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Anterior Surgical Treatment of Scoliosis in a Patient With Loeys-Dietz Syndrome


Farshad, Mazda; Winkler, Elin; Betz, Michael (2017). Anterior Surgical Treatment of Scoliosis in a Patient With Loeys-Dietz Syndrome. Journal of American Academy of Orthopaedic Surgeons, 1(7):e039.

Abstract

Loeys-Dietz syndrome (LDS) is an autosomal dominant disorder affecting the connective tissue, resulting in laxity, and can be challenging if surgical treatment is needed. Literature concerning scoliosis and its treatment in LDS is limited. This is a report of scoliosis surgery in a 12-year-old girl with LDS. She underwent anterior instrumented spinal fusion of the segments T12 to L3 because of a left-sided thoracolumbar scoliosis of 42° with dysplastic pedicles. The scoliosis was reduced by 40%, from 42° to 25°, resulting in a satisfactory clinical outcome with a minimal amount of surgical invasion and lack of complications. A minimal approach and anterior instrumented spinal fusion surgery can be considered a valuable surgical treatment alternative for scoliosis in patients with LDS, avoiding the placement of pedicle screws in dysplastic pedicles and using the abnormal laxity of the connective tissue as an advantage.

Abstract

Loeys-Dietz syndrome (LDS) is an autosomal dominant disorder affecting the connective tissue, resulting in laxity, and can be challenging if surgical treatment is needed. Literature concerning scoliosis and its treatment in LDS is limited. This is a report of scoliosis surgery in a 12-year-old girl with LDS. She underwent anterior instrumented spinal fusion of the segments T12 to L3 because of a left-sided thoracolumbar scoliosis of 42° with dysplastic pedicles. The scoliosis was reduced by 40%, from 42° to 25°, resulting in a satisfactory clinical outcome with a minimal amount of surgical invasion and lack of complications. A minimal approach and anterior instrumented spinal fusion surgery can be considered a valuable surgical treatment alternative for scoliosis in patients with LDS, avoiding the placement of pedicle screws in dysplastic pedicles and using the abnormal laxity of the connective tissue as an advantage.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > Balgrist University Hospital, Swiss Spinal Cord Injury Center
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:October 2017
Deposited On:18 Sep 2018 14:35
Last Modified:24 Sep 2019 23:37
Publisher:American Academy of Orthopaedic Surgeons
ISSN:1067-151X
OA Status:Green
Free access at:PubMed ID. An embargo period may apply.
Publisher DOI:https://doi.org/10.5435/JAAOSGlobal-D-17-00039
Official URL:https://journals.lww.com/jaaosglobal/Fulltext/2017/10000/Anterior_Surgical_Treatment_of_Scoliosis_in_a.4.aspx
PubMed ID:30211363

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