Recurrent Spontaneous Pneumothorax Revealing Marfan Syndrome: A Case Report

Abstract

The present study reports about recurrent spontaneous pneumothorax revealing Marfan syndrome. Marfan syndrome is a rare autosomal dominant disease that affects connective tissue and affects several organs, including the lung. It appears at a variable age and can be unrefined. This is a case of patient aged 16 years, eldest of two siblings, without toxic habits, treated for pulmonary tuberculosis confirmed bacteriologically 2 years ago and operated on 5 years ago for a left inguino-scrotal hernia. The patient presented to the department on day 7 after discharge following spontaneous stabbing left chest pain associated with dyspnea at the slightest effort. On clinical examination, there was a syndrome of air effusion in the left hemithorax. The frontal chest X-ray revealed a large total left pneumothorax which was drained. Lung involvement rarely modifies the respiratory functional prognosis. The occurrence of a pneumothorax is rarely a mode of entry into the disease, hence the interest in discussing the diagnosis in cases of recurrent spontaneous pneumothorax or thoracic deformation.