PURPOSE: Poland’s syndrome is a rare congenital anomaly characterized by hypoplasia of the breast and nipple, scarcity of subcutaneous tissue, absence of the costosternal portion of the pectoralis major muscle, lack of the pektoralis minor muscle, aplasia or deformity of the costal cartilages or ribs, alopecia of the axillary and mammary region, and unilateral brachysyndactyly. Poland’s syndrome occurs in approximately one in every 7.000 to 100.000 live births, being more prevalent in males than females ( ratio of 2:1 to 3:1 ) and more frequently ( 60-70% ) affecting the right side of the body.
METHODS: Thirty eight patients were diagnosed as Poland’s syndrome in our clinic. 2 of the patients were female and others were male. Mean age was 23.5 (5-37). The admitting complaints included shortness of breath with exercise, and restriction in movement and weakness in affected extremity. All patients had detailed radiological investigation, routine blood investigation, respiratory function test, peripheric smear and also cardiology consultation.
RESULTS: Thirty-eight patients were examined. Thirty-six of them were male and 2 female. Right-sides were involved in 19 patients, and left-sided in 19 patients. The sternal portion of the pectoralis major muscles were absent in all, and pectoralis minor muscles were absent in 6 patient. Breast and nipple hypoplasia were seen in 28 patients. Amastia were detected in 11 patients. Deficiencies of the skin, subcutaneous tissues, sweat glands, and hair of affected areas were seen in all patients. Eight patients had involvement of the ribs on the affected side, 4 of them with marked chest deformity. Involvement of the hand and arm varied greatly. In 4 patients the hands were normal. One patient had hand hypoplasia including lack of 2nd finger and hypoplasia of 3rd, 4th and 5th fingers. One patient had ectrodactyly and others had brachysyndactyly. Involved arms of the patients were thinner and shorter. Family histories were obtained in two cases.
CONCLUSIONS: As a conclusion, a multidisciplinary approach is needed for the diagnoses of Poland’s syndrome.Despite they are seen less, the examination and investigations for the search of tumor or other syndromes sould be carried out.
CLINICAL IMPLICATIONS: Poland’s syndrome is a rare congenital anomaly and it need to investigate carefully all body.
DISCLOSURE: The following authors have nothing to disclose: Akin Yildizhan, Esra Yildizhan, Suat Patlakoglu, Deniz Yorgancilar
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