title: Vanishing bone disease of chest wall and spine with kyphoscoliosis and neurological deficit: A case report and review of literature.
abstract: 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 4th-7thribs 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.
authors.affiliation: Department of Orthopaedics, Seth G.S. Medical College and KEM Hospital, Mumbai, Maharashtra, India.
authors.forename: Sudhir Kumar
authors.initials: SK
authors.lastname: Srivastava
authors.affiliation: Department of Orthopaedics, Seth G.S. Medical College and KEM Hospital, Mumbai, Maharashtra, India.
authors.forename: Rishi Anil
authors.initials: RA
authors.lastname: Aggarwal
authors.affiliation: Department of Orthopaedics, Seth G.S. Medical College and KEM Hospital, Mumbai, Maharashtra, India.
authors.forename: Pradip Sharad
authors.initials: PS
authors.lastname: Nemade
authors.affiliation: Department of Orthopaedics, Seth G.S. Medical College and KEM Hospital, Mumbai, Maharashtra, India.
authors.forename: Sunil Krishna
authors.initials: SK
authors.lastname: Bhoale
keywords: Chest wall; Gorham disease; Osteolysis; bone; grafting; idiopathic osteolysis; kyphosis; spine. chest wall; vanishing bone disease