Acute respiratory failure due to intercostal artery rupture and bilateral thoracic meningoceles in a patient with neurofibromatosis type 1: a case report.

BACKGROUND

Thoracic meningoceles (tMCs) are extremely rare, yet the majority occur in patients with Neurofibromatosis type 1 (NF1). Optimal treatment remains to be determined.

METHODS

A 50-year-old female was admitted with acute dyspnea. Workup showed bilateral thoracic masses, suspicious for tMCs, given a history of NF1. External lumbar drainage (ELD) was placed, which relieved the symptoms, yet after two days, the patient went into cardiac arrest due to a ruptured intercostal artery. After successful cardiopulmonary resuscitation and embolization, the patient was stabilized, and a thoracic-neurosurgical procedure was planned. Via a posterior thoracotomy, the larger of the two meningoceles was resected using a linear stapler and removed en bloc. Dura was reinforced with TachoSil®, and the thoracic wall was reinforced with expanded polytetrafluoroethylene (e-PTFE).

RESULTS

The patient developed postoperative pneumonia and was extubated on the 12th postoperative day. She was re-intubated after developing sepsis, re-extubated on the 61st postoperative day, then transferred to the pulmonology ward, and ultimately discharged to rehabilitation. At six months postoperatively, she remains clinically stable, with no new neurological deficits.

DISCUSSION: Thoracic MCs remain challenging in diagnosis and treatment, and should be kept in mind as a differential diagnosis of acute respiratory failure in patients with NF1.