Colorectal Quiz Episode 13: Newborn ARM Part 2
With Dr. Jason Frischer & Dr. Mark Levitt · hosted by Dr. Amanda Jensen & Dr. Rod Gerardo · Colorectal Channel
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
Indications for surgery in female ARM include: hole too small, hole not in center of sphincter, and inadequate perineal body
Perineal groove with mucosal lining will keratinize and look like normal perineal body over time
Surgical intervention for perineal groove is indicated only if secreting mucus, causing irritation, or developing ulcers
If anal opening is adequately sized, surrounded by sphincter, and has a perineal body (albeit short), no surgery is indicated
Short perineal body will grow over time and there is nothing to do about it surgically
If half the fistula is within sphincter complex and half outside, patient will leak stool because they cannot close the hole, making surgery worthy
Vestibular fistula is very common in females and needs formal repair with hole transposed to center of sphincter
For vestibular fistula diagnosed in newborn period, options are primary repair if baby is well, or allow stooling through fistula for couple months then elective repair without stoma
Patients diagnosed with vestibular fistula at 6-12 months who have dilated rectosigmoid need diversion as first step, then repair
Purpose of diversion in ARM repair is to avoid perineal body dehiscence
Cloaca presents with single perineal orifice and hypertrophied area around clitoral hood is typical, not ambiguous genitalia
Cloaca patients do not need endocrine workup or steroids and there is no question of gender assignment - they are female
Cloaca patients are still being misdiagnosed as ambiguous genitalia, with some babies not having proper gender assignment for one to two weeks
Key to perineal exam is to push down and flatten the perineal body to assess if it is normal
Normal anus is centered within sphincter, of adequate size, and perineal body is of normal length properly distanced from vestibule
To evaluate for vestibular fistula, pull labia towards examiner with both hands to visualize vaginal opening and urethra
Congenital perineal groove usually epithelializes on its own by age two
Perineal groove can be misdiagnosed as contact dermatitis, trauma, or sexual abuse
Three qualities to assess in anal location are: anal size, location (whether surrounded by sphincter), and perineal body size
Urogenital sinus plus normal anus is an endocrine problem, but no anus and urogenital sinus is a cloaca
Use Hagar dilators starting low and working up for accurate anal size measurement; do not use fingers because every surgeon has different size glove
Female ARM exam requires determining number of perineal orifices: three orifices means perineal or vestibular fistula; two orifices raises question of fistula presence, vaginal atresia, or rectovaginal fistula; one orifice is cloaca