A rare variation of hydranencephaly: case report

Abstract

Hydranencephaly is a rare severe abnormality characterized by replacement of cerebral hemisphere with fluid covered by leptomeninges such that absent cerebral cortex. We present the Ultrasonographic diagnosis of a case of fetal Hydranencephaly at 38 weeks of gestation. Sonography revealed the absence of cerebral cortex, thalami and basal ganglia with disrupted falx and preserved posterior fossa structures. This was the first reported case of Hydranencephaly with absence thalami and basal ganglia along with midbrain. Confirmation of diagnosis was made with postnatal computed tomography. Even though it was diagnosed in the late third trimester, it allowed prompt and finest obstetric management which reduced the risk for both fetus and mother. Furthermore it allowed arrangement of optimal conditions for birth at a unit with accessible specialized pediatric facilities.

Hydranencephaly: A rare case report

“…It is an encephaloclastic abnormality. The midbrain, thalamus, basal ganglia may be preserved, and the flax cerebri is usually present within the skull [3].…”

“…An examination of baby monkey brains revealed HE due to vascular shutdown predominantly when carried out during earlier gestational age. Some case reports suggest that the occlusion of the ICAs may not be direct as temporary spasm can also lead to ischemic destruction of brain structures [3].…”

“…The exact etiology is not known. The most common cause is found to be occlusion of the supra-clinoid part of the internal carotid artery (ICA), which leads to infarction of cerebral structures [3]. So far, no sex or racial preference has been discovered [1].…”

Unveiling what is absent within: illustrating anesthetic considerations in a patient with hydranencephaly – a case report

“…Prenatal diagnosis has historically been lacking, though increasingly improved with the advent of routine fetal ultrasound [2]. Approximately 1% of infants diagnosed with congenital hydrocephalus actually have hydranencephaly; differentiation between these two diagnoses is critical as congenital hydrocephalus has a significantly better prognosis [1]. However, with the quality of medical care and surgical interventions now available, life expectancy in hydranencephaly can extend once a child survives past their first 2 years of life [6].…”

“…Hydranencephaly is a rare congenital condition that occurs in less than 1 in 10,000 births in which brain development is severely restricted [1]. Cerebral structures are often limited to brainstem and thalamus only, with cerebral spinal fluid (CSF) filling a membranous sac normally inhabited by cerebral cortex [2,3].…”