Artikel
CSF leakage in Gorham-Stout disease due to dura mater involvement after progression of an osteolytic lesion in the thoracic spine
Suche in Medline nach
Autoren
Veröffentlicht: | 24. April 2015 |
---|
Gliederung
Text
Introduction: Patients with Gorham-Stout disease (GSD), a rare disease of poorly understood etiopathophysiology, suffer from progressive osteolysis. Destruction of bone matrix is caused by lymphatic vessels, which can lead to CSF leakage if parts of bony structures adjacent to CSF spaces are involved. So far, fewer than 200 patients have been reported in the literature; only 4 of these patients presented with CSF leakage.
Material and methods: We report the case of a 30-year-old man with GSD and CSF leakage due to dura mater involvement after progression of an osteolytic lesion in the thoracic spine. Neurosurgical intervention, including dura repair, was needed. Experimental medical therapy with rapamycin was started, leading to disease control for more than 12 months.
Results: The patient recovered quickly after surgery. Postop- erative MRI demonstrated regression of low-lying cer- ebellar tonsils only 6 mm below the foramen magnum. The patient was discharged from the hospital 14 days after surgery. Rapamycin therapy was started again 4 weeks after surgery. The patient remained in good clini- cal condition without cephalalgia at clinical follow-up 12 months later.
Conclusion: Progression of GSD can lead to destruction of the meninges, causing CSF leakage. We review 4 other cases reported in the literature and discuss therapeutic options.
Figure 1 [Fig. 1]