Neurofibromatosis, gigantism, elephantiasis neuromatosa and recurrent massive subperiosteal hematoma: a new case report and review of 7 case reports from the literature.
The authors report the case of a 13-year-old patient with neurofibromatosis (NF-I), who suffered blunt trauma to the left tibia in 1993. The diagnosis of subperiosteal hematoma was made. Treatment consisted of temporary rest. There was a recurrence in 1996, and the subperiosteal hematoma was drained. In 1997, a shortening osteotomy of the left tibia was performed. However, massive gigantism with elephantiasis of the left leg remained, causing a serious functional and cosmetic problem. In 1999, the leg was amputated above the knee. The literature is reviewed and 7 case reports are compared. The pathogenesis of subperiosteal hematoma is discussed.
Steenbrugge
F
F
Department of Orthopedic Surgery, Ghent University Hospital, B-9000 Gent, Belgium.
Poffyn
B
B
Uyttendaele
D
D
Verdonk
R
R
Verstraete
K
K
D000293:Adolescent; D000671:Amputation, Surgical; D004604:Elephantiasis / Q000150:complications / Q000473:pathology* / Q000601:surgery; D005260:Female; D005877:Gigantism / Q000150:complications / Q000473:pathology* / Q000601:surgery; D006406:Hematoma / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007866:Leg / Q000473:pathology / Q000601:surgery; D017253:Neurofibromatoses / Q000150:complications / Q000473:pathology* / Q000601:surgery; D010521:Periosteum / Q000473:pathology*; D012008:Recurrence