Abstract
Chronic pelvic pain is a debilitating condition that can affect both men and women. Often the symptoms can overlap with multiple systems, causing it to be misdiagnosed, symptoms dismissed, and thereby challenging for patients and clinicians. Many organ systems can be involved including musculoskeletal, gynecologic, urologic, gastrointestinal, rheumatologic, oncologic, neurologic, and/or psychological components. It often affects multiple aspects of a patient’s life from difficulty with social integration, limitations in employment, and issues in personal relationships. A thorough history and physical exam is key to providing a clear picture of the diagnoses that are involved. Laboratory testing and imaging modalities can also further improve diagnostic accuracy. The treatment is typically multimodal, including but not limited to cognitive-behavioral therapy, physical therapy aimed at the pelvic floor, pain interventions such as injections, oral medications, and possibly surgery depending on the diagnosis.
Keywords
Abacterial prostatitis, chronic pelvic pain, endometriosis, pelvic pain syndrome, pudendal neuralgia
Synonyms | |
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ICD-10 Code | |
N41.1 | Chronic prostatitis |
N73.9 | Female pelvic inflammatory disease, unspecified |
N30.10 | Interstitial cystitis (chronic) without hematuria |
N94.10-19 | Dyspareunia |
R10.2 | Pelvic and perineal pain |
Definition
Chronic pelvic pain (CPP), a common condition among women, affects up to one in four women of reproductive age at some point in their lifetime. For men, chronic pelvic pain in literature has been used interchangeably with chronic abacterial prostatitis (CAP). The use of CAP as an all-encompassing term for men suffering from chronic pelvic pain may have led to inaccurate classification and thereby mistreatment and misdiagnosis. For both men and women suffering from CPP, it can be an elusive disorder to diagnose and to treat, challenging even the most experienced clinicians. Despite variable opinions as to what constitutes this disorder, a widely accepted definition of CPP is noncyclic pain localized primarily in the anatomic pelvis, the anterior abdominal wall at or below the umbilicus, the lumbosacral spine, or the buttocks. Traditionally, CPP must be of 6 months’ duration and severe enough to cause functional disability or to require treatment. It can be of gynecologic, urologic, gastrointestinal, musculoskeletal, neurologic etiology, or a combination thereof. The pain that arises from CPP can be categorized into somatic, visceral, neuropathic, or referred pain.
The prevalence of CPP is estimated to be 3.8% in women aged 15 to 73 years, similar to that of asthma, back pain, and migraine headaches. For men, CAP has a prevalence of 10% for men ages 20 to 74 based on studies by the National Institutes of Health Chronic Prostatitis Symptom Index. In primary care practices, it is estimated that 39% of women have complained of pelvic pain. For men younger than 50, prostatitis discomforts are the most common urologic disorder and the third most common urologic disorder for older men.
Discovering the etiology of CPP (and therefore appropriate treatment) can be difficult because of the broad differential diagnoses and their many overlapping symptoms (see List 106.1 for a complete list by organ system). In addition, these diagnoses are not mutually exclusive but in many cases may coexist. For example, endometriosis or prostatitis can often overlap with myofascial pain.
The initial diagnostic approach should begin with a thorough history to narrow the differential diagnosis. The pain history should include pain characteristics such as first occurrence, location, duration, temporal pattern, precipitating and alleviating factors, relationship to urination and defecation, patterns of radiation, intensity, and effect of pain on life activities (such as activities of daily living, sitting, sleep, work, sexual intercourse, and social or recreational activities). A monthly pain calendar, which records episodes, location, severity, and associated factors, is useful to obtain accurate and detailed information. A thorough review of systems and history should also include prior treatments, history of substance abuse, sexual, physical, and psychological abuse, infections history, previous surgeries, and reproductive history. To streamline the process of obtaining a history for patients with CPP, the International Pelvic Pain Society has created the Pelvic Pain Assessment Form, which is an excellent and freely reproducible tool that can be found on its website.
The following are common causes of CPP.
Adhesive Disease
The correlation between abdominal adhesions and CPP is poorly understood, and studies of these are limited. It is thought that certain types of adhesions, particularly densely vascular adhesions to the bowel and peritoneum, cause CPP. Diagnosis can be made only at the time of laparoscopy, as there are no examination findings, laboratory tests, or imaging studies that are reliably useful. Risk factors include a history of prior pelvic surgery including cesarean sections, pelvic inflammatory disease (PID), endometriosis, inflammatory bowel disease, radiation therapy, and peritoneal dialysis.
Chronic Pelvic Inflammatory Disease
PID is an inflammatory disorder of the upper female tract that could involve salpingitis, tubo-ovarian abscess, pelvic peritonitis, and endometritis. PID can initially be a self-limiting acute diagnosis but can progress to a chronic condition, resulting in CPP. The transition from acute PID to chronic PID is incompletely understood and occurs in about 18% to 35% of women with acute PID. Women at risk for development of chronic PID include those who experience delayed or incomplete treatment. Infertility can be a sequela. Diagnostic criteria include clinical findings of lower genital-tract inflammation with clinical presentation of increased leukocytes in vaginal secretions, cervical friability, and cervical exudate in conjunction with three minimal criteria as listed below:
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oral temperature >101°F (>38.3°C);
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abnormal cervical or vaginal mucopurulent discharge;
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presence of abundant numbers of WBC on saline microscopy of vaginal fluid;
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elevated erythrocyte sedimentation rate;
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elevated C-reactive protein; and
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laboratory documentation of cervical infection with Neisseria gonorrhoeae or Chlamydia trachomatis.
Gastrointestinal: Irritable Bowel Syndrome
Irritable bowel syndrome is a common functional bowel disorder of uncertain etiology characterized by a chronic, relapsing pattern of abdominopelvic pain and altered bowel habits in the absence of an organic cause. Although not all patients with this disorder seek treatment, the estimated prevalence is 14.1% in North America with about only 3.3% diagnosed medically. The abdominal and pelvic pain is usually crampy in nature and varies in location, often exacerbated by emotional stress and eating habits and relieved by defecation.
The Rome III criteria are used to diagnose irritable bowel syndrome, and patients must have two of the following: pain relieved with defecation; onset of pain associated with a change in frequency of stool; or onset associated with a change in form (appearance) of stool.
Gynecologic: Endometriosis, Uterine Leiomyomas, and Adenomyosis
Endometriosis is a common gynecologic condition affecting women of reproductive age. It is characterized by the presence of endometrial tissue that is found outside of the uterus. It often affects the ovaries, fallopian tubes, ureter, peritoneum, bowel, bladder, and in rare cases the lungs, cesarean section scars, appendectomy scars, and episiotomies. The extrauterine endometrial implants respond to the hormonal stimuli in the same way as intrauterine endometrium does, causing cyclic bleeding in the sensitive tissues of the peritoneum, ovaries, fallopian tubes, and elsewhere. This process can lead to formation of pelvic adhesions, scar tissue, and endometriomas.
This disorder is found in 10% to 15% of women of reproductive age, in 25% to 40% of women undergoing treatment for infertility, and noted in 33% of women who have undergone laparoscopy for CPP. Risk factors include early menarche, short menstrual cycles (<27 days), and müllerian anomalies that involve vaginal or uterine obstruction of blood flow. Symptoms include pelvic pain, dysmenorrhea, menorrhagia, deep dyspareunia, backache, painful bowel movements, fatigue, infertility, irregular bleeding, constipation, menstrual diarrhea, pain with exercise, painful pelvic exams, painful urination, as well as bloating.
Uterine leiomyomas (uterine fibroids or myomas) are benign smooth muscle tumors of the uterus and the most common neoplasm in women of reproductive age. There appears to be a racial predilection for African Americans. A study carried out in the United States with randomly selected women between the ages of 35 and 49 years showed that the incidence of uterine fibroids by age 35 were noted to be 60% among African American women, increasing to greater than 80% by age 50, whereas Caucasian women showed an incidence of 40% by age 35, and almost 70% by age 50. Fibroids are thought to grow from estrogen stimulation, as the fibroids rarely appear before menarche and regress after menopause. The most common symptoms are sensation of pressure in the pelvis, pain, dysmenorrhea, and abnormal uterine bleeding. The severity of symptoms is related to the size, number, and location of the tumors, although many women with fibroids are asymptomatic. A myoma that grows rapidly after menopause is concerning for leiomyosarcoma. Uterine leiomyosarcoma is a rare neoplasm with an annual incidence of 0.64 per 100,000 women.
Similar to endometriosis, adenomyosis is the presence of ectopic endometrial tissue in the myometrium of the uterus without direct connection to the endometrium. Adenomyosis develops from aberrant glands of the basalis layer of the endometrium and causes pain before, during, and after menses. In addition, women may suffer from menorrhagia, discomfort after orgasm, and vigorous exercise. Some women experience intense pelvic cramping and pressure that radiates to the lower back, groin, rectum, and anterior thighs. Symptomatic adenomyosis usually is manifested in women aged 35 to 50 years, although adenomyosis can be found in asymptomatic women.
Musculoskeletal
Myofascial pelvic pain is pain attributed to short, spastic, weaker, and tender pelvic floor muscles scattered with characteristic trigger points, small hypersensitive areas within a tight band of skeletal muscle. A trigger point can cause local pain and over time lead to regional and diffuse pelvic pain as well as visceral dysfunction, such as constipation or irritative voiding. The exact prevalence of myofascial pelvic pain syndrome is unknown. Determining if CPP is due to a myofascial etiology or caused by another disorder can be challenging. In fact, overlap between pelvic pain disorders is common. In men with chronic pelvic pain syndrome, 13% to 14% have pain in the external and internal pelvic floor. Inflammatory mediators are released locally when a muscle is injured. Over time, the muscle nociceptors become conditioned to the stimulus and a lower response threshold to inflammatory mediators and mechanical stimulation ensues, leading to muscle hyperalgesia.
Physical, mechanical, systemic, and psychological factors have been associated with the development of trigger points. Physical factors include injury to the nerves and muscles at the time of childbirth or surgery. Mechanical factors that contribute to myofascial pelvic pain include abnormal posture, leg length discrepancy, gait disturbances, sacroiliac joint dysfunction, and diastasis recti, all of which can trigger asymmetric use of the levator muscles. Systemic factors such as subclinical hypothyroidism, nutritional inadequacies, chronic allergies, and impaired sleep have also been linked to the development and activation of trigger points.
Patients with myofascial pelvic pain will complain of pain when pressure is placed on myofascial trigger points, which are often found in the pubococcygeus and iliococcygeus muscles of the pelvic floor.
Pelvic Congestion Syndrome
Pelvic congestion syndrome is a condition of vascular engorgement of the ovarian veins or internal iliac veins that leads to pelvic pain. Characteristic findings of gross dilation, incompetence, and reflux of the ovarian veins are seen on venography. Dysfunction in the one-way valves of the ovarian veins is postulated as the underlying etiology. There is limited understanding of the prevalence of this condition, as there are no definitive diagnostic criteria. Pelvic congestion syndrome has been described only in premenopausal women, typically in late 20s or early 30s, and are more likely to have had children than not. Typically, pain is worse after prolonged standing, walking, or lifting and improves in the morning after rest and while lying down.
Urologic
Chronic bladder pain in the absence of other identifiable etiology has been termed painful bladder syndrome (PBS). PBS has also been known as interstitial cystitis (IC); however, this is actually a misnomer, as there is no evidence of underlying evidence of inflammation. The prevalence of this syndrome varies country to country from 8 in 100,000 in the Netherlands to 500 in every 100,000 in the United States. Accurately diagnosing this syndrome is difficult, in part due to the lack of standardized criteria. Symptoms which characterize PBS/IC include nocturia, dysuria, urinary frequency, and pain. PBS/IC often coexists with other chronic pain syndromes, such as fibromyalgia, irritable bowel syndrome, or myofascial pelvic pain syndrome. The underlying etiology has not been clearly elucidated, but may be related to altered integrity of the glycosaminoglycan layer of the bladder, possibly as a result of infective or inflammatory pathways. Sensory nerve sensitization and mast cell upregulation are other possible underlying etiologies.
Symptoms
The specific symptoms of CPP vary greatly but are important to elicit because they can give diagnostic clues. When pain is cyclic in nature, this suggests a hormonal component and should consider endometriosis or polycystic ovary syndrome. Entry dyspareunia may be a symptom of lichen sclerosis, atrophic vaginitis, or vulvodynia; deep dyspareunia may be a symptom of endometriosis, myofascial pelvic pain syndrome, or chronic PID. Nearly 90% of women with localized vulvar pain syndrome describe their symptoms as a burning sensation localized to the vulvar vestibule. Improvement of pain while supine and aggravation of pain while upright suggest pelvic congestion syndrome.
Pain accompanied by symptoms of poor urinary flow, urinary hesitancy, or constipation suggests myofascial pelvic pain, prostatitis, pelvic floor muscle dysfunction, or an anatomic defect such as pelvic organ prolapse. Pain that increases with bladder filling and is associated with urinary frequency, nocturia, and painful voiding suggests PBS/IC. Lateralizing pain accompanied by hematuria may be indicative of urolithiasis or urinary tract obstruction.
Pain with radiation to the lower extremities may indicate myofascial pelvic pain, sacroiliac joint dysfunction, or spinal disease, such as radiculopathy or cord compression.
Crampy abdominal pain with diarrhea or constipation suggests irritable bowel syndrome or diverticular disease.
Physical Examination
After a thorough history and review of systems, the clinician narrows the differential diagnosis. The goal of the physical examination is to reproduce, whenever possible, the patient’s pain. Therefore it is important for the clinician to remember that this process can be a stressful and unpleasant experience for the patient. The physical examination—indeed, the entire evaluation—should be systematic and methodical. In the case of CPP, a multispecialty approach to both diagnosis and treatment is now considered standard of care. Therefore clinicians commonly treating patients with CPP should be familiar with the basic physical examination of the multiple organ systems around the pelvis that may give rise to pain symptoms. These clinicians ideally function within a team of experts from various specialties such as gastroenterologists, colorectal surgeons, gynecologists, primary care physicians, psychiatrists, psychologists, and urologists to ensure a complete diagnostic approach.
After a screening examination of the lumbosacral spine is performed, the examination of the pelvis continues with the patient supine. The patient should be asked to identify the areas of most pain. A four-quadrant abdominal examination is then performed with superficial and deep palpation (ideally with the patient’s abdomen relaxed), leaving the area of most pain until last, and taking note of discomfort, hernia, masses, and surgical scars. If hypersensitivity is found on the superficial examination, this can suggest myofascial trigger points. A simple maneuver, the Carnett test, can be used to differentiate myofascial pain from visceral abdominal pain. For the Carnett test to be performed, the area of pain on the abdomen is palpated with one or two digits, and then the patient voluntarily contracts the abdomen by raising legs and head off the table. If pain at the palpation site increases, this is likely a myofascial trigger point, and if pain decreases, it is likely visceral in origin.
The examination continues in the dorsal lithotomy position. The patient should again show the clinician where the area of most pain is and this area should be examined last. First, begin with examination of the external pelvis, looking at the groin, external genitalia, and perineum for asymmetry or dermatologic abnormality such as thickened skin or tears. For females, the soft end of a cotton swab can be used to assess pain in the vestibule, vestibular fossa, and labia ( Fig. 107.1 ); if pain is present, it can be indicative of vulvodynia or vulvar skin disorders. When examining male patients, notice the urethral meatus, presence or absence of foreskin, perineum, corona, shaft, scrotum, contents of spermatic cord, testicles, and presence of hernias ( Fig. 107.2 ). Male and female external anal sphincter must be evaluated for tone as well as for any gross abnormalities that may be causing pain ( Fig. 107.3 ).