National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Swyer syndrome

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I was born without ovaries. I have everything else that a normal woman has but I have streaks where ovaries would be. I was diagnosed when I was 15. My doctor put me on triphasil and soon after I started showing signs of puberty. None of my doctors seem to know about this condition. I am now 31. I am wondering if I should opt to have my streaks removed and if I should be on some other form of estrogen. I have very little breast development.

The following information may help to address your question:


How might Swyer syndrome be treated?

The treatment of a person with Swyer syndrome may depend on the specific characteristics that each person has. Some people need surgery to repair the external genitalia and to create and/or enlarge the vagina. Hormone replacement therapy (HRT) is typically needed from puberty onward and usually includes estrogen and progesterone.[1] In addition to helping with normal development of secondary sexual characteristics, HRT can help prevent bone loss and thinning (osteoporosis) later in life. Abdominal dysgenetic gonads (testes or ovaries with abnormal development) or streak gonads, which are common in people with Swyer syndrome, are at increased risk for gonadal tumors such as gonadoblastoma and should be surgically removed. Although women with Swyer syndrome are infertile, they may become pregnant and carry to term through egg donation.[1]
Last updated: 11/16/2015

Should streak gonads be removed in individuals with Swyer syndrome?

It is typically recommended that individuals with streak gonads have them surgically removed. Individuals with streak gonads are at an increased risk for gonadal tumors.[5128]
Last updated: 3/30/2012

We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please contact us.

Warm regards,
GARD Information Specialist

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  1. Harry Ostrer. 46,XY Disorder of Sex Development and 46,XY Complete Gonadal Dysgenesis. GeneReviews. September 15, 2009; http://www.ncbi.nlm.nih.gov/books/NBK1547/. Accessed 3/30/2012.