Long-term follow-up of successful treatment for dandy-walker syndrome (DWS).

BACKGROUND

Various managements of Dandy-Walker syndrome (DWS) from open excision of the cysts to CSF diversion have been reported. However, optimal treatment for DWS remains elusive.

METHODS

Cyst fenestration was employed firstly, but failed 15 days after the surgery. Then a ventriculoperitoneal (VP) shunt was inserted and the patient discharged from hospital 13 days after the second surgery. During the 12-year follow-up, CT scanning, MRI, and X-radiation were performed.

RESULTS

CT scanning showed that the size of ventricular system gradually returned to normal. MRI revealed similar changes of the ventricular system, and further revealed the development of supratentorial brain. The results of X-radiation during the follow-up years showed that the peritoneal end of the shunt might be already out of the abdominal cavity. Slow refilling of the shunt valve may suggest that the shunt could be probably not functional.

CONCLUSION

The treatment of the patient was successfully, and the abnormal cerebral ventricle system gradually recovered during the 12-year follow-up. Because the patient might be probably independent on the shunt, further choice of the shunt removal should be thinking thoroughly, considering both safety and quality of life for the patient.