female hypospadias with neurogenic bladder

Pediatr Surg Int (1995) 10:572-573 © Springer-Verlag 1995

R. Rav ichandran • V. Sripathi • S. Ramana than • N. Prasad

Female hypospadias with neurogenic bladder

Accepted: 16 December 1994

Abstract F e m a l e hypospadias is a rare anoma ly in wh ich the urethral o r i f ice is loca ted on the anter ior vagi- nal wal l d o s e to the introitus. The condi t ion is also assoc ia ted with a neurogen ic vo id ing disorder that is poor ly unders tood [3]. We repor t two chi ldren aged 3 and 9 years who were m a n a g e d with c lean in termi t tent cathe- ter iza t ion after a Mi t ro f ano f f opera- tion.

K e y w o r d s F e m a l e hypospadias • Urogen i t a l sinus • N e u r o g e n i c b ladder

Case reports

Case 1 A 3-year-old female was admitted for dribbling of urine since birth. During an episode of acute urinary retention a physician performed a suprapubic catheter diversion and referred the child to our hospital for further management. As the suprapubic catheter was not draining well, the child was sedated and the bladder catheterized through the urethra. The urethral orifice could be visualized well on the anterior vaginal wall 0.5 cm inside the introitus. Following 72 h of catheter drainage the serum creatinine (Cr) was noted to fall from 2.6 to 0.7 rag%. A urodynamic study

R. Ravichandran • V. Sripathi • S. Ramanathan • N. Prasad Second Surgical Unit, Department of Paedi- atric Surgery, Institute of Child Health & Hospital for Children, Halls Road Egmore, Madras - 600008, India

V. Sripathi ( ~ ) 1. Damodara Mudali Street, Panchavati, Chetput, Madras - 600 03 I, India

done with a 5 Fr catheter inserted suprapubi- cally showed a poorly-compliant, small-ca- pacity bladder with marked detrusor instabil- ity. Urinary flow studies and sphincter elec- tromyography could not be done as effective voiding could not be initiated. Ultrasound examination showed bilateral ureterohydrone- phrosis and voiding cystourethrography showed a neurogenic bladder with marked sacculation. No vesicoureteric reflux was noted. With the patient under general anesthe- sia a 10 Fr cystoscope was inserted into the bladder; the bladder mucosa showed marked trabeculation and the ureteric orifices could not be visualized clearly.

After discussion with the mother, a Mi- trofanoff procedure [2] was done. The appen- dix was isolated on its blood supply. The tip excised and implanted into the bladder above the right ureteric orifice. The cecal end was brought out in the right iliac fossa. Postopera- tively, 4-hourly clean intermittent catheteriza- tion (CIC) was instituted. To our surprise, the dribbling from the urethra ceased completely 1 week following institution of CIC. The Cr has remained at 0.7 rag% and the upper tracts showed good reduction in size at the end of 6 months; the child has also gained 2 kg body weight.

Case 2 A 9-year-old child was admitted for dribbling of urine since birth. Investigations revealed a female hypospadias with neuro- genic bladder. As the idea of a Mitrofanoff procedure was not well received by the par- ents, urethral tubularization was attempted. The tubularized segment became severely narrowed due to ischemia; CIC was thus abandoned. The child developed recurrent episodes of severe pyelonephritis and acute- on-chronic renal failure, which necessitated peritoneal dialysis. Two years later the parents and child requested that the Mitrofanoff pro- cedure be done. The appendix was implanted anterolaterally in the thick bladder wall in the manner of a Lich-Gregoir extravesical ure- teral reimplantation procedure [1], CIC was commenced in the postoperative period. The child stopped wetting and became infection-

free, but has chronic renal failure with Cr above 3 rag%.

Discussion

F e m a l e hypospadias represents the mi ldes t f o r m of a urogeni ta l sinus. The cause o f an associa ted neurogen ic b ladder o f the H i n m a n var ie ty [ 1 ] wi th upper- t ract changes is poor ly under- stood, as there is no obv ious obstruc- t ion to ur inary dra inage apart f rom a lack o f easy access ibi l i ty to the urethra f rom the vulva. The exac t inc idence o f neurogen ic b ladder in associa t ion wi th f emale hypospadias is not known.

The p rob lems in the m a n a g e m e n t o f these chi ldren are twofold . Firstly, regular CIC is necessary to p reven t upper- t ract deter iorat ion. The use o f the Mi t ro f ano f f p rocedure to p rov ide an easi ly access ib le s toma for CIC is w e l l - d o c u m e n t e d [5]. Regu la r CIC m a y reduce or even abol ish wet t ing, as in our two cases. Secondly, in sexu- al ly ac t ive adult females , a hypospa- diac urethra leads to recurrent ur inary infec t ions [6]. To a l lev ia te d i scomfor t due to the urethral syndrome, urethral mea ta l t ransposi t ion has been r ecom- mended . We be l i eve that this is ideal ly done at or after puberty. Prepuber ta l urethral tubular iza t ion for CIC is not f avored by us, as even m i n i m a l nar- r o w i n g of the recons t ruc ted urethra can lead to inabi l i ty to pe r fo rm CIC, as in our second case. I f upper- t ract changes and chronic renal insuffi- c i ency are to be avoided , the condi t ion

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needs to be recognized and corrected in infancy. In our second case, late referral and delay in instituting CIC led to irreversible renal insufficiency.

In a female child with a hypospa- diac urethral orifice, neurogenic void- ing dysfunction needs to be looked for and treated. The Mitrofanoff proce- dure, which provides an easily acces- sible stoma for CIC, would be ideal to preserve upper-tract function. Urethral meatal transposition can be done at or after puberty.

References

1. Allen TD (1977) The non-neurogenic neurogenic bladder. J Urol 117:232-238

2. Duckett JW, Snyder HM (1986) Use of the Mitrofanoff principle in urinary re- construction. Urol Clin North Am 13:271-274

3. Janskowski A, Schafer M (1990) Female hypospadias: a rare cloacal abnormality. Z Urol Nephrol 83:313-316

4. Marberger M, Altwein JE, Straub E et al. (1978) The Lich - Gregoir antireflux plasty: experiences with 371 children. J Urol 120:216-219

5. Merguerian PA, Mclorie GA (1992) Dis- orders of the female genitalia. In: Kelalis PE King LR, Belman BA (eds) Clinical pediatric urology, 3rd edn. WB Saunders, Philadelphia, pp 1084-1105

6. Van Bogaert LI (1992) Surgical repair of hypospadias in women with symptoms of urethral syndrome. J Urol 147: 1263- 1264