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Segmental Spinal Dysgenesis–“Redefined”
STUDY DESIGN: Retrospective single institutional observational study. PURPOSE: Segmental spinal dysgenesis (SSD), a complex spinal dysraphic state caused by notochord malformation disorders, is named after its morphological presentation where a spine segment is dysgenetic, malformed or absent. This...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Korean Society of Spine Surgery
2019
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6454287/ https://www.ncbi.nlm.nih.gov/pubmed/30472824 http://dx.doi.org/10.31616/asj.2018.0076 |
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author | Chellathurai, Amarnath Ayyamperumal, Balaji Thirumaran, Rajakumari Kathirvelu, Gopinathan Muthaiyan, Priya Kannappan, Sivakumar |
author_facet | Chellathurai, Amarnath Ayyamperumal, Balaji Thirumaran, Rajakumari Kathirvelu, Gopinathan Muthaiyan, Priya Kannappan, Sivakumar |
author_sort | Chellathurai, Amarnath |
collection | PubMed |
description | STUDY DESIGN: Retrospective single institutional observational study. PURPOSE: Segmental spinal dysgenesis (SSD), a complex spinal dysraphic state caused by notochord malformation disorders, is named after its morphological presentation where a spine segment is dysgenetic, malformed or absent. This study’s objective was to examine and reassess SSD imaging findings and correlate them with an embryological explanation. OVERVIEW OF LITERATURE: Scott and his colleagues defined SSD as segmental agenesis or dysgenesis of the lumbar or thoracolumbar vertebrae and underlying spinal cord. Tortori-Donati and his colleagues defined it as a morphologic continuum ranging from hypoplasia to an absent spinal cord segment. METHODS: Fifteen children, whose imaging findings and clinical features were consistent with SSD, were included in the study. Magnetic resonance imaging (MRI) was performed per institutional spine protocol. RESULTS: Five children (33.3%) presented with a high-ending bulbous cord with no caudal segment, six (40%) presented with a dorsal or lumbar segmental dysgenetic cord with a low-lying, bulky caudal cord but without significant spinal canal narrowing, and four (26.6%) presented with segmental caudal dysgenesis with severe kyphoscoliosis, gibbus deformity, and spinal canal narrowing with a normal distal segment (normal or low-lying). CONCLUSIONS: SSD is a complex spinal anomaly in children requiring clinical-radiological assessment followed by multidisciplinary management based on the extent and severity of the dysgenetic cord and the type of SSD. MRI plays a crucial role in both diagnosing and classifying SSD prior to surgical treatment to prevent further impairment. |
format | Online Article Text |
id | pubmed-6454287 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Korean Society of Spine Surgery |
record_format | MEDLINE/PubMed |
spelling | pubmed-64542872019-04-19 Segmental Spinal Dysgenesis–“Redefined” Chellathurai, Amarnath Ayyamperumal, Balaji Thirumaran, Rajakumari Kathirvelu, Gopinathan Muthaiyan, Priya Kannappan, Sivakumar Asian Spine J Clinical Study STUDY DESIGN: Retrospective single institutional observational study. PURPOSE: Segmental spinal dysgenesis (SSD), a complex spinal dysraphic state caused by notochord malformation disorders, is named after its morphological presentation where a spine segment is dysgenetic, malformed or absent. This study’s objective was to examine and reassess SSD imaging findings and correlate them with an embryological explanation. OVERVIEW OF LITERATURE: Scott and his colleagues defined SSD as segmental agenesis or dysgenesis of the lumbar or thoracolumbar vertebrae and underlying spinal cord. Tortori-Donati and his colleagues defined it as a morphologic continuum ranging from hypoplasia to an absent spinal cord segment. METHODS: Fifteen children, whose imaging findings and clinical features were consistent with SSD, were included in the study. Magnetic resonance imaging (MRI) was performed per institutional spine protocol. RESULTS: Five children (33.3%) presented with a high-ending bulbous cord with no caudal segment, six (40%) presented with a dorsal or lumbar segmental dysgenetic cord with a low-lying, bulky caudal cord but without significant spinal canal narrowing, and four (26.6%) presented with segmental caudal dysgenesis with severe kyphoscoliosis, gibbus deformity, and spinal canal narrowing with a normal distal segment (normal or low-lying). CONCLUSIONS: SSD is a complex spinal anomaly in children requiring clinical-radiological assessment followed by multidisciplinary management based on the extent and severity of the dysgenetic cord and the type of SSD. MRI plays a crucial role in both diagnosing and classifying SSD prior to surgical treatment to prevent further impairment. Korean Society of Spine Surgery 2019-04 2018-11-27 /pmc/articles/PMC6454287/ /pubmed/30472824 http://dx.doi.org/10.31616/asj.2018.0076 Text en Copyright © 2019 by Korean Society of Spine Surgery This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Clinical Study Chellathurai, Amarnath Ayyamperumal, Balaji Thirumaran, Rajakumari Kathirvelu, Gopinathan Muthaiyan, Priya Kannappan, Sivakumar Segmental Spinal Dysgenesis–“Redefined” |
title | Segmental Spinal Dysgenesis–“Redefined” |
title_full | Segmental Spinal Dysgenesis–“Redefined” |
title_fullStr | Segmental Spinal Dysgenesis–“Redefined” |
title_full_unstemmed | Segmental Spinal Dysgenesis–“Redefined” |
title_short | Segmental Spinal Dysgenesis–“Redefined” |
title_sort | segmental spinal dysgenesis–“redefined” |
topic | Clinical Study |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6454287/ https://www.ncbi.nlm.nih.gov/pubmed/30472824 http://dx.doi.org/10.31616/asj.2018.0076 |
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