Reddy J, Laufer MR
Congenital anomalies of the female reproductive tract in a patient with Goltz syndrome. [Journal Article]
J Pediatr Adolesc Gynecol 2009 Aug; 22(4):e71-2.
BACKGROUND: Goltz syndrome, or focal dermal hypoplasia, is a rare congenital mesoectodermal syndrome. It is characterized primarily by extensive cutaneous and musculoskeletal defects. CASE: We report on a 17-year-old menarchal girl diagnosed with a severe form of Goltz syndrome who was referred to the Gynecology Division at the Children's Hospital Boston. A complete evaluation revealed a septate hymen, a normal vagina with a single cervix, a bicornuate uterus, and a unilateral kidney. To our knowledge, there have been no documented cases of congenital anomalies of the female reproductive tract in patients with Goltz syndrome.
CONCLUSIONS: The development of the female reproductive tract is a complex process involving all three embryonic cell layers. Female patients with multiple abnormalities of ectodermal and mesodermal origin warrant complete evaluations of their reproductive tracts.
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