Ovarian remnant syndrome

Title

Summary Summary

Ovarian remnant syndrome (ORS) is characterized by the presence of residual ovarian tissue after a woman has had surgery to remove one ovary or both ovaries (oophorectomy). Signs and symptoms may include pelvic pain, a pelvic mass, or the absence of menopause after oophorectomy. The condition may be caused by surgical factors leading to incomplete removal of ovarian tissue, including factors that limit surgical exposure of the ovary or compromise surgical technique. Factors may include pelvic adhesions (limiting ability to see the ovary or causing it to adhere to other tissues); anatomic variations; bleeding during surgery; or poor surgical technique. Treatment is indicated for people with symptoms and typically involves surgery to remove the residual tissue. Therapy for those who refuse surgery, cannot have surgery, or do not have a pelvic mass may include hormonal therapy to suppress ovarian function.^[1]

Last updated: 12/2/2016

Symptoms Symptoms

The most common symptom of ovarian remnant syndrome (ORS) is pelvic pain. Less common symptoms include a pelvic mass and/or the absence of menopausal symptoms after oophorectomy. Some people have symptoms consistent with endometriosis, including difficult or painful intercourse; urinary symptoms; or bowel symptoms. It is likely that some people with ORS don't have any symptoms, but the rate of this is unknown.^[1]

In most cases, symptoms occur within the first five years of the oophorectomy, although there are reports of ORS presenting 20 years after the initial surgery.^[1]

Last updated: 12/2/2016

Cause Cause

The risk of ovarian remnant syndrome (ORS) is increased by incomplete removal of the ovarian tissue at the time of oophorectomy. Surgical factors that contribute to incomplete removal include those that limit surgical exposure of the ovary, or compromise surgical technique. Factors may include:

Pelvic adhesions – these can limit visualization of the ovary and may also cause it to adhere to surrounding tissues. Adhesions are often present due to preexisting conditions and/or prior surgeries. In the majority of cases reported since 2007, endometriosis was the most common indication for the initial oophorectomy in patients who subsequently had ORS. Endometriosis increases the risk for functional ovarian tissue being embedded into adjacent structures, making complete excision of tissue challenging.
Anatomic variations - unusual location of ovarian tissue, for example
Intraoperative bleeding
Poor surgical technique – this may include failure to obtain adequate exposure or restore adequate anatomy, or imprecise choice of incision site^[1]

Last updated: 12/2/2016

Diagnosis Diagnosis

Ovarian remnant syndrome (ORS) may first be considered in women who have undergone oophorectomy and have suggestive symptoms, the presence of a pelvic mass, or evidence of persistent ovarian function (by symptoms or laboratory testing). A history of oophorectomy is required, by definition, to make the diagnosis. Notes regarding the indication for the procedure and the procedure itself should be reviewed and may include prior abdominal or pelvic surgery, endometriosis, and/or poor surgical visualization. If ORS is possible, pelvic ultrasound should be performed to evaluate for a pelvic mass.^[1]

Surgical exploration and biopsy of possible ovarian tissue is required for the definitive diagnosis of ORS, and treatment by excision of the remnant ovarian tissue may be performed during the same procedure. For women who are not candidates for surgery, a clinical diagnosis can be made based on the symptoms and hormone levels (follicle-stimulating hormone and estradiol, after bilateral oophorectomy) and/or imaging findings consistent with the presence of residual ovarian tissue.^[1]

Last updated: 12/2/2016

Treatment Treatment

Treatment for ovarian remnant syndrome (ORS) is generally indicated for women with suspected ORS who have symptoms (such as pelvic pain); have a pelvic mass; or need or desire complete removal of tissue to decrease the risk of ovarian cancer (for example, BRCA mutation carriers). The mainstay of treatment is surgery to remove the residual ovarian tissue. Women with ORS with a pelvic mass should have appropriate evaluation for malignancy (cancer). Hormonal therapy to suppress ovarian function is an alternative treatment for those who refuse surgery, or those who are not candidates for surgery.^[1]

Last updated: 12/2/2016

Statistics Statistics

The incidence of ovarian remnant syndrome is difficult to determine. The available data are limited to case reports or to retrospective case series. The best available data are from a study describing the frequency and outcome of laparoscopy in women with chronic pelvic pain and/or a pelvic mass who were found to have ovarian remnants.^[1] In 119 women who underwent hysterectomy and oophorectomy by laparoscopy, ovarian remnants were known in 5 and were found during surgery in 21 patients (18%).^[2] However, this was a small study and the participants were only symptomatic women.^[1] Therefore, it is not known whether the data can be extrapolated to include all women who have undergone oophorectomy.

Last updated: 12/2/2016

Do you have updated information on this disease? We want to hear from you.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

PubMed is a searchable database of medical literature and lists journal articles that discuss Ovarian remnant syndrome. Click on the link to view a sample search on this topic.

News & Events News & Events

News

NCATS Rare Diseases Are Not Rare! Challenge
October 9, 2018
The NIH Undiagnosed Diseases Network Expands
September 26, 2018

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

What are the statistics on getting ORS? I was told it was rare but how rare? I got diagnosed with it about a week ago. I am in excruciating pain with it. Can it rupture? It's at a little under tennis ball size. See answer
I can not find any help in regards to this disease. Since I was diagnosed with this I have been left to deal with it myself and its getting to the point of no return. So any help in getting me pointed in the right direction would be helpful. See answer

Have a question? Contact a GARD Information Specialist.

References References

Rosanne M Kho, Mauricio S Abrao. Ovarian remnant syndrome. UpToDate. Waltham, MA: UpToDate; October, 2016;
Basim Abu-Rafeh, George A. Vilos, Millie Misra. Frequency and Laparoscopic Management of Ovarian Remnant Syndrome. The Journal of the American Association of Gynecologic Laparoscopists. February, 2003; 10(1):33-37.

Do you know of a review article? We want to hear from you.

You can help advance
rare disease research! You can help advance rare disease research!