Anorectal Malformations with Dr. Andrea Bischoff
With Dr. Andrea Bischoff · hosted by Dr. Todd Ponsky · StayCurrentMD
Cued at 23:11 · stops at 23:56 · press play
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
Patients with anorectal malformation with good prognosis for bowel control will have well-formed buttocks with a good midline groove and a good anal dimple, while patients with bad prognosis will have a flat bottom and no clear delineation of the anal dimple.
In a newborn baby, a normal caliber anus should accommodate a number 12 Hegar dilator.
In a rectal perineal fistula, the sphincter mechanism is in a horseshoe shape with posterior and lateral portions having sphincter but the anterior portion lacking sphincter.
8% of patients with anorectal malformation will have esophageal atresia.
30% of patients with anorectal malformation will have cardiac anomalies, but in only 10% of them are these anomalies hemodynamically significant.
50% of patients with anorectal malformation have associated urological defects.
25% of patients with anorectal malformation have tethered cord.
If no fistula is identified on initial examination, the patient should be re-examined after 24 hours because it takes time for air to travel distally and for meconium to pass through a tiny fistula.
Cross-table lateral film with pelvis elevated should never be done before 24 hours of life because it will give the false impression of a high malformation due to muscle tone.
Spinal ultrasound is adequate to detect tethered cord in babies less than 3 months of age; after 3 months, MRI is needed due to ossification.
Tethered cord has more influence on the urinary tract rather than the gastrointestinal tract in terms of prognosis.
Presacral masses are most commonly found in malformations with good prognosis such as rectal perineal fistula, rectal vestibular fistula, and rectal atresia, but when present, the prognosis changes.
The advantage of primary newborn repair is that bowel preparation is not required since meconium is considered sterile.
It is better to open a colostomy and have a perfect operation than to do a primary repair and have a complication such as dehiscence retraction that requires re-operation.
Patients with anorectal malformation have one chance to have the right operation; secondary operations or re-operations usually change the prognosis for bowel control.
An 8 French feeding tube should be used to catheterize a suspected vestibular fistula if it cannot be visualized.
Vestibular fistula is the most common type of anorectal anomaly in females.
The ideal colostomy should be totally diverting, located in the descending colon to leave enough distal bowel for pull-through and avoid prolapse.
The proximal stoma should be located in the center of a triangle formed by the left rib, umbilicus, and iliac crest to ensure it is surrounded by normal skin for stoma bag application.
During colostomy creation, all distal bowel should be irrigated with plenty of normal saline to remove all meconium.
For high pressure distal colostogram, a number 8 French Foley catheter is normally used in the mucous fistula.
During distal colostogram, the patient should be turned to perfect lateral position with knees at 90 degrees and one femur exactly in front of the other, and the radiologist must show all reference points including mucous fistula, sacrum, and anal marker.
In rectal urethral bulbar fistula, the most important portion of the operation is separation between the rectum and the long common wall with the urethra, with minimal rectal mobilization needed.
In rectal urethral prostatic fistula, the common wall with urethra is shorter than bulbar fistula but more rectal dissection is needed to gain length.
In rectal bladder neck fistula, the rectum joins the urinary tract in a T fashion and the challenge is gaining enough length to reach the perineum while preserving good blood supply through selective ligation of mesenteric vessels.
Cloaca patients have never been seen with disorder of sexual differentiation; they are all females with normal ovaries.
Cloaca with common channel less than 3 centimeters can be repaired posterior sagittally with total urogenital mobilization.
Rectal perineal fistula patients with normal sacrum and no tethered cord have 100% chance of bowel control.
Malformations with better prognosis for bowel control will suffer from more constipation.
Rectal vestibular fistula patients with normal sacrum and no tethered cord have 95% chance of bowel control.
Rectal urethral bulbar fistula has 85% chance of bowel control.
Anorectal malformation without fistula has 80% chance of bowel control.
Rectal urethral prostatic fistula has 60% chance of bowel control.
Rectal bladder neck fistula has 20% chance of bowel control.
Cloaca with common channel less than 3 centimeters and normal sacrum has generally about 70% chance of bowel control.
The only indication to keep a colostomy is incapacity to form solid stool; most patients with anorectal malformation have normal colon and can form solid stool.
Patients prefer the quality of life of a pull-through with bowel management rather than with a colostomy.
All children with anorectal malformations should be out of diapers at the same age that other children are normally out of diapers, which in the United States is usually at 3 years of age.
After 3 years of age, if the child has not potty trained, formal bowel management should start: enemas for children with bad prognosis, enemas on temporary basis for borderline bowel control, and laxatives for children with bowel control and constipation.
Bowel management for fecal incontinence consists of finding the enema that completely cleans the colon and allows the child to be clean in underwear for 24 hours.
Determining which enema works for each child is a trial and error process that usually takes about one week.
Contrast enema without bowel preparation can divide fecally incontinent patients into two groups: those with dilated colon and tendency for constipation, and those with non-dilated colon and tendency for diarrhea.
For dilated hypomotile colon, a large and concentrated enema is needed to clean the colon, but after cleaning there is natural tendency not to produce bowel movements.
For non-dilated hypermotile colon, a small enema (sometimes just normal saline) is needed, but the challenge is keeping the colon from moving between enemas, usually requiring constipating diet and medication to slow the colon.
Enema base is normal saline 200 to 1000 mLs, with additives to increase concentration: liquid glycerin 10-40 mLs, Castile soap 9-27 mLs, or Fleet sodium phosphate (33 mLs for ages 2-4, 66 mLs for ages 7-10, 133 mLs for over 10 years).
The enema should be done at the same time every day and the entire process (administration, holding, evacuation) should last one hour.
Enema adjustments are made based on parent/patient report and abdominal radiograph; if real stool accidents occur and radiograph is not clean, concentration should be increased.
Enema frequency is always once daily; concentration is adjusted rather than volume or frequency.
The Malone procedure (appendicostomy) is not the treatment for fecal incontinence; the treatment is finding the enema that works. The appendicostomy provides independence.
Appendicostomy is offered when the child wants to do the enema independently or for patients with borderline bowel control who remain enema-dependent after yearly trials off enemas.
For constipation management, disimpaction is required before determining laxative dosage to avoid severe cramping.
Disimpaction protocol consists of 3 enemas per day for 3 days; most children are disimpacted after day 3.
If still impacted after 3 days of enemas, admit for nasogastric tube with GoLYTELY for 2 days plus enemas; disimpaction under anesthesia is extremely rare (approximately 4 cases in 8 years).
Senna-based laxative is preferred, given at 6 PM to produce bowel movement the next day, with dosage range from 8.8 mg to 175 mg.
Laxative dosage is adjusted daily based on bowel movement frequency, consistency, and abdominal radiograph showing stool burden; if no bowel movements in 24 hours, give enema and increase laxative dose.