Endoscopic third ventriculostomy to treat hydrocephalus associated with macrocephaly-cutis marmorata telangiectatica congenita.
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.
Department of Clinical Neurosciences, Alberta Children's Hospital, University of Calgary, Alberta, Canada.
Alim P
AP
Mitha
Kelly J
KJ
Bullivant
Julie L
JL
Lauzon
Walter J
WJ
Hader
D000015:Abnormalities, Multiple / Q000175:diagnosis; D002508:Cephalometry; D002531:Cerebellum / Q000473:pathology; D018450:Disease Progression; D004677:Encephalocele / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D005260:Female; D006801:Humans; D006849:Hydrocephalus / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery*; D007223:Infant; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D013577:Syndrome; D013684:Telangiectasis / Q000151:congenital*; D020542:Third Ventricle / Q000601:surgery*; D014057:Tomography, X-Ray Computed; D014696:Ventriculostomy / Q000379:methods*