Body Image in Patients with Marfan Syndrome.

Laura Birke Hansen,Yskert Von Kodolitsch,Dieter Benninghoven,Friedrich Schroeder

doi:10.3390/jcm9041015

Laura Birke Hansen, Yskert Von Kodolitsch + Show 2 more

Open Access

https://doi.org/10.3390/jcm9041015

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Abstract

This study aimed to assess body image impairments of individuals with Marfan syndrome and to determine to what extent psychological, physical, and sociodemographic factors influence body image. We assessed the physical fitness and psychosocial health of 42 patients with Marfan syndrome at the beginning of an inpatient rehabilitation program. All participants filled out a body image questionnaire consisting of two scales: (1) Negative Body Evaluation and (2) Vital Body Dynamics. We compared body image data of the study sample with the German representative norming sample and carried out two separate regression analyses in order to determine which variables were associated with the two dimensions of patients’ body image. Body image of individuals with Marfan syndrome appeared to be significantly impaired, with high percentile ranks for Negative Body Evaluation (women = 88, men = 91) and low percentile ranks for Vital Body Dynamics (women = 11, men = 4). Vital Body Dynamics was predicted by age (p = 0.016) and by depression (p < 0.001), and Negative Body Evaluation was predicted by anxiety (p = 0.001). Body image in individuals with Marfan syndrome is not primarily determined by objective measures of fitness or by objective cardiac impairment but by psychological variables like depression and anxiety and by age. This finding can inform treatment and rehabilitation concepts. Accepting Marfan syndrome, including the acceptance of being visually different, may not only demand medical treatment and physical rehabilitation but also psychological treatment for depression and anxiety.

Highlights

Marfan syndrome is an autosomal dominant genetic disorder of the connective tissue
All patients met the following criteria: (1) diagnosis of Marfan syndrome (Q87.4 according to ICD-10- GM) in stable condition proven by a Marfan specialist located in one of the Marfan units in a German university clinic, (2) New York Heart Association (NYHA) stage < III, (3) time since last cardiovascular surgery > 3 months, and (4) no relevant increase in aortic diameter within the last 12 months
The high percentile ranks for Negative Body Evaluation and the low percentile ranks for Vital Body Dynamics indicate that, in line with our hypotheses, Marfan syndrome patients do not feel well in their body, tend to evaluate their body much more negatively, and perceived themselves as much less powerful and energetic than healthy individuals of a comparable age

Summary

Introduction

Clinical manifestations are variable and affect different organ systems. Patients with Marfan syndrome have to cope with reduced visual acuity and even blindness due to lens dislocation. Obesity is often a problem in older patients [1]. Skeletal abnormalities may include an elongated face, torso, and limbs, a long, slender body shape, chest deformities, scoliosis, and joint hypermobility [2]. These skeletal abnormalities, in combination with the frequent use of very thick eyeglasses, may result in a characteristic appearance that is different from the norm, and which is subjectively perceived by affected individuals and visible to others

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