Page 8 - CUA Best Practice Report: Pediatric hemorrhagic cystitis

Hannick & Koyle

may also be considered in patients for whom conservative tive surgery to remove the source of HC. As this is an irrevers-
and other less invasive therapies have proven ineffective and ible option, it should only be considered in intractable, life-
HC has persisted for months. García-Gámez et al noted com- threatening HC. The surgical approach and urinary diversion
plete resolution of HC within 1‒2 weeks in their three patient selection are at the discretion of the patient and surgeon,
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case series of children with post-BMT/SCT HC. Han et al however, most reports describe the construction of an ileal
reported on their aggressive case series of pediatric and adult neobladder either with preservation of the bladder trigone
patients with grade III‒IV HC following post-allogeneic SCT and ureteral insertion or without trigonal preservation and
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who failed to improve with hyperhydration, platelet/blood ureteral reimplantation. As with neobladder creation for
transfusion, CBI, and pain management. Patients had HC other indications, the detubularized Studer type has largely
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for a median of 20 days prior to undergoing SVAE. SVAE become the surgical approach of choice due to its increased
yielded a complete response in 60% and partial response in bladder capacity and decreased bladder spasms from gut
20%, with a median time to complete response of 26 days. peristalsis. Regarding management of the trigone, Sèbe et
Morbidity was noted in 20% of patients who complained of al favor subtotal cystectomy with bladder neck preservation
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gluteal claudication the day following embolization, however, if the ureters are unobstructed. Should there be concern
this spontaneously resolved within one day of the intervention for irreversible ureteral obstruction, however, ureteral reim-
in all patients. Data are lacking on the long-term sequelae of plantation is recommended.
bladder function following SVAE. Recommendation: Cystectomy is reserved as a treatment
Recommendation: SVAE offers modest control of HC option when all other efforts to control HC have failed.
refractory to conservative therapy, however, outside of Long-term implications of this option must be considered
unstable patients, the appropriate time point for its use in the pediatric population, as well as the fitness and hemo-
along the HC treatment pathway and the long-term safety dynamic stability of the patient for whom it is chosen (Level
of its application require further investigation (Level 3 evi- 4 evidence, Grade C recommendation).
dence, Grade C recommendation).
Experimental therapies
Cutaneous vesicostomy
Numerous experimental therapies are being studied for
Short of proceeding with cystectomy, an alternative to bilat- patients with HC that either focus on rebuilding injured bladder
eral PNTs for urinary diversion is the cutaneous vesicosto- mucosa or augmenting the coagulation cascade. Recombinant
my. Among the case reports describing its use, it is typically human keratinocyte growth factor, epidermal growth factor,
employed in the setting of hemodynamic instability with placenta-derived stromal cells, mesenchymal stem cells, and
high transfusion requirements or in patients with terminal adoptive transfer of viral-specific T cells for virus-associated
disease. 25,58 In Gander et al’s case, a gel-port was used to HC in patients with slow immune reconstitution have been
secure the bladder mucosa against the abdominal wall, as studied in case reports in patients refractory to conservative
severe bladder wall edema prevented adequate suture fixa- therapies such as CBI and HOT. While preliminary reports
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tion. A urinary ostomy bag can be applied overlying the with some of these therapies are promising, minimal evidence
vesicostomy to collect urine, more easily remove clots, and exists to support their effectiveness and safety. 60-63
continue CBI through a urethral catheter if so desired. In Recommendation: Due to the experimental nature of
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the author’s experience, bladder-packing with lidocaine with therapies such as recombinant human keratinocyte growth
epinephrine soaked sponges can also aid with local analgesia factor, epidermal growth factor, placenta-derived stromal
and vasoconstriction to reduce hematuria. This approach can cells, adoptive transfer of viral-specific T cells, factor VII,
allow for removal of urethral catheters, which, particularly in factor XIII, and mesenchymal stem cells, their therapeutic
children, can be a source of much distress and discomfort. use to treat HC is not recommended until further robust tri-
Recommendation: Despite a lack of high-quality studies, als demonstrate safety and benefit (Level 4 evidence, Grade
cutaneous vesicostomy in the terminal or refractory pediatric D recommendation).
patient offers potentially reversible direct bladder access for
clot evacuation and application of topical therapies, while Conclusions
also allowing for possible removal of painful urethral cath-
eters (Level 4 evidence, Grade C recommendation). Although the age-old saying, “children are not little adults,”
is true when dealing with most pediatric illnesses, many of
Cystectomy the management strategies used to manage adult HC have
been tested and successful in pediatric populations as well.
When all conventional and invasive options have been HC in children more frequently rises from complications
exhausted, and HC persists, the urologist can turn to extirpa- of oncological regimens and immunosuppression, while in

E332 CUAJ • November 2019 • Volume 13, Issue 11