Aboud, MM

Eshleman, JL

Mbwambo, JS

Mteta, KA

Oyieko, W

English

AJOL - African Journals Online

The urinary diversion in children with bladder exstrophy and epispadias: alternative to primary bladder closure. K A Mteta MMed J S Mb\irambo hIhled J L Eshleman ;\ID, ABU M M Aboud hIhIed W Oyieko hlhled Institute of Urolog!;IiChlC, P 0 Box 3010, hloshi,TANZANIA. Key words: Urinary diversion, children, bladder esstroph!; epispadias. The main objective of this study was to evaluate the outcome of management of bladder exstrophy and epispadias with continent urinary diversion. A total of 15 children, 10 females and 5 males underwent continent urinary diversion at Kilimanjaro Christian Medical Centre (KCMC) between 1985 and 1997. Their ages ranged between one month and 13 years with an average of 5.4 years. Eight (53%)of them had exstrophy epispadias complex, 4 (27%) had incontinent epispadias, 2 (13%) presented with neurological conditions and 1 (7%) had traumatic destruction of the bladder neck and urethra. Seven (47%) had Mainz pouch I1 procedure, 6 (40%) underwent the classical ureterosigmoidostomy while 2 (13%) had appendicovesicostomy. The mean duration of follow up was 3.2 years. Three patients developed mild non-progressive hydronephrosis, which required no surgical intervention. One patient with a solitary kidney developed ureteral stenosis at the implantation site that was nndivcrted. Metabolic acidosis was well compensated with none of the patients requiring sodium bicarbonate supplements. Only one patient had night soiling that required wearing of diapers. Our experience with continent urinary diversion in children with other benign bladder conditions has been favourable and in our view, it offers a viable treatment method in children with exstrophy epispadia complex. Introduction In children with epispadias-esstroph~l comples, the aim of management is to achieve urinary continence, preserve upper urinary tracts and provide adequate external genitalia. Despite the fact that various methods of treatment have been reported, the ultimate surgical procedure remains elusive or even debatable. Most urologists in the West prefer bladder closure with or without osteotomy in the first 24-72 hours of life and this is followed by bladder neck reconstruction and bilateral ureteroneoc~~stostomy with reconstruction of the external genitalia at 2 to 5 years. Most of the time additional procedures are ~-cc~uiycc\ to chic\-c contisicncc. Contincncc rate after these procedures has been reported to range from 32 to 80°/04. However, in most of these series patients are considered continent even if they are only capable of emptying their bladder by clean intermittent self-catheterisation, which in males can be complicated, by recurrent epididymitis and urethral stricture5. Primary bladder closure and subsequent bladder neck reconstruction has also been reported to be complicated by l~ydronephrosis, reflus and poor preservation of the upper urinary tracts6. In developing regions where the health budget is meagre and where patients have to travel long distances to reach referral hdspitals manned by consultants, which also makes follow-up of  patients difficult, it is not feasible to schedule patients for multiple staged and therefore espensive surgical procedures. It  is for those reasons that we decided to seek for simple and appliance free techniques of continent urinary &version rather than primary bladder closure. The rate o f  comple te  cont inence  after  ureterosigmoidostomy has been reported to be as high as 92.3%'. Since 1992, we have been using the sigma rectum pouch (Main2 pouch 11), a modification of ureterosigmoidostomy as our treatment of choice. For the paraplegic paediatric patients, our procedure o f  choice has been appendicovesicostomy with or  without bladder augmentation as these patients have insufficient anal sphincter tone. In this paper we report data obtained from children who underwent urinary diversion over a period of 12 years at IGlimanjaro Christian Medical Centre (ICCMC). Patients and methods Continent urinary diversion for non-malignant conditions at I<CMC during the period under review starting from 1983 were studied. Most of the patients were referrals from district and regional hospitals from all overTanzania. Fot tlic classical ureterosigmoidostomy,a midline laparotomy incision was used, the colon was opened antimesenterically and the ureters were tunnelled submucosal~r for antireflus mechanisms as described by Coffeys. For the sigma rectum pouch, after opening of the rectum and sigmoid for 10 to 12 cm proximal and dstal to the rectosigrnoid junction, the median margins were sutured.  Fixation o f  the rectosigmoid junction to the sacral promontory as well as parallel ureteral implantation guaranteed a straight ureteral path and prevented kinliing and the subsequent obstruction. T h e  use o f  the  appendix as a continent  vesicostomy (appendicovesicostomy) was first described by Mitrofanoff in 1 9809. A continent valvular mechanism was achieved with a submucosal tunnel of the mobhsed appendx into the bladder with the other end brought out to the skin as a catheterizable stoma. Postoperatively, the children were followed up by regular upper urinary tract imaging analysis of serum creatinine, blood urea nitrogen electrolytes and rate of bicarbonate usage sphincter control and the need for re-operation. In  our study, continence was defined as having regular normal interval use of toilet, voluntary control of the urinary stream and regular normal regular underclothes without diapers. Results During the period under review, a total of 15 children underwent continent urinary diversion for non-malignant conditions. There were eight cases of exstrophy epispadia complex, 4 of incontinent epispadias, 2 of neurogenid condtions and one of traumatic destruction of the bladder neck and urethra. There were 10 females and 5 males. Their age at diversion ranged between one montl~ ; ~ n d  13 >,cars \~ - i t h  a mc;m ;isc o f  -5.4 \.c;ll.s and a mean follow up of 3.2 pears (range of 1 to 13 years). Sigma-rectum pouch (Mainz pouch 11) was performed in 7 cases, 6 had classical ureterosigmoidostom~i while two patients had appendicovesicostom~~. Post-operatively, two patients developed superficial wound sepsis. N o  other early postoperative complications were recorded. Long-term complication UPPER URINAR Y TRACTS: All patients were followed up with intravenous urography and more recently by renal ultrasound. hlild unilateral, non-progressive hydronephrosis developed in three patients, none requiring sursical intervention. All patients had their serum creatinine and blood urea nitrogen within normal range. N o  cases of pyelonephritis or  urolithiasis were recorded. One female clild with incontinent epispadias who had undergone ureterosigmoidostom~~ developed stenosis at the ureteral implantation site. Tl i s  cMd had a solitary Iudney; she was undiverted and simultaneously underwent Marshal Marchet bladder neck reconstruction six months after the primary diversion. METABOLIC COMPLICATION: Patients had regular determinations of serum electrolytes (Na+, I<+, C1-) and recently C 0 2  levels. All patients were metabolically well compensated with none  requiring sodium bicarbonate supplements. CONTINENCE: All seven children who had sigma rectum pouch were fully continent .  A m o n g  the  six who  underwent ure teros igmoidostom~~,  one  had nighttime soiling requiring diapers. This was a female child who had exstrophy epispadias complex and was being considered for conversion to sigma rectum pouch. The two paraplegic patients who were done appen&covesicostomy were fully cont inent ,  doing in termi t tent  catheterisation through their stomas. N o  stoma complications had developed. Discussion I n  most  developed countries, the standard treatment for exstrophj~ epispadia complex is primary bladder closure during the neonatal period wi th  subsequent  bladder neck reconstruction at the age of 2 to 5 years. Often additional procedures are required to achieve continence. Despite these multiple procedures however, continence rate vary, ranging between 32 and 80%. Primary bladder closure and subsequent bladder neck reconstruction is not alwaos successful. The reason for the failure of primary bladder closure is the fact that creation of a functional sphincter mechanism is ultimately impossible. To achieve complete continence, further procedures beyond Young - Dees- Leadbetter bladder neck reconstruction are often necessary. These additional procedures include intestinal bladder augmentation, a pubovaginal sling or artificial sphincter implantation". In those cases where outlet resistance is high, clean intermittent self-catheterisation is required. This is an additional risk procedure especially for boys in whom it has been associated with increased rate of epididymitis, urethral stricture and even perforation of the augmented bladder5. Another hypothesis for failure of primary bladder closure may be insufficiency of the detrusor muscle itself. Hollowell et al" demonstrated urine leakage due to involuntary detrusor contractions in 10 out of 18 patients with poor continence. Normal bladder function during filling and contractile voiding was demonstrated in only 8% of their patients. They therefore concluded that < < evolution to completely normal lower urinary tract function after bladder neck reconstruction is unrealistic espectation for the majority of  children with esstrophy and severe epispadias." In  our series, metabolic complications were minimal and so were changes in the upper urinary tracts. Continence rate after sigma rectum pouch was 100% while after ureterosigmoidostomy, it was 83%; findings which were comparable with the continence rates of SO - 100% reported in literature from elsewhere. Since 1992, we have been performing sigma rec tum pouch in preference o the classical ureterosigmoidostomy. For the paraplegcs with insufficient anal sphincter unction, our procedure of  choice has been appendecovesicostomy. The overall continence rate among our 15 children was 93%. In conclusion, we have presented our 12 years' esperience with primary urinary diversion in children with esstrophy epispadias comples. A continence rate of more than 90% mas achieved with good preservation of upper tracts as well as adequate metabolic  compensat ion .  O u r  experience has therefore been most favourable and we conclude that continent urinary diversion is a viable alternative treatment method for children with bladder esstrophy epispad comples. References 1. Koo H P, Arollo L, Duckett J \V. Long-term results of ureterosigmoidostomy in children with bladder esstrophy. 2. Stein R, Fisch XI. Stockle hl, Hohenfellner R. Urinary diversion in bladder esstrophy and incontinent epispadias: 25 years experience, J Urol 1995; 154:1177. 3. Geahart JP, Jeff RD. Esstrophy of the bladder, epispadias and other bladder anomalies. In \\'Ash PC, Retik AB, Stamey TA, Vaughan ED Jr , eds. In Campbell's 6th edn. Philadelphia: \Y!B. Saunders Co., 1992; 1701.2: 1772-1 821. 4. Ansell JS. Sursical treatment of esstrophy of the bladder with emphasis on neonatal primary closure: personal esperience with 28 consecutive cases treated at the Unil-ersity of \\ishington hospitals ft-om 1962 to 1997 techniques and results. 1979; 121:650. 5. \Yryndaele J J, maes d,. Clean intermittent catheterisation: a 12 !.ears foUo\r up. J Urol 1990; 143: 906. 6. hlasrobian H, Icelais PP, [Gamer S A,. Long term follon.up of 103 patients with bladder esstrophy. J Urol 1988; 139:719. 7. Stockle AI, Becht E, Voges G, Riedmiller H,  Hohenfellner R. Ureterosi~moidostomy an outdated approach to bladder essnophy? J Urol 1990; 143:770. 8. Coffey R C. Ph!.siologic implantation of the severed ureter or common bile duct into the intestine. J Am hled Assoc 191 1; 56: 397. 9. Ashken hI H. Reriav of continence mechanism; InHohenfellner R, \\kmmack R, eds Continent urinary diversion 1st etln: Churchill Livingstone, 1992:63-83. 10. Connor J R, Hesle T \\s; Letimer J I.;, Burbige I< A. Long term follou. up of 207 patients with bladder esstrophy: an evolution in treatment. J Urol 1989; 142:793. 11. Hollo\vell J G, Hill P D, Duffy P G, Ransley P G. Evaluation and treatment of incontinence after neck reconstruction in exstropliy and epispadias. BrJ Urol 1993; 71:743. 

The urinary diversion in children with bladder exstrophy and epispadias: alternative to primary bladder closure

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The urinary diversion in children with bladder exstrophy and epispadias: alternative to primary bladder closure

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