A FETUS WITH SIRENOMELIA, OMPHALOCELE, AND MENINGOMYELOCELE, BUT NORMAL KIDNEYS

Sirenomelia, which has been reported for more than 450 years, is the m ost severe form of caudal dysplasia. This disorder is classically asso ciated with agenesis or severe dysplasia of the urinary tract. A 27-ye ar-old gravida 3 para 0020 presented at 19 weeks' gestation with monoa mniotic twins, one normal and one with sirenomelia. The pregnancy misc arried following invasive diagnostic testing. Autopsy confirmed the fi ndings of sirenomelia, meningomyelocele, omphalocele, persistent cloac a, and two normal kidneys. The abnormalities accompanying sirenomelia appear as a spectrum. The pathogenic theories of sirenomelia are revie wed in light of this case with normal kidneys. (C) 1994 Wiley-Liss, In c.