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Background Congenital anterior urethral diverticulum (CAUD) is a rare condition in children. This condition can present at any age; however, it is more commonly identified in infants and older children. The patient may present with difficulty in micturition, dribbling of urine, poor urinary stream, or urinary tract infection. Children with large anterior urethral diverticulum with poor spongiosal support may also complain of cystic ventral penile swelling dur- ing micturition. Methods In this series, we report seven such cases of congenital anterior urethral diverticulum treated over a period of 12 years (2008–2020). All cases presented to the Pediatric Surgery Department with dysuria, dribbling of urine, recurrent urinary tract infection, and/or fluctuant ventral penile swelling. They were further evaluated with retrograde urethrography with micturating cysto-urethrogram and cysto-urethroscopy. Results Three cases had anterior urethral valves that were managed by cystoscopic fulguration of valves. Surpris- ingly, all these three cases had concomitant posterior urethral valves, while the other four cases presented with relatively larger and saccular anterior urethral diverticulum required excision of the diverticulum and primary urethral reconstruction. Conclusions The cases with congenital anterior urethral diverticulum secondary to anterior urethral valves may also be associated with posterior urethral valves as seen in our case series. Surgeons should be aware of this association, and both valves should be fulgurated in the same sitting. A larger and saccular anterior urethral diverticulum requires excision and urethroplasty as a definitive procedure. Keywords Urethral diverticulum, Congenital, Anterior urethral valves, Posterior urethral valves, Cysto-urethroscopy Background Congenital anterior urethral diverticulum (CAUD) is an uncommon condition in children. It is a rare cause of lower urinary tract obstruction and has been clas- sified into the saccular variety and globular variety. *Correspondence: It can be located anywhere on the anterior urethra Rajat Piplani but commonly seen at the distal bulbar and proxi- email@example.com Deepak Bagga mal penile urethra. The cause-and-effect relationship firstname.lastname@example.org between the anterior urethral diverticulum (AUD) and Department of Paediatric Surgery, All India Institute of Medical Sciences, anterior urethral valve (AUV) is still debatable. AUVs Uttarakhand 249203 Rishikesh, India Department of Pediatric Surgery, V.M.M.C & Safdarjang Hospital, are congenital semilunar obstructing folds arising from 110029 New Delhi, India © The Author(s) 2023. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http:// creat iveco mmons. org/ licen ses/ by/4. 0/. Piplani et al. Annals of Pediatric Surgery (2023) 19:9 Page 2 of 6 the ventral floor of the anterior urethra. It can occur Results as an isolated entity or in association with a proximal Out of the total seven cases, three cases were managed by diverticulum, probably representing a spectrum of the cystoscopic fulguration of the valves as all of them had con- disease . Some authors do not distinguish between comitant posterior urethral valves (PUV) along with ante- AUD and AUV, while others believe that these are dif- rior urethral valves. The remaining four cases with relatively ferent entities. The diverticulum that is associated large anterior urethral diverticulum required excision of the with AUV is not a true diverticulum because in AUD, diverticulum and primary urethral reconstruction. Only an acute angle is formed between the proximal part of one child (case no. 4) had back pressure changes on MCUG dilated portion and the ventral floor whereas this acute with left-sided vesico-ureteric reflux (VUR) associated sec - angle is not present in AUV. Congenital anterior ure- ondary to PUV (Table 1). Case no. 7 had urethra-cutaneous thral diverticulum may present itself at any age, from st fi ula and underwent repair after six months. However, all infant to adult . The diverticulum and valve cause cases recovered well with a good urinary stream. On fol- obstruction of the male urethra, and patient com- low-up of these cases, a post-operative MCUG was done in plaints of dysuria, dribbling of urine, recurrent urinary all patients at three months post-surgery. tract infection, or a fluctuant ventral penile swell- ing . The treatment options in AUD include endo- Discussion scopic division of the distal lip of the diverticulum , Congenital anterior urethral diverticulum (CAUD) is a excision of the diverticulum with primary repair , rare cause of bladder outlet obstruction in children. It is marsupialization with staged urethroplasty, or even usually located between the bulbous and the mid-penile suprapubic diversion followed by definitive repair . area. It is infrequent for an anterior urethral diverticu- Methods. lum to be in the distal urethra near the coronal level, This combined study was conducted at the Depart- as present in case numbers 5 and 7 of our series. Two ment of Pediatric Surgery at Safdarjang hospital, New varieties of CAUD have been described radiologically: Delhi, and All India Institute of Medical Sciences, saccular and globular . However, the embryology Rishikesh, and a total of seven cases of congenital ante- of AUD still remains uncertain, and various proposed rior urethral diverticulum were included over a period hypotheses have been studied. These include a develop - of 12 years (2008–2020). The study was exempted ment defect of the corpus spongiosum, cystic dilatation from the need to review by the Institutional review of the urethral glands, and sequestration of an epithelial committees in view of retrospective data analysis. All nest after the closure of the urethral folds. With a lack of cases presented with a history of dysuria, dribbling of a corpus spongiosum, a urethral dilatation in this region urine with recurrent urinary tract infection, and/or may develop into a diverticulum . Suter proposed fluctuant ventral penile swelling. These children with the theory that a diverticulum of the urethra develops ages ranging from 3 months to 10 years were further because of epidermal pockets communicating with the evaluated with retrograde urethrography (RGU) with ventral urethral wall. As the anterior urethral tube forms, micturating cysto-urethrogram (MCUG) and cysto- the urethral groove may leave behind epithelial cells that urethroscopy. Furthermore, a definitive surgery was form a congenital cyst. Cysts in this region developing a planned according to the radiological and cystoscopic communication with the urethra could lead to diverticu- findings, the presence or absence of urethral valves, lum formation as a result of the spontaneous rupture of and the size of the urethral diverticulum. the cyst into the urethral lumen . Table 1 Back pressure changes on MCUG with left-sided vesico-ureteric reflux ( VUR) associated secondary to PUV S.No. (age) CAUD on RGU CAUD on AUV PUV Associated Surgical procedure cystoscopy VUR Case 1 (7 years) + + + + – Cystoscopic valve fulguration/resection Case 2 (10 years) + + + + – Cystoscopic valve fulguration/resection Case 3 (3 years) + + – – – Excision of diverticulum and reconstructive urethroplasty Case 4 (3 months) + + + + + Cystoscopic valve fulguration/resection Case 5 (9 years) + + – – – Excision of diverticulum and reconstructive urethroplasty Case 6 (4 years) + + – – – Excision of diverticulum and reconstructive urethroplasty Case 7 (5 years) + + – – – Excision of diverticulum and reconstructive urethroplasty P iplani et al. Annals of Pediatric Surgery (2023) 19:9 Page 3 of 6 Most of these children with AUD present with difficulty penoscrotal mass clinically and the proximal lip radio- in micturition, dribbling of urine, poor urinary stream, logically which is seen as an arcuate filling defect should or urinary tract infection. The clinical presentation var - readily distinguish the diverticulum from the valve . In ies and depends on the age and the degree of obstruc- addition, the proximal lip forms an acute angle with the tion caused by the associated valves and diverticulum. A normal caliber proximal urethra in AUD, whilst in AUV, careful history will reveal that the child never had a good it forms an obtuse angle . MCUG has an additional urinary stream since birth, and a tell-tale sign is a cystic advantage of indicating proximal changes like megacys- swelling at the penile urethra . On examination, in an tis, VUR, or any other associated anomaly. VUR has been uninfected anterior urethral diverticulum without com- reported in 20% of patients with AUD . Ultrasonog- plications, the mass is usually unattached to the overlying raphy (USG) has a role that compliments the contrast skin, non-tender, and mobile laterally. While the urine studies to diagnose the condition. It has the advantage will be seen dribbling out of the external meatus on com- of evaluating the upper tracts as well. Moreover, voiding pression, the swelling tends to reduce in size. The diagno - USG has been found to be an alternative to contrast stud- sis of AUD is typically made by retrograde urethrogram ies in making a diagnosis of AUD . Cysto-urethroscopy [Fig. 1] or MCUG [Fig. 2]. The presence of a penile or is both diagnostic and therapeutic [Figs. 3 and 4]. A diver- ticulum typically appears as an outpouching from the ven- tral wall of the urethra and has a proximal and distal rim. In our series, we did cysto-urethroscopy in all the patients including children with CAUD. In one of the significant findings, we observed in these patients during urethros - copy is that a true anterior urethral diverticulum lightens up like a bulb through the defect in the spongiosa that Fig. 3 Cystoscopic view of the anterior urethral valve (case 2) and Fig. 1 RGU. a Case 1 with AUV. b Case 2 with AUV and small anterior resection of the valves done urethral diverticulum. c Case 3 with large saccular anterior urethral diverticulum at the distal and mid urethra. d Case 5 with diffuse anterior urethral diverticulum Fig. 2 MCUG. a Case 4 with AUV and PUV with dilated posterior urethra and left-sided VUR. b Case 5 with diffuse diverticular formation of the anterior urethra Piplani et al. Annals of Pediatric Surgery (2023) 19:9 Page 4 of 6 Fig. 4 Cysto-urethroscopy (case 7) showing “orange bulb” sign with large ventral urethral diverticula typically depicts like an “orange bulb” [Fig. 4]. Moreover, urethral urine. Given their non-obstructive nature, the cystoscopy should be done in all patients with obstructed MCUG is not associated with any proximal problem in symptoms and upper tract changes. the urinary tract as a direct consequence of its presence. Diverticula can be described as saccular or diffuse. The treatment entails a careful reduction of urethroplasty The saccular type is a localized protrusion from the followed in the long term by penile prosthesis insertion urethral lumen into the ventral wall of the anterior ure- for those with severe corporeal deficiency, whereas AUVs thra, and the diffuse type is a generalized dilatation of are known to be primarily obstructive in nature and not the entire anterior urethra. A congenital saccular diver- associated with corporeal deficiency. They cause imped - ticulum may produce anterior urethral obstruction by a ance to urinary flow across the urethra by their distal valve-like mechanism of its distal lip, which obscures the shelving semilunar lip that collects urine flowing ante - urethral lumen during filling [12, 13]. The presence of a gradely to the diverticulum which in turn expands and penile or penoscrotal mass clinically and radiologically, obstructs the urethral lumen. Their mode of presentation which is seen as an accurate filling defect, distinguishes is related to their obstructive nature across a spectrum the diverticulum from the valve. Also, the proximal lip involving antenatal pelvic dilatation, antenatal urinoma of the diverticulum forms an acute angle with the rest due to a ruptured bladder or calyceal fornix, or post- of the urethra, while the anterior valve forms the obtuse natally by repeated urinary tract infections, poor stream, angle [14, 15]. The primary differential diagnostic con - dysuria, deteriorating bladder function, and a worsening siderations include AUV, dilated Cowper’s gland ducts, upper tract later in childhood. The mainstay of treatment and posttraumatic diverticulum . In dilated Cowper’s involves endoscopic de-roofing or incision nullifying its gland ducts, a tubular channel is seen in the ventral sur- obstructive effect with very rarely a need to resort to sur - face of the bulbous urethra which it parallels. Its termina- gical excision as an upfront treatment. tion is in the urogenital diaphragm [14, 15]. The treatment of AUD depends on the degree of Despite being on the spectrum of mesenchymal obstruction, size of the diverticulum, and upper uri- defects, megalourethra and anterior urethral diverticula nary tract changes. Transurethral resection (TUR) with are not the same and require different management strat - a pediatric resectoscope is the treatment of choice for egies. Megalourethra are characterized by being primar- small, well-supported diverticula [Fig. 3] wherein the dis- ily non-obstructive, with the well-established associated tal obstructing lip is resected . But in the larger and deficiency of the corpus spongiosum (scaphoid variant) saccular variety of anterior urethral diverticula, as also in or deficiency of both the corpus spongiosum and one our cases, open diverticulectomy and primary repair are or both corpora cavernosa (fusiform variant). These are recommended [Fig. 5]. This approach may provide a more rarely seen as an isolated defect and are usually associated uniform caliber of the urethra, but there is a risk of ure- with other congenital pathologies of the paediatric uri- thra-cutaneous fistula formation . The technique of nary tract (e.g., Prune-Belly syndrome) or other systems making a triangular flap which is fitted into the distal lip, . Their mode of presentation is usually evident by and double breasting of the urethral suture line has also birth related to the evident swelling of the phallus along been described in the literature . Some authors have with dribbling and repeated infection of the stagnant also advocated the plication of the redundant diverticular P iplani et al. Annals of Pediatric Surgery (2023) 19:9 Page 5 of 6 Fig. 5 Case 3. a Preoperative—showing large saccular anterior urethral diverticulum. b Intraoperative—separation of the diverticulum from the surrounding skin. c Intraoperative—excision of the diverticulum. d Postoperative—after urethroplasty wall with good results . Furthermore, where there are Conclusions back-pressure changes of the upper tracts with deranged A history of poor urinary stream and dribbling, recur- renal function, urinary diversion either by marsupializa- rent UTI, and a ventral penile or penoscrotal swelling or tion of the diverticulum or even suprapubic cystostomy mass on examination strongly suggest the diagnosis of or vesicostomy [4, 6, 19] are better options. Moreover, CAUD. Micturating cysto-urethrogram and retrograde the prognosis in these patients will further depend on the urethrogram with full-length visualization of the urethra status of their upper tracts. will confirm the diagnosis. Both anterior and posterior CAUD has been associated with various urologic and urethral valves can co-exist but can be missed on initial non-urologic anomalies like vesico-ureteral reflux, ante - assessment. A meticulous cysto-urethroscopy should fol- rior urethral valve, penile torsion, patent ductus arterio- low for confirming the diagnosis and endoscopic ablation sus, and polydactyly. However, its association with the or resection of the valves for AUV and small but well- posterior urethral valve is a rare occurrence with very supported diverticula. However, in a large saccular and few cases that have been previously reported [20, 21]. diffuse CAUD, excision of the diverticulum and recon - Although the presence of persistent urinary obstruction structive urethroplasty is required. after posterior valve ablation usually suggests incomplete valve ablation, it is also good to bear in mind the rare Abbreviations obstructive defects in the anterior urethra as a differential CAUD Congenital anterior urethral diverticulum diagnosis in these patients . Concomitant PUV and AUD Anterior urethral diverticulum AUV Ant erior urethral valves AUD cases have characteristic micturating cystourethrog- PUV Posterior urethral valves raphy (MCUG) features of dilated posterior urethra, non- RGU Retrograde urethrography dilated membranous urethra, and dilated bulbar urethra MCUG Micturating cysto-urethrography VUR Vesico-ureteric reflux while retrograde urethrography better delineates the valve TUR Trans-urethral resection along with distal urethra dimensions . USG Ultrasonography Piplani et al. Annals of Pediatric Surgery (2023) 19:9 Page 6 of 6 Acknowledgements 14. Kirks DR, Grossman H. Congenital saccular anterior urethral diverticulum. None Radiology. 1981;140:367–72. 15. Kretschmer HL. Diverticula in the anterior urethra in male children. Surg Authors’ contributions Gynecol Obstet. 1936;62:634–40. The authors have read and approved the final manuscript. RP drafted the 16. Stephens FD, Fortune DW. Pathogenesis of megalourethra. J Urol. manuscript, contributed to the study design and interpretation of the data, 1993;149:1512–6. and assisted and operated the cases. SKA revised the manuscript and assisted 17. Paulhac P, Fourcade L, Lesaux N, Alain JL, Colombeau P. Anterior urethral in the study design and data compilation. DB operated the cases and edited valves and diverticula. BJU Int. 2003;92(5):506–9. and concluded the study. 18. Heaton BW, Snow BW, Cartwright PC. Repair of urethral diverticulum by plication. Urology. 1994;44:749–52. Funding 19. Rushton HG, Parrott TS, Woodard JR. The role of vesicostomy in the This research did not receive any specific grant from funding agencies in the management of anterior urethral valves in neonates and infants. J Urol. public, commercial, or not-for-profit sectors. 1987;138:107–9. 20. H. Abebe, H. Girma, F. Temesgen. “Congenital anterior urethral diver- Availability of data and materials ticula with posterior urethral valve: a rare combination, case report” The datasets with more images and patient data are available from the cor- Urol Case Rep. 2021;34:101447. responding author upon reasonable request. 21. Menon P, Rao KLN, Congenital anterior urethral valves and diverticulum: a case series and association with posterior urethral valves. Journal of Pediatric Urology 2022;18:p663.e1–663.e9. Declarations Ethics approval and consent to participate Publisher’s Note Not applicable. Springer Nature remains neutral with regard to jurisdictional claims in pub- lished maps and institutional affiliations. Consent for publication Written informed consent was obtained from the patient’s legal guardian for the publication of the case and any accompanying image. Competing interests The authors declare that they have no competing interests. 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Annals of Pediatric Surgery – Springer Journals
Published: Feb 25, 2023
Keywords: Urethral diverticulum; Congenital; Anterior urethral valves; Posterior urethral valves; Cysto-urethroscopy
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