Endoscopic Endonasal Odontoidectomy in Pediatric Patients with Complex Cervicomedullary Junction Anomalies

Background Multiple factors contribute to the severity and complexity of basilar invagination or ventral brain stem compression. Surgery in the craniovertebral junction needs a careful preoperative review of the occasionally variable anatomical structures. The characterization of Chiari malformation continues to evolve, and recently, there has been extensive elucidation on the management of complex Chiari cases especially in pediatric patients. Attempts have been made to assess the sequelae of growth disruption and alignment of the cervical spine in pediatric patients after posterior fusion.

Objectives The purpose of this study was to evaluate the outcomes and effects of endonasal endoscopic odontoidectomy (for ventral brain stem compression) coupled with posterior upper cervical fusion in the developing spine. Considering the paucity of reported cases of endonasal endoscopic odontoidectomy for pediatric patients with craniovertebral junction abnormalities, we report a consecutive series of such cases at this institution.

Method Over a period of 5 years, between April 2011 and March 2016, eight patients with craniovertebral junction abnormalities underwent endonasal endoscopic odontoidectomy with posterior instrumented fusion at the New York Presbyterian Hospital, Weill Cornell Medical College. A majority of the cases were basilar invagination and Chiari malformation. All diagnoses were confirmed with neuroimaging, and standardized radiological parameters were assessed.

Results A total of 16 operations were done on eight pediatric patients for the treatment of craniocervical junction abnormalities. One patient had Ehlers–Danlos’ syndrome, while the majority had Chiari malformation and/or basilar invagination. Six patients were male, and two were female. No complications were recorded.

Conclusion The propensity of Chiari malformation to occur in the pediatric population, the laxity of the ligaments in this unique anatomical region, that is, craniocervical junction, spinal alignment in the pediatric population, rarity of certain syndromic pathologies, and the advent of minimally invasive techniques suitable for surgical management makes these patient subgroup an important one in which this pathology has to be carefully reviewed. The adult experience of this pathology has been widely reported unlike the pediatric experience (with only two cases reported in the literature). Our outcomes appeared more favorable in comparison to data reported with the adult cases.