Open AccessVanishing bone disease of chest wall and spine with kyphoscoliosis and neurological deficit: A case report and review of literature
Author(s) -
Sudhir Srivastava,
R. K. Aggarwal,
Pradip Sharad Nemade,
Sunil Krishna Bhoale
Publication year - 2017
Publication title -
indian journal of orthopaedics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.434
H-Index - 33
eISSN - 1998-3727
pISSN - 0019-5413
DOI - 10.4103/0019-5413.197559
Subject(s) - medicine , kyphoscoliosis , surgery , bone grafting , kyphosis , asymptomatic , deformity , decortication , nonunion , scoliosis , radiography
Vanishing bone disease is an extremely rare disorder of unknown etiology characterized by idiopathic osteolysis of bone. We describe a case of vanishing bone disease of chest wall and spine with kyphoscoliosis and neurological deficit. A 17-year-old male presented with gradually progressive deformity of back and dorsal compressive myelopathy with nonambulatory power in lower limbs. Radiographs revealed absent 4 th -7 th ribs on the right side with dorsal kyphoscoliosis and severe canal narrowing at the apex. The patient was given localized radiotherapy and started on a monthly infusion of 4 mg zoledronic acid. Posterior instrumented fusion with anterior reconstruction via posterolateral approach was performed. The patient had a complete neurological recovery at 5 weeks following surgery. At 1 year, anterior nonunion was noted for which transthoracic tricortical bone grafting was done. Bone graft from the patient's mother was used both times. At 7 months following anterior grafting, the alignment was maintained and the patient was asymptomatic; however, fusion at graft-host interface was not achieved. Bisphosphonates and radiotherapy were successful in halting the progress of osteolysis.