A subarachnoid pleural fistula with massive crystal-clear pleural fluid caused by a lumbar epidural teratoma

ABSTRACT A subarachnoid pleural fistula — a connection between the pleural cavity and the subarachnoid space — generally presents after trauma or surgery. A 1-year 11-month-old girl without a history of trauma or surgery presented with fatigue, cyanosis and dyspnoea. A chest radiograph and computed tomography (CT) demonstrated a massive pleural effusion in the right hemithorax. About 300 ml of a crystal-clear pleural effusion, which looked like pure water, was removed by insertion of a chest drain, but it continued to collect. Cisternography and CT myelography confirmed leakage of cerebral spinal fluid into the right pleural cavity around the thoracolumbar region. Magnetic resonance imaging demonstrated an 11-mm enhanced nodule in the epidural space around the right lumbar (L) 1/2 intervertebral foramen. The patient underwent surgery and epidural tumours attached to the L1 nerve root foramen were completely resected and a fistula of the dura adjacent to the tumour was sutured. Histopathological examination demonstrated a mature teratoma containing a pancreatic component. On retrospective analysis of stored pleural fluid, a raised level of pancreatic enzymes was detected. It is presumed that digestive enzymes secreted by the pancreatic component of the teratoma lysed the dura, resulting in formation of the fistula. When a crystal-clear pleural effusion is present, even in the absence of trauma or surgery, a subarachnoid pleural fistula should be considered. As far as we know, this is the first report of a subarachnoid pleural fistula caused by a paravertebral teratoma. Abbreviations: CSF: cerebrospinal fluid; CT: computed tomography; 111In-DTPA: indium-111 diethylene triamine penta-acetic acid; MRI: magnetic resonance imaging; NIPPV: non-invasive positive pressure ventilation.