Marc Levitt · Anorectal Malformations Complications
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Podcast48 min·Published Feb 2016Older

Anorectal Malformations Complications

With Dr. Mark Levitt · hosted by Dr. Todd Ponsky · Marc Levitt
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What the experts said54 expert statements
Perineal fistulas in males are commonly missed in the newborn period because the baby passes meconium through the small fistulous opening, and no one notices the abnormal anal anatomy. These patients typically present in the first year of life with severe constipation.
ClinicalMarc Levitt
By the time a missed perineal fistula is diagnosed, the rectum and sigmoid have dilated because stool has been passing through a very tiny fistulous orifice which is not normal anal or rectal mucosa.
ClinicalMarc Levitt
The current standard is to check temperature on the forehead or in the ear rather than rectally, so if you don't look at the anus, you might not know there is a malformation.
ClinicalMarc Levitt
Relocating a perineal fistula into the sphincters does not completely fix the constipation, though it improves the anatomy by making the hole adequately sized and lined by mucosa.
ClinicalMarc Levitt
Patients with uncorrected perineal fistulas can have some semblance of continence with formed stool, but with loose stool or athletic activity they will soil because they cannot completely close the anteriorly located hole when squeezing their sphincters.
ClinicalMarc Levitt
A newborn anus should accept a size 12 Hagar dilator, and a one-year-old should accept a size 15.
ClinicalMarc Levitt
A bucket handle (a lifted skin tag that you can pass a probe underneath) is consistent with a perineal fistula even if you cannot see the fistula itself.
ClinicalMarc Levitt
Perineal fistula in females is probably the most confounding diagnosis in pediatric colorectal surgery, with many patients either being missed or overdiagnosed.
OpinionMarc Levitt
Diagnostic criteria for perineal fistula in females: inadequate perineal body (hole too close to vagina), inadequate hole size, and hole not centered in the sphincter.
ClinicalMarc Levitt
If the anal opening in a female is adequate size and centered in the sphincter, even if it appears slightly anterior with a short perineal body, that patient does not need surgery. The perineal body will lengthen with growth.
ClinicalMarc Levitt
An anesthesia nerve stimulator ($150) with appropriate needle probes works as well as a dedicated Pena stimulator ($15,000) for intraoperative sphincter mapping.
ClinicalMarc Levitt
The vast majority of male ARM patients have a rectourethral fistula. It is important not to approach these primarily because you don't know where the rectum is—it could be at bladder neck, prostatic, or bulbar level.
ClinicalMarc Levitt
If you open posterior sagittal looking for a rectourethral fistula without knowing the location, you will find something midline, white, and shiny that might be the urinary tract, not the rectum.
ClinicalMarc Levitt
Cloacas can be missed in the newborn period. Dr. Levitt saw a six-month-old who presented with constipation and was found to have an undiagnosed cloaca with no hint of an anal opening.
ClinicalMarc Levitt
Ambiguous genitalia (clitoromegaly from endocrine stimulation) presents with a urogenital sinus but a completely normal anus. This is different from a cloaca, which has no anus and no endocrine problem.
ClinicalMarc Levitt
The most common colostomy error is opening too distal in the sigmoid, which restricts the ultimate pull-through by the location of the colostomy or mucous fistula.
ClinicalMarc Levitt
Incompletely diverting loop colostomies allow stool to spill across and contaminate the distal segment, leading to urinary tract infections.
ClinicalMarc Levitt
Transverse colostomies are problematic because they can prolapse, and if there is a large rectourethral fistula, the left colon absorbs all the urine (which doesn't come out the mucous fistula), causing acidosis.
ClinicalMarc Levitt
Dr. Levitt's preference is a very proximal sigmoid colostomy with separated stomas, leaving the entire sigmoid loop for the pull-through. He makes the mucous fistula very tiny and flat.
ClinicalMarc Levitt
Prolapse is related to where in the colon you choose to do the colostomy. Mid-transverse: both sides can prolapse. Hepatic flexure: only distal can prolapse. Proximal sigmoid: only distal (mucous fistula) can prolapse because left colon is fixed to retroperitoneum.
ClinicalMarc Levitt
Dr. Levitt marks the anoplasty location by drawing a circle around the pinkish ellipse where it stimulates on the skin surface BEFORE making the incision, to avoid getting lost when looking at jumping muscles from the stimulator.
ClinicalMarc Levitt
Really good surgeons have put anuses in crazy places because they don't have a sense of what's the center once everything is disrupted and open.
ClinicalMarc Levitt
The distal colostogram is an absolutely vital study. Many mistakes are made because of a poorly done study and misinterpretation.
ClinicalMarc Levitt
The basic questions the distal colostogram must answer: Where is the rectum? How low is it? Is it reachable posterior sagittally or better approached laparoscopically? What is its relationship to the urinary tract?
ClinicalMarc Levitt
The common colostogram mistake is not giving enough contrast and pressure into the distal segment, giving a false impression that the rectum is high or that there is no fistula.
ClinicalMarc Levitt
If you see a straight line flattening of the rectum corresponding to the pubococcygeal line, the radiologist did not give enough contrast or pressure. You need to overcome the PC line (the sphincters compressing the distal rectum) to see the bulging rectum and fistula.
ClinicalMarc Levitt
Fistula classification: if the fistula is at the urethral 'elbow' or below, it's bulbar. Above the elbow is prostatic. At the bladder neck is bladder neck fistula.
ClinicalMarc Levitt
If the rectum is bulbous, it might be reachable posterior sagittally and hard to do laparoscopically because of the girth. If it's tapered, you're better off laparoscopically.
ClinicalMarc Levitt
If you don't know where the rectum is and open posterior sagittal, you will find a whitish, shiny structure and may think it's the rectum. Often it's the bladder neck.
ClinicalMarc Levitt
You avoid bladder neck injury by knowing exactly where the rectum is from a properly done distal colostogram. When you open posterior sagittal, you know the rectum is right under the coccyx (prostatic) or distal to the coccyx (bulbar), or it isn't posterior sagittal at all (bladder neck—do laparoscopy).
ClinicalMarc Levitt
Dr. Levitt will do posterior sagittal for bulbar fistulas and low prostatic fistulas with a bulge. High prostatic with tapered rectum and bladder neck fistulas are best served by laparoscopy.
ClinicalMarc Levitt
If you try laparoscopy for a rectum bulging below the peritoneal reflection at low prostatic or bulbar level, you may leave behind a remnant of the original fistula (ROOF)—the distal rectum left behind that causes trouble later.
ClinicalMarc Levitt
Laparoscopy replaces laparotomy, not PSARP. Dr. Levitt does a mini-PSARP when doing laparoscopy to safely enter the pelvis and tack the rectum to the posterior edge of the muscle complex to avoid prolapse.
ClinicalMarc Levitt
Dr. Levitt calls his approach 'laparoscopic-assisted PSARP' rather than pure laparoscopy.
ClinicalMarc Levitt
Prolapse prevention: put the rectum in the right location, close the levators properly, close the posterior wall to the posterior edge of the muscle complex for 3-4 stitches, don't dissect the rectum more than necessary.
ClinicalMarc Levitt
Prolapse occurs in about 3% of cases, particularly in those without great muscles.
EpidemiologicalMarc Levitt
Rectal prolapse causes bleeding, mucus, and for patients with good continence potential, it inhibits bowel control because they can't close the opening with prolapsed tissue through it.
ClinicalMarc Levitt
Dr. Levitt trims prolapse of more than about 3mm. For circumferential prolapse, he does half the circumference in two different ambulatory settings so families don't need hospitalization and the patient doesn't need dilation (half the circumference is untouched so they won't stricture).
ClinicalMarc Levitt
Perineal body dehiscence is the most common cause of reoperation Dr. Levitt performs in female ARM repairs.
ClinicalMarc Levitt
The key to preventing perineal body dehiscence is mobilizing the rectum well—you must get the anterior rectal wall completely separated from the posterior vaginal wall to the areolar plane. If you don't, the anoplasty will be under tension and can pull back, leak into the perineal body space, and dehisce.
ClinicalMarc Levitt
Dr. Levitt uses 3-0 suture for perineal body closure in a baby and 4-0 Vicryl on the perineal skin, then watches the perineum very closely.
ClinicalMarc Levitt
Traditionally Dr. Levitt kept patients NPO for 7 days on 10% dextrose after female ARM repair. Recently he has been trialing clear liquids only for a week because the major problem is hard stool—clear liquids won't make hard stool.
ClinicalMarc Levitt
If perineal body dehiscence is detected on days 5-8, Dr. Levitt will take the patient back to the OR and re-suture the perineal body, which can salvage the situation. This happens in maybe 1-2 cases out of about 200.
ClinicalMarc Levitt
Laparoscopy causes trouble if you try to dissect a rectum that's too low—you get too close to the urinary tract or you're too timid and leave behind the distal rectum (remnant of original fistula).
ClinicalMarc Levitt
For high rectums, particularly bladder neck fistulas, the dissection of the distal rectum is quite challenging to make it reach with good blood supply. You must preserve the IMA because the colostomy may have disrupted collaterals down the left colic, making the rectum completely dependent on the IMA.
ClinicalMarc Levitt
The rectum has an excellent intramural blood supply from the IMA. If you take the IMA or take branches too close to the aorta, the rectum will die because there's no collateralization down the left colic.
ClinicalMarc Levitt
The biggest problem with posterior sagittal incision is going after a rectum when you don't know where it is. You open and find the bladder neck, urethra, seminal vesicles, vas deferens, ectopic ureter—everything but the distal rectum.
ClinicalMarc Levitt
There are famous cases of pull-through of bladder neck made into beautiful anoplasties, and post-op the patient was draining liquid out their anoplasty—it was the bladder neck.
ClinicalMarc Levitt
To determine if an ARM patient has potential for bowel control, Dr. Levitt looks at three factors: original type of malformation, quality of sacrum and calculated sacral ratio, and quality of spine. He calls this the ARM continence index.
ClinicalMarc Levitt
Three A's (excellent malformation type, sacrum, and spine) predicts a continent patient. Three C's predicts an incontinent patient. Dr. Levitt's group is working on quantifying the in-between grades.
ClinicalMarc Levitt
A bulbar fistula with a good sacrum (sacral ratio of 1) and normal spine should absolutely have bowel control. A bladder neck fistula with poor sacrum (sacral ratio 0.4) and tethered cord or myelomeningocele has no real chance of good bowel control.
ClinicalMarc Levitt
For a soiling 4-year-old ARM patient, Dr. Levitt's first step is to get them clean mechanically with bowel management using enemas. For those with continence potential, when they're older and more mature, he tries to switch them to laxatives to achieve voluntary bowel movements.
ClinicalMarc Levitt
Indications for redo pull-through: any patient with potential for bowel control whose anatomy is not perfect—improperly located anus, anal stricture, rectal prolapse, or remnant of original fistula (posterior urethral diverticulum).
ClinicalMarc Levitt
One of the biggest problems with ARM is that if you don't get it right, you don't know for a few years. Most surgical problems become obvious immediately, but with ARM, patients may not present with soiling until age 4, making it hard for surgeons to learn what to fix about their technique.
OpinionMarc Levitt