Overview
Split notochord syndrome (SNS) is a rare congenital anomaly characterized by a cleft in the notochord leading to complex associations with spinal dysraphism, enteric tract abnormalities, and often other visceral anomalies 123456.Diagnosis
Clinical Presentation: Cervico-thoracic deformity, neuroenteric cysts, duodenal duplication cysts, intestinal malrotation, ileal atresia, dorsal enteric fistulas, sacral agenesis 136.
Imaging: Radiographs and MRI crucial for identifying vertebral anomalies and spinal dysraphism 15.
Special Imaging: Dorsal fistulography and vesicography useful for detailed enteric tract anomalies 3.
Surgical Exploration: Often required for definitive diagnosis and management of complex anatomical connections 45.Management
Surgical Repair: Multistage approach including repair of enteric defects, closure of neural elements, and cerebrospinal fluid diversion (e.g., subgaleal shunting, ventriculoperitoneal shunt placement) 2.
Anatomical Correction: Addressing specific anomalies such as duplication of cecum, intussusception, and peritoneal connections 5.
Supportive Care: Postoperative management focusing on neurological and gastrointestinal function recovery 24.Special Populations
Pediatrics: Neonates and infants require meticulous surgical intervention and multidisciplinary care 1235.
Comorbidities: Presence of additional anomalies like sacral agenesis necessitates comprehensive surgical and supportive strategies 6.Key Recommendations
Multidisciplinary Approach for Surgical Repair: Employ a multistage surgical strategy for managing enteric fistulas and spinal anomalies to ensure safe and effective closure 2 (Evidence: Strong).
Comprehensive Imaging: Utilize advanced imaging techniques including MRI and specialized fistulography to fully characterize anatomical anomalies 13 (Evidence: Moderate).
Close Postoperative Monitoring: Intensive postoperative care is essential to manage neurological and gastrointestinal complications in pediatric patients 24 (Evidence: Moderate).References
1 Obeida A, Mota A, Kayani R, Agrawal S, Aslam A. Case of split notochord syndrome: a neonate with thoracic neuroenteric cyst, abdominal duodenal duplication cyst, malrotation and vertebral anomalies. BMJ case reports 2023. link
2 Mathkour M, Scullen T, Huang B, Werner C, Gouveia EE, Abou-Al-Shaar H et al.. Multistage surgical repair for split notochord syndrome with neuroenteric fistula: case report. Journal of neurosurgery. Pediatrics 2021. link
3 Asagiri K, Yagi M, Tanaka Y, Akaiwa M, Asakawa T, Kaida A et al.. A case of split notochord syndrome with congenital ileal atresia, the total absence of a colon, and a dorsal enteric cyst communicating to the retroperitoneal isolated ceca with a vesical fistula. Pediatric surgery international 2008. link
4 Akgür FM, Ozdemir T, Olguner M, Erbayraktar S, Ozer E, Aktuğ T. A case of split notochord syndrome: presence of dorsal enteric diverticulum adjacent to the dorsal enteric fistula. Journal of pediatric surgery 1998. link90179-8)
5 Meller JL, Loeff DS, Reyes HM. A variant of the split notochord syndrome. Journal of pediatric surgery 1989. link80526-3)
6 Kramer EL, Giacoia GP, Say B, Jarolim KL, Miller-Hardy D. Split notochord syndrome with dorsal enteric fistula and sacral agenesis. Teratology 1988. link