Sprengel deformity and Klippel-Feil syndrome leading to cervical myelopathy presentation in old age
A 50-year-old woman admitted to the Alzahra Hospital, Isfahan, Iran. She presented with a 2 years history of neck pain and ataxia for 1 year. She hadn't urinary incontinence and she was referred to a neurosurgeon by a Neurologist because of her progressive gait ataxia.\nNeurological examination showed intact cranial nerves and no motor deficit, but we found impairment in pain sensory and light touch in both legs that was prominent on the right side of the body, hyperreflexia in the left knee, ankle jerk, and mild gait ataxia based on tandem and blind walking. Blood tests and urinalysis were normal. Physical examination revealed a short neck, a low occipital hairline and diminished cervical range of motion. The patient also had an elevated left scapula and a bony prominence extending from the shoulder to the neck. The range of motion of the neck and left shoulder was restricted. Anteroposterior and lateral radiographs revealed fused vertebral body of C5-C6 without spina bifida [Figures , and ].\nRadiographs in flexion and extension position demonstrated neither vertebral instability nor narrowing of the retrodental distance in ante- and retroflection. Plain thoracic and lumbar radiographs showed scoliosis with no other spinal anomalies. Subsequent Magnetic Resonance (MR) and computed tomography imaging with three dimensional reconstructions of the neck and shoulder revealed an atypical bone configuration of the left shoulder with elevation and dysplasia of the scapula and an aberrant bony structure extending from the superomedial border of the scapula to the C5 transverse process leading to constriction of the spinal canal [Figures –3]. These findings were interpreted as omovertebral bone with associated unilateral SD and KFS. Resection of the omovertebral bone and decompression of the spinal canal were indicated with respect to patient's clinical myelopathy.\nA curved incision was performed over the omovertebral bone from its cervical origin to its scapular termination. The intraspinal localized aberrant bony fragment, which we considered responsible for the neurological syndrome, was removed from the cleft in the posterior arch of C5, a laminectomy was performed at that level, and the omovertebral bone was partially resected.\nIn terms of gait ataxia the patient was neurologically unchanged, but she experienced a significant reduction in her neck pain, improved range motion of the neck, and the cosmetic result was good immediately after the operation. She recovered well from the procedure and was discharged from the hospital 3 days post-operatively. Follow-up radiographs demonstrated no further bony compression. There was no evidence of new instability resulting from the operative procedure occurred.