Macrocephaly-cutis marmorata telangiectatica congenita is a rare overgrowth syndrome commonly associated with hydrocephalus. Although the pathophysiological characteristics of the hydrocephalus in this syndrome is not fully known, previous reports have described its treatment with ventriculoperitoneal shunt placement. The authors describe 2 cases of macrocephaly-cutis marmorata telangiectatica congenita successfully treated for progressive hydrocephalus with endoscopic third ventriculostomy. Both patients experienced clinical and radiographic stabilization following treatment, and these findings offer insight into the pathophysiology of the hydrocephalus and its ideal management.
Alim P. Mitha, Kelly J. Bullivant, Julie L. Lauzon and Walter J. Hader
Kelly J. Bullivant, Alim P. Mitha and Mark G. Hamilton
The PS Medical Strata valve is a programmable shunt valve used in the treatment of hydrocephalus that allows for noninvasive changes in the pressure setting using a magnet. The Strata valve is sensitive to magnetic fields, and reprogramming is frequently necessary after MR imaging. A known but rare complication of the Strata valve is that the rotor can become locked, causing shunt malfunction. This complication can only occur in a first generation Strata valve.