Abstract
A 16-day-old girl was admitted to our hospital with a history of feeding difficuty, convulsions and cranial enlargement, and died at the age of 4 months. Autopsy disclosed hydranencephaly accompanied by subdural effusion and hematoma.
She was born at term but resuscitation was necessary. Shortly after birth, she developed feeding difficulty, convulsion, hypertonicity and hypothermia. On admission, she was apparently jaundiced and her head was enlarged with bulging anterior fontanel. Skull x-rays revealed widening of cranial sutures. CT scan at the age of 41 days disclosed only low density area in the supratentorial region. Transillumination of the head, cerebral angiography and electroencephalography agreed with the diagnosis of hydranencephaly. Her symptoms were alleviated after ventriculo-peritoneal shunting but she died of acute subdural hematoma complicated with systemic infection.
Postmortem examination revealed bilateral subdural effusion and hematoma. Both cerebral hemispheres were replaced by the fluid-filled membranous sac. The wall of the membranous sac has a continuity with the remnants of limbic and temporal lobes, basal ganglia, thalamus and midbrain. Aqueductal stenosis was present and the pyramids absent. Microscopically, the wall of the membranous sac was composed of leptomeninges and glial tissue. Multiple sections of the membranous sac disclosed the absence of ependymal lining on the inner surface of the sac. Both internal carotid arteries were very narrow and anterior, middle and posterior cerebral arteries were also hypolastic.
Pathogenesis of hydranencephaly and differential diagnosis from congenital hydrocephalus were discussed. Clinical implication of subdural effusion (hematoma) in the present case was also stated.
