Clinical analysis of 113 patients with Poland syndrome

Abstract

Background: Poland syndrome is a rare congenital anomaly characterized by the partial or complete absence of pectoral muscles, varying thoracic deformities, and hand anomalies. To date, many variants of this syndrome and its accompanying anomalies have been reported.

Methods: In our clinic, 113 patients were diagnosed with Poland syndrome between 1990 and 2014. A latissimus dorsi muscle transfer was performed on 6 of these patients.

Results: Out of 113 patients, 63 (55.7%) were diagnosed with the syndrome on the right side, 42 (37.1%) were diagnosed on the left side, and 8 (7%) had a bilateral diagnosis. The partial or complete absence of the pectoralis major muscle was detected in all patients. Although 81 (71.6%) patients had a complete absence of the pectoralis major muscle, 32 (28.3%) were lacking only the sternocostal head of the muscle. In the analyzed cases, Poland syndrome was also found to be accompanied by specific anomalies. The most common anomaly accompanying Poland syndrome in these patients was Sprengel deformity, seen in 18 patients. Symmetry and stabilization of the chest wall were performed in 6 patients through transfer of the latissimus dorsi muscle.

Conclusions: Poland syndrome is a rare congenital anomaly, which has several variants and accompanying anomalies. The absence of several muscles in addition to the pectoral muscle can be seen in patients with Poland syndrome. Sprengel deformity is the most common accompanying anomaly. Several surgical procedures have been reported for the syndrome; for example, transposing the latissimus dorsi muscle is an effective procedure in terms of stabilizing the chest wall and providing optimum symmetric body appearance.