High Cervical Partial Posterior Cord Cleft in a Case of Klippel-Feil Syndrome

Posterior high cervical spinal cord cleft has been rarely reported in association with Klippel - Feil syndrome (KFS). Its presence may have prognostic value in long term neurological outcome. If there is segmentation anomaly of two or more vertebrae during embryonic life mainly involving the cervical vertebrae, it can result in Klippel - Feil syndrome. It is a rare anomaly and is present with the incidence of 1 : 42000 births.1 During 2nd to 8th week of embryonic life, the segmentation of the mesodermal somites of the spine in the cervical region fails and leads to cervical spine synostosis which is also known as KFS. Although most commonly the KFS patients present with the symptoms of short length of the neck, hairline posteriorly is low and the mobility in the upper part of the spine is restricted. It is also sometimes associated with other congenital anomalies like Sprengel’s deformity, hemivertebra, basilar impression, cleft palate, and many more rare anomalies.2 Most commonly the patients having this syndrome show restriction of the motion in the neck region.3