|Year : 2014 | Volume
| Issue : 3 | Page : 143-146
Idiopathic urethritis in children: Classification and treatment with steroids
Sivasankar Jayakumar, Kirsty Pringle, George K Ninan
Department of Paediatric Urology, University Hospitals of Leicester, Leicester, United Kingdom
|Date of Web Publication||9-Jul-2014|
George K Ninan
Consultant Paediatric Urology Surgeon, Department of Paediatric Urology, University Hospitals Leicester, Infirmary Road, Leicester, United Kingdom
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Background: Idiopathic urethritis [IU] in children is of unknown etiology and treatment options are limited. We propose a classification for IU based on cystourethroscopy findings and symptoms (Grade 1 - 4) and report our experience with use of topical and oral steroids in IU. Materials and Methods: Retrospective data collection of all male children (0-16 years) diagnosed with IU over a period of 8 years between 2005 and 2012 at our institution. Data was collected on patient demographics, laboratory and radiological investigations, cystourethroscopy findings, management and outcomes. Results: A total of 19 male children were diagnosed with IU. The median age of the patients was 13(7-16) years. Presenting symptoms included dysuria in 12; hematuria in 9; loin pain in 6; and scrotal pain in 2 patients. Both patients with scrotal pain had previous left scrotal exploration that revealed epididymitis. Serum C-reactive protein and Full blood count was tested in 15 patients and was within normal limits in all of them. Cystourethroscopy revealed urethritis of grade-I in 2; grade-II in 11; and grade-III in 3 patients. There were 3 patients with systemic symptoms from extra-urethral extension of inflammation (grade-IV). Mean follow up was 18.9(1-74) months. All patients had steroid instillation at the time of cystourethroscopy. Three patients with IU grade IV required oral steroids (prednisolone) in view of exacerbation of symptoms and signs despite steroid instillation. Complete resolution of symptoms and signs occurred in 18(94.7%) patients. Significant improvement in symptoms and signs was noted in 1(5.3%) patient who is still undergoing treatment. Conclusions: IU in male children can be successfully managed with steroid instillation, especially in grade I and II. Grade III, will need steroid instillation but treatment of scarring and stricture will necessitate longer duration of treatment. In children with IU and extra-urethral symptoms (grade IV), oral steroids may be required.
Keywords: Children, idiopathic urethritis, male, ninan classification, steroid instillation, urethritis
|How to cite this article:|
Jayakumar S, Pringle K, Ninan GK. Idiopathic urethritis in children: Classification and treatment with steroids. J Indian Assoc Pediatr Surg 2014;19:143-6
|How to cite this URL:|
Jayakumar S, Pringle K, Ninan GK. Idiopathic urethritis in children: Classification and treatment with steroids. J Indian Assoc Pediatr Surg [serial online] 2014 [cited 2020 Jul 9];19:143-6. Available from: http://www.jiaps.com/text.asp?2014/19/3/143/136464
| Introduction|| |
Urethritis of childhood affecting male children was first described by Williams and Mikhael in 1971.  The cause of urethritis in some children is unknown and described as idiopathic urethritis (IU). Association with dysfunctional elimination syndrome  and immunological conditions like Reiter's syndrome  has been described but scientific data is lacking. The first series of IU in male children included 19 boys diagnosed on clinical grounds after exclusion of bacterial infection.  Since then various articles have been published on this topic; however, treatment of IU in male children still remains as a challenge. Nonsteroidal anti-inflammatory drugs (NSAIDs) and antibiotics have been tried without much success.  We have previously reported successful treatment of IU with steroid instillation.  We hereby propose a classification for IU in male children and present our experience with steroids in a single center over a period of 8 years.
| Materials and methods|| |
Data was collected retrospectively on all male children (< 16 years of age) diagnosed with IU and managed by a single surgeon at our institution over a period of 8 years (2005-2012). Patients with balanitis xerotica obliterans (BXO) and positive urine culture at presentation were excluded from the study. Data was collected on patient demographics, laboratory and radiological investigations, cystourethroscopy findings, management, and outcomes. Data was incorporated into a spreadsheet (Microsoft Excel 2007) for analysis. A new classification was proposed based on the cystourethroscopy findings and symptoms and this is shown in [Figure 1].
|Figure 1: Ninan classifi cation of Idiopathic urethritis in male children, (Grade-I) Infl ammation involving urethra distal to external urethral sphincter, (Grade-II) Infl ammation extending into urethra proximal to external urethral sphincter, (Grade-III) Infl ammation of urethra with stricture formation or scarring, (Grade-IV) Infl ammation of urethra with retrograde extension to the upper tracts or epididymis with systemic symptoms|
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Steroid instillation procedure
Diagnostic cystourethroscopy was offered to all patients and performed under direct visualization. Steroids were instilled alone or mixed with lignocaine gel and instilled directly into the urethra. Single dose of intravenous gentamicin (dose: 2 mg/kg weight) was administered intraoperatively during the procedure. Triamcinolone or methylprednisolone was used for steroid instillation. The dose of triamcinolone used for each episode of steroid instillation was a standard dose of either 40 mg if patient is < 14 years of age or 80 mg if > 14 years of age. Dose of methylprednisolone used was 30 mg for steroid instillation. Oral steroids (2 mg/kg of prednisolone) were used for 6 weeks in three patients. No urinary catheter was inserted or left in situ during or after the procedure. Postoperatively, simple oral analgesics were given, but no antibiotics were prescribed.
| Results|| |
A total of 19 male children were diagnosed with IU. The median age of the patients was 13 (7-16) years. Presenting symptoms included dysuria in 12, hematuria in nine, loin pain in six, and scrotal pain in two patients [Figure 2]. The two patients with unilateral scrotal pain had previous scrotal exploration and were treated as epididymitis with antibiotics, however the scrotal pain persisted. These two patients were subsequently diagnosed to have posterior urethritis on cystourethroscopy. Past medical history included circumcision in two patients for religious reasons and in two patients for foreskin pathology. Two patients had treatment for suspected urinary tract infection in the past; however, both patients had a negative urine culture at the time of presentation. Serum C-reactive protein (CRP) and full blood count was tested in 15 patients and was within normal limits in all of them. Renal ultrasound scan (USS) was performed in 17 patients and was normal in 12, debris seen within the bladder in two, thickened bladder wall seen in two, and renal pelvis dilatation/hydronephrosis noted in three patients. None of our patients gave history of being sexually active at the time of presentation. But we did chlamydial studies in the adolescent age group and all of them were negative.
|Figure 2: Symptoms of IU patients in our series as per the IU Grade noted on cystourethroscopy|
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Cystourethroscopy revealed urethritis of grade I in two (10.5%) patients, grade II in 11 (57.8%), and grade III in three (15.8%). There were three (15.8%) patients with systemic symptoms from extra-urethral extension of inflammation (grade IV). Mean follow-up was 18.9 (1-74) months. All patients had steroid instillation at the time of cystourethroscopy. Twelve patients required more than one episode of steroid instillation. Three patients with IU grade IV required oral steroids (prednisolone) in view of exacerbation of symptoms and persistent urethritis noted on cystourethroscopy, despite steroid instillation. Complete resolution of symptoms and signs occurred in 18 (94.7%) patients. Significant improvement in symptoms and signs was noted in one (5.3%) patient, who is still undergoing treatment (grade III). The resolution of IU was confirmed by cystourethroscopy findings.
Most patients in our series (62.5%) required more than one episode of steroid instillation [Figure 3]. However, no significant difference in presenting symptoms and signs were noted among patients who required one or more episodes of steroid instillation. At an average follow-up of 18.9 months no complications were noted in our patients, suggesting a good therapeutic efficacy of steroids in IU.
|Figure 3: Comparision of IU patients with 1 or more episodes of steroid instillation and oral steroids [n = 18, one patient is still ongoing treatment]|
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| Discussion|| |
IU is a disease of unknown etiology in male children and diagnosed after exclusion of urinary bacterial infection. The exact pathogenesis of IU in children is so far unknown. Presenting symptoms in IU usually include frank hematuria, dysuria, and occasional loin pain. Some of these symptoms can be debilitating with significant effect on the child psychology and physical health, resulting in absence from school.  We have previously described urethrovasal reflux (UVR) enabling extension of inflammation from posterior urethra into the epididymis.  Our current series includes this patient and another similar patient who presented with persistent unilateral scrotal pain. The scrotal pain disappeared after steroid instillation into the urethra. We believe that UVR of steroids into the epididymis resulted in resolution of inflammation and symptoms. 
Cystourethroscopy is helpful in establishing the diagnosis of IU. Endoscopy findings in IU usually reveal erythematous and inflamed and fragile urethral mucosa with contact bleeding. Some have advised against cystourethroscopy in IU for fear of causing urethral strictures,  but this has not been our experience. All our patients, including the patients with stricture/scarring were diagnosed at cystourethroscopy and followed-up endoscopically until resolution of the disease occurred. Routine use of steroid instillation at endoscopy may be a reason for not causing stricture formation in our patients. In another series on seven adolescent male patients with IU, two patients presented with urethral strictures prior to any intervention, supporting that the strictures in IU are likely to be secondary to the disease and not a consequence of instrumentation. 
IU is classically described as confined to anterior bulbar urethra.  However, in our experience most children (57.8%) had inflammation extending to the posterior urethra (grade II). Anterior urethral inflammation alone (grade I) was noted in only 10.5% of our patients in our series. Squamous metaplasia of the urethra has been reported in association with prepubertal urethrorrhagia and may predispose to stricture formation.  We did biopsy of urethra in our first patient and the histology revealed nonspecific inflammation only. Based on this result and for fear of subsequent stricture formation, we have not biopsied the urethra in any other patient.
It has been suggested that IU can be self-limiting.  However, we believe that the duration of symptoms can be prolonged and complications can occur in the interim, if no treatment is offered. We have already reported extra-urethral complication including ascent of inflammation into the upper renal tract.  Therefore, offering no treatment can be associated with increased morbidity. Treatment with antibiotics and non-steroidal anti-inflammatory drugs (NSAIDs) in IU has been ineffective in our experience. Local inflammation can be treated with topical anti-inflammatory agents. Steroids are effective when used as topical anti-inflammatory and have been used as enemas to treat rectal disease in ulcerative colitis.  We first used steroid instillation for IU in 2005 and reported in 2009  (this patient is included in our current series). Since then we have used steroid instillation as a first line of treatment in all patients diagnosed endoscopically as IU. Both methylprednisolone and triamcinolone were used in our series; however, we believe that other topical steroids should also be equally effective.
| Conclusions|| |
IU in male children can be successfully managed with steroid instillation, especially in grade I and II. IU grade III will need steroid instillation, but treatment of scarring and stricture will necessitate longer duration of treatment. In children with IU and systemic symptoms (grade IV), oral steroids may be required.
| References|| |
|1.||Harrison SC, Whitaker RH. Idiopathic urethritis in male children. Br J Urol 1987;59:258-60. |
|2.||Herz D, Weiser A, Collette T, Reda E, Levitt S, Franco I. Dysfunctional elimination syndrome as an etiology of idiopathic urethritis in childhood. J Urol 2005;173:2132-7. |
|3.||Cuttica RJ, Scheines EJ, Garay SM, Romanelli MC, Maldonado Cocco JA. Juvenile onset Reiter's syndrome: A retrospective study of 26 patients. Clin Exp Rheumatol 1992;10:285-8. |
|4.||Eradi B, Ninan GK. Intravesical steroid instillation--a novel therapeutic intervention for idiopathic urethritis of childhood. Eur J Pediatr Surg 2009;19:105-7. |
|5.||Ninan GK, Bhishma P, Patel R. Steroid treatment for recurrent epididymitis secondary to Idiopathic urethritis and Urethrovasal reflux. Eur Jnl Ped Surg Reports 2013;1:56-9. |
|6.||Kaplan GW, Brock WA. Idiopathic urethrorrhagia in boys. J Urol 1982;128:1001-3. |
|7.||Dewan PA, Wilson TM. Idiopathic urethritis in the adolescent male. Eur Urol 1996;30:494-7. |
|8.||Walker BR, Ellison ED, Snow BW, Cartwright PC. The natural history of idiopathic urethrorrhagia in boys. J Urol 2001;166:231-2. |
|9.||Docimo SG, Silver RI, Gonzalez R, Müller SC, Jeffs RD. Idiopathic anterior urethritis in prepubertal and pubertal boys: Pathology and clues to etiology. Urology 1998;51:99-102. |
|10.||Ninan GK, Pringle K, Patel R. Upper urinary tract inflammation and dilatation secondary to idiopathic urethritis: A plea for steroid treatment. Int J Urol. 2013 Sep 3. doi: 10.1111/iju.12241. [Epub ahead of print] PubMed PMID: 24000904. |
|11.||Frei P, Biedermann L, Manser CN, Wilk M, Manz M, Vavricka SR, et al. Topical therapies in inflammatory bowel disease. Digestion 2012; 86:36-44. |
[Figure 1], [Figure 2], [Figure 3]