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A very rare syndrome combining ocular and neurologic signs.

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Hydrocephalus Cataract Microphthalmos Syndrome.

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Only a single case has been described. Autosomal recessive inheritance has been suggested because the parents were consanguineous.

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Bilateral cataracts, microcornea, microphthalmos, hydrocephaly, high vaulted palate, generalized hypertonia and hyperreflexia have been described in a 9-month-old girl.

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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.

Cennamo G, Gangemi M, Magli A: Hydrocephalus combined with congenital cataract and microphthalmia. J Pediatr Ophthalmol Strabismus 16:382, 1979.  [PubMed: 521881]

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