Irreducible thoracic spondyloptosis in a child with neurofibromatosis: a rationale for treatment.
METHODS
A case report and review of literature are presented.


OBJECTIVE
To describe the surgical management of a midthoracic spondyloptosis associated with kyphosis in a child with neurofibromatosis, an extremely rare but a potentially high-morbidity complication.


BACKGROUND
Dystrophic kyphoscoliotic spinal deformity is the most common orthopedic sequela of neurofibromatosis. Spondyloptosis is a rare complication but with the potential for high morbidity if the diagnosis is missed or undertreated. Reported cases are rare.


METHODS
A severe thoracic spondyloptosis occurred in a 7-year-old girl with peripheral neurofibromatosis who presented with transient paraparesis after a fall. The kyphosis was reduced by cantilever correction forces, achieving side-to-side (bayonet) apposition rather than anatomic reduction of the spondyloptosis. This was followed by anterior spinal arthrodesis and structural grafting.


RESULTS
Two and a half years after the surgery there is no loss of correction, and the patient has remained neurologically recovered.


CONCLUSIONS
Posterior correction of the gibbus in a bayonet apposition and stabilization with a two-rod construct followed by anterior spinal arthrodesis and structural grafting seem to offer efficient surgical treatment.
Children's Hospital of Philadelphia, Division of Orthopaedic Surgery, Philadelphia, Pennsylvania 19104, USA.
B Guirguis
BG
Hanna
Stephan G
SG
Pill
Denis S
DS
Drummond
D002648:Child; D005260:Female; D006801:Humans; D007738:Kyphosis / Q000150:complications / Q000601:surgery*; D009456:Neurofibromatosis 1 / Q000150:complications*; D011859:Radiography; D012600:Scoliosis / Q000150:complications / Q000601:surgery*; D013168:Spondylolisthesis / Q000150:complications / Q000601:surgery*; D013904:Thoracic Vertebrae / Q000000981:diagnostic imaging / Q000601:surgery*; D016896:Treatment Outcome