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.
Abbreviations used in this paper: ETV = endoscopic third ventriculostomy; M-CMTC = macrocephaly-cutis marmorata telangiectatica congenita; VP = ventriculoperitoneal.
Address correspondence to: Walter J. Hader, M.D., Department of Clinical Neurosciences, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, Alberta T3B 6A8 Canada. email:
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