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Colorectal Channel
Complications of Anorectal Malformations with Dr. Marc Levitt
With Dr. Marc Levitt · hosted by Dr. Todd Ponsky
Chapter 1 of 12 · Complications
Missed diagnoses
Introduction and Missed Diagnoses in the Newborn Period
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Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
Anorectal malformations are commonly not properly diagnosed or completely missed in the newborn period.
Male babies with perineal fistula may pass meconium and no one notices anything wrong with their anorectal anatomy, typically presenting in the first year of life with severe constipation.
The standard practice of checking temperature on forehead or ear rather than rectally makes it easier to miss anorectal malformations in newborns.
Relocating a perineal fistula into the sphincters improves anatomy but does not completely fix constipation; patients will inherently have some constipation requiring aggressive treatment.
If a perineal fistula is not centered in the sphincter, patients with loose stool will soil, and athletic activity will cause soiling because sphincter squeeze cannot completely close the hole.
A newborn anus should accept a size 12 Hagar dilator and a 1-year-old should accept a size 15.
In females, diagnostic criteria for perineal fistula are: hole too close to vagina (inadequate perineal body), inadequate hole size by Hagar dilators, and hole not centered in sphincter.
If a female's anal opening is adequate size and centered in the sphincter, even if appearing slightly anterior with short perineal body, no surgery is needed; the perineal body will lengthen with growth.
An anesthesia nerve stimulator costing $150 with appropriate needle probes works as well as commercial $15,000 stimulators for identifying sphincter muscles.
The most common colostomy error is opening too distal in the sigmoid, restricting the ultimate pull-through by the location of the colostomy or mucous fistula.
Incompletely diverting loop colostomies allow stool to spill across and contaminate the distal segment, leading to urinary tract infections.
With transverse colostomy and large rectourethral fistula, the left colon absorbs urine which doesn't exit the mucous fistula, potentially causing acidosis from urine absorption.
Prolapse risk depends on colostomy location: mid-transverse allows bilateral prolapse, hepatic flexure allows only distal prolapse, proximal sigmoid allows only distal prolapse because left colon is fixed to retroperitoneum.
Marking the sphincter ellipse on skin surface before making any incision prevents confusion from muscle stimulation after dissection is open, avoiding misplaced anoplasty.
A distal colostogram showing flattening of the rectum corresponding to the pubococcygeal line indicates insufficient contrast or pressure; more pressure is needed to overcome the sphincters and reveal the true rectal position and fistula.
Fistula level is determined by viewing the urethra as a reverse C or elbow: fistula at or below the elbow is bulbar, above the elbow is prostatic, at bladder neck is bladder neck fistula.
Bulbous rectum on colostogram may be reachable posterior sagittally and difficult laparoscopically; tapered rectum is better approached laparoscopically.
Opening posterior sagittally without knowing exact rectal location risks finding and potentially mobilizing bladder neck instead of rectum.
Bulbar and low prostatic fistulas with bulbous rectum are best approached posterior sagittally; high prostatic with tapered rectum and bladder neck fistulas are best approached laparoscopically.
Attempting laparoscopy for bulging rectum below peritoneal reflection at low prostatic or bulbar level risks leaving behind a remnant of the original fistula (roof) if the surgeon is timid.
Laparoscopy replaces laparotomy, not PSARP; a mini-PSARP during laparoscopy allows safe entry through peritoneal reflection and tacking rectum to posterior muscle complex to prevent prolapse.
Rectal prolapse occurs in about 3% of cases, particularly in patients without great muscles.
Rectal prolapse more than 3 millimeters should be treated because it causes bleeding, mucus, and can inhibit bowel control in patients with good muscle potential.
Circumferential prolapse can be trimmed in two separate ambulatory sessions (half circumference each), avoiding hospitalization and eliminating need for dilation since half the circumference remains untouched.
Complete anterior rectal wall mobilization to the areolar plane between rectum and vagina is essential to avoid tension on the anoplasty that can lead to perineal body dehiscence.
Perineal body dehiscence is the most common cause of reoperation in female ARM repairs.
Clear liquids only for one week postoperatively prevents hard stool formation while allowing more stool volume, showing good perineal body healing results without traditional 7-day NPO period.
If perineal body dehiscence is recognized on days 5-8, taking the patient back to OR to re-suture can salvage the repair; by 3-4 weeks the entire perineal body is dehisced and unsalvageable.
During laparoscopic approach for high rectums, the IMA must be preserved because prior colostomy may have disrupted collaterals down the left colic, making the rectum completely dependent on IMA blood supply.
Continence potential in ARM patients is predicted by three factors: original malformation type, sacral ratio, and spine quality (ARM continence index). Three A's predicts continence, three C's predicts incontinence.
A bulbar fistula with sacral ratio of 1 and normal spine should have bowel control; a bladder neck fistula with sacral ratio of 0.4 and tethered cord or myelomeningocele has no chance of good bowel control.
Initial management of soiling 4-year-old with continence potential is bowel management with enemas to achieve cleanliness, then trial of laxatives when older and more mature to attempt voluntary bowel movements.
Indications for redo pull-through include any patient with continence potential who has improperly located anus, anal stricture, rectal prolapse, or remnant of original fistula (posterior urethral diverticulum).
The major problem with anorectal malformations is that surgical errors may not become apparent for years; an improperly placed anus appears successful initially but presents with soiling at age 4.