Congenital pseudarthrosis of the ulna and radius in two cases of neurofibromatosis type 1.
Two children with neurofibromatosis type 1 who presented at birth with congenital pseudarthrosis of the ulna and radius are described. The patients were treated with broad resections. As a consequence, the forearms were reduced in length. The osteotomies were stabilized in one patient first with endomedullary nailing and then with a free vascularized fibular graft. In the second patient the osteotomy was stabilized by external fixation. Using these techniques, rapid and excellent healing and normal function were achieved. In contrast to the lower extremity, reduction of the length of the forearm can be accepted to a certain extent. If necessary, an extension osteotomy can be performed at a later date.
Ramelli
G P
GP
Department of Pediatrics, Ospedale San Giovanni, 6500 Bellinzona, Switzerland.
Slongo
T
T
Tschäppeler
H
H
Weis
J
J
D016025:Bone Transplantation; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D005594:Fracture Fixation, Intramedullary; D005598:Fractures, Spontaneous / Q000235:genetics* / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009456:Neurofibromatosis 1 / Q000235:genetics* / Q000601:surgery; D010027:Osteotomy; D011542:Pseudarthrosis / Q000235:genetics* / Q000601:surgery; D011885:Radius Fractures / Q000235:genetics* / Q000601:surgery; D014458:Ulna Fractures / Q000235:genetics* / Q000601:surgery