What is Deep Infiltrating Fibrotic Endometriosis?

What is Endometriosis?

About 10-15% of endometriosis are found to be more aggressive and tend to invade deep into the affected tissues and organs, forming dense scarring and producing more severe clinical symptoms such as pelvic pain, menstrual cramps, painful sexual intercourse, and painful defecation. Deep infiltrating fibrotic endometriosis most often involves deep pelvic structures such as uterosacral ligaments (the supporting ligament connecting the sacrum and posterior cervix), cul-de-sac (the space just above the vagina between the rectosigmoid colon and posterior wall of cervix), apex of vagina, rectovaginal septum (the tissue between the rectum and vagina), rectosigmoid colon, pelvic sidewalls, ovaries, tubes, bladder, and sometimes ureters.

Deep infiltrating fibrotic endometriosis usually does not respond well to hormonal suppressive therapy. Adequate surgical excision of the lesions provides the best long-term results and symptomatic relief. However, because of the deep invasive nature of the disease and the frequency of the disease involving the vital pelvic organs, the gynecologist must be experienced and competent in performing bowel, bladder, and ureteral surgery. If not proficient in handling bowel, bladder, and ureteral defects repair, the gynecologist tends to avoid resecting the endometriosis adjacent to and/or on those organs, resulting in an incomplete or inadequate excision of endometriosis and treatment failure. Laparoscopic surgery, in our opinion, is the best way to excise endometriosis. With very bright fiberoptic light shining directly onto the operative field and a sophisticated video camera magnifying the surgical area onto TV monitors, the surgeon and the entire operating team can view in precise detail the operative field with its embedded pathology, enabling them to perform a bloodless dissection and adequate resection of endometriosis with minimal blood loss. Our advice to those patients suffering from deep infiltrating fibroitc endometriosis is to find a gynecologist experienced and surgically competent in performing laparoscopic excision of severe endometriosis. Since our practice is primarily a referral one, many of our patients are from out of state. We videotape all our surgeries and provide each of our patients a copy of the tape of their surgery. Thus each patient can see exactly what she had before surgery, how the entire surgery was performed, and the results of the surgery.

Symptoms of Endometriosis

Patients with endometriosis can have symptoms varying from constant excruciating pelvic pain to no symptoms whatsoever. Paradoxically, the extent of endometriosis has no correlation to the amount of pain a women will experience. Some women with severe endometriosis do not have any symptoms and may not know they have endometriosis until a pelvic mass is detected on a routine pelvic examination or a problem with infertility is discovered. The following are the common symptoms of endometriosis, but remember that women with endometriosis may have all, some, or none of these symptoms.

  • Pain and Discomfort in the Pelvic area. Women with endometriosis most commonly experience increasing pain and discomfort right before and during their monthly cycles. Painful sexual intercourse, pressure and bloating in the lower abdomen, and severe, sometimes incapacitating, cramps during this period are not uncommon. Some women with endometriosis, however, have constant pelvic pain, cramps, and painful intercourse which may not be associated with monthly cycles.
  • Abnormal Uterine Bleeding. A women with endometriosis may have some vaginal spotting a few days before and/or after her period, or she may have abnormally heavy and long periods.
  • Gastrointestinal symptoms. Gastrointestinal dysfunction ranges from abdominal bloating or nausea to intestinal cramps, cycles of diarrhea and/or constipation. Rectal bleeding and painful bowel movements may occur when the rectum and sigmoid colon are invaded by the endometriosis.
  • Infertility. The most common cause of unexplained female infertility is endometriosis.
  • Urinary symptoms. Frequency, urgency, bladder pain, and occasionally bloody urine may occur when endometriosis has involved the bladder. Endometriosis can invade the ureter (tube between the kidney and the bladder), may cause obstruction of the ureter, and damage the kidney.
  • Lower back pain which may radiate down the legs.

What causes Endometriosis?

The real cause of endometriosis remains unknown. The following theories represent current thinking of the etiology of endometriosis, but none of them can explain all cases of endometriosis. We do know, however, that endometriosis is not caused by anything that the patient has done.

Sampson’s Theory

endometriosisThis theory was proposed by Dr. John Sampson of Boston in the 1920’s. His theory that menstrual blood refluxed through the fallopian tubes and was deposited and grew on the pelvic peritoneum and pelvic organs remains popular, but the initial attachment of single or multiple endometrial cells on the peritoneal surface has not been demonstrated. Additionally, the time-related geographic spread of endometriosis throughout the pelvis that would be predicted to occur with repeated seeding of the peritoneum by refluxed endometrium has not been demonstrated.

The fact that 90% of women have retrograde flow but only 15% of women develop endometriosis further repudiate the validity of the theory. This theory of origin also implies that older age groups of patients with endometriosis have more disease than younger age group and a high and progressively increasing rate of recurrence after complete surgical resection. The literature, however, as well as our own experience has shown that the actual rate of persistent or recurrent disease is surprisingly low after aggressive conservative surgical excision at laparoscopy or laparotomy, and the rate of recurrent or persistent disease does not appear to increase with the passage of time following excision. Because of these deficits, Sampson’s theory is losing favor to more modern concepts.

Theory of Embryonically Patterned Metaplasia or Rests

Metaplasia means a transformation from one type of tissue to another type. During the embryonic stage, the primitive cells migrate and undergo differentiation to form the pelvic organs. This embryogenesis is controlled and directed by a sophisticated, but still incompletely understood, fetal system. This fetal developmental control system may be the fetal analog of the adult immune system.

If a developmental or inheritable defect in this controlling system exists, then differentiation and migration of cells may be aberrant or incomplete. Cellular morphology and functionality might be expressed over-abundantly or inadequately. Such cells or tracts of cells are laid down in the migratory pathway of fetal organogenesis (forming the organs) across the posterior pelvic floor, although location anywhere might be possible, depending on the degree of aberrant differentiation or migration. These cells may be endometrium-like initially, or may possess the ability to undergo metaplasia into endometriosis after puberty. Arrest of migration across the posterior pelvis would conveniently explain the observation that endometriosis is most commonly and predictably found in the cul-de-sac, uterosacral ligaments, and medial broad ligaments.

Abnormalities of the fetal development control system may be preserved into adult life, giving rise to detectable abnormalities of the adult immune system. The degree of residual abnormality of the adult immune system may control the aggressiveness of the endometriosis that develops, with the result that some patients may develop invasive disease or adhesions, while most do not. The competence of the adult immune system might be impaired by exposure to environmental toxins (e.g. Dioxin), with endometriosis emerging as a possible result, as occurs in experimental laboratory primates, and endometriosis in male bladder or prostate in later life during estrogen treatment of advanced prostate cancer.

Hematogenous metastasis of endometriosis

This theory has been proposed to explain the remote occurrence of the disease. According to this theory, exfoliated endometrial cells are swept into the venous drainage of the uterus, with subsequent deposition possible anywhere in the body. Venous blood draining from the uterus must pass through the capillary bed to the lungs. If this is the case, then hematogenous spread should also result in a high rate of secondary pulmonary endometriosis unless a high rate of atrial or ventricular septal defects also exists among patients with extrapelvic endometriosis. Since neither pulmonary endometriosis nor cardiac atrial or ventricular septal defects has been reported to be more frequent in patients with endometriosis, hematogenous spread remains speculative.

Genetic Predisposition

Multicenter studies conducted by Stephen Kennedy, M.D. Professor at Oxford University show that first degree relatives of women with endometriosis are more likely to develop endometriosis. And when there is a hereditary link, the disease tends to be worse in the next generation. Chattanooga Women’s Laser Center is one of the participating study centers for the genetic predisposition factors in endometriosis.

Diagnosis of Endometriosis

Laboratory and imaging tests (ultrasound and x-rays) are not as helpful as the history and physical examination in diagnosing endometriosis. Indeed, in our own experience, the high suspicion and persistence in seeking the diagnosis of the patient are the two most important factors in leading to the majority of the diagnoses of endometriosis. Visualization of endometriosis on the lower genital tract (vagina and cervix) and tender nodules palpable above the vagina during routine pelvic examination is considered by many physicians to be diagnostic of endometriosis. However, laparoscopy (laparo = abdomen, scopy = inspection, thus, laparoscopy = inspection of abdominal cavity) to directly inspect the abdominal and pelvic cavity is the only definitive diagnosis of endometriosis.

Treatment of Endometriosis

Hormonal Therapy

Hormonal therapy is based on the knowledge of the response of existing estrogen and progesterone receptors on ectopic endometrial tissue to certain hormonal agents. Since endometrial lesions are not all the same with regard to the number and response of receptors to the hormonal agents, the result of the hormonal treatment may vary from patient to patient. Hormonal therapy is considered as suppressive, thus temporary. Endometriosis will recur after the treatment is discontinued. Following are some of the currently available hormonal agents for treatment of endometriosis.

  1. Birth Control Pills: Birth control pills, or oral contraceptives, are the combination of estrogen and progestin pills to regulate a patient’s hormonal levels and suppress endometrial growth. While a patient is on birth control pills, ovulation usually ceases and endometrial lesions shrink. The common side effects of taking birth control pills are weight gain, nausea, headache, mood changes, depression, irregular vaginal bleeding, and loss of sexual desire.
  2. GnRH Agonists: This agent works by suppressing the pituitary hormones (FSH and LH) to stop the ovary from producing estrogen, putting the patient into a menopausal state, and thus shrinking the endometrial lesions. The names of GnRH agonists include Lupron Depot, Synarel, and Zoladex. The drug can be given by injection or nasal spray. The main side effects of menopausal syndromes are hot flashes, cold sweat, insomnia, vaginal dryness, loss of sexual interest, and depression. The FDA has approved GnRH agonists for no longer than six months in a lifetime due to the risk of osteoporosis.
  3. Progestins: Progestins work by keeping the ovarian hormone (estrogen and progesterone) levels low to prevent ovulation and suppress endometrial growth. Common names of progestins are Provera, Cycrine, Megace, Micronor, Amen, Nor-Q.D., and Depo-Provera. The main side effects include irregular vaginal bleeding, depression, breast tenderness, moodiness, weight gain, headache, and fluid retention.
  4. Danazol: Danazol exhibits some androgen (male hormone) effect and reduces ovarian hormonal production. Most women will stop ovulation and menstruation when they are on Danazol, and their endometrial lesions will shrink and become inactive. The main side effects of Danazol are weight gain, decrease in breast size, acne, oily skin, male-pattern hair growth, and deepening of the voice.

Surgical Treatment

endometriosis between the rectum and uterusLaparoscopic surgery is the primary and definitive way to diagnose and treat endometriosis. Ideally, all the endometrial lesions should be excised through the laparoscope at the initial diagnosis of endometriosis. Unfortunately, most gynecologists are not well trained in treating extensive endometriosis through laparoscopy. Thus many women with extensive endometriosis often require more than one surgery and suffer from many unfortunate outcomes of poorly performed surgery. It cannot be emphasized enough the importance of finding a well trained gynecologist at initial diagnosis and treatment of endometriosis.

The definite treatment of endometriosis is NOT hysterectomy and bilateral salpingo-oophorectomy (removal of both uterus and ovaries). Many gynecologists currently still mistakenly believe that removal of both uterus and ovaries is the cure for endometriosis. At our Center, we are convinced that the correct way of treating endometriosis is the complete excision of the endometrial lesions, whether it appears on the pelvic organs, bowel, ureter (draining tube between the kidney and the bladder), or bladder.

The decision of whether to perform hysterectomy or oophorectomy at the time of endometriosis surgery is purely based on whether there are any coexisting uterine or ovarian pathology in addition to the presence of endometriosis and the patient’s desire for future childbearing. For example, if she is known to have severe endometriosis and no longer desires to bear a child, and meanwhile fibroids (benign tumor of womb which may cause heavy menstrual bleeding and cramps) are found on the uterus, then hysterectomy along with excision of all endometriosis should be recommended. Throughout the years, we have performed numerous laparoscopic surgeries for extensive endometriosis with excellent results without having removed either a normal uterus or ovaries.

The Role of Presacral Neurectomy in Conservative Surgical Treatment for Endometriosis

We sometimes perform Presacral Neurectomy (resection of part of presacral nerve which is in charge of pain sensation in the midline pelvic area). This procedure excises endometrial implants while reducing pain and cramps in the midpelvic area without affecting sexual feelings or sensations for patients desiring future childbearing.