1. Perianal Poop-pourri: Disorders of the Anorectum
Elizabeth Schaefer, M.D.
St. Vincent Pediatric Gastroenterology
8402 Harcourt Rd. Suite #402
Indianapolis, IN
(317)

2. Objectives Review clinical presentations of classic perianal disorders
Make the diagnosis
Review the management and identify when and who to consult

4. Is this normal?
Document anal opening not in the center of the perineal pigmented area
API (Anal Position Index):
Normal: halfway between coccyx and introitus or scrotum
Female: anus-fourchette/coccyx-fourchette 0.45+/- 0.08
Male: anus-scrotum/coccyx-scrotum /- 0.07
4% of infants
Refer to surgery if severe constipation associated with API <2SD from the mean
<0.29 in girls, <0.40 in boys
Anterior Ectopic Anus
Anteriorly displaced anus partially within the anal sphincter complex
Examination of a newborn includes the inspection of the perineum. The absence of an anal orifice in the correct position leads to further evaluation. Mild forms of imperforate anus are often called anal stenosis or anterior ectopic anus. These are probably imperforate anus with a perineal fistula. The normal position of the anus on the perineum is approximately halfway (0.5 ratio) between the coccyx and the scrotum or introitus. Although symptoms, primarily constipation, have been attributed to anterior ectopic anus (ratio <0.34 in girls, <0.46 in boys), many patients have no symptoms.

5. What does this “bucket handle” bridge represent?
Rectum passes through the levator ani
Fistulous tract extends to perineal region
Prognosis favorable for low lesions because they lie within the levator ani complex
Low Imperforate Anus
A spot of meconium is visible beneath a “bucket handle” bridge of skin in an infant with a low imperforate anus.
These rectal anomalies are classified on the basis of the position of the rectum and the levator ani muscle complex. Low imperforate anus is associated with the passage of the rectum through the levator ani, and a fistulous tract extends to the perineal region ending in the center of a ridge of tissue on the anus (“bucket handle” deformity) or anterior to these structures as a perineal fistula (Fig ). In male infants the fistula may travel in the median raphe of the scrotum, and the meconium may be seen as a string of white or black beads (Fig ). The prognosis is generally favorable for low lesions because they lie within the levator ani complex.

6. Rectal Fissure
Superficial tears of anoderm, inferior to the dentate line
90% posterior
Due to constipation, although history only elicited in 25% of cases
Presentation: pain, bleeding
Diagnosis:
acute fissures are typically small
chronic fissures assoc w/ skin tag or fibrosis
Remember if fissure is large or there is bruising, consider abuse

7. Rectal Fissure Management Chronic fissures Decrease trauma
Stool softeners
Lubricant laxative
Fiber
Reduce anal sphincter tone
Warm sitz baths
Good hygiene
>80% heal
Chronic fissures
>6 weeks
Uncommon in kids
Dilation to reduce anal spasm
Nitric oxide (0.2% glycerol trinitrate)
Botulism toxin
Surgery:
lateral internal sphincterotomy

8. Perianal Strep Presentation Diagnosis: Treatment:
Well demarcated rash
6 mo – 10 yrs old
Cellulitis in 90%, pruritis in 80%
Pain, pruritis, bleeding
Familial spread possible
Diagnosis:
Group A B-hemolytic streptococcal infections found on perianal cx
Treatment:
10 days of oral penicillin
EES for PCN allergic patient
Clindamycin +/- mupirocin
40-50% recurrence rate
Contemporary Pediatrics, Publish date: Mar 1, 2011 By: Sapna Kansal Mukherjee, MD

9. Chronic Pruritis Ani Enterobius vermicularis
Presentation: anal pruritis
Dead parasites and eggs in the perianal area may also cause abscesses and granulomas
Conditions Associated with Pruritus Ani
Systemic illness
Diabetes mellitus
Hyperbilirubinemia
Leukemia
Aplastic anemia
Thyroid disease
Mechanical factors
Chronic diarrhea
Chronic constipation
Anal incontinence
Soaps, deodorants, perfumes
Over-vigorous cleansing
Hemorrhoids producing leakage
Prolapsed hemorrhoids
Alcohol-based anal wipes
Rectal prolapse
Anal papilloma
Anal fissure
Anal fistula
Tight-fitting clothes
Allergy to dyes in toilet paper
Intolerance to fabric softener
Skin sensitivity from foods
Tomatoes
Caffeinated beverages
Beer
Citrus products
Milk products
Dermatologic conditions
Psoriasis
Seborrheic dermatitis
Intertrigo
Neurodermatitis
Bowen's disease
Various squamous disorders
Atopic dermatitis
Lichen planus
Lichen sclerosis
Contact dermatitis
Infections
Erythrasma (Corynebacterium)
Intertrigo (Candida)
Herpes simplex virus
Human papillomavirus
Pinworms (Enterobius)
Scabies
Local bacterial abscess
Gonorrhea
Syphilis
Medications
Colchicine
Quinidine
Adapted with permission from Zuber TJ. Diseases of the rectum and anus. In: Taylor RB, ed. Family medicine: principles and practice. 5th ed. New York: Springer-Verlag, 1998:792.

10. Perianal Fistula
Chronic track of granulation tissue connecting two epithelial lined surfaces
Most fistulas originate below the dentate line
A fistulous abscess becomes a fistula when it ruptures
Surgical drainage
Except in known or suspected Crohn’s disease
Pack the cavity or catheter to drain
Sitz or tub baths, analgesics
Antibiotics
When fistula present, surgeon inserts a probe in external opening; the internal opening in infants is radially opposite the external opening (unlike in adults where it is often in the posterior midline (Goodsall’s rule)

11. Perianal Fistula
The internal opening in children is on the pectinate line radially opposite the external orifice
Unroof the fistula
Keep area clean with soap and water

12. Infliximab in Patients with Fistulizing Crohn’s Disease
Perianal Fistula Case Study
Pretreatment
2 Weeks
In more complex forms of abscess and fistula in Crohn’s disease, frequency of fistula seen in 17-41% of Crohn’s pts; Simple fistula is low with single external opening, no pain or fluctuation
Complex fistula is high and may have multiple external openings, assoc with perianal abscess, rectovaginal fistula, active rectal disease
Treatment: antibiotics, immunomodulators, infliximab
10 Weeks
18 weeks
Present D, et al. NEJM. 1999; 340:

13. Perirectal Abscess Majority result from a crypt of Morgagni infection
Classification determined by anatomic location of lesion relative to the levator ani and sphincteric muscles

14. Perirectal Abscesses Management Presentation Sitz baths Antibiotics
Surgical options:
If chronic fistulae beyond 3 months despite medical management
Fistulectomy
Fistulotomy
Seton loop
Consider evaluation for neutropenia, leukemia, HIV, diabetes, IBD
Presentation
Males > Females
98% report persistent perirectal pain
Abscesses identified in 95% of cases when an external perianal exam in combined with a digital rectal exam

15. Rectal Prolapse Mucosal vs full thickness Males > Females
Etiologies:
Constipation
Diarrhea
Cystic fibrosis
Other: intra-abdominal pressure, polyps, parasites, malnutrition, pelvic floor weakness
Usually self limited
If recurrent and pronounced
Sweat chloride
Screen for parasites
Rectal prolapse is an uncommon condition that is most often idiopathic in children (Fig ). The peak incidence of idiopathic rectal prolapse occurs in the second year of life, often precipitated by episodes of diarrheal illnesses, efforts to toilet train or severe constipation. This process responds spontaneously after the resolution of the acute illness or with dietary and medical manipulations to treat the constipation. Nonidiopathic cases are often related to neurologic conditions or chronic diseases. Abnormalities in the development of the muscles of the pelvic floor or the innervation occur in patients with spina bifida and related spinal cord abnormalities. Refractory cases should be evaluated for chronic hookworm infestation with stool evaluations for ova and parasites, which may cause severe tenesmus and straining. Rectal polyps may precipitate prolapse by acting as a lead point for this form of rectal intussusception. Evaluation by contrast enema and sigmoidoscopy are important components of the assessment of children with recurrent episodes. Cystic fibrosis is another common cause of prolapse and should be evaluated in patients with this condition. Surgery is rarely indicated. Circumferential submucosal injections with concentrated dextrose functions as a sclerosant that prevents prolapse from recurring.

17. Rectal Prolapse
Treatment: Manual reduction, treat primary inciting factor
If persistent: surgical – injection of sclerosant or hypertonic saline submucosally or submuscularly above dentate line
Prognosis generally good

18. Hemorrhoids Small asymptomatic: not uncommon Symptomatic:
Due to chronic straining
Anal infection spreading to hemorrhoidal veins
Underlying Crohn’s disease
Male = Female
Presentation: Bleeding, pruritis, prolapse, pain
Diagnosis: Clinical history and careful exam

19. Hemorrhoids External Hemorrhoids Internal Hemorrhoids
From ectoderm and arise distal to dentate line
Stratified squamous epithelium
Inferior rectal nerve - painful
Internal Hemorrhoids
Above the dentate line from embryonic endoderm
Simple columnar epithelium
Painless
Classified by the degree of prolapse
Pathogenesis: ?
Low fiber diets
Decreased venous return
Prolonged sitting on toilet
aging

20. Hemorrhoids: Treatment
Conservative Options
Indication: Grade I & II internal; non-thrombosed external
Sitz baths bid-tid
High-fiber diet
Fluid intake
Stool softeners
Topical/systemic analgesic
Proper anal hygiene
Short term topical steroid (hydrocortisone acetate 2.5% and pramoxine HCL1% cream)
Non-surgical Options
Indication: Recalcitrant hemorrhoids
Rubber band ligation*
Infrared coagulation*
Injection sclerotherapy
Laser therapy
Cryosurgery
Surgical Management
Nonsurgical treatment failure
Grade III & IV internal with severe symptoms
5-10% eventually require surgery
Hemorrhoidectomy
Prolonged steroid use can cause skin sensitization and rectal absorption may lead to systemic side effects

21. More is not necessarily better

22. References
Browning J, Levy M. Cellulitis and Superficial Skin Infections. In: Long SS, Pickering LK, Prober CG, ed. Principles and Practice of Pediatric Infectious Diseases. 3rd ed. Hamilton, Ontario: Churchill Livingstone; Chapter 72.
Davari HA. The anal position index: a simple method to define the normal position of the anus in neonate. Acta Paediatr. 2006;95:877
Gourgiotis S, Baratsis S. Rectal prolapse. Int J Colorectal Dis. 2007;22:
Langer M, Modi BP: Benign Perianal Lesions. In Kleinman RE, Goulet O, et al, eds. Pediatric Gastrointestinal Disease. 5th ed. Hamilton, Ontario: BC Decker Inc; 2008”
Pfefferkorn M, Fitzgerald J. Disorders of the Anorectum: fissures, fistulae, prolapse, hemorrhoids, tags. In: Wyllie R, Hyams JS, eds. Pediatric Gastrointestinal and Liver Disease, 3rd ed., 2006;
Walker W, et al, eds. Pediatric Gastrointestinal Disease. 4th ed. Hamilton, Ontario: BC Decker, 2004: Chapter 35