Limb body wall complex

Fabrice Cuillier, MD

2004-08-16-08 Limb-Body wall complex © Cuillier www.thefetus.net/


Dept of Obstetrics and Gynecology, H√īpital F√©lix Guyon, rue des Topazes, 97400 Saint-Denis, Reunion Island, France. Ph : 0262 90 55 22.

This is a 26 year old woman scanned at 12 weeks of gestation. A transvaginal scan demonstrated a live fetus with an omphalocele, measuring 2 X 2 cm, protruding from the anterior abdominal wall and a meningocele.The bladder was not visualized, but amniotic bands were present and the lower limbs were malpositioned.

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Three-dimensional trans-vaginal ultrasound clearly demonstrated the omphalocele as a solid mass protruding from the ventral wall and the lower limb behind.

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Initially, the parents refused interruption, but after genetic consultation and amniocentesis they chose to terminate the pregnancy. The fetus was delivered vaginally. A mid line spherical mass which contained liver and bowel protruded from the anterior abdominal wall of the fetus and the lower limbs were deformed. An autopsy confirmed LBWC without cranio-facial defect. Unfortunately, the rest of the pelvis was destructed during delivery. There were broad adhesive bands overlying the omphalocele. The karyotype was 46 XX.

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The Limb Body Wall Complex is a rare polymalformative syndrome. Two phenotypes have been described: one form with placento-cranial adhesion and the other with placento-abdominal adhesion. Omphaloceles are found in all cases, and variably coexists with encephalic, vertebral, visceral and limb anomalies. This syndrome should be better known and it has a good prognosis for a later pregnancy because there is no reccurence.

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