Children With Marfanʼs Syndrome and Pectus Excavatum

Es, Golladay; Char, F; Dl, Mollitt

doi:10.1097/00007611-198511000-00012

Cited by 18 publications

(11 citation statements)

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“…Patients with Marfan's syndrome are more likely to have delayed wound healing following repair of pectus excavatum. 59,60 Surgical correction in children should be avoided, as recurrence is common in this age group. 60 Spontaneous pneumothorax occurs in 4 -11% of patients and may be associated with apical bullae.…”

Section: Respiratory Systemmentioning

confidence: 99%

Marfan syndrome: clinical diagnosis and management

2007

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In association withMarfan syndrome: clinical diagnosis and management Marfan syndrome is a multisystem connective tissue disorder usually associated with mutation in fibrillin, and occasionally with mutation in TGFBR1 or 2. The clinical diagnosis is made using the Ghent nosology, which will unequivocally diagnose or exclude Marfan syndrome in 86% of cases. Use of a care pathway can help implementation of the nosology in the clinic. The penetrance of some features is age dependent, so the nosology must be used with caution in children. Molecular testing may be helpful in this context. The nosology cannot be used in families with isolated aortic dissection, or with related conditions such as Loeys -Dietz syndrome, although it may help identify families for further diagnostic evaluation because they do not fulfill the nosology, despite a history of aneurysm. Prophylactic medical (eg b-blockade) and surgical intervention is important in reducing the cardiovascular complications of Marfan syndrome. Musculoskeletal symptoms are common, although the pathophysiology is less clear -for example, the correlation between dural ectasia and back pain is uncertain. Symptoms in other systems require specialist review such as ophthalmology assessment of refractive errors and ectopia lentis. Pregnancy is a time of increased cardiovascular risk for women with Marfan syndrome, particularly if the aortic root exceeds 4 cm at the start of pregnancy. High-intensity static exercise should be discouraged although low-moderate intensity dynamic exercise may be beneficial. The diagnosis and management of Marfan syndrome requires a multidisciplinary team approach, in view of its multisystem effects and phenotypic variability.

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Section: Respiratory Systemmentioning

confidence: 99%

Marfan syndrome: clinical diagnosis and management

2007

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“…Multiple surgical techniques were used in their series, which may have further impacted their outcomes. Golladay et al [6] demonstrated an increased rate of wound problems but acceptable cosmetic and functional results after Ravitch repair in 8 Marfan syndrome patients compared with non-Marfan patients with PE. Limited long-term follow-up in both of these studies may also contribute to the conclusion that there are differences in PE repair surgical outcomes for patients with Marfan syndrome.…”

Section: Discussionmentioning

confidence: 99%

“…Although the incidence of PE is between 1 in 400 and 1 in 1000 live births [4], it is present in two thirds of patients with Marfan syndrome [5]. In fact, it has been recommended that any child with PE be screened for Marfan syndrome [6]. It is well established that PE affects cardiopulmonary functioning in both normal and Marfan patients, but there has been very little research assessing whether there are differences in these effects between these populations [7].…”

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confidence: 99%

“…It is well established that PE affects cardiopulmonary functioning in both normal and Marfan patients, but there has been very little research assessing whether there are differences in these effects between these populations [7]. In correcting PE in patients with Marfan syndrome, the use of sternal support has been noted as essential to ensuring a good result [6]. However, "there is very little information regarding the outcome of patients with Marfan syndrome who undergo repair of pectus excavatum" [8].…”

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confidence: 99%

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

Redlinger

Rushing

Moskowitz

et al. 2010

Journal of Pediatric Surgery

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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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Children With Marfanʼs Syndrome and Pectus Excavatum

Cited by 18 publications

References 0 publications

Marfan syndrome: clinical diagnosis and management

Marfan syndrome: clinical diagnosis and management

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

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

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