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Split Notochord Syndrome with Spinal Column Duplication and Spinal Cord Lipoma: A Case Report

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 colu...

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Autores principales: Alelyani, Fayez, Aronyk, Keith, Alghamdi, Hashim, Alnaami, Ibrahim
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9406422/
https://www.ncbi.nlm.nih.gov/pubmed/36010029
http://dx.doi.org/10.3390/children9081138
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author Alelyani, Fayez
Aronyk, Keith
Alghamdi, Hashim
Alnaami, Ibrahim
author_facet Alelyani, Fayez
Aronyk, Keith
Alghamdi, Hashim
Alnaami, Ibrahim
author_sort Alelyani, Fayez
collection PubMed
description 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.
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spelling pubmed-94064222022-08-26 Split Notochord Syndrome with Spinal Column Duplication and Spinal Cord Lipoma: A Case Report Alelyani, Fayez Aronyk, Keith Alghamdi, Hashim Alnaami, Ibrahim Children (Basel) Case Report 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. MDPI 2022-07-29 /pmc/articles/PMC9406422/ /pubmed/36010029 http://dx.doi.org/10.3390/children9081138 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Alelyani, Fayez
Aronyk, Keith
Alghamdi, Hashim
Alnaami, Ibrahim
Split Notochord Syndrome with Spinal Column Duplication and Spinal Cord Lipoma: A Case Report
title Split Notochord Syndrome with Spinal Column Duplication and Spinal Cord Lipoma: A Case Report
title_full Split Notochord Syndrome with Spinal Column Duplication and Spinal Cord Lipoma: A Case Report
title_fullStr Split Notochord Syndrome with Spinal Column Duplication and Spinal Cord Lipoma: A Case Report
title_full_unstemmed Split Notochord Syndrome with Spinal Column Duplication and Spinal Cord Lipoma: A Case Report
title_short Split Notochord Syndrome with Spinal Column Duplication and Spinal Cord Lipoma: A Case Report
title_sort split notochord syndrome with spinal column duplication and spinal cord lipoma: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9406422/
https://www.ncbi.nlm.nih.gov/pubmed/36010029
http://dx.doi.org/10.3390/children9081138
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