Abstract
Aim. To investigate the possible connection of Poland's syndrome with the presence of lung bullae and, thus, with an increased risk for recurrent pneumothorax. Patients-methods. Two male patients, aged 19 and 21 years respectively were submitted to our department after their second incident of pneumothorax. Both had Poland's syndrome (unilaterally hypoplastic chest wall with pectoralis major muscle atrophy) and both had multiple bullae to the ipsilateral lung based on CT findings. The patients were treated operatively (bullectomy, lung apicectomy, partial parietal pleurectomy and chemical pleurodesis) due to the recurrent state of their pneumothorax. Results. The patients had good results with total expansion of the affected lung. Conclusions. Poland's syndrome can be combined with ipsilateral presence of lung bullae, a common cause of pneumothorax. Whether this finding is part or a variation of the syndrome needs to be confirmed by a larger number of similar cases.
Highlights
Poland’s syndrome is a developmental deformity characterized by hypoplastic 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
Cases Presentation Two young male patients with Poland’s syndrome were admitted to our department after their second episode of pneumothorax for which they had to be treated with the placement of closed chest
Thorax CT imaging revealed the presence of 5-6 bullae to the ipsilateral with the syndrome lung (Figures 1 and 2). Both patients were submitted to surgery with total lung expansion and excellent results both on discharge and on the 3-month follow-up control
Summary
Introduction 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.
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