Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + At a Glance Download Section PDF Listen ++ A very rare syndrome combining ocular and neurologic signs. + Synonym Download Section PDF Listen ++ Hydrocephalus Cataract Microphthalmos Syndrome. + Incidence and Genetic Inheritance Download Section PDF Listen ++ Only a single case has been described. Autosomal recessive inheritance has been suggested because the parents were consanguineous. + Clinical Aspects Download Section PDF Listen ++ Bilateral cataracts, microcornea, microphthalmos, hydrocephaly, high vaulted palate, generalized hypertonia and hyperreflexia have been described in a 9-month-old girl. + Anesthetic Considerations Download Section PDF Listen ++ Anesthesia in this condition has not been described. Hydrocephalus is most likely associated with raised intracranial pressure. In this case, anesthesia should focus on normal ventilation or mild hyperventilation, preventing seizures and hyperthermia, and using a (preferably intravenous) anesthesia technique not resulting in increased cerebral blood flow. + References Download Section PDF Listen ++Cennamo G, Gangemi M, Magli A: Hydrocephalus combined with congenital cataract and microphthalmia. J Pediatr Ophthalmol Strabismus 16:382, 1979. [PubMed: 521881]