Combining Correction of Pectus Excavatum and Open Heart Surgery in a Single-Stage Procedure

Media, Ara Shwan; Pilegaard, Hans K.; Paoli, Frank Vincenzo de

doi:10.1016/j.athoracsur.2019.07.043

Cited by 7 publications

(4 citation statements)

References 6 publications

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“…After the NUSS-procedure, the metal bar has to remain in this position for approximately three years which hinders quick access to the aorta in case of an aortic dissection [ 12 ]. It is therefore important to ensure the correct timing of corrective chest surgery in patients with MFS or to combine open heart and chest surgery in one procedure [ 29 ]. We did not find any cases with a need for aortic surgery after a NUSS-procedure, which indicates that these patients were young and well selected.…”

Section: Discussionmentioning

confidence: 99%

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%

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

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“…The proportion of symmetrical deformity in them was as high as 90.3%, and deformity often occurred within 1 year of cardiac surgery (71%), which is significantly different from the primary. Some studies showed the feasibility of concurrent surgery for congenital heart disease and PE [ 17 , 18 ]. However, we prefer to correct the cardiac disease first, especially in those under 3 years old, unless the surgery can be completed by minimally invasive incision or intervention.…”

Section: Discussionmentioning

confidence: 99%

Modified Nuss procedure with a novel steel bar in patients with pectus excavatum post-congenital heart surgery

Liu

Wang

Zhang

et al. 2021

Interactive CardioVascular and Thoracic Surgery

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OBJECTIVES Pectus excavatum (PE) can be secondary in patients who underwent sternotomy for cardiac surgery. Retrosternal adhesions increase the complexity and risk of traditional Nuss repair. Thus, we summarized the outcomes of our modified Nuss procedure using a newly designed bar. METHODS A retrospective analysis was performed on 35 patients who underwent modified PE repair after open heart surgery from January 2011 to July 2019. The surgery was performed using a novel bar with no need for intraoperative reshaping and rotation, assisted by thoracoscopy and subxiphoid incision when necessary. RESULTS There were 19 males and 16 females with a median age of 5.3 years (interquartile range, 4.1–10.9) at PE repair. All patients underwent the modified procedure uneventfully with no death. The median operating time was 70 min. Twenty-nine (82.9%) patients required subxiphoid incision assistance. There was 1 case (2.8%) with unexpected sternotomy due to intraoperative bleeding. The median length of postoperative hospital stay was 4 days. During the median 3.5 years of follow-up, no bar dislocation was found and 30 (85.7%) patients had their bars removed with no recurrence recorded. After PE repair, the Haller index improved significantly (2.6 ± 0.4 vs 4.9 ± 1.3, P < 0.05) and further decreased till the time of bar removal (2.5 ± 0.4 vs 2.6 ± 0.4, P < 0.05). All patients were satisfied with the cosmetic outcome. CONCLUSIONS The novel bar can be placed and removed easily with a low rate of adverse events. This modified Nuss procedure seems to be a safe, effective and convenient approach for the management of PE after cardiac surgery.

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“…After the NUSS-procedure, the metal bar has to remain in this position for approximately three years which hinders quick access to the aorta in case of an aortic dissection [11]. It is therefore important to ensure the correct timing of corrective chest surgery in patients with MFS or to combine open heart and chest surgery in one procedure [29]. We did not nd any cases with a need for aortic surgery after a NUSSprocedure, which indicates that these patients were young and well selected.…”

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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Combining Correction of Pectus Excavatum and Open Heart Surgery in a Single-Stage Procedure

Cited by 7 publications

References 6 publications

Musculoskeletal diseases in Marfan syndrome: a nationwide registry study

Musculoskeletal diseases in Marfan syndrome: a nationwide registry study

Modified Nuss procedure with a novel steel bar in patients with pectus excavatum post-congenital heart surgery

Musculoskeletal Diseases in Marfan Syndrome – A Nationwide Registry Study.

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