- Email: [email protected]
Anterior Urethral Valve – A Commonly Misdiagnosed Entity in Adolescent Boys
Journal Pre-proof
Anterior urethral valve- A commonly misdiagnosed entity in adolescent boys Dinesh Reddy , Ashwin Shekar P PII: DOI: Reference:
S0090-4295(20)30181-3 https://doi.org/10.1016/j.urology.2020.02.006 URL 21990
To appear in:
Urology
Received date: Revised date: Accepted date:
4 December 2019 30 January 2020 4 February 2020
Please cite this article as: Dinesh Reddy , Ashwin Shekar P , Anterior urethral valve- A commonly misdiagnosed entity in adolescent boys, Urology (2020), doi: https://doi.org/10.1016/j.urology.2020.02.006
This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2020 Published by Elsevier Inc.
1
Manuscript type: Pediatric case report Title: Anterior urethral valve- A commonly misdiagnosed entity in adolescent boys Authors: a
Dinesh Reddya, Ashwin Shekar. Pa*.
Department of Urology, Sri Sathya Sai Institute of Higher Medical Sciences,
Prashantigram, Puttaparthi, Andhra Pradesh 515134, India. *Corresponding Author-Ashwin Shekar P, Consultant Urologist, Department of Urology, Sri Sathya Sai Institute of Higher Medical Sciences, Prashantigram, Puttaparthi, Andhra Pradesh 515134, India. Ph no: +91-9940658701 Email address: [email protected] (Ashwin Shekar.P). Keywords: anterior urethral valve ,adolescent Acknowledgement: None Conflict of interest: None Funding: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
2
Abstract Anterior urethral valves are a rare cause of obstructed voiding in adolescent children and are often unheard by adult urologists. In this case report, we discuss the management of two adolescent patients who were referred to us with obstructive voiding symptoms with a diagnosis of neurogenic bladder and posterior urethral valves respectively but on evaluation were found to have anterior urethral valves. This article highlights the need for considering anterior urethral valve as an important differential diagnosis in children and adolescents presenting with obstructive voiding symptoms so as to avoid delay in diagnosis and management in young boys.
Introduction:
Urethral valves are a common cause of obstructive uropathy in boys. Though posterior urethral valves (PUV) are a more frequent cause, the obstructive effects of anterior urethral valves (AUV) can cause equal damage to the kidneys. AUV is 10-30 times less common than posterior urethral valve. 1 Due to its rarity, knowledge of this entity is quite less among practicing adult urologists. This article highlights two cases of AUV who were mismanaged by adult urologists as a case of neurogenic bladder and posterior urethral valve respectively resulting in delayed treatment and unnecessary multiple procedures.
Case report:
Case 1: A 10-year-old boy presented with history of poor urinary stream and straining at micturition since birth. He had history of recurrent UTI. He was initially evaluated
3
in an outside center by an adult urologist who had got a voiding cystourethrogram (VCUG) and provisionally diagnosed as a case of anterior urethral stricture. He had undergone a cystoscopy and was found to have no “stricture” leading to the patient being labelled as having neurogenic bladder and advised to practice CIC. He presented to us for second opinion. On reevaluation of the old VCUG films we noted an open bladder neck with dilated prostatic and bulbar urethra with a sudden decrease in caliber at penobulbar junction with proximal dilatation in the distal bulb suggestive of anterior urethral valve (Fig 1A). Ultrasonography showed right moderate hydroureteronephrosis, thick-walled urinary bladder and high residual urine. Uroflowmetry showed a straining pattern suggestive of obstruction (Fig 1B). An endoscopic examination revealed an AUV extending from 3 to 9'o clock position in the penobulbar urethra along with a diverticulum proximal to the valve (Fig 2A, B). Back pressure changes were noted in the bladder. The valve was fulgurated at the 6'o clock position using a hook electrode in a pediatric resectoscope (Fig 2C). Postoperatively, the catheter was removed on post-operative day 2 and he had marked improvement in flow. At 3 months follow-up, VCUG confirmed a marked decrease in the diameter of proximal bulbar urethra (Fig 3A) and a good improvement of flow with Qmax of 14ml/sec (Fig 3B).
Case 2: A 11-year-old boy presented with history of poor urinary stream, straining during micturition and recurrent urinary tract infection (UTI) since birth with an elevated creatinine of 1.12 mg/dl. He had been diagnosed with obstructive uropathy due to PUV with elevated creatinine of 1.34mg/dl and fulguration was done at the age of 2 years. In spite of valve fulguration, patient continued to have recurrent UTI and elevated creatinine, hence a vesicostomy had been done which led to decrease in
4
creatinine to 0.8mg/dl. Vesicostomy closure and right ureteric reimplant was done after a year. The patient however, continued to have poor urinary stream with nocturnal urinary incontinence with a creatinine is 1.12mg/dl. Ultrasonography shows bilateral hydroureteronephrosis and thickened bladder wall with high PVR. Uroflowmetry showed a straining pattern with Qmax of 5ml/sec. MCU showed a good capacity but trabeculated bladder with a dilated posterior urethra extending upto penobulbar junction. A cystopanendoscopy shows anterior urethral valve at penobulbar junction with no evidence of valves in posterior urethra. Patient underwent fulguration of valve at 6’O clock position. After surgery, patient voided with good urinary stream and although his creatinine continued to be high, it was stable.
Discussion: AUV are rare congenital entities which cause obstructive uropathy in the lower urinary tract of children and can be difficult to diagnose. Because of the rarity they are often missed by urologists who don’t anticipate it and sometimes the diagnosis is delayed and made in adolescence and adulthood.2 AUV can be located anywhere distal to the membranous urethra and, in terms of distribution, these valves are most common in the bulbar urethra (40%), and equally occurring in the penile urethra and the penobulbar junction (30%).3 Some cases also have been reported in the navicular fossa.4 AUV are congenital mucosal folds that arise during micturition and flatten against the urethral roof, constituting an obstruction that can be severe. The etiology is still controversial, but faulty union of the glandular and penile urethral segments,
5
incomplete formation of the corpus spongiosum, and congenital cystic dilatation of the periurethral glands have been proposed as possible mechanisms.5
The anterior urethral valve is often found in association with an anterior urethral diverticulum. Anterior urethral diverticula communicate with the urethra and are found on the ventral aspect of the urethra between the bulbous and mid-penile urethra. Little consensus exists on the usage of the terms anterior urethral valve and diverticulum. It has been proposed that valves cause proximal urethral dilatation with the formation of a saccular diverticulum. Conversely, progressive enlargement of a diverticulum can result in a distal valve-like flap. While some authors distinguish between anterior urethral valves and diverticula as separate entities, others consider them as just variations of a single entity.6,7
The clinical presentation of AUV is highly variable, depending on age of patient and the degree of obstruction. Unless suspected, diagnosis can be difficult, hence AUV may escape diagnosis until adulthood. The common presenting complaints include difficulty in voiding, dribbling on micturition, incontinence, poor urinary stream and recurrent UTI.8 In the neonatal period and infancy, AUV may cause severe obstruction
resulting
in
megacystis,
bladder
rupture,
bilateral
severe
hydroureteronephrosis, azotemia and urinary ascites.9 In one-third of patients there is VUR, and in half of them upper tract damage is present .10 If not diagnosed early, they can lead to irreversible renal damage as highlighted in our second case who ended up with chronic kidney disease.
6
VCUG remains the most important imaging technique for the identification of these valves .11 Typically, the urethra appears dilated proximal to the valve and narrow distal to it. VCUG also allows the identification of VUR or an associated diverticulum.8 Cystoscopy may be less accurate, because it is easy to miss the flap valve if it is not looked for specifically. Recognition of the valve may be difficult because retrograde flow during urethroscopy flattens the valve against the urethral wall.12 Typically, the valve appears as a filmy, ventrally located cusp-like or semilunar flap of tissue, or a circumferential iris-like membrane.7,9 Both of our patients had cusp like valves.
Treatment varies according to age of presentation, extent of upper tract damage, extent of anterior urethral deformity. 3
In premature or small infant, vesicostomy
may be required to facilitate relief of obstruction until the infant can accommodate a cystoscope or undergo further reconstruction. 13 In majority of cases, transurethral valve ablation is the initial treatment. 10 In severe cases in which a gross urethral diverticulum is seen, transurethral or open diverticulectomy and urethral reconstruction required.7
To conclude, anterior urethral valves being a rare cause of obstructive uropathy in children can be missed by adult urologists unless specifically searched for. This case report highlights the need for awareness among adult urologists about a rare entity like AUV which can cause the same damage like a PUV and further by comparing two cases (one with an early and another with a late diagnosis) shows how if the condition is detected and treated early, irreversible damage to the kidneys can be averted.
7
References : 1. Paulhac P, Fourcade L, Lesaux N, et al. Anterior urethral valves and diverticula. BJU Int. 2003;92:506-509. 2. Khim A, Harris RD, Raffel J. Anterior urethral valve presenting in an adult male. Urol Radiol 1991;12:196-8. 3. Kibar Y, Coban H, Irkilata HC, et al. Anterior urethral valves: an uncommon cause of obstructive uropathy in children. J Pediatr Urol. 2007;3:350-353. 4. Scherz HC, Kaplan GW, Packer MG. Anterior urethral valves in the fossa navicularis in children. J Urol 1987;138:1211-3. 5. Firlit RS, Firlit CF, King LR. Obstructing anterior urethral valves in children. J Urol 1978;119:819-21. 6. Brueziere J, Guerrieri M. Congenital diverticula and valves of the anterior urethra. Ann Urol (Paris). 1985;19:101-107. 7. Keihani S, Kajbafzadeh AM. Concomitant anterior and posterior urethral valves: a comprehensive review of literature. Urology 2015;86:151-157. 8. Zia-ul-Miraj M. Anterior urethral valves: a rare cause of infravesical obstruction in children. J Pediatr Surg 2000;35:556-8. 9. Glazier DB, Underberg-Davis SJ, Cummings KB, Barone JG. Neonatal bladder rupture due to anterior urethral valves. Br J Urol 1997;80:819-20. 10. Van Savage JG, Khoury AE, McLorie GA, Bagli DJ. An algorithm for the management of anterior urethral valves. J Urol 1997; 158:1030-2. 11. Kajbafzadeh A. Congenital urethral anomalies in boys. Part II. Urol J. 2005;2:125-131.
8
12. Kajbafzadeh AM, Payabvash S, Karimian G. Urodynamic changes in patients with anterior urethral valves: before and after endoscopic valve ablation. J Pediatr Urol. 2007;3:295-300. 13. Rushton HG, Parrott TS, Woodard JR, Walther M. The role of vesicostomy in the management of anterior urethral valves in neonates and infants. Urol 1987;138:107-9.
9
Figure legends: Figure 1. A Preoperative VCUG showing the valve (black arrow) and the diverticulum (D). B Preoperative uroflowmetry showing interrupted flow.
10
Figure 2. Intraoperative pictures of the anterior urethral valve A. Pre-fulguration B. Post-fulguration
11
Figure 3. A Post-operative VCUG showing successful fulguration of valve. B Post-operative uroflowmetry showing good uninterrupted flow.
Anterior urethral valve- A commonly misdiagnosed entity in adolescent boys Dinesh Reddy , Ashwin Shekar P PII: DOI: Reference:
S0090-4295(20)30181-3 https://doi.org/10.1016/j.urology.2020.02.006 URL 21990
To appear in:
Urology
Received date: Revised date: Accepted date:
4 December 2019 30 January 2020 4 February 2020
Please cite this article as: Dinesh Reddy , Ashwin Shekar P , Anterior urethral valve- A commonly misdiagnosed entity in adolescent boys, Urology (2020), doi: https://doi.org/10.1016/j.urology.2020.02.006
This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2020 Published by Elsevier Inc.
1
Manuscript type: Pediatric case report Title: Anterior urethral valve- A commonly misdiagnosed entity in adolescent boys Authors: a
Dinesh Reddya, Ashwin Shekar. Pa*.
Department of Urology, Sri Sathya Sai Institute of Higher Medical Sciences,
Prashantigram, Puttaparthi, Andhra Pradesh 515134, India. *Corresponding Author-Ashwin Shekar P, Consultant Urologist, Department of Urology, Sri Sathya Sai Institute of Higher Medical Sciences, Prashantigram, Puttaparthi, Andhra Pradesh 515134, India. Ph no: +91-9940658701 Email address: [email protected] (Ashwin Shekar.P). Keywords: anterior urethral valve ,adolescent Acknowledgement: None Conflict of interest: None Funding: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
2
Abstract Anterior urethral valves are a rare cause of obstructed voiding in adolescent children and are often unheard by adult urologists. In this case report, we discuss the management of two adolescent patients who were referred to us with obstructive voiding symptoms with a diagnosis of neurogenic bladder and posterior urethral valves respectively but on evaluation were found to have anterior urethral valves. This article highlights the need for considering anterior urethral valve as an important differential diagnosis in children and adolescents presenting with obstructive voiding symptoms so as to avoid delay in diagnosis and management in young boys.
Introduction:
Urethral valves are a common cause of obstructive uropathy in boys. Though posterior urethral valves (PUV) are a more frequent cause, the obstructive effects of anterior urethral valves (AUV) can cause equal damage to the kidneys. AUV is 10-30 times less common than posterior urethral valve. 1 Due to its rarity, knowledge of this entity is quite less among practicing adult urologists. This article highlights two cases of AUV who were mismanaged by adult urologists as a case of neurogenic bladder and posterior urethral valve respectively resulting in delayed treatment and unnecessary multiple procedures.
Case report:
Case 1: A 10-year-old boy presented with history of poor urinary stream and straining at micturition since birth. He had history of recurrent UTI. He was initially evaluated
3
in an outside center by an adult urologist who had got a voiding cystourethrogram (VCUG) and provisionally diagnosed as a case of anterior urethral stricture. He had undergone a cystoscopy and was found to have no “stricture” leading to the patient being labelled as having neurogenic bladder and advised to practice CIC. He presented to us for second opinion. On reevaluation of the old VCUG films we noted an open bladder neck with dilated prostatic and bulbar urethra with a sudden decrease in caliber at penobulbar junction with proximal dilatation in the distal bulb suggestive of anterior urethral valve (Fig 1A). Ultrasonography showed right moderate hydroureteronephrosis, thick-walled urinary bladder and high residual urine. Uroflowmetry showed a straining pattern suggestive of obstruction (Fig 1B). An endoscopic examination revealed an AUV extending from 3 to 9'o clock position in the penobulbar urethra along with a diverticulum proximal to the valve (Fig 2A, B). Back pressure changes were noted in the bladder. The valve was fulgurated at the 6'o clock position using a hook electrode in a pediatric resectoscope (Fig 2C). Postoperatively, the catheter was removed on post-operative day 2 and he had marked improvement in flow. At 3 months follow-up, VCUG confirmed a marked decrease in the diameter of proximal bulbar urethra (Fig 3A) and a good improvement of flow with Qmax of 14ml/sec (Fig 3B).
Case 2: A 11-year-old boy presented with history of poor urinary stream, straining during micturition and recurrent urinary tract infection (UTI) since birth with an elevated creatinine of 1.12 mg/dl. He had been diagnosed with obstructive uropathy due to PUV with elevated creatinine of 1.34mg/dl and fulguration was done at the age of 2 years. In spite of valve fulguration, patient continued to have recurrent UTI and elevated creatinine, hence a vesicostomy had been done which led to decrease in
4
creatinine to 0.8mg/dl. Vesicostomy closure and right ureteric reimplant was done after a year. The patient however, continued to have poor urinary stream with nocturnal urinary incontinence with a creatinine is 1.12mg/dl. Ultrasonography shows bilateral hydroureteronephrosis and thickened bladder wall with high PVR. Uroflowmetry showed a straining pattern with Qmax of 5ml/sec. MCU showed a good capacity but trabeculated bladder with a dilated posterior urethra extending upto penobulbar junction. A cystopanendoscopy shows anterior urethral valve at penobulbar junction with no evidence of valves in posterior urethra. Patient underwent fulguration of valve at 6’O clock position. After surgery, patient voided with good urinary stream and although his creatinine continued to be high, it was stable.
Discussion: AUV are rare congenital entities which cause obstructive uropathy in the lower urinary tract of children and can be difficult to diagnose. Because of the rarity they are often missed by urologists who don’t anticipate it and sometimes the diagnosis is delayed and made in adolescence and adulthood.2 AUV can be located anywhere distal to the membranous urethra and, in terms of distribution, these valves are most common in the bulbar urethra (40%), and equally occurring in the penile urethra and the penobulbar junction (30%).3 Some cases also have been reported in the navicular fossa.4 AUV are congenital mucosal folds that arise during micturition and flatten against the urethral roof, constituting an obstruction that can be severe. The etiology is still controversial, but faulty union of the glandular and penile urethral segments,
5
incomplete formation of the corpus spongiosum, and congenital cystic dilatation of the periurethral glands have been proposed as possible mechanisms.5
The anterior urethral valve is often found in association with an anterior urethral diverticulum. Anterior urethral diverticula communicate with the urethra and are found on the ventral aspect of the urethra between the bulbous and mid-penile urethra. Little consensus exists on the usage of the terms anterior urethral valve and diverticulum. It has been proposed that valves cause proximal urethral dilatation with the formation of a saccular diverticulum. Conversely, progressive enlargement of a diverticulum can result in a distal valve-like flap. While some authors distinguish between anterior urethral valves and diverticula as separate entities, others consider them as just variations of a single entity.6,7
The clinical presentation of AUV is highly variable, depending on age of patient and the degree of obstruction. Unless suspected, diagnosis can be difficult, hence AUV may escape diagnosis until adulthood. The common presenting complaints include difficulty in voiding, dribbling on micturition, incontinence, poor urinary stream and recurrent UTI.8 In the neonatal period and infancy, AUV may cause severe obstruction
resulting
in
megacystis,
bladder
rupture,
bilateral
severe
hydroureteronephrosis, azotemia and urinary ascites.9 In one-third of patients there is VUR, and in half of them upper tract damage is present .10 If not diagnosed early, they can lead to irreversible renal damage as highlighted in our second case who ended up with chronic kidney disease.
6
VCUG remains the most important imaging technique for the identification of these valves .11 Typically, the urethra appears dilated proximal to the valve and narrow distal to it. VCUG also allows the identification of VUR or an associated diverticulum.8 Cystoscopy may be less accurate, because it is easy to miss the flap valve if it is not looked for specifically. Recognition of the valve may be difficult because retrograde flow during urethroscopy flattens the valve against the urethral wall.12 Typically, the valve appears as a filmy, ventrally located cusp-like or semilunar flap of tissue, or a circumferential iris-like membrane.7,9 Both of our patients had cusp like valves.
Treatment varies according to age of presentation, extent of upper tract damage, extent of anterior urethral deformity. 3
In premature or small infant, vesicostomy
may be required to facilitate relief of obstruction until the infant can accommodate a cystoscope or undergo further reconstruction. 13 In majority of cases, transurethral valve ablation is the initial treatment. 10 In severe cases in which a gross urethral diverticulum is seen, transurethral or open diverticulectomy and urethral reconstruction required.7
To conclude, anterior urethral valves being a rare cause of obstructive uropathy in children can be missed by adult urologists unless specifically searched for. This case report highlights the need for awareness among adult urologists about a rare entity like AUV which can cause the same damage like a PUV and further by comparing two cases (one with an early and another with a late diagnosis) shows how if the condition is detected and treated early, irreversible damage to the kidneys can be averted.
7
References : 1. Paulhac P, Fourcade L, Lesaux N, et al. Anterior urethral valves and diverticula. BJU Int. 2003;92:506-509. 2. Khim A, Harris RD, Raffel J. Anterior urethral valve presenting in an adult male. Urol Radiol 1991;12:196-8. 3. Kibar Y, Coban H, Irkilata HC, et al. Anterior urethral valves: an uncommon cause of obstructive uropathy in children. J Pediatr Urol. 2007;3:350-353. 4. Scherz HC, Kaplan GW, Packer MG. Anterior urethral valves in the fossa navicularis in children. J Urol 1987;138:1211-3. 5. Firlit RS, Firlit CF, King LR. Obstructing anterior urethral valves in children. J Urol 1978;119:819-21. 6. Brueziere J, Guerrieri M. Congenital diverticula and valves of the anterior urethra. Ann Urol (Paris). 1985;19:101-107. 7. Keihani S, Kajbafzadeh AM. Concomitant anterior and posterior urethral valves: a comprehensive review of literature. Urology 2015;86:151-157. 8. Zia-ul-Miraj M. Anterior urethral valves: a rare cause of infravesical obstruction in children. J Pediatr Surg 2000;35:556-8. 9. Glazier DB, Underberg-Davis SJ, Cummings KB, Barone JG. Neonatal bladder rupture due to anterior urethral valves. Br J Urol 1997;80:819-20. 10. Van Savage JG, Khoury AE, McLorie GA, Bagli DJ. An algorithm for the management of anterior urethral valves. J Urol 1997; 158:1030-2. 11. Kajbafzadeh A. Congenital urethral anomalies in boys. Part II. Urol J. 2005;2:125-131.
8
12. Kajbafzadeh AM, Payabvash S, Karimian G. Urodynamic changes in patients with anterior urethral valves: before and after endoscopic valve ablation. J Pediatr Urol. 2007;3:295-300. 13. Rushton HG, Parrott TS, Woodard JR, Walther M. The role of vesicostomy in the management of anterior urethral valves in neonates and infants. Urol 1987;138:107-9.
9
Figure legends: Figure 1. A Preoperative VCUG showing the valve (black arrow) and the diverticulum (D). B Preoperative uroflowmetry showing interrupted flow.
10
Figure 2. Intraoperative pictures of the anterior urethral valve A. Pre-fulguration B. Post-fulguration
11
Figure 3. A Post-operative VCUG showing successful fulguration of valve. B Post-operative uroflowmetry showing good uninterrupted flow.