Syringomyelia caused by an arachnoid web in a patient with shunted Dandy-Walker malformation

Autor: Hee Chang Lee, Seung-Ki Kim, Ji Yeoun Lee, Byung Kyu Cho, Jung Won Choi, Kyu-Chang Wang, Ji Hoon Phi
Rok vydání: 2016
Předmět:
Zdroj: Child's Nervous System. 33:665-670
ISSN: 1433-0350
0256-7040
DOI: 10.1007/s00381-016-3293-x
Popis: Dandy-Walker malformation (DWM) is a congenital brain anomaly characterized by dysgenesis of the cerebellar vermis and the presence of a posterior fossa cyst. The association of syringomyelia with DWM is extremely rare. A 10-year-old patient who was diagnosed with DWM in infancy presented with progressive scoliosis and fecal incontinence. He had been treated with cystoventriculoperitoneal shunting with a Y-connection during infancy, which was followed by a revision 6 years later. During the revision surgery, intraventricular bleeding occurred and was managed conservatively. Imaging studies for the current visit revealed syringomyelia along the cervicothoracic spinal cord and a membranous structure around the cervicomedullary junction. Phase-contrast cine magnetic resonance imaging (MRI) revealed disturbed cerebrospinal fluid (CSF) flow across the membrane. We excised the arachnoid web that was tethering the brainstem and blocking CSF flow. Postoperatively, the patient experienced symptom relief, and the follow-up imaging study demonstrated a dramatic decrease in the size of the syringomyelia. We suggest that syrinx formation in this patient was possibly caused by disturbed CSF flow and tethering of the brainstem. We experienced an unusual case of DWM with syringomyelia which was caused by an arachnoid web blocking CSF flow and tethering the brainstem. The arachnoid web seems to be formed by previous bleeding which occurred at the time of shunt revision. After excision of the arachnoid web, the patient showed good outcome.
Databáze: OpenAIRE
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