Embryologically, the combination of transdiaphragmatic duplication, mediastinal cystic lesion, anterior spina bifida, and hemivertebra suggest notochord malformation.
A half of the patients with Klippel Feil syndrome KFS have hemivertebrae; however, the incidence of hemivertebrae showed no significant difference in congenital scoliosis (CS) patients with KFS and without KFS (P>0.05).
The incidence of KFS was 5.42% in patients with CS. Congenitally fused cervical patterns are more common in the mid and lower cervical spine region. The incidence of rib anomalies, intraspinal abnormities, and hemivertebra was not increased in CS patients with KFS 1)
Posterior cervical hemivertebra associated with instability is a very rare finding. The anterior approach with corpectomy and anterior plate enables suitable stabilization 2).
A 18-year-old female with intractable shoulder and neck pain and progressive weakness in the upper extremities, harbouring a cervical intradural lipoma with intramedullary extension, along with concomitant scoliosis.
Despite its benign nature, surgical treatment of these lesions in symptomatic patients generally provides satisfactory relief of symptoms. Radical removal of spinal intradural lipomas is not recommended since attempts at complete excision carry an unacceptable risk of postoperative morbidity and sufficient decompression with or without duraplasty generally provides a successful clinical outcome 3).