Minimally invasive repair of pectus excavatum in patients with Marfan syndrome and marfanoid features

Redlinger, Richard E.; Rushing, Gregory D.; Moskowitz, Alan D.; Kelly, Robert E.; Nuss, Donald L.; Kuhn, Ann M.; Obermeyer, Robert J.; Goretsky, Michael J.

doi:10.1016/j.jpedsurg.2009.10.037

Cited by 34 publications

(42 citation statements)

References 12 publications

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“…3,10 Many have not had PE repair and present as adults with aortic and valve pathology requiring operative intervention. 5,10 Simultaneous cardiac surgery and repair of the chest wall deformity can be safely performed. Our modification of the MIRPE included direct visualization when passing the introducer and bars across the thoracic cavity through a partially approximated median sternotomy.…”

Section: Discussionmentioning

confidence: 99%

“…Bilateral anterior axillary incisions were made centered between the intercostal spaces identified for bar placement. [8][9][10] The pectoralis major muscle was dissected from the chest wall bilaterally and the thoracic entry and exit sites in the intercostal sites made close to the sternum with a large introducer (Lorenz Surgical, Jacksonville, FL, USA). Because the sternum was still not completely approximated, full visualization and safe guidance of the introducer across the defect could be performed without thoracoscopy.…”

Section: Procedures Detailsmentioning

confidence: 99%

“…4,6,7 Since its introduction by Nuss et al, 8 clinical results have shown the Minimally Invasive Repair PE (MIRPE) to be safe and successful in the Marfan syndrome patient. [8][9][10] We describe simultaneous cardiac surgery and chest wall repair in two Marfan adults using a modified MIRPE. Good surgical exposure, excellent sternal stability and satisfactory cosmetic appearances were obtained.…”

Section: Introductionmentioning

confidence: 99%

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Simultaneous repair of cardiac pathology and severe pectus excavatum in Marfan patients using a modified minimally invasive repair

2011

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Pectus excavatum (PE) deformity is present in the majority of Marfan patients. Many have not had PE repair and present as adults with aortic and valve pathology requiring operative intervention. We present our preliminary report of simultaneous cardiac surgery and repair of the chest wall deformity. Utilizing this modified minimally invasive excavatum repair provided a quick, safe repair with good cosmetic results.

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Section: Discussionmentioning

confidence: 99%

Section: Procedures Detailsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Simultaneous repair of cardiac pathology and severe pectus excavatum in Marfan patients using a modified minimally invasive repair

2011

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“…Gurnett CA et al found that adolescent idiopathic scoliosis and pectus excavatum may have a similar genetic etiology, and the prevalence of scoliosis among pectus excavatum patients is higher than the prevalence within the general population 10) . Furthermore, pectus excavatum is frequently associated with heritable disorders of the connective tissue, such as Marfan syndrome in which almost half the patients have pectus excavatum and a large number have scoliosis [11][12][13][14][15] . However, to our knowledge, although a few studies have reported on the clinical characteristics of patients having both scoliosis and pectus excavatum, they do not give a detailed discussion and the degree of scoliosis is not severe 6,8,9,16,17) .…”

Section: Introductionmentioning

confidence: 99%

Clinical Characteristics and Thoracic factors in patients with Idiopathic and Syndromic Scoliosis Associated with Pectus Excavatum

Tauchi

Suzuki

Tsuji

et al. 2018

Spine Surg Relat Res

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Abstract:Introduction: The purpose of this study is to demonstrate the clinical characteristics and thoracic factors such as sternal tilt angle and Haller index in patients with idiopathic or syndromic scoliosis associated with pectus excavatum.Methods: We performed a retrospective review on a cohort of 70 patients (37 males and 33 females) diagnosed with idiopathic and syndromic scoliosis associated with pectus excavatum between 1985 and 2014. We investigated age, location and Cobb angle of the main curve, and thoracic factors including sternal deviation and tilting angle and Haller index using radiographs and computed tomography of the chest.Results: Patients' mean age at the first visit to our hospital was 10.3 years (1-18 years old). There were 41 patients with idiopathic scoliosis and 29 with syndromic scoliosis. Main curve locations were thoracic in 52 patients, thoracolumbar in 10, and lumbar in 8. The mean Cobb angle of the main curve was 45.0 degrees (11-109 degrees). The sternum was displaced on the left side in 72% of patients, central in 23%, and right in 5%. Mean sternal tilt angle was 12.4 degrees (2.3-34 degrees), and mean Haller index score was 4.9 (2.9-9.2). There was no significant correlation between Cobb angle and sternal tilt angle/Haller index. However, a significant difference was found between sternal tilt angle and Haller index.Conclusion: Most patients with both scoliosis and pectus excavatum have left side deviated sternum and a higher Haller index score; therefore this can negatively impact cardiac function. Prone positioning and the corrective force applied during scoliosis surgery as well as thoracic compression during cast or brace treatment may have a negative effect on cardiac function in these patients.

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“…Pectus excavatum is often observed in children with muscular disorders, such as in children with Marfan syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, or homocystinuria. The deformity is more common in children with congenital heart disease and in children with Down syndrome [2,5,7].…”

Section: Introductionmentioning

confidence: 99%

REHABILITATION Functional testing of muscles in patients with pectus excavatum – indications for preoperative rehabilitation

Jarosz¹,

Barinow‐Wojewódzki²,

Wiernicka³

et al. 2013

kitp

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Introduction: Studies of people with pectus excavatum usually relate to physiological abnormalities caused by the deformity, as well as its impact on posture. Little is known about muscle length disorders associated with this defect. Aim of the study: Determining whether the presence of pectus excavatum is associated with changes in muscle length; developing guidelines for preoperative rehabilitation of pectus excavatum patients. Material and methods: 19 patients aged 13-19 years with diagnosed pectus excavatum were examined and qualified for the Nuss procedure at the Wielkopolska Centre of Pulmonology and Thoracosurgery in Poznań. The control group consisted of 30 healthy boys of similar age. Each patient underwent a total of 42 functional tests measuring the length of individual muscles. Results: A comparative analysis of the study and control groups in terms of muscle flexibility showed significant differences in the results of 8 tests of the 42 tests carried out (19.04%). These tests assessed the length of the pectoralis major (clavicular part, right and left), the internal rotators of both humeral joints, the iliopsoas muscle (left lower extremity) and the rectus femoris muscles (right and left), and the ischiocrural muscles (left lower extremity) -the muscles were shortened significantly more often in boys without pectus excavatum. Boys with this deformity had significantly lower body weight and a significantly lower BMI (body mass index) compared to boys in the control group. Conclusions: When planning a physiotherapy programme for pectus excavatum patients, an individualized approach should be used to achieve the best possible results.

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Minimally invasive repair of pectus excavatum in patients with Marfan syndrome and marfanoid features

Cited by 34 publications

References 12 publications

Simultaneous repair of cardiac pathology and severe pectus excavatum in Marfan patients using a modified minimally invasive repair

Simultaneous repair of cardiac pathology and severe pectus excavatum in Marfan patients using a modified minimally invasive repair

Clinical Characteristics and Thoracic factors in patients with Idiopathic and Syndromic Scoliosis Associated with Pectus Excavatum

REHABILITATION Functional testing of muscles in patients with pectus excavatum – indications for preoperative rehabilitation

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