Klippel Feil Syndrome Type III With Associated Rare Congenital Anomalies (Sprengel Deformity,Scoliosis,and Atlanto-Occipital Assimilation): A Rare Case Report

Abstract

Klippel Feil Syndrome (KFS) is a congenital anomaly having fusion of 2 or more cervical vertebral bodies characterized by the presence of classic triad of low posterior hairline, short neck, and restricted range of motion. We have reported a rare case of Type III Klippel Feil Syndrome having classical clinical triad with Sprengel deformity and atlantooccipital assimilation. The baby was born at Sanjay Gandhi Memorial Hospital, Mangolpuri, New Delhi, India. The detailed discussion is done in the case report. Prognosis is based on radiological classification by Samartzis et al which classifies KFS under 3 types with type II as the commonest variety. Associated anomalies include scoliosis or kyphosis, renal disease, Sprengel deformity, loss of hearing, synkinesis or mirror movements, congenital heart defects, craniofacial malformations, and skeletal abnormalities of ear, nose, mouth and larynx. Proper management requires multidisciplinary approach including neurologist, orthopedic surgeon, pediatrician, nurse practitioner, physical therapist, and neurosurgeon.