Current Diagnosis & Treatment of Pain, 1st Edition

17. Chronic Pelvic Pain

Fred M. Howard MS, MD

Essentials of Diagnosis

  • Pain lasting 6 months or more.
  • Pain located in the anatomic pelvis or anterior abdominal wall below the umbilicus.

General Considerations

Chronic pelvic pain (CPP) is nonmenstrual pelvic pain lasting 6 months or more that causes functional disability or requires medical or surgical treatment. The mean age of women with CPP is about 30 years. Its estimated prevalence is 4%, which is similar to the prevalence of migraine headaches, asthma, and low back pain in women. CPP is the indication for 12% of all hysterectomies and for over 40% of gynecologic diagnostic laparoscopies. Direct costs of health care for CPP in the United States are estimated at $880 million per year, and direct and indirect costs may total over $2 billion per year.

On average, women have CPP for 2 to 5 years before they seek medical help. CPP often leads to years of disability and suffering, with loss of employment, marital discord and divorce, as well as numerous visits to physicians with unsuccessful results.

CPP may be due to disorders of the reproductive tract, urologic organs, gastrointestinal system, musculoskeletal system, and psychoneurologic system (Tables 17-1, 17-2, 17-3, 17-4 and 17-5). Occasionally, one of these disorders is the only diagnosis and curative treatment is possible. More often the etiology of CPP is not discernible or it is associated with several diagnoses that contribute to pain (“pain generators”). For example, endometriosis, irritable bowel syndrome (IBS), poor posture, and emotional stress may all be pain generators in a single patient, yet treatment of each of these disorders still may not result in cure of pelvic pain. Thus, a multidisciplinary approach is often ideal.

Clinical Findings

Obtaining a complete history is a crucial component of the clinical evaluation. In addition to a comprehensive pain history (discussed in Chapter 2), a thorough review of symptoms relating to the most commonly involved systems-the gastrointestinal, urologic, reproductive, and musculoskeletal systems-should be done. Intake pain questionnaires greatly facilitate the ability to obtain a detailed history. A useful form is available from the International Pelvic Pain Society (http://www.pelvicpain.org). Intake questionnaires should be used to supplement-not replace-history-taking, allowing the patient to tell her story. Not only does using intake questionnaires allow the chance to obtain a more detailed history, they also allow the clinician to observe the patient's reaction to critical aspects of the history, thereby enhancing rapport and trust. A patient is often reluctant to talk about bowel, bladder, and sexual functions, so it is important to ask her specific questions about these functions and their relationship to pain.

It is useful to have the patient mark the location of her pain on a pain map (Figure 17-1). Pain of visceral origin is usually not well localized and may be depicted as fairly diffuse. Somatic pain is more likely to show a dermatomal distribution or a myotomal pattern.

Cyclic pain related to menses is often characteristic of gynecologic disorders such as endometriosis, adenomyosis, and pelvic congestion, but such a pattern also may occur with nonreproductive tract disease, such as interstitial cystitis (IC) and IBS. A history of pain or increased pain with coitus (dyspareunia) is frequently interpreted as pathognomonic of psychological or gynecologic disease. In fact, dyspareunia is present in about 50% of women with CPP and occurs as commonly in urologic, gastrointestinal, and musculoskeletal disorders as in psychological and gynecologic disorders.

Obtaining a history regarding pregnancy and delivery is another aspect of pain evaluation. Onset during or immediately after pregnancy may suggest a musculoskeletal disorder, particularly peripartum pelvic pain syndrome. Pregnancy and childbirth are traumatic events to the musculoskeletal system, especially the pelvis and back. In addition, the hormonal changes of pregnancy cause laxity of ligaments, and this may lead to pain. Risk factors associated with pregnancy and pain include lumbar lordosis, delivery of a large infant, muscle weakness and poor physical conditioning, a difficult delivery,

P.251


vacuum or forceps delivery, and use of gynecologic stirrups for delivery.

Table 17-1. Gynecologic Diseases That Cause or Are Associated with Chronic Pelvic Pain in Women.

Extrauterine

· Adhesions

· Adnexal cysts

· Chronic ectopic pregnancy

· Chlamydial endometritis or salpingitis

· Endometriosis

· Endosalpingiosis

· Neoplasia of the genital tract

· Ovarian retention syndrome (residual ovary syndrome)

· Ovarian remnant syndrome

· Ovarian dystrophy or ovulatory pain

· Pelvic congestion syndrome

· Pelvic inflammatory disease (PID)

· Postoperative peritoneal cysts

· Residual accessory ovary

· Subacute salpingo-oophoritis (chronic PID)

· Tuberculous salpingitis
Uterine

· Adenomyosis

· Atypical dysmenorrhea or ovulatory pain

· Cervical stenosis

· Chronic endometritis

· Endometrial or cervical polyps

· Intrauterine contraceptive device

· Leiomyomata

· Symptomatic pelvic relaxation (genital prolapse)

Table 17-2. Urologic Diseases That Cause or Are Associated with Chronic Pelvic Pain in Women.

· Bladder neoplasm

· Chronic urinary tract infection

· Interstitial cystitis

· Radiation cystitis

· Recurrent, acute cystitis

· Recurrent, acute urethritis

· Stone/urolithiasis

· Uninhibited bladder contractions (detrusor dyssynergia)

· Urethral diverticulum

· Urethral syndrome

· Urethral caruncle

Table 17-3. Gastroenterologic Diseases That Cause or Are Associated with Chronic Pelvic Pain in Women.

· Carcinoma of the colon

· Chronic intermittent bowel obstruction

· Colitis

· Constipation

· Diverticular disease

· Hernias

· Inflammatory bowel disease

· Irritable bowel syndrome

A thorough physical examination is also essential. Because the physical examination, especially the pelvic examination, is often painful for the woman with CPP, it is essential that the physician go slowly enough to allow her to recover and relax between various portions of the examination. Even a “routine” pelvic examination is emotionally and physically stressful for many patients with CPP.

A major goal of the examination is to detect, in as much as possible, the exact anatomic locations of tenderness and correlate these with areas of pain. This requires a

P.252


systematic and methodical attempt to duplicate the pain by palpation or positioning.

Table 17-4. Musculoskeletal Diseases That Cause or Are Associated with Chronic Pelvic Pain in Women.

· Abdominal wall myofascial pain (trigger points)

· Chronic coccygeal pain

· Compression of lumbar vertebrae

· Degenerative joint disease

· Disk herniation or rupture

· Faulty or poor posture

· Fibromyalgia

· Fibromyositis

· Hernias: ventral, inguinal, femoral, Spigelian

· Low back pain

· Muscular strains and sprains

· Neoplasia of spinal cord or sacral nerve

· Neuralgia of iliohypogastric, ilioinguinal, and/or genitofemoral nerves

· Pelvic floor myalgia (levator anispasm)

· Piriformis syndrome

· Rectus tendon strain

· Spondylosis

Table 17-5. Psychoneurologic Disorders That Cause or Are Associated with Chronic Pelvic Pain in Women.

· Abdominal cutaneous nerve entrapment in surgical scar

· Abdominal epilepsy

· Abdominal migraine

· Bipolar personality disorders

· Depression

· Familial Mediterranean fever

· Neurologic dysfunction

· Porphyria

· Shingles

· Sleep disturbances

· Somatic referral

 

Figure 17-1. Example of a pain map in a pelvic pain patient.

Logistically, the examination is facilitated if it is performed in the following order:

  1. Standing examination.
  2. Sitting examination.
  3. Supine examination.
  4. Lithotomy examination.

Only the lithotomy portion of the examination is reviewed in this chapter. Once the patient is in the lithotomy position, initially inspect the external genitalia for redness, discharge, abscess formation, excoriation, perineal fistula, ulcerations, pigment changes, condylomata, atrophic changes (thinning, paleness, loss of vaginal rugae, protruding urethral mucosa), or signs of trauma. Look for fistulas and fissures because they may occasionally be the first objective evidence of inflammatory bowel disease.

Perform basic sensory testing to sharpness, dullness, and light touch, and test the bulbocavernosus and anal wink reflexes. Use a cotton-tipped swab to evaluate the vestibule for the localized tenderness of vulvar vestibulitis (localized vulvodynia); hold the labia apart and gently palpate the vestibule, vulva, hymen, and the area of the minor vestibular glands with a cotton-tipped swab. Patients with vulvar vestibulitis demonstrate exquisite tenderness in localized areas at the minor vestibular glands just external to the hymen, with normal sensation in adjacent vulvar areas (Figure 17-2). Use a cotton-tipped applicator or single digit palpation to evaluate the vulva and pubic arch for trigger points and for skin or mucosal lesions that reproduce the patient's symptoms. Pay particular attention to areas of previous vulvar or vaginal trauma and scars from surgeries or deliveries.

Assess the pelvic floor muscles for pain or tension by insertion of a Sims retractor or a single blade of the speculum into the posterior vagina while asking the patient to relax. The resistance to downward or posterior pressure can be evaluated to reveal increased muscle tone, tension, or spasm. This maneuver may also reproduce part of the patient's symptom complex. Single speculum blade or Sims type retractor examination may also reveal evidence of pelvic relaxation, with uterine descensus, cystocele, enterocele, or rectocele.

The traditional speculum examination is done for full visual inspection and to obtain requisite cytologic and bacteriologic specimens. It is reasonable to test for sexually transmitted diseases with cervical cultures or smears for gonorrhea and chlamydia, as well as syphilis serology, hepatitis B surface antigen screening, and HIV testing as deemed appropriate. Note the position of the cervix, as lateral displacement suggests possible ipsilateral

P.253


uterosacral endometriosis. Use a cotton-tipped swab to evaluate the cervicalos and the paracervical and cervical tissues for tenderness. In patients who have had a hysterectomy, palpate the full vaginal cuff for tenderness with a cotton-tipped applicator. If localized tender points are elicited, it may be worthwhile to block them with 1 % lidocaine and reevaluate for tenderness after 5 minutes.

 

Figure 17-2. Areas of tenderness to cotton tip palpation with localized vulvodynia (vulvar vestibulitis).

Always initiate the manual portion of the pelvic examination with a single index finger, first noting any introital tenderness or spasm. Vaginismus can be identified by involuntary introital spasm at this point in the examination. Next, directly palpate the levator animuscles for tone and tenderness. The levator animuscles are easily palpated during vaginal examination. They lie adjacent to the lateral vaginal walls just above the hymeneal ring. The medial margins of the muscles are slightly thicker than a standard pencil, running in an anteroposterior direction. Identification may be confirmed by having the patient contract her pelvic muscles. The anus elevates when the levators are contracted. Normally this palpation causes only a pressure sensation, but in patients with pelvic floor myalgia, it may cause pain consistent with at least part of the patient's clinical symptoms. In some patients with pelvic floor myalgia, there will also be tenderness of the coccyx, lateral sacrum, or sacrococcygeal ligaments. Digital pressure on the involved muscle characteristically reproduces or intensifies the patient's pain symptoms. It is not unusual for the tenderness to be unilateral.

Palpate the piriformis, coccygeus, and obturator internus muscles bilaterally seeking tenderness that reproduces the patient's pain. The piriformis muscles are somewhat more difficult to palpate than the levators. Rectal examination may allow an easier evaluation than vaginal examination. Next, the sacrospinous ligament and area of Alcocks canal are palpated to elicit any tenderness suggestive of pudendal neuralgia.

Palpate the anterior vaginal, urethral, and trigonal areas to reveal any areas of tenderness, induration, or thickening. Urethral tenderness with or without discharge is consistent with chronic urethritis or chronic urethral syndrome. Next, evaluate the “gutter” on either side of the urethra for any fullness, fluctuance, or discomfort that might suggest a urethral diverticulum or vaginal wall cyst. Also evaluate the bladder base for tenderness; its presence is consistent with trigonitis or IC.

Next, still using a single digit, palpate the cervix, paracervical areas, and vaginal fornices for tenderness or trigger points. Cervical tenderness may suggest problems such as cervicitis, repeated cervical trauma (usually from intercourse), or pelvic infection. Vaginal forniceal tenderness may suggest problems such as pelvic infection, endometriosis, ureteral tenderness, or trigger points.

Compress the uterus against the sacrum to evaluate uterine tenderness. Uterine tenderness may be consistent with diseases such as adenomyosis, pelvic congestion syndrome, pelvic infection, or premenstrual syndrome. A uterus that is immobile and fixed in position, especially a retroflexed position, may suggest endometriosis or adhesions. Next, palpate the coccyx with the single digit and attempt to move it 30 degrees or less. This part of the examination may also be done during the bimanual or rectovaginal examination. Normally the coccyx moves 30 degrees without eliciting pain, but in patients with coccydynia this movement elicits pain.

Palpate the adnexal areas next, still using a single digit without the use of the abdominal hand. This is often a

P.254


more accurate manner of assessing intrinsic tenderness of the ovaries or tube than the traditional bimanual examination, especially in patients with abdominal wall tenderness or trigger points. All ovaries are tender, so it is the degree of tenderness and the similarity to the chief pain complaint that are clinically useful.

Palpate the cecum on the right and the rectosigmoid on the left for masses, hard feces, and tenderness. Either or both may be abnormally tender in patients with IBS.

Perform traditional bimanual pelvic and rectal or rectovaginal examinations last. Marked discomfort with digital rectal examination often accompanies IBS or chronic constipation, as may hard feces in the rectum. Evaluate function of the internal and external anal sphincter by reflex wink and voluntary constriction. Carefully examine the rectovaginal septum for nodularity and tenderness, suggestive of endometriosis. Rectal examination should also include evaluation for rectal masses as many colorectal carcinomas are palpable this way. Tenderness of the anal canal may suggest abscess or fissures in the canal. Fecal occult blood test should be obtained.

Laboratory and imaging studies are not universally useful and should be done based on the clinical findings and differential diagnosis. Laparoscopy is commonly performed, especially by gynecologists, as a major part of the evaluation, but it also should be done based on the clinical findings and differential diagnosis.

A new approach to diagnostic laparoscopy, “conscious laparoscopic pain mapping,” has been suggested as a way to improve the diagnostic capability of laparoscopy. Conscious laparoscopic pain mapping is a diagnostic laparoscopy under local anesthesia, with or without conscious sedation, directed at the identification of sources of pain. The technique used with conscious laparoscopic pain mapping is a gentle probing or tractioning of tissues, lesions, and organs with a blunt probe or forceps. Diagnosis of an etiologic lesion or organ is based on the severity of pain elicited and on replication of the pain that is the patient's presenting symptom. However, CPP is a multifaceted and complicated problem, and it is premature to assume that the findings with mechanically elicited tenderness at conscious pain mapping directly translate into cause and cure. For example, a study at the University of Rochester Medical Center evaluated laparoscopic diagnosis and treatment. A historical cohort of 65 patients treated before the introduction of conscious pain mapping was compared with 50 patients treated after introduction of conscious laparoscopic pain mapping. Data failed to show any improvement in outcomes.

Treatment

Clinically, there are two approaches to the treatment of CPP. The first is to treat chronic pain itself as a diagnosis, and the second is to treat diseases or disorders that might be a cause of or a contributor to CPP. These two approaches are not mutually exclusive, and in many patients, effective therapy is best achieved by using both.

Although several disorders may be associated with CPP (see Tables 17-1, 17-2, 17-3, 17-4 and 17-5), the most frequent conditions seen in clinical practice include endometriosis, adhesions, IBS, and IC.

Cramer DW et al. The epidemiology of endometriosis. Ann N Y Acad Sci. 2002;955:11. [PMID: 11949940]

Howard FM et al. Conscious pain mapping by laparoscopy in women with chronic pelvic pain. Obstet Gynecol. 2000;96:934. [PMID: 11084181]

Lippman SA et al. Uterine fibroids and gynecologic pain symptoms in a population-based study. Fertil Steril. 2003;80:1488. [PMID: 14667888]

Tripp DA et al. Predictors of quality of life and pain in chronic prostatitis/chronic pelvic pain syndrome: findings from the National Institutes of Health Chronic Prostatitis Cohort Study. BJU Int. 2004;94:1279. [PMID: 15610105]

Zondervan K et al. Epidemiology of chronic pelvic pain. Baillieres Best Pract Res Clin Obstet Gynaecol. 2000;14:403. [PMID: 10962634]

Zubor P. Laparoscopy in chronic pelvic pain-a prospective clinical study. Ceska Gynekol. 2005;70:225. [PMID: 16047928]

American Pain Society http://www.ampainsoc.org

International Association for the Study of Pain http://www.iasp-pain.org

The Cochrane Collaboration http://www.cochrane.org/cochrane

The International Pelvic Pain Society http://www.pelvicpain.org

Adhesions

Pathogenesis

An adhesion is fibrous tissue by which anatomic structures abnormally adhere to one another. Adhesions may cause intestinal obstruction and infertility, but their role as a cause of CPP is controversial. Pelvic inflammatory disease, endometriosis, perforated appendix, prior abdominopelvic surgery, and inflammatory bowel disease are known causes of pelvic adhesions.

Prevention

At the time of surgery, adhesion barriers (biopolymers used at surgery to reduce adhesion formation), can be used and have been shown to have some, although not complete, efficacy in preventing surgical adhesions.

Clinical Findings

Pelvic pain due to adhesions is exacerbated by sudden movements, intercourse, or physical activities. The pain is often consistent in its location, although over time the area of involvement may expand. A history of one of the

P.255


classic causes of adhesions is present in 50% of women with adhesions.

Complications

Surgical treatment of adhesions appears to have limited efficacy, yet may result in significant complications, especially intestinal injuries.

Treatment

Uncontrolled, observational studies suggest that laparoscopic adhesiolysis reduces pain in 60 to 90% of patients with CPP, but the only randomized trial (adhesiolysis by laparotomy, not laparoscopy) failed to show any significant improvement in pain symptoms. Only a subgroup of women with severe, stage IV adhesions showed any improvement in pain that could be attributed to adhesiolysis. A more recent randomized trial of laparoscopic adhesiolysis for chronic abdominal pain (not just pelvic pain) also failed to show significant improvement that could be attributed to adhesiolysis. Currently, no effective treatment has been confirmed with clinical trials.

Hammoud A et al. Adhesions in patients with chronic pelvic pain: a role for adhesiolysis? Fertil Steril. 2004;82:1483. [PMID: 15589847]

Swank DJ et al. Laparoscopic adhesiolysis in patients with chronic abdominal pain: a blinded randomised controlled multi-centre trial. Lancet. 2003;361:1247. [PMID: 12699951]

Endometriosis

Pathogenesis

Endometriosis is the presence of endometrial tissue in any location other than the uterine mucosa or muscularis. By definition, it requires histologic documentation of ectopic endometrial glands and stroma. Endometriosis is one of the most common gynecologic causes of CPP and is found in at least 33% of women who undergo a laparoscopy to evaluate CPP. The precise mechanisms by which it causes pain are not completely understood.

Prevention

At this time, there are no known ways to prevent the development of endometriosis. There is no evidence that early treatment with oral contraceptives prevents endometriosis.

Clinical Findings

Most women with endometriosis-associated pain are between 20 and 45 years of age. However, endometriosis can occur in adolescents and may be a more common cause of pain in teenagers than is generally recognized. It is rare in postmenopausal women but can occur if they are receiving estrogen replacement therapy.

Classically, the woman with endometriosis seeks medical attention complaining of one or more of the following triad: an adnexal mass (endometrioma), infertility, and pelvic pain. Endometriosis-associated pelvic pain often starts as dysmenorrhea, and about 75% of women with endometriosis-associated pelvic pain have dysmenorrhea as a component of their pain symptoms. Dyspareunia with deep vaginal penetration is also a frequent component of endometriosis-associated pain. Although CPP, dyspareunia, and dysmenorrhea are characteristic symptoms of endometriosis, they are not as specific or diagnostic as is commonly thought and by themselves do not justify a diagnosis of endometriosis.

In many women with endometriosis-associated pelvic pain, there is detectable tenderness only during menses. Therefore, it is sometimes helpful to do the examination during the first day or two of menstrual flow in women with suspected endometriosis. This may also increase the likelihood of finding tender endometriotic nodules in the pelvis or rectovaginal septum.

Complications

Complications of medical treatment include side effects of weight gain, edema, hot flushes, headaches, nausea, acne, hirsutism, abnormal uterine bleeding, decreased breast size, decreased libido, vaginal dryness, weakness, decreased bone density, and thromboembolic disease. Surgical complications vary with the severity of disease, but injury to pelvic viscera is a potential risk in women with endometriosis. Untreated endometriosis is rarely life-threatening, although there are cases of ureteral and bowel obstruction due to endometriosis as well as invasion of the urinary and gastrointestinal tracts.

Treatment

Treatment of endometriosis-associated pelvic pain is complex and none of the options for therapy are ideal for all patients (Table 17-6). The patient's age, reproductive plans, presence of infertility, pain severity, and attitude toward surgery or hormonal medications are a few of the factors that must be considered in designing a treatment plan. Plans may need to be modified based on the tolerance of drug therapies or persistence or worsening of symptoms.

Surgical treatment can be done at the time of laparoscopic diagnosis in symptomatic women. Conservative, laparoscopic surgical treatment has been shown to significantly improve pain in women with stage II, III, and IV endometriosis, with a number needed to treat to be effective being 2 to 2.5. Surgery for advanced-stage

P.256


endometriosis can be challenging, tedious, frustrating, and prone to complications.

Table 17-6. Some of the Treatment Options for Women with Endometriosis-Associated Pelvic Pain.

· Observation with palliative treatment

· Conservative surgery
    - Excision and ablation of endometriotic lesions
    - Presacral neurectomy

· Hormonal treatment
    - Combined oral contraceptives

· Low dose pills, continuously; double dose for 5 days if breakthrough bleeding occurs

· Low dose pills, cyclically
    - Medroxyprogesterone acetate, 10-100 mg/d
    - Norethindrone acetate, 10-40 mg/d
    - Danazol, 200-400 mg bid
    - Gonadotropin-releasing hormone analogues

· Nafarelin, 200-400 mcg bid

· Depot-leuprolide, 3.75-7.5 mg every 28 days

· Goserelin, 3.6 mg every 28 days

· Combined medical and surgical treatments

· Definitive extirpative surgery
    - Hysterectomy
    - Salpingo-oophorectomy

Presacral neurectomy (resection of the superior hypogastric plexus) and uterosacral neurectomy (uterine nerve resection or transection of the uterosacral ligament) have been recommended for relief of CPP associated with endometriosis. Data from clinical trials show that presacral neurectomy somewhat improves pain relief obtained with surgical treatment of endometriosis. However, presacral neurectomy may lead to intractable constipation in up to 5% of patients and bothersome urinary urgency in 5%. Data from clinical trials clearly show that uterosacral neurectomy does not improve pain relief when included in surgical treatment of endometriosis.

Medical treatment with gonadotropin-releasing hormone (GnRH) agonists, progestins, danazol, or combined oral contraceptives often relieves endometriosis-associated pelvic pain. The number needed to treat to be effective is 2 to 2.5.

GnRH agonists inhibit the production and release of luteinizing hormone and follicle-stimulating hormone, leading to a dramatic decline in estradiol levels, induction of amenorrhea, and improvement of pain levels. Examples of GnRH agonists available in the United States are depot leuprolide (3.75 mg intramuscularly every 28 days) and goserelin (3.6 mg subcutaneous implant every 28 days). When patients have a recurrence of pain within 1 year after treatment with GnRH analogues, re-treatment appears to be reasonably effective, with about 67% of patients showing a significant reduction of pain levels during retreatment.

Loss of bone density with GnRH analogues is a serious concern. Clinical trials with GnRH agonists show that add-back therapy with conjugated equine estrogen or norethindrone acetate significantly decreases bone loss.

Danazol (200 to 400 mg orally twice a day), a 17-ethinyl-testosterone derivative, has efficacy similar to that of GnRH agonists. However, danazol is not as frequently used as GnRH agonists because of possible androgenic side effects, including significant weight gain, mood changes, and musculinizing symptoms.

Medroxyprogesterone acetate has been a recommended treatment for many years. Although a high dose of 100 mg/d was used in the only placebo-controlled trial of medroxyprogesterone acetate, lower doses are generally used in clinical practice.

Oral contraceptive treatment of endometriosis is a longstanding approach, using either cyclical or continuous dosing. Efficacy appears to be similar or somewhat less than the other hormonal treatments.

Combining medical and surgical treatments appear to result in the best relief of pain.

Prognosis

Endometriosis is often a progressive disease without treatment. Resolution of pain without treatment is uncommon. Surgical and medical treatments are efficacious, although only complete extirpation including hysterectomy and bilateral oophorectomy appears to have a high cure rate.

Abbott J et al. Laparoscopic excision of endometriosis: a randomized, placebo-controlled trial. Fertil Steril. 2004;82:878. [PMID: 15482763]

Busacca M et al. Post-operative GnRH analogue treatment after conservative surgery for symptomatic endometriosis stage III-IV: a randomized controlled trial. Hum Reprod. 2001;16:2399. [PMID: 11679528]

Gambone JC et al. Consensus statement for the management of chronic pelvic pain and endometriosis: proceedings of an expert-panel consensus process. Fertil Steril. 2002;78:961. [PMID: 12413979]

Howard FM. An evidence-based medicine approach to the treatment of endometriosis-associated chronic pelvic pain: placebo-controlled studies. J Am Assoc Gynecol Laparosc. 2000;7:477. [PMID: 11044498]

Jain KA. Sonographic spectrum of hemorrhagic ovarian cysts. J Ultrasound Med. 2002;21:879. [PMID: 12164573]

P.257

 

Jarrell J et al. Laparoscopy and reported pain among patients with endometriosis. J Obstet Gynaecol Can. 2005;27:477. [PMID: 16100643]

Lamvu G et al. The role of laparoscopy in the diagnosis and treatment of conditions associated with chronic pelvic pain. Obstet Gynecol Clin North Am.2004;31:619. [PMID: 15450323]

Luciano AA. Leuprolide acetate in the management of endometriosis-associated pain: a multicenter, evaluator-blind, comparative clinical trial. Glob Cong Gynecol Endo. 2004;11(Suppl): S5.

Olive DL. Optimizing gonadotropin-releasing hormone agonist therapy in women with endometriosis. Treat Endocrinol. 2004;3:83. [PMID: 15743104]

Scarselli G et al. Diagnosis and treatment of endometriosis. A review. Minerva Ginecol. 2005;57:55. [PMID: 15758866]

Sutton C et al. A prospective, randomized, double-blind controlled trial of laparoscopic uterine nerve ablation in the treatment of pelvic pain associated with endometriosis. Gynaecol Endoscopy. 2001; 10:217.

Tsai YL et al. Short-term postoperative GnRH analogue or danazol treatment after conservative surgery for stage III or IV endometriosis before ovarian stimulation: a prospective, randomized study. J Reprod Med. 2004;49:955. [PMID: 15656211]

Valle RF et al. Endometriosis: treatment strategies. Ann N Y Acad Sci. 2003;997:229. [PMID: 14644830]

Vercellini P et al. Laparoscopic uterosacral ligament resection for dysmenorrhea associated with endometriosis: results of a randomized, controlled trial. Fertil Steril. 2003;80:310. [PMID: 12909493]

Yap C et al. Pre–and postoperative medical therapy for endometriosis surgery. Cochrane Database Syst Rev. 2004;(3):CD003678. [PMID: 15266496]

Zullo F et al. Effectiveness of presacral neurectomy in women with severe dysmenorrhea caused by endometriosis who were treated with laparoscopic conservative surgery: a 1-year prospective randomized double-blind controlled trial. Am J Obstet Gynecol. 2003; 189:5. [PMID: 12861130]

Zullo F et al. Long-term effectiveness of presacral neurectomy for the treatment of severe dysmenorrhea due to endometriosis. J Am Assoc Gynecol Laparosc.2004;11:23. [PMID: 15104826]

Endometriosis Association http://www.endometriosisassn.org/

MedlinePlus: A service of the United States Library of Medicine and the National Institutes of Health http://www.nlm.nih.gov/medlineplus/endometriosis.html

Irritable Bowel Syndrome

Pathogenesis

IBS is one of the most common disorders associated with CPP in women. Symptoms suggestive of IBS are present in 50 to 80% of women with CPP. Approximately 25 to 50% of all referrals to gastroenterologists are related to this diagnosis. In most Western countries, IBS is three times more common in women than in men.

IBS is a functional disorder, which means that, by definition, no structural or biochemical abnormalities are present that explain the symptoms. It is one of several functional digestive disorders. The pathophysiologic mechanisms that cause IBS are not completely understood and likely are multifactorial. One alteration that may account for some symptoms of IBS is increased visceral sensitivity; patients with IBS have abnormal pain levels with intestinal distention.

Clinical Findings

IBS is defined by the presence of abdominal pain associated with bowel movements and changes of bowel function in the absence of other pathologies to explain the symptoms. The ROME II criteria are generally accepted as the clinical definition of this syndrome (Table 17-7).

A detailed history must be obtained about bowel function and the association with pain to discern whether the ROME II criteria are met and whether other diagnoses are likely. Abdominal pain must be present to have IBS. Pain is most often in the left lower quadrant but may be located in the middle or right lower abdomen as well. Many patients have two or more sites of pain. Eating commonly precipitates pain and defecation often relieves it. A complete history includes questions about anorexia, early satiety, nausea, vomiting, number of bowel movements per day, number of bowel movements per week, urgency to defecate, prolonged evacuation attempts, straining to defecate, stool color, weight loss without dieting, and increase of symptoms with sex or menses. A detailed dietary history is also important, particularly related to

P.258


lactose, sucrose, fructose, caffeinated products, and gas-producing foods.

Table 17-7. ROME II Criteria for Irritable Bowel Syndrome.

· At least 12 weeks, which need not be consecutive, in the preceding 12 months of abdominal discomfort or pain that has two of the following three features:
    - Relieved with defecation
    - Onset associated with a change in frequency of stool
    - Onset associated with a change in form (appearance) of stool

· Symptoms that support diagnosis:
    - < 3 bowel movements/week
    - <3 bowel movements/day
    - Hard or lumpy stools
    - Loose or watery stools
    - Straining during a bowel movement
    - Urgency
    - Feeling of incomplete bowel movement
    - Passing mucus
    - Abdominal fullness, bloating, or swelling

A detailed medication history, including all current and past medications, both prescribed and over-the-counter, is necessary. Many medications alter bowel motility and may exacerbate symptoms of IBS. In particular, many patients take laxatives and do not realize that laxatives contribute to their symptoms. Antacids containing magnesium or aluminum can cause diarrhea or constipation, respectively.

Travel history, particularly overseas, is often important in the differential diagnostic evaluation of symptoms suggesting IBS. In addition, family history of gastrointestinal diseases, especially inflammatory bowel disease; colon cancer; or malabsorption states, such as sprue, is important in the differential diagnostic evaluation.

A history of rectal bleeding suggests a diagnosis other than IBS unless the bleeding is related to hemorrhoids or a fissure from straining. Similarly, a history of weight loss suggests the diagnosis is not IBS. Weight loss is unusual in a patient with IBS unless there is concomitant depression.

The symptoms of IBS are chronic, although variable in severity. Pain and bowel symptoms that are of a steadily progressive nature suggest a diagnosis other than IBS. Also, as a chronic disorder, IBS usually has an onset of symptoms that is gradual and vague, and it is unusual for the patient to be able to relate an exact date of onset of symptoms. If so, it is likely that she does not have IBS.

Either diarrhea or constipation, or alternating episodes of both, may be present. It is helpful to ask the patient to describe her bowel movements precisely. In particular, many patients complain of constipation if they do not have a bowel movement daily and do not realize that normal stool frequency is anywhere from 3 times a day to every 3 days. In patients with IBS and diarrhea, the volume of diarrhea is small (< 200 mL/d). Diarrheic stool volumes greater than 200 mL/d suggest the diagnosis is not IBS.

Characteristically, both pain and diarrhea resolve during sleep. Also, diarrhea associated with IBS usually resolves during a 24-hour fast. Awakening and noting pain is not the same as being awakened by pain, and it is important to try to have the patient make this distinction if possible.

IBS symptoms are often exacerbated during menses, so direct questions should be asked about a cyclic pattern corresponding with menses, but this correlation should not be assumed to mean the pain is of gynecologic origin. Even in women without IBS there is an increased occurrence of diarrhea, constipation, and increased gas at menses. Women with IBS also have an increased frequency of dyspareunia, compared with women without IBS.

The general physical examination is usually normal. Abdominal examination may reveal mild to moderate distention and mild to moderate tenderness, especially in the left lower quadrant. Rebound tenderness is not a common finding. Rectal and pelvic examinations are important to assess for masses or anal disease, such as hemorrhoids or fissures that could explain some of the symptoms.

In the patient with suspected IBS, a complete blood cell count with differential, chemistry profile, and sedimentation rate are suggested. With IBS, the chemistry profile should be normal, whereas in inflammatory bowel disease electrolyte abnormalities are more likely. To rule out infection with Giardia, amoeba, and other parasites, three stool specimens should be sent for ova-and-parasite testing.

Also, stool should be checked for occult blood; results should be negative in patients with IBS. Similarly, methylene blue stain of stool to look for white blood cells should be negative with IBS, as the presence of large numbers of white blood cells is diagnostic of inflammation. Stools should be checked for Clostridium difficile toxin if there is significant persistent diarrhea.

In women younger than 40 years of age, proctosig-moidoscopy with biopsy should be performed. Although the mucosa may appear grossly normal, biopsy may reveal microscopic or collagenous colitis. In patients older than 40 years of age, a barium enema and flexible sigmoidoscopy or a full colonoscopy may be indicated to rule out neoplasia. The insufflation during an air-contrast barium enema or colonoscopy often reproduces IBS symptoms.

Complications

Women with IBS have a disproportionately high predisposition to undergo hysterectomy. Of women with IBS, 21% of those 18 to 40 years of age have undergone hysterectomies; this is significantly higher than the national average of about 6%.

Clearly, it is important that accurate diagnosis and comprehensive treatment modalities be tried before hysterectomy is performed in women with pelvic pain and symptoms suggestive of IBS. Whether hysterectomy is capable of causing or worsening IBS symptoms is not clear.

Treatment

Dietary treatment is the mainstay of therapy, but most dietary interventions have not been experimentally validated. Elimination of dietary lactose, sorbitol, and fructose is advised. Lactose intolerance can mimic IBS and contribute to IBS symptoms; about 40% of patients with IBS also have lactose intolerance. Sorbitol, which is a common sweetening agent used in “sugar free” and other dietetic foods, may also contribute to symptoms.

P.259

 

Fructose, a major sugar component of fruit and an additive to a variety of processed foods, also can cause significant abdominal distress. Caffeinated products, including coffee, tea, and cola, carbonated products, and gas-producing foods may contribute to bloating. Smoking and chewing gum lead to more swallowed air and may increase gas and bloating. Excessive alcohol consumption may lead to increased rectal urgency.

Medical treatment of IBS is directed to symptomatic relief. Categorizing patients into one of three major sub-classifications can help in selecting medical therapy, depending on which symptoms are predominant:

  • Abdominal pain, gas, and bloating.
  • Constipation.
  • Diarrhea.

Unfortunately, many patients do not fall clearly into one of these three groups; rather, they have overlapping symptoms.

With predominantly abdominal pain, gas, and bloating, an antispasmodic may be useful. The commonly used antispasmodics are dicyclomine, hyoscyamine, atropine-hyoscyamine-phenobarbital-scopolamine formulation, and chlordiazepoxide with methscopolamine. Potential side effects of antispasmodic, anticholinergic medications include urinary retention, xerostomia, and mydriasis. It is helpful to discuss these side effects with the patient before initiating treatment. Because many patients have these symptoms postprandially, the timing of the dosing is crucial. Generally, it is best to give each of these medications 30 minutes before meals. However, if a sublingual preparation is prescribed, then it can be given at the time that the discomfort begins. Gas and bloating symptoms may be decreased by α-D-galactosidase (Beano) or a simethicone preparation. Peppermint oil may decrease abdominal distention and reduce flatulence.

With predominantly constipation, a trial of increased roughage and psyllium is often beneficial. However, many patients have increased gas with increased fiber, and about 15% cannot tolerate fiber therapy. In such cases, a stool softener or osmotic laxative can be tried. Long-term use of stimulant laxatives should be discouraged. The 5-HT4 receptor agonist tegaserod maleate (6 mg PO twice daily) is effective and approved for short-term use in women.

With predominantly diarrhea, loperamide is the most commonly used agent. A particular advantage of loperamide is that it does not cross the blood-brain barrier, unlike other antidiarrheal agents. Tricyclic antidepressants may be another option for diarrhea-predominant patients.

Combining psychological treatment with medical therapies improves the clinical response in patients with

IBS. Factors that predict a good response to psychotherapy include predominately diarrhea and pain symptoms, the association of overt psychiatric symptoms, intermittent pain exacerbated by stress, short durations of bowel complaints, and few sites of abdominal pain. Patients with constant abdominal pain do poorly with psychotherapy or hypnotherapy.

Kamm MA et al. Tegaserod for the treatment of chronic constipation: A randomized, double-blind, placebo-controlled multinational study. Am J Gastroenterol.2005; 100:362. [PMID: 15667494]

Kellow J et al. An Asia-Pacific, double blind, placebo controlled, randomised study to evaluate the efficacy, safety, and tolerability of tegaserod in patients with irritable bowel syndrome. Gut. 2003;52:671. [PMID: 12692051]

Lackner JM et al. Psychological treatments for irritable bowel syndrome: a systematic review and meta-analysis. J Consult Clin Psy chol. 2004;72:1100. [PMID: 15612856]

Longstreth GF et al. Irritable bowel syndrome and surgery: a multivariable analysis. Gastroenterology. 2004; 126:1665. [PMID: 15188159]

Novick J et al. A randomized, double-blind, placebo-controlled trial of tegaserod in female patients suffering from irritable bowel syndrome with constipation. Aliment Pharmacol Ther. 2002; 16:1877. [PMID: 12390096]

Nyhlin H et al. A double-blind, placebo-controlled, randomized study to evaluate the efficacy, safety and tolerability of tegaserod in patients with irritable bowel syndrome. Scand J Gastroenterol. 2004;39:119. [PMID: 15000272]

Spiller RC. Irritable bowel syndrome. Br Med Bull. 2004;72:15. [PMID: 15767561]

Whitehead WE et al. The usual medical care for irritable bowel syndrome. Aliment Pharmacol Ther. 2004;20:1305. [PMID: 15606392]

Williams RE. Recognition and treatment of irritable bowel syndrome among women with chronic pelvic pain. Am J Obstet Gynecol. 2005;192:761. [PMID: 15746669]

Ziegenhagen DJ et al. Cisapride treatment of constipation-predominant irritable bowel syndrome is not superior to placebo. J Gastroenterol Hepatol.2004;19:744. [PMID: 15209619]

American Gastroenterological Association http://www.gastro.org

International Foundation for Functional Gastrointestinal Disorders http://www.iffgd.org

Irritable Bowel Syndrome Association http://www.ibsassociation.org/

National Digestive Diseases Information Clearinghouse (NDDIC): A service of the National Institute of Diabetes and Digestive and Kidney Diseases http://www.digestive.niddk.nih.gov/ddiseases/pubs/ibs/

Interstitial Cystitis/Painful Bladder Syndrome

Pathogenesis

Interstitial cystitis/painful bladder syndrome (IC/PBS) is a chronic inflammatory condition of the bladder characterized by CPP associated with bladder

P.260


dysfunction. The disease appears to be unrelated to menopausal status, occurring both premenopausally and postmenopausally.

The etiology of IC/PBS is unknown. It is possible that more than one etiology and more than one disease are encompassed in the syndrome. Current thinking is that patients with IC/PBS have defects in the glycosaminoglycan layer of the bladder wall. The glycosaminoglycans of the bladder surface are extremely hydrophilic polysaccharides that form a layer of micelles of water on the bladder epithelium. This micellar layer acts as a barrier between the transitional epithelial cells and urine. It is hypothesized that a defect in this layer allows “leaking” of the epithelium, resulting in a dysfunctional epithelium with excessive permeability and exposure of the transitional epithelium and muscularis to noxious substances in the urine.

An autoimmune cause of this leakiness seems possible. Several researchers have demonstrated an increased number of mast cells in the bladder wall of patients with IC/PBS, potentially consistent with an autoimmune process. Detection of antinuclear antibodies and increased excretion of eosinophilic cationic protein in the urine also support an autoimmune mechanism. Other proposed mechanisms include viral infection, toxin exposure, or other inflammatory mediators. However, the failure to culture an organism and the failure of antibiotic therapy to alleviate symptoms argues against a bacterial infectious cause.

The physiologic causes of pain with IC are also not clear. The inflammatory reaction of the bladder wall may, via algesic substances released by this reaction, cause nociceptor stimulation of visceral neural pathways. This neuroinflammation may cause pain as well as urgency and frequency.

Clinical Findings

The definition and diagnostic criteria of IC/PBS are imprecise, but most commonly it is defined clinically by the following triad:

  1. Irritative voiding symptoms.
  2. Absence of objective evidence of another disease that could cause the symptoms.
  3. A characteristic cystoscopic appearance of the bladder (glomerulations).

Dysuria or pain with voiding is not a characteristic symptom, but pain with the urge to void or after voiding is common. The urgency and frequency experienced is so severe that women with IC/PBS often have histories of recurrent treatment for urinary tract infections. This may be why there is typically a history of 3 to 7 years of symptoms before the diagnosis is established. Nocturia, with voiding at least two times per night, is also a characteristic and troublesome symptom. Incontinence is not a characteristic symptom of IC/PBS.

Pain is typically suprapubic and may radiate to the low back or the groin. Pain with intercourse is also common. Pain in the pelvic floor muscles, especially the levator ani, piriformis, and obturators is commonly associated with IC/PBS.

The physical examination in women with IC/PBS is usually normal. Many women may have anterior vaginal wall tenderness under the trigone and suprapubic pelvic tenderness, but this is variable. Tenderness of the pelvic floor muscles may be noted.

In patients with IC/PBS without infection, a urinalysis is generally normal. In the occasional patient with hematuria, urine cytology or cystoscopy is essential. Urine culture should be negative.

Cystoscopy with hydrodistention, with the finding of characteristic petechial, submucosal hemorrhages (termed “glomerulations”) is the gold standard diagnostic criteria for IC/PBS, although clearly there are false-positives and false-negatives. Because the bladder needs to be significantly distended, which is painful in women with IC/PBS, general or spinal anesthesia is usually necessary. Occasionally, Hunner ulcer may also be noted, but it is not a consistent finding.

The potassium challenge also may be a useful diagnostic test for IC/PBS. With this test, the bladder is infused with 40 mL of sterile water, and the patient is asked to rate her pain level and sensation of urgency after the water is retained for 3 to 5 minutes. Patients with IC/PBS generally do not have a change of pain level with this volume of sterile water. The water is then drained, and 40 mL of a 400 mEq/L solution of potassium chloride is infused into the bladder. (This solution is easily made by mixing 40 mEq of potassium chloride with sterile water and bringing the volume to 100 mL.) The patient is again asked to rate her symptoms. A positive test is when the patient has a significantly greater increase of pain and urgency with the potassium chloride solution. Patients with radiation cystitis also have positive responses to the potassium challenge test.

Treatment

The treatments approved by the US Food and Drug Administration for IC/PBS are intravesical dimethylsulfoxide and oral pentosan polysulfate sodium. Dimethylsulfoxide is administered intravesically every 1 to 2 weeks for 2 to 3 months. Dimethylsulfoxide treatments result only in remission of disease, not cure. Other intravesical therapies for IC have been less extensively studied and efficacies are not established. Intravesical capsaicin, Bacillus Calmette-Guérin, clorpactin, heparin, and local anesthetics are examples of other medications used as intravesical treatments.

P.261

 

Pentosan polysulfate sodium is a polyanionic analogue of heparin. Reported results of its effectiveness have been mixed, but at least one placebo-controlled, double-blinded study showed a clinically significant response. Other nonsurgical treatments are often used, but evidence of their effectiveness is scant. Cyclosporine, L-arginine, nifedipine, antihistamines, and tricyclic antidepressants are some of the reported treatments.

Based on clinical observations, the mainstay of urologic treatment of IC for more than 50 years has been hydrodistention of the bladder. This procedure can be performed at the time of diagnostic cystoscopy if general or spinal anesthesia is used. It is too painful to be done without anesthesia.

Prognosis

In some patients, IC/PBS is a progressive disease with severe compromise of bladder function and incapacitating loss of bladder capacity. At times, this has led to the need for surgical treatments via augmentation cystoplasty and cystectomy-urethrectomycontinent diversion with a Kock or Indiana pouch.

Bernie JE et al. The intravesical potassium sensitivity test and urodynamics: implications in a large cohort of patients with lower urinary tract symptoms. J Urol. 2001;166:158. [PMID: 11435846]

Nordling J. Interstitial cystitis: how should we diagnose it and treat it in 2004? Curr Opin Urol. 2004; 14:323. [PMID: 15626873]

Parsons CL et al. Gynecologic presentation of interstitial cystitis as detected by intravesical potassium sensitivity. Obstet Gynecol. 2001;98:127. [PMID: 11430970]

American Urological Association Foundation, Inc http://www.afud.org

American Urogynecologic Society http://www.augs.org

Interstitial Cystitis Association http://www.ichelp.org

National Kidney and Urologic Diseases Information Clearinghouse (NKUDIC): A service of the National Institute of Diabetes and Digestive and Kidney Diseases http://www.kidney.niddk.nih.gov/kudiseases/pubs/interstitialcystitis/

Adenomyosis

Pathogenesis

Adenomyosis is growth of endometrial glands and stroma into the myometrium at least 2 to 3 mm below the endometrial surface. The reported incidence of adenomyosis ranges from 5 to 70%, varying with the scrutiny of histologic evaluation and the patient's symptoms, age, and parity.

Clinical Findings

Symptoms of adenomyosis are menorrhagia and dysmenorrhea, but many women with adenomyosis are asymptomatic. Many women with adenomyosis have tender uteri symmetrically enlarged up to 12 weeks' gestational size. Often, the uterus of a woman with adenomyosis is diffusely boggy to palpation, or it may have a nodular consistency, reminiscent of multiple small intramural fibroids.

Magnetic resonance imaging may be useful for the preoperative diagnosis of adenomyosis. On T2 weighted images, diffuse adenomyosis distorts normal zonal anatomy of the uterus, causing enlargement of the functional zone, seen as a wide band with low signal intensity adjacent to the endometrium.

Diagnostic hysteroscopy may show small diverticula when there is a connection between the ectopic sites of adenomyosis in the endometrial cavity. In symptomatic patients with a normal appearing endometrial cavity, a hysteroscopic or laparoscopic myometrial biopsy may be helpful.

Treatment

Gonadotropin suppression with GnRH agonists, such as depot leuprolide or goserelin, may relieve symptoms, but they recur when suppression is discontinued. There may be a role for endometrial ablation or resection in some patients with adenomyosis. The principal method of diagnosis and therapy of adenomyosis is still hysterectomy.

Wang PH et al. Treatment of infertile women with adenomyosis with a conservative microsurgical technique and a gonadotropin-releasing hormone agonist. Fertil Steril. 2000;73:1061. [PMID: 10785242]

Dysmenorrhea

Pathogenesis

Dysmenorrhea is severe, cramping pain in the lower abdomen, lower back, and upper thighs during menses. It is termed “primary dysmenorrhea” when it is not a symptom of another disorder. Secondary dysmenorrhea is the term used when dysmenorrhea is a symptom of pelvic pathology. Dysmenorrhea is not only a significant pain problem itself but is frequently a component of CPP.

Primary dysmenorrhea appears to be due principally to prostaglandins, in particular F2α and E2 released from the endometrium at menses. Both estradiol and progesterone levels influence the synthesis and levels of endometrial PGF2α.

Clinical Findings

Primary dysmenorrhea usually begins 6 to 12 months after menarche and coincides with the onset of ovulatory cycles. However, some patients complain of pain from the first cycle. Patients complain of spasmodic or

P.262


cramping lower abdominal pain that may radiate suprapubically, to the low back, and to the anteromedial aspect of the thighs. The pain may also be described as continuous, dull, and aching. Other symptoms, such as headache, nausea, vomiting, diarrhea, and fatigue, often accompany the menstrual pain. Symptoms typically last 72 hours or less. They may also start 1 or 2 days before the onset of menses. Occasionally, the accompanying vaso-constriction in the acute phase may be so marked that the patient appears to be shocky.

The pelvic examination should be normal in women with primary dysmenorrhea and may be normal or abnormal in women with secondary dysmenorrhea. Sometimes a laparoscopy may have to be performed to rule out pelvic pathology, particularly endometriosis.

Treatment

Successful management of dysmenorrhea can be challenging. A healthy lifestyle (including nutritional supplements) and aerobic exercise (such as walking, swimming, and bicycling) may produce an overall benefit and decrease the impact of dysmenorrhea on the patient's daily activities. For appropriate selection of treatment, it is usually helpful to determine whether dysmenorrhea is primary or secondary.

Oral contraceptives provide significant relief of primary dysmenorrhea and are a good first-line therapy for many young women, especially if contraception is also needed.

Nonsteroidal anti-inflammatory drugs (NSAIDs) have had a pivotal role in treating primary dysmenorrhea. More than 70% of women obtain significant relief with NSAIDs. Unlike oral contraceptives, NSAIDs need to be taken only 2 to 5 days per month and do not suppress the hypothalamic-pituitary-ovarian axis. A trial of up to 3 to 6 months may be needed to demonstrate effective relief of symptoms. If a particular NSAID is ineffective, it is worth trying a different one, since there is significant variability of individual responsiveness to the NSAIDs (see Table 3-1).

Surgical interventions include hysterectomy, presacral neurectomy, uterosacral neurectomy, and cervical dilation but generally should be considered only if medical treatment has failed.

Leiomyomata Uteri

Pathogenesis

A leiomyoma is a benign tumor of smooth muscle; other names are fibroid, fibromyoma, or myoma. Uterine leiomyomas are the most common tumors of the female pelvis and occur in one of four to five women, with the highest incidence in the fifth decade.

Clinical Findings

About 33% of women with uterine leiomyomas experience pain, but it is usually dysmenorrhea. CPP is more likely to be produced by associated pathology (eg, endometriosis or adhesions) than by a uterine fibroid. Occasionally, pressure-type symptoms, either directly from the leiomyoma or from pressure on the bladder or rectum, become severe and present as CPP. The onset of pain in such cases is usually gradual. Ureteral compression from very large myomas can produce hydronephrosis and back pain. Intermittent torsion of a pedunculated leiomyoma can cause sharp pelvic pain.

Leiomyomata can be diagnosed by finding an enlarged, firm, irregularly shaped uterus at the time of pelvic examination. With degeneration, the consistency of the myomas may become softer, or even cystic, to palpation. Rarely, the uterus may be tender. Sometimes, it is difficult to differentiate uterine myomata from an adnexal tumor.

Imaging studies, especially ultrasonography, can usually distinguish uterine from ovarian tumors. It also is valuable to document the number of fibroids, their location, degree of calcification, and rate of growth. Laparoscopy is helpful to discover associated pathology in patients with CPP.

Complications

Abnormal bleeding, including significant menorrhagia, is more common with leiomyomas than is pelvic pain. Such bleeding can at times become life-threatening and mandate intervention.

Treatment

Treatment of CPP associated with uterine fibroids includes expectant management, medical therapy, radio-logic embolization, surgical removal or destruction of the myomas, and hysterectomy. Expectant management should include repeat examinations every 6 months to assure there is no rapid growth. NSAIDs can be used to treat dysmenorrhea and iron therapy may be necessary to treat anemia due to abnormal bleeding. GnRH agonists have been effective in reducing the size and symptoms of leiomyoma, but regrowth to pretreatment size occurs within 12 weeks after cessation of treatment. GnRH agonist therapy has been useful to reduce blood loss, treat iron deficiency anemia, and convert an abdominal to a vaginal hysterectomy.

Arterial embolization has been demonstrated to decrease the size of fibroids, but long-term results regarding pain relief are lacking.

Hysterectomy is usually the optimal way to ensure successful treatment with complete removal of all myomas and no recurrences. Myomectomy is an option

P.263


for those patients wishing to retain their reproductive potential. Other medical and nonmedical therapies, including myolysis and mifepristone, are under investigation.

Prognosis

Uterine leiomyomas are almost always benign and rarely transform into malignancies, so the overall prognosis is good. Treatment improves quality of life.

Ovarian Retention Syndrome

Pathogenesis

Ovarian retention syndrome, also called residual ovary syndrome, is the presence of pelvic pain or dyspareunia (or both) after deliberate conservation of one or both ovaries at the time of hysterectomy. The reported incidence of ovarian retention syndrome ranges from 0.9% to 4.9%. Proposed mechanisms for pain from retained ovaries include the following:

  1. Adhesions interfere with ovarian function and ovulation, leading to multiple cystic, atretic, or hemorrhagic follicles.
  2. Adhesions hinder the ovary's ability to cyclically expand because of its encapsulation in dense adhesions.
  3. Adhesions undergo distention with ovarian function and follicular development.

Prevention

Removal of the ovaries at the time of hysterectomy prevents ovarian retention syndrome, but obviously this is not clinically appropriate in many patients. Whether adhesion prevention barriers would decrease the likelihood of this syndrome is not known.

Clinical Findings

Patients with ovarian retention syndrome may complain of cyclic or continuous pain that may range from a bothersome ache to recurrent colicky pain to incapacitating cramps. Pain is often localized to the side of the retained ovary. The pain can also radiate to the lower back and into the legs. Deep dyspareunia occurs in at least 20% of patients.

On physical examination, most patients have a tender pelvic mass at the vaginal vault. Imaging studies may confirm the location and cystic status of the ovary or ovaries. Relief of pain with GnRH agonist suppression of ovarian function may confirm the retained ovary or ovaries as a source of pain. Laparoscopy may also be used as a diagnostic test and has the advantage of allowing simultaneous oophorectomy if indicated.

Treatment

Medical treatment with hormonal replacement therapy, continuous medroxyprogesterone, oral contraceptives, or GnRH agonists is often used, but studies confirming efficacy are lacking. Surgical treatment with salpingo-oophorectomy is the most common treatment but can be difficult due to extensive adhesions. Surgery can sometimes be performed laparoscopically but more often has been done by laparotomy due to the likelihood of severe adhesive disease.

Prognosis

Surgical removal of the retained ovary or ovaries has a high success rate, especially if the prior hysterectomy was not done for CPP.

Baxter N et al. The effect of gonadotropin-releasing hormone analogue as first-line management in cyclical pelvic pain. J Obstet Gynecol. 2004;24:64. [PMID: 14675984]

Howard FM. The role of laparoscopy as a diagnostic tool in chronic pelvic pain. Baillieres Best Pract Res Clin Obstet Gynaecol. 2000; 14:467. [PMID: 10962637]

Mahdavi A et al. Laparoscopic management of ovarian remnant. Obstet Gynecol Clin North Am. 2004;31:593. [PMID: 15450320]

Ovarian Remnant Syndrome

Pathogenesis

Ovarian remnant syndrome is the persistence of functional ovarian tissue inadvertently not removed at the time of intended extirpation of one or both ovaries, with or without hysterectomy. The incidence of ovarian remnant syndrome is not known, but it is probably more common than has previously been appreciated. The major predisposing factor to ovarian remnant syndrome is a difficult oophorectomy, usually due to adhesive disease, difficulty in hemostasis, or alteration of normal anatomy. It has been shown experimentally that incompletely excised, devascularized ovarian tissue can reimplant and function.

Prevention

Ensuring complete removal of all ovarian tissue at the time of oophorectomy is essential to prevent ovarian remnant syndrome. However, this may not always be achievable in particularly difficult cases.

Clinical Findings

Ovarian remnant syndrome should be considered in any woman with pelvic pain after hysterectomy and bilateral salpingo-oophorectomy. Too often, clinicians assume

P.264


that it is impossible for these women to have a gynecologic disease. Ovarian remnant syndrome should also be considered in any woman with ipsilateral pelvic pain after a unilateral oophorectomy.

It usually presents as chronic pain of varying quality and severity in the abdominopelvic area, with or without a palpable pelvic mass. The absence of hot flushes in a woman not receiving hormonal replacement following bilateral oophorectomy should be a signal to investigate carefully for ovarian remnant.

Ovarian remnants are often too small to be palpated. Tenderness to palpation by pelvic examination is usually present on the same side as the remnant.

Without hormonal replacement therapy, women with ovarian remnant syndrome frequently have serum follicle stimulating hormone and estradiol concentrations at pre-menopausal levels. The diagnosis can also be confirmed using GnRH agonist stimulation. During the first week of administration, continuous GnRH agonist administration initially stimulates estrogen production. Thus, estradiol levels increase significantly 1 week after administration of a GnRH agonist, such as depot leuprolide, from their baseline levels in women with ovarian remnant syndrome.

Vaginal ultrasound shows a pelvic mass in 50 to 85% of cases. The diagnostic accuracy of ultrasound may be improved by stimulating follicular cyst formation with a 5- to 10-day course of clomiphene citrate.

Treatment

Ovarian remnant syndrome is most effectively diagnosed and treated surgically. Surgical extirpation is often difficult and complex, however, and has a postoperative recurrence rate of 8 to 15%. Alternatives include medical treatment via hormonal suppression of ovarian function and radioablative therapy. Sonographically directed aspiration of cysts has also been suggested.

Prognosis

Malignancies have rarely been reported in ovarian remnant syndrome.

Fleischer AC et al. Sonographic features of ovarian remnants. J Ultrasound Med. 1998; 17:551. [PMID: 9733172]

Kaminski PF et al. Clomiphene citrate stimulation as an adjunct in locating ovarian tissue in ovarian remnant syndrome. Obstet Gynecol. 1990;76(5 Pt 2):924. [PMID: 2216258]

Narayansingh G et al. Ovarian cancer developing in the ovarian remnant syndrome. A case report and literature review. Aust N Z J Obstet Gynaecol.2000;40:221. [PMID: 10925917]

Scott RT et al. Use of the GnRH agonist stimulation test in the diagnosis of ovarian remnant syndrome. A report of three cases. J Reprod Med. 1995;40:143. [PMID:7738926]

Vavilis D et al. Ovarian remnant syndrome: a case report and review of the literature. Clin Exp Obstet Gynecol. 2000;27:121. [PMID: 10968351]

Pelvic Congestion Syndrome

Pathogenesis

Pelvic congestion syndrome is characterized by pelvic pain and dyspareunia associated with pelvic varicosities and pelvic venous congestion or stasis.

Clinical Findings

Women with pelvic congestion tend to have dull, aching, constant pain with premenstrual exacerbation. They also have episodes of severe, acute pelvic pain. The location of their pain tends to change or move, in contrast to the more consistent location of pain in women with CPP and other pelvic diseases. Women with pelvic congestion syndrome experience exacerbation of pain by walking, standing, lifting, bending, and stress. Deep dyspareunia and postcoital aching occurs in more than 50% of cases.

Deep abdominal palpation at the ovarian point, which lies at the junction of the upper and middle third of a line drawn from the anterior superior iliac spine to the umbilicus (Figure 17-3), reproduces the pelvic pain complained of by the patient. On pelvic examination, patients with pelvic congestion usually have bilateral ovarian tenderness.

Pelvic venography is performed to confirm the presence of pelvic varicosities and venous stasis and congestion. The two most common techniques used are selective retrograde ovarian venography and transuterine venography.

Treatment

Medical treatment with medroxyprogesterone acetate, 20 to 40 mg orally per day, or with GnRH agonists, such as goserelin at 3.6 mg subcutaneously implanted every 28 days, has been shown to significantly decrease pain. Adding psychotherapy to medical treatment may improve the clinical outcome. Radiologic transcatheter embolization of the ovarian or internal iliac veins has shown efficacy in observational studies.

Surgical treatment with hysterectomy and bilateral salpingo-oophorectomy has shown efficacy in women with venographically documented pelvic congestion syndrome who have not obtained long-term relief with medical therapy. Since most women with pelvic congestion syndrome are young, the benefits of extirpative surgery must be carefully weighed against the drawbacks of loss of fertility and the necessity of long-term hormone

P.265


replacement therapies. Also, the use of objective diagnostic criteria is crucial.

 

Figure 17-3. Location of the “ovarian point” a characteristic finding in pelvic congestion syndrome.

Soysal ME et al. A randomized controlled trial of goserelin and medroxyprogesterone acetate in the treatment of pelvic congestion. Hum Reprod. 2001;16:931. [PMID: 11331640]

Venbrux AC et al. Pelvic congestion syndrome (pelvic venous incompetence): impact of ovarian and internal iliac vein embolotherapy on menstrual cycle and chronic pelvic pain. J Vasc Interv Radiol. 2002;13(2Pt 1):171. [PMID: 11830623]

Pelvic Floor Tension Myalgia

Pathogenesis

Pelvic floor tension myalgia pain is caused by or associated with pain and tenderness of the levator ani, coccygeus, obturator, iliopsoas, or piriformis muscles or their associated fascia or insertions; pain is most often associated with the levator ani muscles. The pelvic floor muscles are major components of the musculoskeletal system of the pelvis and are frequently a source of CPP. The pain is often assumed to be analogous mechanistically to tension headaches. Many names (coccygodynia, pelvic floor myalgia, piriformis syndrome, levator ani spasm syndrome, and diaphragma pelvis spastica) have been used for pelvic floor pain, but it is not clear whether these multiple names contribute to further understanding of this syndrome.

Prevention

Abnormalities of posture are major contributors to the etiology of pelvic floor muscle pain. A marked kyphosis-lordosis, termed “typical pelvic pain posture,” has been noted in most women with CPP. Occasionally, the abnormal posture is just a marked lordosis. Correct posture may prevent pelvic floor tension myalgia in some cases.

Clinical Findings

Most women with pelvic floor tension myalgia have been treated for numerous other problems, such as endometriosis, pelvic inflammatory disease, low back pain, lumbar disc disease, or degenerative joint disease, prior to the diagnosis of pelvic floor tension myalgia. Symptoms are usually vague and poorly localized. Pain may be diffuse within the pelvis, more localized about the rectum or the anterior pelvis, or unilateral. Low back pain and radiation to the sacrum at the area of insertion of the levator ani is not uncommon (>80% of patients). Radiation to the hip and down the back of the thigh, like sciatica, may also be noted and is particularly characteristic of piriformis spasm. Pain is most often described as aching, throbbing, or heaviness. Similar to patients with pelvic relaxation disorders, patients may describe the sensation as “everything falling out or dropping.” Pain may be quite severe, and in some patients, has a characteristic of acute attacks that awaken the patient from sleep with rectal pain (“proctalgia fugax”) or vaginal pain (“colpalgia fugax”). Characteristically, pain from spasm of the levator ani starts in the afternoon and becomes progressively worse. Pain is increased by prolonged sitting or standing in one position. The pain is not usually worsened by bowel movements. However, dyspareunia is a common symptom.

The most common physical finding with pelvic floor tension myalgia is tenderness and spasm of one or more of the levator ani muscles. Digital pressure on the involved muscle typically reproduces or intensifies the patient's pain. It is not unusual for the tenderness to be unilateral. In some patients with pelvic floor tension myalgia, there is also tenderness of the coccyx, lateral sacrum, or sacrococcygeal ligaments.

The diagnosis of pelvic floor tension myalgia is by clinical history and physical findings. No imaging or laboratory evaluations are useful in establishing the diagnosis. However, as pelvic floor tension myalgia may frequently occur secondary to other pelvic pathology, evaluations may be needed to rule out diagnoses such as

P.266


endometriosis or adhesions. This is especially true if pelvic floor tension myalgia does not respond to appropriate physical therapy and medical treatment.

Treatment

The classic treatment for pelvic floor tension myalgia involves massaging the tender muscles with a firm sweeping motion. At each treatment session, 15 to 20 strokes are done, taking about 5 minutes. Treatments are repeated daily for 4 to 5 days, then every other day until improvement. About six sessions are usually needed. The initial sessions are usually quite uncomfortable, and most patients note an exacerbation of pain after the first one or two treatments. Many women find it less unpleasant if the technique is modified to transvaginal massage rather than transrectal.

Hot sitz baths may also be helpful. Vaginal and rectal diathermy, ultrasound, bed rest, relaxation exercises, biofeedback training, analgesics, skeletal muscle relaxants, Kegel exercises, transcutaneous electrical nerve stimulation, acupuncture, vaginal electrical stimulation, and infiltration with corticosteroid or local anesthetic (especially if trigger points are present) are all treatments for pelvic floor tension myalgia. There are no well-performed studies that allow a recommendation of any specific treatment modality or combination at this time. Coccygectomy is sometimes done for this syndrome but probably has little, if any, role in the modern management of the problem.

Tu FF et al. Musculoskeletal causes of chronic pelvic pain: a systematic review of diagnosis: part I. Obstet Gynecol Surv. 2005;60:379. [PMID: 15920438]