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Disease Profile

Ovarian remnant syndrome

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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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]

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]

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]

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]

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]

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.

References

  1. Rosanne M Kho, Mauricio S Abrao. Ovarian remnant syndrome. UpToDate. Waltham, MA: UpToDate; October, 2016;
  2. 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.

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