*   Radiologist , UltraCare , Coimbatore, India;
**  Obstetrician, Surya Hospital, Coimbatore, India.

Definition

Iniencephaly by definition is a complex neural tube defect, characterized by accentuated retroflexion of  the head, varying degree of defect in the occiput and upper spine, fusion of the skin of the face and neck and nearly absent neck. The defect of occiput can vary from acrania to minimal defect.

Case report

We present a case of a 25 year-old G2P1 referred to our unit for a targeted scan at 27 weeks. The fetus presented with acrania combined with extreme degree of rachischisis of cervical and dorsal vertebra. Associated pleural effusion with pulmonary hypoplasia was also present. Here are some of the ultrasonographic and pathological images we obtained.

Images 1 and 2: 27 weeks. Image 1 shows axial image of the skull aligned through the orbits and brain tissue protruding dorsally out of skull. Image 2 shows pleural effusion and pulmonary hypoplasia of the fetus.

Images 3 and 4: 27 weeks. 3D images showing the head of the fetus kept in retroflection with dorsally protruding brain tissue.

Images 5: 27 weeks;  Image 5 shows the head of the fetus kept in retroflection with dorsally protruding brain tissue.

Image 6 shows coronal view of the fetal spine with cervical rachischisis.

Images 7 and 8: Pathological images.

Images 9 and 10: Pathological images.