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Complications of Anorectal Malformations with Dr. Marc Levitt
With Dr. Marc Levitt · hosted by Dr. Todd Ponsky
Chapter 1 of 14 · Fundamentals
Newborn diagnosis
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 have the malformation go unnoticed, typically presenting in the first year of life with severe constipation.
In perineal fistula, the hole is too small and anterior to the center of the sphincters, causing the rectum and sigmoid to dilate as stool passes through a tiny fistulous orifice.
The standard practice of checking temperature on the forehead or ear rather than rectally means the anus may not be examined, potentially missing malformations.
Relocating a perineal fistula into the sphincters does not completely fix constipation, though it improves anatomy by making the hole adequately sized and lined by mucosa.
Patients with uncorrected perineal fistula may have some continence with formed stool but will soil with loose stool or athletic activity because sphincter contraction cannot completely close the anteriorly located hole.
A newborn anus should accept a size 12 Hagar dilator and a one-year-old should accept size 15.
A bucket handle skin tag is consistent with perineal fistula; a probe can be passed underneath it even when the fistula itself is not visible.
Diagnosing perineal fistula in females is probably the most confounding thing in pediatric colorectal surgery, with many patients either missed or overdiagnosed.
Criteria for perineal fistula in females: hole too close to vagina (inadequate perineal body), inadequate hole size by Hagar dilators, and hole not centered in sphincter.
If the hole is adequate size and centered in the sphincter, even if appearing slightly anterior with short perineal body, the patient does not need surgery; the perineal body will lengthen with growth.
Examination under anesthesia with stimulation can confirm whether a questionable hole is properly centered within the sphincter.
An anesthesia nerve stimulator costing $150 with appropriate needle probes works as well as commercial $15,000 sphincter stimulators.
In rectourethral fistula, there is no anal opening and no hope for a hole; some babies pee meconium, making the diagnosis obvious.
Rectourethral fistulas should not be approached primarily because the rectum location (bladder neck, prostatic, or bulbar level) is unknown; attempting posterior sagittal incision may find urinary tract structures instead of rectum.
Rectourethral fistula patients should be managed with colostomy and distal colostogram, except in exceedingly rare cases where cross-table lateral at 20 hours shows very low rectum allowing safe primary posterior sagittal approach.
Cloaca can be missed in the newborn period; a recent case presented at 6 months with constipation when someone finally noticed no anus.
Ambiguous genitalia (clitoromegaly from endocrine stimulation) with urogenital sinus has a completely normal anus, distinguishing it from cloaca which has no anus.
Cloaca patients have no endocrine problem and two completely normal ovaries, unlike urogenital sinus with virilization.
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.
Transverse colostomies can prolapse and, with large rectourethral fistula, the left colon absorbs urine causing acidosis; they also make distal colostogram difficult.
Preferred technique is very proximal sigmoid colostomy leaving entire sigmoid for pull-through, with tiny flat mucous fistula separated from proximal stoma.
Prolapse is related to colon mobility: mid-transverse colostomy both sides can prolapse, hepatic flexure only distal prolapses, proximal sigmoid only distal can prolapse because left colon is fixed to retroperitoneum.
Ileostomies prolapse frequently because they are free-floating unless tacked to anterior abdominal wall.
The anoplasty site should be marked before making the incision by drawing a circle around the pinkish ellipse where it stimulates on the skin surface, preventing confusion when anatomy is disrupted.
Without pre-marking, surgeons can choose the wrong place when seeing muscles jumping with stimulator after opening posterior sagittal incision, requiring re-operation despite good muscle potential.
The distal colostogram is an absolutely vital study; many mistakes result from poorly done studies and misinterpretation.
The colostogram must answer: where is the rectum, how low is it, is it reachable posterior sagittally or better laparoscopically, and what is its relationship to the urinary tract.
Common colostogram error is insufficient contrast and pressure, giving false impression of high rectum and no fistula.
If the distal rectum shows a straight line corresponding to the pubococcygeal line, the radiologist did not give enough contrast or pressure; more pressure will show bulging rectum and fistula.
Fistula classification using urethra as reverse C or elbow: fistula at or below elbow is bulbar, above elbow is prostatic, at bladder neck is bladder neck fistula.
Bulbous rectum may be reachable posterior sagittally and hard laparoscopically due to girth; tapered rectum is better approached laparoscopically.
Opening posterior sagittally without knowing rectum location will find a whitish shiny structure that may be bladder neck, not rectum.
Adjunct techniques to locate rectum include balloon catheter in mucous fistula inflated with fluid or gastroscope to look for light, though the speaker has not used these.
Never go to the operating room without knowing exactly what anatomy to expect from a proper distal colostogram; the key question is where is the rectum and is it the most posterior structure.
Bulbar or low prostatic rectum with bulge is more easily approached posterior sagittally; high prostatic tapered rectum is best served by laparoscopy; bladder neck fistulas are certainly best by laparoscopy.
Laparoscopy for bulging rectum below peritoneal reflection at low prostatic or bulbar level requires unnecessary extra work and risks leaving remnant of original fistula (roof) if surgeon is timid.
Posterior sagittal approach for high rectum is very difficult to mobilize and risks urinary tract injury.
Laparoscopy replaces laparotomy as elegant dissection from above but should not give away advantages of PSARP; a mini-PSARP during laparoscopy allows safe pelvic entry and rectal tacking to prevent prolapse.
Preferred terminology is laparoscopic-assisted PSARP rather than laparoscopy versus PSARP.
Prolapse prevention: proper levator closure, tacking rectum to posterior edge of muscle complex for 3-4 stitches, not dissecting rectum more than necessary, avoiding excessive trimming.
Prolapse occurs in about 3% of cases, particularly in those without great muscles.
Rectal prolapse causes bleeding, mucus, and can inhibit bowel control in patients with good muscle potential because they cannot close the opening with prolapsed tissue through it.
Prolapse more than 3 millimeters should be treated; ideal time is when colostomy is still present.
For circumferential prolapse, performing half the circumference in two different ambulatory settings is preferred by families over hospitalization and avoids need for dilation since half is untouched.
Perineal body dehiscence prevention requires complete anterior rectal wall separation from posterior vaginal wall to reach areolar plane, avoiding tension on anoplasty.
Perineal body dehiscence is the most common cause of reoperation in female ARM repairs.
Traditional postoperative management is NPO for 7 days on 10% dextrose (hyperalimentation only if longer than 7 days); recently trialing clear liquids only for a week to avoid hard stool while allowing some oral intake.
Daily perineal examination during the first week is critical; if dehiscence is detected on day 5-8, taking the patient back to OR to re-suture can salvage the repair, but by 3-4 weeks later nothing can be done.
Laparoscopy causes trouble if dissecting a rectum that is too low, getting too close to urinary tract or being too timid and leaving remnant of original fistula (distal rectum).
For high rectums, particularly bladder neck fistulas, dissection to make the rectum reach with good blood supply is challenging; the IMA must be preserved because prior colostomy may have disrupted left colic collaterals.
The rectum has excellent intramural blood supply from the IMA; taking tiny distal vessels along the rectal wall preserves this, but taking IMA or branches too close to aorta will cause rectal necrosis.
The biggest PSARP problem is exploring without knowing rectum location and finding bladder neck, urethra, seminal vesicles, vas deferens, or ectopic ureter instead of distal rectum.
Famous cases exist of bladder neck being pulled through and made into beautiful anoplasties, with the patient postoperatively draining liquid (urine) from the anoplasty.
To determine continence potential, assess three factors: original malformation type, sacral quality and calculated sacral ratio, and spine quality (ARM continence index).
Three A's in continence index (malformation type, sacrum, spine) predicts continence; three C's predicts incontinence; intermediate grades are being quantified through data collection.
Bulbar fistula with sacral ratio of 1 and normal spine should absolutely have bowel control; bladder neck fistula with sacral ratio 0.4 and tethered cord or myelomeningocele has no chance of good bowel control.
For 4-year-old with soiling and continence potential, first step is mechanical cleaning with bowel management enemas to gain confidence, then when older try switching to laxatives for voluntary bowel movements.
If patient cannot be weaned from enemas, consider antegrade option like Malone procedure.