KLIPPEL FEIL SYNDROME - A CASE REPORT

Klippel Feil syndrome (KFS) - a rare congenital anomaly with a prevalence of around 1 in 40,000 live births (1) characterised by fusion of 2 or more cervical vertebra with a triad of low posterior hair line, short neck and restricted cervical range of motion with associated abnormalities which includes scoliosis, renal abnormalities, sprengel deformity (2), deafness, synkinesis (3), congenital heart diseases (4). Fewer than 50 percent of patients with KFS have the classical triad (5). Here we present a case report of a 11 year old boy with classical triad of KFS with severe neurologic pain in neck and both upper limbs. Child also had associated anomalies like syringomyelia(C3-L3), aqueductal stenosis, bilateral omovertebral bone. Child was managed by sub-occipital craniectomy, foramen magnum decompression, C5,6,7 hemilaminectomy and physiotherapy. Postoperatively his neurological pain subsided significantly. This case is reported here not only because of its rarity, but also to emphasize that early surgical intervention will prevent neurological deficits as in our case.