Obliterative portal venopathy: a study of 48 children

Stéphanie Franchi-Abella; Monique Fabre; Emmanuel Mselati; Marise Elia De Marsillac; Mohamed Bayari; Danièle Pariente; Emmanuel Jacquemin; Olivier Bernard

doi:10.1016/j.jpeds.2014.03.025

Obliterative portal venopathy: a study of 48 children

J Pediatr. 2014 Jul;165(1):190-193.e2. doi: 10.1016/j.jpeds.2014.03.025. Epub 2014 Apr 25.

Authors

Stéphanie Franchi-Abella¹, Monique Fabre², Emmanuel Mselati³, Marise Elia De Marsillac⁴, Mohamed Bayari⁴, Danièle Pariente¹, Emmanuel Jacquemin⁵, Olivier Bernard⁶

Affiliations

¹ Department of Pediatric Radiology, Hôpital Bicêtre AP-HP and Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
² Department of Histopathology, Hôpital Bicêtre AP-HP and Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
³ Department of Hematology and Biological Immunology, Hôpital Bicêtre AP-HP and Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
⁴ Department of Pediatric Hematology, Center de Référence National de l'atrésie des voies Biliaires, Hôpital Bicêtre AP-HP and Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
⁵ Department of Pediatric Hematology, Center de Référence National de l'atrésie des voies Biliaires, Hôpital Bicêtre AP-HP and Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France; Inserm U 757, Université Paris-Sud 11, Orsay, France.
⁶ Department of Pediatric Hematology, Center de Référence National de l'atrésie des voies Biliaires, Hôpital Bicêtre AP-HP and Université Paris-Sud 11, Hôpital Bicêtre, Le Kremlin-Bicêtre, France. Electronic address: [email protected].

PMID: 24768253
DOI: 10.1016/j.jpeds.2014.03.025

Abstract

Childhood obliterative portal venopathy presents at any age and may be genetic in origin. We report 48 children with obliterative portal venopathy, based on strict histologic criteria, investigated between 1972 and 2011. Diagnosis requires histology and is suggested by ultrasonography findings. Portal hypertension is the main complication but is absent in some cases. Prognosis is relatively good, but the detection of cardiopulmonary complications is essential.

MeSH terms

Adolescent
Child
Child, Preschool
Female
Humans
Hypertension, Portal / complications*
Hypertension, Portal / diagnosis
Infant
Infant, Newborn
Liver / pathology*
Male
Portal Vein / pathology*
Prognosis
Vascular Diseases / complications*
Vascular Diseases / diagnosis