal-Blood-Flow-to-the"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,3,1]]},"references-count":45,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2006,3,1]]},"published-print":{"date-parts":[[2006,3,1]]}},"URL":"https:\/\/doi.org\/10.1542\/peds.2005-1177","relation":{},"ISSN":["0031-4005","1098-4275"],"issn-type":[{"value":"0031-4005","type":"print"},{"value":"1098-4275","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,3,1]]}}}