Marfan’s syndrome presenting as bilateral spontaneous pneumothorax

Abstract

Summary A case of bilateral spontaneous pneumothorax in a 14-year-old girl with previously undiagnosed Marfan’s syndrome is described. The pulmonary abnormalities of Marfan’s syndrome are not commonly encountered and bilateral pneumothorax is itself a rare event which, in most instances, has been reported following invasive procedures.

Pulmonary Histologic Changes in Marfan Syndrome

“…Previous studies have reported spontaneous pneumothorax (unilateral and bilateral), congenital lobar abnormalities, bullae, bronchiectasis, cystic disease and changes, honeycombing, apical fibrosis, and emphysema. 5,[11][12][13][14][15][16][17][18][19][20][21][22][23][24][25] These studies have largely relied on imaging and gross examination, with spontaneous pneumothorax being the pathologic change most often described. The few studies that explore histologic classification tend to report nonspecific microscopic descriptions.…”

Chest Pain in an Adolescent with Marfan Syndrome

“…Chest pain is almost universal in spontaneous pneumothoraces, whereas dyspnea is the second most common symptom (5,6). Spontaneous pneumothorax has been reported in patients with MFS (7,8).…”

VATS for Bilateral Syncronized Spontaneous Pneumothorax

“…The operation time was shortened in this way. Bilateral spontaneous pneumothorax could result from sarcoidosis, lymphangioleiomyomatosis, Marfan's syndrome, metastasis, tuberculosis, and mesothelioma (5)(6)(7)(8). For this reason, systemic physical examination should be conducted on these patients.…”