Prevalence:

• 1 in 10,000 pregnancies.
• More often in a younger age group and cocaine abuser.

Ultrasound diagnosis:

• Presence of a major abdominal wall defect, severe kyphoscoliosis and short or absent umbilical cord. Typically, the liver is directly attached to the placenta without interposed umbilical cord and there is major distortion of the spine.

Associated abnormalities:

• The incidence of chromosomal abnormalities and genetic syndroms is not increased.
• Exencephaly or encephalocoele, facial cleft, and limb amputations are common.

Investigations:

• Detailed ultrasound examination.

Follow up:

• If the pregnancy continues then follow-up should be standard.

Delivery:

• Routine obstetric care and delivery.

Prognosis:

• Lethal condition either in utero or in early neonatal period.

Recurrence:

• No increased risk of recurrence.