A very rare syndrome combining ocular and neurologic
Hydrocephalus Cataract Microphthalmos Syndrome.
Only a single case has been
described. Autosomal recessive inheritance has been suggested because the
parents were consanguineous.
Bilateral cataracts, microcornea, microphthalmos,
hydrocephaly, high vaulted palate, generalized hypertonia and hyperreflexia
have been described in a 9-month-old girl.
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