Presumptive limb-body wall complex detected by ultrasonography

Author's Introduction:
Limb-body wall complex refers to a rare combination of disruptive and lethal abnormalities which start early in the gestational process. Abnormalities commonly associated with this disorder include cranio-facial abnormalities, scoliosis, ventral body wall defect (thoraco-abdominoschisis), limb deformations, short umbilical cord, and others. Other terms used to describe similar findings include short umbilical cord syndrome, body-stalk anomaly, and amniotic band syndrome. The causes of this disorder are not fully understood.

Although limb-body wall complex has been widely reported in the literature, the case that follows had several unusual features. First of all, it involved a twin pregnancy. Secondly, the abnormal twin (referred to as "twin B") progressed from oligohydramnios in early pregnancy to polyhydramnios. This complication resulted in severe compression of the sac of the normal twin (referred to as "twin A"), and a therapeutic amniocentesis was consequently performed. Twin A continued to develop normally, whereas twin B developed oligohydramnios once again, and subsequently died. As of this writing, the mother's pregnancy with twin A continues, and this surviving twin is progressing normally. Please review the following case and accompanying images.

It is my hope that this case will stimulate lively discussion and healthy debate. It will give all of us an opportunity to learn something new about these rare disorders.

-- Martin Necas, RDMS, RVTHistory:
A 39-year-old female presented to a local diagnostic center for an obstetrical dating scan prior to undergoing elective termination of her pregnancy. A living twin gestation of 12 weeks was identified with one twin suffering unspecified fetal abnormalities. The patient decided against termination of her pregnancy and was subsequently referred to our department for further ultrasonographic evaluation

Findings:
The first obstetrical ultrasound was performed at 15 weeks, with the following findings:

• Living dichorionic twin pregnancy

• Twin A: LT lateral location, average ultrasound age 14w5d, posterior placenta

• Abnormalities: Echogenic Intracardiac Focus (a.k.a. echogenic chordae tendinae), no other anatomic abnormality detected

• Twin B: RT lateral location, average ultrasound age 13w3d, anterior placenta

• Abnormalities: ventral body defect (thoraco-abdominoschisis), 90-degree lateral scoliosis of the lumbar spine, highly prominent nuchal region, ectopia cordis, two vessel cord, oligohydramnios.

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