{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:37:31Z","timestamp":1787027851428,"version":"build-2736575974"},"reference-count":20,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2022,7,29]],"date-time":"2022-07-29T00:00:00Z","timestamp":1659052800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Background and Importance: Split notochord syndrome (SNS) is an exceedingly rare type of spinal dysraphism. SNS is sometimes associated with other congenital dysraphic defects but, as in our case, the association with spinal cord lipoma, tethered cord, and spinal deformity in the form of spinal column duplication would be exceedingly rare. Herein, the authors report a three-year-old child presented with SNS associated with complex spinal deformity and other associated congenital anomalies. The patient underwent microsurgical release of the tethering element with excellent short- and long-term outcomes. Clinical Presentation: A male newborn with healthy nonconsanguineous parents was born with multiple gastrointestinal and genitourinary anomalies, and duplicated vertebral columns at the lumbosacral area consistent with split notochord syndrome. The patient was initially managed for the gastrointestinal and genitourinary anomalies. As there was no obvious neurological deficit initially, the neurosurgical intervention was postponed till the child reached 30 months of age, when he underwent uneventful release of both spinal cords at their spit point. Conclusions: SNS is an exceedingly rare developmental anomaly that is usually associated with varying degrees of complex congenital dysraphic defects. Early clinical diagnosis, understanding of the pathophysiology of spinal cord tethering, and microsurgical cord untethering are the important steps in optimal management.<\/jats:p>","DOI":"10.3390\/children9081138","type":"journal-article","created":{"date-parts":[[2022,7,31]],"date-time":"2022-07-31T21:49:02Z","timestamp":1659304142000},"page":"1138","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Split Notochord Syndrome with Spinal Column Duplication and Spinal Cord Lipoma: A Case Report"],"prefix":"10.3390","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7989-8153","authenticated-orcid":false,"given":"Fayez","family":"Alelyani","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, King Khalid University Medical City, King Khalid University, Abha 61413, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keith","family":"Aronyk","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, University of Alberta, Edmonton, AB T6G 2B7, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3961-7820","authenticated-orcid":false,"given":"Hashim","family":"Alghamdi","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Abha Maternity and Children Hospital, Abha 62562, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6433-1990","authenticated-orcid":false,"given":"Ibrahim","family":"Alnaami","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, Department of Surgery, College of Medicine, King Khalid University, Abha 62523, Saudi Arabia"},{"name":"Department of Pediatric Neurosurgery, Abha Maternity and Children Hospital, Abha 62562, Saudi Arabia"},{"name":"Department of Neurosurgery, Aseer Central Hospital, Abha 62523, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,7,29]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1136\/adc.35.179.76","article-title":"Developmental posterior enteric remnants and spinal malformations: The split notochord syndrome","volume":"35","author":"Bentley","year":"1960","journal-title":"Arch. Dis. Child."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1227\/00006123-199209000-00011","article-title":"Split Cord Malformation: Part II: Clinical Syndrome: Neurosurgery","volume":"31","author":"Pang","year":"1992","journal-title":"Neurosurgery"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"626","DOI":"10.1111\/j.1440-1789.2011.01212.x","article-title":"A case of split notochord syndrome: An adult with a spinal endodermal cyst mimicking an intramedullary tumor","volume":"31","author":"Wakao","year":"2011","journal-title":"Neuropathology"},{"key":"ref_4","first-page":"1943","article-title":"Split Spinal Cord","volume":"Volume 2","author":"Raskin","year":"2022","journal-title":"Youmans and Winn Neurological Surgery"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"544","DOI":"10.1080\/10790268.2018.1547531","article-title":"Spine duplication or split notochord syndrome\u2014Case report and literature review","volume":"43","author":"Jasiewicz","year":"2018","journal-title":"J. Spinal Cord Med."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1016\/S1042-3680(18)30467-4","article-title":"Split Cord Malformations","volume":"6","author":"Dias","year":"1995","journal-title":"Neurosurg. Clin. N. Am."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1227\/00006123-199209000-00010","article-title":"Split Cord Malformation: Part I: A Unified Theory of Embryogenesis for Double Spinal Cord Malformations","volume":"31","author":"Pang","year":"1992","journal-title":"Neurosurgery"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1016\/0092-8674(95)90397-6","article-title":"Floor plate and motor neuron induction by different concentrations of the amino-terminal cleavage product of sonic hedgehog autoproteolysis","volume":"81","author":"Roelink","year":"1995","journal-title":"Cell"},{"key":"ref_9","unstructured":"Sarnat, H., and Flores-Sarnat, L. Integrative Classification of Morphology and Molecular Genetics in Central Nervous System Malformations, Academia.edu. Available online: https:\/\/www.academia.edu\/es\/20668358\/Integrative_classification_of_morphology_and_molecular_genetics_in_central_nervous_system_malformations."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1590\/0100-3984.2015.0251","article-title":"Split notochord syndrome: A case in point","volume":"51","author":"Sousa","year":"2018","journal-title":"Radiol. Bras."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"101","DOI":"10.4103\/2249-4847.116412","article-title":"Caudal duplication syndrome","volume":"2","author":"Ramzan","year":"2013","journal-title":"J. Clin. Neonatol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"23","DOI":"10.21699\/jns.v6i1.487","article-title":"A Rare Case of Split Notochord Syndrome","volume":"6","author":"Singh","year":"2017","journal-title":"J. Neonatal Surg."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1159\/000120535","article-title":"Diastematomyelia in 172 Children: The Impact of Modern Neuroradiology","volume":"16","author":"McHugh","year":"1990","journal-title":"Pediatric Neurosurg."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1007\/s00381-006-0205-5","article-title":"Diastematomyelia in children: Treatment outcome and natural history of associated syringomyelia","volume":"23","author":"Gan","year":"2006","journal-title":"Child\u2019s Nerv. Syst."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"204","DOI":"10.4103\/1817-1745.123665","article-title":"Tethered cord with tandem lipomyelomeningoceles, split cord malformation and thick filum","volume":"8","author":"Hanif","year":"2013","journal-title":"J. Pediatric Neurosci."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"e601","DOI":"10.1016\/j.wneu.2020.01.092","article-title":"Split Cord Malformation: Presentation, Management, and Surgical Outcome","volume":"136","author":"Alnefaie","year":"2020","journal-title":"World Neurosurg."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3171\/foc.2001.10.1.7","article-title":"Ventral tethering in split cord malformation","volume":"10","author":"Pang","year":"2001","journal-title":"Neurosurg. Focus"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"S41","DOI":"10.4103\/1817-1745.85708","article-title":"Split cord malformation\u2014A study of 300 cases at AIIMS 1990\u20132006","volume":"6","author":"Mahapatra","year":"2011","journal-title":"J. Pediatric Neurosci."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"151","DOI":"10.3171\/2020.7.PEDS20441","article-title":"Multistage surgical repair for split notochord syndrome with neurenteric fistula: Case report","volume":"27","author":"Mathkour","year":"2020","journal-title":"J. Neurosurg. Pediatrics"},{"key":"ref_20","first-page":"622","article-title":"The split notochord syndrome with dorsal enteric fistula","volume":"14","author":"Hoffman","year":"1993","journal-title":"Am. J. Neuroradiol."}],"container-title":["Children"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2227-9067\/9\/8\/1138\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T23:58:59Z","timestamp":1760140739000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2227-9067\/9\/8\/1138"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,7,29]]},"references-count":20,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2022,8]]}},"alternative-id":["children9081138"],"URL":"https:\/\/doi.org\/10.3390\/children9081138","relation":{},"ISSN":["2227-9067"],"issn-type":[{"value":"2227-9067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,7,29]]}}}