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Spontaneous pneumothorax as manifestation of Marfan syndrome
Carolina Viveiro1, Patricia Rocha, Cristiana Carvalho
1Department of Pediatria, Centro Hospitalar de Leiria-Pombal, Leiria, Portugal.
Abstract:
The authors describe a 16-year-old boy, previously healthy, who was admitted to our hospital for left-sided spontaneous pneumothorax. On physical examination he presented with marfanoid habitus. Pneumothorax was managed conservatively with resolution. Four months later he had a recurrence of left-sided pneumothorax and 1 week after that he presented with contralateral pneumothorax. He underwent video-assisted thoracoscopic surgery twice for bullectomy and pleurodesis. No further recurrence was stated. Additional investigation showed a prolapsed cardiac mitral valve and Marfan syndrome was confirmed genetically.
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