Fig. 36.1
Chronic or recurrent rectal pain. 1. Pain present for at least 6 months is required for a diagnosis of functional anorectal pain syndrome. Pain associated with bowel movements, menses, or eating excludes the diagnosis of functional anorectal pain. 2–3. The history and physical exam should identify alarm and other features suggesting structural disease, such as severe throbbing pain, sentinel piles, fistulous opening, anal strictures, induration, and anal tenderness during digital examination or while gently parting the posterior anus. Relevant organic causes of pain including inflammatory bowel disease, perianal abscesses, anal fissure, and painful gynecological conditions should be considered and identified by appropriate testing. If pain is associated with and worsened by menses, conditions that may include endometriosis, dysfunctional uterine bleeding, or another gynecological pathology should be evaluated by pelvic examination, pelvic ultrasound, and/or referral to a gynecologist. The minimum diagnostic workup (in the absence of alarm signs) includes the following: CBC, ESR, biochemistry panel, flexible sigmoidoscopy, and perianal imaging with ultrasound or MRI. If there is a high index of suspicion for anal fissures, anoscopy should be considered. 4–7. If the diagnostic workup for alarm signs or symptoms identifies an abnormality (i.e., evidence of another disease explaining the anorectal pain), treat accordingly. If treatment of the other disease resolves the pain, this excludes the diagnosis of proctalgia fugax or levator ani syndrome. If treatment does not resolve the pain, go to Box 8. However, if the diagnostic workup for alarm signs or symptoms does not identify an abnormality, continue evaluation for proctalgia fugax or levator ani syndrome (Box 8). 8. An important feature of the history is whether the pain is episodic with pain-free intervals. 9. Patients with proctalgia fugax have brief episodes of pain lasting seconds to minutes with no pain between episodes. 10–12. Patients with levator ani syndrome and unspecified functional anorectal pain have chronic or recurrent anorectal pain; if recurrent, the pain lasts for 30 min or longer during episodes. Levator ani syndrome, unlike unspecified functional anorectal pain, is associated with tenderness during posterior traction of the puborectalis. Used with permission from: Bharucha AE, Rao SC, Wald A. Anorectal Disorders. In Kellow J, Drossman DA, Chang L, Chey W, Tack J, Whitehead WE (eds). Rome IV Diagnostic Algorithms for Common GI symptoms. Chap. 6, pp. 112–131. Rome Foundation, Raleigh, NC, 2016
Many treatments, largely directed at reducing tension of the striated muscles of the pelvic floor, have been used with little supporting evidence. These include electrogalvanic stimulation [4, 5], biofeedback training [6], muscle relaxants [3], digital massage of the levator ani muscles [7], and sitz baths. There is no evidence to support the use of botulinum toxin A injections [8], and surgery to cut the puborectalis muscle should be avoided.
The most rigorous study to date evaluated biofeedback, electrogalvanic stimulation, and digital massage in a group of patients with chronic proctalgia [9]. Patients were stratified into those with “levator ani syndrome” or “unspecified functional anorectal pain” on the basis of the examination. Patients in each group were further divided into those who exhibited a dyssynergic defecation pattern on the basis of anorectal manometry and balloon expulsion testing. The results were impressive. In the levator ani group, those with evidence of dyssynergia had an 87% response to biofeedback treatment (consisting of 5 weekly sessions) at 1 month but no benefit was observed in patients who did not have this finding. Response to biofeedback exceeded that of 9 sessions of electrogalvanic stimulation (45%) which in turn exceeded the response to 9 sessions of digital massage (22%). There were no differences in patients with either levator ani syndrome or unspecified anorectal pain if they did not exhibit dyssynergia. While this single center study was impressive, future studies are needed to confirm these findings. However, this study suggests a therapeutic pathway for many of these patients.
Future Studies
More studies of this difficult disorder are needed to determine best treatments. The results of the Chiarioni study need to be confirmed by independent centers. Electrogalvanic stimulation should be studied further as a possible alternative to biofeedback for patients who reside in areas where biofeedback expertise is not available.
While chronic proctalgia is not a fatal illness, quality of life is significantly impaired, as it is in most patients with chronic pain syndromes. Above all, selecting a treatment without harmful risks should remain paramount.
Proctalgia Fugax
Clinical situation: An otherwise healthy man presents with a several year history of recurrent self-limited attacks of severe anal pain lasting up to 15 min. He has had at least six episodes during the past 2 years and feels well between episodes.
Question: What is the cause of my pain and is there anything that can prevent these attacks from occurring?
Answer: You have a condition called proctalgia fugax. While the pain is very uncomfortable, it is not associated with permanent damage to the rectum or surrounding muscles.
It is thought that the pain is a result of spasm of some of the muscles of the anus or pelvic floor. Because most persons do not consult with physicians and because it is uncommon to see patients when they are symptomatic, there are no studies to determine how or why these attacks occur. Also, there is no clear evidence for treatment which is often not necessary if the attacks are brief or infrequent. Some physicians recommend albuterol inhalants, amylnitrate or diltiazem ointment or clonidine in patients whose episodes last more than 30 min, but there is little or no evidence that they are effective.
Although the episodes are unpleasant, there is no permanent damage and the disorder is considered “harmless.”
Commentary: The prevalence of proctalgia fugax in the population may be as high as 18%, but only about one in five patients consult a health care professional [10]. The diagnosis is made on clinical criteria, which specify that episodes of pain are unrelated to defecation and last no more than 30 min, and by the exclusion of other disorders such as prostatitis, coccygodynia, and major structural alterations of the pelvic floor [2] (Fig. 36.1).
The short duration and episodic nature of these attacks have made this disorder virtually impossible to study and characterize. Studies have suggested that abnormal smooth muscle contractions may be responsible for the pain [11]. Several families with a hereditary form of proctalgia fugax were found to have hypertrophy of the internal anal sphincter [12, 13]. If this finding were universal, drugs to reduce internal anal sphincter tone would be theoretically appropriate, but there is no data to support this in the vast majority of cases.
The appropriate approach to patients is an explanation about the disorder, that it is unpleasant but harmless and to provide reassurance. For patients who have frequent attacks with a duration more than 20 min, the use of an albuterol inhalant (a beta adrenergic agonist) has been reported to shorten attacks [14]. Others have recommended clonidine [15], or nitrate ointments and diltiazem ointments based upon anecdotal reports. There are no studies of anxiolytic or antidepressant agents in proctalgia fugax but these may be indicated in patients exhibiting depression, anxiety, and other mood disorders.
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