Cystoperitoneal shunt as alternative treatment for a giant thoracic meningocele in a patient with neurofibromatosis.
BACKGROUND
Thoracic meningoceles are associated with neurofibromatosis 1 in 60% to 85% of all cases. Usually, these meningoceles remain asymptomatic, but back pain, headache, cough, and dyspnea are possible manifestations. Often, there is an associated kyphoscoliotic deformity of the thoracic spine.


METHODS
A 60-year-old woman known in our department after a fossa posterior decompression for an Arnold-Chiari malformation was admitted through the emergency department because of progressive dyspnea. A giant intrathoracic meningocele was already diagnosed earlier but was left untreated because the patient was asymptomatic at that time. She now had dyspnea, and on chest x-ray and CT scan, there was an obvious shift of the mediastinum to the right. Because of the long-existing hemithoracic meningocele, we assumed that this patient actually had only 1 functional lung, and so, left-sided thoracotomy with resection of the meningocele and closure of the defect included a high operative mortality. Instead, we chose to obtain a permanent drainage of the meningocele by putting a shunt between the meningocele and the peritoneum. Postoperatively, the patient recovered well and became oxygen-independent.


CONCLUSIONS
Treatment of giant intrathoracic meningoceles in patients with progressive dyspnea can be challenging, and different options can be found in the literature. Treatment with a cystoperýtoneal shunt, as in our case, can be a less invasive alternative in patients with a high operative mortality risk. To our knowledge, this is the first report of a patient with neurofibromatosis 1 treated in this way.
Vanhauwaert
Dimitri J
DJ
Department of Neurosurgery, Heilig Hart Ziekenhuis, Roeselare, Belgium, 8800. vanhauwaertdimitri@telenet.be
Deruytter
Marc J
MJ
D002404:Catheterization; D004322:Drainage / Q000379:methods*; D005260:Female; D006801:Humans; D008588:Meningocele / Q000150:complications / Q000473:pathology / Q000601:surgery*; D008875:Middle Aged; D017253:Neurofibromatoses / Q000150:complications* / Q000473:pathology / Q000601:surgery; D010537:Peritoneum; D013904:Thoracic Vertebrae