, Le suivi régulier de ces enfants est nécessaire, afin d"éventuellement détecter une aggravation de l"HTP,adapter la prise en charge et diagnostiquer précocement les complications secondaires pour les traiter avant leur irréversibilité

C. Heller, R. Schobess, K. Kurnik, R. Junker, G. Günther et al., Abdominal venous thrombosis in neonates and infants: role of prothrombotic risk factors-a multicentre case-control study, Br J Haematol, vol.111, issue.2, pp.534-543, 2000.

B. L. Shneider, J. De-ville-de-goyet, D. H. Leung, A. Srivastava, S. C. Ling et al., Primary prophylaxis of variceal bleeding in children and the role of MesoRex Bypass: Summary of the Baveno VI Pediatric Satellite Symposium, Hepatol Baltim Md. avr, vol.63, issue.4, pp.1368-80, 2016.

P. Klemperer, Cavernomatous transformation of the portal vein: Its relation to Banti's disease, Arch.path, vol.6, p.353, 1928.

J. B. Gibson and R. L. Richards, Cavernous transformation of the portal vein, J Pathol Bacteriol. juill, vol.70, issue.1, pp.81-96, 1955.

J. Fraser and A. K. Brown, A clinical syndrome associated with a rare anomaly of the vena portae system, 1944.

H. Guo, F. Hao, C. Guo, and Y. Yu, Outcome of the Pediatric Patients with Portal Cavernoma: The Retrospective Study for 10 Years Focusing on Recurrent Variceal Bleeding, Gastroenterol Res Pract, p.7953870, 2016.

J. H. Kim, Y. S. Lee, S. H. Kim, S. K. Lee, M. K. Lim et al., Does umbilical vein catheterization lead to portal venous thrombosis? Prospective US evaluation in 100 neonates, Radiology. juin, vol.219, issue.3, pp.645-50, 2001.

A. Keir, R. Giesinger, and M. Dunn, How long should umbilical venous catheters remain in place in neonates who require long-term (?5-7 days) central venous access?, J Paediatr Child Health. août, vol.50, issue.8, pp.649-52, 2014.

I. Morag, M. Epelman, A. Daneman, R. Moineddin, B. Parvez et al., Portal vein thrombosis in the neonate: risk factors, course, and outcome, J Pediatr. juin, vol.148, issue.6, pp.735-744, 2006.

E. M. Fuchs, A. G. Sweeney, and J. W. Schmidt, Umbilical venous catheter-induced hepatic hematoma in neonates, J Neonatal-Perinat Med. 1 janv, vol.7, issue.2, pp.137-179, 2014.

S. K. Sethi, P. Dewan, M. Faridi, A. Aggarwal, and L. Upreti, Liver abscess, portal vein thrombosis and cavernoma formation following umbilical vein catherisation in two neonates, Trop Gastroenterol Off J Dig Dis Found. juin, vol.28, issue.2, pp.79-80, 2007.

H. Pergantou, M. Avgeri, A. Komitopoulou, P. Xafaki, Z. Kapsimali et al., Venous thromboembolism at uncommon sites in neonates and children, J Pediatr Hematol Oncol, vol.36, issue.8, pp.624-633, 2014.

C. Dubuisson, C. Boyer-neumann, M. Wolf, D. Meyer, and O. Bernard, Protein C, protein S and antithrombin III in children with portal vein obstruction, J Hepatol. juill, vol.27, issue.1, pp.132-137, 1997.

M. Caropreso, R. Campanile, S. Maddaluno, C. Veropalumbo, C. Piscopo et al., Genetic Prothrombotic Risk Factors in Children With Extrahepatic Portal Vein Obstruction, J Pediatr Gastroenterol Nutr. sept, vol.51, issue.3, p.374, 2010.

R. Sawamura, P. Louzada-junior, V. Ferriani, L. Ramalho, and M. Fernandes, Hepatoportal sclerosis and extrahepatic portal venous obstruction associated with anti-phospholipid antibody syndrome in child, J Trop Pediatr. juin, vol.57, issue.3, pp.222-226, 2011.

J. N. Schouten, J. Verheij, and S. Seijo, Idiopathic non-cirrhotic portal hypertension: a review, Orphanet J Rare Dis, vol.10, issue.1, 2015.

S. Franchi-abella, M. Fabre, E. Mselati, D. Marsillac, M. E. Bayari et al., Obliterative Portal Venopathy: A Study of 48 Children, J Pediatr. juill, vol.165, issue.1, pp.190-193, 2014.

N. Tantemsapya, R. Superina, D. Wang, G. Kronauer, P. F. Whitington et al., Hepatic Histology and Morphometric Measurements in Idiopathic Extrahepatic Portal Vein Thrombosis in Children, Correlated to Clinical Outcome of Meso-Rex Bypass, Ann Surg. déc, vol.1, 2016.

H. M. El-karaksy, N. El-koofy, N. Mohsen, H. Helmy, N. Nabil et al., Extrahepatic portal vein obstruction in Egyptian children, J Pediatr Gastroenterol Nutr. janv, vol.60, issue.1, pp.105-114, 2015.

B. Weiss, E. Shteyer, A. Vivante, D. Berkowitz, S. Reif et al., Etiology and long-term outcome of extrahepatic portal vein obstruction in children, World J Gastroenterol, vol.16, issue.39, pp.4968-72, 2010.

, Lakhdar Idrissi Mounia,1 et Hida Moustapha1. Le cavernome porte chez l'enfant : à propos de 11 cas, Tadmori Ilham, vol.1, 201419277.

M. Sbihi, A. Abkari, S. Chafai, N. Mikou, and H. Hadj-khalifa, Hypertension portal chez l'enfant : à propos de 75 cas, 2000.

U. Poddar, B. R. Thapa, K. Rao, and K. Singh, Etiological spectrum of esophageal varices due to portal hypertension in Indian children: is it different from the West?, J Gastroenterol Hepatol. sept, vol.23, issue.9, pp.1354-1361, 2008.

A. E. Oestreich and S. N. Zingula, Umbilical vein catheter tip-slight rightward or anterior shift and trajectory can indicate liver penetration. Pediatr Radiol, vol.44, pp.1470-1471, 2014.

A. Plessier, P. Rautou, and D. Valla, Management of hepatic vascular diseases, J Hepatol, vol.56, issue.1, pp.25-38, 2012.

A. Shukla, A. Gupte, V. Karvir, P. Dhore, and S. Bhatia, Long Term Outcomes of Patients with Significant Biliary Obstruction Due to Portal Cavernoma Cholangiopathy and Extra-Hepatic Portal Vein Obstruction (EHPVO) With No Shuntable Veins, J Clin Exp Hepatol. déc, vol.7, issue.4, pp.328-361, 2017.

M. Gauthier-villars, S. Franchi, F. Gauthier, M. Fabre, D. Pariente et al., Cholestasis in children with portal vein obstruction, J Pediatr. avr, vol.146, issue.4, pp.568-73, 2005.

B. Chiu and R. Superina, Extrahepatic portal vein thrombosis is associated with an increased incidence of cholelithiasis, J Pediatr Surg. juill, vol.39, issue.7, pp.1059-61, 2004.

R. N. Mehrotra, V. Bhatia, P. Dabadghao, and S. K. Yachha, Extrahepatic portal vein obstruction in children: anthropometry, growth hormone, and insulin-like growth factor I, J Pediatr Gastroenterol Nutr, vol.25, issue.5, pp.520-523, 1997.

S. K. Sarin, A. Bansal, S. Sasan, and A. Nigam, Portal-vein obstruction in children leads to growth retardation, Hepatol Baltim Md. févr, vol.15, issue.2, pp.229-262, 1992.

M. A. Bellomo-brandão, A. M. Morcillo, G. Hessel, S. R. Cardoso, F. Servidoni-m-de et al., Growth assessment in children with extra-hepatic portal vein obstruction and portal hypertension, Arq Gastroenterol. déc, vol.40, issue.4, pp.247-50, 2003.

R. J. Sokol, Portopulmonary Hypertension: Opportunities for Precision Pediatrics, J Pediatr Gastroenterol Nutr. sept, vol.61, issue.3, pp.268-277, 2015.

A. A. Condino, D. D. Ivy, O. Connor, J. A. Narkewicz, M. R. Mengshol et al., Portopulmonary Hypertension in Pediatric Patients, J Pediatr. 1 juill, vol.147, issue.1, pp.20-26, 2005.

D. Alagille, J. C. Carlier, M. Chiva, R. Ziadé, M. Ziadé et al., Long-term neuropsychological outcome in children undergoing portal-systemic shunts for portal vein obstruction without liver disease, J Pediatr Gastroenterol Nutr. déc, vol.5, issue.6, pp.861-867, 1986.

C. L. Mack, F. A. Zelko, J. Lokar, R. Superina, E. M. Alonso et al., Surgically restoring portal blood flow to the liver in children with primary extrahepatic portal vein thrombosis improves fluid neurocognitive ability, Pediatrics. mars, vol.117, issue.3, pp.405-412, 2006.

D. 'antiga, L. Dacchille, P. Boniver, C. Poledri, S. Schiff et al., Clues for minimal hepatic encephalopathy in children with noncirrhotic portal hypertension, J Pediatr Gastroenterol Nutr. déc, vol.59, issue.6, pp.689-94, 2014.

D. S. Goldberg and M. B. Fallon, Lung and Heart Disease Secondary to Liver Disease, Clin Gastroenterol Hepatol Off Clin Pract J Am Gastroenterol Assoc, vol.13, issue.12, p.2118, 2015.

V. V. Borkar, U. Poddar, A. Kapoor, S. Ns, A. Srivastava et al., Hepatopulmonary Syndrome in children: a comparative study of non-cirrhotic vs. cirrhotic portal hypertension, Liver Int. 1 juin, vol.35, issue.6, pp.1665-72, 2015.

K. Noli, M. Solomon, F. Golding, M. Charron, and S. C. Ling, Prevalence of hepatopulmonary syndrome in children, Pediatrics. mars, vol.121, issue.3, pp.522-527, 2008.

E. Ecochard-dugelay, V. Lambert, J. Schleich, M. Duché, E. Jacquemin et al., Portopulmonary Hypertension in Liver Disease Presenting in Childhood, J Pediatr Gastroenterol Nutr. sept, vol.61, issue.3, pp.346-54, 2015.

C. Liang, S. Ko, C. Chen, and A. M. Concejero, Impact of mild and moderate intrapulmonary shunting in children with end-stage liver disease undergoing liver transplantation, World J Surg. juill, vol.31, issue.7, pp.1474-1483, 2007.

P. Lykavieris, F. Gauthier, P. Hadchouel, M. Duche, and O. Bernard, Risk of gastrointestinal bleeding during adolescence and early adulthood in children with portal vein obstruction, J Pediatr. juin, vol.136, issue.6, pp.805-813, 2000.

J. P. Molleston, Variceal Bleeding in Children, J Pediatr Gastroenterol Nutr, vol.37, issue.5, pp.538-545, 2003.

C. L. Mack, R. A. Superina, and P. F. Whitington, Surgical restoration of portal flow corrects procoagulant and anticoagulant deficiencies associated with extrahepatic portal vein thrombosis, J Pediatr. févr, vol.142, issue.2, pp.197-206, 2003.

M. Odièvre, G. Pigé, and D. Alagille, Congenital abnormalities associated with extrahepatic portal hypertension, Arch Dis Child. mai, vol.52, issue.5, pp.383-388, 1977.

P. J. Mckiernan, K. Sharif, and G. L. Gupte, The role of endoscopic ultrasound for evaluating portal hypertension in children being assessed for intestinal transplantation, Transplantation, vol.86, issue.10, pp.1470-1473, 2008.

F. Alvarez, O. Bernard, F. Brunelle, P. Hadchouel, M. Odièvre et al., Portal obstruction in children. I. Clinical investigation and hemorrhage risk, J Pediatr. nov, vol.103, issue.5, pp.696-702, 1983.

A. Bertocchini, P. Falappa, C. Grimaldi, G. Bolla, L. Monti et al., Intrahepatic portal venous systems in children with noncirrhotic prehepatic portal hypertension: anatomy and clinical relevance, J Pediatr Surg. août, vol.49, issue.8, pp.1268-75, 2014.

C. Grimaldi, J. De-ville-de-goyet, and V. Nobili, Portal hypertension in children, Clin Res Hepatol Gastroenterol. juin, vol.36, issue.3, pp.260-261, 2012.

. Etiology, Hypertension in Children:A Single Center's Experiences

. Disponible,

S. Williams and A. Chan, Neonatal portal vein thrombosis: diagnosis and management, Semin Fetal Neonatal Med. déc, vol.16, issue.6, pp.329-368, 2011.

G. Schettino, E. Fagundes, M. Roquete, A. R. Ferreira, and F. J. Penna, Portal vein thrombosis in children and adolescents, J Pediatr (Rio J). juin, vol.82, issue.3, pp.171-179, 2006.

D. Alberti, M. Colusso, M. Cheli, P. Ravelli, A. Indriolo et al., Results of a stepwise approach to extrahepatic portal vein obstruction in children, J Pediatr Gastroenterol Nutr. nov, vol.57, issue.5, pp.619-645, 2013.

P. M. Ferri, A. R. Ferreira, E. Fagundes, S. M. Liu, M. Roquete et al., Portal vein thrombosis in children and adolescents: 20 years experience of a pediatric hepatology reference center, Arq Gastroenterol. mars, vol.49, issue.1, pp.69-76, 2012.

L. D'antiga, Medical management of esophageal varices and portal hypertension in children, Semin Pediatr Surg. août, vol.21, issue.3, pp.211-219, 2012.

T. B. Lautz, S. S. Sundaram, P. F. Whitington, L. Keys, and R. A. Superina, Growth impairment in children with extrahepatic portal vein obstruction is improved by mesenterico-left portal vein bypass, J Pediatr Surg, vol.44, issue.11, pp.2067-70, 2009.

J. Duché, M. Fabre, and A. , Survey on Clinical Practice of Primary Prophylaxis in Portal Hypertension in Children, J Pediatr Gastroenterol Nutr. avr, vol.64, issue.4, pp.524-531, 2017.

E. Fagundes, A. R. Ferreira, M. Roquete, F. J. Penna, E. Goulart et al., Clinical and laboratory predictors of esophageal varices in children and adolescents with portal hypertension syndrome, J Pediatr Gastroenterol Nutr. févr, vol.46, issue.2, pp.178-83, 2008.

D. Celioska-cedro, M. Teisseyre, M. Woynarowski, P. Socha, J. Socha et al., Endoscopic ligation of esophageal varices for prophylaxis of first bleeding in children and adolescents with portal hypertension: preliminary results of a prospective study, J Pediatr Surg. juill, vol.38, issue.7, pp.1008-1019, 2003.

M. Duché, B. Ducot, O. Ackermann, F. Guérin, E. Jacquemin et al., Portal hypertension in children: High-risk varices, primary prophylaxis and consequences of bleeding, J Hepatol. févr, vol.66, issue.2, pp.320-327, 2017.

Y. Eroglu, K. M. Emerick, P. F. Whitingon, and E. M. Alonso, Octreotide therapy for control of acute gastrointestinal bleeding in children, J Pediatr Gastroenterol Nutr. janv, vol.38, issue.1, pp.41-48, 2004.

O. 'meara, M. Cicalese, M. P. Bordugo, A. Ambrosi, A. Hadzic et al., Successful use of longacting octreotide for intractable chronic gastrointestinal bleeding in children, J Pediatr Gastroenterol Nutr. janv, vol.60, issue.1, pp.48-53, 2015.

A. El-hamid, N. Taylor, R. M. Marinello, D. Mufti, G. J. Patel et al., Aetiology and management of extrahepatic portal vein obstruction in children: King's College Hospital experience, J Pediatr Gastroenterol Nutr, vol.47, issue.5, pp.630-634, 2008.

T. Mitsunaga, H. Yoshida, K. Kouchi, T. Hishiki, T. Saito et al., Pediatric gastroesophageal varices: treatment strategy and long-term results, J Pediatr Surg. déc, vol.41, issue.12, pp.1980-1983, 2006.

D. A. Bambini, R. Superina, P. S. Almond, P. F. Whitington, and A. E. , Experience with the Rex shunt (mesenterico-left portal bypass) in children with extrahepatic portal hypertension, J Pediatr Surg. janv, vol.35, issue.1, pp.13-18, 2000.

G. Chocarro, P. T. Junco, E. Dominguez, M. V. Amesty, N. Cerezo et al., Portal Cavernoma in the Era of Mesoportal Shunt (Rex) and Liver Transplant in Children, Eur J Pediatr Surg Off J Austrian Assoc Pediatr Surg Al Z Kinderchir. févr, vol.26, issue.1, pp.7-12, 2016.

M. Zhang, C. Pu, Y. Li, and C. Guo, Sixty-four-slice computed tomography in surgical strategy of portal vein cavernous transformation, World J Gastroenterol, vol.17, issue.38, pp.4334-4342, 2011.

Y. Lv, C. He, W. Guo, Z. Yin, J. Wang et al., Transjugular Intrahepatic Portosystemic Shunt for Extrahepatic Portal Venous Obstruction in Children, J Pediatr Gastroenterol Nutr. févr, vol.62, issue.2, pp.233-274, 2016.

N. Gibelli, A. Tannuri, M. L. Pinho-apezzato, J. G. Maksoud-filho, and U. Tannuri, Extrahepatic portal vein thrombosis after umbilical catheterization: is it a good choice for Rex shunt?, J Pediatr Surg. janv, vol.46, issue.1, pp.214-220, 2011.

J. Li, W. Chen, C. Xu, R. Wang, and Y. Chen, Treatment of portal hypertension from portal vein cavernoma with the meso-Rex bypass, Chin Med J (Engl). mars, vol.126, issue.5, pp.971-974, 2013.

K. Sharif, P. Mckiernan, and J. De-ville-de-goyet, Mesoportal bypass for extrahepatic portal vein obstruction in children: close to a cure for most!, J Pediatr Surg. janv, vol.45, issue.1, pp.272-278, 2010.

R. Dasgupta, E. Roberts, R. A. Superina, and P. C. Kim, Effectiveness of Rex shunt in the treatment of portal hypertension, J Pediatr Surg. janv, vol.41, issue.1, pp.108-112, 2006.

J. Fuchs, S. Warmann, R. Kardorff, H. Rosenthal, B. Rodeck et al., Mesenterico-left portal vein bypass in children with congenital extrahepatic portal vein thrombosis: a unique curative approach, J Pediatr Gastroenterol Nutr. févr, vol.36, issue.2, pp.213-219, 2003.

Y. R. Krishna, S. K. Yachha, A. Srivastava, D. Negi, R. Lal et al., Quality of life in children managed for extrahepatic portal venous obstruction, J Pediatr Gastroenterol Nutr. mai, vol.50, issue.5, pp.531-537, 2010.

C. Vasilescu, O. Stanciulea, M. Popa, A. Colita, and C. Arion, Subtotal laparoscopic splenectomy and esophagogastric devascularization for the thrombocytopenia because of portal cavernoma-case report, J Pediatr Surg. juill, vol.43, issue.7, pp.1373-1378, 2008.

R. B. Pinto, T. R. Silveira, E. Bandinelli, and L. Röhsig, Portal vein thrombosis in children and adolescents: the low prevalence of hereditary thrombophilic disorders, J Pediatr Surg. sept, vol.39, issue.9, pp.1356-61, 2004.

B. Koot, M. Alders, J. Verheij, U. Beuers, and J. M. Cobben, A de novo mutation in KCNN3 associated with autosomal dominant idiopathic non-cirrhotic portal hypertension, J Hepatol. avr, vol.64, issue.4, pp.974-981, 2016.

S. Vilarinho, S. Sari, G. Yilmaz, A. L. Stiegler, T. J. Boggon et al., Recurrent recessive mutation in deoxyguanosine kinase causes idiopathic noncirrhotic portal hypertension, Hepatol Baltim Md. juin, vol.63, issue.6, pp.1977-86, 2016.

C. Besmond, D. Valla, L. Hubert, K. Poirier, B. Grosse et al., Mutations in the novel gene FOPV are associated with familial autosomal dominant and non-familial obliterative portal venopathy, Liver Int Off J Int Assoc Study Liver. févr, vol.38, issue.2, pp.358-64, 2018.

M. Casanelles, J. J. Gil-fernández, L. Casero, M. G. Bengoechea, R. Serrano et al., Portal hypertension in Williams syndrome: report of two patients, Am J Med Genet A. 1 mai, vol.118, issue.4, pp.372-378, 2003.

F. Guérin, V. Bidault, E. Gonzales, S. Franchi-abella, D. Lambert et al., Meso-Rex bypass for extrahepatic portal vein obstruction in children, Br J Surg, vol.100, issue.12, pp.1606-1619, 2013.

, A Embryology.ch, schéma des veines vitellines au stade embryonnaire

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, D Seminars in Pediatric Surgery, 2005.

S. Hippocrate,

, Au moment d"être admis à exercer la médecine, en présence des maîtres de cette école et de mes condisciples, je promets et je jure d"être fidèle aux lois de l"honneur et de la probité qui la régissent

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