Source: Annual Congress 2008 - Mediastinal lesions; rare pathology

Abstract

Poland‘s syndrome is a congenital chest wall deformity. It has incidence of 1 in 30.000. Poland‘s syndrome includes agenesis or hypoplasia of the pectoralis major and minor muscles, agenesis of costal cartilages and absence of anterior parts of ribs, abnormalities of breast, hypoplasia of subcutaneous tissue and brachysyndactyly in unilateral hand.
The diagnosis of Poland‘s syndrome was identified in 48 patients between January 1990 and December 2007. Forty-seven patients were male and their mean age was 21.46 (19-28). Other patient was 6 year-old girl.
Poland‘s syndrome was localized at right side in 33 patients and at left side in 15 patients. There were pectoral agenesis in 36 patients and pectoral hypoplasia in 12 patients. In all of the patients there were hypoplasia of subcutaneous tissue. There were amastia or hypomastia in 26 patients, athelia or hypothelia in 25 patients, agenesis of costal cartilages and absence of anterior parts of ribs in 16 patients, lung herniation via chest wall defect in 10 patients, paradox respiration in 5 patients, brachydactyly in 7 patients and syndactyly in 8 patients. Most common presenting symptoms were restriction of upper limb movement, chest pain with effort and dyspnea. In pulmonary function studies there was mild decreasing in 5 patients and moderate decreasing in 2 patients. There were dextracardia in three patients and patent foramen ovale in other one. Transposition of latissimus dorsi flap to anterior chest wall was performed to five patients with rib defects in our clinic.
For protection of visceral organs in patients with multiple rib agenesis and for stabilization of chest wall in patients with rib agenesis accompanying paradox respiration, we suggest surgical procedure.

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T. Isitmangil, N. Yiyit, R. Gorur, F. Candas, H. Turut, O. Erdik, S. Selcuk, A. Yildizhan (Istanbul, Turkey). An overview about 48 patients with Poland‘s syndrome. Eur Respir J 2008; 32: Suppl. 52, 2146

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