Sudden unexpected death in young adults with chronic hydrocephalus

Abstract

We present four cases of sudden unexpected death in young adults with chronic hydrocephalus. The patients were between 20 and 28 years of age and had suffered from aqueduct stenosis (two patients), spina bifida in combination with Arnold-Chiari malformation (type II) and fragile X-syndrome. The patients suddenly collapsed with cardiorespiratory failure and could not be resuscitated and none had a history of headache or seizures. The post-mortem examinations revealed no unusual findings and a definite cause of death could not be established. Neuropathological examination revealed chronically hydrocephalic brains without any signs of uncal or tonsillar herniation. We hypothesise that a sudden pressure-induced decompensation of cerebral neuronal pathways involving insular and limbic cortex, hypothalamus and brain stem nuclei, may have caused disturbances of the cardiopulmonary control centres in the reticular formation of the brain stem, which in turn may have led to instantaneous cardiorespiratory arrest resulting in sudden "neurogenic" cardiac death.

Perioperative Management of Children With Encephalocele

“…18 Although fluid within the encephalocele sac is essentially content of "third space fluid shift" and not in direct continuation with the systemic circulation, a sudden decompression of the ventricular system causes traction of cerebral neuronal pathways involving brainstem nuclei, which may lead to instantaneous cardiopulmonary arrest. 19 Thus, controlled drainage of CSF is always advocated. A reduction of sac or reintroduction of the brain tissue contained in the sac back into cranial cavity can induce subsequent increase in ICP even in children with enlarged cranial vault.…”

Chronic Hydrocephalus and Alcohol Abuse in a Young Male Suicide

“…10 -15%), or postinfection (9%). 19,22 The discrepancy between production and elimination of CSF causes an increase in volume, with consequent acute or chronic hydrocephalus. 3,5 Chronic hydrocephalus leads to an increase in intraventricular pressure, causing ventricular expansion and dislocation of adjacent cerebral structures.…”

“…In 30%-50% of children with hydrocephalus, the IQ is normal. 22 Intellectual deficits occur with increasing intracranial pressure and reduced thickness of the cerebral cortex (IQ of under 80 with cortex thickness under 2 cm; normal IQ with thickness above 2.8 cm 18 ). The IQ has been related with the type of hydrocephalus, 28 size of ventricles, thickness of cerebral cortex before and after surgery, 29 shunt 30 and any complications, and degree of myelinization.…”

Neuropathological Findings in a Child with Slit Ventricle Syndrome

“…The cause of the respiratory arrest could have been medullary compression by the descending cerebellar tonsils, although diffuse neurologic dysfunction in the presence of elevated intracranial pressure without tonsillar herniation has been postulated in some circumstances [9]. Unfortunately, this clinical scenario is repeated about once a year in our hospital jurisdiction, which serves a sizeable number of remote communities.…”