ARMs in Neonates: Pediatric Colorectal Controversies 2014
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
There is no urgency to operate on an ARM patient on the day of birth as long as the abdomen is soft and not distended
About 95% of Down syndrome patients with ARM have no fistula
A flat bottom in a newborn with ARM usually indicates a high fistula and predicts poor continence
Every ARM patient needs to be evaluated in context of three factors: type of malformation, quality of sacrum, and quality of spine (the 'ARM index') to predict continence
A bladder neck fistula patient is very different from a perineal fistula patient in terms of continence prognosis
The critical cutoff point for timing of ARM repair is when infants transition from breast milk/formula to solid food (around 6 months), as stool character changes and rectal dilation begins
An anus is defined as a properly sized hole in the center of the sphincter that is mucosa-lined
Trans-scrotal fistulas are generally low-type malformations in more than 90% of cases, suitable for primary neonatal repair
Perineal ultrasound for determining rectal position requires a skilled radiologist who does not push too hard and a quiet baby who is not valsalving
In newborn male perineal fistula repair, the urethra is incredibly close to the rectum, and circumferential anterior mobilization risks urethral injury
A cutback technique (extending rectum posteriorly without anterior dissection) in males avoids dangerous anterior dissection and reduces stricture, and does not require daily dilation postoperatively
If anal opening is left outside the sphincter mechanism, patients may have anterior leakage during athletics or with loose stool when they try to close the sphincter
What looks like a very small perineal body in a newborn female actually gets much bigger as the child grows
From a gynecological standpoint, building as good a perineal body as possible is important for separation of reproductive organs from GI tract, sexual functioning, and possibility of vaginal delivery
Women can become incontinent from vaginal delivery even without ARM history, so the risk after ARM repair is too high to recommend vaginal delivery
Vaginal delivery after ARM repair is possible with lateral episiotomy if needed and proper planning with a pediatric gynecologist familiar with ARM repairs
Cloaca (single perineal opening with no anus) does not require endocrine evaluation and electrolytes will be normal, unlike urogenital sinus with normal anus which may indicate congenital adrenal hyperplasia
The incidence of congenital adrenal hyperplasia in the ARM population is almost zero
Perineal fistula is distinguished from vestibular fistula by presence of perineal body; vestibular fistula has no perineal body
Spinal ultrasound can detect presacral masses if the radiologist is specifically looking for them and increases depth of examination, though very small masses may be missed
Missing a presacral mass can result in teratoma becoming malignant; at least two cases are known where presacral mass was missed on newborn evaluation
Cutback technique preserves more rectal tissue including potential sensory fibers at the dentate line
Every ARM patient with anal stenosis must have presacral mass ruled out with plain x-ray of sacrum and ultrasound looking specifically at presacral space
All ARM patients need esophageal atresia ruled out before proceeding with colostomy
The 'Twitter sign' (air visible in fistula tract on cross-table lateral) is consistent with high rectum with probable rectourethral fistula
It is exceedingly rare to have perineal meconium and a long fistula; almost uniformly these patients have very reachable rectum suitable for primary repair
If normal physical exam, normal chest x-ray, and normal ECG, significant cardiac problems that would interfere with anesthesia are never found in ARM patients
Cross-table lateral films showing very short distance between skin and rectum are most useful; long distances may be falsely elevated due to meconium preventing air from reaching the rectum
Colostomy should be opened at the very proximal sigmoid (where sigmoid begins at left retroperitoneal attachments) so that part of colon won't prolapse
Complete evacuation of distal meconium at time of colostomy is a very important job that should not be neglected
Laparoscopic colostomy allows precise identification of proximal sigmoid and creation of stomas without skin bridge, avoiding wound complications
What affects UTI rate in ARM patients with colostomy is presence of vesicoureteral reflux or neurogenic bladder, not stoma type
Loop stomas may be acceptable when fistula is very small or bladder is normal with no other problems, and especially if repair is done early (within 3 months)
Opening colostomy too distal leaves insufficient bowel for the pull-through procedure
Rollins' data showed approximately 30% of perineal fistula patients had associated anomalies across GU and cardiac systems
Loop stomas theoretically allow distal spillage and have high prolapse rates, but recent data from Toronto shows no difference in UTI rates between loop and divided stomas