Prevalence:

• 1 in 1,200 births.

Ultrasound diagnosis:

• Spectrum of features involving extremities, craniofacial region and trunk, which may be isolated or appear in combination. Demonstration of bands in not necessary for the diagnosis of amniotic band syndrome.
  • Extremities: absent digits or portions of limbs, swollen distal arm or leg resulting from constrictive amniotic bands.
  • Craniofacial region: facial cleft, asymmetric microphthalmia, severe nasal deformity, encephalocele.
  • Trunk: severe spinal deformities, large abdominal wall defects with herniated intestine. The most extreme manifestation is body stalk anomaly.

Associated abnormalities:

• The incidence of chromosomal abnormalities or genetic syndromes is not increased.

Investigations:

• Detailed ultrasound examination.

Follow up:

• Ultrasound scans every 2-3 weeks to assess the evolving consequences of the bands.

Fetal therapy:

• In a few cases fetoscopic lysis of the bands has been used in cases of constriction of the umbilical cord or threatened limb amputations.

Delivery:

• Place: hospital with neonatal intensive care and facilities for pediatric surgery.

Prognosis:

• Prognosis and treatment depends on the nature of amniotic band syndrome and the severity of deformation. Facial deformities often require extensive reconstructive procedures.
• Body stalk anomaly is lethal.

Recurrence:

• No increased risk of recurrence.