A rare case of Sprengel deformity associated with spondylocostal dysostosis in a four-year child

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dc.contributor.authorMohamed, Syeden_US
dc.contributor.authorKumar, Radhaen_US
dc.date.accessioned2020-09-24T07:43:12Z
dc.date.available2020-09-24T07:43:12Z
dc.date.issued2019-03
dc.description.abstractSprengel deformity is a complex congenital anomaly affecting one or both scapulae with mispositioning and dysplasia of scapula. It occurs due to failure of descent of scapula during intrauterine development and is the commonest congenital skeletal deformity of the scapula, often associated with other skeletal deformities. spondylocostal dysostosis is a rare genetic disorder which is characterized by malformation of the bones of the spine and ribs. It occurs in approximately 1 in 2,00,000 people worldwide. In spondylocostal dysostosis, affected children have wedge shaped vertebrae along with ribs that are fused, forked or sometimes missing. Based on the severity of the defect's children may present with short stature, scoliosis or hypoplasia of lungs causing difficulty in breathing and recurrent respiratory tract infections. Management of both these skeletal deformities depends on their severity and the presence of associated deformities. Surgical intervention might be required for cosmetic and functional recovery of the shoulder. Authors had a rare and interesting case of a 4-year-old boy who had features of Sprengel deformity affecting left scapula which was associated with features of spondylocostal dysostosis.en_US
dc.identifier.affiliationsDepartment of Paediatrics, Saveetha Medical College Hospital, Chennai, Tamil Nadu, Indiaen_US
dc.identifier.citationMohamed Syed, Kumar Radha. A rare case of Sprengel deformity associated with spondylocostal dysostosis in a four-year child. International Journal of Contemporary Pediatrics. 2019 Mar; 6(2): 886-889en_US
dc.identifier.issn2349-3283
dc.identifier.issn2349-3291
dc.identifier.placeIndiaen_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/203918
dc.languageenen_US
dc.publisherMedip Academyen_US
dc.relation.issuenumber2en_US
dc.relation.volume6en_US
dc.source.urihttps://dx.doi.org/10.18203/2349-3291.ijcp20190749en_US
dc.subjectBirth defectsen_US
dc.subjectHigh scapulaen_US
dc.subjectSpondylocostal dysostosis (SCDO)en_US
dc.subjectSprengel deformityen_US
dc.titleA rare case of Sprengel deformity associated with spondylocostal dysostosis in a four-year childen_US
dc.typeJournal Articleen_US
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