Persistence of Mullerian derivatives and intestinal lymphangiectasis in two newborn brothers:: Confirmation of the Urioste syndrome

We describe two newborn brothers with a pattern of malformation characterized by the persistence of Mullerian duct derivatives, intestinal lymphangiectasia, hypertrophied alveolar ridges, and early death. Postmortem examination showed the presence of a rudimentary uterus, fallopian tubes, the upper third of a vagina, a prostate of normal shape, a dilated colon, and generalized intestinal and pulmonary lumphangiectasia. The syndrome was first delineated by Urioste and co-workers [1993: Am J Med Genet 47:494-503]. These cases confirm the existence of a definite and distinct entity. (C) 2001 Wiley-Liss, Inc.