Limb body wall complex

Last revised by Daniel J Bell on 8 Nov 2018

The limb-body wall complex (LBWC) is a rare variable group of congenital limb and body wall defects (involving mainly the chest and abdomen). They can include:

The estimated incidence is at around 1 in 10-14,000 live births.

The etiology is controversial; proposed mechanisms include:

  • early amnion rupture
  • early vascular disruption
  • embryologic malformation with abnormal development of the body folds

Internal organs are frequently affected with a high incidence of cardiac and diaphragmatic defects, bowel atresia, renal agenesis, and hydronephrosis.

  • maternal cocaine abuse (probable) 8-9

May show a large abdominal wall defect with the fetus being adherent to the placenta. The umbilical cord insertion site is challenging to find or is absent, and there is direct apposition of the membranous sac to the amniochorionic membrane. An accompanying kyphoscoliosis/scoliosis may also be detected on ultrasound. Early first-trimester scanning may also show an increased nuchal translucency 10.

The condition carries an extremely poor prognosis and is invariably fatal 3,4. Management is often supportive. Future pregnancies are however not thought to carry an increased risk of redeveloping the condition.

General imaging considerations include:

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Cases and figures

  • Case 1
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