SYRINGOMYELIA ASSOCIATED WITH HAJDU-CHENEY SYNDROME - CASE-REPORT

OBJECTIVE AND IMPORTANCE Hajdu-Cheney syndrome is a rare idiopathic bo ne disease based on a generalized bone-dysplasia accompanied by acro-o steolysis. We describe a surgical case of this syndrome that was accom panied by neurological signs associated with cervical syringomyelia. C LINICAL PRESENTATION: A 41-year-old woman was referred to our hospital with mild quadriparesis and sensory disturbance resulting from a car accident. there was a neck injury. She showed almost all of the major characteristic clinical features and roentgenographic findings of Hajd u-Cheney syndrome with syringomyelia. INTERVENTION: Surgical treatment was indicated because of the progressive sive neurological deficits. Foramen magnum decompression and C1 laminectomy were performed, and th e dura was exposed. The dura was opened at the area of the foramen mag num and C1. The occipitocervical posterior fusion-was carried out with an iliac bone graft titanium wires. CONCLUSION: Postoperatively, quad riparesis and sensory disturbance improved and the patient showed impr oved ambulation. Magnetic resonance imaging disclosed the well-decompr essed foramen magnum. The syringomyelia disappeared in the segmental a rea of C2 and was decreased in the segmental areas of C5-T6. The treat ment of this syndrome is symptomatic. In this patient magnetic resonan ce imaging disclosed compression of the brain stem by basilar invagina tion and platybasia, disturbance of cerebrospinal fluid flow at the le vel of the foramen magnum, and syringomyelia. It was suspected that th e obstruction of cerebrospinal fluid-flow at the level of foramen magn um caused the cervical syringomyelia. However, the long-term prognosis remains uncertain. Follow-up is necessary to assess the final result of the treatment.