Abstract
Hydranencephaly is a rare congenital abnormality characterized by the absence and replacement of the cerebral hemispheres with cerebrospinal fluid. Here, we present an ultrasonographic diagnosis of a case of a rare variant of fetal hydranencephaly at 38 weeks of gestation. Obstetric sonography revealed the absence of the cerebral cortex, thalami and basal ganglia with a disrupted falx and preserved posterior fossa structures. This is the first reported case of hydranencephaly with the absence of the thalami and basal ganglia, which was diagnosed prenatally. The diagnosis was confirmed with postnatal computed tomography. The early prenatal diagnosis allowed for prompt obstetric attention at a tertiary care hospital which had specialized pediatric facilities including prenatal counseling and support.
Highlights
Is a rare congenital abnormality characterized by the absence and replacement of the cerebral hemispheres with cerebrospinal fluid
The ultrasonographic findings that we described, led to the diagnosis of hydranencephaly being the most likely outcome and this was confirmed by postnatal computed tomography (CT) of the fetal head
Remnant or preserved midbrain structures were observed in the most reported cases of hydranencephaly[28,29] with the thalamus or basal ganglia structures preserved in the majority of cases
Summary
Is a rare congenital abnormality characterized by the absence and replacement of the cerebral hemispheres with cerebrospinal fluid. Other etiologies of hydranencephaly include intrauterine infections, which leads to the local destruction of brain tissue e.g. congenital toxoplasmosis[10] or other viral infections (adenovirus, cytomegalovirus, enterovirus, Epstein-Barr virus, herpes simplex virus, parvovirus, and respiratory syncytial viruses)[3,6,11]. Another etiology that has arisen is the maternal exposure to carbon monoxide or butane gas, which can result in fetal hypoxia which in turn leads to massive tissue necrosis with cavitations, resorption of necrotized tissue and necrotizing vasculitis[3,12]. Has been associated with various congenital anomalies, including Fowler syndrome, arthrogryposis, renal aplastic dysplasia, poly-valvular heart defect, and trisomy[17,18]
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