title: [Portal cavernoma in children revealed by gastrointestinal haemorrhage: about a case].
abstract: Portal cavernoma is a venous vascular anomaly characterized by the formation of a network of veins whose caliber is increased and carrying portal blood. It is due to a thrombotic and always chronic occlusion of the extra-hepatic portal venous system. This is one of the most common causes of portal hypertension in children. Its severity is mainly associated with an high risk of gastrointestinal haemorrhage. Very few cases have been described mainly in African literature. We report the case of a 4-year old boy admitted with very abundant haematemesis, melena and dizziness associated with anemic syndrome on examination. Laboratory tests showed severe microcytic hypochromic anemia with normal renal and hepatic function. Gastrointestinal endoscopy showed esophageal varices (grade III) with red signs. Abdominal ultrasound showed portal vein formation resulting in the classic "spiderweb", in favor of a cavernoma. Abdominal CT scan confirmed portal cavernoma associated with portal hypertensive syndrome and vascular anomaly like an ectopic splenic vein anastomosis with the trunk formed by the gonadal vein and the inferior mesenteric vein. Therapeutic approach was based on blood transfusion and beta-blocker treatment. Portal cavernoma can be a major complication of vascular malformations often unknown. In case of gastrointestinal haemorrhage in children, diagnosis should be suspected. Its management requires early treatment and should be adapted to the patient's condition in order to prevent a fatal evolution.
authors.affiliation: Hôpital pour Enfants de Diamniadio, Université de Thiès, Thiès, Sénégal.
authors.forename: Idrissa
authors.initials: I
authors.lastname: Basse
authors.affiliation: Hôpital pour Enfants de Diamniadio, Université de Thiès, Thiès, Sénégal.
authors.forename: Ndéye Rama Diagne
authors.initials: NR
authors.lastname: Guèye
authors.affiliation: Hôpital pour Enfants de Diamniadio, Université de Thiès, Thiès, Sénégal.
authors.forename: Dina Cyrienne Obambi
authors.initials: DC
authors.lastname: Diop
authors.affiliation: Hôpital pour Enfants de Diamniadio, Université de Thiès, Thiès, Sénégal.
authors.forename: Ndiémé Ndiaye
authors.initials: NN
authors.lastname: Diawara
authors.affiliation: Hôpital d'Enfants Albert Royer, Université Cheikh Anta Diop de Dakar, Dakar, Sénégal.
authors.forename: Aïssatou
authors.initials: A
authors.lastname: Ba
authors.affiliation: Hôpital Régional de Saint-Louis, Service de Pédiatrie, Université de Saint-Louis, Saint-Louis, Sénégal.
authors.forename: Ndiogou
authors.initials: N
authors.lastname: Seck
authors.affiliation: Hôpital d'Enfants Albert Royer, Université Cheikh Anta Diop de Dakar, Dakar, Sénégal.
authors.forename: Aliou
authors.initials: A
authors.lastname: Thiongane
authors.affiliation: Hôpital d'Enfants Albert Royer, Université Cheikh Anta Diop de Dakar, Dakar, Sénégal.
authors.forename: Abou
authors.initials: A
authors.lastname: Ba
authors.affiliation: Hôpital pour Enfants de Diamniadio, Université de Thiès, Thiès, Sénégal.
authors.forename: Aliou Abdoulaye
authors.initials: AA
authors.lastname: Ndongo
authors.affiliation: Hôpital d'Enfants Albert Royer, Université Cheikh Anta Diop de Dakar, Dakar, Sénégal.
authors.forename: Amadou Lamine
authors.initials: AL
authors.lastname: Fall
authors.affiliation: Hôpital Abass Ndao, Service de Pédiatrie, Université Cheikh Anta Diop de Dakar, Dakar, Sénégal.
authors.forename: Djibril
authors.initials: D
authors.lastname: Boiro
authors.affiliation: Hôpital de la Paix de Ziguinchor, Université de Ziguinchor, Ziguinchor, Sénégal.
authors.forename: Lamine
authors.initials: L
authors.lastname: Thiam
authors.affiliation: Hôpital pour Enfants de Diamniadio, Université de Thiès, Thiès, Sénégal.
authors.forename: Marie
authors.initials: M
authors.lastname: Mbengue
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D001803:Blood Transfusion; D002675:Child, Preschool; D016099:Endoscopy, Gastrointestinal / Q000379:methods; D004932:Esophageal and Gastric Varices / Q000473:pathology; D006471:Gastrointestinal Hemorrhage / Q000209:etiology*; D006392:Hemangioma, Cavernous / Q000150:complications / Q000000981:diagnostic imaging* / Q000628:therapy; D006396:Hematemesis / Q000209:etiology; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology*; D008297:Male; D011169:Portal Vein / Q000000981:diagnostic imaging / Q000473:pathology*; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed
chemical_list: D000319:Adrenergic beta-Antagonists
keywords: Portal cavernoma; abdominal CT scan; abdominal ultrasound; child; endoscopy