تأثیر یک برنامه تمرین اصلاحی متقارن بر ناهنجاری اسکولیوز در فرد مبتلا به سندرم شبه مارفان (مطالعه موردی)

Background and Aims: Marfan syndrome is a genetic disorder whose diagnosis is based on clinical findings in the cardiovascular, vision, musculoskeletal systems and positive family history. Marfan-like patients often have symptoms and musculoskeletal abnormalities related to Marfan syndrome, but they do not have the problems and complications of the cardiovascular and vision systems. The prevalence of this disease is 1 in 3000-10000 and it affects men and women of all races and ethnicities equally. Musculoskeletal symptoms and abnormalities of Marfan and Marfan-like patients, including: Pectus excavatum and chest asymmetry, loss of plantar arches and flat feet, long and abnormal length of upper limbs, different degrees of dorsal kyphosis and high degrees of scoliosis in different areas of the spine. It is the vertebrae. Research has shown that 40% of people with Marfan syndrome have kyphosis more than 50 degrees, and about 52-63% of them have scoliosis.

In general, exercise-based approaches, including corrective and therapeutic exercises based on efficient scientific resources, have been useful in reducing the complications of deformity and preventing the progression of scoliosis severity. On the other hand, based on researches, exercise and physical activity have been recommended for Marfan patients. There are limited articles on the research relating to the effect of corrective exercises in the treatment of scoliosis, indicating that there is a lack of high-quality research to finalize the use of exercises for the management of scoliosis. On the other hand, among the review articles examined by the researcher and the search in various databases, there is no article about the use of corrective exercises on subjects with Marfan and Marfan-like syndrome. Most of the studies related to these patients have investigated the results of exercise and physical activity in general and with emphasis on cardiorespiratory problems, and the correction of their skeletal and muscular abnormalities has been less investigated. Therefore, the purpose of this research is to investigate the effect of symmetrical corrective exercise program on scoliosis deformity of a Marfan- like patient.

Materials and Methods: This research is a case study with a pre-test and post-test design, which was conducted on a subject with Marfan-like syndrome and scoliosis. Descriptive statistics including obtaining the pre-test and post-test differences in terms of degree of scoliosis and angle between vertebrae and percentage were used to analyze the data. In this way, the angles obtained after the test were subtracted from the angles before the test, and for the uniformity of the data and to simplify their interpretation, they were converted to percentages by multiplying them by percentage fractions.

In this research, the subject performed a 12-week (24 sessions) symmetric corrective exercise. The research protocol was designed including symmetrical stretching exercises in the frontal and sagittal plane, symmetrical mobility exercises in the transverse plane and bilateral strengthening-rotational exercises in the transverse plane, in which the stretching exercises were started in the initial sessions with the minimum possible duration and then increased until the final sessions. Strengthening and mobility exercises also started with the minimum repetitions and progress was made according to the principle of individual characteristics and characteristics of the subject. It should be noted that during the twelve weeks of the implementation of the research protocol, the subject did not participate in any sports activities, and if he needed advice, he would consult his specialist doctor and inform the researcher. Before and after the exercises, the overall angle of the scoliosis arches was measured using Cobb method in thoracic and lumbar areas on the radiograph. Also, the Cobb angle between vertebrae involved in scoliosis arches was measured segment by segment using radiographs.

Results: The findings showed that the post-test size of the thoracic angle has decreased by 3.8 degrees (15.56%) compared to the pre-test and the post-test value of the lumbar angle has decreased by 6.2 degrees and 9.3% compared to the pre-test. Also, the following table shows the changes in the Cobb angle between all the vertebrae involved in the S-shaped curve of scoliosis (L4) to (T6) before and after the exercises. The results showed that the changes in the angle between the sixth and seventh vertebrae (T7-T6) in the thoracic region and the third and fourth vertebrae (L4-L3) in the lumbar region also decreased by 22.2 and 62.5%, respectively. Also, the amount of changes in the angle between the twelfth thoracic-first lumbar vertebrae (T12-L1) and the eleventh twelfth thoracic (T12-T11) and the tenth and eleventh thoracic (T10 T11) (Pivotal and near-pivotal points in the S-curve bend) was minimum and zero.

Variable

(Degree) Angel Value (Degree) Difference of Angels (Degree) Reduction Percentage

Pre-test Post-test

Angel Between T6-T7 9.0 7.0 2.0 %2.22

Angel Between T7-T8 3.1 1.1 2.0 %15.38

Angel Between T8-T9 1.1 9.0 2.0 %18.18

Angel Between T9-T10 6.1 4.1 2.0 %12.5

Angel Between T10-T11 5.1 5.1 0 0

Angel Between T11-T12 1.1 1 1.0 %9.09

Angel Between 12-L1 2 2 0 0

Angel Between L1-L2 9.0 9.0 0 0

Angel Between L2-L3 8.1 6.1 2.0 %11.11

Angel Between L3-L4 8.0 3.0 5.0 %62.5

*T: thoracic vertebrae

**L: Lumbar vertebrae

Conclusion: Symmetrical corrective exercises have significantly reduced the scoliosis angle of the Marfan-like subject and improved the alignment of his spine. Although other musculoskeletal abnormalities were not investigated in this research, the results of the current study have shown the positive effect of symmetrical corrective exercises. The review of previous studies shows that the present study is one of the few studies that has been able to prove the effect of exercise on scoliosis in people with Marfan-like syndrome by using the method of symmetric corrective exercises. The results of the present study showed that if the corrective exercises are designed and prescribed in the correct way benefiting scientific methods, they can be effective in correcting the scoliosis dissonance of people with Marfan-like syndrome.