Endometriosis with Ovarian Endometrioma Formation Case File

Endometriosis with Ovarian Endometrioma Formation Case File

Eugene C. Toy, MD, Konrad P. Harms, MD, Keith O. Reeves, MD, Cristo Papasakelariou, MD, FACOG

Case 13

A 32-year-old G0P0 woman complains of painful menses during the last year as well as pelvic nonmenstrual pain and dyspareunia of recent onset. Although she stated that her menarche was at age 13 and her menses were painless and regular, have recently become somewhat irregular. She denies vaginal discharge or prior sexually transmitted disease. Also, she has stopped using any contraceptive method since being married. Her blood pressure is 110/70 mm Hg, heart rate 85 beats/min, and temperature 95°F. Heart and lung examinations are normal. On palpation, she had slight abdominal tenderness at the right iliac fossa, but without guarding. On pelvic examination, a retroverted and displaced uterus seems to move together with a palpable cystic mass of her right adnexa. Her pregnancy test is negative. Her hemoglobulin level is 11 g/dL, leukocyte count 8500/mm3, and platelet count 230,000/mm3. Ultrasound examination reveals a smooth 9-cm homogenic hypoechoic cystic mass of the right ovary attached to the posterior surface of a retroverted uterus with a small quantity of free hypodense fluid in the pouch of Douglas.

➤ What is the most likely diagnosis?

➤ What is your next step?

ANSWERS TO CASE 13:

Endometriosis with Ovarian Endometrioma Formation

Summary: This is a 32-year-old nulliparous woman with a history of recent onset of dysmenorrhoea, dyspareunia, nonmenstrual pelvic pain, and perhaps infertility, because she has never become pregnant despite her unprotected sexual intercourse. Her past gynecological and medical history were unremarkable. Complete blood count is within the normal range. Pregnancy test is negative. The right adnexa have a cystic mass on palpation and is attached to the retroverted uterus.

➤ Most likely diagnosis: Endometriosis with ovarian endometrioma formation

➤ Next step: Laparoscopy

ANALYSIS

Objectives

1. Know the symptoms of endometriosis.
2. Understand the mechanism of the symptoms.
3. Differential diagnosis of endometriomas from other adnexal cystic masses in reproductive aged women.

Considerations

The history of this 32-year-old woman is highly suggestive of endometriosis with concurrent endometrioma formation because dysmenorrhea begins after years of pain-free menses and is gradually accompanied by nonmenstrual pain, dyspareunia, and possible infertility. Proposed mechanisms that explain the pain in patients with endometriosis are the alteration of peritoneal environment due to inflammatory reaction of pelvic peritoneum associated with increased concentration of macrophages, prostaglandins, angiogenesis-promoting substances, and cytokines in the peritoneal fluid. This inflammatory response results in adhesion formation as a sort of healing process, fibrotic thickening of the invaded organs, and collection of shed menstrual blood in endometriotic implants with subsequent painful traction with the physiological movements of tissues. So, in this case the retroverted displacement and attachment of the uterus to the right adnexal mass in conjunction with the absence of previous laparotomies, as well as the negative history of previous pelvic inflammatory disease or vaginal discharge are additional arguments in favor of endometriosis with endometrioma formation. In addition, adhesion formation due to endometriosis appears in the advanced stage of disease and chronic pelvic pain appears later as it progresses. However, either the hostile peritoneal environment and/or the anatomical changes of pelvic structures are often associated with ovarian dysfunction and/or tubal obstruction due to adhesions. These factors, in relation to the use of no contraceptive method, could possibly justify the nulliparity of this woman. Furthermore, ruptured corpus luteum cyst is excluded because this condition is clinically characterized by sudden onset of severe abdominal pain with various degrees of hemoperitoneum. Moreover, the diagnosis of borderline or invasive adnexal mass is quite remote in this case due to the reproductive age of this woman, the protective role of oral contraceptives use in the past, and the ultrasound findings, which are not pathognomonic but strongly indicative of the endometriotic feature of this cyst. Of course, a negative pregnancy test is mandatory in every woman of reproductive age with pelvic pain, adnexal mass, and free fluid in Douglas pouch to exclude ectopic pregnancy. In contrast to postmenopausal women, CA-125 tumor marker cannot provide additional information to ultrasonographic findings as a diagnostic tool for endometriomas. But CA-125 measurements could be useful to detect the recurrence of endometriosis during the posttreatment follow-up period. Finally, laparoscopy has been established as the “gold standard” for definitive visual diagnosis and staging of endometriosis with simultaneously histological confirmation of uncertain lesions and exclusion of rare instances of malignancy.

APPROACH TO

Endometriosis With Ovarian Endometrioma Formation

DEFINITIONS

ENDOMETRIOSIS: The presence of functional endometrial glands and stroma outside the uterine cavity that responds to cyclical changes of ovarian steroid hormones. According to epidemiological, surgical, and pathological data, it manifests as peritoneal, ovarian, and deep endometriosis.

PERITONEAL OR SUPERFICIAL ENDOMETRIOSIS: It appears as typical red, black, and white superficial endometrial implants on serosal surface of organs or peritoneum according to the degree of cellular activity and fibrosis. According to its location, it is distinguished in pelvic and extrapelvic endometriosis.

OVARIAN ENDOMETRIOSIS OR ENDOMETRIOMA: It is defined as a pseudocyst formation, with ectopic endometriotic lining within the ovary after invagination of the ovarian cortex. Implantation theory and metaplasia of coelomic epithelium covering the ovary or secondary involvement of functional ovarian cysts (induction theory) have been proposed as possible pathogenetic mechanisms.

DEEP RECTOVAGINAL ENDOMETRIOSIS: It is defined as endometriotic nodular or polypoid mass invading the retroperitoneal space, more than 5 mm underneath the peritoneum. This lesion consists essentially of dense fibrotic tissue, hyperplasia of smooth muscle with active endometrial glands, and scanty stroma. Metaplasia of müllerian remnants into endometriotic glands often involves the rectovaginal septum or secondary infiltration of the pouch of Douglas by peritoneal endometriotic implants.

ADENOMYOSIS: It is defined as the presence of endometrial glands and stroma deep within the myometrium. It is considered as a separate pathological entity as it affects a different population of women and has a different etiology.

CLINICAL APPROACH

Although endometriosis is the second most common gynecological condition, its exact cause and pathogenesis are still controversial. It is estimated that the prevalence of endometriosis in the general population is 7% to 10% with a peak incidence between 35 and 40 years and is associated with pelvic pain and infertility. In women with infertility or pelvic pain, various incidences have been reported, ranging between 20% and 90%. This discrepancy is justified because a significant proportion of affected women are asymptomatic.1

Nulliparity, heavy and long menstrual flow, or short cycle length are considered risk factors for retrograde menstruation. A relative risk of 7.2 is found among first-degree relatives as it is inherited in polygenic-multifactorial manner.1,2

Extrapelvic endometriosis should be suspected when symptoms of pain or a palpable mass outside the pelvis present a cyclical pattern. Pelvic pain and infertility raise the suspicion for underlying endometriosis, but those symptoms alone do not establish the diagnosis, because there are other gynecological, urological, and gastroenterological conditions that can cause the same symptoms. As a result, there is often a delay in diagnosis of 8 to 12 years. There is no relation between the severity of symptoms and the location or stage of disease. Rectovaginal examination during menstruation is sometimes informative of uterosacral or rectovaginal painful nodules, and lesions of posterior vaginal fornix are visible during speculum inspection.3

Transvaginal and transrectal ultrasounds are useful noninvasive diagnostic tools for the diagnosis of ovarian endometriomas and deep infiltrating retroperitoneal nodules but not conclusive. Round-shaped homogenous cyst with low-level echoes, thick capsule with pericystic flow at the level of the ovarian hilus, and scattered vascularity are considered typical sonographic features of endometriomas that differentiated them from corpus luteum or other cysts. Elevated CA-125 may be indicative of advanced stage or recurrence but has no value as a diagnostic tool. MRI and barium enema studies are useful for mapping the extent of deep endometriosis when there is strong clinical suspicion.2,3

Laparoscopy is the “gold standard” for the diagnosis and treatment of endometriosis. It facilitates direct visual inspection of endometriotic lesions under magnification and determines the stage of disease, the type, the site, and extent of all lesions and adhesions according to the American Society of Reproductive Medicine classification system (r-AFS). Superficial peritoneal lesions typically have blue-black powder burn appearance and represent advanced, active lesions. Subtle lesions include red early active implants (petechial, vesicular, polypoid, hemorrhagic, red flame-like) and serous or clear vesicles. Moreover, white plaques or scarring, yellow-brown discoloration of the peritoneum, and subovarian adhesions occur in healed and inactive lesions. Neoangiogenesis is observed in active lesions. Laparoscopic features of ovarian endometrioma include (1) ovarian cyst not greater than 12 cm in diameter, (2) adhesions to the pelvic side wall and/or the posterior broad ligament, (3) burns and minute red or blue spots adjacent puckering on the surface, and (4) tarry, thick, chocolate-colored fluid content. Biopsies are recommended only from suspicious areas in peritoneal disease, and should be obtained in all cases of ovarian endometriomas and in deep infiltrating nodules to exclude malignancy. Negative histology is possible in 24% of cases due to the limited experience of the surgeon or pathologist to recognize subtle or atypical endometriotic lesions.4-8

The rationale for treatment of endometriosis is to treat symptoms, to remove the lesions, and to prevent recurrences. Considering that endometriosis is a chronic inflammatory disease, NSAIDs are effective in reducing endometriosis-associated pain. In addition, as an oestrogen-dependent disease, suppression of ovarian function or a pseudopregnancy status is induced by oral contraceptives, danazol, gonadotropin-releasing hormone agonists (GnRH-a), progestins, and medroxyprogesterone acetate. All of them are equally effective in pain reduction but with different side effects.5

Laparoscopic treatment of endometriosis can be either conservative as in most cases preservation of reproductive function is desirable or radical procedures such as oophorectomy or total abdominal hysterectomy with bilateral salpingo-oophorectomy in refractory severe cases, in which postoperative hormone replacement therapy (HRT) regimen with progestin is necessary.

Laparoscopic excision, electrocoagulation, or laser vaporization are comparable alternatives in the treatment of superficial-peritoneal or serosal endometriosis and should be treated at the same time during diagnostic laparoscopy. However, laparoscopic cystectomy of endometriomas (Figure 13–1), in terms of symptoms recurrence and pregnancy rate, seems to be the method of choice in comparison to laparoscopic electrocoagulation or laser vaporization of the inner cyst lining after one-step procedure or after drainage followed by administration of 12 weeks of GnRH-a in the three-step procedure. However, there is great concern about the impact of excisional methods on ovarian reserve and function due to inadvertent damage to healthy ovarian tissue. Interdisciplinary approach by laparotomy or laparoscopy is applied for excision of rectovaginal

Figure 13–1. Right ovarian endometrioma is seen. Note the adhesions also of the uterus to the cul-de-sac. (Courtesy of Dr.Cristo Papasakelariou.)

or rectosigmoidal nodules, but it can be associated with bowel, ureteral perforations, and peritonitis in 2% to 3% of cases though treated by experts. Preoperative hormonal treatment does not improve the success or ease of surgery. Postoperative medical treatment only with GnRH-a for 3 to 6 months achieved greater pain relief scores and fewer recurrences, but it is not recommended in patients with infertility because it prevents pregnancy. Concerning endometriosis-associated fertility, laparoscopic treatment especially in advanced stages improves pregnancy rates, and the highest spontaneous pregnancy rates occur during the first 6 to 12 months after conservative surgery.7-9

Comprehension Questions

13.1 A 34-year-old woman is noted to have chronic pelvic pain. The gynecologist has evaluated the patient and on the basis of history does not believe that this patient’s symptoms are due to endometriosis. Which of the following is present in this patient that would be inconsistent with endometriosis?

A. Parity

B. Short cycles length

C. Early menarche

D. Heavy menstruation

13.2 In a nulliparous woman, unilateral endometrioma should not be treated by

A. Oophorectomy

B. One-step procedure: drainage and electrocoagulation/laser vaporization at the same time

C. Three-step procedure: drainage, GnRH-agonist for 3 months, and then laser vaporization

D. Cystectomy

13.3 Which of the following statements regarding endometriosis is most accurate?

A. Deep endometriosis is typically a straightforward diagnosis by laparoscopy.

B. Endometriosis-related ovarian cancer usually affects older, multiparous women.

C. There is no clear justification to treat minimal endometriosis in women with infertility.

D. Endometriosis once treated surgically has a low risk of recurrence provided all the lesions were excised.

ANSWERS

13.1 A. Parity is inversely related to the risk of endometriosis, whereas the other findings are found with endometriosis.

13.2 D. Oophorectomy is contraindicated in women of reproductive age if there is no malignancy. However, the best conservative laparoscopic approach in nulliparous women is a controversial issue.

13.3 C. There is no proven benefit to treating minimal endometriosis in women with infertility. Deep endometriosis is often not a peritoneal disease and may be difficult to diagnose. Endometriosis associated ovarian cancer typically affects younger, nulliparous women and is usually well differentiated with a good prognosis. Endometriosis has a high recurrence risk approaching 40% in 5 years post therapy.

Clinical Pearls

See Table 1-2 for definition of level of evidence and strength of recommendation

➤ Peritoneal, ovarian, and deep endometriotic lesions constitute different manifestations of a single disease (Level B).

➤ Nulliparity,heavy menstruation, short cycle length, and early menarche are considered risk factors for endometriosis (Level B).

➤ The best means to diagnose peritoneal endometriosis is by direct laparoscopic visualization with histological confirmation where uncertainty persists (Level A).

➤ Transvaginal ultrasound is the preferred noninvasive diagnostic tool for confirming endometriomas, and MRI can be useful for diagnosing the presence and extent of deep endometriosis (Level B).

➤ The medical treatment based on induction amenorrhoea to prevent cyclical changes and menstruation and the choice of drug are determined by side effects and cost (Level B).

➤ Laparoscopy is indicated in all cases with ovarian endometriomas and rectovaginal adenomyotic nodules (Level C).

REFERENCES

1. D’Hooghe T, Hill J. Endometriosis. In: Berek J, ed. Berek and Novak’s Gynaecology. 14th ed. Philadelphia, PA: Lippincott Williams &Wilkins; 2007:1137-1182. 

2. Kennedy S, Bergqvist A, Chapron C, et al. on behalf of the ESHRE Specialist Interest Group for Endometriosis and Endometrium Guideline Development Group. ESHRE guideline for the diagnosis and treatment of endometriosis. Hum Reprod. 2005;20(10):2698-2704. 

3. Shaw R. Endometriosis. In: Shaw R, Soutter P, Stanton S, eds. Gynaecology. 3rd ed. Philadelphia, PA: Churchill Livingstone; 2003:493-510. 4. Arulkumaran S, Brosens I. Endometriosis. Best Pract Res Clin Obstet Gynaecol. 2004;18(2):177-371. 5. Pados G, Tsolakidis D, Bontis J. Laparoscopic management of the adnexal mass. Ann N Y Acad Sci. 2006;1092:211-228. 6. Catenacci M, Sastry S, Falcone T. Laparoscopic surgery for endometriosis. Clin Obstet Gynecol. 2009 Sep;52(3):351-361. 7. Yeung PP Jr, Shwayder J, Pasic RP. Laparoscopic management of endometriosis: comprehensive review of best evidence. J Minim Invasive Gynecol. 2009 May- Jun;16(3):269-281. 8. Wykes CB, Clark TJ, Khan KS. Accuracy of laparoscopy in the diagnosis of endometriosis: a systematic quantitative review. BJOG. 2004 Nov;111(11):1204-1212. 9. Garry R. The effectiveness of laparoscopic excision of endometriosis. Curr Opin Obstet Gynecol. 2004 Aug;16(4):299-303.

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