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 characteriz ed by the persistence of Mullerian duct derivatives, intestinal lymphangiec tasia, hypertrophied alveolar ridges, and early death. Postmortem examinati on showed the presence of a rudimentary uterus, fallopian tubes, the upper third of a vagina, a prostate of normal shape, a dilated colon, and general ized intestinal and pulmonary lumphangiectasia. The syndrome was first deli neated by Urioste and co-workers [1993: Am J Med Genet 47:494-503]. These c ases confirm the existence of a definite and distinct entity. (C) 2001 Wile y-Liss, Inc.