Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Chellathurai, Amarnath, Ayyamperumal, Balaji, Thirumaran, Rajakumari, Kathirvelu, Gopinathan, Muthaiyan, Priya, Kannappan, Sivakumar
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Korean Society of Spine Surgery 2019
Materias:
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
_version_ 1783409547209080832
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
work_keys_str_mv AT chellathuraiamarnath segmentalspinaldysgenesisredefined
AT ayyamperumalbalaji segmentalspinaldysgenesisredefined
AT thirumaranrajakumari segmentalspinaldysgenesisredefined
AT kathirvelugopinathan segmentalspinaldysgenesisredefined
AT muthaiyanpriya segmentalspinaldysgenesisredefined
AT kannappansivakumar segmentalspinaldysgenesisredefined