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[Anencephaly and diprosopy: 2 cases].

H Journel, P Parent, H Leguern

    Journal De Gynecologie, Obstetrique Et Biologie De La Reproduction
    |January 1, 1986
    PubMed
    Summary
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    Two cases of diprosopy (facial duplication) linked to anencephaly were observed. This condition, a form of conjoint twinning, can disrupt neural tube closure, leading to birth defects like spina bifida.

    Area of Science:

    • Developmental Biology
    • Teratology
    • Medical Genetics

    Background:

    • Diprosopy is a rare congenital anomaly characterized by partial or total duplication of the face.
    • It arises from incomplete embryonic division, specifically of the cephalic neural plate between days 16-18, resulting in conjoint twins.
    • This condition is associated with other severe developmental abnormalities.

    Observation:

    • Two cases of diprosopy were documented in Brittany between 1975 and 1984.
    • One case presented as diprosopos distomos dirhinos diophthalmos, and the second as diprosopos distomos dirhinos triophthalmos.
    • Both cases were associated with anencephaly; the second also exhibited spina bifida and diaphragmatic hernia.

    Findings:

    • Diprosopy can disrupt subsequent embryological events, particularly neural tube development.

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  • Delayed nerve formation in diprosopy cases can lead to incomplete neural tube closure, explaining associated anencephaly and spina bifida.
  • Monochorial, monozygotic conjoint twins may interfere with early embryonic development, increasing risks of neural tube closure failure.

    • Implications:

    • Understanding the embryological basis of diprosopy is crucial for diagnosing and managing associated congenital anomalies.
    • This study highlights the link between twinning events and neural tube defects, suggesting a common pathway in early embryonic development.
    • Further research into conjoint twinning and its impact on neural tube closure could improve prenatal risk assessment and counseling.