Surgical Management and Follow-Up: Cloaca and Complex ARMs 2015

Neurogenic bladder is defined as either the inability to store or empty urine.

Voiding may be possible, but in some cases voiding can actually be harmful.

If management is individualized based on beliefs or biases without consistent treatment algorithms, there are no demonstrable outcomes to measure.

The management of neurogenic bladder requires a multifaceted approach; surgery alone cannot make things better.

The ultimate goal of neurogenic bladder management is to protect the upper tracts.

A 30-year-old female with long common channel cloaca underwent PSARVUP with neovagina as a child, tolerated a voiding trial, but over her lifetime had multiple UTIs, vesicoureteral reflux treated with Deflux, left dysplastic kidney removed, and presented with hydronephrosis of solitary kidney, incomplete bladder emptying, chronic kidney disease stage 3, and hypertension.

Post-intervention with suprapubic tube, bladder cycling with Ditropan, ventriculofenestration, and Malone, the patient's hydronephrosis completely resolved.

When children are one month of age, they have about 30 to 40% of their actual adult GFR; GFR is still developing.

Tolerating hydronephrosis or urinary retention in infants may hurt developing kidneys.

A successful voiding trial is not predictive of future renal health.

Bladder management encompasses assessing bladder capacity and whether storage or emptying puts the patient at risk from an upper tract perspective, using urodynamic studies.

There is not a lot of data on voiding pressures, but certain cohorts of children with cloacal exstrophy do have deterioration of renal function long term with voiding.

A child with a 2 cm common channel and normal spine, born with normal kidneys and normal birth VCUG and ultrasound, developed multiple febrile UTIs, sepsis, pyelonephritis, and significant bilateral hydroureteronephrosis within a few months.

During cystoscopy at 18 months, the child had a massively trabeculated bladder despite short common channel and normal spine.

After repair, intermittent catheterization, and Ditropan, the child's massive reflux and hydronephrosis resolved, but she was left with chronic kidney disease stage 3 (GFR less than half of expected).

When catheterization was stopped because the child had a good voiding stream, reflux recurred, she had multiple UTIs, and eventually required a vesicostomy and is now on the transplant list.

The 2 cm common channel, normal spine, normal kidneys at birth, and passing a voiding trial did not portend a good prognosis for this child.

Family support is critical; these patients are resource-intensive, requiring nurses and specialized teams to teach catheterization and manage DME.

Yearly measurement of GFR is advised.

Baseline urodynamics are performed after about a year of age in selected high-risk cases.

Delayed urinary control is not normal in anorectal malformation.

Urinary tract infection is not normal in anorectal malformation.

Incontinence, volitional stream, or strong urinary stream does not necessarily mean children are healthy or suggest absence of a problem.

A lot of information can be gleaned from a simple ultrasound and VCUG, especially in the below 12-month age group.

Urodynamics under 12 months of age are challenging to interpret; high-pressure voiding is normal in that age group.

The trigone may be the area where the confluence of nerves interacts with the rest of the bladder, and that anatomy is altered especially with separation.

In posterior cloaca, the single perineal orifice is posteriorly deviated instead of at the normal urethral location, so it can appear the patient has a normal anus.

Posterior cloaca patients have a very short common channel and a very thick pubic bone, making it challenging to accommodate three structures, but mobilization is not usually difficult.

In a personal series of 560 cloacas, there is a spectrum of defects from very short to very long common channels, tethered cord to normal cord, normal to abnormal sacrum, born with or without hydronephrosis or reflux, some with giant low-pressure bladders at birth, some with small bladders, and some switch from big bladder to small hypertonic bladder.