Gorham's Disease of Spine
A 23-year-old male patient presented to us in June 2011 with a deformity of the back, weakness of both lower limbs, and with a history of multiple surgeries on the back. At three years of age, spontaneous disappearance of the right clavicle and upper humerus was noticed without any preceding trauma. Biopsy was taken from the lesion and diagnosis of Gorham's disease was made based on the histopathological examination of the tissue. The disease was subsequently not progressive. In May 2010, he noticed progressive deformity of the upper back, spasticity, and an altered gait. He was diagnosed with myelopathy and kyphotic deformity of the spine. On August ninth 2010 he pedicle screw instrumentation with decompression of the cord. The patient experienced an uneventful post-op period. His spasticity reduced and his gait improved over a period of two months and he was then able to walk normally. On May 25th 2011, a routine follow up X-ray showed breaking of the implant for which a revision surgery was performed the same day. Post-surgically, the patient developed complete weakness of both lower limbs with loss of bladder and bowel control. He was taken for immediate decompression. As the patient's neurology did not improve, a revision decompression was performed on 3 days later. The patient noticed the return of some movement in his right lower limb a few days after surgery. His bladder control returned one month later. He was referred to us for rehabilitation (, ).\nOn examination, the patient showed dyspnoea, tachypnoea, and paradoxical breathing. Neurological examination revealed complete absence of motor power on the left lower limb and the power in the right lower limb was 1-2/5. Sensation was decreased from T5 below. Deep tendon reflexes were exaggerated with ankle clonus and extensor plantars. A loss of the rounded contour was observed on the right shoulder with painless exaggerated passive movements. A chest X-ray revealed pleural effusion on the left side. Chylous looking fluid was aspirated from the left side of the chest and on examination showed lymphocytes. The patient was booked for a thoracic duct ligation. He was also given the option of radiotherapy and pleural ablation. The patient refused any of these treatments. Since the patient's condition remained stable and the osteolysis was not progressing, he was kept under observation.\nAfter 8 months of rehabilitation, the motor power in the lower limbs gradually improved and he was able to stand and walk in parallel bars with the help of left Anklefoot Orthrosisand bilateral knee gaiters. The disease did not show any progression clinically.