Multistage surgical repair for split notochord syndrome with neuroenteric fistula: case report

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  • 1 Department of Neurosurgery, Tulane/Ochsner Medical Center, New Orleans, Louisiana;
  • 2 Department of Neurological Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania;
  • 3 Department of Pediatric Surgery, Ochsner Medical Center, New Orleans; and
  • 4 Departments of Plastic and Reconstructive Surgery, LSUHSC School of Medicine, New Orleans, Louisiana
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Split notochord syndrome (SNS) is a rare congenital defect of the central nervous system and has been associated with several anomalies affecting multiple organ systems. One association has been communication with the gastrointestinal tract and the spine, previously identified as a neuroenteric fistula (NEF). Here, the authors describe the unique case of a female infant with SNS and NEF treated with a multistage surgical repair. The three-stage operative plan included a two-stage repair of the defect and temporary subgaleal shunting followed by delayed ventriculoperitoneal shunt placement. The infant recovered well postsurgery and over a 5-year follow-up. A case description, surgical techniques, and rationale are reported. Additionally, a systematic review of the literature utilizing the MEDLINE database was performed.

Treatment of SNS with NEF using a multidisciplinary multistaged approach to repair the intestinal defect, close the neural elements, and divert cerebrospinal fluid to the peritoneum is shown to be a safe and viable option for future cases.

ABBREVIATIONS CNS = central nervous system; CSF = cerebrospinal fluid; GI = gastrointestinal; MMC = myelomeningocele; NEF = neuroenteric fistula; NICU = neonatal intensive care unit; NTD = neural tube defect; SCM = split cord malformation; SNS = split notochord syndrome; VPS = ventriculoperitoneal shunt.

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Contributor Notes

Correspondence Mansour Mathkour: Ochsner Health System, New Orleans, LA. mathkour.mansour@gmail.com.

INCLUDE WHEN CITING Published online December 4, 2020; DOI: 10.3171/2020.7.PEDS20441.

Disclosures Dr. Maulucci is a consultant for Globus Medical.

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