Congenital Penile Urethrocutaneous Fistula - Springer Link

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)ept. of Pediatric Surgery, Sylhet MAG Osmani Medical College, Bangladesh. ~.bstracL Congenital Penile Urethrocutaneous Fistula (CPUF) is an extremely rareĀ ...
Clinical Brief

Congenital Penile Urethrocutaneous Fistula rid. K a b i r u l Islam

)ept. of Pediatric Surgery, Sylhet MAG Osmani Medical College, Bangladesh. ~.bstracL Congenital Penile Urethrocutaneous Fistula (CPUF) is an extremely rare anomaly. Commonly congenital Jrethrocutaneous fistulae are associated with anorectal anomalies and urethral duplication. The posterior urethra s the part, which is mostly affected. To the best of our knowledge, only 11 such cases have been reported so far n the English literature. 1-7A case of CPUF with a short review of literature. indian J Pedlatr 2001; 68 (8) : 785-786] 9 ey words : Urethra; Congenital fistula; Urethrocutaneous fistula

CASE REPORT

3-year-old otherwise healthy boy presented to the Department of Paediatric Surgery, Sylhet MAG ~gsmani Medical College, with an abnormal opening on the ventral side of penis. The urethrocutaneotrs fistula was present since birth. There was no history of trauma, surgery, and stone impaction or strangulation injury to the penis. He used to pass urine mostly through the fistula and only a small amount through the normal external urethral meatus. On examination, the fistula was ovoid in shape, measuring 2 cm by 1 cm and was about 2 cm. distal to the penoscrotal junction. He was uncircumcised and had normal prepuce, glans and external urethral meatus. The urethra distal to the fistula was absolutely n o r m a l and was s u r r o u n d e d by n o r m a l c o r p u s ~!~..

spongiosum. There was no associated chordee (Fig. 1). Investigations like urinanalysis, intravenous urography and a b d o m i n a l u l t r a s o u n d were n o r m a l . Cystourethroscopy was not possible due to lack of facility at that period. The fistula was closed by repairing the urethral plate over an 8 Fr tube (Fig. 2). An a d d i t i o n a l layer of vascularized tissue was superimposed on the repair taking from the under surface of prepucial skin. The tube was kept in the urethra for 9 days without any u r i n a r y diversion. After removal, the fistula was completely closed. The child was healthy without any complain up to I year of follow-up.

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Fig. 2. A No. 8 Fr tube in the urethra

Fig. 1. The Congenital Urothrocutaneous Fistula Near penoscrotal junction.

DISCUSSION

Reprint requests : Prof. Md. Kabirul Islam, l/E, Surma Building, Old Medical Colony, Sylhet 3100, Bangladesh. Email: [email protected]

Congenital urethral fistula is considered as a spectrum of anomalies. The fistulae that run from the penile, scrotal or posterior urethra to the skin form the

Indian Journal of Pediatrics, Volume 68---August, 2001

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Kablrul Islam components of this spectrum. 3 The CPUF is the rarest form of this spectrum. Previously, this condition has been described as an u n u s u a l type of h y p o s p a d i a s , 2 Congenital u r e t h r o c u t a n e o u s fistula 3 and cryptospadiasP The penile urethra up to glans penis develops from e n d o d e r m a l cells of the u r e t h r a l plate. 7 Most of the urethra within the glans is developed from in g r o w t h of surface epithelial cells, which subsequently canalize to f o r m a tube. In the m e a n t i m e , the u r e t h r a l fold d e v e l o p s o n e i t h e r s i d e of the p l a t e , f o r m i n g the p r i m a r y u r e t h r a l g r o o v e . T h e d e e p e n i n g of this primary groove as well as canalization of the thickened urethral plate ultimately gives rise to the definitive urethral groove. Later on fusion of these urethral folds completes the formation of the urethra. 8 When a focal or temporary defect in urethral plate appears, it results in CPUF. 2.5 As the urethra in the glans and the prepuce are not derived from the urethral plate, they remain intact in this case. Some authors suggest that a CPUF m a y be a result of persisting pressures on the urethra by other parts of the body especially the lower limbs? Heyns ~~described a boy w h o was born with a deep-scarred impression on the p e n o s c r o t a l j u n c t i o n t h r o u g h w h i c h a testis prolapsed. Sharma 7 has also s u p p o r t e d the idea as a cause of these cases too. But in our case, the child had no abnormality in the lower limbs, which could give pressure in the urethra in fetal life. T h o u g h o u r case did not have a chordee, Goldstein ~ reported a case of CPUF with chordee. A n u m b e r of a u t h o r s h a v e d e s c r i b e d different methods of repairing CPUF. They include pedicle flap, 1

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p r e p u c i a l b o u n d skin flap, 3 m o d i f i e d Denis B r o w n u r e t h r o p l a s t y 6 or direct closure. 6 All of t h e m h a v e d i f f e r e n t merits a n d demerits. In o u r case w e p e r f o r m e d a direct closure, as it was easy and m o r e physiological. The extra layer of vascularized tissue from the inner surface of prepuce gave extra s u p p o r t and a very good result without any recurrence. REFERENCES

1. Goldstein M. Congenital urethral fistula with chordee. J Urol 1975; 113 : 138-140. 2. Gupta SC. An unusual type of hypospadias. Br J Plast Surg 1962; 15 : 191-193. 3. Karnak I, Tanyel CF, Hicsconmez A. Congenital urethro-cutaneous fistula : a case report and literature review with nomenclature proposal. ] Pediatr Surg 1995; 30 : 1504-1505. 4. Keppke EM et al. A rare congenital deformity of the external male genetalia : report of a case. Rev Lat Am Cir Plast 1968 : 12 : 210-211. 5. Menlen JC. Hypospadias and Cryptospadias. Br J Plast Surg 1971; 24 : 101-108. 6. Oulbourne NA. Congenital urethral fistula: case reports. Plast Reconstr Surg 1976; 57 : 237-239. 7. Sharma AK, Kothari SK, Goe] D, Chaturvedi V. Congenital urethral fistula. Peaditr Surg Int 2000; 16 : 4243. 8. Glenister TW. Origin and fate of the urethral plate in man. J Anat 1954; 88 : 413-425. 9. Cook WA, Stephens FD. Pathoembryology of the urinary tract. In King LR, ed. Urological Surgery in Neonates and Young Infants. Philadelphia; WB Saunders 1988; 123-124. 10. Heyns CF. Extrophy of the testis. J Uro11990; 144 : 724725.

Indian Journal of Pediatrics, Volume 68~August, 2001