Second Pneumothorax in an Ehlers-Danlos Syndrome Patient Initially Treated by Bullaectomy Twenty-two Years Ago

A 2nd pneumothorax developed in a 40-years-old man after his 1st bilateral pneumothoraces were successfully treated by bullaectomies 22 years ago. For the past 10 years, he has suffered major complications such as repeated bleeding into the digestive tract due to vascular-type Ehlers-Danlos syndrome (vEDS). Although preoperative computed tomography demonstrated multiple bullae at the apex of the right lung, any abnormal findings suggesting tissue fragility was not found. At surgery, visceral pleura of the right lung had an almost normal appearance through a thoracoscope. However, we found that gentle touch with soft surgical equipment resulted in serious pleural damage leading to new air leakage. Our case suggests a progressive fragility of the lung in a vEDS patient, and a less invasive pleural covering technique would therefore be preferable when major complications become evident, despite previous successful bullaectomy.