Childs Nerv Syst. 2025 Dec 6;41(1):407. doi: 10.1007/s00381-025-07075-x.
ABSTRACT
PURPOSE: Composite split cord malformations (SCM) are rare congenital anomalies characterized by spinal cord division at two or more discontinuous levels. Cervical SCMs are particularly uncommon, with very few cases documented. We present what appears to be the first reported case of dual cervical SCM, highlighting its clinical presentation, surgical management, and review of literature.
METHODS: A 15-year-old male presented with congenital neck deformity, webbing, torticollis, and fixed hyperextension, but no neurological deficits. MRI and CT imaging revealed scoliosis with multiple block vertebrae and type I SCM at C3-C4 and C7-D1 levels, along with a tethered cord. The patient underwent staged surgical management consisting of excision of bony spurs, unification of hemicords, and filum terminale sectioning.
RESULTS: The initial procedure was aborted due to an intraoperative pneumothorax requiring bilateral intercostal drains. Definitive surgery was performed 5 days later, with complete excision of spurs at C4 and C7, detethering of the cord, and successful reconstruction. The postoperative course was uneventful, with no new neurological deficits. At 6-month follow-up, the patient remained neurologically intact and clinically stable.
CONCLUSION: Dual cervical SCM is an exceedingly rare entity not previously reported in the literature. Awareness of its embryogenetic basis, careful anesthetic and surgical planning, and timely prophylactic intervention are crucial to prevent neurological deterioration. This case expands the spectrum of SCM presentations and reinforces the importance of early diagnosis and management in pediatric neurosurgery.
PMID:41353524 | DOI:10.1007/s00381-025-07075-x
