Congenital Cervical Scoliosis at the Craniovertebral Junction

Background: Congenital cervical scoliosis at the craniovertebral junction is an exceedingly rare condition, with limited existing research. In this study, we aimed to elucidate the clinical characteristics, surgical strategies, and postoperative outcomes associated with this unique pathology, providing a comprehensive analysis to enhance clinical understanding and management. Methods: We conducted a retrospective analysis involving 27 patients with congenital cervical scoliosis at the craniovertebral junction who underwent surgery at a mean age of 7.81 ± 1.52 years. The median follow-up duration was 36.00 months. Patients were categorized into Group A and Group B on the basis of the absence or presence of concomitant subaxial cervical scoliosis, respectively. Three distinct surgical strategies were employed accordingly. Radiographic parameters were measured preoperatively, within 5 days postoperatively, and at the final follow-up. Other clinical and surgical characteristics were also collected. Results: Patients exhibited preoperative coronal imbalance, with a mean structural Cobb angle of 30.75° ± 13.09° and a mean head shift of 20.34 ± 13.23 mm. At the final follow-up, these parameters had significantly improved to a median of 3.00° and 8.59 mm (both p < 0.05). The mean operative time was 473.74 ± 134.29 minutes, and the mean intraoperative blood loss was 336.11 ± 166.52 mL. Among the 27 patients, 10 were in Group A and the other 17 were in Group B. Twenty-three patients/families reported being “satisfied” or “very satisfied” with the surgical outcome at the final follow-up. Postoperative complications occurred in 11 patients; no nerve root or vertebral artery injuries were observed. Conclusions: Congenital cervical scoliosis at the craniovertebral junction is a complex deformity that severely influences coronal alignment. Nevertheless, tailored surgical strategies have shown promising effectiveness in achieving satisfactory clinical and psychological results. Level of Evidence: Therapeutic Level III . See Instructions for Authors for a complete description of levels of evidence.