Bladder exstrophy: an stark example of regional differences in health care delivery

Male Bladder exstrophy and epispadiasExstrophy repair is unique in medicine: different places in the same country with the same resources treat exstrophy in radically different ways.

Differences in surgical care are common in pediatric urology and in all medicine: open versus laparoscopic reimplant, scrotal versus inguinal orchiopexy, TIP hypospadias repair versus Mathieu repair etc. These minor differences do not translate in significant changes in outcomes, expenses, and hospital stay like the ones found in exstrophy.

On one extreme you have the “complete” repair”:

  1. Saran wrap to cover bladder for 2-6 months.Seran wrap to cover bladder exstrophy
  2. Surgery at 2-6 months:
    1. Oblique osteotomies, 1 on each side (orthopedic surgeons fractures the bone in order to close the pubic bones together)
    2. Bladder closure and simultaneous repair of the penis (epispadias repair with penile disassembly to provide the longest possible penis).
    3. Spica Cast
  3. Transfer to PICU extubated for 1-2 days, then to the floor for 2-4 days then home. Patients are then home in 5-7 days.
  4. Cefazolin antibiotic prophylaxis before incision and for 24 hrs, then Trimethoprim-Sulfamethoxazole for prophylaxis while tubes are in.

Another approach developed at Hopkins is called the “modern” repair:

  1. Newborn repair (first few days, weeks of life)
    1. 2 osteotomies on each side
    2. Bladder closure only, the penis is not repaired right away.
    3. No spica cast, traction is done in the hospital for 4-6 weeks (Bucks or Bryant’s traction)
  2. After surgery transfer to NICU intubated/sedated for 2-7.
  3. IV antibiotics, Ampicillin, and Gentamycin are given at treatment doses for 2 weeks.
  4. The patient is home in 4-6 weeks. 

 

To be fair, these are only the extremes. Some centers use a combination of the above.

Antibiotics

In the complete repair, Cefazolin is given preop and not for more than 24 hrs as recommended in the AUA guidelines. In the modern approach intravenous, Amp and Gent are given for 2 weeks! This would require either a PICC line or multiple IV’s as well as checking Gentamycin levels constantly to make sure the patient does not get renal or ear damage. I would also think that the risk of fungemia or MRSA infection would be higher with IV antibiotics for 2 weeks.

Diet

With the complete approach, the patient can be eating in the first 24 hrs after surgery. With the modern approach patient may not start eating for the first week and thus would require a PICC line and parental nutrition. By now is common knowledge in surgery that early feeding by mouth improves the healing process and decreases the chance of infection. Adding a central line puts the patient at risk of CLABSI (central line-associated bloodstream infection).

Immobilization

With the complete approach, the patient is left on a low maintenance spica cast that the patients can take care of at home within a week from surgery. With the modern approach using Bucks or Bryant’s traction, patients need to be hospitalized for 1 month with close monitoring of the traction because if done wrong it could have serious consequences.

Penile repair

In the complete repair, the urethra is separated from the penis which allows for 2 things:

  1. The bladder and urethra are able to be placed deep inside the pelvis
  2. The penis is able to come out to the surface instead of been tethered by the urethra.

Without this urethral separation, the bladder is left more superiorly and the penis more inside the body.

At the end of the day, most patients that have had bladder exstrophy will need to catheterize and will not be able to void on their own as this study from the PUMA collaboration showed. What male patients tend to care later in life is the appearance of the penis. Leaving the urethra attached to the penis would definitely not provide the best appearance in the future.

Why the discrepancy?

Hopkins has made a career of operating on bladder exstrophy and have published really successful outcomes. Because of that, they get lots of referrals. Many of the referrals are of those patients who have had failed repairs done elsewhere.

A variation of the complete repair is done by other prominent hospitals like CHOP, Boston Children’s, Milwaukee children’s and Seattle. Both Hopkins and these other hospitals are considered centers of excellence for bladder exstrophy repair by the Association of the Bladder Exstrophy Community. 

It seems that the complete repair is performed by the majority of the most prominent hospitals and is likely the most common technique used to repair bladder exstrophy in the US (no data to back this statement up). Because of this, most referrals to Baltimore would have had a complete repair.

From the perspective of Baltimore, the complete repair must be terrible since every single complication they get referred has had that approach. But the problem is that they are not seeing the good outcomes. Imagine 100 repairs are done using the complete approach, of which 5 get complicated: 5% complication rate. If the 5 complications go to Baltimore, for them the repair seems to have a 100% complication rate. I think this is the main reason Hopkins has not changed their approach.

Unbiased studies have shown no difference in outcomes between the 2 approaches. To this, the Baltimore people would say, “if no difference then is surgeon preference and whatever the surgeon feels more comfortable doing”.

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