Surgical emphysema and pneumothorax: Unfamiliar combination in Ehlers-Danlos syndrome

Abstract

A 53-year-old gentleman presented with sudden onset swelling over the neck for the past 5 days. It was associated with dyspnoea on exertion. He was a known patient of Ehlers-Danlos syndrome since sixteen years of age. Initially, the swelling appeared over the neck which gradually progressed over to the anterior chest wall and the nape of the neck. There was no history of trauma, fever, surgery or similar complaints in the past. He was a known smoker. On examination, there was diffuse subcutaneous emphysema over the neck, and the anterior chest wall (Figure 1). Skin crepitus was present. The skin was hyper-extensile, fragile and easily stretchable (Figure 1). The patient was vitally stable. On rigid laryngoscopy, bilateral vocal cords were mobile. We admitted the patient and performed a CT scan of the neck and chest to assess the extent of the emphysema. CT scan confirmed an extensive subcutaneous emphysema over the neck, extending posterior to the oesophagus. There were multiple emphysematous bullae over the apical region of the right lung and an area of pneumothorax just in the vicinity of the bullae (Figure 2). The patient was planned for incision and evacuation of the air. A horizontal incision was given over the most prominent part of the swelling and the air was milked out gently. An Intercostal Chest Drain (ICD) was inserted and the condition was closely monitored. The recovery was uneventful. He was discharged after seven days of hospital stay.