Hydranencephaly with extensive periventricular necrosis and numerous ectopic glioneuronal nests

Abstract

The case reported here relates to a male infant with hydranencephaly who was born at 37 weeks of gestation and died immediately after birth. Post-mortem examination revealed that the cerebral hemispheres had been replaced by fluid-filled cavities within a normal-sized cranium. The membranous hemispheric wall was composed of gliosed tissue with multiple foci of necrosis and hemosiderin-laden macrophages. The ependyma was absent. Many necrotic foci containing hemosiderin were also found around the aqueduct and fourth ventricle. These findings suggest that hemorrhagic necrosis had occurred throughout the periventricular region, and more severely in the cerebral hemispheres. Moreover, numerous glioneuronal nests were found throughout the subarachnoid space and ventricles. Glioneuronal nests, if present, are usually minimal in hydranencephaly, whereas it is one of the pathological features of multicystic encephalopathy. The transition of multicystic encephalopathy to hydranencephaly has been demonstrated repeatedly. The former is a condition resulting from a severe circulatory disturbance, most often at the end of gestation or in the perinatal period. These lesions date later than hydranencephaly. Considering that numerous glioneuronal nests were found in the present case, it is likely that the encephaloclastic process developed toward the end of gestation.

An autopsy case of congenital hydrocephalus and severe thinning of the cerebral cortex in a 4‐year‐old boy

“…Generally, the destruction of the cerebrum is more prominent with that of the brainstem and cerebellum, with only a small part of the cerebrum being preserved. 1 Most of the reports on autopsy findings of hydrocephalus [2][3][4][5][6][7][8] indicate marked hypoplasia of the cerebrum and various abnormalities in the brainstem and cerebellum (Table 1). Neuropathological changes were more severe in the brainstem and cerebellum in cases of short survival than in those of long survival.…”

“…Most of the reports on autopsy findings of hydrocephalus 2–8 indicate marked hypoplasia of the cerebrum and various abnormalities in the brainstem and cerebellum (Table 1). Neuropathological changes were more severe in the brainstem and cerebellum in cases of short survival than in those of long survival.…”

“…Halsay et al 7 2 years 3 months/-Atrophic posterior temporal lobe and parieto-occipital lobes, normal thalamus Normal brain stem, basal ganglia and cerebellum; atrophy of the pyramidal tracts in the spinal cord 8 Hoffman and Liss 2 7 years 6 months/F Atrophic cerebrum, only the occipital and temporal lobes, insular gyri and hippocampus preserved Normal brain stem, cerebellum and spinal cord, except for the absence of pyramidal tracts in the medulla…”

Hidranencefalia

“…Dentro de las múltiples causas que conducen a la lesión isquémica de las carótidas internas, se describen a las infecciones virales como las mas implicadas, entre ellas al herpes virus, parvovirus, citomegalovirus, orbi virus, bunyavirus, virus de la peste, toxoplasmosis congénita 10 y otros; otra posible etiología son los procesos tóxicos (alcohol) 3, 11 o genéticos (síndrome de Fowler, enfermedad de Border, síndrome de PEHO, etc.) 12,13,14 .…”

“…Se encuentra necrosis de las placas germinales del sistema nervioso central e infiltración de células inflamatorias, especialmente macrófagos 15 . La prevalencia de la hidranencefalia depende del tipo de agente y del estado inmunitario materno 13 . El tiempo de sobrevida en ésta patología, depende del compromiso anatómico y de la preservación de las conexiones entre ellas.…”