Cystoperitoneal shunt as alternative treatment for a giant thoracic meningocele in a patient with neurofibromatosis.

Abstract

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.

CASE DESCRIPTION:

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.

CONCLUSION:

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.

• Authors: Dimitri J Vanhauwaert, Marc J Deruytter
• Journal: Surgical neurology (Surg Neurol) Vol. 69 Issue 5 Pg. 535-7 (May 2008) ISSN: 0090-3019 [Print] United States
• PMID: 18262243 (Publication Type: Case Reports, Journal Article)

Topics

• Catheterization
• Drainage (methods)
• Female
• Humans
• Meningocele (complications, pathology, surgery)
• Middle Aged
• Neurofibromatoses (complications, pathology, surgery)
• Peritoneum
• Thoracic Vertebrae