Pectus updates and special considerations in Marfan syndrome
نویسندگان
چکیده
Congenital chest wall or pectus deformities including pectus excavatum (funnel chest) and pectus carinatum (pigeon chest) affect a significant proportion of the general population and up to 70% of patients with Marfan syndrome. Patients often experience significant morbidity and psychological distress, which can worsen with age. Here we discuss new techniques for both operative and non-operative treatment of pectus deformity, the importance of a welltimed intervention and special considerations in patients with Marfan syndrome.
منابع مشابه
Pectus Excavatum: Not Just a Cosmetic Concern
The exact cause of PEX is not known, but the primary theory centers around unbalanced overgrowth in the costochondral regions. One study showed that asymmetric PEX patients had shorter ribs on the more severely depressed side of the deformity. Up to 43% of PEX patients have a family history of the disease. It is thought to be a disease of multifactorial inheritance, and the exact genes implicat...
متن کاملگزارش یک مورد سندرم مارفان همراه با دفورمیتی‌های شدید اسکلتی با توارث اتوزوم مغلوب
A case of Marfan Syndrome with Severe Kyphoscoliosis in Recessive Autosomal from of Inheritance N. Tayebi [1] , M. tashakor [2] Received: 30/09/07 Sent for Revision: 23/04/08 Received Revised Manuscript: 15/07/08 Accepted: 23/08/08 Background and Objective: Marfan syndrome is known as an autosomal-dominant connective tissue disorder which affects the skeletal, ocular and cardiovas...
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Cardiovascular surgery in the setting of chest wall deformities is a clinical challenge. Pectus excavatum, for example, can cause heart displacement to the left thoracic cavity, following the poor operative field. This report highlights a case in which a successful aortic valve-sparing operation via conventional median sternotomy after correction of the heart displacement due to pectus excavatu...
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A 15-year-old boy presented with a moderate pectus excavatum (Figure 1). The preoperative X-rays are shown (Figures 2,3). He complained about cosmetics and did not have any physiological limitations. He plays badminton without symptoms and claims that he has the same level of endurance as his peers. Because of clinical suspicions concerning Marfan syndrome, he had an echocardiography, which was...
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عنوان ژورنال:
دوره 9 شماره
صفحات -
تاریخ انتشار 2017