The Treatment of Cleidocranial Dysostosis (Scheuthauer-Marie-Sainton Syndrome), a Rare Form of Skeletal Dysplasia, Accompanied by Spinal Deformities: A Review of the Literature and Two Case Reports

Cleidocranial dysostosis is a skeletal dysplasia inherited in an autosomal dominant manner and may lead to complications such as scoliosis and kyphosis, concurrent with various orthopedic involvements. Since concurrent spinal deformities are of progressive nature, surgical treatment may be necessary...

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Main Authors: Mehmet Bülent Balioğlu, Deniz Kargın, Akif Albayrak, Yunus Atıcı
Format: Article
Language:English
Published: Wiley 2018-01-01
Series:Case Reports in Orthopedics
Online Access:http://dx.doi.org/10.1155/2018/4635761
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author Mehmet Bülent Balioğlu
Deniz Kargın
Akif Albayrak
Yunus Atıcı
author_facet Mehmet Bülent Balioğlu
Deniz Kargın
Akif Albayrak
Yunus Atıcı
author_sort Mehmet Bülent Balioğlu
collection DOAJ
description Cleidocranial dysostosis is a skeletal dysplasia inherited in an autosomal dominant manner and may lead to complications such as scoliosis and kyphosis, concurrent with various orthopedic involvements. Since concurrent spinal deformities are of progressive nature, surgical treatment may be necessary. In addition to other orthopedic problems, possible accompanying complications such as atlanto-axial subluxation, myelopathy, syringomyelia, congenital spine deformities, spondylosis, and spondylolisthesis should be kept in mind while planning for the treatment of scoliosis and kyphosis. Lengthening the use of growth-friendly systems (growing rod) in patients, like ours, with an early onset of symptoms, and performing posterior instrumentation and fusion once the spinal growth is complete will yield successful results with no complications in the middle and the long term. Further multicenter studies with more comprehensive assessments are required to find solutions to spinal problems related to this rare skeletal dysplasia.
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id doaj-art-120df2d3d3454e8b84c13e3aa34e86cc
institution Kabale University
issn 2090-6749
2090-6757
language English
publishDate 2018-01-01
publisher Wiley
record_format Article
series Case Reports in Orthopedics
spelling doaj-art-120df2d3d3454e8b84c13e3aa34e86cc2025-02-03T05:53:59ZengWileyCase Reports in Orthopedics2090-67492090-67572018-01-01201810.1155/2018/46357614635761The Treatment of Cleidocranial Dysostosis (Scheuthauer-Marie-Sainton Syndrome), a Rare Form of Skeletal Dysplasia, Accompanied by Spinal Deformities: A Review of the Literature and Two Case ReportsMehmet Bülent Balioğlu0Deniz Kargın1Akif Albayrak2Yunus Atıcı3Department of Orthopaedics, Istinye University Liv Hospital, Istanbul, TurkeyDepartment of Orthopedics, Health Science University Baltalimani Bone Diseases Education and Research Hospital, Istanbul, TurkeyDepartment of Orthopedics, Health Science University Baltalimani Bone Diseases Education and Research Hospital, Istanbul, TurkeyDepartment of Orthopaedics, Okan University Hospital, Istanbul, TurkeyCleidocranial dysostosis is a skeletal dysplasia inherited in an autosomal dominant manner and may lead to complications such as scoliosis and kyphosis, concurrent with various orthopedic involvements. Since concurrent spinal deformities are of progressive nature, surgical treatment may be necessary. In addition to other orthopedic problems, possible accompanying complications such as atlanto-axial subluxation, myelopathy, syringomyelia, congenital spine deformities, spondylosis, and spondylolisthesis should be kept in mind while planning for the treatment of scoliosis and kyphosis. Lengthening the use of growth-friendly systems (growing rod) in patients, like ours, with an early onset of symptoms, and performing posterior instrumentation and fusion once the spinal growth is complete will yield successful results with no complications in the middle and the long term. Further multicenter studies with more comprehensive assessments are required to find solutions to spinal problems related to this rare skeletal dysplasia.http://dx.doi.org/10.1155/2018/4635761
spellingShingle Mehmet Bülent Balioğlu
Deniz Kargın
Akif Albayrak
Yunus Atıcı
The Treatment of Cleidocranial Dysostosis (Scheuthauer-Marie-Sainton Syndrome), a Rare Form of Skeletal Dysplasia, Accompanied by Spinal Deformities: A Review of the Literature and Two Case Reports
Case Reports in Orthopedics
title The Treatment of Cleidocranial Dysostosis (Scheuthauer-Marie-Sainton Syndrome), a Rare Form of Skeletal Dysplasia, Accompanied by Spinal Deformities: A Review of the Literature and Two Case Reports
title_full The Treatment of Cleidocranial Dysostosis (Scheuthauer-Marie-Sainton Syndrome), a Rare Form of Skeletal Dysplasia, Accompanied by Spinal Deformities: A Review of the Literature and Two Case Reports
title_fullStr The Treatment of Cleidocranial Dysostosis (Scheuthauer-Marie-Sainton Syndrome), a Rare Form of Skeletal Dysplasia, Accompanied by Spinal Deformities: A Review of the Literature and Two Case Reports
title_full_unstemmed The Treatment of Cleidocranial Dysostosis (Scheuthauer-Marie-Sainton Syndrome), a Rare Form of Skeletal Dysplasia, Accompanied by Spinal Deformities: A Review of the Literature and Two Case Reports
title_short The Treatment of Cleidocranial Dysostosis (Scheuthauer-Marie-Sainton Syndrome), a Rare Form of Skeletal Dysplasia, Accompanied by Spinal Deformities: A Review of the Literature and Two Case Reports
title_sort treatment of cleidocranial dysostosis scheuthauer marie sainton syndrome a rare form of skeletal dysplasia accompanied by spinal deformities a review of the literature and two case reports
url http://dx.doi.org/10.1155/2018/4635761
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