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Endometriosis Overview




Author: Shawn Daly, MD, Consulting Staff, Catalina Radiology, Tucson, Arizona

Shawn Daly is a member of the following medical societies: American College of Radiology, American Medical Association, American Society of Neuroradiology, and Radiological Society of North America

Coauthor(s): Eric Outwater, MD, Professor, Department of Radiology, University of Arizona

Editors: Christopher L Sistrom, MD, Associate Chair for Research, Assistant Professor, Department of Radiology, University of Florida School of Medicine; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; Karen L Reuter, MD, FACR, Professor, Department of Radiology, Lahey Clinic Medical Center; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; Eugene C Lin, MD, Consulting Staff, Department of Radiology, Virginia Mason Medical Center

Author and Editor Disclosure

Synonyms and related keywords: endometriosis, endometrioma, chocolate cyst

Background

Endometriosis is defined as the presence of endometrial glandular tissue outside of the uterus. In contrast, adenomyosis is endometrial tissue within the myometrium. Adenomyosis once was termed endometriosis interna but currently is recognized as a distinct clinical entity.

Two main theories exist for the pathogenesis of endometriosis. One theory is that endometrial tissue is spread by retrograde menstruation or by vascular and/or lymphatic spread. The second theory holds that the serosal epithelium of the peritoneum undergoes metaplastic differentiation into endometrium-like tissue.

The theory that retrograde menstruation causes endometriosis is supported by the analysis of peritoneal fluid in women. As many as 90% of women have blood in the peritoneal fluid during the perimenstrual period. In addition, endometrial cells have been found in the peritoneal fluid. The pattern of endometriosis is consistent with retrograde menstruation and is most common in the ovary, followed by the other dependent areas of the pelvis. Vascular and/or lymphatic spread is supported by noting the occasional distal (extraperitoneal) sites of endometriosis, including the lungs and central nervous system (CNS). In addition, teenage girls with obstructive uterine or vaginal anomalies show retrograde menstrual bleeding, and endometriosis is common in these patients.

Metaplasia is the conversion of peritoneal epithelium into endometrial epithelium. The theory that metaplasia causes endometriosis is supported by the fact that endometrial cells and peritoneal cells derive from the same celomic wall epithelium. This theory also is supported by development of endometriosis in women who lack normal endometrial tissue (ie, in Turner syndrome and uterine agenesis). In addition, rare cases of endometriosis have been found in the prostatic utricle of men. The prostatic utricle is a mullerian remnant.

Clinical presentations of endometriosis often consist of chronic pelvic pain and infertility; however, many patients are asymptomatic.

For excellent patient education resources, visit eMedicine's Women's Health Center. Also, see eMedicine's patient education articles Female Sexual Problems and Endometriosis.

Pathophysiology

The pathophysiologic hallmark of endometriosis is the presence of peritoneal implants of ectopic endometrium. The histologic diagnosis of endometriosis is confirmed when 2 of the following 3 features are identified:

  • Endometrial glands
  • Stroma
  • Hemosiderin pigment

The most frequent site of endometriosis is the ovary. Other frequent locations include the following:

  • Uterine ligaments
  • Pelvic cul-de-sac (pouch of Douglas)
  • Pelvic peritoneum
  • Fallopian tubes
  • Rectosigmoid
  • Bladder

Complications of endometriosis consist of bowel and ureteral obstruction resulting from pelvic adhesions. Rarely, endometriosis is extraperitoneal and occurs in the lungs and CNS.

Ectopic endometrial glandular tissue is influenced by ovarian hormones and undergoes cyclic bleeding. The earliest visible manifestations of endometriosis are whitish peritoneal plaques. The foci of endometrial tissue are small subserosal nodules with a brown appearance on gross examination; termed powder burns, they are seen on laparoscopic examination. Over time, the repeated hemorrhaging can produce extensive fibrosis surrounding the endometrial tissue, which can result in adhesions to adnexal structures or to bowel and can obliterate the pelvic cul-de-sac.

When the ovaries are involved, they can become enlarged with cystic, blood-filled spaces that, on gross examination, are termed chocolate cysts, or endometriomas. Endometriomas can become large and multilocular. The endometrial-cell lining can become obliterated over time, making the pathologic distinction between an endometrioma and a hemorrhagic cyst difficult in some cases.

Frequency

United States

Estimates of the frequency in the general population of menstrual-aged females are variable, depending on the population source. This variability may have resulted from sampling bias, since most women with pathologically proven endometriosis who have been included in such statistics have sought evaluation because of pelvic pain and/or fertility problems. A large-scale laparoscopic evaluation of asymptomatic women has never been performed.

In 1 study, 20% of women undergoing laparoscopic evaluation for infertility and 24% of women with pelvic pain had endometriosis. Overall prevalence in symptomatic and asymptomatic women has been estimated to be 1-10%. A hereditary component may be a factor, because an increased prevalence is found among first-degree relatives.

Mortality/Morbidity

The primary morbidity associated with endometriosis relates to chronic pelvic pain and infertility.

The cause of infertility is probably related to tubal dysfunction secondary to endometrial implants. This may be due to fibrosis and to adhesions distorting the tube, but other factors, such as the production of cytokines, may contribute. The severity of symptoms is not closely related to the extent of endometriosis. Mortality is negligible.

Endometriosis in atypical sites can present with various comorbid clinical symptoms. Endometriosis involving the gastrointestinal (GI) tract usually involves the dependent bowel. The endometrial implants begin in the serosa but can erode into the muscularis and cause bowel obstruction or rectal bleeding. More commonly, endometriosis adhesions or endometriomas can obstruct the ureters and cause hydronephrosis. In the rare patient with intrathoracic endometriosis, pneumothorax associated with menses may occur.

Race

No statistically significant race predilection exists for endometriosis.

Sex

Endometriosis occurs primarily in women, with only rare case reports existing of clinically insignificant occurrences of the disease in men.

Age

Endometriosis occurs primarily in women during their reproductive years, most commonly in women aged 25-29 years. Extraperitoneal endometriosis typically occurs in a slightly older population. Endometriosis in women younger than 20 years often is secondary to obstructive anomalies of the uterus or vagina. Endometriosis usually regresses substantially after menopause.

Anatomy

Endometriosis most commonly affects the dependent portions of the pelvis. The most frequent site is the ovary, and the next most frequent locations include the uterine ligaments, pelvic cul-de-sac, pelvic peritoneum, fallopian tubes, rectosigmoid, and bladder. Rarely, endometriosis can be extraperitoneal, affecting the lungs and CNS.

Staging of endometriosis depends on the degree and complications of endometrial implants. The 1985 Revised Classification of Endometriosis, created by the American Society for Reproductive Medicine, evaluated the characteristics of endometrial implants, such as location and the depth of penetration, as well as the degree of cul-de-sac obliteration and adhesions. The findings on laparoscopy can be used to classify patients into 4 classes, from mild (stage I) to severe (stage IV). The staging correlates with the likelihood of achieving pregnancy but not with the severity of pain.

Clinical Details

Endometriosis affects many women during their reproductive years. The most common symptoms for which women seek medical attention include pelvic pain and infertility. Clinical history and physical examination often do not correlate with the extent of the disease.

Usually, physical examination demonstrates nonspecific pelvic tenderness, although occasionally, tender nodular masses can be palpated in the adnexa or the posterior cul-de-sac. Adhesions can obliterate the cul-de-sac, which correlates with the finding of a frozen pelvis on physical examination. Cystic masses of endometriosis (endometriomas) may present as palpable adnexal masses.

Endometriosis can cause ureteral obstruction and hydronephrosis. This results from endometrial implants on the distal ureter or from mass effect from endometriomas.

It should always be considered that a woman with pelvic pain and/or fertility problems may have more than 1 disease process, such as chronic pelvic inflammatory disease or an adnexal neoplasm.

Young women with endometriosis may have a uterine obstruction and/or malformation as the underlying cause.

Preferred Examination

The initial workup begins with a history and physical examination. These can be nonspecific, but occasionally, tender, nodular masses can be palpated on pelvic examination, representing fibrotic implants in the cul-de-sac.

Usually, the initial imaging examination for suspected endometriosis is pelvic ultrasound (US) scanning. Magnetic resonance imaging (MRI) provides superior anatomic detail and better defines abnormalities found using ultrasonography.

Laparoscopy is the standard modality for the diagnosis of endometriosis. It is the most sensitive means of examination because only laparoscopy can identify superficial peritoneal implants; however, laparoscopy is an invasive surgical procedure. Laparoscopy can be incorporated into treatments in which endometriomas are cauterized or removed and adhesions are lysed.

Limitations of Techniques

Plain film radiography, computed tomography (CT) scanning, and barium studies are not sensitive for the diagnosis of endometriosis. Moreover, the appearance of implants and endometriomas is nonspecific.

US scanning and MRI are not sensitive for superficial lesions. US scanning is not sensitive for the detection of large implants.

Laparoscopy is limited because it is invasive. In addition, laparoscopy can be limited by the presence of dense pelvic adhesions, resulting in limited access to the cul-de-sac and adnexa.



Ectopic Pregnancy
Ovarian Torsion
Ovary, Malignant Tumors
Pelvic Inflammatory Disease/Tubo-ovarian Abscess

Other Problems to Be Considered

History and physical examination findings

Pelvic infection
Ovarian cyst
Ovarian torsion
Ovarian neoplasm
Ectopic pregnancy

MRI and US scan findings

Cystic neoplasms
Functional cysts
Hemorrhagic cysts
Cystadenomas



Findings

Plain film radiographs are not indicated in the radiologic workup of endometriosis. There are no specific findings on plain films. The findings on contrast studies (a barium enema and an intravenous [IV] urogram) are nonspecific.

Uncommonly, women with rectal pain or bleeding from involvement of the bowel with endometriosis may undergo a barium enema examination. A tethered submucosal mass that is centered at the anterior midrectumrepresenting cul-de-sac implants involving the rectal wallis typical.

An IV urogram in patients with endometriosis may show ureteral obstruction at or below the pelvic brim. This obstruction may be from an endometriosis implant invasion into the ureter or from a mass effect from endometrioma.

Degree of Confidence

The appearance of endometriosis on a barium enema contrast study is nonspecific and can be mimicked by rectal carcinoma or serosal metastases. Similarly, the appearance of the ureteral obstruction on urography is nonspecific.



Findings

CT scanning typically is not performed in the radiologic evaluation of endometriosis because the appearance of endometriosis and endometriomas on CT scans is nonspecific. If CT scanning is performed, endometriomas appear as cystic masses. A slightly high attenuation crescent lying dependently within the cyst has been described as a more specific feature. Complications of endometriosis, such as bowel obstruction, are evident on CT scans. Ureteral obstruction may cause hydronephrosis.

Degree of Confidence

The appearance of endometriomas and endometriosis on CT scans is nonspecific and is easily mimicked by pelvic inflammatory disease, as well as by benign or malignant ovarian tumors. CT scanning should not be relied on for the diagnosis.



Findings

  • The appearance of endometriomas on magnetic resonance images is variable and depends on the concentration of iron and protein in the fluid, products of blood degradation. Most endometriomas have the gross appearance of chocolate cysts, representing highly concentrated blood products. MRI demonstrates these endometriomas as cystic masses with very high signal intensity on T1-weighted images and very low signal intensity on T2-weighted images (see Images 5-7). This low signal intensity on the T2-weighted images is termed shading and occasionally occurs in a gradient from higher to lower signal intensity. This pattern of signal intensities results from the high iron concentration in the endometrioma and is rarely seen in other masses of any type.
  • Multiple high-signal lesions, usually in the ovaries, on T1-weighted images also are highly suggestive of endometriosis.
  • Peritoneal implants initially are small serosal lesions and usually escape detection. Larger, fibrotic implants of endometriosis are seen on magnetic resonance images as spiculated nodules of very low signal intensity on T2-weighted images. These commonly occur in the cul-de-sac; they less commonly appear on the bladder dome, rectum, or umbilicus or in pelvic surgical scars. Dilated fallopian tubes occasionally are seen on magnetic resonance images in patients with endometriosis; these demonstrate high signal intensity on T1-weighted images, indicative of bloody fluid.
  • MRI can also demonstrate the complications of endometriosis. Bowel obstructions and hydronephrosis occasionally can be seen. Since longer imaging times are required for MRI, antiperistaltic medication can improve visualization of bowel.

Degree of Confidence

In 1 study, MRI showed a sensitivity of 90-92% and a specificity of 91-98% for the diagnosis of endometrioma in patients with adnexal masses.1 Thus, MRI is an accurate technique in distinguishing endometriomas from other masses.

MRI is not sensitive for superficial implants; therefore, the modality should not be relied on to rule out endometriosis.

False Positives/Negatives

False-negative examinations usually are seen in patients with only peritoneal implants. These are generally too small to resolve with MRI or any noninvasive imaging technique.

False positives can occur because cystic neoplasms or functional cysts rarely can mimic endometriomas. Most hemorrhagic cysts and neoplasms do not show the degree of T1 and T2 shortening shown by endometriomas because the concentration of iron even in whole blood is much less that endometriomas.



Findings

Patients with suspected endometriosis referred for US scanning evaluation should receive a transvaginal study, because this is more sensitive for smaller endometriomas. The kidneys should be examined for hydronephrosis.

The typical US scan finding in endometriosis is a cystic mass with diffuse, low-level echoes (see Image 1, Image 3). However, endometriomas can vary in appearance (for example, they may appear cystic [simple or complex], or they may resemble a solid mass) (see Image 4). Punctate echogenicities in the wall of endometriomas are less commonly seen but add specificity to the diagnosis.

Small implants typically are not seen with US scanning.

Doppler waveform analysis is not helpful in differentiating endometriomas from other masses. Low-resistance waveforms resembling malignancy are encountered in endometriomas (see Image 2).

Degree of Confidence

US scanning is not as specific as MRI in the evaluation of endometriosis. The appearance of a cystic mass with homogeneous, diffuse, low-level echoes is highly suggestive of an endometrioma. However, other appearances are much less specific and can be mimicked by hemorrhagic cysts, tubo-ovarian abscesses, and cystadenomas. US scan characteristics of endometriomas overlap with other pathologies, such as ovarian neoplasms. Endometriomas are commonly bilateral or multicystic, furthering their resemblance to malignancies.

Since small endometrial implants are not seen reliably on US scans, US scanning is not a sensitive technique to diagnose endometriosis.

Guerriero and colleagues found that the sensitivity and specificity of endovaginal ultrasonography were 83% and 89%, respectively, when the technique was used to differentiate endometriomas from other ovarian cysts.2

Low-resistance Doppler waveforms resembling malignancy are encountered in endometriomas.

False Positives/Negatives

False-positive findings may occur because hemorrhagic cysts, tubo-ovarian abscess, and cystadenomas may resemble endometriomas.

False-negative findings can occur because endometrial implants are too small to visualize on US scans. In addition, endometriomas without the typical appearance described above may be misdiagnosed as hemorrhagic cysts, tubo-ovarian abscesses, and cystadenomas.



Findings

Nuclear medicine is not indicated in the radiologic evaluation of endometriosis.



Findings

Angiography is not indicated in the radiologic evaluation of endometriosis.



Treatment for symptomatic endometriosis can be medical or surgical.

Nonsteroidal anti-inflammatory drugs (NSAIDs) are frequently used first in patients with pelvic pain, particularly if the diagnosis of endometriosis has not been definitively established.

The goal of directed medical treatment is to achieve an anovulatory state. This is typically achieved initially using oral contraceptives. This can also be accomplished with a progestational agent (ie, medroxyprogesterone); with danazol, gestrinone, or gonadotropin-releasing hormone agonists (GnRH); or with other less well-known agents. These agents are generally used if oral contraceptives and NSAIDs are ineffective in controlling symptoms. GnRH can be combined with estrogen and progestogen (add-back therapy) without loss of efficacy but with fewer hypoestrogenic symptoms. No evidence exists that any 1 of these agents is most effective in controlling pain; moreover, there is no strong evidence that these drugs improve fertility.

Laparoscopic surgical approaches to endometriosis include the ablation of implants, the lysis of adhesions, and the removal of endometriomas, as well as uterosacral nerve ablation and presacral neurectomy. Conservative surgery can be performed to preserve fertility in young patients. Randomized clinical trials have shown evidence that laparoscopic surgery provides pain relief and improved fertility over diagnostic laparoscopy without surgery. Definitive surgery consists of a hysterectomy and a bilateral oophorectomy.



Media file 1:  Endovaginal ultrasound scan of an endometrioma. Note the characteristic diffuse, low-level echoes of the endometrioma (E) giving a solid appearance.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Image

Media file 2:  Duplex ultrasound scan of an endometrioma. Note the resistive index of 0.48, indicating a low-resistance waveform. This value can also be seen with an ovarian neoplasm, yielding false-positive results.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Image

Media file 3:  Sagittal view shows an endometrioma (e) in the cul-de-sac with diffuse, low-level echoes. The endometrioma lies directly posterior to the uterus (u).
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Media type:  Image

Media file 4:  Transverse view of a left ovarian endometrioma shows a heterogeneous appearance, with diffuse, low-level echoes interspersed with echogenic and anechoic areas.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Image

Media file 5:  T1-weighted magnetic resonance image of an endometrioma. Note the characteristic high signal intensity (similar to that of fat) of this right-sided adnexal endometrioma (arrow).
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI

Media file 6:  Fat-saturated T1-weighted magnetic resonance image of an endometrioma. In this right adnexal endometrioma (same lesion as in Image 5), fat saturation has been applied. Note that the endometrioma's (arrow) signal intensity does not decrease. This signal characteristic differentiates endometriomas from fatty adnexal masses, such as dermoids.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI

Media file 7:  T2-weighted magnetic resonance image of an adnexal endometrioma (arrow; same lesion as in Images 5-6). Note the characteristic low T2-weighted signal. This low T2 signal is a result of the high iron concentration in the endometrioma.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI



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Endometrioma/Endometriosis excerpt

Article Last Updated: Aug 16, 2007