A case of cranio-facial dysostosis (Crouzon's disease) associated with hydrocephalus
Kawai, K.; Shigemori, M.; In, K.; Kuga, S.; Kuramoto, S.
No Shinkei Geka. Neurological Surgery 8(11): 1093-1099
1980
ISSN/ISBN: 0301-2603 PMID: 7453940 Document Number: 163508
A case of cranio-facial dysostosis (Crouzon's disease) associated with enlarged head due to hydrocephalus was reported with special reference to the mechanism of the hydrocephalus. A 2-year-old boy who complained of mental retardation was admitted on Dec 78' when choked disc and dilatation of the lateral ventricle shown on CT scan were also seen. Characteristic signs of hypertelorism, exophthalmos, hypoplastic maxilla and mandibular prognathism indicating cranio-facial dysostosis (Crouzon's disease) were noted on admission. There were no indicative findings of cranial sutures at all on plain skull film, which was seen on previous film at the age of 11 months. Craniometry on plain film indicated narrowed basal angle and shortening of both anterior and posterior cranial fossas. A moderate symmetrical dilatation of the lateral and third ventricle with normal ranged forth ventricle were shown on CT scan. RI cisternography using 111In was normal without ventricular reflex or subarachnoid block. Choked disc was gradually disappeared following shunting operation performed on Dec. 28, 1978. PVG demonstrated the stenosis and ventral displacement of the aqueduct at the site 7 mm from the outlet of the third ventricle. Poststenotic dilatation for 4 mm in width was also noted. These findings suggested that maldevelopment of the enchondral bone at the skull base in cranio-facial dysostosis produced bending of the aqueduct leading to obstructive hydrocephalus as seen in the case.