Congenital anomalies of the female reproductive tract in a patient with Goltz syndrome.
BACKGROUND
Goltz syndrome, or focal dermal hypoplasia, is a rare congenital mesoectodermal syndrome. It is characterized primarily by extensive cutaneous and musculoskeletal defects.


METHODS
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.
Reddy
Jhansi
J
Division of Gynecology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts, USA.
Laufer
Marc R
MR
D000293:Adolescent; D005260:Female; D005489:Focal Dermal Hypoplasia / Q000150:complications*; D006801:Humans; D006924:Hymen / Q000002:abnormalities*; D007668:Kidney / Q000002:abnormalities*; D014599:Uterus / Q000002:abnormalities*