Growth-friendly Spinal Instrumentation in Marfan Syndrome Achieves Sustained Gains in Thoracic Height Amidst High Rates of Implant Failure

Bellaire, Laura; Zhang, Chong; Smith, John T.; Heflin, John; Klatt, Joshua B.; Roye, David P.; Sponseller, Paul D.; Samdani, Amer F.; Fedorak, Graham

doi:10.1097/bpo.0000000000001730

Cited by 3 publications

(3 citation statements)

References 16 publications

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“…These findings are in accordance with other observational studies [ 13 , 21 ]. Scoliosis bracing for children and adolescents can be effective but growth-friendly spinal surgery may be necessary in many cases which includes staged surgery and growth friendly spine implants [ 22 , 23 ]. Both the increased risk of implant failure in these cases and the need for multiple surgical stages is reflected in our findings where the number of surgical procedures was twice as high as in the background population [ 21 , 22 ].…”

Section: Discussionmentioning

confidence: 99%

“…Scoliosis bracing for children and adolescents can be effective but growth-friendly spinal surgery may be necessary in many cases which includes staged surgery and growth friendly spine implants [ 22 , 23 ]. Both the increased risk of implant failure in these cases and the need for multiple surgical stages is reflected in our findings where the number of surgical procedures was twice as high as in the background population [ 21 , 22 ]. However, we did not see a significantly higher number of surgical complications among patients with MFS, as previously reported [ 23 ].…”

Section: Discussionmentioning

confidence: 99%

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Musculoskeletal diseases in Marfan syndrome: a nationwide registry study

Andersen

Hauge

Baad‐Hansen

et al. 2022

Orphanet J Rare Dis

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Background Marfan syndrome is associated with abnormalities in the musculoskeletal system including scoliosis, pectus deformities, protrusio acetabuli, and foot deformities. Over a life span, many patients with Marfan syndrome will need treatment; however, the musculoskeletal morbidity over a life span is not well described. The aim of the present study was to assess the overall burden of musculoskeletal disease in patients with Marfan syndrome. Materials and methods A registry-based, nationwide epidemiological study of patients with a Ghent II verified Marfan syndrome diagnosis from 1977 to 2014. Each patient was matched on age, and sex with up to 100 controls from the background population. Results We identified 407 patients with Marfan syndrome and 40,700 controls and compared their musculoskeletal diagnoses and surgical treatments using Cox proportional hazards ratio (HR). The risk of a registration of a musculoskeletal diagnosis in patients with Marfan syndrome was significantly increased compared to controls (HR: 1.94 (1.69–2.24). One out of six with Marfan syndrome was registered with scoliosis (HR: 36.7 (27.5–48.9). Scoliosis was more common in women with Marfan syndrome compared to men (HR: 4.30 (1.73–1.08)). One out of 11 were registered with a pectus deformity HR: 40.8 (28.1–59.3), and one out of six with a deformity of the foot. Primarily pes planus (HR: 26.0 (15.2–44.3). The proportion of patients with Marfan syndrome (94/407) that underwent musculoskeletal surgery was also significantly higher (HR: 1.76 (1.43–2.16)). The major areas of surgery were the spine, pectups correction, and surgery of the foot/ankle. Ten patients with Marfan syndrome had elective orthopedic surgery without being recognized and diagnosed with Marfan syndrome until later in life. None of these had scoliosis, pectus deformity or a foot deformity. Among patients with an aortic dissection, the age at dissection was 34.3 years in those with at least one major musculoskeletal abnormality. In patients without a major abnormality the age at dissection was 45.1 years (p < 0.01). Conclusions The extent of musculoskeletal disease is quite significant in Marfan syndrome, and many will need corrective surgery during their life span. Surgeons should be aware of undiagnosed patients with Marfan syndrome when treating patients with a Marfan syndrome like-phenotype.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Musculoskeletal diseases in Marfan syndrome: a nationwide registry study

Andersen

Hauge

Baad‐Hansen

et al. 2022

Orphanet J Rare Dis

View full text Add to dashboard Cite

show abstract

“…Scoliosis bracing for children and adolescents can be effective but growth-friendly spinal surgery may be necessary in many cases which includes staged surgery and growth friendly spine implants [22,23]. Both the increased risk of implant failure in these cases and the need for multiple surgical stages is re ected in our ndings where the number of surgical procedures was twice as high as in the background population [21,22]. However, we did not see a signi cantly higher number of surgical complications among patients with MFS, as previously reported [23].…”

Section: Discussionmentioning

confidence: 99%

Musculoskeletal Diseases in Marfan Syndrome – A Nationwide Registry Study.

Andersen¹,

Hauge²,

Baad‐Hansen³

et al. 2021

Preprint

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BackgroundMarfan syndrome is associated with abnormalities in the musculoskeletal system including scoliosis, pectus deformities, protrusio acetabuli, and foot deformities. Over a life span, many patients with Marfan syndrome will need treatment; however, the musculoskeletal morbidity over a life span is not well described. The aim of the present study was to assess the overall burden of musculoskeletal disease in patients with Marfan syndrome.Materials and MethodsA registry-based, nationwide epidemiological study of patients with a Ghent II verified Marfan syndrome diagnosis from 1977-2014. Each patient was matched on age, and sex with up to 100 controls from the background population.ResultsWe identified 407 patients with Marfan syndrome and 40,700 controls and compared their musculoskeletal diagnoses and surgical treatments using Cox proportional hazards regression (HR). The risk of a registration of a musculoskeletal diagnosis in patients with Marfan syndrome was significantly increased compared to controls (HR: 1.94 (1.69-2.24). One out of six with Marfan syndrome was registered with scoliosis (HR: 36.7 (27.5-48.9). Scoliosis was more common in women with Marfan syndrome compared to men (HR: 4.30 (1.73-1.08)). One out of 11 were registered with a pectus deformity HR: 40.8 (28.1-59.3), and one out of three with a deformity of the foot (HR: 1.9 (1.6-2.3)). The proportion of patients with Marfan syndrome (94/407) that underwent musculoskeletal surgery was also significantly higher (HR: 1.76 (1.43-2.16)). The major areas of surgery were the spine, pectus correction, and surgery of the foot/ancle. Ten patients with Marfan syndrome had elective orthopedic surgery without being recognized and diagnosed with Marfan syndrome until later in life. None of these had scoliosis, pectus deformity or a foot deformity.Among patients with an aortic dissection, the age at dissection was 34.3 years in those with at least one major musculoskeletal abnormality. In patients without a major abnormality the age at dissection was 45.1 years (p<0.01).ConclusionsThe extend of musculoskeletal disease is quite significant in Marfan syndrome and many will need corrective surgery during their life span. Surgeons should be aware of undiagnosed patients with Marfan syndrome when treating patients with a Marfan syndrome like-phenotype.

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