Abstract:
OBJECTIVE AND IMPORTANCE:Syringomyelia in association with the Dandy-Walker complex is rare and only 17 cases have been reported, mostly as autopsy findings or incidental reports in series for other conditions. We present a further case report and discuss the possible pathogenetic mechanisms of syrinx formation in the presence of the Dandy-Walker complex. We emphasize the role of foramen magnum obstruction and alteration in cerebrospinal fluid flow dynamics in syrinx formation. The therapeutic importance of disimpaction of the foramen magnum is stressed. CLINICAL PRESENTATION:A 39-year-old man presented with headache. As a child, he had had cystoperitoneal and ventriculoperitoneal shunts inserted for hydrocephalus in association with the Dandy-Walker complex. On examination, the patient had limited upgaze but was otherwise normal. Magnetic resonance imaging revealed a large cervicothoracic syrinx and a Dandy-Walker cyst obstructing the foramen magnum. INTERVENTION:The cystoperitoneal shunt was revised. The patient's headaches resolved, and follow-up imaging demonstrated resolution of the syrinx and disimpaction of the foramen magnum. CONCLUSION:Disimpaction of the foramen magnum can resolve syringomyelia in patients with the Dandy-Walker complex. This can be achieved by a shunt procedure or by formal foramen magnum decompression. The rarity of the association between the Dandy-Walker complex and syringomyelia prevents firm conclusions from being made regarding the best management strategy for this condition.
journal_name
Neurosurgeryjournal_title
Neurosurgeryauthors
Hammond CJ,Chitnavis B,Penny CC,Strong AJdoi
10.1097/00006123-200201000-00028keywords:
subject
Has Abstractpub_date
2002-01-01 00:00:00pages
191-4issue
1eissn
0148-396Xissn
1524-4040journal_volume
50pub_type
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