Poland syndrome: Treatment and management
Advanced Thoracic Surgery, Akademisyen Kitabevi, ss.561-572, 2021
- Yayın Türü: Kitapta Bölüm / Araştırma Kitabı
- Basım Tarihi: 2021
- Yayınevi: Akademisyen Kitabevi
- Sayfa Sayıları: ss.561-572
- Sağlık Bilimleri Üniversitesi Adresli: Evet
Özet
Poland Syndrome is known as a rare congenital anomaly characterized by partial or complete absence of the pectoral muscles. This syndrome typically presents with multiple accompanying thoracic anomalies. The most common component of Poland Syndrome is the complete or partial absence of the pectoral muscles. Additionally, patients who have one or more of the other components are diagnosed with Poland Syndrome. The treatment of Poland Syndrome aims to provide the patient with an aesthetic and symmetrical appearance. There are various options for correcting the deformity, such as bone grafts, patches, prosthetic materials, muscle flaps, and combinations of these methods. Although the etiology of Poland Syndrome has not been fully determined, it can be considered as a non-genetic congenital disease. Due to the rarity of this syndrome, publications are often presented in the form of case reports. The number of published case series on Poland Syndrome is very limited. The largest single-center series of this syndrome has been published in our country. It has been reported that Poland Syndrome generally occurs with a frequency of 1/30000 to 1/32000. The syndrome affects only one side of the body and the anomaly is detected on the right side in 60-75% of patients. The question of whether the syndrome can affect both sides is still controversial. Nine cases of bilateral Poland Syndrome have been reported in the literature. Although Poland Syndrome is a sporadic disease, less than 1% show familial characteristics. In those with familial characteristics, the ratio of female to male and the ratio of right-sided disease to left-sided disease are equal. Despite many hypotheses being reported about the etiology of Poland Syndrome, it has not yet been definitively determined. Poland Syndrome is a very rare congenital anomaly that can be characterized as the partial or complete absence of pectoral muscles. It is generally presented with many coexisting thoracic anomalies. (1-4) Although the title -Poland syndrome-has been used for referring to different combinations of pectoral muscle deficiencies, syndactyly and chest deformities, various variants and accompanying diseases have been reported over time. Even though there are few case series reported in the literature, the largest single-center series had been published in our country. (1) Due to its rarity publications are generally in the form of case reports. The major component seen in patients with Poland syndrome is complete or partial absence of the major pectoral muscle. In addition to that patients having one or more of the other components are diagnosed with Poland syndrome. (5,6) The treatment of Poland syndrome aims to provide an aesthetic and symmetrical appearance tothe patient. There are multiple different options as bone grafts, patches, prosthetic materials, muscle flaps and combinations of these methods are used for the correction of the deformity.