Medical Care
Medical therapy directed at the causative factor is usually initiated when the disease recurs after several trials of surgical treatment. Specific medical therapy depends on the etiology defined.
Some studies show that infliximab therapy in combination with examination under anesthesia/seton drainage is a safe and effective short-term treatment for fistulating anal Crohn disease (CD), but long-term fistula healing rates are low. [26, 27] A study by Lawrence et al found that in CD patients with granulomas, anti–tumor necrosis factor (TNF) therapy greatly reduced the risk of perianal fistula. [28]
Antitubercular therapy is instituted for tubercular causes, usually with three or four drugs for a prolonged course of therapy, and also for pulmonary disease, if present. The causative organisms often display resistance to multiple drugs.
First-line medical treatment of actinomycosis is penicillin or a penicillin-related antibiotic. Classically, intravenous (IV) penicillin G (20 MU/day) is recommended for 4-6 weeks, followed by oral amoxicillin for up to 1 year. The exact duration of therapy depends on the severity of the disease and the immune status of the patient. [8, 29]
Antifungals are used when a fungus is believed to be the etiology. The specific drug depends on the fungus identified. The treatment of choice for basidiobolomycosis is ketoconazole or itraconazole. [30]
Tetracyclines remain the treatment of choice for lymphogranuloma venereum (LGV). Doxycycline or a macrolide antibiotic is required for 3 weeks. [9] Surgical treatment of this condition is particularly difficult because of the degree of tissue destruction often observed in long-standing cases.
Perianal amebiasis in patients with HIV is treated with HIV medications, in order to improve the individual’s immune status, plus oral metronidazole. [31]
Failure of corticosteroids and azathioprine in treating perianal lesions in Hermansky-Pudlak syndrome has led to the use of infliximab with clinical improvement. [13]
Surgical Care
Preparation for surgery
Knowledge of the correct diagnosis greatly increases the likelihood that surgical therapy will be successful in perianal granulomatous disease. Since the diagnosis is often not considered until the disease is advanced or has recurred, surgeons must endeavor to maintain a high index of clinical awareness whenever a presentation seems unusual.
Operative details
Surgical intervention is predicated on presentation. If symptoms are related to fistulous disease, procedures for fistulas may be considered. If the fistula is associated with a perirectal abscess, drainage should be performed to control the sepsis source.
Surgical therapy is very straightforward, and the various procedures used in the surgical treatment of perianal granulomatous disease are largely those also used in the treatment of perianal abscess and fistula. Esoteric causes are not sought until after the disease has recurred, sometimes after several surgical procedures. [32] For symptomatic granulomas, excision may be chosen to relieve symptoms. Simple granulomas may be excised, with the skin left open to close by secondary intention.
Unusual lesions should be generously biopsied, not necessarily excised, because this may not be necessary when the etiology is known. Pus must be drained, taking care to preserve sphincter function and continence. If unforeseen extensive fistulous tracts are found, simple drainage is the correct action, with postoperative investigation of the anatomy using magnetic resonance imaging (MRI), endorectal or endoanal ultrasonography (US), or both.
LGV can cause extensive tissue destruction, sometimes destroying the rectovaginal septum and leaving the patient with a cloacalike abnormality. Reconstruction in this setting can be exceedingly difficult, given that the rectum and, often, the sigmoid are also abnormal, thus making diversion with an end colostomy the only option. HIV testing should be considered.
Postoperative Care
Nonhealing or recurrence after an otherwise simple perianal operative procedure must prompt evaluation of causes other than cryptoglandular disease of the anorectum, sebaceous cysts, or pilonidal disease. CD is always prominent in the differential diagnosis, and tuberculosis is becoming so.
Wounds are cared for as after any anorectal surgical procedure, with damp-to-dry dressings of isotonic sodium chloride solution, avoidance of constipation and diarrhea, and careful cleansing of the region. Granulation may be slow in the patient who is immunocompromised. On occasion, sharp debridement may be needed. If the wound is granulating poorly, switching from isotonic sodium chloride solution to a quarter-strength Dakin solution for 5-10 days can be helpful. Povidone-iodine should not be used for dressings, because it retards healing.
Complications
Aside from recurrence, which may occur until identification of the true etiology of the granulomas, the complications are chiefly those of any anorectal surgical procedure.
However, patients may undergo multiple procedures before the cause is identified, and the underlying etiology may cause anatomic changes in the perianal region as well. Because of this, these patients are at higher risk for complications, such as sphincter dysfunction with impaired continence, stenosis, mucosal ectropion, and rectovaginal fistula. These patients also bear the morbidity associated with the underlying etiology. Some of them may also be immunocompromised.
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Perianal granuloma and multiple fistulous tracts in patient with severe perianal Crohn disease. Note noncutting seton in place.
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Perianal granuloma in patient with severe perianal Crohn disease.
