The growing spine in marfan and loeys–dietz syndromes

Paul D. Sponseller; Jarred Bressner; Ehsan Jazini

doi:10.1007/978-3-662-48284-1_17

The growing spine in marfan and loeys–dietz syndromes

Paul D. Sponseller, Jarred Bressner, Ehsan Jazini

School of Medicine

Research output: Chapter in Book/Report/Conference proceeding › Chapter

2 Scopus citations

Abstract

Marfan and Loeys-Dietz Syndromes both involve connective tissues and TGF-beta signaling. Both may lead to spinal deformity which becomes severe at an early age. Bracing may only be of value if started before 25°. Growth-friendly strategies may be necessary. VEPTR may lead to kyphosis and forward trunk balance in these patients. Magnetically-controlled rods may not be feasible if the patients require periodic MRI for the cardiovascular system. Growing rods produce good deformity control in both conditions. The rate of rod fracture is higher in Loeys-Dietz syndrome, perhaps because of the greater tissue laxity. The surgeon should be aware of cervical deformity in this population, either at presentation or later in the course.

Original language	English (US)
Title of host publication	The Growing Spine
Subtitle of host publication	Management of Spinal Disorders in Young Children, Second Edition
Publisher	Springer Berlin Heidelberg
Pages	293-306
Number of pages	14
ISBN (Electronic)	9783662482841
ISBN (Print)	9783662482834
DOIs	https://doi.org/10.1007/978-3-662-48284-1_17
State	Published - Jan 1 2015

Keywords

Cervical instability
Fibrillin
Kyphosis
Scoliosis

ASJC Scopus subject areas

Medicine(all)

Access to Document

10.1007/978-3-662-48284-1_17

Cite this

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title = "The growing spine in marfan and loeys–dietz syndromes",

abstract = "Marfan and Loeys-Dietz Syndromes both involve connective tissues and TGF-beta signaling. Both may lead to spinal deformity which becomes severe at an early age. Bracing may only be of value if started before 25°. Growth-friendly strategies may be necessary. VEPTR may lead to kyphosis and forward trunk balance in these patients. Magnetically-controlled rods may not be feasible if the patients require periodic MRI for the cardiovascular system. Growing rods produce good deformity control in both conditions. The rate of rod fracture is higher in Loeys-Dietz syndrome, perhaps because of the greater tissue laxity. The surgeon should be aware of cervical deformity in this population, either at presentation or later in the course.",

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AU - Bressner, Jarred

AU - Jazini, Ehsan

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N2 - Marfan and Loeys-Dietz Syndromes both involve connective tissues and TGF-beta signaling. Both may lead to spinal deformity which becomes severe at an early age. Bracing may only be of value if started before 25°. Growth-friendly strategies may be necessary. VEPTR may lead to kyphosis and forward trunk balance in these patients. Magnetically-controlled rods may not be feasible if the patients require periodic MRI for the cardiovascular system. Growing rods produce good deformity control in both conditions. The rate of rod fracture is higher in Loeys-Dietz syndrome, perhaps because of the greater tissue laxity. The surgeon should be aware of cervical deformity in this population, either at presentation or later in the course.

AB - Marfan and Loeys-Dietz Syndromes both involve connective tissues and TGF-beta signaling. Both may lead to spinal deformity which becomes severe at an early age. Bracing may only be of value if started before 25°. Growth-friendly strategies may be necessary. VEPTR may lead to kyphosis and forward trunk balance in these patients. Magnetically-controlled rods may not be feasible if the patients require periodic MRI for the cardiovascular system. Growing rods produce good deformity control in both conditions. The rate of rod fracture is higher in Loeys-Dietz syndrome, perhaps because of the greater tissue laxity. The surgeon should be aware of cervical deformity in this population, either at presentation or later in the course.

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The growing spine in marfan and loeys–dietz syndromes

Abstract

Keywords

ASJC Scopus subject areas

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