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 [
8‐
11]. 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,
14‐
18]. 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 [
22‐
27]. In the absence of an underlying cause of terminal hematuria as detailed so far, in boys, one should then consider idiopathic urethritis (IU) [
5].