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Poland's syndrome with spontaneous pneumothorax: report of two cases
Shi-Ping Luh1, Pan-Chyr Yang, Chun-Jean Lee
1Department of Surgery, Kuang Tien General Hospital, Hong Kuang College of Technology, Taipei, Taiwan.
Abstract:
Poland's syndrome is an uncommon congenital anomaly of the chest wall characterized by the absence of the pectoralis major muscle and other nearby musculoskeletal components. Many associated aberrations over the thoracic cage, intrathoracic organs, and upper limbs have been reported. However, spontaneous pneumothorax in these patients has not been reported. Here, we describe two patients with both Poland's anomaly and spontaneous pneumothorax. One patient was a 16-year-old boy with left chest wall hypoplasia and pneumothorax on the right side. The other was a 27-year-old man with right chest wall hypoplasia, hand brachydactyly, and pneumothorax. Pneumothorax in both patients was treated with bullectomy and mechanical pleurodesis with the aid of videothoracoscopy, and the postoperative courses were smooth. Blood supply disruption has been hypothesized as a pathogenic mechanism of both spontaneous pneumothorax and Poland's syndrome, suggesting an association between these two diseases.