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03.02.2022 | Clinical Quiz

Gross hematuria in a boy — “spot” the diagnosis: Answers

verfasst von: Sandeep Riar, Alonso Carrasco Jr., Tanya Pereira

Erschienen in: Pediatric Nephrology | Ausgabe 7/2022

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Excerpt

1.
What additional question and complementary finding on physical exam might indicate non-glomerular rather than glomerular cause of gross hematuria?
An additional question is about the phasic characteristic of hematuria to clarify if it is initial, total, or terminal. An additional complementary finding on exam for terminal hematuria is “blood spots on underwear,” indicating urethrorrhagia. Unless asked explicitly, a patient may not report phasic hematuria. On enquiry, our patient’s hematuria was described as “a few drops of blood at the end of urination.” On examination, a few drops of blood on his underwear were noted. Urethrorrhagia (urethral bleeding) is defined as bleeding emanating from the urethra at a point distal to the bladder neck (Fig. 1), occurring separate from micturition and marked by the presence of blood spots on the underwear [1]. Some include terminal hematuria in the description of urethrorrhagia and define it as a spotting of blood on the underwear after urination or voiding of clear urine followed by a few drops of blood [2].
The determination of urine discoloration being initial (at the beginning of the stream only), terminal (at the end of the stream only), or total (throughout the entire stream) may indicate the probable site of bleeding within the urinary tract [3]. Initial hematuria generally indicates some type of anterior urethral bleeding that is flushed out by the initial passage of the bladder urine through the urethra. Terminal hematuria is often secondary to posterior urethral, bladder neck, or trigone bleeding and is noted when the bladder finally compresses these areas at the end of micturition. Anterior urethra and posterior urethra are the two major segments of the male urethra (Fig. 1). Anterior urethra extends from the external urethral meatus and includes the fossa navicularis, penile (also called pendulous) urethra, and bulbar urethra. Posterior urethra includes membranous urethra, prostatic urethra, and bladder neck. Total hematuria usually indicates the occurrence of bleeding at the level of the bladder or above, such that all the urine is mixed with blood and is therefore discolored throughout the entire stream. One may not depend too much on this characteristic of gross hematuria though as it may sometimes be absent with non-glomerular hematuria and history may not be available or reliable in some settings.
 
2.
What is the differential diagnosis of gross hematuria with this clinical feature?
The differential diagnoses for terminal hematuria include disorders in the posterior urethra, bladder neck, or trigone area and broadly include infectious and non-infectious etiology.
Infectious causes of terminal hematuria include cystitis or urethritis related to bacterial, viral, or protozoal organisms. The presence of dysuria and urgency indicates cystitis while urethral discharge suggests urethritis [4, 5]. Workup should include urinalysis with urine culture and as appropriate screening for sexually transmitted infections such as Neisseria gonorrhoeae, Chlamydia trachomatis, and Mycoplasma genitalium. Immunocompromised patients may develop hemorrhagic cystitis from BK virus, JC virus, and adenovirus infection but also Ureaplasma urealyticum [6]. Rare but serious infectious causes of terminal hematuria include schistosomiasis and tuberculosis [7]. Terminal hematuria is a classic feature of urogenital schistosomiasis, caused by S. haematobium, a trematode parasite found in Africa, the Middle East, and Corsica, and a single exposure to freshwater ponds, lakes, and rivers is enough to cause infection [811]. Early signs include dysuria, pollakiuria, and terminal hematuria, but severe infection may cause total hematuria [12]. Pollakiuria refers to a very high daytime frequency of micturition (up to 50 times per day) [13]. Definitive diagnosis requires visualization of S. haematobium eggs in urine, detectable from 5 to 13 weeks after initial infection which may need several samples; serological testing is not useful [10].
Non-infectious causes of terminal hematuria include trauma, urethral malformations (posterior or anterior urethral valves, polyps, diverticula, Cowper gland cystic dilation, vascular malformations such as in Klippel–Trenaunay syndrome, prostatic utricle cyst), foreign body, calculi, urethral prolapse (in girls), chemical urethritis (amiodarone induced), autoimmune (reactive arthritis), urethral stricture disease, or neoplasms [1, 5, 1418]. Terminal hematuria has been reported in adult females with interstitial cystitis and migration of intrauterine device [19, 20]. Painless gross hematuria requires consideration of urinary tract neoplasms but is rare in children [3, 15, 21]. Pediatric kidney tumors such as Wilms tumor, renal cell carcinoma, and medullary renal cell carcinoma may cause gross hematuria, and are often associated with flank pain and abdominal mass. Pediatric tumors arising in the bladder or urethra are rare, may be benign (lymphangioma, fibroepithelial polyps, cystitis cystica/glandularis, keratinizing squamous metaplasia etc.) or malignant (rhabdomyosarcoma, botryoid sarcoma etc.), and are to be considered if a mass is noted on exam or imaging [2227]. In the absence of an underlying cause of terminal hematuria as detailed so far, in boys, one should then consider idiopathic urethritis (IU) [5].
 
3.
What is the most likely cause for this patient’s presentation?
The most likely cause of terminal hematuria in this child is idiopathic urethritis (IU), often also referred to as urethrorrhagia, a condition exclusively affecting prepubertal and pubertal males with symptoms as already described [28].
 
4.
What is the significance of absence of hematuria on urine studies?
 
Literatur
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Zurück zum Zitat Murphy JP, Gatti JM (2012) Abnormalities of the urethra, penis, and scrotum. In: Coran AG, Caldamone A, Adzick NS, Krummel TM, Laberge J, Shamberger R (eds) Pediatric surgery, 7th edn. Mosby, pp 1555–1563CrossRef Murphy JP, Gatti JM (2012) Abnormalities of the urethra, penis, and scrotum. In: Coran AG, Caldamone A, Adzick NS, Krummel TM, Laberge J, Shamberger R (eds) Pediatric surgery, 7th edn. Mosby, pp 1555–1563CrossRef
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Zurück zum Zitat Campos-Juanatey F, Osman NI, Greenwell T, Martins FE, Riechardt S, Waterloos M, Barratt R, Chan G, Esperto F, Ploumidis A, Verla W, Dimitropoulos K, Lumen N (2021) European Association of Urology guidelines on urethral stricture disease (part 2): diagnosis, perioperative management, and follow-up in males. Eur Urol 80:201–212. https://doi.org/10.1016/j.eururo.2021.05.032CrossRefPubMed Campos-Juanatey F, Osman NI, Greenwell T, Martins FE, Riechardt S, Waterloos M, Barratt R, Chan G, Esperto F, Ploumidis A, Verla W, Dimitropoulos K, Lumen N (2021) European Association of Urology guidelines on urethral stricture disease (part 2): diagnosis, perioperative management, and follow-up in males. Eur Urol 80:201–212. https://​doi.​org/​10.​1016/​j.​eururo.​2021.​05.​032CrossRefPubMed
19.
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Metadaten
Titel
Gross hematuria in a boy — “spot” the diagnosis: Answers
verfasst von
Sandeep Riar
Alonso Carrasco Jr.
Tanya Pereira
Publikationsdatum
03.02.2022
Verlag
Springer Berlin Heidelberg
Erschienen in
Pediatric Nephrology / Ausgabe 7/2022
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-022-05461-0

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