A Case of Gorham-Stout Disease Treated with Fistula Closure by Transmeatal Approach

Abstract

Gorham-Stout disease, a rare and intractable disease of unknown etiology, causes systemic bone lysis and replacement with lymphoid tissue. Here, we report a case of Gorham-Stout disease with cerebrospinal fluid leakage in a 16-year-old boy. The patient complained of nasal discharge, right ear obstruction, fever, and headache. A computed tomography scan of the head showed osteolysis around the right internal carotid artery, vestibule, and cochlea and osteolytic changes in the left parietal bone. It was suggested that the patient had bacterial meningitis owing to the leakage of cerebrospinal fluid from the fistula caused by the temporal bone osteolysis. He was treated with meropenem, and a transmeatal fistula closure and a bone biopsy of the left parietal bone were performed. Intraoperatively, osteolysis was observed on the promontory and around the internal carotid artery. The fistula was closed by dense filling and compression around the fistula, in the middle ear cavity, and in the external auditory canal. The symptoms disappeared after the surgery. Bone biopsy showed the presence of a lymphangioma, and Gorham-Stout disease was diagnosed. Prophylactic bisphosphonate therapy was initiated. A 4-year follow-up revealed no progression of the disease.