A Rare Association of Posterior Urethral Valve with Anterior Urethral Valve, Large Anterior Urethral Diverticula, and Glanular Hypospadias

Abstract

Background: Multiple urethral anomalies are rare with its own challenges in diagnosis and management. The double urethral obstructions are often missed as proximal obstruction can mask the early diagnosis of distal obstruction. We present a rare case of concomitant posterior urethral valve (PUV) and anterior urethral valve (AUV) with a large anterior urethral diverticula (AUD) and hypospadias.

Case Presentation: An 11-month-old male child after fulguration of PUVs continued to have urinary symptoms. He was subsequently found to have distal urethral obstruction because of AUV and large anterior urethral bulbar diverticula. He also had associated glanular hypospadias. He was managed with endoscopic ablation of AUV and diverticulectomy.

Conclusion: Concomitant PUV, AUV, AUD, and hypospadias is a rare occurrence. The diagnosis and management can be challenging in such cases with multiple anomalies. To avoid a misdiagnosis, a high index of suspicion, proper assessment of urethrogram, and detailed cystourethroscopy is required.

Introduction and Background

Posterior urethral valve (PUV) and anterior urethral valve (AUV) are two important causes of obstructive uropathy in children. AUV is in itself a rare entity being 25 to 30 times less common than PUV. Its association with a large anterior urethral diverticula (AUD) and more so with PUV and hypospadias is even rare.^1,2 The association of AUV and PUV has been rarely described in the literature and only about 22 cases have been reported in the literature.^1–3 This will be the fifth reported case in a child with multiple urethral anomalies of AUV, AUD, PUV, and hypospadias. We present the diagnostic and management challenges encountered in this rare case.

Case

An 11-month-old child presented with complaints of recurrent urinary tract infections and urinary dribbling. He was an antenatally diagnosed case of bilateral hydroureteronephrosis and was evaluated at his native place. Voiding cystourethrogram (VCUG) was suggestive of trabeculated bladder and dilated posterior urethra with thin urinary stream suggestive of bladder outlet obstruction secondary to PUV. Anterior urethra was not properly observed (Fig. 1). He underwent valve ablation at 1 month of age. After ablation, child continued to be symptomatic with recurrent urinary infections and urinary dribbling. At around 8 months of age, parents noted a large swelling on the ventral aspect of penis. When he presented to us, a repeat VCUG revealed a dilated posterior urethra and a new finding of large diverticulum at bulbar urethra (Fig. 2). The renal functions were normal. Cystourethroscopy revealed an additional finding of semilunar valve in the penile urethra arising from the ventral floor of urethra with proximal dilatation mimicking a small diverticulum. A large wide mouth diverticulum was present in the bulbar urethra about 2 to 3 cm proximal to classical valve. It was not related with AUV (Figs. 3, and 4). The posterior urethra was dilated with no residual valves. In addition, he also had a glanular hypospadias.

FIG. 1.

VCUG suggestive of trabeculated bladder, dilated posterior urethra, anterior urethra not observed. VCUG, voiding cystourethrogram.

FIG. 2.

VCUG image with anterior urethral diverticula not seen on previous VCUG.

FIG. 3.

(a, b) Endoscopic view of AUV and intraoperative picture of large AUD. AUD, anterior urethral diverticula; AUV, anterior urethral valve.

The valve could be easily engaged with resectoscope and incised at 6 o'clock position. The classical mucosal fold of valve arising from the ventral floor of urethra, which could be easily engaged, ruled out the possibility of any stricture caused by previous instrumentation. A large saccular diverticulum with wide neck of about 5 cm was excised (Fig. 4a, b) and urethra reconstructed with spongioplasty and the long suture line covered with tunica vaginalis flap. The patient is asymptomatic after 14 months of follow-up with a normal VCUG scan. He is now planned for MAGPI (meatal advancement and glanuloplasty) repair for glanular hypospadias.

FIG. 4.

Sketch diagram showing the urethral anatomy of the case.

Discussion

A combination of multiple urethral anomalies is extremely rare. After the first case of concomitant AUV and PUV reported by Graham et al. in 1982, little is known about the presentation, diagnosis, and management of valves causing double urethral obstruction.¹

A concomitant occurrence PUV and AUV 22% has been reported in a recent series of AUV.³

The association of AUV with proximal diverticula has been well described in the literature, with one third cases of AUV have been found to be associated with diverticula, although anatomically and embryologically their association has been difficult to explain. It is debatable whether they are the part of same pathology or are separate entities.¹ The best anatomical description is by Brueziere and Guerrieri⁴ who differentiates diverticula associated with valves as pseudodiverticula covered by corpus spongiosum with true diverticula that is formed outside the spongiosum. We believe in this case, semilunar AUV leaflet in penile urethra caused the formation of a small proximal pseudodiverticula and a more proximal spongiosal defect resulted in a large true diverticula formation (Fig. 3). As described in the literature, the classically associated AUV and AUD lie in close association where distal prominent lip of diverticulum acts similar to a valve causing urethral obstruction. In this case, AUD, which was 2 to 3 cm proximal to AUV, was not in close association with it and might represent an entirely separate pathology because of the large spongiosal defect.

To explain association of AUV/AUD various theories have been explained such as incomplete closure of urogenital folds, abortive attempt of urethral duplication, failure of alignment of anterior and posterior urethra, and cystic urethral glandular dilatation^1,2; the most appealing seems to be the incomplete migration of mesenchymal cells to form ventral spongiosa and leaving an unsupported area causing diverticula formation.¹ The association of AUV/AUD with PUV is exceedingly rare with not more than 22 cases being reported and association can be just a coincidence.¹ Considering various other associated urethral anomalies such as hypospadias, megalourethra with concomitant AUV and PUV, one proposed explanation is incomplete resorption of mesenchymal tissue in the anterior urethra and defective absorption of wolffian duct in posterior urethra.¹ The role of testosterone has also been proposed in view of association of multiple urethral anomalies with hypospadias.¹

The clinical presentation of this dual obstruction is similar to any other case of bladder outlet obstruction varying from mild to severe symptoms. The diagnosis and management in these patients can be challenging. As a result of proximal obstruction, the clinical presentation and findings of distal valves and diverticula gets masked and are often missed¹ on VCUG scan and cystourethroscopy, as also happened in our case. AUVs are tend to be missed on retrograde urethrography and urethroscopy as they might get flattened with retrograde flow. To avoid a misdiagnosis, a high index of suspicion with a good oblique view of VCUG scan delineating complete urethra and a careful urethroscopy looking specifically for valve is required. AUVs with or without small diverticula with prominent distal obstruction can be managed by endoscopic resection, whereas the large diverticula with spongiosal defects are best managed by diverticulectomy.^1,3

Conclusion

Concomitant AUV/AUD with PUV is rare and frequently missed anomaly. Awareness about the concomitant association and high index of suspicion is required especially in cases who remain symptomatic after valve ablation. This possibility of double urethral obstruction should always be kept in mind when proximal or distal obstruction is suspected and a careful diagnostic urethrocystoscopy will help in avoiding the missed diagnosis.

Abbreviations Used

AUD

anterior urethral diverticula

AUV

anterior urethral valve

PUV

posterior urethral valve

VCUG

voiding cystourethrogram

Disclosure Statement

No competing financial interests exist.

Funding Information

No funding was received for this article.

Cite this article as: Jain P, Prasad A, Jain S (2020) A rare association of posterior urethral valve with anterior urethral valve, large anterior urethral diverticula, and glanular hypospadias, Journal of Endourology Case Reports 6:4, 362–365, DOI: 10.1089/cren.2020.0138.