Perianal Poop-pourri: Disorders of the Anorectum Elizabeth Schaefer, M.D. easchaef@stvincent.org St. Vincent Pediatric Gastroenterology 8402 Harcourt Rd. Suite #402 Indianapolis, IN 46260 (317) 338-9450

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

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 0.54 +/- 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 http://health-7.com/ 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.

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. 17-143). 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. 17-144). The prognosis is generally favorable for low lesions because they lie within the levator ani complex.

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

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

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

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.

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)

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

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; 340:1398-405.

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

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

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. 17-151). 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.

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

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

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

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

More is not necessarily better

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; 2008. 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;22:231-243 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” 368-369. 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; 801-807. Walker W, et al, eds. Pediatric Gastrointestinal Disease. 4th ed. Hamilton, Ontario: BC Decker, 2004: Chapter 35