Urologic and Gynecologic Aspects in Anorectal Malformations: Pediatric...
With Dr. Shamel Elam & Dr. Donald Shaw & Dr. Brad Kropp · hosted by Dr. Mark Levitt · StayCurrentMD
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What the experts said
High-grade reflux with ureteral dilation and renal pelvic dilation indicates a child at relatively high risk who should be followed closely and warrants urologic evaluation.
A normal renal ultrasound is very reassuring that the kidneys at birth are in good shape, but it does not give all the answers—you can have a normal appearing kidney and still have high-grade reflux and bladder pathology.
Every child with an anorectal malformation must have initial ultrasonography of the urinary tract at presentation and in follow-up.
Solitary kidney patients are at higher risk for further injury of that solitary kidney, generally in the case of unrecognized or underdiagnosed neurogenic bladder.
Children with high-grade reflux, solitary kidney, and bladder neck fistula need very close follow-up with serial ultrasonography, maintenance of sterile urine, blood work (serum creatinine, cystatin C), and regular urodynamic studies.
All humans are born with all the nephrons they will have for the rest of their lives, with continued nephron development only for the first 6 months after birth.
Reflux itself does not damage kidneys, but infection does. Reflux in conjunction with bladder dysfunction can damage kidneys.
A creatinine of 0.3 for the first 12-18 months of life may seem normal, but if the child is at the 4th percentile for height and weight, that is not a normal condition—growth parameters are important indicators of renal function.
In high-risk ARM cases (solitary kidney, reflux, bladder neck fistula), a divided colostomy is preferable to a loop colostomy to ensure complete fecal diversion.
Total urogenital mobilization eliminates any future function of the external urinary sphincter, making continence dependent solely on bladder neck function.
Continence after total urogenital mobilization depends more on bladder function and bladder compliance than on the bladder neck itself.
A 12-year-old ex-cloaca patient who is voiding well, has urinary control, and has no UTIs is not necessarily safe—underlying neuropathic bladder can cause long-term renal damage without obvious symptoms.
Neuropathic bladder does not always mean the patient leaks or has urinary tract infections—the functional morbidity can be silent.
The bladder stores urine for about 23 hours and 40 minutes during the day—the storage function is more important than the emptying function for long-term renal health.
Asking if a patient is continent is not enough to assess bladder health—you need evidence of how the bladder is storing urine.
In ARM patients with bladder neck fistula, single kidney, reflux, and tethered cord, expectations should not be set for volitional voiding—these patients are at high risk for neurogenic bladder.
The appendix makes a nice Mitrofanoff that tends to have longer durability than a tapered ileal piece.
When doing split-appendix technique, implant the Mitrofanoff very carefully first, then see where the Malone goes—pushing the Malone to the umbilicus after Mitrofanoff implantation risks blood supply compromise and tissue tearing.
In ARM patients with absent sacrum and malrotation requiring Ladd's procedure, the appendix should be preserved (not removed) for potential future use in Mitrofanoff or Malone procedures.
At the onset of puberty in cloaca patients, parents must be educated to watch for undrained fluid collections, especially if rudimentary uterine structures were left in place.
For fertility potential, a connected system is needed: distal fallopian tube to uterine Müllerian structure to cervix (important for carrying pregnancy) to vaginal outflow tract.
Performing ureteral reimplantation at the time of initial cloaca repair (rather than delayed) makes the secondary operation for continence and augmentation more pleasant and leads to faster patient recovery.
It is very important to know whether the patient has reflux before undertaking a cloaca repair—sometimes cystoscopy is needed to catheterize the bladder for a VCUG.
Reimplanting the ureter after a complex cloaca repair with long common channel and bladder neck mobilization is a totally difficult operation—better to do it at the time of cloaca repair if reflux is present.
Cutaneous ureterostomy should only be done if the ureter is dilated, as the biggest complication is stenosis.
A massively dilated ectopic ureter is a situation where ureterostomy may be safer than primary reconstruction, with reimplantation deferred to a later date.
Five-centimeter common channel cloaca patients rarely have volitional voiding that is functional later in life.
If a newborn requires vaginostomy and/or vesicostomy, those structures must be taken down to perform the cloaca repair—they cannot be left in place.
A circle stent (small 6 or 8 French elastic catheter) coming out of the urethral repair and bladder, tied to itself and protected with suprapubic tube, allows 100% certainty about urethral healing and avoids perineal catheter trauma.
Patients who need vesicostomy probably have some impairment in bladder function and may need intermittent catheterization long-term.
Total urogenital mobilization patients are likely to void and be easily catheterized, but complex urethral reconstructions may not be easily catheterizable, warranting prolonged urethral stenting or vesicostomy.
A vesicostomy with refluxing ureters provides safety and allows waiting until ultimate urologic reconstruction at age 4.
The fundamental principle is to keep the kidneys at low pressure—there are many different ways to accomplish this.
A suprapubic cystostomy tube at definitive cloaca reconstruction diverts urine, keeps the perineum dry, and automatically provides a way to assess bladder emptying.
Teaching families intermittent catheterization gives them control and prevents no-care-zone situations, especially for families traveling long distances.
A febrile urinary tract infection should prompt immediate assumption that the bladder is not successfully emptying—pre- and post-void residuals and assessment for scarring are essential.
Excellent nursing staff who can teach families intermittent catheterization is as important as any surgical procedure—families are scared and need professional guidance.
Teaching catheterization in the OR with the child asleep alleviates huge anxiety for families, allowing them to practice multiple times and learn nuances before the child wakes.
If total urogenital mobilization does not get the urethral opening as far out on the perineum as possible, it leaves a female hypospadias or enteritis that is harder for families to catheterize.