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Exencephaly

2005-05-12-9 Heron Werner, MD*, Pedro Daltro, MD*, Dorothy Bulas, MD# * Clínica de Diagnóstico por Imagem (CDPI) & Instituto Fernandes Figueira (IFF) – FIOCRUZ – Rio de Janeiro – Brazil
# Professor of Radiology and Pediatrics – Children"s National Medical Center – George Washington University Medical Center – 111 Michigan Ave, NW, Washington D.C. 20010

Exencephaly is a rare and fatal anomaly in which the bones of the cranial vault are absent with protrusion of brain tissue into the amniotic cavity. The origin is due to the failure of the closing of the neural tube during the fourth week of embryonic development. It can be detected early in gestation, when the normal calcification of the cranial bones is not present. Later in gestation, the most common finding is the presence of a large quantity of disorganized brain tissue. It can be associated with other anomalies such as amniotic bands, omphalocele and pentalogy of Cantrell.

References

Pretorius DH et al.: Tracheoesophageal fistula in utero. Twenty-two cases. J Ultrasound Med 6: 509-13, 1987.

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