Severe cervical scoliosis in the fetus.

Objective: This study provides prenatal characteristics and postnatal outcomes of patients with severe cervical scoliosis.

Methods: A retrospective analysis of clinical information from cases prenatally diagnosed with severe cervical scoliosis (>90° angulation) in the Fetal Care Center at Cincinnati Children's Hospital Medical Center between 2007 and 2010 was performed. We excluded iniencephaly and tumors of the head or neck.

Results: We identified five patients with severe cervical scoliosis. The fetal spine abnormality was diagnosed between 18 and 27 weeks of gestation. Classical cesarean section was recommended in all patients. There were no immediate airway or neurological complications in the delivery room. Extravertebral anomalies were present in four of the five patients. These patients had a more complicated clinical course and three separate syndromes including a complex collagenopathy, heterotaxy, and Klippel-Feil syndrome were identified. One patient had an isolated cervical deformation secondary to a large uterine fibroid with a benign clinical course. None of the infants have required spinal surgery.

Conclusion: Our patients suggest that connective tissue diseases or heterotaxy may be important risk factors for the development of severe cervical scoliosis. There were no significant long-term complications directly related to cervical scoliosis.