A rare case of split notochord syndrome with neurenteric cyst: clinical challenges, surgical approach, and literature review

Split notochord syndrome (SNS) is a rare congenital anomaly characterized by incomplete midline notochordal integration during gastrulation, leading to segmental clefts of the spine. This case report describes a female patient with symptomatic cervico-dorsal SNS associated with a neurenteric cyst (NEC), intrathoracic gut duplication, and secondary hydrocephalus. Multistep surgical interventions were performed, including hydrocephalus management, excision of the cyst and gut duplication, and detethering with sectioning of the filum. Although there were no procedural complications, bladder and bowel dysfunction persisted on follow-up, while normal motor function was preserved. This case illustrates the diverse and complex clinical manifestations of SNS, underscoring the necessity of interdisciplinary collaboration and personalized patient care. While conservative management may be appropriate for asymptomatic cases, surgical intervention is typically recommended for symptomatic patients to address complications and improve outcomes. Given the lack of established guidelines, customized management based on clinical judgment is essential. Further research is needed to enhance understanding of SNS, improve the quality of care, and achieve better patient outcomes.