Iniencephaly

Philipe Massoc MD, Fernando Heredia MD

Ginecology & Obstetrical Department. Hospital Guillermo Grant Benavente, Concepción — Chile.

Case report:

A 20- year-old patient, with a uneventful prior pregnancy, arrived to our center with an uncontrolled pregnancy and in labour. The baby was in breech presentation so we decided to perform a caesarian section. The baby girl weighting 2600 grams and measuring 33 cm had Apgar scores of 1-1-1-1. The baby died 1 hour after delivery of complications of Iniencephaly.

In the neonatal exam, the most prominent findings were:

  • Head was in fixed retroflection, with short and almost absent neck. A cervical paramedial mass (50 by 50 mm of diameter) was also observed.
  • The newborn’s spine was hyperextended to the back.

The autopsy findings included:

  • Cranium: larger posterior fossa with a two cm defect in right occipital area, and  herniation of the leptomeninges.
  • Nervous system: moderate dilatation of 4th ventricle.
  • Respiratory system: thoracic cage larger than normal with small lungs.
  • Cardiovascular system: very small left ventricle with high interventricular communication. Right-sided truncus arteriosus.
  • Gastrointestinal system: Slight enlargement of the liver.
  • Urinary Tract: Kidneys almost fused, with preserved aspect and size.

The fetus

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The X-ray of the fetus

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