Page 3 - CUA Best Practice Report: Pediatric hemorrhagic cystitis
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BPR: Pediatric hemorrhagic cystitis




       is associated with a greater risk of mortality, particularly if   or diffusely increased in the setting of HC. Doppler investi-
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       HC presents before 200 days post-SCT (p=0.002).  Viral eti-  gation may also reveal focal or diffuse hypervascularity, as
       ology did not affect post-SCT survival, rather hematological   well as distinct bleeding, which may be amenable to targeted
       malignancy (OR 2.74) and ifosfamide exposure (OR 1.988)   cautery during cystoscopic evaluation.
       were associated with higher mortality. 20,23             As there are no direct guidelines on treatment for this
                                                             patient population, there is no definitive algorithm of options
       Initial management/prevention                         and recommendations throughout the progression of treat-
                                                             ment. Fig. 1 provides suggestions for clinicians to guide
       Although the source of hematuria is quite clear in pediatric   them through treatment based on several systematic reviews
       patients undergoing BMT/HSCT, all other potential etiologies   and previously proposed algorithms. 6,21,25  The remainder of
       (i.e., urinary tract infection, urolithiasis, and urothelial/renal   this report will follow a similar progression of severity and
       malignancy) must be ruled out through a thorough history   invasiveness of treatment options.
       and physical examination and laboratory and microbiologi-  Because of the well-reported incidence of gross hematu-
       cal investigations. Imaging with Doppler renal and blad-  ria and HC in patients undergoing conditioning for alloge-
       der ultrasonography can aid with this evaluation.  Upper   neic BMT/SCT, these patients are routinely prophylactically
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       tract assessment may reveal hydronephrosis indicative of   treated with continuous bladder irrigation (CBI), 2-mercap-
       obstruction and subsequent need for operative management.   toethanesulfonic acid (mesna), and hyperhydration during
       Visualization of the bladder should incorporate measure-  their myelosuppressive conditioning. Whereas previously,
       ment of the bladder wall thickness, which may be focally   CBI use was based on anecdotal practice, Hadjibabaie et


                               Preventive/supportive treatment



                              1) Hyperhydration/forced diuresis
                               2) Continuous bladder irrigation
                                       3) Mesna
                                     4) Pain control
                               5) Bladder spasm management
                       6) Hematological optimization (transfusions if indicated)



            Low-grade HC (I–II)                      High-grade HC (III–IV)


          Refractory to preventive                      Cystoscopy, clot
             measures above                               evacuation



              Non-invasive                   Fulguration ± fibrin
         – PPS, estrogen, antivirals                            Intravesical irrigation
                                             glue ± intravesical
           – Hyperbaric oxygen                irrigation (focal   (diffuse bleeding)
                therapy                                             – Formalin
                                                bleeding)

           Invasive (intravesical                 Refractory bleeding/clot retention
               irrigation)
          – Alum, hyaluronic acid,
             PGE, antivirals
                                             Hemodynamically       Hemodynamically stable:
                                                unstable:                – PNTs
                                                – Selective        – Vesicostomy ± packing
            Progression of HC                angioembolization         – Cystectomy
                                                 – PNTs
       Fig. 1. Proposed algorithm for treatment of pediatric hemorrhagic cystitis. HC: hemorrhagic cystitis; PGE: prostaglandin E;
       PNT: percutaneous nephrostomy tubes; PPS: pentosan polysulfate.


                                                CUAJ • November 2019 • Volume 13, Issue 11                   E327
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