PP 76 “I AM TALL AND BREATHLESS FOR A REASON”: MARFAN’S SYNDROME WITH PNEUMOTHORAX

Abstract

INTRODUCTION

We present a case of Marfan’s syndrome presenting at our centre with chest pain and our approach of management.

CASE REPORT

A 17 years old young Indian gentleman who was well previously, presented with right sided chest pain for 4 days. Patient had chest pain and shortness of breath; worse during coughing. There was no sweating or nausea. Patient denied any history of recent trauma, exercise or sports injury. On arrival, he was hemodynamically stable but there was tachypnoea at the rate of 18 per minute while the oxygen saturation was 98% on air. Upon examination, the trachea was central. Slight increase in resonance was noted on percussion on the right lung. The lungs were clear on auscultation but reduced air entry noted over the right upper and middle zone. The chest X-Ray showed pneumothorax of the right lung (apex region) measuring 1cm. It was also noted that patient was was thin and tall (178cm) and his arm span to height ratio was almost 1:1. His fingers were long and he had a high arch palate. Patient was given a high flow mask at 15L/ min on top of nasal prong at 3L/min for 1hour, in the hope that there will be reabsorption of air. Nevertheless, the size increased to 2 cm on repeat X-Ray although patient felt a lot better with the oxygen delivery. A chest tube was inserted and patient was admitted to medical ward. Patient was diagnosed to have pneumothorax as well as Marfan’s syndrome. Patient stayed in ward for 3 days and discharged well.

DISCUSSION & CONCLUSION

This is a case of Marfan’s syndrome presenting with pneumothorax. It is a known fact that one of the features of Marfan’s syndrome is that patients may be proned to developing pneumothorax. Be alert in patients having features of Marfan’s syndrome and chest pain as this may indicate the development of pneumothorax.