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At a glance

A disorder combining ocular and neurologic signs.

Synonym

Hydrocephalus Cataract Microphthalmos Syndrome.

Incidence

Only a single case has been described.

Genetic inheritance

Autosomal recessive inheritance has been suggested because the parents were consanguineous.

Diagnosis

Based on the clinical findings.

Clinical aspects

Hydrocephalus with bilateral microphthalmia, microcornea, cataracts, high-vaulted palate, generalized hypertonia, and hyperreflexia have been described in a 9-month-old girl.

Precautions before anesthesia

Check for signs of increased intracranial pressure. Otherwise, routine preoperative assessment should be sufficient. Decreased vision in a child can cause agitation and stress in the perioperative period, thus anxiolytic and sedative premedication and/or presence of a parent (or primary caregiver) for induction of anesthesia may be beneficial.

Anesthetic considerations

Anesthesia in this condition has not been described. The anesthetic management should take increased intracranial pressure (ICP) into consideration and aim at maintaining an appropriate cerebral perfusion pressure while at the same time avoiding further increases in ICP, prevent seizures, and hyperthermia.

Pharmacological implications

Use a (preferably intravenous) anesthesia technique not resulting in increased cerebral blood flow.

Reference

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Cennamo  G, Gangemi  M, Magli  A: Hydrocephalus combined with congenital cataract and microphthalmia. J Pediatr Ophthalmol Strabismus 16(6):382–385, 1979.  [PubMed: 521881]

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