Portal vein thrombosis after splenectomy for hereditary stomatocytosis in childhood.
Portal vein thrombosis is a rare but potentially lethal complication in children requiring splenectomy. We report on a 15-year-old boy with a dehydrated hereditary stomatocytosis, who underwent splenectomy and presented a postoperative partial portal vein thrombosis. With prompt heparin therapy, neither propagation of the thrombus nor further cavernous transformation in the following occurred 6 years.


CONCLUSIONS
Recent data suggest that hereditary stomatocytosis carries a high risk of thrombotic complications, especially after splenectomy. This procedure, the benefit of which is limited in this condition, should therefore be strongly avoided.
Perel
Y
Y
Unité d'Onco-Hematologie Pediatrique, Hopital des Enfants, Centre Hospitalier et Universitaire de Bordeaux, France. yves.perel@chu-aquitaine.fr
Dhermy
D
D
Carrere
A
A
Chateil
J F
JF
Bondonny
J M
JM
Micheau
M
M
Barbier
R
R
D000293:Adolescent; D000745:Anemia, Hemolytic, Congenital / Q000601:surgery*; D006801:Humans; D008297:Male; D011169:Portal Vein; D013156:Splenectomy / Q000009:adverse effects*; D013927:Thrombosis / Q000209:etiology*