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Volumetric analysis of cerebrospinal fluid and brain parenchyma in patient with hydranencephaly and macrocephaly

Hydranencephaly is a rare disorder where almost all cerebral hemispheres are absent and replaced by cerebrospinal fluid (CSF) often accompanied with extreme increase of head size. We present intracranial volumetric analysis of residual brain parenchyma and CSF in 29 years old women with hydranencephaly and macrocephaly. Head scan was performed using 3T magnetic resonance device and manual segmentation of brain parenchyma and CSF was made separately for supratentorial and infratentorial space using Analyze 8.0 software. Total volume of intracranial space was je 3645, 44 cm³. In supratentorial space volume of CSF was 3375, 19 cm³ while brain parenchyma was reduced to only 88, 69 cm³. In infratentorial space volume of CSF was 101, 29 cm³ and volume of brain parenchyma was 88, 69 cm³. Analysis of structural MR images reveal that in supratentorial space there is no visible remnants of choroidal plexuses and only parts of diencephalon, left temporal lobe and occipital lobes are spared. Infratentorial structures of brain steam and cerebellum are hypoplastic but completely developed. From our volumetric data it is obvious that hydranencephaly is primary consequence of increased CSF volume in supratentorial space. Mechanism of development of macrocephaly assumes presence of hypertensive hydrocephalus before closing of the sutures of the cranial bones. In case of our patient classical hypothesis of CSF pathophysiology in which hypertensive hydrocephalus is interpreted by disorder CSF secretion, circulation or absorption. Taking into account that in our patient there are no visible supratentorial plexuses or obstruction between CSF and dural sinuses, some other pathophysiological mechanisms than proposed by classical hypothesis are more likely responsible for this impressive clinical presentation of hydranencephaly with macrocephaly.

volumetric analysis ; hydranencephaly ; macrocephaly ; cerebrospinal fluid

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