D. Abdomen, Pelvis, and Genitalia

Abdominal pain is one of the most common presenting complaints in the primary care physician’s office and often a diagnostic dilemma for surgeons. Despite recent technologic advances, the diagnosis and treatment of chronic, recurrent abdominal pain remain a challenge. Pain is a subjective sensation that patients often find difficult to describe. By contrast with other areas of the body, the abdominal organs have poorly developed sensory systems that also may contribute to the patient’s difficulty when trying to describe and localize the pain. In the majority of clinical scenarios, no physical course is apparent and symptoms are transient. The purpose of pain is to protect the organ and the patient from injury. After the source of the pain is found, every effort should be made to control or eliminate it. In chronic pancreatitis or diffuse malignancy, for example, pain control may become as much a challenge as in instances where no underlying cause can be found.

For a patient to perceive pain, the autonomic nervous system must be intact. The anatomy and physiology of pain have been described in detail in previous chapters. Abdominal viscera are relatively insensitive to many stimuli compared with a more sensitive organ such as the epidermis. In addition to the relative paucity of sensory nerve endings, the same group of nerves may innervate several viscera. There are a few well-known nociceptive triggers in the abdominal cavity. These include abnormal distention or contraction of hollow organ walls, ischemia of the visceral musculature, direct action of chemical substances on the mucosa, formation of allogenic mediators, and traction or compression of ligaments, vessels, or mesentery. Pain patterns are not well differentiated as to their location or the cause. Nevertheless, there are some recognizable pain patterns, and a careful history often can lead to the correct diagnosis. The history and physical examination provide diagnosis in two thirds of clinical presentations. Laboratory and radiologic tests are important auxiliary tools for investigative workup. The invasiveness and cost-effectiveness of the proposed tests should always be considered.

There is either a physical (organic) or psychogenic (nonorganic) cause to pain, with one or more elements dominating. Abdominal pain can be classified by the duration of the symptoms, etiology, primary diagnosis, anatomic localization, and its response to treatment. It can be either acute or chronic; chronic pain being arbitrary, based on symptoms persisting for more then 6 months or after the healing process is completed. Some clinicians divide pain into nociceptive (somatic or visceral), neuropathic, psychogenic, or referred.

Visceral pain is transmitted from nociceptors found on the walls of the abdominal viscera via sympathetic (thoracic branches and lumbar splanchnic nerves synapsing in subsidiary plexuses: celiac, splenic, hepatic, aorticorenal, superior mesenteric, adrenal) and parasympathetic (vagus and nervi erigentes S2–4; motor and sensory) pathways. This pain is nonspecific because of wide divergence and relatively small number of afferent fibers innervating a large area with extensive ramification. Patients usually have difficulty localizing the source of pain and will describe it as aching, cramping, or burning that fluctuates in intensity. Visceral pain usually is paroxysmal, colicky, deep, squeezing, and diffuse. Still, today, a theoretical explanation for transduction and transmission of visceral pain is being used.

Centralistic and peripheralistic theories of pain are widely recognized. The centralistic theory is subdivided into the convergence projection and convergence facilitation views. The former states that visceral fibers with nociceptive input converge onto somatosensory spinal neurons, and that the viscerosomatic cells project through nociceptive pathways. The convergence facilitation theory focuses on the fact that visceral activation changes the excitation of multiple spinal units (including pain pathways) without direct activation of spinal neurons. The peripheralistic theory claims that vasoactive substances released into cutaneous and deep tissues lead to hyperalgesia and referred pain from these structures.

Differentiating somatic from referred pain can be accomplished by reviewing the patient’s history and selective blocks. Somatic pain tends to be more intense or sharp and is aggravated by movement. It is produced by the stimulation of nociceptors in the parietal peritoneum and intra-abdominal connective tissues. It is usually well localized and described as constant, aching, gnawing, and throbbing. It can be relieved by opiates and peripheral nerve blocks. Referred pain can be explained easily by our knowledge of the segmental distribution of the spinal nerves. The pain can be referred to remote areas of the body if visceral impulses enter the spinal cord at the same level as afferent nerves from another area. These impulses are mistakenly interpreted as pain initiating at the second site. In addition, the pain may spread cranially to adjacent segments. Lower lobe pneumonia may be associated with severe upper abdominal pain and even some associated guarding. This is the most frequently used example of referred pain (Fig. 34-1).

Examination of the patient with abdominal pain must be done through an organized approach. Description of current and previous disease (including psychiatric treatment) in conjunction with a social and family history will develop a picture of the pain and a presumptive diagnosis. A history of allergies and current medications must be elicited. Knowledge of the pain pattern and character is also important. Following this, the patient should be carefully examined. A useful algorithm is presented in Figures 34-2 and 34-3.

The patient can differentiate superficial abdominal pain easily. A local process in the abdominal wall usually causes it. Pain over the xiphoid or costal margins that is exacerbated by movement or touch often is attributable to costochondritis, a form of arthritis that usually responds to appropriate therapy with anti-inflammatory agents or intra-articular injections. Sharp, burning pain near a recently healed incision may indicate nerve irritation from surgical transection, entrapment in the healing tissue, or regeneration with neuroma formation. Pain originating in the abdominal wall frequently can be confirmed by Carnett’s test and obliterated by blocking the appropriate intercostal or paravertebral nerves. Block of the celiac plexus can be helpful in differentiating abdominal wall or genitourinary pain from intraabdominal visceral pain, because the plexus supplies all abdominal viscera except the rectum, sigmoid, bladder, and reproductive organs.

The more common deep abdominal pain is, by definition, either somatic or visceral (autonomic: sympathetic and parasympathetic). Somatic pain originates in the parietal peritoneum, the root of the mesentery, and is elicited in close proximity to the nociceptive source. It is intense, sharp, precisely localized, associated with external stimuli, and represented at the cortical levels. Severity is related to the intensity of stimulus. Visceral pain is described more often as a poorly localized, vague (can be colicky, cramping, squeezing, dull, aching) pain associated with internal factors. Sometimes the pain can be referred to another part of the body. It can be presented as diffuse midabdominal discomfort. The intensity of the stimulus is as essential as its quality (i.e., cutting or coagulating the bowels is painless, but distention will cause pain). It is a primarily reflex and can be represented at the cord levels. Sometimes severe, visceral pain may generate a secondary physiologic reaction mediated by the autonomic nervous system and manifested by nausea, vomiting, sweating, lightheadedness, and salivation

The pain from the stomach, pancreas, and hepatobiliary tree is referred to the epigastrium. Periumbilical localization occurs from the small bowel and right colon; the rest of the colon and the genitourinary organs cause pain that presents in the epigastrium. The common midline pain location is a result of the bilateral innervation of the abdominal organs from both sides of the spinal cord (Fig. 34-4).

To determine the source of the pain, it is important to assess its intensity, location (Fig. 34-5), and character. It is also important to note its onset, whether acute or insidious, and its temporal profile. The circumstances that intensify or alleviate the pain are significant. Relief with eating or antacids suggests ulcer disease or gastroesophageal reflux. Postprandial pain, depending on its location, character, and timing, could be biliary, ischemic, or associated with a more benign condition, such as lactose intolerance or irritable bowel syndrome. Seasonal patterns frequently are seen in ulcer disease and, occasionally, with regional enteritis. The pain of inflammatory bowel disease and irritable bowel syndrome may be relieved by defecation, whereas heat usually relieves pain of musculoskeletal origin. Posture, sudden movement, coughing, straining, and sneezing may worsen the pain from peritoneal irritation or of spinal origin. The abdomen is not exempt from psychogenic pain. This may be manifested as a component of irritable bowel syndrome. Although common, psychological pain should and does remain a diagnosis of exclusion.

Esophagus

Heartburn is the most common symptom referable to the esophagus. It is a burning or hot substernal discomfort that frequently moves up toward the neck, but it may be localized only to the epigastrium. Eating, bending down, lying down after eating, and occasionally vigorous exercise may precipitate it. It is not entirely clear whether heartburn is caused by the chemical irritation of acid or bile, or if secondary muscle spasm plays a role. Occasionally, the pain is described as being a heaviness or tightness in the chest, with secondary restricted respiration and subsequent shortness of breath, simulating myocardial ischemia. The shortness of breath may be caused by an intercostal muscle spasm mediated by spinal reflex arcs.

Usually, the pain from the esophagus is felt at the level of the lesion. In some patients, however, pain caused by a lesion in the lower third of the esophagus is felt in the throat or in the high retrosternal area. The opposite is uncommon. When heartburn is severe, such as that associated with an ulcerating or infiltrating process, esophageal pain can radiate into the back, between the shoulder blades.

Stomach and Duodenum

The character of pain from ulcer disease varies widely. Typically, it is located in the epigastrium. It may be a sharply localized burning or gnawing pain or just a vague discomfort occurring from ½ to 2 hours after eating. Occasionally, it occurs shortly before meals or on an empty stomach and it may wake the patient up in the early hours of the morning. Food or antacids relieve it. The pain may, at times, be more localized to the right or left upper quadrant. When the pain bores through into the back, it usually indicates a posterior duodenal wall ulcer with secondary irritation of, or penetration into, the pancreas. This pain usually is deep, persistent, poorly localized, and does not respond well to treatment. Unlike heartburn, ulcer pain frequently occurs in clusters; several weeks of daily pain may be followed by variably long pain-free intervals. There may be seasonal variation with the symptoms; that is, they may be worse during the spring and fall.

Pain from gastritis tends to be more persistent and may be more difficult to abolish. The associated nausea and vomiting may be particularly troublesome. As in heartburn, it is not known whether the pain is produced by acid irritation of the nerve endings in the ulcer bed or whether it is secondary to a spasm of antral or duodenal smooth muscle.

Epigastric pain occurring soon after eating, unrelieved by antacids, and with lack of periodicity does not necessarily exclude ulcer disease. Pyloric channel ulcers may present in such a manner, and unless there is associated postprandial vomiting, the diagnosis may not be made until frank gastric outlet obstruction occurs.

Small Intestine

As a rule, pain originating in the small intestine is periumbilical in location and crampy or colicky in nature. Jejunal lesions tend to be associated with pain in the left upper quadrant. Ileal pain tends to localize in the right lower quadrant, and it may result from abnormal bowel motility patterns. A lowered threshold to the pain of bowel distention or contraction also can cause it. A lesion obstructing the lumen of the bowel, such as regional enteritis or a malignant process, may be the precipitating factor.

The pain of irritable bowel syndrome frequently is chronic, and at times, it can be incapacitating. It is unusual, however, for it to wake the patient from sleep. The pain usually is in the lower abdomen, in either the right or left lower quadrants. Its description ranges from burning, sharp, and stabbing to dull. Most commonly, it is intermittent, but it may be constant, with superimposed acute attacks. The pain may remain localized or may migrate with time. Eating usually precipitates it; defecation or fasting tends to relieve it. Nausea, bloating, and dyspepsia frequently occur and may simulate peptic ulcer or biliary tract disease. A change in bowel habits is not a universal finding, but, classically, diarrhea alternates with constipation. Predominant diarrhea or constipation, however, can be part of the syndrome.

Pain from partial small bowel obstruction also occurs after meals. The closer the lesion is to the stomach, the earlier the pain occurs. Moreover, nausea and vomiting are more likely to occur when the lesion is close to the stomach. The pain frequently is described as crampy and comes in waves. Regional enteritis is suggested by localization of the discomfort to the right lower quadrant and associated diarrhea, fever, weight loss, or extraintestinal manifestations, such as arthritis and mouth ulcers. Significant weight loss and cachexia may suggest an underlying lymphoma or metastatic disease to the bowel. It may be several months before complete obstruction occurs. At this time, the diagnosis is more obvious. As in appendicitis, the initial pain may be a nonspecific discomfort, but as the underlying process develops and eventually involves the overlying peritoneum, the pain localizes and approximates the site of the underlying disease.

Postoperative adhesions frequently are blamed for chronic or recurrent abdominal pain. Before exploration is considered, definitive evidence of bowel obstruction using plain abdominal x-rays or angulation and proximal dilation of the bowel using a barium study should be documented.

Colon

Pain from the colon usually is poorly localized to the lower abdomen. However, an adenocarcinoma of the colon or diverticula of the colon with secondary microperforation and abscess formation may have localized symptoms overlying the area of disease. Pain from the rectosigmoid area, in addition to being in the left lower quadrant, may be located in the sacral area.

Pancreas, Liver, and Biliary Tract

Because the pancreas, liver, biliary tract, stomach, and duodenum share some of the same afferent neuropathways, it is easy to understand some of the difficulties involved in the differential diagnosis of chronic epigastric pain. Diseases of the pancreas, and, in particularly, pancreatic cancer, are the most difficult to diagnose. Pain resulting from pancreatic cancer usually signifies infiltration of the retroperitoneal area or celiac axis, or spread to surrounding organs. Some of the pain may be a result of pancreatic duct obstruction and surrounding pancreatitis. Tumors in the head of the pancreas cause pain that is more localized to the epigastrium or right upper quadrant. Those in the tail tend to cause pain in the left upper quadrant. Lesions in the body of the pancreas can cause the pain to radiate into the back. Back pain, alone, also can be a presenting symptom. Because pancreatic cancer is rarely resectable, the physician is able to treat only the symptoms. When the retroperitoneal celiac plexus is involved by the tumor, the anesthesiologist frequently is asked to guide and help manage the pain.

The pain of chronic pancreatitis, which is mainly a result of alcohol abuse, can be constant, debilitating, and frequently lead to drug abuse. The persistent inflammation of the pancreas causes some of the pain, as does the ductal distention secondary to ductal obstruction by creation of strictures. The pain may be dull or sharp, burning, and steady. It commonly radiates into the back. Superimposed, more acute attacks last from days to several weeks. Eating, moving, or lying down may aggravate the pain; sitting up or leaning forward may relieve it. In patients who are not surgical candidates, the anesthesiologist may be asked to intervene. Neurolytic celiac plexus block has been used to treat patients who have not responded to conservative or surgical therapies. It often is used as a last resort, because rendering the abdomen insensitive may allow future abdominal disease to be missed.

Placement of stents during endoscopic retrograde cholangiopancreatography was used initially to decompress a dilated biliary tree. Only recently, this technique has been used to decompress the pancreatic duct. Relief may occur if the dilated biliary (or pancreatic) duct was the cause of the pain. Both benign and malignant lesions are amenable to this technique.

Passing a stone or biliary tree dilation secondary to an obstruction usually causes biliary pain. Contrary to the commonly used term biliary colic, the pain tends to have a gradual onset. After it peaks, it tends to reach a plateau until again, hours later, it diminishes. An attack can last from several hours to a day or more. The pain characteristically is localized in the right upper quadrant and may radiate to the right shoulder and shoulder blade, but it also is felt commonly in the epigastrium, with radiation into the back. Vomiting occurs in most patients and may provide some relief. After an acute attack, residual soreness may persist for several days. Commonly, these symptoms occur after eating, but they may become constant if the common bile duct is impacted by a stone or infiltrated by a malignant process. Associated dyspepsia is common in approximately one quarter of all patients. It responds to antacids, further confusing its cause. The appearance of complicating cholangitis (with symptoms of fever and jaundice) usually leads quickly to the correct diagnosis.

The liver parenchyma is insensitive to pain, but relatively rapid distention of the liver capsule will initiate well-localized right upper quadrant pain. Acute processes such as viral hepatitis, alcoholic hepatitis, and cardiac decompensation with secondary liver congestion rarely may appear as right upper quadrant pain, but this pain does not evolve into a chronic complaint. Chronic, active hepatitis may follow a course of recurrent attacks of right upper quadrant pain. The pain usually is well localized and accompanied by worsening liver function tests.

Benign focal nodular hyperplasia or adenomas associated with the use of birth control pills may cause recurrent right upper quadrant discomfort or, occasionally, a dramatic crisis of severe abdominal pain and hypotension from a hemorrhage into the capsule or peritoneum. The recurrent warning pains probably are caused by small bleeding episodes into the lesions. Bleeding into or necrosis of malignant lesions in the liver causes similar pain, but this usually is accompanied by fever and jaundice. The pain may be well localized, sharp, and steady. Any movement producing friction between the liver surface and the ribs may exacerbate it. When sought, a bruit over the lesion may he identified in approximately 25% of patients.

Vascular Diseases of the Bowel

Although mainly asymptomatic, occlusive vascular disease may be associated with chronic, recurrent, dull paraumbilical or epigastric pain. The pain of intestinal angina begins approximately ½ hours after eating and lasts throughout digestion and absorption of the meal. Usually, at least two of the three major splanchnic vessels are affected by significant obstruction from atherosclerotic changes. It is postulated that the collateral supply is insufficient to meet the increased need during digestion, and this state of relative ischemia creates subsequent pain. Classically, this recurrent pain may create a fear of eating and can lead to severe weight loss. When patients with acute ischemia are questioned closely, retrospectively, they often will report postprandial abdominal discomfort preceding the acute event by weeks to months.

Superior mesenteric artery syndrome and celiac compression syndrome frequently are mentioned in the differential diagnosis of chronic abdominal pain, but their validity is controversial. Superior mesenteric artery syndrome is described as occurring in so-called asthenic patients or patients with significant weight loss. The postprandial epigastric pain, vomiting, and distention are believed to be caused by compression of the duodenum by the superior mesenteric artery. The pain of celiac compression syndrome is not necessarily related to meals. The celiac axis frequently has a high take-off and may be compressed by the median arcuate ligament of the diaphragm or by the tissue of the celiac ganglion. Whether bowel ischemia is the cause of the pain or whether the pain originates from the celiac ganglion also is unclear.

Abdominal aortic aneurysm usually presents more acutely, but a slowly expanding or leaking aneurysm may be associated with recurrent, dull midepigastric or back pain over several months. As with pancreatic pain, sitting up or leaning forward occasionally may relieve this pain. The pulsating, sometimes tender, mass can be palpated, and a bruit may be heard.

Peritoneum

The parietal peritoneum is well innervated by the branches of the spinal nerves, and, consequently, the pain perceived is well localized. Such pain frequently is associated with secondary muscle spasm of the overlying abdominal wall. The visceral peritoneum, however, has no pain receptors, and any pain that is generated is poorly defined. A malignant process most often causes chronic pain originating in the peritoneal cavity. Metastatic bowel or ovarian tumors and lymphoma are common. Mesothelioma is the most common primary tumor; teratoma, carcinoid, or sarcoma are much less common. The abdominal discomfort often is compounded by the presence of ascites, which distends the peritoneum further and causes more pain.

In young patients of Mediterranean descent who have chronic recurrent attacks of sudden diffuse or localized peritoneal pain, familial Mediterranean fever should be considered. Abdominal tenderness, fever, and arthritis are common. Despite feeling very ill, the patient recovers in several days and is well until the next attack.

Mesentery and Omentum

Recurrent localized or generalized abdominal pain in an older patient associated with the displacement of the stomach or bowel on x-ray studies should suggest a mesenteric or omental lesion. Fever, weight loss, nausea, vomiting, and a palpable tender mass may be associated with mesenteric panniculitis or retractile mesenteritis. Metastatic tumors to the mesentery are more common than primary lesions. The latter usually are fibromas, myomas, histiocytic or lipomatous tumors. Leiomyomas and leiomyosarcomas tend to involve the omentum.

Genitourinary System

The acute pain of renal colic or pyelonephritis is classic and easy to diagnose. The triad of hematuria, flank pain, and a palpable mass suggests renal cell carcinoma, but this triad occurs infrequently. The tumor often is diagnosed from systemic complaints (dull upper abdominal or flank pain may be included). Perinephric abscess, although uncommon, should be considered in a patient with a history of urinary tract infections or pyelonephritis. Typically, dull upper quadrant or flank discomfort is present, accompanied by malaise and low-grade fevers.

Gynecologic problems usually cause acute pain. Depending on the patient’s age, chronic lower abdominal pain, usually more localized to one of the lower quadrants, could be a presenting symptom of chronic pelvic inflammatory disease, uterine or ovarian cancer. The pain can be dull, steady, or crampy. The local irritation by the mass or inflammatory process can cause changes in the urine or bowel pattern.

Metabolic causes of chronic abdominal pain are rare but should always be sought, particularly when the usual diagnostic avenues have been exhausted. The hepatic porphyrias have many features in common, including their clinical presentation. Abdominal pain is the most prominent symptom. It is thought to result from autonomic neuropathy, which causes disturbances in gastrointestinal motility. Spasm and dilation of the bowel can cause severe pain. The frequent association of fever and leukocytosis mimics an inflammatory process. Although vomiting and constipation frequently are present, the abdomen is soft, without marked tenderness. These attacks may last from days to weeks. Fasting, infections, the menstrual cycle, and drugs whose metabolism involves the hemoproteins of cytochrome P-450 are often the precipitating factors. Such drugs include alcohol, barbiturates, anticonvulsants, estrogens, and contraceptives. Knowledge of so-called safe or probably safe drugs may be useful to the clinician treating the pain. Drugs that are considered safe include morphine and related opiates, which may be required to treat the pain in an acute attack.

In the United States, lead poisoning mainly is encountered in children. Although anemia, peripheral neuritis, and encephalopathy complete the picture, the young patient may have only lead colic, sometimes called painter’s cramps. Severe, migrating, poorly localized guarding and rigidity of the abdominal wall should raise the suspicion of an acute intraabdominal event that may accompany crampy abdominal pain.

When in a hemolytic crisis, a patient with paroxysmal nocturnal hemoglobinuria may have substernal, lumbar, or abdominal pain, in addition to generalized weakness. The pain may be colicky and last for several days. The abdomen may be tender, with some guarding and even a rebound phenomenon. Venous thrombosis occurs with increased frequency in these patients. It always should be considered if a sudden increase in liver size accompanies these attacks, suggesting thrombosis involving the portal system.

Hyperparathyroidism has been called the syndrome of bones, stones, and groans. Associated peptic ulcer disease or pancreatitis usually causes the abdominal pain.

The lightening-sharp abdominal pain of tabes mainly is associated with syphilis, but it also may occur with diabetes and meningeal tumors. This is a radicular syndrome resulting from damage to the large posterior lumbosacral roots.

The diagnosis of chronic recurrent abdominal pain often can be a challenge to both the therapeutic and diagnostic abilities of the involved physician. Unfortunately, many patients with this type of pain undergo multiple surgical procedures without any significant findings. Good knowledge of the underlying anatomy and pain patterns should make the diagnosis and subsequent treatment easier (Figure 34-6).

The abdomen is the third most common site of pain in psychiatric patients. Diagnosis of psychological pain can be made based on exclusion criteria. There are some signs that can be helpful or supportive. Suspicion is raised when location, timing, quality, and distribution of symptoms do not relate to pathophysiologic patterns. There is often marked discrepancy between reported severity of pain and presented behavior. The patient sometimes reports pain in other parts of body and may have a history of many negative diagnostic workups for various complaints. The onset of pain may have an obvious relationship to a stressful event.

Most of this chapter has dealt with the diagnosis of abdominal pain syndromes. When a treatable disorder is identified, appropriate therapy should be instituted. A simplified algorithm was presented at the beginning of this chapter (see Figs. 34-2, 34-3). Unfortunately, in many patients with chronic abdominal pain, a specific diagnosis is not identified despite an extensive workup. In other patients, a condition is diagnosed, but conventional methods fail to treat the disease and pain. Nerve blocks can be helpful in treating acute and chronic abdominal pain (Table 34-1). Nonspecific methods, such as antidepressants, stress reduction techniques, and behavioral approaches, often are useful in treating these difficult problems.

Pelvic pain is a complicated topic and a clinical challenge, because the very definition of this pain can vary. The pelvis has a formal anatomic description; however, pelvic pain can refer to pain experienced in the general pelvic cavity or pain that is synonymous with gynecologic pain. In addition, gynecologic pelvic pain, although potentially acute or chronic, can refer to chronic pain with or without an anatomic lesion. Under these circumstances the parameters for describing pelvic pain also fluctuate. In the most general context, pelvic pain can be visceral, somatic, and neuropathic. Causal categories are traumatic, mechanical, and psychological. Organ systems include the genitourinary, gastrointestinal, neurologic, and musculoskeletal. The treatment of pelvic pain encompasses not only discoveries in clinical physiology and pharmacology, but also the social and intellectual assumptions behind the clinical practice.

It is estimated that 9.2 million women in United States have pelvic pain. Ten percent of all gynecologic office visits are for pelvic pain; 44% of all laparoscopies and 10% to 15% of hysterectomies are for chronic pelvic pain; and 30% of women presenting to a pain clinic have already undergone hysterectomy. The male pelvic pain syndrome comprises of 8% of all urologic visits and 1% of all visits to primary care physicians. The economic impact is enormous, with medical costs of $1.2 billion per year and missed work and productivity totalling $15 billion per year.

Any discussion of pelvic pain requires an appreciation of the spatial relations of the pelvic viscera, along with their vascular supply and innervation. The pelvis has as its walls the bony pelvis and as its floor the pelvic diaphragm. It is lined by a complex vascular structure and traversed by sympathetic and parasympathetic afferent, visceral afferent, and efferent nerves from the lumbar and sacral plexes. The pelvis is divided into a major pelvis and a minor pelvis, separated by the pelvic brim, which also is the boundary between the abdominal and pelvic cavities. The pelvis contains the bladder and the paravesical fossae in front, the rectum and the pararectal fossae in back, and the internal genital organs in the middle.

The bony pelvis includes two hip bones formed from the fusion of the ischium, ilium and pubis, the sacrum, and coccyx. The two innominate bones form the sides of the pelvis. They are joined in front at the symphysis pubis and articulate with the sacrum and coccyx in back.

The pelvic diaphragm, or floor of the pelvis, arises in front from the body of the pubis and continues behind to the coccyx. It includes the levator ani muscles and the coccygeus muscles. The diaphragm holds the lower part of the rectum and supports the bladder, vagina, and prostate by maintaining sufficient intraabdominal pressure. Beneath the diaphragm is the perineum, with the external genitalia.

Although the bony pelvis can be compared to a basin, the pelvic vasculature resembles a woven lining composed of large, thin-walled veins through which the arteries thread their way. The veins are divided into vesicle, uterine and vaginal or prostatic, and rectal venous plexes, which drain into the internal iliac vein and into the inferior mesenteric veins via the superior rectal (hemorrhoidal) vein, eventually reaching the portal vein. The middle rectal vein emerges from the lower part of the side of the rectum, passes to the internal iliac vein, and anastomoses with the superior and inferior rectal veins and with the other plexes of pelvic veins.

The arteries of the pelvis arise from the internal iliac artery, which runs retroperitoneally, is posterior to the ureter, and is divided into anterior and posterior divisions. The anterior division has seven branches, and the posterior division, three. Collateral circulation is abundant.

The major afferent pathways for nociception from the female pelvic organs travel with the sympathetic nerve bundles and have cell bodies in the thoracolumbar distribution. The suprapubic area is innervated by the iliohypogastric nerve (L1, L2). The inguinal area and the base of scrotum or labia are supplied by the ilioinguinal nerve (L1, L2). The skin of the penis is supplied by the two dorsal nerves of the penis, which are branches of pudendal nerves. The skin of the scrotum and perineal skin are supplied by the posterior scrotal nerves (S2, S3, and S4). The skin between the anus and coccyx is innervated by the lower sacral and coccygeal plexus. The lower third of the vagina is supplied by the pudendal nerve. The genital branch of the genitofemoral nerve supplies the lateral side of the scrotum, the vulva, and the cremasteric muscles.

There are sympathetic, parasympathetic, and visceral somatic afferent nerves to the pelvic viscera. The sympathetic nerves cause muscular contraction and vasoconstriction, whereas the parasympathetic nerves relate to relaxation and vasodilation. Most of the autonomic fibers enter the pelvis through the superior hypogastric plexus, which is located bilaterally at the lower third of the fifth vertebral body and the upper third of the first sacral vertebra at the sacral promontory. This plexus is primarily sympathetic and is formed by the confluence of lumbar sympathetic chains and branches of the aortic plexus containing fibers that traverse the celiac and inferior mesenteric plexus (Fig. 35-1). The superior hypogastric plexus divides into the right and the left hypogastric nerves, which descend laterally to the sigmoid colon to reach the inferior hypogastric plexus.

The inferior hypogastric plexus is located against the inside of the pelvis, lateral to the uterovaginal junction and the rectum. It is the major neuronal integrative center in the pelvis. It innervates multiple pelvic organs, including the urinary bladder, proximal urethra, distal ureter, rectum, and internal anal sphincter, as well as genital and reproductive structures via communication through the uterovaginal plexus and the vesical and the inferior rectal plexus. The parasympathetic nerves, which are branches of anterior rami of the S2, S3, and S4 nerve roots, traverse the inferior hypogastric plexus. In contrast to the superior hypogastric plexus, which is situated predominantly in the longitudinal plane, the inferior hypogastric plexus is oriented more transversely, extending posteroanteriorly and parallel to the pelvic floor. The location and configuration of the inferior hypogastric plexus does not lend itself to surgical or chemical extirpation.

The sensory nerves from the uterus accompany the sympathetic nerves and enter the spinal cord at the T11 and T12 level, and refer pain to the abdomen. The S2, S3, and S4 afferents from the cervix are referred to the lower back and lumbosacral area.1,2

The ganglion impar (also known as ganglion of Walther) is a solitary retroperitoneal structure located at the level of the sacrococcygeal junction that marks the termination of the paired paravertebral chains. This structure receives fibers from the lumbar and sacral portions of the sympathetic and parasympathetic nervous system. It provides sympathetic innervation to portions of the perineum, rectum, and genitalia.

Pelvic pain is often considered synonymous with gynecologic pain. Such pain can be acute or chronic. Acute pain is caused by structural disruption or physiologic dysfunction, whereas chronic pain may be the result of persistent pressure on nerves, inflammation of arteries, diseases of the female reproductive organs, and factors not demonstrable by present-day imaging techniques.

Acute Pain

The differential diagnosis of acute pelvic pain includes ectopic pregnancy and complications of pregnancy, ruptured cysts, ovarian and adnexal torsion, uterine fibroids, and pelvic inflammatory diseases. Ninety-eight percent of women with ectopic pregnancy experience unilateral pain, which may be accompanied by light or missed menses. Symptoms of pregnancy, such as nausea and breast tenderness, also may be present.3

The pain of ovarian cysts may be dull and aching, localized to the side of the abscess, and accompanied by pelvic tenderness. An ovarian cyst with a twisted pedicle can cause acute pain, which becomes intermittent when the pedicle untwists. Other symptoms include nausea and vomiting, diarrhea or constipation, and leukocytosis.

Symptoms of a palpable mass, delayed menses, and pelvic tenderness also occur with corpus luteum cysts, which can bleed into the peritoneum and mimic pelvic inflammatory disease (PID). PID is defined as a spectrum of upper genital tract inflammatory disorders that may include endometritis, salpingitis, tubo-overian abscess, and pelvic peritonitis. The diagnosis should not be considered conclusive without additional findings, such as a positive cervical culture. The primary pathogens are Neisseria gonorrhea and Chlamydia trachomatis. Cervical cultures for Chlamydia can detect up to 80% of cervical infections; antibody testing, enzyme-linked immunosorbent assay, and DNA probe testing can detect 60% to 90% of infections. Because of this wide range in positive findings, culturing of specimens from the urethra and anus also should be considered. Tubo-ovarian abscess may occur as a complication of PID as well as in postpartum and postoperative patients and in women who use intrauterine devices. The bacteria present in tubo-ovarian access occur in the lower genital tract and may not be the same agents involved in PID.4

Chronic Pain

Chronic pain may begin as an acute episode, change into episodic pain, and then persist for 6 months or longer. From a clinical perspective, chronic pelvic pain can be a symptom of a disease or part of a syndrome. Endometriosis and malignancies are examples of primary illnesses, whereas syndromes include dysmenorrhea, premenstrual syndrome, and chronic pelvic pain disorders.

Endometriosis is the presence of ectopic endometrial glands and stroma outside the uterine cavity. Patients with endometriosis may have dysmenorrhea, dyspareunia, back pain, and rectal discomfort in addition to persistent pelvic pain. The symptoms are related to the site of the endometrial implant. However, the intensity of the pain may not correlate with the size of the implant.5 The incidence is 1% to 2% of the general female population and 15% to 25% in infertile women. Patients may remain asymptomatic until they are being evaluated for infertility. Definitive diagnosis can only be made by laparoscopy, although elevated serum CA-125 levels correlate with severity and reflect the course of the disease. Treatment includes medical and surgical options. The medical treatment for endometriosis includes oral contraceptives, medroxyprogesterone acetate, danazol, and gonadotrophin-releasing analogues. The goals of surgery are to restore normal pelvic anatomy and to resect, coagulate, or vaporize all endometriotic implants.

Adenomyosis is a benign invasion of the wall of the uterus by endometrial tissue. Most patients are 35 to 50 years of age and have had children. Presenting complaints usually dysmenorrhea and menorrhagia.

Uterine leiomyomas (fibroids) are painful when they press on or become entangled in other structures, and cause discomfort when they grow. Dyspareunia, dysmenorrhea, and pelvic pressure are frequent symptoms, along with intermenstrual, postmenstrual, and heavy menstrual bleeding. A degenerating leiomyoma may cause only episodic pain at first and then fever, increased pain, and leukocytosis with a left shift later. Leaking purulent material can cause peritonitis. Leiomyomas undergo malignant transformation in 2 to 3 per 100,000 women, but grow slowly. Surgery used in the treatment of women with heavy bleeding, growths of large size, and additional symptoms.6

Cervical cancer may cause pelvic pain as it metastasizes. Ovarian cancer may be asymptomatic until late stages, when it causes symptoms that are vague and nonspecific. Endometrial cancer in 90% of cases presents with vaginal bleeding or discharge. Pelvic pressure or discomfort occurs from uterine enlargement and then spread of the tumor into extrauterine structures. Any tumor that compresses the bladder or rectum can cause pressure, pain, or dyspareunia.7

Women with ovarian remnant syndrome present with unilateral consent or cyclical pain, as well as postcoital ache, and post-micturition or post-defecation pain. They may have a history of the removal of one or both residual ovaries for pain associated with pelvic adhesions or endometriosis. Although localized abdominal pain is a constant feature of the syndrome, often no mass can be found on pelvic examination. Ultrasound examination is usually diagnostic, revealing a mass that must be distinguished from accessory ovaries, an embrylogic variant of normal development. Treatment is surgical removal of the remnant.8

Women with major gynecologic diseases can have pelvic pain in the form of dysmenorrhea. This term is applied to severe cramping in the lower abdomen, lower back, and upper thighs that occurs during menstruation. Primary dysmenorrhea is the most prevalent source of chronic episodic pain in premenopausal women. It is caused primarily by prostaglandin release from the endometrium at menses, in particular prostaglandins F2 alpha and E2. The cramping pain is often accompanied by nausea, vomiting, headache, diarrhea, and fatigue. Secondary dysmenorrhea and atypical cyclic pain are caused by underlying intrauterine or extrauterine pathology, such as endometriosis, adenomyosis, and other pathology that alters blood flow, increases pressure, or causes irritation of the pelvic organs.

The treatment of primary dysmenorrhea includes nonsteroidal anti-inflammatory drugs (NSAIDs) and oral contraceptive pills. NSAIDs are effective in up to 80% of cases. Other modes of menstrual hormonal suppression are recommended for patients who obtain no relief with NSAIDs and oral contraceptives. Secondary dysmenorrhea requires a thorough evaluation and then treatment of the underlying cause.

Premenstrual syndrome (PMS) includes mood, behavioral, and physical changes that occur during the luteal phase of most menstrual cycles. Studies suggest that about one third of premenopausal women experience some degree of PMS. As in the treatment of dysmenorrhea, underlying illnesses, such as endometriosis and leiomyomas, should be ruled out. Treatment includes NSAIDs for pain, diuretics such as spironolactone for fluid retention, and antidepressants for dysphoria.

Pelvic congestion syndrome is usually seen in premenopausal women, thus suggesting that there is a hormonal factor involved in the venous dilation.9 The capacity of pelvic veins to increase 60-fold by the end of pregnancy makes them, in the nonpregnant state, vulnerable to chronic dilation and stasis. Weakened fascial supports during parturition and the vasodilating effects of cyclically fluctuating hormones aggravate the tendency to dilation.10 The common symptom is a dull, aching pain in the pelvic area that worsens on standing, walking, and lifting and is relieved by lying down. Deep dyspareunia is one of the most consistent symptoms of pelvic congestion syndrome, with an incidence ranging from 71% to 78%. The severity of the pain is determined by the extent of the venous stasis, which leads to hypoxemia and local tissue damage followed by the release of pain-producing substances. The presence of dilated veins in the infundibulopelvic ligament, ovarian hilum, or broad ligament, combined with polycystic changes in the ovary (which may be apparent only on close inspection), is diagnostic of pelvic congestion. Ultrasound and computed tomography (CT) scans are important for revealing polycystic changes in the ovaries and dilated veins in the broad ligament and uterus. Surgical treatment includes hysterectomy and ligation of the ovarian vein. Medical therapy consists of oral contraceptives to suppress ovarian function and intermittent courses of anti-inflammatory agents and antibiotics when inflammation occurs secondary to local infection.11 Radiologic transcatheter embolization of the ovarian veins has been used with varying success.

Sympathetic pelvic syndrome is a chronic pelvic pain disorder that has been recognized because of developments in the study of chronic pain. It is assumed that visceral illness occurs, with pain transmitted to cutaneous areas. The area of innervation includes the cervix and vagina (with innervation from the pudendal nerves, having derivation from S2 through S4), along with the uterus, fallopian tubes, and ovaries (with innervation from the sympathetic pelvic branches of T10 through T12). Repeated local anesthetic nerve blocks are recommended in addition to the medications used in patients with chronic pain.

Another chronic pelvic pain syndrome is focal vulvitis, characterized by burning vulvar pain and superficial dyspareunia. One study found that a majority of patients continued to have symptoms of vulvitis after Woodruff perineoplasty. The authors concluded that surgery was not the best treatment. They noted that these women often had insufficient lubrication or hypertonia of the pelvic floor, or both, during sexual intercourse. Thus, an integrated approach was recommended, including protection of the vulvar skin, relaxation of pelvic muscles, and treatment of psychosexual and relational aspects of the disorder.12

These treatment recommendations are similar to those for patients with chronic pain for whom no pathologic findings have been found. Some studies of chronic pelvic pain in women report that 10% to 50% experience pelvic pain without pathology; other studies cite 15% as the percentage of women who have chronic pelvic pain. The studies citing these statistics then tend to look into symptom clusters for this group of patients. The wide incidence range cited suggests there may be some ambiguity in the definition of chronic pain and in the procedures used to evaluate patients with pelvic symptoms.

The initial patient group can be modified after further study and treatment. Nevertheless, there remains a group for whom no physiologic mechanisms have been elucidated.13,14 These patients have a chronic pelvic pain syndrome that is considered a somatoform disorder similar to irritable bowel syndrome. The prevalence of this syndrome in primary care patients (38%) is comparable to that of asthma and back pain.15 A syndrome of chronic pelvic pain without obvious pathology (CPPWOP) has also been described, but is unclear in these cases whether pathology has been overlooked or limited to findings for specific diseases, such as endometriosis, thus possibly excluding damage to ligaments and smooth and striated muscle. Patients with CPPWOP have a group of symptoms that includes intermittent pain that can vary with the menstrual cycle and is localized to the low abdomen and back. Dyspareunia may be present. Patients tend to be in their 20s and 30s and have a history of childhood sexual abuse, rape, or incest. Present relationships are marked by abuse, divorce, and prostitution.16

Treatment of chronic pelvic pain syndrome or CPPWOP is similar to that of other chronic pelvic pain syndromes and includes NSAIDs and antidepressants. Psychological interventions also have played a role in the treatment of patients with these syndromes. The treatment strategies resemble those for patients with chronic pain with a specific pathology and include methods that help women distract themselves from the pain and encourage them to cope despite the pain.

Women with negative investigations that do not result in an organic diagnosis may then be referred for psychiatric treatment. Despite the apparent presence of anxiety, depression, and possible history of sexual trauma, referral for psychiatric treatment may be resisted. This response is not surprising, given the role psychobiological factors have played in the treatment of women with chronic pelvic pain. Studies of women with chronic pelvic pain syndrome and chronic vulvar pain syndrome have shown a significantly higher incidence of sexual abuse in the two chronic pain groups compared with women who do not have these symptoms. In addition to physical and emotional trauma, there is a significant association between sexual victimization before age 15 years and chronic pelvic pain. Sexual abuse and somatization are highly predictive factors for chronic pain. There also is a higher incidence of depressive symptoms in this group of patients.

The association of sexual abuse, affect and pelvic pain was the basis for psychoanalytic studies of personal development throughout the past century. The first case studies were women with the diagnosis of hysteria, whose symptoms had psychological meaning. The term hysterus is Greek for uterus, and the concept of hysteria (derived from a theory of a “wandering” womb as the basis for inexplicable symptoms in women) historically was the basis for treatments that reflected sociocultural values about the status of women. Hysteria is no longer considered a valid diagnosis; it has been replaced by in current practice by the diagnoses of conversion and somatoform disorders, which may affect both men and women. Nevertheless, the legacy of hysteria as a medical condition persists.

Hysterical conversion has been defined as a special type of somatization in which physical symptoms express a psychological conflict (often sexual) that includes affects that were not sufficiently expressed at the time of the incident, and now represented symbolically. In classical psychotherapy, after elucidating the traumatic precipitant, the next step was to clarify how affects were not expressed directly and were converted into physical symptoms. Patients were said to be using the defenses of repression, denial, and somatization to deal with their feelings. Because of social pressure to limit their direct expression of aggression and their vulnerability to sexual abuse, women were more likely to have chronic pain and to express themselves somatically. Eventually, such women came to be identified with their symptoms; the so-called painful woman who resisted the interpretations put forth for her benefit.17,18

Even today pelvic pain may be considered psychogenic when there is no explicit organic finding because of the persistence of the triad of affect, sexual abuse, and somatization in clinical and psychometric testing studies. Because not all women with chronic pelvic pain have been abused, an alternative explanation has been sought for pelvic pain that occurs without confirming laparoscopic and imaging findings. The biopsychosocial model attempts to integrate physiologic and psychological causes of pain, suggesting the existence of a mind-body dualism in which psychological factors are secondary to physical symptoms. Keeping in mind that physical symptoms in at least one third of cases have no demonstrable organic findings, this model can lead to clinical situations that are difficult to distinguish from early models of hysteria and thus are subject to patient skepticism. This approach is being replaced by theories of central pain mechanisms focusing on neurotransmitters and neural networks that also express affective and cognitive functions. However, a major deconstruction of the studies for chronic pelvic pain needs to be carried out before the diagnosis of hysteria can be considered obsolete.

Whereas women with chronic pain have been referred to multidisciplinary treatment centers to treat complex chronic pain, evaluation and treatment for men has included attempts to establish objective criteria for diagnosis of chronic pelvic pain syndrome. Chronic prostatitis is a poorly understood form of chronic pelvic pain syndrome in men. The diagnosis is based on symptoms, and no measurable parameter can help in defining the presence of the disease, its severity, or cause. A National Institutes of Health (NIH) classification system showed that 38% of men with chronic prostatis had chronic bacterial prostatitis; with 7% of cases resulting from inflammatory chronic pain syndrome, and 55% from noninflammatory chronic pelvic pain syndrome.19

The objectives of current studies include refining and standardizing the evaluation of men with symptoms of chronic prostatitis. Some men have elevated levels of tumor necrosis factor-α and interleukin-1β proinflammatory cytokines. Seminal cytokine levels may provide an objective measure of disease in these patients and suggest specific therapeutic strategies.20 Patients with prostatitis report more perineal, lower abdominal, testicular, penile, and ejaculatory pain than patients with benign prostatic hypertrophy and sexual dysfunction.

Another attempt at determining objective criteria for chronic pelvic pain syndrome is to look for evidence of T-cell reactivity with normal prostatic proteins in autoimmune prostatitis. In one study, the CD4 T-cell proliferative response to seminal plasma was found to be statistically significant when compared with medium alone in men with a history of chronic prostatitis and chronic pelvic pain syndrome, but it was not statistically significant in normal men. The responses of subjects in the chronic prostate/chronic pelvic pain syndrome group and normal subjects to the recall antigens tetanus toxoid and Candida extract were equivalent.21 In studies using color Doppler ultrasonography, chronic prostatitis was associated with abnormal prostate blood flow in men with and without inflammation in comparison with controls.22

In a study of men with pelvic pain, the reported locations of the pain were the prostate or perineal region, or both, in 45.6% of cases, the scrotum or bladder in 38.8% of cases, the penis in 5.8%, the bladder in 5.8%, and the lower abdomen and back in 1.9%.23 Regardless of the location, most subjects had increased urethral sensitivity, suggesting an apparent association of pelvic floor dysfunction with pelvic pain. Treatment was aimed at modulating the pelvic floor with biofeedback, medication, and sacral anterior root stimulation.24

Pelvic floor tension myalgia may contribute to the symptoms in men with chronic pelvic pain syndrome. The presence of detrusor instability, hypersensitivity to filling, or bladder-sphincter pseudodyssynergia on pretreatment urodynamic studies is not predictive of treatment results. Treatment recommendations include a formalized program of neuromuscular reeducation of the pelvic floor muscles plus interval bladder training to improve objective measures of pain, urgency, and frequency in patients with chronic pelvic pain syndrome.25

Pelvic varicocele and seminal vesicle dystrophy are other causes of pelvic pain. In men, diagnosis of varicocele is based on clinical findings (in contrast with women, in whom instrument evaluation is needed to identify causes such as incontinence of ovarian veins or development of adnexal varicosities). Treatment of male varicocele involves percutaneous sclerotization, surgical excision, or therapeutic embolization.26

One study of women with chronic pelvic pain found that more than 75% had irritable bowel syndrome. Of women referred to a gynecologic clinic for dysmenorrhea, dyspareunia, and abdominal pain, half had significantly more symptoms of irritable bowel syndrome than did those referred for symptoms other than pain. Irritable bowel syndrome is a functional bowel disorder of uncertain etiology characterized by a chronic relapsing pattern of abdominal-pelvic pain and bowel dysfunction with constipation or diarrhea, or both. It affects 15% of the population. Symptoms include abdominal pain, bloating, belching, excessive flatus, diarrhea, constipation, passage of mucus, and painful defecation with a sense of incomplete evacuation. Symptoms often are worse during stressful periods and the premenstrual phase of the cycle, and may accompany anxiety and depression.

The pathophysiology of irritable bowel syndrome is multifactorial, involving altered bowel motility, visceral hypersensitivity, and psychosocial factors. Other contributing factors include diet and prior infection. Therapeutic options include increasing dietary fiber to prevent constipation, opioid agents for diarrhea, low-dose antidepressants or infrequent use of antispasmodics for pain, psychotherapy, and hypnotherapy.27 The diagnosis of irritable bowel syndrome is made by taking a careful history and by use of colonoscopy or barium enema to exclude other conditions.

Inflammatory bowel disease may manifest the same symptoms as irritable bowel syndrome. More than 50% of patients with Crohn’s disease and ulcerative colitis present with pain. About 90% of patients with Crohn’s disease have a bloody diarrhea, whereas patients with ulcerative colitis have moderate cramping that resolves with defecation. In one study, chronic pelvic pain was reported in 35% of patients with irritable bowel syndrome and in 13.8% of those with inflammatory bowel disease.28 Women with both irritable bowel syndrome and chronic pelvic pain were more likely to have a lifetime history of dysthymic disorder, current and lifetime panic disorder, somatization disorder, childhood sexual abuse, and hysterectomy than women who had irritable bowel syndrome alone. Logistic regression has shown that the mean number of somatization symptoms was the best predictor of a history of both irritable bowel syndrome and chronic pelvic pain compared with either inflammatory bowel disease or irritable bowel syndrome alone.29

Interstitial cystitis is a chronically progressive, severely debilitating, heterogeneous syndrome that affects the urinary bladder and is characterized by urgency frequency and pain. Its etiology is poorly understood; current hypotheses focus on occult or resistant microorganisms, urothelial hyperpermeability from a defective glycosaminoglycan mucus layer, neurogenic or hormonal dysfunction, mast cell activation, and genetic susceptibility as possible causative factors. The diagnosis is made clinically, by cystoscopy with hydrodistention, and sometimes by biopsy when other pathologies have been excluded.30 This condition is underdiagnosed in women with pelvic pain. Symptoms of urinary frequency and urgency, dysuria, hematuria, and nocturia occur along with the pelvic pain. Patients have often been treated for recurrent urinary tract infections. Urinalysis results may be normal, but microscopic hematuria without white blood cells is sometimes noted. Patients must have at least two of the following symptoms: pain in the suprapubic, perineal, and urethral region; pain on bladder filling that is relieved by emptying; decreased compliance on cystsometrogram; and glomerulations on endoscopy. When these criteria are not met, patients are considered to have urgency-frequency syndrome.

Treatment approaches includes use of systemic agents, instillation therapy, and surgical management. Trials of systemic agents, including antihistamines, azathioprine, corticosteroids, heparin, pentosan polysulfate, and tricyclic compounds have shown inconsistent results. Dimethyl sulfoxide (DMSO) has been used for intravesical therapy, with varying response rates. Surgical treatments include urinary diversion procedures and augmentation cystoplasty. Denervation procedures are reserved for patients with severe, intractable disease. Hypogastric plexus block and neuromodulatory procedures have been used with some success.

Infectious cystitis manifests symptoms of suprapubic pain, dysuria, and frequency and urgency, along with pyuria and a positive urine culture. These symptoms respond to antibiotics. In about 25% of women with irritative urinary symptoms and pyuria, C. trachomatis is found in the urethra. The urethral syndrome occurs when the urinary bacterial count is low, urinalysis is negative, and the Chlamydia study is negative. The etiology of this syndrome is unclear, but it may be caused by chronic inflammation of the periurethral glands, or urethral spasticity with periurethral muscle fatigue. Treatment is similar to that of interstitial cystitis and urgency-frequency syndrome, and consists of a combination of medication, biofeedback, and re-education of voiding habits.31

Evolving lumbar disk disease and intradural neoplasms in the upper lumbar area produce symptoms that may be interpreted as pelvic pain. Symptoms consistent with radiculopathy occur late in the course of these diseases. Musculoskeletal pain can be the result of decreased abdominal and pelvic muscle strength, decreased range of motion of the hip, and unequal leg lengths. Trigger points for this pain may be near surgical incisions.

Surgical procedures can lead to serious pain that develops months to years afterward. An example is the Pfannensteil incision, a horizontal incision across the lower pelvis, which can lead to pulling along the pathway of the ilioinguinal and iliohypogastric nerves. The stretching and trauma arising from labor and delivery also can lead to pelvic pain. The pain is from the lower pelvis, although it may be experienced as visceral in origin.32 Pelvic pain during pregnancy has been associated with symphyseal distention. However, the degree of pain was not proportional to the degree of distention. Studies of pain in pregnancy have attempted to correlate pain with elevated hormones, such as serum concentrations of relaxin and propeptide of type III procollagen measured in early pregnancy.33,34

A similar disproportion occurs in adhesions, which may be responsible for pelvic pain as sequelae of past infection, chronic active inflammatory state, endometriosis, and postoperative adhesions. Celioscopy is the key diagnostic procedure after history taking and physical examination. There is no systematic relation between the clinical picture and anatomic findings. A diagnosis of chronic pelvic pain is recommended in the absence of any macroscopic, histologic, and bacteriologic lesions.35

In laparoscopic studies, about 25% of cases of acute pain and 35% of cases of chronic pain have been attributed to adhesions. The primary sources for the adhesions were in the bowel and omentum. It has been suggested that this pain develops when adhesions fix the pelvic organs in place, hampering their motility. The pain of adhesions can be aggravated by activity. Lysis of adhesions can reduce symptoms of pain, but the duration of relief cannot be predicted. There has been no scientific support for using crystalloids, macromolecular solutions, introperitoneal heparin, corticosteroids, mechanical barriers such as intercede and Gore-Tex, or biodegradable barriers to reduce adhesions.36

A study of laxity in the posterior ligament support of the uterus suggests that the concurrent pelvic pain is attributable to parasympathetic pain at the T12 to L1 level. The pain may result from gravitational pull on nerves that are not being supported by the lax uterosacral ligaments.37

The term vulvar vestibulitis refers to pain that occurs during sexual intercourse. The diagnosis is based on the presence of dyspareunia that lasts for at least 6 months and red areas in the vestibulum that are extremely sensitive to touch. The etiology is multifactorial and includes repeated use of antibiotics, local treatment of Candida and human papillomavirus infections, use of hormonal contraceptives, frequent use of local substances that may be irritative, lack of arousal, vaginismus, and tense pelvic floor muscles. There is increased intraepithelial innervation and no inflammation. Treatment includes biofeedback, tricyclic antidepressants, psychotherapy, and surgery.38

Pudendal nerve entrapment should be considered as a differential diagnosis in patients with anoperineal pain. The idea that pudendal nerve entrapment is the cause of some pelvic pain syndromes has gained momentum in the past 15 years.39,40 The pudendal nerve navigates along a tortuous path, supplying sensation to virtually the entire pelvic area including the penis, scrotum, perineum, and rectum, as well as motor function to the pelvic floor musculature and urethral sphincter. The two major areas of entrapment include the junction of the sacrotuberous and sacrospinous ligaments, and the pudendal canal of Alcock. Evaluation of this pathology includes reproduction of pain on application of intrarectal digital pressure at the ischial spine. In addition, intrarectal stimulation of the pudendal nerve trunk and a recording response in the bulbospongiosus muscle with distal motor latency longer than 5.0 msec is suggestive of pudendal neuropathy. The pain can be abolished by fluoroscopy or CT-guided local anesthetic and corticosteroid injection at the ischial spine or the canal of Alcock.41

Coccygodynia is a common problem, characterized by pain and tenderness at the tip of the spine or in the coccyx. The pain frequently radiates to the perineal, gluteal, and posterior sacral areas; worsens on sitting; and is eased on standing. Treatment consists of massage, local heat, injection of the sacococcygeal joint with local anesthetic and corticosteroid, and, if indicated, psychological therapies. Tension myalgia refers to spasm of the involved muscles, which can be the levator ani, the piriformis, or the coccygeus muscle groups. Treatment includes heat and massage, local anesthetic injection of the muscles, and follow-up exercises.42

Pelvic joint instability and persistent pelvic pain can occur in instances of precocious puberty and use of oral contraceptives prior to the age of reproduction. Diagnosis is based on a history of early onset of menarche. Osteoporotic sacral fractures are associated with pelvic pain.

Sports-related injuries and pain include pyramidal muscle hematoma, osteitis pubis, and adductor tendonitis. Pyramidal hematomas can cause impingement of the sciatic, inferior gluteal, and pudendal nerves when the nerves are compressed between the muscle and the iliac spine. Osteitis pubis, considered to be the most common inflammatory disease of the pubic symphysis, is a self-limiting inflammation secondary to trauma, pelvic surgery, childbirth, or overuse. It occurs more often in men during the third and fourth decades of life, causing pain in the pubic area, one or both groins, and in the lower rectus abdominis muscle. The pain may be exacerbated by exercise or by specific movements, such as running, kicking, or pivoting on one leg, and is relieved with rest. During the physical examination, pain can be elicited by resisted long and flexed adductor contraction. A waddling antalgic gait and symphysis tenderness also can occur. Initial therapy should focus on decreasing inflammation with active rest, ice, NSAIDs, and physical therapy. If necessary, judicious use of local corticosteroids may be attempted.43

Medical Therapy

NSAIDs have been studied extensively in the treatment of primary dysmennorrhea and have proven efficacy in various causes of pelvic pain. Combination estrogen-progestin oral contraceptives, danazol, and gonadotropin-releasing hormone agonist are used in a stepwise sequence in the management of pelvic pain secondary to endometriosis. Other adjuvant medications, such as tricyclic antidepressants, have a role in some chronic pelvic pain conditions.

Neural Blockade

Hypogastric Plexus Block

This type of block is used to modulate pain that results from a sympathetic mechanism originating in the pelvic viscera. Several techniques have been described for performing hypogastric plexus block. Needle placement with fluoroscopy from the posterior approach, using landmarks and coaxial imaging technique, is commonly used. A transdiskal approach through the L5-S1 disk using fluoroscopy or CT guidance has been reported. An anterior approach under CT guidance may be used in selected cases. Neurolytic hypogastric plexus is quite effective in relieving pelvic pain in patients with gynecologic, colorectal, or genitourinary cancer.43

Ganglion Impar Block

This block is used to evaluate and manage pain of sympathetic origin that has its root in the perineum, rectum, or genitalia. There are various techniques for performing this block. One technique uses fluoroscopic guidance and an approach via the trans-sacrococcygeal with a straight needle44 (Fig. 35-2). Another technique involves entering at the level of the anococcygeal ligament with a curved needle45 (Fig. 35-3). Neurolysis of ganglion impar is used for refractory cases of perineal pain. Neurolysis can be achieved by using chemicals such as phenol or by cryoablation or radiofrequency lesioning.

Pudendal Nerve Blockade

Pudendal neuralgia should be considered in the differential diagnosis of long-term anoperineal pain. In women, the block is performed transvaginally with the patient in the lithotomy position. The needle tip must be advanced through the sacrospinous ligament just past the ischial spine. The transperineal approach is used in men, and in women in whom anatomical constraints preclude a transvaginal approach. CT-guided pudendal nerve block has also been reported as a very effective technique in the treatment of pudendal neuralgia.46

Neuromodulation

Sacral neuromodulation, which involves insertion of sacral nerve stimulators, has been performed for patients with voiding disorders, including urinary urge continence, urgency-frequency, and nonobstructive urinary retention; for patients with intractable interstitial cystitis; and for those with chronic intractable pelvic pain.47 This technique is especially beneficial in patients with bladder sphincter dysfunction because it restores balance between the sacral reflexes. Electrical stimulation of the S3 nerve activates the pelvic floor and modulates innervations of the bladder, sphincter, and pelvic floor.

Neuraxial Drug Delivery

Delivery of an analgesic into the epidural or subarachnoid space is used most often in the management of patients with pain secondary to malignancy. Opioids remain the most commonly used drugs for this therapy; however, several other agents, such as local anesthetics and clonidine, can be used in conjunction.

Neuroablation

In patients with unilateral pelvic pain secondary to malignancy cordotomy or rhizotomy can be used in selected patients. The side effects include paresis, ataxia, and bladder dysfunction.

Surgical Therapy

Laparoscopy

In a review of 1,524 gynecologic laparoscopies performed for chronic pelvic pain, endometriosis was the most commonly diagnosed disorder (33%), followed by adhesive disease (24%), chronic PID (5%), ovarian cysts (3%), pelvic varicosities (< 1%), leiomyomata (< 1%), and a variety of other diagnoses (4%). No visible pathology was detected in 35% of patients.12 Resection and ablation of the endometriotic lesions, and lysis of adhesions are the two therapeutic approaches commonly employed laparoscopically.

Nerve Transection Procedures

Laparoscopic uterosacral nerve ablation involves the destruction of the uterine nerve fibers that exit the uterus through the uterosacral ligaments. There seems to be little evidence to support the performance of this procedure.48

Presacral neurectomy is a procedure designed to interrupt the sympathetic innervation of the uterus at the level of superior hypogastric plexus. It is performed by incising the pelvic peritoneum over the sacrum and then identifying and transecting the sacral nerve plexus. This procedure is more helpful in the management of patients with persistent midline pelvic pain.

Role of Gonadectomy and Hysterectomy

Bilateral oophorectomy with or without hysterectomy is the most effective procedure for women who have recurrent symptomatic endometriosis and who have no desire to retain reproductive function.

Multidisciplinary Treatment

Pelvic pain is the end result of disease-initiated stimuli and the interpretation of these stimuli by the central nervous system. It is a complex interaction that involves sensory, psychological, and environmental factors. Hence, applying a multidisciplinary approach, which employs an integrative approach to the psychosocial-mind-body paradigm, is sometimes the most effective strategy in the management of patients with chronic pain states. This approach integrates medical intervention with identification and management of socioenvironmental problems, cognitive behavioral pain strategies, and treatment of concurrent psychological morbidity. Available evidence suggests that outcomes, including pain severity, general health, and functional status and disability, improve more significantly after this approach than after isolated medical or surgical interventions.

Physical Therapy

Pelvic floor manual therapy, which involves decreasing the pelvic floor hypertonicity, may ameliorate the symptoms of urgency-frequency syndrome and interstitial cystitis.49 Other physical therapy modalities, including heat, electrical stimulation, deep ultrasound, and massage, may be used depending on the individual need of the patient.

Psychological Treatment

The various psychological modalities of treatment should be selected based on individual need, which can vary dramatically among patients. Behavioral therapy, progressive relaxation training, guided imagery, self-hypnosis, breathing exercises, and biofeedback are some of the techinques employed. Other options that may be more useful in the setting of chronic pelvic pain include group therapy, couples therapy, and sex therapy.

Alternative Medicine

Alternative or complementary medicine is receiving greater interest from both patients and health care providers. Yoga, meditation, chiropractic treatment, acupuncture, and magnet therapies are some of the common forms of these modalities used in the treatment of pelvic pain.

Pelvic pain remains a diagnostic and therapeutic challenge for the health care provider. It is a source of frustration to both the physician and patient. Physicians have been ill equipped through their training to confront the multifaceted nature of the complaints of patients with chronic pelvic pain. Patients presenting with these pain syndromes are best assessed and treated using a multidisciplinary approach. Close collaboration among gynecologists, urologists, proctologists, neurologists, pain specialist, gastroenterologists, psychiatrists, psychologists, physiatrists, and neurologists is needed. Special emphasis should be placed on preventive strategies, such as screening and treatment of avoidable conditions (e.g., lower genital infection) that can result in PID. Preventive measures also are needed to tackle the growing incidence of childhood and sexual abuse, which play a role in chronic pain syndromes.

Chronic, nonmalignant pain syndromes of the perineal area have been well described in the medical literature dating back to the last century. However, the etiology of these focal pain syndromes is poorly understood. The patient who is experiencing pain in the perineal area is often embarrassed, because these areas of the body are considered taboo in our society. These pain syndromes are frequently under-reported and under-recognized.

Patients with these pain syndromes often have seen a variety of specialists in different subspecialties—including urologists, gynecologists, gastroenterologists, proctologists, and internists—and despite an extensive evaluation, no specific etiology has been found in the majority of cases. Not surprisingly, many of these patients are frustrated, because they have suffered from chronic pain for many years, but the disease has not been “given a name” and the pain is not controlled. It is important to recognize that these focal chronic pain syndromes of the perineal area do exist. The etiology of these pain syndromes is not known, and a specific secondary cause can be identified in a minority of patients. Although these patients often are depressed, rarely are these pain syndromes the only manifestation of a psychiatric disease. Currently available treatment strategies are empirical, only. Although complete cures are uncommon, effective treatment modalities exist to lessen the impact of pain and offer reasonable expectations of an improved functional status.

The intent of this chapter is to first give a brief overview of the current knowledge of the neurobiology of the perineal area, and then to review the clinical characteristics and treatment strategies of the different perineal pain syndromes.

The perineum is a highly specialized area of the body, responsible for carrying out a host of basic biologic functions, including defecation, micturition, copulation, and reproduction. The display of these diverse functions relies on precise nervous system control, coordinated with endocrine and other local control mechanisms. Compared with other areas of the body, there has been fairly little research on the neuroanatomy, neurophysiology, and neuropharmacology of the perineum. The complexity of the perineum in carrying out many different specialized functions has largely been considered to account for the slow progress in our understanding of the neurobiology of this area. The fact that these areas of the body often are considered taboo in our society also may account for the scarcity of research on this topic.

A detailed review of the neurobiology of the pelvic floor is provided by Burnett and Wesselmann.1 Briefly, the innervation of the perineum is served by both components of the autonomic nervous system, the sympathetic and parasympathetic divisions, as well as the somatic nervous systems2,3 (Figs. 36-1 and 36-2). Sensations from the pelvic floor are mainly conveyed via the sacral afferent parasympathetic system, with a far lesser afferent supply from afferents traveling with the thoracolumbar sympathetics.4 However, sensations of the testis and epididymis may predominantly involve thoracolumbar afferents.4 Somatic efferent and afferent innervation to the perineum originates from sacral spinal cord levels S2 to S4. Sacral nerve roots emerge from the spinal cord to form the sacral plexus, from which arises the pudendal nerve.5 The pudendal nerve also receives postganglionic axons from the caudal sympathetic chain ganglia.3 The pudendal nerve runs medial to the internal pudendal vessels along the lateral wall of the ischiorectal fossa dorsal to the sacrospinous ligament. First a branch splits off to become the dorsal nerve of the penis (or clitoris), then the remaining pudendal nerve fibers distribute a medial branch to the anal canal, dorsal branches to the urethral sphincter, and dorsolateral branches to the anterior perineal musculature. The posterior perineal musculature is supplied by nerves originating predominantly from sacral level S4. Branches of the S4-5 nerve roots form the coccygeal plexus, distributing fibers to the perineal, perianal, and scrotal (labial) skin.6

Neuropeptide release appears to account for perineal sensations.4 Numerous peptides have been associated with afferent pathways of the pelvic floor, although a preponderance of evidence supports the roles of substance P and calcitonin gene–related-peptide (CGRP) as the primary chemicals released from these sensory neurons.7

Vulvodynia

Detailed descriptions of hyperesthesia of the vulva can already be found in U.S. and European textbooks of gynecology from the last century.8,9 Surprisingly, despite these early reports the medical literature did not mention vulvar pain again until the early 1980s, when a new awareness of this chronic pain syndrome developed. In 1984, the International Society for the Study of Vulvar Disease Task Force defined vulvodynia as chronic vulvar discomfort, characterized by the patient’s complaint of a burning and sometimes stinging sensation in the vulvar area.10 Vulvodynia includes several subgroups: vulvar dermatosis, cyclic vulvovaginitis, vulvar vestibulitis, vulvar papillomatosis, and dysesthetic vulvodynia.11

The incidence or prevalence of vulvodynia is not known. A recent survey of sexual dysfunction, analyzing data from the National Health and Social Life Survey, reported that 16% of women between the ages of 18 and 59 years living in households throughout the United States experience pain during sex.12 When these data were analyzed by age group, the highest number of women reporting pain during sex was in the age group of 18 to 29 years. The location and etiology of pain was not analyzed in this study. Goetsch13 reported that 15% of all patients seen in her general gynecologic private practice fulfilled the definition of vulvar vestibulitis, a major subgroup of vulvodynia. It is important to point out that these patients had not come for a gynecologic evaluation because of vulvar pain, but for a routine gynecologic checkup. Fifty percent of these patients had always experienced entry dyspareunia and pain with inserting tampons, and most of these, since their teenage years.

It is important to be aware of the different subtypes of vulvodynia, because treatment varies according to diagnosis. The clinical features and treatment strategies for these different subtypes are discussed in the remainder of this section. It is likely that the current classification of different subtypes of vulvodynia will be modified in the future, as physicians learn more about the etiologies of the vulvar pain syndromes. Chronic infections of the vulvar area should be treated before a diagnosis of vulvodynia is made. A number of genital infections have been found to be a frequent cause of chronic vulvar pain,14 and a thorough evaluation for infections of the vulva and vagina should be carried out by a gynecologist or dermatologist experienced in this area.

Vulvar dermatoses are a frequent cause of chronic vulvar pain. A prospective study15 of patients presenting with vulvar pain showed that the majority of patients had a corticosteroid-responsive dermatosis. Unlike most other subsets of vulvodynia, vulvar dermatoses are associated with physical signs: redness, blisters, and erosions that can be recognized during a careful physical examination.16 For the physician who specializes in pain management, these physical signs on examination of the patient should be an indication to evaluate more extensively for an etiology of the pain. The differential diagnosis of these physical signs is complex and may include local as well as systemic disease. Inflammatory dermatoses, chronic contact dermatitis, lichen planus, lichen sclerosus, lichen simplex chronicus, seborrheic dermatitis, psoriasis, herpetic infections, and systemic autoimmune diseases such as Beháet’s disease and systemic lupus erythematosus have to be considered.17 The diagnosis usually needs to be confirmed by vulvar punch biopsy. Vulvar and vaginal redness and erosions also may occur in diabetes mellitus. These patients, however, typically present with vaginal itching and not pain.

Cyclic vulvovaginitis is characterized by episodic “flares” of vulvar pain, often after sexual intercourse (pain is typically worst the next day) or during the luteal phase of the menstrual cycle.18 Cyclic symptoms have been reported after hormonal changes, such as starting or discontinuing oral contraceptives, or during pregnancy. Cyclic vulvovaginitis might be multifactorial. Several contributing factors have been suggested: hypersensitivity to the Candida antigen,19 immunoglobulin A deficiency,20 and cyclic changes in the vaginal environment.21 Prolonged maintenance therapy with antimycotics, topically or systemically, is usually effective if cultures show the presence of Candida.16,18

Vulvar vestibulitis is characterized by a history of entry dyspareunia, pain at the introitus of the vagina when inserting a tampon, and painful sensations when wearing pants, or with bicycle and horseback riding. On examination of the vulva, there is often some vestibular erythema. A typical characteristic of this type of vulvodynia during examination is that touch of the vestibule is very painful.22 Often, the patient herself can point to a specific spot at the vaginal introitus that is painful to touch. The clinical observation of vestibular erythema associated with dyspareunia was described in 1928 by Kelly23: “Exquisitely sensitive deep-red spots in the mucosa of the hymenal ring are a fruitful source of dyspareunia—tender enough at times to make a vaginal examination impossible. Inflamed caruncles with or without these spots often stand guard at the introitus labeled ‘noli me tangere’.” Earlier studies implicated persistent infection as the cause of vulvar vestibulitis, but histologic and molecular studies have not supported this supposition.24–28 Interestingly, two recent studies reported vestibular neural hyperplasia,29,30 which might provide a morphologic and neurologic explanation for the pain in vulvar vestibulitis syndrome.

Approximately half of the patients with clinical symptoms of vulvar vestibulitis eventually experience spontaneous remission.31 Treatment for vulvar vestibulitis is difficult. Most treatments have been reported as uncontrolled case reports, and no long-term follow-up data have been provided. As a first step in the treatment of vulvar vestibulitis, local irritants and potential allergens should be identified and eliminated. A change to a mild, hypoallergenic laundry detergent should be considered.

The role of urinary oxalate excretion in vulvar vestibulitis is controversial. One initial case report associated vulvar vestibulitis with oxalate crystalluria32: the patient experienced pain relief with calcium citrate and a low-oxalate diet. Withdrawal of calcium citrate resulted in reoccurrence of vulvar pain, and reinstitution of calcium citrate alleviated the pain again. A more recent study (published as a commentary, only, without details of the patient population studied) claimed successful pain relief in 75% of patients treated with low-oxalate diet and calcium citrate.33 However, a controlled study in 130 patients with vulvar pain and 23 volunteers without symptoms showed that urinary oxalates may be nonspecific irritants that aggravate vulvodynia, but that the role of oxalates as instigators is doubtful.34 Because a low-oxalate diet has no side effects, a trial of this treatment modality is certainly indicated in patients interested in pursuing a dietary approach. Controlled, prospective studies with clearly defined outcome parameters are necessary to further elucidate the role of urinary oxalates in this chronic pain syndrome.

Biofeedback of the pelvic floor musculature has been reported to result in pain improvement in 83% of patients suffering from vulvar vestibulitis.35 Given that this treatment modality is not associated with any side effects and given the high response rate reported in the literature, a trial of biofeedback is warranted, before considering any more invasive treatment strategies, especially irreversible surgical approaches (see below). Various topical creams or ointments applied to the vulvar vestibule have resulted in pain relief in some patients. A mild hydrocortisone cream or ointment or an estrogen cream applied to the vaginal introitus have been helpful. In mild cases of vulvar vestibulitis, topical lidocaine applied as a 4% solution is often sufficient to make intercourse possible without serious discomfort.36 Intralesional α interferon injections have been reported to provide substantial or partial improvement in about 50% of patients.37 Isoprinosine, another agent known to enhance immune function, was found to improve pain in 60% of patients.38

Surgical approaches have been suggested with the aim of removing the painful skin area. The most common procedure is perineoplasty. The vulvar vestibule (the hyperalgesic area) is excised, and the vaginal mucosa is then advanced to cover the defect.39 Risks include general anesthesia, prolonged healing period, intraoperative bleeding, and postoperative disfigurement.36 A simplified surgical revision under local anesthesia has been suggested in a pilot study,36 resulting in complete resolution of pain in the majority of patients.

In vulvar papillomatosis, small papillae are seen around the vulvar vestibule. These papillae can be seen in conjunction with lichen simplex chronicus, or with subclinical infection with human papillomavirus. In other cases, this is a normal variant. Colposcopy and biopsy are important to rule out an infection with human papillomavirus. Treatment of the papillomatosis is usually not necessary.16 If pain persists, symptomatic treatment of the vulvar pain can be considered.

The term essential vulvodynia was originally used to describe chronic vulvar pain for which no secondary cause could be determined.21 When it was subsequently found that treatment of cutaneous neuralgia was effective in some of these patients, the term essential was replaced by the more appropriate term dysesthetic.40 Dysesthetic vulvodynia is more common in perimenopausal or postmenopausal women. In contrast to vulvar vestibulitis, in which pain is localized at the vulvar vestibule and evoked by touch, patients with dysesthetic vulvodynia report diffuse, constant hyperalgesia in the vulvar area, often extending throughout the perineum. These women complain less about dyspareunia. Some women present with features of both vulvar vestibulitis and dysesthetic vulvodynia. One article has suggested use of the term vestibulodynia for this symptom complex.41 It has been hypothesized that the vulvar hyperalgesia in dysesthetic vulvodynia is caused by a neuropathic pain syndrome, possibly of the pudendal nerve.17 In support of this hypothesis, Sonni and coworkers42 found that the pain threshold for acid solutions is decreased in women with dysesthetic vulvodynia. Many patients can be helped with medications recommended for other neuropathic pain syndromes.43 Case reports have demonstrated that low-dose amitriptyline may be effective in women with dysesthetic vulvodynia, especially in elderly women.40,44 However, no controlled studies have been reported so far, and further clinical research is urgently needed.

Iatrogenic causes have to be considered when evaluating a patient with vulvodynia. Especially as the use of potent topical corticosteroids becomes more widespread, steroid rebound dermatitis in the vaginal area is seen more frequently.18 Vulvodynia has been recognized as a complication of CO2 laser therapy to the vulva.17 The literature on the long-term effects of episiotomy on vaginal sensation is sparse. In one older, retrospective study,45 16% to 47% of the women interviewed continued to report dyspareunia 1 to 5 years after episiotomy.

In summary, vulvodynia is a recognized disease entity, and an emerging body of literature is reporting on several different etiologic factors, attesting to the multifactorial aspects of this disease. For the treating physician, it is important to realize that many women with vulvodynia are in their reproductive ages and previously had satisfying sexual relationships. In contrast to many other chronic pain syndromes, vulvodynia may only interfere to a moderate extent with the daily activities of a woman, but the disease usually interferes 100% with her sexual life. To confirm the diagnosis of vulvodynia, excluding secondary causes such as dermatitis or gynecologic infections, and to design a treatment plan, a multidisciplinary approach involving collaborations of gynecologists, dermatologists, neurologists, pain specialists, psychologists, and psychiatrists is necessary.

Clitoral Pain

In contrast to the large body of literature that has emerged over the past 15 years on vulvodynia, few reports exist on clitoral pain. In clinical practice, clitoral pain occasionally is seen in women presenting with dysesthetic vulvodynia, if the pain is extending throughout the whole perineum, and the ongoing pain (often a burning, stinging sensation) is usually exacerbated by mechanical stimuli such as tight clothing and sexual contact. Chronic pain is reported as one of the complications of female circumcision. This procedure involves excision of the clitoris and the labia minora, and is still performed on young females in many parts of the world before or as they reach puberty, as an instrument to control female sexuality and maintain cultural pride.46–48 As mobility is increasing, some of these women have moved to western countries; for example, it is estimated that 2,000 young women living in the United Kingdom undergo this ritual per year.47 Few of these women seem to seek medical attention, and the incidence of chronic clitoral pain in this group is not known.

Urethral Syndrome

Many women present to the urologist, gynecologist, or family physician with painful micturition but no evidence of organic disease, and the urine culture is negative by standard techniques. Gallagher and colleagues49 in 1965 coined the term urethral syndrome to describe this problem. It has been estimated that urethral syndrome accounts for as many as 5 million office visits a year in the United States.50 This syndrome is defined as a disease entity characterized by urinary urgency, frequency, dysuria, and, at times, by suprapubic and back pain and urinary hesitancy in the absence of objective urologic findings. Urethral syndrome typically occurs in women during their reproductive years, but it also has been reported in children and in men.51,52 In contrast to other chronic perineal syndromes involving nonmalignant pain, the rates of spontaneous remission are very high in this patient population.53,54

Several different theories have been proposed to explain the etiology of urethral syndrome, most, however, with little supporting evidence. It has been suggested that symptoms are caused by urethral obstruction and thus are surgically treatable.55,56 It is important to note that there rarely is evidence to support an anatomically obstructive etiology. Although surgical procedures aimed at relieving a urethral obstruction claim excellent results, it must be cautioned that long-term follow-up rarely is provided. These procedures involve some risk of incontinence and are of uncertain and usually temporary efficacy.57,58 Urinary hesitance, which often is reported by patients with urethral syndrome, might be the result of spasms of the external urethral sphincter, rather than an anatomic obstruction. Several studies reported a staccato or prolonged flow phase during uroflowmetry and increased external sphincter tone detected on urethral pressure profilometry in patients with urethral syndrome.51 However, these urodynamic findings may also be produced voluntarily in a neurologically intact person, and are, therefore, difficult to interpret.59 To date, an inflammatory or infectious etiology of the urethral syndrome has not been supported (reviewed in ref. 59), and controlled studies using molecular techniques to assess for infection are necessary to further clarify whether an occult infection is maintaining the chronic pain syndrome.

A thorough diagnostic evaluation is very important, because the symptoms of urethral syndrome are indistinguishable from those caused by urinary infections, tumors, stones, interstitial cystitis, and many other urologic diseases. Urethral syndrome is a diagnosis of exclusion. The urologic evaluation includes urine analysis, culture, and cytology. Radiographic studies, urodynamic studies, and cystoscopy are indicated in selected patients.59 Systemic diseases affecting the innervation of the urogenital area, including multiple sclerosis, collagen diseases, and diabetes mellitus, have to be included in the differential diagnosis. In female patients a gynecologic examination is necessary to rule out symptoms that may be secondary to a gynecologic cause. As in other chronic pain syndromes, a psychological evaluation should be part of the multidisciplinary evaluation to rule out a psychogenic etiology and to assess for symptoms of depression associated with the chronic pain problem.

Various invasive and medical treatment options have been suggested for patients with urethral syndrome59; most are anecdotal clinical reports, and controlled clinical studies are urgently needed to assess which approach might be most successful for this painful disorder. Endoscopic and open surgical procedures have been suggested to eliminate a presumed urethral stenosis. Fulguration, scarification, resection, or cryosurgery has been considered to obliterate cystoscopically apparent urethritis. Bladder instillations with a variety of anti-inflammatory or cauterizing agents and systemic therapy with anticholinergics, α-adrenergic blockers, and muscle relaxants have been advocated. High rates of success were found with skeletal muscle relaxants or electrostimulation combined with biofeedback techniques.51,58 Realizing the different surgical and nonsurgical treatment options discussed in the literature, a conservative treatment approach has been recommended as the first choice, because this usually is as effective as surgery, less expensive, and, most importantly, less subject to risk.54,60

Testicular Pain

Similar to women who suffer from pain syndromes of the reproductive organs, men with chronic testicular pain are usually embarrassed to talk about it, and often wonder if they have a hidden sexual aversion that presents itself as a pain syndrome in the genital region. Many patients cannot recall any precipitating event that led to the onset of the chronic pain syndrome. Secondary causes of chronic testicular pain include infection, tumor, testicular torsion, varicocele, hydrocele, spermatocele, trauma (bicycle accident), and previous surgical interventions.61,62 The differential diagnosis includes referred pain from the ureter or the hip or lumbar facet joints and entrapment neuropathies of the ilioinguinal or genitofemoral nerve. Chronic testicular pain has been reported as a complication of vasectomy.63 This chronic genital pain syndrome is usually not associated with erectile or ejaculatory dysfunction.61

A careful history, physical examination, and urologic evaluation reveal most secondary causes of chronic testicular pain. In selected patients, a gastroenterologic evaluation might be indicated to rule out referred pain from the lower pelvic organs or herniography to evaluate for an occult hernia. The neurologic evaluation is directed toward the lumbosacral roots, the ilioinguinal, genitofemoral, and pudendal nerves, and the autonomic nerve supply to the testis. In many cases, however, the pain remains unexplained despite a very thorough diagnostic workup. Treatment of chronic testicular pain is directed toward the underlying etiology, if an underlying etiology can be identified. A hydrocele, varicocele, or spermatocele rarely is the cause of chronic testicular pain, but rather is a coincidental finding.64

Traditionally, pain management for chronic testicular pain in the urology clinics consisted of a trial of antibiotics and nonsteroidal anti-inflammatory drugs (NSAIDs), with the aim of treating a possible occult inflammatory process. Case reports suggest that medical management, including medications used for other chronic pain syndromes such as low-dose antidepressants, anticonvulsants, membrane-stabilizing agents, and opiates, often are effective for treatment of chronic testicular pain62,65,66; however, no placebo-controlled studies have been published yet. Transcutaneous electrical nerve stimulation (TENS) might be helpful.65 Repeated lumbar sympathetic blocks with local anesthetic and oral sympatholytic drugs have been reported to result in marked pain relief in selected patients in whom a sympathetic component is suspected in the maintenance of chronic testicular pain.67 In the past, drastic surgical procedures have been recommended for the treatment of chronic testicular pain, such as epididymectomy and orchiectomy. As an alternative to surgical removal of these organs, microsurgical denervation has been suggested.68

Prostatitis and Prostatodynia

“Prostatitis” is a diagnosis that is often given to patients presenting with unexplained symptoms or condition that might possibly originate from the prostate gland.69 In the United States, approximately 25% of men presenting with genitourinary tract problems are diagnosed with prostatitis.70,71 Drach and colleagues72 defined four categories of prostatitis: (1) acute bacterial prostatitis, (2) chronic bacterial prostatitis, (3) nonbacterial prostatitis (including nonbacterial infections, allergic and autoimmune prostatitis), and (4) prostatodynia. Prostatodynia is defined as persistent complaints of urinary urgency, dysuria, poor urinary flow, and perineal discomfort and pain, without evidence of bacteria or purulence in the prostatic fluid.72 In addition to the perineal pain, patients often report that the pain is radiating to the lower back, suprapubic area, and groin. In contrast to patients with chronic testicular pain, patients with prostatodynia often complain about pain with ejaculation. Prostatodynia accounts for approximately 30% of patients presenting with prostatitis, the age range is from 20 to 60 years of age.73,74

Physical examination of the prostate is typically normal, without any signs of tenderness. A thorough urologic evaluation is indicated, including urinalysis, urine culture, urine cytology, and urethral cultures.75 Referred pain from the colon or rectum needs to be ruled out. Prostatodynia is a diagnosis of exclusion, in which it is assumed that the chronic pain syndrome is related to the prostate, but no inflammatory prostatic process can be identified.

The most frequently advocated treatment is antibiotics despite the fact that usually no infectious etiology can be found. The urodynamic abnormalities observed in some patients with prostatodynia suggest that there is increased sympathetic tone. Oral α-adrenergic blockers have been shown to improve the voiding abnormalities as well as pain; however, their use is often limited by side effects, most frequently hypotension.76 It has been suggested that there is an increase in pelvic floor muscle tone in patients presenting with prostatodynia, and pelvic floor relaxation techniques and muscle-relaxing agents have been reported to result in marked improvement of the symptomatology.77

Coccygodynia

Pain localized to the coccyx is a common perineal pain syndrome. The term coccygodynia was first used by Simpson78 to describe a chronic pain syndrome characterized by tenderness and pain in the area of the coccyx, most severe with sitting. This chronic pain syndrome occurs more frequently in women and among elderly debilitated patients.79,80 Some patients can remember a history of acute trauma to the coccyx, either a fall in the sitting position or birth trauma. Chronic trauma to the coccyx might result from poor sitting positions in which continued pressure occurs on the coccyx. Although one series related 70% of all cases of coccygodynia to a traumatic etiology,81 others have suggested that trauma is an unlikely cause of coccygodynia, and instead a rheumatic etiology should be considered.82

On physical examination the coccyx is usually tender on palpation. Because pain can be referred to the coccyx from the lumbosacral spine, sacrum, anus, rectum, pelvis, and genitourinary tract, a thorough history is important, including questions regarding a precipitating cause. It is important to evaluate for anal fissures, hemorrhoids, anorectal or gynecologic infections, or rare causes such as space-occupying lesions, including tumors. As in many other perineal pain syndromes, despite a thorough evaluation, no cause can be found in many patients with coccygodynia.

The first step in the treatment of coccygodynia is protection of the painful coccyx from further irritation by sitting in such a position that no pressure occurs on the coccyx.80 This measure alone often results in significant pain improvement after a few weeks. Physical therapy interventions, including hot sitz bath, pelvic relaxation techniques, and pelvic massage, have been reported to result in pain relief.77,83 Local infiltrations with local anesthetic of the painful area, coccygeal nerve blocks, and caudal injections with local anesthetic, alone or in combination with corticosteroids, often are helpful.83,84 Wray and coworkers84 reported that manipulations of the coccyx under anesthesia resulted in marked pain relief. In these manipulations, the coccyx is repeatedly flexed and extended with the aim of stretching ligaments so that the ordinary ranges of motion were no longer painful. In patients with intractable pain who experience significant temporary pain relief with caudal local anesthetic injections, cryoanalgesia of the posterior rami of the lower sacral nerve roots and the coccygeal nerve should be considered.85 Surgical removal of the coccyx, the treatment of choice during the nineteenth and early twentieth centuries, is rarely necessary today because conservative measures are usually sufficient.80,84 In selected patients whose pain is clearly related to the coccyx and in whom conservative measures have failed, coccygectomy is indicated and has a high success rate.84,86 However, before considering this irreversible surgical procedure, it is important to make use of conservative measures first. Importantly, Wray and coworkers84 reported a 20% relapse rate in patients who had initial pain relief with conservative measures; however, repeat therapy was usually effective in providing permanent pain relief.

Rectal Pain

Pain in the rectal-anal area can occur as constant pain—proctodynia—or as paroxysms of pain—proctalgia fugax.

Proctodynia is often caused by local disease of the anus or rectum or it can be referred from the urogenital tract or the lumbosacral spine. A comprehensive workup is indicated, because in most cases of proctodynia—in contrast to many of the other perineal pain syndromes—the underlying etiology can be found. Intractable rectal pain has been associated with pudendal neuralgia in 24% of the cases in one study, and has been treated successfully with neuropathic pain medications.87 A pudendal nerve block with local anesthetic might be helpful to assess the contribution of the pudendal nerve to the chronic pain syndrome. Chronic idiopathic anal pain has been associated with abnormal anorectal manometric profiles, probably resulting from a dysfunction of the striated external anal sphincter. Biofeedback training has been shown to be effective in these cases.88

Proctalgia fugax is characterized by sudden attacks of intense pain of short duration in the region of the internal anal sphincter and the anorectal ring. The incidence of proctalgia fugax has been reported to be as high as 14% in the general population, and as high as 33% in patients with gastrointestinal disease.89,90 Familial forms of proctalgia fugax have been described, and it is important to take a family history. The immediate cause of proctalgia fugax seems to be muscle spasms, but the etiology of this syndrome remains unclear.91 A consistent phenomenon in all studies seems to be gastrointestinal smooth muscle dysfunction. In the majority of patients with proctalgia fugax, the physical examination is normal, and common anorectal diseases such as hemorrhoids and anal fissures seem to be unrelated to the paroxysmal pain problem, in contrast to patients with constant rectal pain—proctodynia.

Simple and effective remedies have been suggested to end the acute pain attack associated with proctalgia fugax: immediate taking of food or drink, dilation of the anorectum (by digital dilation, attempting a bowel movement or inserting a tap-water enema), hot sitz baths, and firm pressure to the perineum.92–94 A variety of drugs has been suggested in anecdotal reports: antispasmodics, nitroglycerin, nifedipine, carbamazepine, diltiazem, and salbutamol (reviewed in ref. 43). Eckhardt and colleagues95 showed in a controlled crossover trial that salbutamol inhalation significantly shortened the duration of the severe pain. Because this is an easy-to-use medication during the acute attack, and given that this is the only controlled study on medications for proctalgia fugax, salbutamol should be the first choice, if a decision is made to use medications to abort pain attacks. It is important to reassure the patient that the symptoms, although quite troublesome (the pain often reaches an intensity of 10/10 on a Visual Analog Scale) are not signs of a life-threatening disease and may improve with time. Psychological assessment is important to rule out a depressive symptomatology contributing to the chronic pain syndrome.

Generalized Perineal Pain

Perineal pain can be localized to a specific area of the perineum, as previously discussed. In some cases, the perineal pain syndrome starts at a specific area and over time extends over the whole perineum. In other cases, perineal pain starts as a diffuse discomfort involving, from the beginning, most of the perineum and gradually increasing in intensity—this is a chronic generalized perineal pain syndrome.

The differential diagnosis is complex: gastrointestinal, proctologic, urologic, gynecologic, and neurologic etiologies have to be excluded. Systemic diseases associated with painful peripheral neuropathies, such as diabetes mellitus and acquired immunodeficiency syndrome (AIDS), have to be considered. French studies have reported a diagnosis of pudendal nerve entrapment in 91% of patients with perineal pain referred to a pain clinic.

Surgical neurolysis-transposition was recommended as the treatment of choice, with best results obtained in patients in whom pudendal nerve entrapment was diagnosed early.96,97 In patients presenting with perineal pain and sacral meningeal cysts (Tarlov cysts), surgical resection of the cysts has been reported to result in excellent pain relief in the majority of the patients.98 Perineal pain has been reported in the context of movement disorders. Chronic perineal pain occurred as a complication of neuroleptic drug exposure. Catecholamine depletors resulted in complete resolution of the painful sensations.99 In addition, chronic perineal pain has been reported in the context of Parkinson’s disease, and excellent pain relief was achieved using medications regularly used for Parkinson’s disease.100

The literature examining psychological factors in chronic perineal pain has been reviewed in detail.43 As with other chronic pain syndromes, in the absence of obvious organic pathology, many etiologies regarding a purely psychogenic origin of perineal pain have been entertained. Many of these studies have neglected to examine whether the psychological findings were likely to be preexisting or reactive. It would not be surprising, or necessarily indicative of psychopathology, if a patient with a chronic perineal pain syndrome was depressed. The critical issue is whether this patient was depressed before the chronic pain syndrome started, and whether his or her mood returned to normal after successful therapy for the chronic pain syndrome.

It is important that health care providers treating patients with chronic perineal pain realize that these patients are often embarrassed to talk about their chronic pain syndrome. In addition, they often are afraid of being labeled as not really suffering from a pain syndrome but having a psychosomatic or psychiatric illness, or being hypochondriacs. They also may be afraid that conclusions about their sexual life will be drawn (because the genitalia are either directly affected by the chronic pain syndrome or are close to the painful area), which might further isolate them. The difficulties that many of these patients have in talking about their chronic perineal pain syndrome becomes more obvious if one thinks about two situations in which a patient is in so much pain that he or she cannot come to work: in the first scenario, someone takes a day off from work, telling coworkers that he or she has really bad back pain, in the second scenario, a patient has to take sick leave because of an exacerbation of chronic perineal pain. The patient with perineal pain might invent an excuse, a more legitimate and accepted disease, to prevent any gossip among the coworkers.

Location of pain may be a significant predictor for appraisals of pain and disclosure of pain complaints. Klonoff and colleagues101 demonstrated that subjects asked to imagine pain in their genitals appraised themselves as more ill than if they were asked to imagine chest, stomach, head, and mouth pain. Further, subjects reported that they would be least likely to disclose genital pain and would be more worried, depressed, and embarrassed by pain in the genitals than in all other areas of the body.

Although the chronic perineal pain syndromes discussed in this chapter are quite frequent and some were described in detail more than 100 years ago, many of the patients suffering from these pain syndromes do not receive adequate pain management, and some patients do not receive any pain treatment at all. In many cases, the focus is on finding and possibly treating the underlying etiology, and patients go from physician to physician in different subspecialties, hearing after extensive evaluations that nothing abnormal can be found. However, the pain persists, and many patients who have suffered for many years from chronic perineal pain without finding a cure are quite frustrated and angry.

As a first step, it is important to acknowledge that these chronic perineal pain syndromes do exist, and that they are well-described but poorly understood. A thorough workup is mandatory, often including several medial subspecialties. Perineal pain can be a symptom of a malignant or nonmalignant disease, for which specific treatment is available, and the diagnosis of a chronic perineal pain syndrome is a diagnosis of exclusion.

In many patients who present with chronic perineal pain, the workup does not reveal any underlying pathology. In the future, novel treatment strategies might become available, targeted specifically against the pathophysiologic mechanisms of the chronic pain syndromes discussed here. Although these developments are still on the horizon, it is very important to realize what can be done now for patients who suffer from chronic perineal pain. Most currently available treatment options for these pain syndromes are empirical, only. Although these pain syndromes can rarely be cured, some pain relief can be provided to almost all patients using a multidisciplinary approach that includes pain medications (antidepressants, anticonvulsants, membrane-stabilizing agents, and opioids), local treatment regimens, nerve blocks, selected surgical procedures, physical therapy, and psychological support.