Bilateral absence of the ovaries and distal fallopian tubes. A case report.
BACKGROUND
Bilateral tuboovarian absence is extremely rare and is associated with infantile sexual development, primary amenorrhea and primary infertility.


METHODS
A 23-year-old woman presented for evaluation of primary amenorrhea. Her examination revealed hypoplastic breasts, genitalia and uterus; ovaries could not be identified. Marked estrogen deficiency was confirmed by endocrinologic testing. The karyotype was normal female. The patient was started on combined hormone replacement therapy and subsequently developed normal menses; physical maturation progressed normally. At the age of 29 she underwent diagnostic laparoscopy for evaluation of her fertility potential, at which time the absence of both ovaries and distal fallopian tubes was confirmed.


CONCLUSIONS
Bilateral tuboovarian absence is an extremely rare cause of primary amenorrhea and is associated with infantile sexual development and primary infertility. Its etiology includes tuboovarian torsion and congenital malformation. In this case, congenital malformation appears to have been the more likely cause.
Gold
M A
MA
Department of Obstetrics and Gynecology, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, USA.
Schmidt
R R
RR
Parks
N
N
Traum
R E
RE
D000328:Adult; D000568:Amenorrhea / Q000188:drug therapy / Q000209:etiology*; D015914:Estrogen Replacement Therapy; D005187:Fallopian Tubes / Q000002:abnormalities*; D005260:Female; D006801:Humans; D010535:Laparoscopy; D010053:Ovary / Q000002:abnormalities*