Connatal (fetal) hydrocephalus: an acquired pathology?

Abstract

We investigated the etiology of hydrocephalus present at birth, i.e. of fetal hydrocephalus. Both inherited and dysraphic major malformations are very rare. Intraventricular hemorrhages and viral infections during pregnancy are among the proposed etiologies; they are supported more by anatomical, physiopathological and experimental findings than by clinical evidence. Cases of fetal intraventricular hemorrhages cited in the literature are anecdotical, and the reports fail to identify maternal or fetal predisposing factors. The role of viruses in the etiology of connatal hydrocephalus has been postulated on the basis of epidemiological considerations in human pathology and of a considerable amount of experimental studies in animals. Investigations were generally focused on aqueduct ependyma, but research should also address other structures involved in the genesis of hydrocephalus (choroid plexus, extraventricular CSF pathways, including arachnoid villi). Furthermore, experimental evidence has emerged concerning a number of toxins and of drugs administered during pregnancy, which are thought to be involved in the genesis of hydrocephalus: once more, the conclusions reached in these experimental trials lend further credence to the human epidemiological data linking pregnancy disturbances with fetal hydrocephalus. Since most of these toxic agents are also thought to induce major malformations, we could assume the degree of their effect to depend on the embryonal stage affected: the earlier the action, the worse the malformation.

Follow-up studies of newborn-babies with congenital ventriculomegaly

“…These did not match the cases mentioned by Guiffré et al [12]. These did not match the cases mentioned by Guiffré et al [12].…”

“…From 10 patients born by induction of delivery in 7 newborns early ventriculo-peritoneal shunts were implanted (patients No 1,8,9,10,12,19,30). Similarly there was no difference between the outcome of preterm and term newborns.…”

Cerebral Ventricular Asymmetry and Ventriculomegaly Interact to Increase Risk for Schizophrenia: A Case Report and Recommendation for Routine Fetal Sonography

“…3,11 Relative to the more commonly occurring intraventricular hemorrhages and viral infections during pregnancy, genetic etiologies of fetal hydrocephalus are rare. 12 Causes of post-hemorrhagic hydrocephalus include maternal hypertension, trauma in pregnancy, and coagulation disorders. However, the true incidence of coagulation disorders appears to be low.…”

“…Also, twin pregnancies are associated with an increased incidence of fetal hydrocephalus. 12 Many viral candidates have an ependymal tropism that can lead to aqueductal atresia and, possibly, hydrocephalus due to viral-related involvement of the choroid plexuses and arachnoid villi. 12 Although genetic factors may not be prominent in terms of etiology, a higher than expected percentage of malformations was reported in first-degree relatives of children with congenital hydrocephalus.…”

A Male Fetus with Aqueductal Stenosis and Four Accessory Spleens

“…However, the causes leading to FH are still a matter for debate. Recently, FH has been linked with acquired pathology during pregnancy [4]; the most frequent cause was considered to be viral infection of the mother [4], We report here a case of FH affecting a male fetus presenting with an associated visceral malforma tion, namely the presence of 4 accessory spleens. We con sider this association as a marker for a developmental defect which can be explained by common regulatory pathways involved in the control of both splenic and brain stem development.…”