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–7th 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.

Vanishing Bone Disease of the Thoracic Cage: Challenges in the Management of a Rare Entity

“…Srivastava SK et al reported on a patient presenting with VBD of the ribs on the right side and spine. He presented with flattening of the chest on the right side and a thoracic kyphoscoliosis which was surgically corrected 13 . Kakhaki AD et al reported a 48-year-old man who presented with dyspnea and recurrent episodes of pleural effusion following a blunt trauma of the chest who was later diagnosed to have VBD with massive osteolysis of the ribs.…”

“…As far as other cases in the literature are concerned, cases involving the rib cage are limited. Srivastava et al 13 reported the case of a patient presenting with VBD of the ribs on the right side and spine. He presented with flattening of the chest on the right side and thoracic kyphoscoliosis, which was surgically corrected.…”

Gorham‑Stout disease in the rib and thoracic spine with spinal injury treated with radiotherapy, zoledronic acid, vitamin D, and propranolol: A case report and literature review

“…However, various locations, such as the humerus, scapula, clavicle, ribs, sternum, pelvis, and femur, can be affected by GSD (21). In spinal GSD, 46% of cases reportedly occurred in the thoracic vertebrae (22,23). Involvement of both the thoracic vertebrae and ribs is extremely rare, with only three cases being reported in the literature (23–25).…”

“…In spinal GSD, 46% of cases reportedly occurred in the thoracic vertebrae (22,23). Involvement of both the thoracic vertebrae and ribs is extremely rare, with only three cases being reported in the literature (23–25). One of these reports was a 17-year-old man who had osteolysis in his ribs and thoracic spine, with nonambulatory power in lower limbs due to myelopathy.…”

“…Thereafter, posterior instrument fixation with anterior reconstruction was performed. These treatments succeeded in halting the progression of osteolysis and the patient showed complete neurological recovery (23). Another report was for a 2-year-old girl, who had lesions in the femur in addition to the thoracic spine and ribs.…”