Transoral Approach to the Craniovertebral Junction: A Case Series of Three Rare Pathologies

The transoral approach (TOA) to the craniovertebral junction (CVJ) provides direct midline access to ventral compressive pathologies but remains associated with notable technical challenges and complications. We present our institution's case series of rare CVJ pathologies, highlighting operative nuances and patient outcomes. Three extremely rare and complex CVJ cases, including Klippel-Feil syndrome with severe stenosis, anterior spinal artery aneurysm, and ventral cervicomedullary pial arteriovenous malformation, undergoing tailored transoral interventions were retrospectively analyzed for technical details, outcomes, and complications. Successful ventral decompression was achieved in all cases. Unique operative techniques, including transmandibular and transpalatal osteotomies, were critical for accessing these rare lesions. Complications, including cerebrospinal fluid leakage, prolonged dysphagia, and staged posterior fusion, underscored the surgical complexity. At the six-month follow-up, all patients showed neurological stability or improvement. The TOA remains an essential technique for the management of ventral CVJ pathology, particularly in cases where posterior or endonasal approaches are insufficient. Optimal outcomes are achieved through detailed preoperative planning, multidisciplinary collaboration, and vigilant management of approach-related complications. This series illustrates the ongoing role of TOA in the management of rare and technically demanding CVJ pathologies where alternative methods may prove inadequate.