Ó Springer 2006
International Urology and Nephrology (2006) 38:163–165 DOI 10.1007/s11255-005-2947-5
Urethrocutaneous fistula: A case report Kadir Ceylan1, Burhan Ko¨seog˘lu2, O¨nder Tan3 & Bekir Atik3 1
Department of Urology, Faculty of Medicine, Yu¨zu¨ncu¨ Yıl University, 65100, Van, Turkey; 2Department of Pediatric surgery, Faculty of Medicine, Yu¨zu¨ncu¨ Yıl University, Van, Turkey; 3Department of Plastic Surgery, Faculty of Medicine, Yu¨zu¨ncu¨ Yıl University, Van, Turkey
Abstract. Background: A 16-year-old boy with penilurethra anomaly was admitted to our clinic. This is a case description of a hypospadiac congenital urethrocutaneous fistula of the anterior urethra. Methods/ Results: After the chordee was corrected by removing dysgenetic tissue in urethral plate, urethral defect was repaired with tranverse preputial island flap. Conclusions: Congenital urethrocutaneous fistula is rare. Thirty-two cases of urethrocutaneous fistula have been reported previously. Embryological events that cause anterior urethrocutaneous fistula are unclear. We reported a case with hypospadiac congenital urethrocutaneous fistula of the anterior urethra treated with tranverse preputial island flap. Key words: Penis, Urethra, Urethral plate, Urethrocutaneous fistula
Introduction Congenital urethrocutaneous fistula is a rare anomaly with only 32 cases previously reported [1]. The mechanism of urethrocutaneous fistula is a deficiency of the urethral plate and fusion of urethral folds. The authors should more clearly state that although the cause of this anomaly is not definitively known, there are several theories which have been proposed [2]. We report a case of hypospadiac congenital urethrocutaneous fistula of the penil urethra and review of the literature.
both the skin and urethra was located on the anterior surface of midshaft penis. Urethral chordee was weak. (Figure 1). Both testicles were in the scrotum. He was neither circumcised nor had an imperforate anus. There was neither trauma nor strangulation injury or infection history about the defective area. After the chordee was corrected by removing dysgenetic tissue in urethral plate, urethral defect was repaired with transverse preputial island flap. Postoperatively, the patient was voiding successfully and no recurrence of fistula.
Discussion Case report A 16-year-old boy with penil urethra anomaly was admitted to our clinic. His systemic physical examination was normal, but on his local examination a usual congenital anomaly of the midshaft urethra associated with intact distal and proximal urethral segment (with intact preputial, glans and glandular urethra) was observed. Prepisium and glans were normal and defect of 2.5 cm involving
In fetus, sexual differentiation and urethral development approximately begin in urethra at 8 weeks and are completed in 15 weeks. The urethra is formed by the fusion of the urethral folds along the ventral surface of the penis, which extends to the corona on the distal shaft [3]. The embryonic male urethra has three separate segments. The proximal portion was formed by the portion above the wolffian duct opening including
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Figure 1. Prepisium and glans were normal. The defect of urethra involved both the skin and the anterior surface of mid-shaft penis with a slight urethral chordee.
verumantanum utricle and urogenital sinus. The second enlarge forms the segment that extends from the verumantanum to the base of the glans. The glans segment is formed separately. Failure of fusion of urethral folds leads on to various levels of hypospadias. According to the level at which the lack of fusion occurs, urethral meatus can be localized anywhere from perineum to glans. The development of urethral tube distal to urethral meatus does not exist in all cases encountered [4]. The etiology of congenital urethrocutaneous fistula is not clear yet, but several hypotheses have been proposed [5]. Olbourne [6] suggested that the formation of a congenital urethral fistula result in arrested distal migration of the urethral plate or a localized deficiency of a portion of the plate, which prevents fusion of the urethral folds. The etiology is probably multifactorial, because of the varied appearance of the congenital fistula in the cases with and without hypospadias or a normal prepuce [1, 5]. The condition has been described as an unusual type of hypospadias [7] criptospadias [8] or congenital urethrocutaneous fistula [9]. Posterior congenital urethral fistula is most commonly seen in association with anorectal malformations, such as rectourethral, perineourethral fistula or imperforate anus [2]. Anterior urethrocutaneous fistula appears as two different types. An isolated type is associated with normal foreskin, no chordee and intact distal urethra and spongiosum. The other type is
associated with hypospadias-like characteristics [1]. The type congenital anterior urethral fistula is a rare anomaly [5]. There have been 32 cases reported in the literature. This condition may represent a form of congenital urethral fistula. Most of the cases reported have been of the hypospadias type with chordee [1]. However, we could not explain about certain etiologic forming of the second segment of the urethra which might be caused by some factors. In our case, intact distal urethral tube with chordee was observed. We also confirmed it by inserting a stile into the distal urethral tube (Figure 1). Our case has no anorectal malformations and associated with hypospadias-like characteristics. Surgical treatment of this pathology is easier than the other hypospadias type because of the isolated defect and intact distal urethral tube from the meatus. It was not necessary to reconstruct the whole urethral tube to the glandular tip. We prefer the transverse prepusial island flap to repair the defect, because the island flap maintains a stable well vascularized thick adequately durable tissue to cover the lesion but also, different methods, such as buccal mucosal graft, can be used as the same in large congenital urethral defect [10]. The dimensions of the defect were not allowed to be repaired by local tissues such as fistula repair. Due to the features of urethral defect and its surgical treatment, this case has been presented.
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Caldamone AA, Chen SC, Elder JS et al. Congenital anterior urethrocutaneous fistula. J Urol 1999; 162(11): 430–432. Harjai MM. Congenital urethrocutaneous fistula. Pediatr Surg Int 2000; 16: 386–387. McAninch JW, Disorders of the penis and male urethra, In Tanagho EA, McAninch JW: Smith’s General Urology, 14th edn, int. ed, Appleton and Lange, 1995; 658– 671. Duckett JW, Laurence SB. Hypospadias. In: Neill O’ JA, Rowe MI, Grosfeld JL, Ewand Coran AG (eds) Pediatric Surgery2 Mosby, Boston 1998, pp 1761–1781. Nakane A, Hayashi Y, Kojima Y et al. Congenital urethrocutaneous fistula. Int J Urol 2000; 7: 343–344. Olbourne NA. Congenital urethral fistula. Plast Reconstr Surg 1976; 57: 237–238.
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Address for correspondence: Kadir Ceylan, Department of Urology, Yu¨zu¨ncu¨ Yıl University, 65100 Van, Turkey Phone: +90-432-2164710-1151; Fax: +90-432-2167519 E-mail:
[email protected] 