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ORIGINAL ARTICLE Table of Contents   
Year : 2009  |  Volume : 6  |  Issue : 2  |  Page : 102-105
Congenital anterior urethral valves and diverticula: Diagnosis and management in six cases


Department of Pediatric Surgery, CSM Medical University (Upgraded KGMC), Lucknow, Uttar Pradesh, India

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Date of Web Publication29-Jul-2009
 

   Abstract 

Background: Anterior urethral valves (AUVs) are rare congenital anomalies causing lower urinary tract obstruction in children. Although they are referred to as valves, these obstructive structures often occur in the form of a diverticulum. The urethra in these cases shows saccular or bulbar dilatation known as anterior urethral diverticulum (AUD). They typically occur where there is a defect in the corpus spongiosum, leaving a thin-walled urethra. This segment of the urethra balloons out during voiding, simulating a mass that is sometimes visible along the ventral wall of the penis. The swelling is fluctuant and urine dribbles from the meatus on compression. The present study highlights the clinical approach in identifying the condition and its treatment options, especially for those, presenting with urethral diverticula. Materials and Methods: We have studied children with congenital anterior urethral valves and diverticula. Six patients of AUVs with diverticula were admitted during the period of 2000-2007 and were prospectively evaluated. The mean age of presentation was 16 months (15 days to 4 years). Straining at micturition and a palpable penile swelling were the most common presenting features. The diagnosis was established by voiding cystourethrogram (VCUG) and supported by ultrasonography (USG). All patients were treated with single-stage open surgical excision except one who died preoperatively due to urosepsis. Initial lay opening of the penoscrotal urethra and delayed repair were done in one patient. Results: The surgical outcome was successful in all but one patient, who died of delayed presentation with severe back pressure changes, urinary ascitis and urosepsis. On long-term follow-up, all patients demonstrated good stream of urine. The renal functions were normal and the patients had no evidence of urinary infections. Conclusion: We propose that, the patients of AUVs, if not excessively delayed for treatment are otherwise well in terms of general condition as opposed to patients of posterior urethral valves. The diagnosis is easily established by VCUG and the severity is revealed by a sonogram. Open surgical excision is the method of choice for patients with a urethral diverticulum; however, cystoscopic fulguration is also feasible in selected patients. The outcome is excellent with minimal morbidity and mortality.

Keywords: Anterior urethral valves, congenital, open surgery, urethral diverticulum

How to cite this article:
Rawat J, Khan TR, Singh S, Maletha M, Kureel S. Congenital anterior urethral valves and diverticula: Diagnosis and management in six cases. Afr J Paediatr Surg 2009;6:102-5

How to cite this URL:
Rawat J, Khan TR, Singh S, Maletha M, Kureel S. Congenital anterior urethral valves and diverticula: Diagnosis and management in six cases. Afr J Paediatr Surg [serial online] 2009 [cited 2019 Nov 14];6:102-5. Available from: http://www.afrjpaedsurg.org/text.asp?2009/6/2/102/54773

   Introduction Top


Congenital anterior urethral valves (AUVs) are an uncommon condition, causing lower urinary tract obstruction in male urethra. They can occur as an isolated entity or in association with the proximal diverticulum, both of which probably represent the spectrum of disease. [1] It is suggested that the valves cause proximal dilatation that eventually results in a saccular diverticulum. [2] The diverticula typically occur where there is a defect in the corpus spongiosum, leaving a thin-walled urethra. The distal edge of the diverticulum forms a flap that obstructs the flow of the urine. Commonest in the penoscrotal region, approximately one-third is seen in the penile urethra.[3] The clinical presentation of AUVs is highly variable, depending on the age of the patient and the degree of obstruction. Unless suspected, the diagnosis at times may be difficult. The present discussion highlights the clinical approach in identifying this condition and its treatment options.


   Materials and Methods Top


Six patients with varied presentations were prospectively evaluated during the period of 2000-2007. The mean age of presentation was 16 months (15 days to 4 years). The patients presented with dribbling of urine, poor stream and ventral penile swelling. The swelling became more prominent while the patient was in the act of micturition. These patients were difficult to catheterize as the catheter got coiled in the anterior urethra, as described elsewhere. [5] There were no associated anomalies in any of the patients. The serum creatinine levels were normal in all patients but one, who had neonatal presentation with severe bilateral hydrouteronephrosis. Voiding cystourethrogram (VCUG) was diagnostic in all cases and confirmed the dilated urethra, proximal to the valve and narrow distal to it, or by a dilated urethra, ending in a smooth bulge. An urethrogram suggestive of meatal stricture in the presence of normal meatus was suggestive of a valve in fossa navicularis. In addition to defining the lesion in the urethra, VCUG also showed an associated diverticulum, and Vesico-ureteric reflux (VUR). Initial endoscopy was done in all cases preoperatively, to confirm the diagnosis which showed out-pouching on the ventral wall. All patients were operated by open surgical excision by a single surgeon. The operative procedure included an initial degloving of the penis up to its base by a circumferential sub-coronal incision. The urethra was tailored over an appropriate-sized catheter after the excision of the diverticulum, and reinforcement was done by the local dartos tissue. Excessive tissue was excised and the edges approximated. Initial proximal urethrostomy and delayed urethral repair were done in one patient because of the proximally located diverticulum (bulbar urethra). The clinical presentation, investigatory findings and surgical treatments of the patients are summarized in [Table 1].


   Results Top


The surgical outcome was successful in all patients except one, who died of severe back pressure changes, urinary ascitis and urosepsis. Postoperatively, urethral obstruction was relieved in all cases, as indicated by a good urinary stream. All patients remained symptom free and had normal renal functions at follow-up ranging up to 3 years. Serum creatinine levels were normal in follow-up and patients were continent and free of infections.Ultrasonography (USG) has showed a complete disappearance of hydrouteronephrosis within a period of 3-6 months. Only one patient had the evidence of urethral stenosis that was presented to us about 1 month back only, and is being planned for cystoscopy.


   Discussion Top


Congenital anterior urethral valve/diverticulum (AUV/AUD) is a rare condition capable of producing significant obstructive uropathy, if not recognized in time or left untreated. The first description of the condition was by reported by Watts in 1906, [4] ≠and the total number of descriptions in the literature is small. The embryological origin remains uncertain; various proposed aetiological mechanisms include an abortive attempt at urethral duplication; [6] failure of alignment between the proximal and distal urethra; [7] imbalanced tissue growth in the developing urethra resulting in excessive tissue remnant acting as a valve [7] and congenital cystic dilation of periurethral glands, resulting in a flap-like valve. [5] AUVs present with a spectrum of variable severity. Obstructive changes accompanying AUVs have been classified by Firlit, [2] as a continuity of pathology, who divided them into four basic types depending on the degree of urethral dilatation, presence of a diverticulum and the grade of upper tract dilatation.

The common presenting complaints include difficulty in voiding, dribbling on micturition, poor urinary stream or recurrent UTI. In the neonatal period or in infancy, AUVs may cause severe obstruction resulting in severe hydrouteronephrosis and azotaemia with urinary ascitis, and usually carries a bad prognosis. The same was true with one of our cases. Older children with minimal obstruction may present with enuresis, postvoid dribbling or failure to thrive.

Patients presenting with recurrent urinary tract infections and straining at micturition are first ordered a USG by the attending clinician. It is only after seeing the back pressure changes a suspicion arises and VCUG is ordered. At times, AUVs may escape diagnosis until adulthood. VCUG is the diagnostic investigation of choice for AUVs and shows dilatation of the anterior urethra in various forms. Saccular dilatation and uniform urethral dilation with a small flap-like valve were the common types observed in our patients [Figure 1] and [Figure 2]. In addition to defining a lesion in the urethra, VCUG may show an associated diverticulum, VUR or any other associated anomaly [Figure 3].

Various methods of treatment include open urethrotomy and excision of the valve, [8] segmental uretherectomy of the valve-bearing area along with a primary end-to-end anastomosis [9] and transurethral resection of the valves. Open excision may result in complications like extravasation of urine, stricture formation, and urethrocutaneous fistula, [10] fortunately not seen in any of our patients. Presence of a valve-like finding will make the option of cystoscopic fulguration feasible. In this manner, urethroscopy can be diagnostic as well as therapeutic. Success rate by cystoscopic fulguration is variable. Five of the 14 patients treated by cystourethroscopy required secondary surgical procedures in one of the large series reported. [11] It is believed that open primary reconstruction is better as the complete exposure of the diverticulum is possible and the surgical repair offers treatment in a single stage. [12]

The most commonly used surgical option is open excision of the valve/diverticulum with urethral repair. This can be accomplished as a single-stage procedure except in patients with deranged renal function, azotaemia and sepsis. In such instances, one can give a chance of prolonged catheter drainage and wait for the general condition to improve. Another feasible option is doing a perineal urethrostomy just proximal to the valve/diverticulum with delayed excision and repair. Suprapubic urinary diversion is another viable alternative. Few of the series reviewed used this as an initial treatment option for patients presenting with gross pyuria. [12] We performed open surgical excision in all the patients of our series [Figure 3] and [Figure 4], after excising the excess tissue, as open primary reconstruction was better providing a complete exposure of the diverticulum and the surgical repair offered treatment in a single stage. The surgical outcome was successful and was comparable to the reported literature.

Long-term follow-up in the patients is also good with milder grades of reflux resolved within 1-2 years of treatment. The back pressure changes to the upper tracts also underwent rapid resolution. A recurrence after surgical correction is not known.


   Conclusion Top


Although rare, AUVs must be considered in the differential diagnosis of lower urinary tract obstruction. These patients if not excessively delayed for treatment are otherwise well in terms of their general condition as opposed to the patients of posterior urethral valves. The diagnosis is easily established by VCUG and severity is revealed by a USG. Transurethral valve ablation with the help of a paediatric resectoscope is feasible in early cases; however, open excision and urethroplasty are indicated in the presence of severe urethral dilatation and deformity. The long-term prognosis is excellent.[Figure 5]

 
   References Top

1.Burstein JD, Firlit CF. Anterior urethra. In: Kelalis PP, King LR, Belman AB, editors. Clinical Pediatric Urology. Philadelphia:PA, WB Saunders; 1985. p. 558-61.  Back to cited text no. 1    
2.Firlit CF. Urethral abnormalities. Urol Clin North Am 1978;5:31-55,  Back to cited text no. 2  [PUBMED]  
3.Josep BD. Pediatric Urology. 2 nd ed. WB Saunders; 2001. p, 347.  Back to cited text no. 3    
4.Watts SH. Urethral Diverticulum in Male with Report of a Case. Johns Hopkins Hosp Rep 1906:13:48-89.  Back to cited text no. 4    
5.Anthony J.Castle. Posterior urethra valves and other urethral anomalies. 9 th ed. Campbell Walsh Urology; 2007. p. 3600.  Back to cited text no. 5    
6.Williams DI, Retik AB. Congenital valves and diverticula of theanterior urethra. Br J Urol 1969;41:228-30.  Back to cited text no. 6    
7.Karnak I, Senocak ME, BŁyŁkpamukηu N, Hiηsφnmez A. Rare congenitalabnormalities of the anterior urethra. Pediatr Surg Int 1997;12:407-9.  Back to cited text no. 7    
8.Yaxley RP. Congenital valves of the anterior urethra. Med J Aust 1969;2:295-7.  Back to cited text no. 8    
9.Golimbu M, Orca M, Al-Askari S, Morales P, Golimbu C. Anterior urethral valves. Urology 1978;12:343-6.  Back to cited text no. 9    
10.Rushton HG, Parrott TS, Woodard JR. The role of vesicostomy in the management of anterior urethral valves in neonates and infants. J Urol 19871;38:107-9.  Back to cited text no. 10    
11.Huang CJ, Bai JW, Liang RX, Sun N. Congenital anterior urethral valves and diverticula-analysis of 50 cases. Ann Acad Med Singapore 1989:18:665-8.  Back to cited text no. 11    
12.Gupta DK, Srinivas M. Congenital anterior urethral diverticulum in children. Pediatr Surg Int 2000:16:565-8.  Back to cited text no. 12    

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Correspondence Address:
Jiledar Rawat
Department of Pediatric Surgery, C. S. M. Medical University (Upgraded KGMC), Lucknow - 226 003, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0189-6725.54773

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
 
 
    Tables

  [Table 1]

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