kongenitaler Hydrozephalus

Fetal

hydrocephalus • Fetal hydrocephalus - Ganzer Fall bei Radiopaedia

Abdrabou, A. Fetal hydrocephalus. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-25225 • CC by-nc-sa 3.0 (modified)

Fetal

hydrocephalus • Fetal lumbar meningocele with hydrocephalus - Ganzer Fall bei Radiopaedia

Babu, V. Fetal lumbar meningocele with hydrocephalus. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-51862 • CC by-nc-sa 3.0 (modified)

Fetal

hydrocephalus • Fetal cardiac failure - acardiac twin - monochorial pregnancy - Ganzer Fall bei Radiopaedia

Ho, F. Fetal cardiac failure - acardiac twin - monochorial pregnancy. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-57858 • CC by-nc-sa 3.0 (modified)

Fetal

hydrocephalus • Craniofacial malformations (antenatal ultrasound) - Ganzer Fall bei Radiopaedia

El-Feky, M. Craniofacial malformations (antenatal ultrasound). Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-92604 • CC by-nc-sa 3.0 (modified)

Autosomal

recessive polycystic kidney disease • Autosomal recessive polycystic kidney disease associated with severe hydrocephalus - Ganzer Fall bei Radiopaedia

Abbas, A. Autosomal recessive polycystic kidney disease associated with severe hydrocephalus. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-79351 • CC by-nc-sa 3.0 (modified)

Radiopaedia • CC-by-nc-sa 3.0 • de

Fetal hydrocephalus often refers to an extension of fetal ventriculomegaly where the ventricular dilatation is more severe. It is usually defined when the fetal lateral ventricular diameter is greater than 15 mm .

Epidemiology

The estimated incidence is 0.5-3% per 1000 live births. There may be a very slight increased female predilection .

Pathology

It can be either obstructive or non-obstructive and each can arise from a number of etiologies. In a small proportion of cases, it carries a familial X-linked inheritance (congenital X linked hydrocephalus).

Causes

in utero infection(s)

Associations

The vast majority of conditions are associated with other intracranial and cranial anomalies . The list includes:

central nervous system anomalies: (more common and reported in more than 80% of cases)
- aqueductal stenosis: one of the commonest causative associations
- Chiari malformations
- neural tube defect(s)
- Dandy-Walker malformation
- encephalocoele
- alobar holoprosencephaly
- posterior fossa cysts
- polymicrogyria
non-central nervous system anomalies
- craniofacial
  - cleft lip +/- palate
  - low set ears
  - bilateral optic atrophy
  - facial bone anomalies
  - acrocephalosyndactylia
- congenital cardiovascular anomalies
- gastrointestinal anomalies
- genitourinary anomalies
  - congenital renal fusion
- skeletal anomalies
  - clubfeet
syndromes
- Meckel-Gruber syndrome
- Miller-Dieker syndrome
chromosomal anomalies: may be present in ~20% of cases
- trisomy 21
- triploidy

Radiographic features

Antenatal ultrasound

Will demonstrate enlarged ventricles with variable degrees of parenchymal thinning. The choroid may be seen floating within the ventricle giving a dangling choroid sign. Often a separation of more than 3 mm between the choroid plexus and the margin of the ventricle is considered abnormal. In some cases, there may also be evidence of macrocephaly.

Treatment and prognosis

The overall prognosis will depend on the underlying cause and associated anomalies. Some cases can slowly progress during the fetal period. Antenatal shunting has been considered in a small proportion of selected cases .