A case presentation with bilateral Pneumothoraces secondary to tuberous sclerosis

Abstract

Bilateral pneumothoraces is a rare presentation. Here we discuss a case with Bilateral spontaneous pneumothoraces secondary to Tuberous sclerosis. Tuberous sclerosis is a rare autosomal dominant disease characterized by numerous benign tumors in different parts of the body caused by mutations in either of two different genes ,TSC1 and TSC2. We had a 16 years old girl presented with bilateral spontaneous pneumothoraces. She had a history of left nephrectomy for angiomyolipoma (AML) of left kidney at the age 13 and an episode of fits at the age of 1 month. On examination she had multiple papules on face and neck (adenomasebaceum).High resolution Computed Tomography (HRCT) chest showed bilateral multiple small cysts and pneumothoraces both sides. She was managed with prompt chest intubation bilaterally for lungs re-expansion, oxygen therapy, intravenous antibiotics and chest physiotherapy with resolution of the pneumothoraces. nical presentation, in particular IgG4- Related disease (IgG4-D) is not clear.