African Journal of Paediatric Surgery

SYSTEMATIC REVIEW
Year
: 2018  |  Volume : 15  |  Issue : 2  |  Page : 63--68

Congenital anterior urethrocutaneous fistula: A systematic review


Yang Lin, Changkai Deng, Qiang Peng 
 Department of Pediatric Surgery, Chengdu Women's and Children's Central Hospital of Chongqing Medical University, Chengdu, China

Correspondence Address:
Dr. Changkai Deng
Department of Pediatric Surgery, Chengdu Women's and Children's Central Hospital of Chongqing Medical University, No. 1617, Riyue Avenue, Qinyang District, Chengdu
China

Abstract

Congenital anterior urethrocutaneous fistula (CAUF) is a rare anomaly characterized by fistulization of penile urethra to skin. It's usually seen as an isolated deformity or may accompany genitourinary or anorectal malformations. We aim to define the common properties of patients mentioned in literatures by systematic review. A comprehensive search of PubMed, Embase, Web of Science, and Cochrane Library was performed including cross-referencing independently by two assessors. Selections were restricted to human studies in English. Based on the systematic review, 63 patients in 34 articles were included in the study. Most common fistula site was subcoronal in 29 (46.0%) patients. Chordee was in 8 (14.5%) and associated genitourinary anomaly was detected in 19 (30.2%) of patients. Fistula recurrence ratio was 6/59 (11.3%) using different surgical techniques and 3/6 was closed spontaneously. CAUF is frequently located in subcoronal level and usually an intact urethra distal to it. Success rates are high with the principles of hypospadias surgery.



How to cite this article:
Lin Y, Deng C, Peng Q. Congenital anterior urethrocutaneous fistula: A systematic review.Afr J Paediatr Surg 2018;15:63-68


How to cite this URL:
Lin Y, Deng C, Peng Q. Congenital anterior urethrocutaneous fistula: A systematic review. Afr J Paediatr Surg [serial online] 2018 [cited 2019 Dec 7 ];15:63-68
Available from: http://www.afrjpaedsurg.org/text.asp?2018/15/2/63/261632


Full Text

 Introduction



Congenital anterior urethrocutaneous fistula (CAUF) is an uncommon anomaly of the penile urethra. It's usually seen as an isolated deformity or may accompany genitourinary or other malformations.[1],[2],[3] The cause is unclear but probably reflects a focal defect in the urethral plate that prevents fusion of the urethral folds.[4],[5] Over the past few decades, more and more cases were reported. However, the information gathered about this condition mostly comes from case reports and a few original articles with limited number of patients. To overcome the limitation of individual studies, we carried out this systematic review to provide a more precise and comprehensive properties of CAUF.

 Methods



Four databases (PubMed, Web of Science, Embase, and Cochrane Library) were electronically searched to retrieve studies on CAUF by September 10, 2017. Searching terms were (“urethral” AND “fistula” AND “congenital”) OR (“urethrocutaneous fistula” AND “congenital”). In additional, we evaluated all associated publications to identify the most eligible literature. Their reference lists were hand-searched to obtain other relevant publications. This systematic review was based on the preferred reporting items for systematic reviews and meta-analyses' guidelines.

Inclusion criteria and exclusion criteria

Titles and abstracts of all relevant papers were reviewed first. Then, full texts were reviewed as a second screening. The studies were considered eligible if they met all of the following criteria: (i) the study explored CAUF; (ii) when multiple publications reported on the same or overlapping data, the most recent article or the article based on the largest study population was selected; (iii) the publication language was English. Studies met any of the following exclusion criteria were excluded: (i) researches based on animals or cells rather than general population; (ii) reviews, editorials, meeting abstracts, and commentaries; and (iii) articles with no target data or no relevant outcomes.

Two reviewers (Changkai Deng and Yang Lin) reviewed all eligible publications independently according to the aforementioned inclusion and exclusion criteria; then, we extracted the relevant data in accordance with the preformed data extraction form. Disagreements were solved by discussion and a third party (Qiang Peng) was involved when necessary. The basic information was extracted from each article: first author, year of publication, country where study was conducted, sample size of case, history of circumcision, location of fistula, associated urinary system anomalies, associated other anomalies, type of surgical repair, number of recurrence, number of catheter days, and auxiliary examination.

 Results



We initially identified 996 potentially eligible studies. Most of them were excluded after the screening of titles and abstracts. The main excluded reason was duplication and irrelevant to CAUF. After assessing the full-text of 44 potentially relevant articles, we identified 34 eligible articles. The main reasons for exclusion were as follows: five studies were no relevant outcome, three studies were commentaries, and two studies reported on the same data [Figure 1].{Figure 1}

Thirty-four studies with 63 cases were included in the analysis. The basic characteristics of included studies are presented in [Table 1]. All studies were published between 1962 and 2017. The included studies were conducted in India, USA, UK, Turkey, France, Italy, and so on. Subcoronal fistula was detected in 29 patients, midpenile in 24, proximal penile to subcoronal level in 4, and penoscrotal in 1. Eleven cases were circumcised. Fistula recurrence ratio was 6/59 and 3/6 was closed spontaneously. Eight cases with chordee, 19 cases with associated genitourinary anomalies, and 11 cases with associated other anomalies. Complicated cases were 19 and isolated cases were 44.{Table 1}

 Discussion



CAUF is defined as a localized defect in penile urethra of congenital origin. It's usually seen as an isolated disorder or may accompany genitourinary or other penile anomalies. Limited information exists about this topic since it is a rare anomaly and the clinical characteristics are not properly defined. The etiology of CAUF is still obscure, and several pathogenetic theories have been used to explain its causes.[5],[6],[7] Olbourne suggested that fistulae located in the penile shaft probably reflect a focal or temporary defect in urethral plate function and this would result in a complete defect or a partial deficiency of urethral fold fusion.[7] Goldstein theorized that a transient deficiency in testicular evocator substance could produce congenital urethral fistula with chordee.[6] Karnak regarded congenital urethrocutaneous fistulas (excluding those associated with anorectal malformations) as one set of anomalies.[5]

Based on systematic review, 63 patients in 34 articles were included since 1962. Most of the articles report a few number of cases except 2 articles cover 14 and 9 cases, respectively.[8],[9] Subcoronal fistula was detected in 29 patients, midpenile in 24, proximal penile to subcoronal level in 4, and penoscrotal in 1. Subcoronal and midpenile locations seem to be typical for CAUF. Presentation of patients after circumcision raises the question whether this fistula might be acquired caused by the procedure or congenital and it becomes obvious after circumcision. Eleven of 63 patients reported in the literature were circumcised, and in some cases, the fistula is located proximal to prepuce making it unlikely to be iatrogenic.[4],[9],[10],[11],[12],[13]

Associated penile and urethral anomalies may be encountered in these patients as complicated cases. Eight patients with chordee and 19 with associated genitourinary anomalies were reported up to date with CAUF cases.[1],[6],[7],[8],[9],[13],[14],[15],[16],[17],[18],[19],[20] Meanwhile, associated other anomalies may be detected in patients with CAUF. Based on our systematic review, anomalies which may be noticed by physical examination such as undescended testes (n = 5), inguinal hernia (n = 2), penoscrotal transposition (n = 1), bifid scrotum (n = 1), duplicated urethra (n = 1), megalourethra (n = 2), anorectal malformation (n = 11), and congenital heart disease (n = 2) were detected in cases mentioned in the literature. This indicated that it's better to advise a through physical examination in all patients and these additional anomalies may necessitate different surgical techniques in fistula repair and make the procedure more complicated.[2]

The management of these anterior urethrocutaneous fistulae depends on the size and location. Small fistulas which are <0.5 cm can be easily closed primarily after refreshing the edges and covered by skin.[21] Fistulas of size >0.5 cm but <1 cm can be closed with turnover flap.[3],[6] Larger fistulas which are >1 cm can be closed by tubularized incised plate urethroplasty using the Thiersch-Duplay technique.[1],[9],[12],[20] Fistulae larger than 2 cm are associated with significant defect in urethra and skin cover which can be dealt with bilamellar preputial island flap.[22] Based on our systematic review, success rates are high with all the principles of hypospadias surgery. Fistula recurrence ratio was 6/59 (11.3%) using different surgical techniques and 3/6 (50.0%) was closed spontaneously.[2],[5],[7],[9],[10]

 Conclusion



This systematic review suggested that CAUF is chiefly located in subcoronal level and usually an intact urethra distal to it and treatment of this entity is personalized according to site of fistula, associated anomalies, and condition of the distal urethra. Success rates are high with all the principles of hypospadias surgery.

Financial support and sponsorship

Nil.

Conflicts of interest

Yang Lin and Changkai Deng contributed equally to this work.

References

1Gupta AK, Kumar M, Singh K, Sokhal AK. Rare association of congenital penile urethrocutaneous fistula with Y-type urethral duplication. BMJ Case Rep 2017;2017. pii: bcr2016217331.
2User IR, Karakus SC, Akçaer V, Özokutan BH, Ceylan H. Congenital anterior urethrocutaneous fistula: 3 new cases and review of literature. Arch Esp Urol 2016;69:238-43.
3Akman RY, Cam K, Akyuz O, Erol A. Isolated congenital urethrocutaneous fistula. Int J Urol 2005;12:417-8.
4Ritchey ML, Sinha A, Argueso L. Congenital fistula of the penile urethra. J Urol 1994;151:1061-2.
5Karnak I, Tanyel FC, Hiçsönmez A. Congenital urethrocutaneous fistula: A case report and literature review, with a nomenclature proposal. J Pediatr Surg 1995;30:1504-5.
6Goldstein M. Congenital urethral fistula with chordee. J Urol 1975;113:138-40.
7Olbourne NA. Congenital urethral fistula. Case reports. Plast Reconstr Surg 1976;57:237-9.
8Biswas S, Ghosh D, Das S. Congenital urethrocutaneous fistula-our experience with nine cases. Indian J Surg 2014;76:156-8.
9Caldamone AA, Chen SC, Elder JS, Ritchey ML, Diamond DA, Koyle MA, et al. Congenital anterior urethrocutaneous fistula. J Urol 1999;162:1430-2.
10Alhazmi HH. Congenital anterior urethrocutaneous fistula: Two case reports and review of literature. Urol Ann 2014;6:239-41.
11Jindal T, Kamal MR, Mandal SN, Karmakar D. Isolated congenital urethrocutaneous fistula of the anterior urethra. Korean J Urol 2011;52:368-70.
12Rashid KA, Kureel SN, Tandon RK. Congenital anterior penile isolated urethrocutaneous fistula: A case report. Afr J Paediatr Surg 2008;5:52-3.
13Shiraki IW. Congenital megalourethra with urethrocutaneous fistula following circumcision: A case report. J Urol 1973;109:723-6.
14Raj P, Birua H. Type V pouch colon, prune belly syndrome, and congenital anterior urethrocutaneous fistula. J Neonatal Surg 2017;6:38.
15Cheng SY, Chen SJ, Lai HS. Congenital anterior urethrocutaneous fistula at the penoscrotal junction with proximal penile megalourethra: A case report. J Radiol Case Rep 2016;10:33-7.
16Galinier P, Mouttalib S, Carfagna L, Vaysse P, Moscovici J. Congenital anterior urethrocutaneous fistula associated with a stenosis of the bulbar urethra in the context of high anorectal malformation without fistula. J Plast Reconstr Aesthet Surg 2009;62:e11-3.
17Ceylan K, Köseoǧlu B, Tan O, Atik B. Urethrocutaneous fistula: A case report. Int Urol Nephrol 2006;38:163-5.
18Merrot T, Pankevych T, Chaumoitre K, Alessandrini P. Congenital anterior urethrocutaneous fistula associated with urethral duplication. Pediatr Surg Int 2003;19:744-5.
19Nakane A, Hayashi Y, Kojima Y, Mizuno K, Okada A, Sasaki S, et al. Congenital urethrocutaneous fistula. Int J Urol 2000;7:343-4.
20Barwell J, Harris D. Case report: Congenital urethrocutaneous fistula. J Anat 1997;190(Pt 1):155-6.
21Shukla RM, Mukhopadhyay B, Mandal KC, Barman SS. Congenital penile urethrocutaneous fistula: A rare anomaly and review of literature. Urol Ann 2011;3:161-3.
22Islam MK. Congenital penile urethrocutaneous fistula. Indian J Pediatr 2001;68:785-6.