Page 6 - CUA Best Practice Report: Pediatric hemorrhagic cystitis

Hannick & Koyle

Hyaluronic acid (HA) index of suspicion for toxicity should any neuropsychiatric
symptoms arise.
Much like PPS, the use of HA has predominantly been seen Recommendation: Alum bladder irrigation is effective in
in the interstitial cystitis population for its role in enhancing treating pediatric HC, although the patient’s bladder must
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connective tissue healing within the bladder. Additionally, be fully cleared of blood clots prior to initiation of therapy.
it has been proposed that the HA may alter a step of the poly- Patients must also be monitored closely for potential alu-
omavirus’ life cycle and that its breakdown products may minum toxicity, particularly in the setting of renal failure
trigger the secretion of various cytokines and chemokines (Level 3 evidence, Grade C recommendation).
by macrophages that inhibit viral replication. In their case
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report of a 7.5-year-old boy with HC refractory to conserva- Cystoscopy, clot evacuation, and fulguration
tive therapy post-BMT, Cipe et al administered intravesical
HA (40 mg in 50 ml solution) via catherization, clamped for Following failure of conservative therapies, cystoscopy offers
one hour, drained, and then repeated dosing a week later both a diagnostic and therapeutic opportunity for the urolo-
after an initial partial response, and later complete resolution gist to escalate management of the HC patient. Cystoscopic
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within four days of the second dose. Miodosky et al used evaluation aids the clinician in ruling out any potential
a similar protocol prospectively in a group of pediatric and malignant causes of HC while also distinguishing bleeding as
adult post-SCT HC patients with no control group and repeat originating from a focal vessel or a diffuse source. The small
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dosing after one week if no response in HC was noted. Of diameter of the pediatric urethra, especially in prepubertal
the seven patients treated, four responded after an initial males, can be quite problematic, hence limiting the ability
dose, and two responded after a second dose. Complete to pass a reasonably sized scope that will allow effective
response was noted in five of the seven patients at a median irrigation and evacuation of clots. When possible, with the
of 12 days (range 7‒23). larger diameter of the cystoscope relative to a Foley catheter,
Recommendation: HA intravesical therapy may be of effective clot evacuation can proceed, possibly concomitant
benefit in treating HC via bladder instillation, although with the placement of a suprapubic catheter for more facile
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repeated applications may be required and, thus, more postoperative bladder irrigation. Focal bleeding vessels can
robust evidence is required to demonstrate its efficacy be cauterized either with a Bugbee electrode or laser fiber,
(Level 4 evidence, Grade D recommendation). though aggressive, diffuse fulguration risks potential late scar
formation and bladder capacity compromise. 8,21
Alum Recommendation: Although evidence is lacking, cystos-
copy, clot evacuation, and fulguration of bleeding are a
The use of intravesical alum in patients with gross hematuria mainstay of therapy in management of pediatric HC for
is well-noted in the adult literature. Alum is thought to work their capacity to diagnose and treat HC, as well as provide
by adhering to raw protein surfaces, resulting in decreased symptom relief from patients in clot retention (Level 4 evi-
vascular permeability, vasoconstriction, and reduced inflam- dence, Grade C recommendation).
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mation. As its mode of administration is relatively similar
to CBI, it is commonly used when patients demonstrate HC Fibrin glue
refractory to CBI alone. Typically, a solution of 1% alum (10
g aluminum potassium sulfate mixed in 1 L distilled water) Though a relatively new phenomenon, fibrin glue applica-
is instilled via a three-way Foley catheter at a rate of 300 tion at the time of cystoscopy has demonstrated substantial
ml/hour or less. Prior to initiation, patients must be cleared success in HC refractory to non-invasive conservative thera-
of all intravesical blood clots, either manually or surgically; pies. Tirindelli et al have published multiple accounts of their
otherwise, they may suffer from difficult to resolve “alum work with fibrin glue. 51,52 The fibrin glue is generated in 6 ml
balls,” which can precipitate urinary obstruction. Praveen at aliquots from 120 mls of the patient’s own virus-inactivated
al evaluated the effectiveness of alum for treating hematuria blood or fresh-frozen plasma over 30 minutes. In their cases,
in a prospective, randomized, controlled comparison with cystoscopy is performed with a 24 Fr nephroscope and the
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PGF2 instillation. Of the nine patients treated with alum, bladder insufflated with carbon dioxide at 12 mmHg, and
six had complete resolution of hematuria and three had the glue then sprayed evenly over the bleeding, raw surfaces,
partial resolution; however, three had recurrent bleeding. where it polymerizes on contact and sets over several days.
Along with bladder spasms, as were seen in all of Praveen’s A catheter is left in place without CBI postoperatively. In
alum patients, alum toxicity can lead to encephalopathy and their earlier study, 4/5 (80%) patients had a rapid, good
seizures, particularly in patients with renal insufficiency. 6,28,50 response, one of whom was a 17-year-old female (the rest
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Patients receiving alum therapy should be monitored with were adults). In a later study with 35 patients, there was
serial aluminum levels and evaluated regularly with a high 83% complete response rate, which was achieved after one,

E330 CUAJ • November 2019 • Volume 13, Issue 11