Management of Scoliosis in Patients With Loeys-Dietz Syndrome

Bressner, Jarred A.; MacCarrick, Gretchen; Dietz, Harry C.; Sponseller, Paul D.

doi:10.1097/bpo.0000000000000833

Cited by 15 publications

(28 citation statements)

References 28 publications

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“…Of these, 16 were excluded because they did not discuss the management of orthopaedic conditions. Finally, 13 articles were eligible for inclusion in this study, including four retrospective cohort studies 7 , 10 , 11 , 13 and nine case reports. 8 , 14 15 16 17 18 19 20 21 …”

Section: Resultsmentioning

confidence: 99%

“…These manifestations include idiopathic talipes equinovarus, scoliosis, spondylolisthesis, instability of the cervical spine, joint hypermobility, pectus deformity, osteoporosis, osteoarthritis, and deformity of the hip. 2 , 7 8 9 10 11 The reported orthopaedic conditions in LDS can vary considerably from patient to patient and can often be quite severe. Because early detection and improved management of the vascular aspects of the disease help patients with LDS live longer, fuller lives, 12 understanding these musculoskeletal manifestations will become increasingly important to optimize physical function and quality of life.…”

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confidence: 99%

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Orthopaedic Management of Loeys-Dietz Syndrome: A Systematic Review

Lynch¹,

Patel²,

Seeley³

et al. 2021

JAAOS Glob Res Rev

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Introduction: Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder associated with aortic aneurysm/dissection in children. However, LDS may also present with a host of orthopaedic conditions. This study aimed to elucidate the management of orthopaedic conditions and associated outcomes in patients with LDS. Methods: PubMed, Ovid MEDLINE, and Cochrane Library were systematically searched for primary articles regarding the management of orthopaedic conditions in patients with LDS. The goals and findings of each included study were described. Data regarding demographics, conditions studied, treatment modalities, and outcomes were extracted and analyzed. Results: Three hundred sixty-two unique articles were retrieved, 13 of which were included, with 4 retrospective cohort studies and 9 case reports representing 435 patients. In total, 19.8% of patients presenting with orthopaedic conditions received surgical treatment;54.3% of them experienced adverse outcomes, and 44.4% required revision surgery. The mean age at surgery was 9.0 ± 2.1 years. Conclusion: Patients with LDS may require early surgical intervention for a variety of orthopaedic conditions and may be at an increased risk for surgical complications. The current LDS literature is primarily focused on spinal conditions with a relative paucity of data on the management of hip deformity, joint subluxation, clubfoot, and trauma. Additional research is required regarding orthopaedic management for this unique population.

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Section: Resultsmentioning

confidence: 99%

mentioning

confidence: 99%

Orthopaedic Management of Loeys-Dietz Syndrome: A Systematic Review

Lynch¹,

Patel²,

Seeley³

et al. 2021

JAAOS Glob Res Rev

View full text Add to dashboard Cite

show abstract

“…Another aspect of Loeys–Dietz syndrome involves vertebral anomalies with scoliosis being reported in as many as 62% of cases . Midline defects of the upper cervical spine involving cervical vertebrae 1–3 have been associated with subluxations and instability, in some instances associated with apnea and paresthesia, necessitating surgery .…”

Section: Discussionmentioning

confidence: 99%

Manifestations and Medicolegal Significance of Loeys–Dietz Syndrome

Byard

2017

Journal of Forensic Sciences

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Loeys-Dietz syndrome is a recently described autosomal dominant disorder with underlying vasculopathy characterized by aortic and other vascular aneurysmal dissection/rupture. A 61-year-old man is reported who died suddenly and unexpectedly and at autopsy was found to have a ruptured abdominal aortic aneurysm. Additional findings included dolichostenomelia, high-arched palate, and pectus excavatum. There was a strong family history of Loeys-Dietz syndrome, although the decedent had never been tested. Death was, therefore, due to a ruptured abdominal aortic aneurysm in a case of probable Loeys-Dietz syndrome. Although Loeys-Dietz syndrome shares common characteristics with Marfan and other connective tissue syndromes, it is a distinct entity with a much more aggressive clinical course. Lethal events may not occur until later adult life; however, the identification of the syndrome and differentiation from other connective tissue disorders at autopsy is important so that genetic investigation of close relatives can be undertaken with prophylactic surgical treatment if necessary.

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“…The most recent published study, conducted by Bressner et al 6 discussing scoliosis in patients with LDS and comparing different treatment options, showed that bracing failed in 11 of 15 patients. Contrarily, the same group noted high rates of complications in patients with LDS who underwent growing rod surgery or spinal fusion.…”

Section: Discussionmentioning

confidence: 99%

“…However, no revisions due to failure of fixation of screws or hooks were needed. 6 Controversies exist concerning the surgical approach in syndromic scoliosis.…”

Section: Discussionmentioning

confidence: 99%

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

2017

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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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Management of Scoliosis in Patients With Loeys-Dietz Syndrome

Abstract: Level IV-retrospective cohort study.

Cited by 15 publications

References 28 publications

Orthopaedic Management of Loeys-Dietz Syndrome: A Systematic Review

Orthopaedic Management of Loeys-Dietz Syndrome: A Systematic Review

Manifestations and Medicolegal Significance of Loeys–Dietz Syndrome

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

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