Poland's syndrome and recurrent pneumothorax: is there a connection?

Poland's syndrome is a developmental deformity characterized by hypoplastic (unilaterally) chest wall with pectoralis major muscle atrophy, sometimes combined with syndactyly. It has an incidence of 1:7000 to a: 100000 live births and affects with a higher frequency (2:1) the right hemithorax [1,2]. It was first described by British Alfred Poland [3] and about 100 years later studied by the British doctor Patrick Clarkson [2] who named it after him. The syndrome is in rare cases combined with severe ocular pathologies, such as hamartoma of the retina and retinal pigment epithelium [4]. Herein, we are presenting two case reports of patients with the syndrome which was combined with bullae of the ipsilateral lung and suffered from recurrent episodes of pneumothorax.Two young (aged 19 and 21 years old) male patients with Poland's syndrome were admitted (with an interval of 6 months from each other) to our department after their second episode of pneumothorax for which they had to be treated with the placement of closed chest tube thoracostomy. Thorax CT imaging revealed the presence of 5-6 bullae to the ipsilateral (right) with the syndrome lung (Figures 1 and 2). Both patients were submitted to surgery (bullectomy, lung apicectomy, partial parietal pleurectomy, and chemical pleurodesis with hyperosmotic 35% Dextrose solution) with total lung expansion and excellent results both on discharge and on the 3-month follow-up control. The patients' informed consent was obtained prior to their inclusion in this report.Although the complex genetic and embryological mechanisms behind the genesis of developmental deformities are in most cases unknown, the knowledge of such clinical syndromes can always be useful for practicing physicians. In the current study we are reporting the cases of two young male patients with Poland's syndrome who were admitted with recurrent pneumothorax due to the presence of lung bullae affecting the ipsilateral to the syndrome side. Although more cas