Pediatric Case Report Anterior Urethral Valve Associated With Posterior Urethral Valves: Report of 2 Cases and Review of the Literature Christine N. Tran, Chad A. Reichard, Daniel McMahon, and Audrey Rhee Anterior urethral valve (AUV) associated with posterior urethral valves (PUVs) is an extremely rare congenital urologic anomaly resulting in lower urinary tract obstruction. We present our experience with 2 children with concomitant AUV and PUV as well as a literature review. The clinical presentation of concomitant AUV and PUV is variable. Successful endoscopic management can result in improvement in renal function, reversal of obstructive changes, and improvement or resolution of voiding dysfunction. UROLOGY 84: 469e471, 2014. 2014 Elsevier Inc.
CASE 1
A
male infant was born at 38 5/7 weeks gestation via vaginal delivery to a 28-year-old primigravida. Prenatal ultrasound demonstrated bilateral hydronephrosis. Amniotic fluid index was normal. Physical examination was notable for left undescended testicle but otherwise normal external genitalia. The infant was started on antibiotic prophylaxis, and a foley catheter was placed. Renal/bladder ultrasound obtained on day of life (DOL) 0 demonstrated severe left and mild right hydroureteronephrosis, as well as significant renal size discrepancy concerning for an atrophic right kidney. Voiding cystourethrogram (VCUG) revealed a small trabeculated bladder with an urachal remnant, a very dilated posterior urethra consistent with posterior urethral valves (PUV), normal anterior urethra, and bilateral grade V vesicoureteral reflux (VUR; Fig. 1). The infant remained in the neonatal intensive care unit. His serum creatinine plateaued at 0.49 mg/dL by DOL 5. On DOL 6, the decision was made to proceed with operative intervention, and the patient was taken to the operating room for cystoscopy and transurethral resection of urethral valves. Cystourethroscopy was performed with an 8.5F cystoscope. A circumferential diaphragm-type membrane was encountered in the bulbar urethra, which represented an anterior urethral valve (AUV; Supplementary Fig. 1). Type 1 PUV was then encountered, as well as a dilated posterior urethra and high bladder neck. The AUV was incised at the 9, 12, and 3 o’clock positions with hook electrocautery. The Financial Disclosure: The authors declare that they have no relevant financial interests. From the Glickman Urological & Kidney Institute, Cleveland Clinic Foundation, Cleveland, OH Reprint requests: Chad A. Reichard, M.D., Glickman Urological & Kidney Institute, Cleveland Clinic, Desk Q10-1, 9500 Euclid Avenue, Cleveland, OH 44195. E-mail: [email protected] Submitted: March 5, 2014, accepted (with revisions): April 21, 2014
ª 2014 Elsevier Inc. All Rights Reserved
PUV was incised at 5 and 7 o’clock in a similar fashion. The urethra appeared widely patent at the conclusion of the operation and an 8F catheter was placed and secured. VCUG performed on post-operative day 4 demonstrated good bladder emptying without significant postvoid residual; however, the bilateral grade V VUR persisted. The catheter was discontinued. The patient continued to have adequate urine output and renal function stabilized at creatinine 0.4 mg/dL. The infant was discharged home at 2 weeks of age in stable condition. He was seen for follow-up at 6 weeks old. Renal ultrasound showed an atrophic right kidney and improved left hydroureteronephrosis with normal appearance of the renal parenchyma. Renal function had improved further to creatinine 0.32 mg/dL.
CASE 2 A male infant was born full-term. Prenatal history was notable for a 2-vessel umbilical cord and bilateral mild hydronephrosis with anterior-posterior diameter of 45 mm. Postnatal renal/bladder ultrasound showed bilateral pelviectasis. VCUG at 2 months of age showed bilateral grade V VUR; however, the posterior urethra was not well visualized. Physical examination revealed a sacral dimple but spinal ultrasound was negative for spinal abnormalities. Renal function was normal. Urodynamics showed high-pressure contractions starting at 10 mL, with leakage at 60-cm H2O. Repeat ultrasound at 3 months showed resolution of renal dilatation. He was started on antibiotic prophylaxis with no breakthrough urinary tract infections. On follow-up at age 3, his parents reported normal voiding. A repeat VCUG showed bilateral grade III VUR as well as a dilated posterior urethra concerning for PUV with a change in caliber at the bulbomembranous junction and urachal remnant (Supplementary Fig. 2). VCUG may not have demonstrated AUV (ie, dilation of anterior urethra) because of a poor urinary http://dx.doi.org/10.1016/j.urology.2014.04.034 0090-4295/14
469
Figure 1. Case 1: VCUG revealed a small trabeculated bladder with an urachal remnant, a very dilated posterior urethra, and bilateral grade V VUR. VCUG, voiding cystourethrogram; VUR, vesicoureteral reflux.
Figure 2. Case 2: Cystourethroscopy demonstrated a narrowed concentric ring in the bulbar urethra representing an AUV as well as a type 1 PUV extending from the verumontanum. AUV, anterior urethral valve; PUV, posterior urethral valve. (Color vesion available online.)
stream distal to the PUV, which was similarly described in a prior reported case.1 Cystourethroscopy was notable for a 50% narrowed concentric ring in the bulbar urethra representing an AUV as well as a type 1 PUV extending from the verumontanum (Fig. 2). The PUV did not appear to be obstructing but did extend into the urethral lumen. The AUV and PUV were resected with cold hook knife. The patient did well on follow-up.
COMMENT For many years, PUVs and the bladder neck were generally considered to be the key sites of lower urinary tract obstruction in boys.2 Although PUV remains the most common cause of congenital obstructive uropathy, more prevalent use of VCUG allowed for the more frequent recognition of anterior urethral pathology. AUVs are an uncommon cause of obstructive uropathy. They are reported to be 7 times less common than the incidence of PUVs. However, the obstructive sequelae of AUV may be equally devastating. The association of these 2 anomalies is extremely rare. Only 5 such cases have previously been reported in the literature (Table 1).1,3-6 We present our experience with 2 children with concomitant AUV and PUV as well as a literature review. 470
The concomitant presence of AUV and PUV cannot be explained on an embryologic basis, as their development is different.6 The etiology of AUV is still unclear.1,7 Proposed theories include an abortive attempt at urethral duplication, failure of alignment between the proximal and distal urethra, imbalanced tissue growth in the developing urethra resulting in excess tissue that acts as a valve, congenital cystic dilation of the periurethral glands, and incomplete formation of the corpus spongiosum.7 AUV may be located anywhere distal to the membranous urethra. They are most commonly found in the bulbar urethra (40%) whereas 30% present at the penoscrotal junction and 30% at the pendulous urethra.6,8 The clinical presentation of concomitant AUV and PUV is variable.2-6 Affected individuals can be present in the neonatal period with changes on perinatal ultrasound suggestive of bladder outlet obstruction.3 Alternatively, patients may present later in infancy or childhood on workup for febrile urinary tract infection or for poor urinary stream with difficult voiding, dribbling on micturition, or urinary incontinence.2,4-6 The spectrum of severity is variable as well and ranges from mild urethral dilatation to bilateral hydronephrosis with renal insufficiency or, in extreme circumstance, even urinary ascites and bladder rupture.6,7 UROLOGY 84 (2), 2014
Table 1. Anterior urethral valve associated with posterior urethral valves N 1 Case
1 Case
1 Case
1 Case
1 Case
Presentation
Management
Five-d-old boy presented with poor urinary stream, crying with micturition, and palpable bladder. Renal function was normal, ultrasound with thickened bladder, and bilateral hydronephrosis. VCUG is suggestive of AUV and PUV and no reflux. Fifteen-d-old boy presented with stranguria. VCUG with left grade 5 VUR and dilated posterior urethra.
Cystoscopic AUV and PUV ablation with hook electrocautery. Normal renal function and resolution of hydronephrosis at 8-mo follow-up.
Rao et al
Cystoscopic PUV ablation. Post-op VCUG showed anterior urethral diverticulum for which a vesicostomy was performed. Repair of diverticulum at 6 mo of age, and left nephroureterectomy and vesicostomy closure at 9 mo of age. At 2 year follow up, Cr was 0.8mg/dL and voiding was satisfactory. Cystoscopic AUV and PUV ablation with Bugbee electrode. Perineal urethrostomy closed 1 y later. Follow-up at 5 y showed no obstruction and normal voiding.
Kumar et al
Cystoscopic valve ablation with electrocautery and subsequent Snodgrass hypospadias repair. Improved serum Cr to 0.6 mg/dL, improved hydronephrosis, and resolution of voiding symptoms on follow-up.
Bhagat et al
Cystoscopic valve ablation with hook electrocautery at 2 mo old and, again, at 5 mo old for residual valves. Uneventful post-op and 2-y follow-up.
Lassaad et al
Three-y-old boy presented with febrile UTI since age of 7 mo. Patient initially underwent bilateral ureteral reimplant at age of 8 mo for bilateral grade 3 VUR, and vesicostomy and, subsequently, perineal urethrostomy for inability to void. VCUG showed AUV and PUV. Eight-y-old boy presented with recurrent febrile UTI, continuous dribbling, and stranguria. Subcoronal hypospadias on examination. Serum Cr was 0.9 mg/dL. Ultrasound with bilateral hydroureteronephrosis and trabeculated bladder. VCUG and RUG with AUV and PUV and no reflux. Two-mo-old boy presented with febrile UTI and continuous dribbling. VCUG revealed concomitant AUV and PUV and no reflux.
Source
Kajbafzadeh et al
AUV, anterior urethral valve; Cr, creatinine; PUV, posterior urethral valve; RUG, retrograde urethrogram; UTI, urinary tract infection; VCUG, voiding cystourethrogram; VUR, vesicoureteral reflux.
Workup initially consists of assessment of renal function, renal bladder ultrasound, and VCUG. Typical findings on VCUG include a thickened trabeculated bladder, dilated or elongated posterior urethra, and dilatation of the anterior urethra proximal to the AUV. Obtaining a good quality VCUG is paramount as diagnosis has been delayed in the absence of adequate films, resulting in the treatment of obstructive consequences (eg, bladder neck plasty or ureteral reimplant) without addressing the initial problem.1,5 It is easy to miss an AUV on cystoscopy if not looked for specifically because retrograde flow flattens the valve against the urethral wall.7 On cystoscopy, an AUV tends to appear either as a circumferential iris-like membrane or a ventrally located cusp-like tissue flap. The management of AUV and PUV initially included open valve resection and reconstruction of the diverticulum. However, over the past 2 decades, endoscopic valve ablation has become the procedure of choice.2-6 Successful resection can result in improvement in renal function, reversal of obstructive changes, and improvement or resolution of voiding symptoms. If endoscopy or urethral catheterization is not possible in the unstable infant with febrile urinary tract infection, temporary diversion with vesicostomy is also a consideration. UROLOGY 84 (2), 2014
References 1. Kumar A, Bajpai M, Gupta AK. Double urethral obstruction in a neonate—a case report. Eur J Pediatr Surg. 2005;15:449-451. 2. Paulhac P, Fourcade L, Lesaux N, et al. Anterior urethral valves and diverticula. BJU Int. 2003;92:506-509. 3. Lassaad S, Rachida L, Manel BS, et al. Anterior and posterior urethral valves: a rare association. Open J Pediatr. 2011;1:34-36. 4. Rao KL, Eradi B, Menon P. Anterior and posterior urethral valves: a rare association. J Pediatr Surg. 2003;38:E23-E24. 5. Kajbafzadeh AM, Jangouk P, Ahmadi Yazdi C. Anterior urethral valve associated with posterior urethral valves. J Pediatr Urol. 2005;1: 433-435. 6. Bhagat SK, Gopalakrishnan G, Kekre NS, et al. Anterior and posterior urethral valves with subcoronal hypospadias: a rare association. J Pediatr Surg. 2008;43:e23-e25. 7. 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. 8. Karnak I, Senocak ME, B€uy€ukpamukc¸u N, et al. Rare congenital abnormalities of the anterior urethra. Pediatr Surg Int. 1997;12: 407-409.
APPENDIX SUPPLEMENTARY DATA
Supplementary data associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/j.urology. 2014.04.034. 471
CASE 1
A
male infant was born at 38 5/7 weeks gestation via vaginal delivery to a 28-year-old primigravida. Prenatal ultrasound demonstrated bilateral hydronephrosis. Amniotic fluid index was normal. Physical examination was notable for left undescended testicle but otherwise normal external genitalia. The infant was started on antibiotic prophylaxis, and a foley catheter was placed. Renal/bladder ultrasound obtained on day of life (DOL) 0 demonstrated severe left and mild right hydroureteronephrosis, as well as significant renal size discrepancy concerning for an atrophic right kidney. Voiding cystourethrogram (VCUG) revealed a small trabeculated bladder with an urachal remnant, a very dilated posterior urethra consistent with posterior urethral valves (PUV), normal anterior urethra, and bilateral grade V vesicoureteral reflux (VUR; Fig. 1). The infant remained in the neonatal intensive care unit. His serum creatinine plateaued at 0.49 mg/dL by DOL 5. On DOL 6, the decision was made to proceed with operative intervention, and the patient was taken to the operating room for cystoscopy and transurethral resection of urethral valves. Cystourethroscopy was performed with an 8.5F cystoscope. A circumferential diaphragm-type membrane was encountered in the bulbar urethra, which represented an anterior urethral valve (AUV; Supplementary Fig. 1). Type 1 PUV was then encountered, as well as a dilated posterior urethra and high bladder neck. The AUV was incised at the 9, 12, and 3 o’clock positions with hook electrocautery. The Financial Disclosure: The authors declare that they have no relevant financial interests. From the Glickman Urological & Kidney Institute, Cleveland Clinic Foundation, Cleveland, OH Reprint requests: Chad A. Reichard, M.D., Glickman Urological & Kidney Institute, Cleveland Clinic, Desk Q10-1, 9500 Euclid Avenue, Cleveland, OH 44195. E-mail: [email protected] Submitted: March 5, 2014, accepted (with revisions): April 21, 2014
ª 2014 Elsevier Inc. All Rights Reserved
PUV was incised at 5 and 7 o’clock in a similar fashion. The urethra appeared widely patent at the conclusion of the operation and an 8F catheter was placed and secured. VCUG performed on post-operative day 4 demonstrated good bladder emptying without significant postvoid residual; however, the bilateral grade V VUR persisted. The catheter was discontinued. The patient continued to have adequate urine output and renal function stabilized at creatinine 0.4 mg/dL. The infant was discharged home at 2 weeks of age in stable condition. He was seen for follow-up at 6 weeks old. Renal ultrasound showed an atrophic right kidney and improved left hydroureteronephrosis with normal appearance of the renal parenchyma. Renal function had improved further to creatinine 0.32 mg/dL.
CASE 2 A male infant was born full-term. Prenatal history was notable for a 2-vessel umbilical cord and bilateral mild hydronephrosis with anterior-posterior diameter of 45 mm. Postnatal renal/bladder ultrasound showed bilateral pelviectasis. VCUG at 2 months of age showed bilateral grade V VUR; however, the posterior urethra was not well visualized. Physical examination revealed a sacral dimple but spinal ultrasound was negative for spinal abnormalities. Renal function was normal. Urodynamics showed high-pressure contractions starting at 10 mL, with leakage at 60-cm H2O. Repeat ultrasound at 3 months showed resolution of renal dilatation. He was started on antibiotic prophylaxis with no breakthrough urinary tract infections. On follow-up at age 3, his parents reported normal voiding. A repeat VCUG showed bilateral grade III VUR as well as a dilated posterior urethra concerning for PUV with a change in caliber at the bulbomembranous junction and urachal remnant (Supplementary Fig. 2). VCUG may not have demonstrated AUV (ie, dilation of anterior urethra) because of a poor urinary http://dx.doi.org/10.1016/j.urology.2014.04.034 0090-4295/14
469
Figure 1. Case 1: VCUG revealed a small trabeculated bladder with an urachal remnant, a very dilated posterior urethra, and bilateral grade V VUR. VCUG, voiding cystourethrogram; VUR, vesicoureteral reflux.
Figure 2. Case 2: Cystourethroscopy demonstrated a narrowed concentric ring in the bulbar urethra representing an AUV as well as a type 1 PUV extending from the verumontanum. AUV, anterior urethral valve; PUV, posterior urethral valve. (Color vesion available online.)
stream distal to the PUV, which was similarly described in a prior reported case.1 Cystourethroscopy was notable for a 50% narrowed concentric ring in the bulbar urethra representing an AUV as well as a type 1 PUV extending from the verumontanum (Fig. 2). The PUV did not appear to be obstructing but did extend into the urethral lumen. The AUV and PUV were resected with cold hook knife. The patient did well on follow-up.
COMMENT For many years, PUVs and the bladder neck were generally considered to be the key sites of lower urinary tract obstruction in boys.2 Although PUV remains the most common cause of congenital obstructive uropathy, more prevalent use of VCUG allowed for the more frequent recognition of anterior urethral pathology. AUVs are an uncommon cause of obstructive uropathy. They are reported to be 7 times less common than the incidence of PUVs. However, the obstructive sequelae of AUV may be equally devastating. The association of these 2 anomalies is extremely rare. Only 5 such cases have previously been reported in the literature (Table 1).1,3-6 We present our experience with 2 children with concomitant AUV and PUV as well as a literature review. 470
The concomitant presence of AUV and PUV cannot be explained on an embryologic basis, as their development is different.6 The etiology of AUV is still unclear.1,7 Proposed theories include an abortive attempt at urethral duplication, failure of alignment between the proximal and distal urethra, imbalanced tissue growth in the developing urethra resulting in excess tissue that acts as a valve, congenital cystic dilation of the periurethral glands, and incomplete formation of the corpus spongiosum.7 AUV may be located anywhere distal to the membranous urethra. They are most commonly found in the bulbar urethra (40%) whereas 30% present at the penoscrotal junction and 30% at the pendulous urethra.6,8 The clinical presentation of concomitant AUV and PUV is variable.2-6 Affected individuals can be present in the neonatal period with changes on perinatal ultrasound suggestive of bladder outlet obstruction.3 Alternatively, patients may present later in infancy or childhood on workup for febrile urinary tract infection or for poor urinary stream with difficult voiding, dribbling on micturition, or urinary incontinence.2,4-6 The spectrum of severity is variable as well and ranges from mild urethral dilatation to bilateral hydronephrosis with renal insufficiency or, in extreme circumstance, even urinary ascites and bladder rupture.6,7 UROLOGY 84 (2), 2014
Table 1. Anterior urethral valve associated with posterior urethral valves N 1 Case
1 Case
1 Case
1 Case
1 Case
Presentation
Management
Five-d-old boy presented with poor urinary stream, crying with micturition, and palpable bladder. Renal function was normal, ultrasound with thickened bladder, and bilateral hydronephrosis. VCUG is suggestive of AUV and PUV and no reflux. Fifteen-d-old boy presented with stranguria. VCUG with left grade 5 VUR and dilated posterior urethra.
Cystoscopic AUV and PUV ablation with hook electrocautery. Normal renal function and resolution of hydronephrosis at 8-mo follow-up.
Rao et al
Cystoscopic PUV ablation. Post-op VCUG showed anterior urethral diverticulum for which a vesicostomy was performed. Repair of diverticulum at 6 mo of age, and left nephroureterectomy and vesicostomy closure at 9 mo of age. At 2 year follow up, Cr was 0.8mg/dL and voiding was satisfactory. Cystoscopic AUV and PUV ablation with Bugbee electrode. Perineal urethrostomy closed 1 y later. Follow-up at 5 y showed no obstruction and normal voiding.
Kumar et al
Cystoscopic valve ablation with electrocautery and subsequent Snodgrass hypospadias repair. Improved serum Cr to 0.6 mg/dL, improved hydronephrosis, and resolution of voiding symptoms on follow-up.
Bhagat et al
Cystoscopic valve ablation with hook electrocautery at 2 mo old and, again, at 5 mo old for residual valves. Uneventful post-op and 2-y follow-up.
Lassaad et al
Three-y-old boy presented with febrile UTI since age of 7 mo. Patient initially underwent bilateral ureteral reimplant at age of 8 mo for bilateral grade 3 VUR, and vesicostomy and, subsequently, perineal urethrostomy for inability to void. VCUG showed AUV and PUV. Eight-y-old boy presented with recurrent febrile UTI, continuous dribbling, and stranguria. Subcoronal hypospadias on examination. Serum Cr was 0.9 mg/dL. Ultrasound with bilateral hydroureteronephrosis and trabeculated bladder. VCUG and RUG with AUV and PUV and no reflux. Two-mo-old boy presented with febrile UTI and continuous dribbling. VCUG revealed concomitant AUV and PUV and no reflux.
Source
Kajbafzadeh et al
AUV, anterior urethral valve; Cr, creatinine; PUV, posterior urethral valve; RUG, retrograde urethrogram; UTI, urinary tract infection; VCUG, voiding cystourethrogram; VUR, vesicoureteral reflux.
Workup initially consists of assessment of renal function, renal bladder ultrasound, and VCUG. Typical findings on VCUG include a thickened trabeculated bladder, dilated or elongated posterior urethra, and dilatation of the anterior urethra proximal to the AUV. Obtaining a good quality VCUG is paramount as diagnosis has been delayed in the absence of adequate films, resulting in the treatment of obstructive consequences (eg, bladder neck plasty or ureteral reimplant) without addressing the initial problem.1,5 It is easy to miss an AUV on cystoscopy if not looked for specifically because retrograde flow flattens the valve against the urethral wall.7 On cystoscopy, an AUV tends to appear either as a circumferential iris-like membrane or a ventrally located cusp-like tissue flap. The management of AUV and PUV initially included open valve resection and reconstruction of the diverticulum. However, over the past 2 decades, endoscopic valve ablation has become the procedure of choice.2-6 Successful resection can result in improvement in renal function, reversal of obstructive changes, and improvement or resolution of voiding symptoms. If endoscopy or urethral catheterization is not possible in the unstable infant with febrile urinary tract infection, temporary diversion with vesicostomy is also a consideration. UROLOGY 84 (2), 2014
References 1. Kumar A, Bajpai M, Gupta AK. Double urethral obstruction in a neonate—a case report. Eur J Pediatr Surg. 2005;15:449-451. 2. Paulhac P, Fourcade L, Lesaux N, et al. Anterior urethral valves and diverticula. BJU Int. 2003;92:506-509. 3. Lassaad S, Rachida L, Manel BS, et al. Anterior and posterior urethral valves: a rare association. Open J Pediatr. 2011;1:34-36. 4. Rao KL, Eradi B, Menon P. Anterior and posterior urethral valves: a rare association. J Pediatr Surg. 2003;38:E23-E24. 5. Kajbafzadeh AM, Jangouk P, Ahmadi Yazdi C. Anterior urethral valve associated with posterior urethral valves. J Pediatr Urol. 2005;1: 433-435. 6. Bhagat SK, Gopalakrishnan G, Kekre NS, et al. Anterior and posterior urethral valves with subcoronal hypospadias: a rare association. J Pediatr Surg. 2008;43:e23-e25. 7. 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. 8. Karnak I, Senocak ME, B€uy€ukpamukc¸u N, et al. Rare congenital abnormalities of the anterior urethra. Pediatr Surg Int. 1997;12: 407-409.
APPENDIX SUPPLEMENTARY DATA
Supplementary data associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/j.urology. 2014.04.034. 471
