Etiology and long-term outcome of extrahepatic portal vein obstruction in children

Weiss, Batia; Shteyer, Eyal; Vivante, Asaf; Berkowitz, Drora; Reif, Shimon; Weizman, Zvi; Bujanover, Yoram; Shapiro, Rivka

doi:10.3748/wjg.v16.i39.4968

Cited by 71 publications

(64 citation statements)

References 21 publications

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“…As observed in the present study, PVT does not show gender predominance and its more common initial manifestations in children are upper gastrointestinal bleeding and splenomegaly, which corroborates results reported in the literature (1,6,41,47,50,53) . In the present casuistic, upper gastrointestinal bleeding was the main initial clinical manifestation in 52.7% of patients, followed by splenomegaly in 36.4%.…”

Section: Discussionsupporting

confidence: 81%

“…(1) and Weiss et al (53) reported a greater proportion of patients with splenomegaly as the initial finding (63% and 43.3%, respectively). Thus, the possibility of PVT should be raised and a Doppler ultrasound exam should be requested in children and adolescents with UGIB or isolated finding of splenomegaly during the clinical exam.…”

Section: Discussionmentioning

confidence: 99%

“…Cases that do not fit in these groups are denominated as idiopathic PVT and, due to the great percentage of these cases, recent studies, mainly in adults, have focused on the evaluation of new etiological factors (7,16,17,22,23) . In the pediatric age group, the few available literature has been demonstrating that approximately 50% of the cases still remained with an unknown etiology (1,2,20,53) . This fact was also observed in the present study.…”

Section: Discussionmentioning

confidence: 99%

“…It should also be emphasized that a clinical situation of hypersplenism, manifested through thrombocytopenia associated with splenomegaly, is also frequently observed in pediatrics cases and, most of the time, the patients are referred firstly to the hematologist (6,41,44,50) . Today, the PVT etiology is considered multifactorial, with association of prothrombotic and local trigger factors found even in the pediatric age group (6,10,21,23,29,33,34,37,47,52,53) . The causes of PVT may be grouped into three categories: direct injury of the portal vein with subsequent thrombus formation; vascular malformation which includes stenosis or even atresia of the portal vein; and hypercoagulable conditions which favor the thrombus formation (6,15,19,22,39,49,50,52) .…”

Section: Discussionmentioning

confidence: 99%

“…Approximately 79% of the children diagnosed with PVT will show at least one episode of upper gastrointestinal bleeding during their lives (2,41) . In the literature there are few reports on PVT in children and adolescents, being some of them also studies about casuistic descriptions and others about specific characteristics of the disease as thrombophilia (1,17,20,32,43,45,48,53,54,55) . Due to the scarcity of studies in the pediatric age group and the importance of the theme, this study`s objective is to describe the clinical characteristics, diagnosis, evolution and treatment of the portal hypertension in children and adolescents with diagnosis of portal vein thrombosis assisted at the Pediatric Hepatology Clinic of the Hospital das Clínicas of Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brazil.…”

Section: Introductionmentioning

confidence: 99%

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Portal vein thrombosis in children and adolescents: 20 years experience of a pediatric hepatology reference center

Ferri

Ferreira

Fagundes

et al. 2012

Arq. Gastroenterol.

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-Context -Portal vein thrombosis refers to a total or partial obstruction of the blood flow in this vein due to a thrombus formation. It is an important cause of portal hypertension in the pediatric age group with high morbidity rates due to its main complication -the upper gastrointestinal bleeding. Objective -To describe a group of patients with portal vein thrombosis without associated hepatic disease of the Pediatric Hepatology Clinic of the Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil with emphasis on diagnosis, presentation form and clinical complications, and the treatment of portal hypertension. Methods -This is a descriptive study of a series of children and adolescents cases assisted from January 1990 to December 2010. The portal vein thrombosis diagnosis was established by ultrasound. Results -Of the 55 studied patients, 30 (54.5%) were male. In 29 patients (52.7%), none of the risk factors for portal vein thrombosis was observed. The predominant form of presentation was the upper gastrointestinal bleeding (52.7%). In 20 patients (36.4%), the initial manifestation was splenomegaly. During the whole following period of the study, 39 patients (70.9%) showed at least one episode of upper gastrointestinal bleeding. The mean age of patients in the first episode was 4.6 ± 3.4 years old. The endoscopic procedure carried out in the urgency or electively for search of esophageal varices showed its presence in 84.9% of the evaluated patients. The prophylactic endoscopic treatment was performed with endoscopic band ligation of varices in 31.3% of patients. Only one died due to refractory bleeding. Conclusions -The portal vein thrombosis is one of the most important causes of upper gastrointestinal bleeding in children. In all non febrile children with splenomegaly and/or hematemesis and without hepatomegaly and with normal hepatic function tests, it should be suspect of portal vein thrombosis. Thus, an appropriate diagnostic and treatment approach is desirable in an attempt to reduce morbidity and mortality.

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Section: Discussionsupporting

confidence: 81%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Portal vein thrombosis in children and adolescents: 20 years experience of a pediatric hepatology reference center

Ferri

Ferreira

Fagundes

et al. 2012

Arq. Gastroenterol.

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Primary prophylaxis of variceal bleeding in children and the role of MesoRex Bypass: Summary of the Baveno VI Pediatric Satellite Symposium

et al. 2015

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Approaches to the management of portal hypertension and variceal hemorrhage in pediatrics remain controversial, in large part because they are not well informed by rigorous clinical studies. Fundamental biological and clinical differences preclude automatic application of approaches used for adults to children. On April 11-12, 2015, experts in the field convened at the first Baveno Pediatric Satellite Meeting to discuss and explore current available evidence regarding indications for MesoRex bypass (MRB) in extrahepatic portal vein obstruction and the role of primary prophylaxis of variceal hemorrhage in children. Consensus was reached regarding MRB. The vast majority of children with extrahepatic portal vein obstruction will experience complications that can be prevented by successful MRB surgery. Therefore, children with extrahepatic portal vein obstruction should be offered MRB for primary and secondary prophylaxis of variceal bleeding and other complications, if appropriate surgical expertise is available, if preoperative and intraoperative evaluation demonstrates favorable anatomy, and if appropriate multidisciplinary care is available for postoperative evaluation and management of shunt thrombosis or stenosis. In contrast, consensus was not achieved regarding primary prophylaxis of varices. Although variceal hemorrhage is a concerning complication of portal hypertension in children, the first bleed appears to be only rarely fatal and the associated morbidity has not been well characterized

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Wedged hepatic vein portovenography for assessment of Rex vein patency in children with extrahepatic portal venous obstruction

Kaur,

Khanna,

Sood

et al. 2024

J. pediatr. gastroenterol. nutr.

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BackgroundMeso‐Rex bypass is the surgical intervention of choice for children with extrahepatic portal vein obstruction (EHPVO). Patency of Rex vein, umbilical recessus of the portal vein, is a prerequisite for this surgery. Conventional diagnostic modalities poorly detect patency, while transjugular wedged hepatic vein portography (WHVP) accurately detects patency in 90%.ObjectivesWe aimed to assess Rex vein patency and portal vein branching pattern in children with EHPVO using transjugular WHVP and to identify factors associated with Rex vein patency.MethodsTransjugular WHVP was performed in 31 children with EHPVO by selective cannulation of left and right hepatic veins. Rex vein patency, type of intrahepatic portal venous anatomy (Types A–E), and factors associated with patency of Rex vein were studied.ResultsThe patency of Rex recess on transjugular WHVP was 29%. Complete obliteration of intrahepatic portal venous radicles was the commonest pattern (Type E, 38.7%) while Type A, the favorable anatomy for meso‐Rex bypass, was seen in only 12.9%. Patency of the Rex vein, but not the anatomical pattern, was associated with younger age at evaluation (patent Rex: 6.6 ± 4.9 years vs. nonpatent Rex: 12.7 ± 3.9 years, p = 0.001). Under‐5‐year children had a 12 times greater chance of having a patent Rex vein (odds ratio: 12.22, 95% confidence interval: 1.65–90.40, p = 0.004). Patency or pattern was unrelated to local factors like umbilical vein catheterization, systemic thrombophilia, or disease severity.ConclusionLess than one‐third of our pediatric EHPVO patients have a patent Rex vein. Younger age at evaluation is significantly associated with Rex vein patency.

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Etiology and long-term outcome of extrahepatic portal vein obstruction in children

Abstract: EHPVO is a rare disorder. The etiological factors are still mostly unknown, and the endoscopic and surgical treatment options ensure a good long-term prognosis.

Cited by 71 publications

References 21 publications

Portal vein thrombosis in children and adolescents: 20 years experience of a pediatric hepatology reference center

Portal vein thrombosis in children and adolescents: 20 years experience of a pediatric hepatology reference center

Primary prophylaxis of variceal bleeding in children and the role of MesoRex Bypass: Summary of the Baveno VI Pediatric Satellite Symposium

Wedged hepatic vein portovenography for assessment of Rex vein patency in children with extrahepatic portal venous obstruction

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