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Fetopathology and developmental pathology of the embryo and fetus
Marta Ježová, Josef Feit et al.
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Anencephaly
Clinical signs
  • severe malformation which occurs when the cephalic end of the neural tube fails to close and major parts of the brain and the cranial vault fail to develop
  • incidence is 1 : 1000 (therefore not very rare)
  • anencephaly can often be associated with rachischisis and other congenital defects
  • incompatible with survival
  • diagnosis is made by ultrasound: missing skull and polyhydramnion
Macroscopic appearance
  • absent cranial vault
  • the base of the skull is small with absent or decreased anterior and middle cranial fossa
  • the cranial base is covered by a vascular mass, so-called area cerebrovasculosa, structurally close to chorioid plexus, with remnants of immature nervous tissue; area cerebrovasculosa merges into skin at the edges.
  • eyes are prominent no optical nerves usually.
  • hypophysis is present, but usually hypoplastic

Anencephalus (72119)

Anencephalus (72120)

Pictures

Anencephalus: Anencephalus, Macro, autopsy (72119) Anencephalus, Macro, autopsy (72120) Anencephalus, Macro, autopsy (72121)

Anencephalus, amniotic bands: Anencephalus, amniotic bands, Macro, autopsy (73621)

Anencephalus: Anencephalus, Macro, autopsy (73626) Anencephalus, Macro, autopsy (73627)

Anencephalus: Anencephalus, Macro, autopsy (73628) Anencephalus, Macro, autopsy (73629) Anencephalus, Macro, autopsy (73630) Anencephalus, Macro, autopsy (73631)

Anencephalus: Anencephalus, Macro, autopsy (73632) Anencephalus, Macro, autopsy (73633) Anencephalus, Macro, autopsy (73634) Anencephalus, Macro, autopsy (73635)

Anencephalus: Anencephalus, Macro, autopsy (73966)

Histology

Area cerebrovasculosa consists of dilated blood vessels and variable amount of immature nervous tissue, glial in particular.

Pictures

Area cerebrovasculosa: Area neurovasculosa, HE 20x (73002)