Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_927_Библиотеки_им_академика_М_И_Перельмана.pdf
P H
S
Mark L. Manwaring
idradenitis suppurativa (HS), which was rst described by Alfred
Velpeau in the early 1800s, is a chronic inammatory condition
H
of apocrine sweat glands aecting between 2% and 4% of young adults.
HS results in signicant morbidity in many patients. Although it
most frequently involves the axillae and groin, perianal HS confronts
the surgeon with a sometimes frustrating refractory inammation
that can mimic other types of perianal sepsis. Given the signicant
variability in presentation, the Hurley staging system provides a
measure of disease severity (Table 8-1).
ETIOLOGY
Most investigators agree that HS is caused by follicular occlusion
and secondary infection of apocrine sweat glands. However, genetic,
immunologic, and hormonal factors may inuence the development
of the disease. Pathologic analysis of resected specimens suggests that
compromised support of the follicular unit results from the specic
abnormalities seen immunohistochemically. Elevated levels of interleukin (IL)-1β, tumor necrosis factor (TNF)-α, IL-10, and matrix
metalloproteinase-2 have been found in tissues of patients with HS.
Upon recognition that TNF inhibitors aect the disease, studies have
been performed in an attempt to identify the relevant pathways.
Although involvement is limited to areas of the body with apocrine
glands, these glands are present with the same density and size in
patients with HS as in control subjects. Deodorants and shaving do
not seem to aect disease development. Obesity seems to be associated with HS, but it is unclear whether this association is due to
related hormonal changes or associated dermal trauma.
Patients with HS oen have a family history of the disease (in one
study, 18 of 70 patients were reported to have a family history), supporting a genetic predisposition. However, no consistent genetic cause
has been identied. Smoking is signicantly more common in patients
with HS than in matched control subjects. HS occurs in patients with
other chronic inammatory conditions, most notably Crohn disease,
although causal relationships have not been established. Negative
bacterial cultures from HS lesions are common, and the exact role of
TABLE 8-1: Hurley Staging of Hidradenitis
Suppurativa
Stage Features
I Isolated abscess(es) without scarring, sinus tracts
II Recurrent abscesses with tract formation and cicatri-
zation; single or multiple widely separated lesions
III Diuse or near-diuse involvement or multiple inter-
connected tracts and abscesses over an entire area
infection in persons with HS is controversial. Infection probably plays a
secondary role and likely worsens the progressive scarring seen in later
stages of the disease. Cultures of deeper tissues may not have the same
results as those of more supercial lesions.
CLINICAL PRESENTATION AND EVALUATION
HS initially appears as painful, round, subcutaneous nodules that may
resolve or progress over days or weeks to abscesses. ese abscesses
do not typically point to the surface as with staphylococcal infections but rather become progressively inamed and swollen, causing deeper dermal involvement. ese abscesses oen spontaneously
rupture and may drain seropurulent malodorous debris. Symptoms
may spontaneously improve in one area with no signicant treatment
but oen recur in another area and frequently become progressive.
Disease may aect only one site or multiple areas simultaneously.
Areas of suppuration may coalesce and stulize to adjacent tissues,
resulting in subcutaneous tracts or sinuses with signicant associated induration. In later stages of the disease, chronic inammation
results in multiple draining sinuses from underlying suppuration and
leads to signicant scarring. Skin contractures in the axilla or groin
may eventually develop. Over the long term, patients with HS have
a 2% to 4% risk of developing squamous cell carcinoma in the scars.
Although it is rarely life threatening, HS is a debilitating, painful,
recurrent condition that causes signicant alteration in quality of life.
Many patients experience embarrassment and social isolation and struggle with job retention because of recurrent exacerbations of the disease.
Because perianal sepsis frequently has a cryptoglandular source and
other perianal dermatological diseases exist, the diagnosis of perianal
hidradenitis may not be obvious. e dierential diagnosis includes
pilonidal disease, anal stula, or perianal Crohn disease. Careful examination with appropriate lighting and anoscopy is required to establish
the diagnosis and rule out other causes. At times, examination aer
induction of anesthesia is necessary to trace stulous connections and
conrm that the anal canal is not involved. A diagnosis of HS is suggested by the multiple supercial cutaneous scars. None of the sinuses
or stulas extends to the dentate line, in contrast to cryptoglandular
sepsis, which originates in the anal crypts.
ANTIBIOTIC TREATMENT
Treatment of HS is based on presentation, distribution of disease,
and stage (see Table 8-1). Medical treatment is standard for stage
I disease and includes a wide range of agents. Long-course antibiotic
therapy has shown some ecacy and typically includes tetracycline,
erythromycin, lymecycline, or doxycycline. Topical clindamycin
lotion has shown similar ecacy. ese regimens have a temporary,
likely suppressive eect on the disease but are not generally curative.
41
Perianal Hidradenitis suPPurativa42
Stage IIIStage IIStage I
Stop smoking Antibiotics Minimize trauma
Biologics if indicated for concurrent disease
Consider targeted
local resection
Primary closure
Local flap closure
Secondary intention
FIGURE 8-1 Treatment choices.
Consider radical
resection
Flap closure
Skin graft
Secondary intention
Cultures can be helpful to tailor antibiotic therapy but are oen negative, and deeper tissues may be infected by other organisms.
NONANTIBIOTIC TREATMENT
Oral isotretinoin, which is used for acne, does not help patients with
HS, but oral acitretin showed ecacy in a small study. is drug is teratogenic, however, and thus its use accordingly is limited. Small studies
support the use of dapsone, which has temporary ecacy in fewer than
40% of patients. Hormonal modulation with estrogen agents and nasteride has had positive results, and some evidence indicates that hormonal
modulation may be more eective than use of antibiotics. Some case
reports suggest improvement with zinc supplementation, metformin,
and other agents, but data are insucient to support their routine use.
Initial recognition that persons with Crohn disease who had HS
experienced improvement in symptoms with TNF-α inhibitors has
prompted an evaluation of these biologic agents in treating refractory
HS. Although data are limited, iniximab and adalimumab have shown
short-term response rates from 60% to 80%. ese agents should be
considered in patients with concurrent inammatory bowel disease.
SURGICAL TREATMENT
For acutely infected lesions with undrained pus, adequate drainage is
required. Most drainage procedures, including marsupialization, or
limited local excision, have a temporizing eect. Unlike drainage of
simpler abscesses, isolated drainage in HS is rarely curative. Antibiotics are used liberally given the underlying chronic inammation. Most
clinicians regard the morbidity of excision excessive for stage I disease,
and medical regimens are used to suppress the process and prevent
progression, although this approach has had limited success (Fig. 8-1).
Surgical excision is the best chance for cure for patients with stage
II or III HS, because it is the only treatment that removes chronically
scarred tissues. In general, the more radical the resection, the fewer
the recurrences of disease. is nding underscores the predilection
for recurrence in any residual hair-bearing skin. Resection should
remove the entire grossly involved area with a negative margin of so,
healthy underlying tissue, which frequently requires excision down
to the fascia.
e main challenge aer excision is to select the best closure
method. Primary closure is appropriate when wound edges can be
approximated and contamination is minimal. Local advancement
aps can decrease the size of the defect or assist in primary closure.
ese techniques oer faster healing at the risk of postoperative
infections and recurrence. Healing by secondary intention is best
for grossly contaminated wounds, and with meticulous wound care,
this approach oers the lowest recurrence rates at the expense of prolonged healing. Immediate and delayed skin graing may shorten
healing time and lessen the incidence of contractures, as well as oer
a better cosmetic result.
FIGURE 8-2 Anal stenosis after excision of hidradenitis.
Care should be taken in circumferential excision of perianal
hidradenitis because anal stenosis may result from postsurgical scarring (Fig. 8-2). Flap anoplasty can be used to avoid or treat this complication. As with all excisions, infectious complications and delayed
healing are common. Although unroong of lesions, laser ablation,
and photodynamic therapy have been reported, none of these techniques is currently recommended.
SUMMARY
Perianal HS is frequently a frustrating disease for patients, and correct
diagnosis demands that other causes of perianal sepsis be excluded.
Although temporizing medical regimens may have limited success,
surgery is oen required. Localized lesions may be treated with incision and drainage, but recurrence should be expected, and patients
with frequent recurrences should be treated with excision. Whether
the wound is le to heal by secondary intention or closed with or
without advancement aps, progressive recurrent infection oen
carries much greater long-term morbidity for patients than surgery.
S u g g e S t e d R e a d i n g
Boer J, Nazary M. Long-term results of acitretin therapy for hidradenitis sup-
purativa. Is acne inversa also a misnomer? Br J Dermatol. 2011;164:170–175.
Ellis LZ. Hidradenitis suppurativa: surgical and other management tech-
niques. Dermatol Surg. 2012;38:517–536.
Kra JN, Searles GE. Hidradenitis suppurativa in 64 female patients: ret-
rospective study comparing oral antibiotics and antiandrogen therapy.
J Cutan Med Surg. 2007;11:125–131.
Morgan WP, Hughes LE. e distribution, size and density of the apocrine
glands in hidradenitis suppurativa. Br J Surg. 1979;66:853–856.
Morgan WP, Leicester G. e role of depilation and deodorants in hidradeni-
tis suppurativa. Arch Dermatol. 1982;118:101–102.
Nesmith RB, Merkel KL, Mast BA. Radical surgical resection combined with
lymphadenectomy-directed antimicrobial therapy yielding cure of severe
axillary hidradenitis. Ann Plast Surg. 2013;70:538–541.
Randhawa HK, Hamilton J, Pope E. Finasteride for the treatment of
hidradenitis suppurativa in children and adolescents. JAMA Dermatol.
2013;149:732–735.
Slade DE, Powell BW, Mortimer PS. Hidradenitis suppurativa: pathogenesis
and management. Br J Plast Surg. 2003;56:451–461.
van Rappard DC, Limpens J, Mekkes JR. e o-label treatment of severe
hidradenitis suppurativa with TNF-alpha inhibitors: a systematic review.
J Dermatolog Treat. 2013;24:392–404.
M
P A
J. Andres Cervera-Servin, Rodney J. Kratz, and Daniel P. Froese
INTRODUCTION
Patients with pruritus ani experience symptoms of perianal itching,
burning, or soreness. ese symptoms may occur during the day or
night, with higher intensity aer bowel movements, and may even
interfere with sleep. e prevalence may increase during the warm
summer months with increased sweating and moisture. Pruritus ani
aicts 1% to 5% of the population, with men experiencing symptoms
more oen than women at a 4:1 ratio, possibly because men have a
longer anal canal. e condition predominantly aects persons aged
20 to 40 years and is less common in elderly persons even though
they have more frequent episodes of perianal soiling. Itching leads
to scratching, which provides temporary relief. However, continued
scratching damages the protective skin surface, causing further seepage, which in turn exacerbates the itching. In this manner a vicious
cycle of itching and scratching can develop, and the scratching may
lead to a secondary infection. Overuse of wipes, ointments, or other
topical treatments that contain chemicals caustic to the skin may
worsen the symptoms, and any ointment, although it provides temporary relief, ultimately prolongs symptoms because of its wetness.
ETIOLOGY
e causes of pruritus ani can be classied as primary (idiopathic)
and secondary. Primary pruritus ani is most common, but secondary
causes must be sought and ruled out before a diagnosis of primary
pruritus ani can be made. Secondary causes (listed in Table 9-1) are
dealt with initially because they are the easiest to treat once diagnosed. Diagnosis is determined on the basis of a thorough history
and physical examination and a careful examination of the perianal
skin.
PRIMARY CAUSES OF PRURITUS ANI
Aer secondary causes have been ruled out, the remaining cases are
the result of idiopathic or primary disease. Perianal skin contact with
feces has been established as a cause of pruritus ani, and thus any
fecal contamination of perianal skin may be related to the condition.
Food may cause the stool to become excessively liquid and acidic.
Types of food that may contribute to pruritus ani are listed in Box 9-1.
Each of these foods has an intake threshold above which susceptible
patients will have pruritus ani and below which they will not have it.
ese thresholds vary among individuals.
Compulsive anal cleaning can lead to pruritus ani. Frequent wiping with continuous and excessive cleaning can damage the skin and
sometimes leads to a secondary bacterial infection that perpetuates
and intensies the problem. In the absence of an identiable underlying cause, these patients are oen misdiagnosed and mistreated and
have persistent symptoms.
Pathophysiology
Pathophysiologic research has helped us understand the underlying
disorder. Eyres and ompson noted that symptomatic patients had
an exaggerated rectal anal inhibitory reex with rectal distention,
leading to subsequent soiling. Allan and coworkers compared symptomatic patients with control subjects, and in patients with pruritus,
earlier leakage from the anal canal was noted when the rectum was
lled with saline solution. Smith etal used anal manometry to demonstrate diminished sphincter tone aer ingestion of coee. Farouk
etal made three important observations: rst, an internal sphincter
pressure decrease that was greater in pruritic subjects than in control
subjects; second, a prolonged duration of internal sphincter relaxation aer rectal distention; and third, symptoms of seepage that correlated with abnormal sphincter relaxation. All these observations
describe an abnormality in internal sphincter function, either as a
primary defect or aer ingestion of coee.
HISTORY
e investigation of pruritus ani is centered on possible causes.
Obtaining a complete history is important in narrowing down the
possible cause (Box 9-2).
EXAMINATION
Examination involves careful inspection of the perianal skin for the
presence of any seepage or soiling. e appearance of the skin changes
may be classied as follows, although it is of limited clinical signicance:
Stage 0: Normal skin
Stage 1: Red and inamed skin
Stage 2: White, lichenied skin
Stage 3: Coarse ridging of the skin with ulceration superimposed on
lichenication
Distribution of the rash is important in that diet-induced pruritus ani is centered symmetrically around the anus, whereas an infection is located asymmetrically at the anal orice. is asymmetric,
infected rash may occur as a result of scratching during sleep and
usually corresponds to the side of the patient’s dominant hand. e
diagnosis of specic infections, including scabies and the dermatophytoses, is sometimes possible with a discerning eye. Upon further
inspection one should note the presence of anal disease, including
prolapsing hemorrhoids, an anal ssure or stula, skin tags, rectal
prolapse, or mucosal ectropion (Box 9-3). Suspicion of any dermatologic condition mandates examination of the entire body. Anoscopy
may reveal redundant internal hemorrhoids or possibly prolapsing
rectal mucosa, both of which allow mucus to leak from the anus. In
these cases, elastic band ligation may be benecial.
43
ManageMent of Pruritus ani44
TABLE 9-1: Secondary Causes of Pruritus Ani
Cause Mechanism
Anorectal conditions Rectal prolapse
Hemorrhoids
Fistula-in-ano
Anal ssure
Skin tags, mucosal ectropion
Villous adenoma
Hidradenitis suppurativa
Seepage either of mucus or
stool leading to inadequate
cleanliness; prevention of
the anus from closing
eectively leads to seepage
Infectious STD
Viral: human papilloma virus, condyloma acuminatum, herpes II
Bacterial: gonorrhea, syphilis
Non-STD
Bacterial: tuberculosis, Corynebacterium minutissimum (erythrasma)
Fungal: Candida albicans and dermatophytes (e.g., Epidermophyton occosum,
Trichophyton mentagrophytes, and T. rubrum) cause a characteristic unilateral
ringworm appearance on the skin
Parasites: pinworm (Enterobius vermicularis), pubic louse (Pediculosis pubis), and
scabies (Sarcoptes scabiei)
Alterations of
fecal continence
Surgical: restorative proctocolectomy with ileoanal anastomosis;
proctosigmoidectomy with coloanal anastomosis
Trauma: anal sphincter damage; sexual practices causing chronic anal dilatation
Medical: diseases that cause sphincter function loss or deterioration (neurologic:
CVA, MS, and MND)
Primary skin
disorders
Contact dermatitis
Psoriasis
Eczema, atopic dermatitis, and seborrheic dermatitis
Lichen planus and lichen simplex
Lichen sclerosus
Leukoplakia
Radiation dermatitis
Neoplastic Extramammary Paget disease
Bowen disease, anal intraepithelial neoplasia, perianal neoplasms
An underlying irritation or
inability to maintain perianal hygiene
Increased exposure of perianal
skin to fecal material as a
result of a weakened
sphincter and/or absent
rectal vault
Contact dermatitis due to
allergens in soaps, detergents, bleaches, and even
perfumes
Drugs Antidepressants (e.g., Prozac and Zolo), ACE inhibitors (e.g., Captopril and
Vasotec), antibiotics (e.g., ampicillin, amoxicillin, tetracycline, and cephalosporins),
colchicine, digoxin, diuretics, laxatives, and many chemotherapeutic treatments
Systemic disorders Diabetes mellitus
Jaundice
Uremia
Leukemia, lymphoma
Diarrhea is the common
denominator in most drugs
related to pruritus ani
Diabetes mellitus causes
pruritus as a result of internal sphincter weakening,
decreased sensation, or
fungal overgrowth
ACE, Angiotensin-converting enzyme; CVA, cerebrovascular accident; MND, motor neuron disease; MS, multiple sclerosis; STD, sexually transmitted disease.
BOX 9-1: Foods Possibly Causing Pruritus Ani
• C o e e
• Beer
• T e a
• Milk
• C o l a s
BOX 9-2: Pruritus Ani History
1. Duration and timing of symptoms
2. Obvious precipitating factors: exposure to other people with
intense itching, pets, strange environments, chemicals, use of
soaps, tissue paper, and even the type of underclothes worn
3. Contact allergens are ruled out by addressing the aforemen-
• Tomatoes
• Chocolate
• Citrusfruits,vitaminC
• Hotspicesandassociatedfoods
ments (including those prescribed to treat pruritus)
4. Hygiene habits are noted
5. Chronic diarrhea: primary or secondary
Continued
ANAL AND PERIANAL REGION 45
BOX 9-2: Pruritus Ani History—Cont’d
6. Drughistory:ruleoutapossiblepharmaceuticalcause,such
as use of antibiotics
7. Dermatologic conditions of a systemic nature
8. Prior sexually transmitted disease diagnoses and treatment
9. Systemic conditions that may be related, such as diabetes mellitus, biliary disease, and uremia
10. Dietary habits, especially use of coee, tea, colas, chocolate, beer,
milk, tomatoes, citrus fruits, and vitamin C, even in amounts
not expected to cause symptoms, such as in multivitamins;
excessive uid intake is also noted, along with food allergies,
which may necessitate keeping a food and liquid intake diary
11. Presence of soiling, seepage, or full fecal incontinence
BOX 9-3: Examination and Further Diagnostic Testing
Routine
• Carefulinspectionofperianalregion
• Digitalrectalexamination,anoscopy,orrigidproctoscopy
Case-by-Case Basis
• Examinationofskinontheentirebody
• Swabculture(performedbeforetheuseofalubricant)
• Skinscrapingsformycologicexamination
• Skinbiopsy(toruleoutneoplasticchanges)
• Woodlamptestingiferythrasmaissuspectedasaresultof
Corynebacterium minutissimum (the classic nding is pink
uorescence due to porphyrin production); erythrasma results in scaly red-brown patches on the perianal skin, axillae,
groin, or intergluteal folds
• Scotchtapetestofperianalskin(todetecteggsinpersons
with a pinworm infestation)
• Laboratorytests:completebloodcellcountandliverand
renal tests; glucose testing and thyroid function tests may be
considered
• Anorectalmanometrytoassessinternalsphincterfunction
(in specic cases)
• Colonoscopy(toruleoutproximalneoplasiainpersonswith
refractory pruritus)
TREATMENT
Once a cause is established or excluded, treatment may begin. Certain general therapies are of benet in most cases despite the underlying cause. However, sometimes treatment is started without knowing
the underlying cause of the pruritus because patients may have tried
over-the-counter therapies and may have seen several physicians in
their eorts to obtain relief of symptoms (Box 9-4).
SECONDARY PRURITUS ANI TREATMENT
Any specic cause should be identied and treated aer starting the
general measures described in Box 9-4.
BOX 9-4: General Measures for Treatment of Pruritus Ani
General measures aim to keep the perianal region dry and free of
further pruritic stimuli:
1. Stop applying soaps, topical creams, or any other therapies
to the perianal skin and avoid use of scented toilet paper and
wipes, which contain chemicals
2. Stop scratching: Although scratching temporarily relieves
symptoms, it perpetuates the problem; it may be necessary
to cut ngernails and even wear cotton gloves at night, when
most scratching occurs
3. Avoid overcleaning of the perianal region, avoid excessive friction with toilet paper aer bowel movements, and use moist
cotton or toilet paper to gently blot the area
4. Wear loose underclothes to allow the perianal skin to dry
5. Review hygiene habits; cleaning with water or hygienic cleansing lotion (Balneol) and drying with a cool hair dryer or gentle
blotting with a clean cloth are recommended
6. Keeptheperianalareadry:
• Avoiddrugsthatcausediarrheaandothercausesofdiarrhea
(such as certain foods)
• Usedietaryber(e.g.,Metamucilwafers)toaddbulkto
stools
• Considercorrectionofanorectalconditionsthatcauseseep-
age (see Table 9-1)
7. Avoid foods possibly related to pruritus (see Box 9-1)
Infections
e following infections may be encountered:
• Sexuallytransmitteddiseases, includinghumanpapillomavirus,
condyloma acuminatum, and herpes simplex (see Chapter 12 for
specic treatment)
• Erythrasmawhichistreatedwitha10-daycourseoferythromy-
cin, 250 mg by mouth three times a day
• Tuberculosis(rare)istreatedforafull6months,usuallywithiso-
niazid, rifampin, and pyrazinamide with streptomycin or ethambutol; an infectious disease consultation is recommended
• Secondary infection (signaled by excess scratching) is usually
staphylococcal and is treated with oral dicloxacillin
• β-Hemolytic streptococcal infections, which tend to recur, are
treated with oral penicillin
• Pinwormistreatedwithmebendazole,100mgbymouthinasin-
gle dose and repeated in 2 weeks; the cure rate is 90% but requires
follow-up and treatment of family members in the household
• Pubiclice andscabiesaretreatedwithlindane1%(Kwell),whichis
applied to the infected area, le in place for 8 to 12 hours, and then
washed o to prevent adverse eects; this treatment must be repeated
in 1 week; alternatives include permethrin and pyrethrin creams
• FungalinfectionsareusuallyduetoaCandidaspecies;treatment
of the underlying systemic condition that fosters this infection is
required with topical nystatin, clotrimazole, miconazole, or other
antifungal agents
• Dermatophytic infections may require a more specic therapy,
such as salicylate/benzoate compound (Whiteld’s) ointment, if
they do not respond to azole agents
Anorectal Conditions
Causes of seepage and soiling can be corrected by treating these conditions with dietary modications and medications. If the conditions
fail to improve, surgery or oce procedures, such as hemorrhoidal
rubber band ligation, may be considered. Any fecal incontinence
should be treated.
Dermatologic Conditions
Perianal irritation is oen only part of a more extensive condition
involving other parts of the body. A dermatology consultation is
strongly recommended.
Eczema (atopic dermatitis), psoriasis, lichen planus, lichen simplex, and lichen sclerosus respond to general measures and application of topical steroids.
ManageMent of Pruritus ani46
Leukoplakia requires a ruling out of neoplasia and responds well
to removal of the lesion.
Contact dermatitis requires removal of the allergen and use of topical, mild steroids. Long-term use of a strong steroid can break down
the skin barrier, creating a chronic scenario, and is to be avoided.
Neoplastic Causes
e most important step is to diagnose and recognize neoplastic conditions as the cause of pruritus ani and treat the patient accordingly.
Extramammary Paget disease, Bowen disease, and anal intraepithelial
neoplasia should always be considered with a high index of suspicion.
Any lesions of concern warrant a biopsy to rule out any carcinomas
or melanoma for both diagnosis and potential relief of symptoms
prior to denitive treatment (see specic chapters for detailed treatment protocols).
Systemic Disease
Diabetes, jaundice, and uremia require therapy to improve pruritus
symptoms by bringing blood levels of glucose, bilirubin, and urea to a
normal range. In persons with biliary disease and uremia, cholestyramine may be helpful to control the diarrhea.
TREATMENT OF PRIMARY
PRURITUS ANI
Traditionally, primary (idiopathic) pruritus has mainly been considered to be due to dietary factors (see Box 9-1). Fecal soiling is also
an important cause. It is possible that most of the “idiopathic” cases
are due to causes that have not been identied but result in perianal
irritation in susceptible persons, as demonstrated by Caplan’s study.
Treatment includes dietary restriction of foods that are possibly
related to the condition (see Box 9-1). A thorough and systematic
dietary history is required to determine which foods might be contributing to the problem. In recalcitrant cases, it may be necessary to
keep a detailed food and uid intake diary. Patients are instructed to
eliminate any substance from their diet that seems to be related
to their condition. Aer removal of the oending agent, at least 2 to 3
weeks may elapse before the symptoms cease. In long-standing cases
even more time may be required, and thus perseverance is encouraged. Once relief is obtained, some of the foods may be reintroduced
to an identied “threshold,” which will be dierent for each individual. Staying below that threshold is then appropriate in the long term
to prevent symptom recurrence.
REFRACTORY OR PERSISTENT PRURITUS ANI
When symptoms remain severe despite dietary restrictions, the
patient will need to take further steps to obtain relief (Box 9-5). ese
steps should be applied together with the general measures described
in Box 9-4 and the dietary restrictions previously outlined.
SUMMARY
Pruritus ani is common, and in almost all cases the cause may be
identied by a careful history and examination of the perianal area
(Fig. 9-1). Most patients will benet from use of the general mea-
sures described in Box 9-4, as well as specic treatment of the primary cause. Further investigation and/or treatment may be required
BOX 9-5: Options for Treating Refractory Pruritus Ani
1. Topical treatment
• Irrigatetherectumaerabowelmovementtoremovefecal
residue and then place a cotton ball or white so tissue in the
anal canal to block fecal seepage and absorb moisture away
from the skin; cornstarch or talc on the perianal skin may aid
in drying the skin
• Useofbarriercreams,includingcalamine,Calmoseptine,or
zinc oxide, is safe; they may be used on a long-term basis to
avoid chronic moisture on the skin
• Useofasteroid-basedointmentissafeandeectiveinalow
dose (e.g., hydrocortisone 1%), but such an ointment should
be used for a limited amount of time (no more than 3
weeks)
• Hydrocortisone,aglucocorticoidwithanti-inammatory,
antipruritic, and vasoconstrictive properties, decreases local
inammation, itching, and swelling
• Steroidcombinedwithothermedications:
Hydrocortisone acetate 1%, but never more than 2.5%, com-
bined with pramoxine (a local anesthetic), is also eective
A combination of hydrocortisone/iodoquinol (Vytone) and
clotrimazole (Lotrimin) is used when infection is suspected, with dicloxacillin recommended for persons with a
proven, severe infection
Antifungal creams and ointments, with rst-line azole deriva-
tives (e.g., clotrimazole) used for specic infections
Capsaicin: an alkaloid derivative used to relieve pain and
itching; the precise mechanism of action is unknown, but
evidence suggests that capsaicin depresses the synthesis,
storage, and release of substance P (a mediator for pain and
itch impulses to the central nervous system); a study by
Lysy etal demonstrated relief of perianal itching in 31 of 44
subjectsusing0.006%capsaicincomparedwithonesubject
in a placebo group
2. Consultation with dermatology
• Ifthepatientisnotrespondingtorst-linetherapy,anunder-
lying dermatologic condition should be excluded; Dasan etal
found that 34 of 41 patients studied in a combined colorectal
and dermatological clinic had a dermatosis, which supports
referral to a dermatologist in the rst instance
3. Surgical therapy
• Correctionofanyrelatedanorectalconditions(seeTable 9-1)
• Subcutaneousinjectionofatopicalanestheticandmethylene
blue; the exact mechanism of action is unknown, although it is
believed it may be toxic to nerves supplying the perianal skin;
Sutherlandetalreporteda96%improvementand57%resolution in 49 patients with an 8-week follow-up (complications
included transitory incontinence and a decrease in perianal
sensation), whereas Salamavicius reported a 20% success rate
with a long-term follow-up of 47 months; previous studies
have reported severe complications, including full-thickness
skin necrosis and cellulitis (some investigators believe these
complications were due to the amount and concentration
of methylene blue used); we have no experience in use of
this technique and thus cannot make any recommendations
regarding its use
4. Alternatives under research
• Topicaltacrolimus:Ucaketalperformedarandomized
controlledtrialthatincluded16patientswithpruritusaniand
atopic dermatitis who were treated with 0.03% tacrolimus and
placebo; the investigators concluded that topical tacrolimus
was well tolerated and eective in controlling pruritus ani in
this setting
ANAL AND PERIANAL REGION 47
History and physical
(Box 9-2)
Anoscopy, rigid proctoscopy
SECONDARY CAUSE IDENTIFIED
NO
General measures (Box 9-4)
Consider dermatology consult
RESOLUTION OF SYMPTOMS
NO YES
Dermatology consult
Consider options for refractory or persistent
pruritus ani (Box 9-5)
Case-by-case basis examinations as
needed (Box 9-3)
YES
General measures (Box 9-4)
and treat secondary cause
Follow-up
Continue treatment (chronic condition)
FIGURE 9-1 Recommended
algorithm for pruritus ani.
in patients who do not respond to rst-line therapy. A dermatology
consultation should always be considered, particularly when treatment is not eective.
S u g g e S t e d R e a d i n g
Allan A, Ambrose NS, Silverman S,Keighley MRB. Physiological study of
pruritus ani. Br J Surg.1987;74:576–579.
Caplan RM. e irritant role of feces in the genesis of perianal itch. Gastroen-
terology.1966;50:19–23.
Dasan S, Neill SM, Donaldson R, Scott HJ. Treatment of persistent pru-
ritus ani in a combined colorectal and dermatological clinic. Br J Surg.
1999;86:1337–1340.
Eyres AA, ompson JPS. Pruritus ani: is anal sphincter dysfunction impor-
tant in etiology? BMJ.1979;2:1549–1551.
Farouk R, Duthie GS, Pryde A, Bartolo DCC. Abnormal transient internal
sphincter relaxation in idiopathic pruritus ani: physiological evidence
from ambulatory monitoring. Br J Surg.1994;81:603–606.
Farouk R, Lee PWR. Intradermal methylene blue injection for the treatment
of intractable idiopathic pruritus ani. Br J Surg.1997;84:670.
Friend WG. e causes and treatment of idiopathic pruritus ani. Dis Colon
Rectum.1977;20:40–42.
MarkellK, Billingham R. Pruritus ani: etiology and management. Surg Clin N
Am.2010;90:125–135.
Nasseri Y, Osborne M. Pruritus ani: diagnosis and treatment. Gastroenterol
Clin North Am.2013;42:801–813.
Pirone E, Infantino A, Masin A, etal. Can proctologic procedures resolve peri-
anal pruritus and mycosis? Int J Colorect Dis.1992;7:18–20.
Salamavicius NE, Poskus T, Gupta RK, Lunevicius R. Long-term results of
single intradermal 1% methylene blue injection of intractable idiopathic
pruritus ani: a prospective study. Tech Coloproctol.2012;16:295–299.
Siddiqi S, Vijay V, Ward M, et al. Pruritus ani. Ann R Coll Surg Engl.
2008;90:457–463.
Smith LE, Henrichs D, McCullah RD. Prospective studies in the etiology and
treatment of pruritus ani. Dis Colon Rectum.1982;25:358–363.
Sutherland AD, Faragher IG, Frizelle FA. Intradermal injection of meth-
ylene blue for the treatment of refractory pruritus ani. Colorect Dis.
2009;11:282–287.
Ucak H, Demir B, Cicek D, etal. Ecacy of topical tacrolimus for the treat-
ment of persistent pruritus ani in patients with atopic dermatitis. J Derma-
tolog Treat.2013;24(6):454–457.
C M
A S
Theodore E. Eisenstat and Jason Penzer
INTRODUCTION
Anal stenosis (also referred to as anal stricture) is an abnormal narrowing of the anus that oen occurs aer colon and rectal surgery.
e diagnosis of anal stenosis is suggested by a history of constipation
and diculty in passing stool. Patients oen have a history of anal
surgery, inammatory bowel disease, radiation treatment, or anorectal infection. e diagnosis is conrmed by physical examination.
DEFINITION
e anal canal may be dened as the part of the alimentary tract from
the anal verge to the anorectal ring (at the level of the levator muscle).
Scarring and contracture may occur in a narrow bandlike fashion
at the anal verge or occasionally may involve the entire anal canal
with a thick, unyielding contracture. Severe stenosis may be dened
by the inability to pass an 11-mm scope or the index nger into the
anal canal. Stenosis of this degree is usually symptomatic and oen
requires operative treatment.
Sometimes the stenosis is due to an abnormally high tone in the
internal sphincter. is condition is sometimes referred to as anismus
(internal sphincter spasm). Anal manometry may be of value in documenting the spasm; a “saw tooth” pressure pattern is common. If the
stenosis is due to scarring or a tumor, manometry is not very helpful. However, measurement of rectal compliance, anorectal sensation,
and the integrity of the rectoanal inhibitory reex will complete the
picture of the patient’s adaptation to his or her condition.
CLASSIFICATION OF ANAL STENOSIS
Cause
Anal stenosis may be broadly classied by its cause (Box 10-1).
Spasm
Anal spasm (anismus) causes a tight anal canal that is painful and
impossible to examine in the oce. One of the common causes of
internal sphincter spasm is an anal ssure. However, the spasm of
a ssure is not a true stenosis. Painful anal spasm is an indication
for examination with use of an anesthetic. Narrowing associated
with spasm will disappear as the anesthetic relaxes the sphincters,
whereas a stricture due to scarring will persist. Stenosis associated
with internal sphincter spasm and a painful anal ssure is generally
treated with a lateral internal sphincterotomy, which is discussed in
Chapter 3.
Postoperative Scarring
Postoperative scarring is the most common cause of an anal stenosis.
e anal canal is a small oval tube, and incisions made in the mucosa
heal with contracture, producing a circular scar that inevitably tightens. is outcome is characteristic of all circular wounds, such as anastomoses. Ileal pouch–anal anastomoses can be hand sewn or stapled.
Stapled anastomoses are prone to weblike strictures because of the
diverting ileostomy, but these strictures are easily dilated at the time
of stoma closure. Hand-sewn anastomoses can form denser strictures
that may be symptomatic, oen requiring repeated dilations. Anal
stenosis is also common aer a hand-sewn coloanal anastomosis for
rectal cancer, especially when neoadjuvant radiation has been administered. e radiation makes stenosis relatively resistant to dilation,
and thus frequent stretching with use of an anesthetic may be needed.
Anal mucosal stripping aer stapled ileal pouch–anal anastomosis for retained anal transition zone is a potent cause of a dense anal
stenosis. To avoid this consequence, consider stripping half the circumference at one operation and the other half later, aer the rst
half has healed.
Postoperative strictures at the anal verge or in the external anal
canal are usually due to excision of excessive amounts of anoderm
during anorectal operations—especially hemorrhoidectomy—or
overambitious electrocoagulation/excision of anal warts.
Stenosis Due to Chronic Diarrhea
e anal canal relies on passage of a formed stool for maintenance
of its suppleness and dilatability. In persons with chronic diarrhea,
the anus never naturally stretches, and over time it tends to lose the
ability to dilate. is tendency is worse in patients with anal inammation, such as that found in association with inammatory bowel
disease (Crohn-related colitis and ulcerative colitis). e presence of
active inammatory bowel disease, especially with rectal involvement
or suppuration, may also prevent surgical correction of a stricture.
Occasionally, fecal diversion will be required in this group of patients.
Gentle dilation aer induction of anesthesia with a lubricated dilator
has been reported to have a good eect in some patients. is success is thought to be related to the absence of new wounds in these
patients with inammatory bowel disease and carries the caveat of
using minimal dilation that is just adequate to allow the passage of
semiliquid stool without disrupting the sphincters.
Age-Related Stenosis
Anal stenosis may develop in elderly and senile patients or patients
with Alzheimer disease who chronically abuse laxatives. Care must
be taken to avoid surgery in these patients whose continence may,
in part, depend upon the presence of anal stenosis. Both the anal
48
ANAL AND PERIANAL REGION 49
BOX 10-1: Common Causes of Anal Stenosis
• Sphincterspasm(analssure)
• Postoperativescarring
• Anastomoticstenosis
• Inammatoryboweldisease
• Chronicsuppuration
• Chronicdiarrhea
• Radiation
• Venerealdisease
• Congenitalmalformation(imperforateanus,stenosis,or
membrane)
• Neoplasm(benignormalignantanal,perianal,orrectallesions)
• Trauma(lacerations,crush,thermalinjury,chemicalinjury)
• Infection(tuberculosis,lymphogranulomavenereum,schisto-
somiasis, syphilis, actinomycosis)
• Ischemia
stenosis and the atrophy of the sphincters are due to passage of only
liquid stools and the lack of natural dilatation by formed movements.
Use of mineral oil has been implicated in these patients. Caution is
advised in management because correction of the anal stenosis may
result in severe incontinence. Education with regard to bowel habits
and diet may alleviate symptoms.
Other causes of stenosis are listed in Box 10-1.
Other Classification Schemes for Anal Stenosis
Other classication schemes for anal stenosis have been described.
On the basis of severity, Milsom and Mazier distinguished mild,
moderate, and severe anal stenosis. Persons with mild anal stenosis
have a tight anal canal that can be traversed by a well-lubricated index
nger or a medium Hill-Ferguson retractor. In persons with moderate anal stenosis, forceful dilatation is required to insert either the
index nger or a medium Hill-Ferguson retractor. In persons with
severe anal stenosis, neither the little nger nor a small Hill-Ferguson
retractor can be inserted unless a forceful dilatation is employed. Furthermore, stenoses may be diaphragmatic (characterized by a thin
strip of constrictor tissue in the presence of inammatory bowel disease), ringlike or anular (aer development of surgical or traumatic
lesions of a length <2 cm), or tubular (with a length >2 cm). Based
on the level of anal canal aected, stenosis also may be distinguished
as low, middle, high, or diuse. Low stenosis, which occurs in 65%
of patients, is located at the distal anal canal at least 0.5 cm below the
dentate line. Middle stenosis, which occurs in 18.5% of patients, is 0.5
cm proximal to 0.5 cm distal to the dentate line. High stenosis, which
is found in 8.5% of patients, is proximal to 0.5 cm above the dentate
line. Diuse stenosis, found throughout the anal canal, aects 6.5%
of patients.
SYMPTOMS
Symptoms associated with anal stenosis include constipation, because
the stool will not easily traverse the stenotic area; incontinence,
because liquid seeps uncontrollably around the fecal bolus that lies
above the stricture; and pain from the anus itself and the overfull rectum above. Persons with chronic obstruction from the stenosis can
experience abdominal pain and bloating.
Examination Findings
Examination may reveal evidence of surgery, such as scarring, or distortion of the perianal tissues. Signs of inammatory bowel disease
also may be present, such as tags or chronic ssures.
e anal tone can be gauged through a gentle attempt at digital
examination using a large amount of lubricant and starting with the
h nger. At the same time, physical stenosis can be detected. For
many patients, the examination will need to be performed with use of
an anesthetic to avoid discomfort.
PREVENTION OF POSTOPERATIVE ANAL STENOSIS
Postoperative anal stenosis is usually preventable. Good surgical
technique preserves anoderm during excisional procedures, such
as a hemorrhoidectomy. When performing the classic closed Ferguson hemorrhoidectomy, three (or at most, four) hemorrhoids
may be removed. During the procedure, adequate bridges of anoderm between excisions must be preserved. If, at the completion of
the procedure, the largest Hill-Ferguson (3.5 cm) anoscope can be
introduced into the anal canal, development of postoperative strictures will be unlikely. Severe postoperative stenosis has occurred
aer the “Whitehead” hemorrhoidectomy has been performed,
although when it is performed correctly, this procedure should
not result in anal stenosis or ectropion. Rather than risk a postoperative stenosis by sacricing too much anoderm, it is preferable to leave behind some hemorrhoids; later banding of residual
internal hemorrhoids and excision of skin tags is far easier than
correction of a stenosis from overaggressive excision. e surgeon
should be especially conservative when operating in the presence
of acute hemorrhoidal disease. Postoperatively, ber or a bulking
agent should be prescribed to ensure daily bowel movements. e
dilating eect of a well-formed stool daily is key in preventing postoperative stenosis.
TREATMENT
Nonoperative Management
Treatment of anal stenosis depends on its severity and symptoms.
Asymptomatic anal stenosis requires no treatment, unless access
to the rectum is needed for endoscopy or for a procedure. Patients
with chronic diarrhea may have a stenosis but no symptoms. In
such cases, use of a bulk-forming agent may precipitate symptoms,
and it is better just to let the diarrhea continue. Patients with mild
to moderate symptoms may require only dietary adjustment and
counseling. Dietary manipulation (generally with reduced ber
intake) and the use of stool soeners will provide symptomatic
relief. Severe stenosis and symptoms usually require surgical
correction.
Stenosis that follows anorectal surgery initially should be managed conservatively. A waiting period of 3 to 6 months is appropriate
to allow maturation of the scar and adaptation of the mechanism of
defecation. Symptoms sometimes resolve with conservative management and regular oce dilation. Anal dilators may be used, but use of
an index nger is preferable.
Anal Dilation
Anal dilation with use of an anesthetic allows assessment of the
diameter, length, intensity, and nature of the stenosis. Dilation to
permit insertion of a closed anoscope or a Hill-Ferguson retractor
can be performed with a well-lubricated finger or a set of graduated anal dilators. Scar tissue can be broken during the dilation.
A weblike stricture can be incised in four quadrants using diathermy, which will be a permanent cure. A lengthier stricture is
dilated and the scarring is injected with steroids (triamcinolone
[Kenalog], 40 mg [1 mL] in 5 mL of saline solution). The steroid
reduces the tendency for the stricture to re-form. In some patients
Cause and ManageMent of anal stenosis50
FIGURE 10-1 Simple anotomy. (From Oliver GC, Rubin RJ. Anoplasty. In:
Fielding LP, Goldberg SM, eds. Rob & Smith’s Operative Surgery: Surgery of
the Colon, Rectum and Anus. 5th ed. London: Butterworth-Heinemann; 1993.)
a series of elective dilations can be advised because the scar tissue
will stabilize over time.
Surgical Management
Surgery is indicated for symptomatic patients who are not helped by
conservative treatment. A careful sphincterotomy or a Botox injection
are options for stenosis caused by spasm of the internal sphincter. e
sphincterotomy is performed with a closed technique, incising for
the length of the sphincter on either side. e blade is drawn across
the medial surface of the muscle, and a few bers are divided. It is better to be more conservative, even though the patient may have to return
for more sphincter division, than aggressive and risk incontinence.
For a moderate stenosis due to an actual stricture, simple release of
the stricture with a lateral incision (anotomy) may suce (Fig. 10-1).
is incision is generally performed in an open fashion, with the
wound le open to heal by secondary intention. Granulation of this
wound may result in a more adequate orice in contrast to primary
closure. Occasionally, a repeat stricture will occur with healing, and
multiple anotomies are sometimes necessary (Fig. 10-2).
For severe anal strictures, simple release of the stricture or scar tissue is usually inadequate because as healing occurs, the contracture
re-forms. erefore, transposition of healthy, viable epithelium into
the defect that results aer release of the stricture is required. is
transposition can be accomplished either by advancing the mucosa
outward or by moving skin into the defect. Mucosal ectropion and
scar tissue should be excised during release of the stricture. Because
advancement of the mucosa beyond the dentate line will create an
ectropion, it is usually preferable to bring skin into the anal canal.
FIGURE 10-2 Multiple anotomies. (From Oliver GC, Rubin RJ. Anoplasty.
In: Fielding LP, Goldberg SM, eds. Rob & Smith’s Operative Surgery: Surgery of
the Colon, Rectum and Anus. 5th ed. London: Butterworth-Heinemann; 1993.)
Anoplasty
When loss of anoderm has been signicant and the stricture is severe,
anoplasty is required. One of several ap-type procedures may be
used, depending on the severity of the stricture and the resultant
deformity aer excision of the scar tissue. In increasing order of mag-
nitude,these proceduresarea“Y-V”advancementap,an“island”
advancement ap, and an “S” plasty. Advancement aps provide
viable tissue to ll the defect that remains aer stricture excision and
prevent recurrent contracture. All but the most extensive of these
procedures can be performed using intravenous sedation and a local
anesthetic, although induction of general anesthesia provides a comfortable experience for the patient and avoids the distortion caused
by a local anesthetic; exposure is best with the patient in the prone
position.
e operation begins by excising scar tissue and restoring the
length and diameter of the anus, which will result in a “bare” area
that used to be contracted within the stricture but now needs to be
covered by epithelium. is coverage is generally achieved with a ap.
Historically, a “Y-V”–type advancement ap has been used to
replace anoderm. An anotomy is performed and continued into a
Y-shaped incision to create a ap, which may be advanced into the
anal canal. is procedure can be performed in any of the four anal
quadrants. Although we most frequently use a single lateral site, the
procedure may be performed bilaterally. Limitations relate to the distance this ap can be moved and the resultant tension on the ap.
Occasionally, necrosis of the tip may occur but will not result in restenosis, as long as the width of the interposed ap remains viable.
“Island” aps are recommended for larger defects that require
further advancement of viable tissue. “Island” aps should be constructed in such a manner that they include the attached subcutaneous tissue and an adequate blood supply. Closure of the skin defect
behind these aps aids in securing the ap within the anal canal and
relieving tension; this maneuver also tends to push the ap into the