Definition

The levator ani syndrome is also called levator spasm, puborectalis syndrome, chronic proctalgia, pyriformis syndrome, and pelvic tension myalgia. The levator ani syndrome is characterized by relatively constant or frequent dull anorectal pain, often associated with tenderness to palpation of the levator ani but not urinary symptoms or an organic disease that can explain pain.

Epidemiology

The prevalence of symptoms compatible with levator ani syndrome in the general population is 6.6%. More than 50% of affected people are aged 30 to 60 years, and it is more common in women (7.4% of all women) than in men (5.7% of all men). Although disability associated with levator ani syndrome can be significant, only 29% of people with levator pain had consulted a physician. In a postal survey of 5430 adults, people with levator ani syndrome reported missing an average of 17.9 days from work or school in the past year, and 11.5% reported that they were currently too sick to work or go to school. There are no published data on the frequency with which the levator ani syndrome is encountered in medical practice.

Pathophysiology

Tenderness to palpation of pelvic floor muscles in chronic pelvic pain and levator ani syndrome may reflect visceral hyperalgesia or increased pelvic floor muscle tension, the pathophysiology of which is unknown. Uncontrolled observations suggest that patients may have increased anal pressures or electromyogram activity. Higher anal pressures may reflect increased external or internal anal sphincter tone. Inability to relax pelvic floor muscles suggests pelvic floor dysfunction. It is unclear if the association between chronic pelvic pain and psychosocial distress on multiple domains (eg, depression and anxiety, somatization, and obsessive-compulsive behavior) reflects an underlying cause or an effect of pain.

Clinical features

The diagnosis is based on characteristic symptoms in the absence of anorectal and pelvic pathophysiology. The diagnostic criteria are 12 weeks, which may not be consecutive, of the following:

• 1.
  

  Chronic or recurrent rectal pain or aching, and

  
  
• 2.
  

  Episodes last 20 minutes or longer, and

  
  
• 3.
  

  Other causes of rectal pain, such as ischemia, inflammatory bowel disease, cryptitis, intramuscular abscess, fissure, hemorrhoids, prostatitis, and solitary rectal ulcer, have been excluded

The pain is often described as a vague, dull ache, or pressure sensation high in the rectum. It is often worse with sitting than with standing or lying down. Physical examination may reveal overly contracted levator ani muscles and pelvic floor tenderness to palpation. For unknown reasons, tenderness is often asymmetric and more frequently affects the left than the right side. The diagnosis is considered to be highly likely if patients have symptoms and abnormal physical signs and possible if patients have symptoms but not tenderness to levator palpation.

Patients with levator ani syndrome have significant elevations on the hypochondriasis, depression, and hysteria scales of the Minnesota Multiphasic Personality Inventory. This pattern occurs in chronic pain patients and is often referred to as the “neurotic triad”. Although clinical observations suggest that levator ani syndrome is frequently associated with an impaired quality of life, there is limited evidence in this regard.

Treatment

Appropriate testing (eg, sigmoidoscopy, defecography, ultrasound, or pelvic MRI) to exclude other causes of pain (eg, Crohn’s disease, anal fissures) and to identify associated conditions (eg, defecatory disorders) should be performed as necessary. There are no controlled studies of treatments for chronic intractable anorectal pain. Although uncontrolled studies suggest that electrogalvanic stimulation, biofeedback training, digital massage of the levator ani muscles, and sitz baths may be effective, management of chronic intractable anorectal pain can be a “frustrating endeavor”. Electrogalvanic stimulation improved pain in 10 of 27 patients in one study. In a study of 316 patients with the levator syndrome, 68% of patients reported “good results” after a combination of massage; sitz baths; muscle relaxants; and diathermy (method unspecified). The technique for levator massage is described later. Biofeedback therapy improved pain but had variable effects on anal pressures. Hot sitz baths may alleviate pain not only by counterirritation but also because immersion in hot water may reduce anal pressures in patients with anorectal pain. Ultrasound-guided injection of local anesthetics or alcohol for pelvic nerves (eg, pudendal nerve) is of unproved efficacy.

If the patient’s distress or other circumstances require that treatment be undertaken, the only advice that can be offered at present is to do no harm; select a treatment, such as biofeedback, that has no significant adverse consequences. Surgery should be avoided.

Definition

Proctalgia fugax is defined by sudden, severe intermittent pain in the anal area lasting several seconds or minutes in the absence of an organic disorder to explain pain.

Epidemiology

The prevalence of proctalgia fugax has been difficult to determine because sufferers tend not to report episodes to their physician except in the most severe cases. The estimated prevalence ranges from 8% to 18% and is comparable in men and women. Symptoms rarely begin before puberty.

In the US Householder Study, subjects with proctalgia fugax missed an average of 12.8 days from work or school in the past year, and 8.4% of them reported that they were currently too ill to work or attend school. It is unknown, however, if the reported disability was the result of proctalgia fugax, which seems unlikely, or other disorders in these patients.

Pathophysiology

The pathophysiology of proctalgia fugax is unclear and is entirely based on small case reports, which observed increased myoelectric activity and anal resting pressure during episodes of proctalgia. An uncontrolled study suggested that most patients were perfectionistic, anxious, or hypochondriacal. A hereditary form of proctalgia fugax associated with hypertrophy of the internal anal sphincter has also been reported.

Clinical features

The diagnosis is based on characteristic symptoms in the absence of anorectal and pelvic pathophysiology. The criteria are 12 weeks, which may not be consecutive, of the following:

• 1.
  

  Recurrent episodes of pain localized to the anus or lower rectum, and

  
  
• 2.
  

  Episodes last from seconds to minutes, and

  
  
• 3.
  

  There is no anorectal pain between episodes.

Attacks are generally not related to a specific triggering factor, are often precipitated by stressful life events or anxiety, and may last from a few seconds to as long as 30 minutes. In a study of 148 patients of whom one third had proctalgia fugax, the pain was localized to the anus in 90% of patients, occurred less than five times a year in 51%, and lasted less than 1 minute in 57%. In most, activity was not interrupted by this pain and only 20% had ever reported it to a physician. The pain has been described as cramping, gnawing, aching, or stabbing; may range from uncomfortable to unbearable; and radiates infrequently.

Management

For most patients, the episodes of pain are so brief that remedial treatment is impractical. Because symptoms occur infrequently, prevention is not feasible. The emphasis is on reassurance and explanation. For patients with frequent symptoms, treatment may be considered. In a randomized controlled trial, the inhaled β ₂ -adrenergic agonist salbutamol was more effective than placebo for shortening the duration of episodes of proctalgia. The α ₂ -adrenergic agonist clonidine reduced symptoms in a single patient. Coexistent psychologic issues should be addressed with behavioral or pharmacologic therapies.