The 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 bicarbonate supplements. Only one patient evaluate the outcome of management of had night soiling that required wearing of bladder exstrophy and epispadias with diapers. Our experience with continent continent urinary diversion. A total of 15 urinary diversion in children with other children, 10 females and 5 males underwent benign bladder conditions has been continent urinary diversion at Kilimanjaro favourable and in our view, it offers a viable Christian Medical Centre (KCMC) between treatment method in children with exstrophy 1985 and 1997. Their ages ranged between one epispadia complex. month and 13 years with an average of 5.4 years. Eight (53%)of them had exstrophy Introduction epispadias complex, 4 (27%) had incontinent In children with epispadias-esstroph~lcomples, epispadias, 2 (13%) presented with the aim of management is to achieve urinary neurological conditions and 1 (7%) had continence, preserve upper urinary tracts and traumatic destruction of the bladder neck and provide adequate external genitalia. Despite the . Seven (47%) had Mainz pouch I1 fact that various methods of treatment have been procedure, 6 (40%) underwent the classical reported, the ultimate surgical procedure remains ureterosigmoidostomy while 2 (13%) had elusive or even debatable. Most urologists in the appendicovesicostomy. The mean duration West prefer bladder closure with or without of follow up was 3.2 years. Three patients osteotomy in the first 24-72 hours of life and this developed mild non-progressive is followed by bladder neck reconstruction and hydronephrosis, which required no surgical bilateral ureteroneoc~~stostomy with intervention. One patient with a solitary reconstruction of the external genitalia at 2 to 5 developed ureteral stenosis at the years. Most of the time additional procedures are implantation site that was nndivcrted. ~-cc~uiycc\to chic\-c contisicncc. Contincncc rate Metabolic acidosis was well compensated after these procedures has been reported to range with none of the patients requiring sodium from 32 to 80°/04. However, in most of these series patients are considered continent even if they are opened antimesenterically and the were only capable of emptying their bladder by clean tunnelled submucosal~rfor antireflus mechanisms intermittent self-catheterisation, which in males as described by Coffeys. can be complicated, by recurrent epididymitis and urethral stricture5. For the sigma rectum pouch, after opening of the rectum and sigmoid for 10 to 12 cm proximal Primary bladder closure and subsequent bladder and dstal to the rectosigrnoid junction, the median neck reconstruction has also been reported to be margins were sutured. Fixation of the complicated by l~ydronephrosis,reflus and poor rectosigmoid junction to the sacral promontory preservation of the upper urinary tracts6. as well as parallel ureteral implantation guaranteed a straight ureteral path and prevented kinliing and In developing regions where the health budget is the subsequent obstruction. meagre and where patients have to travel long distances to reach referral hdspitals manned by The use of the appendix as a continent consultants, which also makes follow-up of vesicostomy (appendicovesicostomy) was first patients difficult, it is not feasible to schedule described by Mitrofanoff in 19809. A continent patients for multiple staged and therefore valvular mechanism was achieved with a espensive surgical procedures. It is for those submucosal tunnel of the mobhsed appendx into reasons that we decided to seek for simple and the bladder with the other end brought out to the appliance free techniques of continent urinary skin as a catheterizable stoma. &version rather than primary bladder closure. The rate of complete continence after Postoperatively, the children were followed up by ureterosigmoidostomy has been reported to be regular upper urinary tract imaging analysis of as high as 92.3%'. Since 1992, we have been using serum creatinine, urea nitrogen electrolytes the sigma rectum pouch (Main2 pouch 11), a and rate of bicarbonate usage sphincter control modification of ureterosigmoidostomy as our and the need for re-operation. In our study, treatment of choice. For the paraplegic paediatric continence was defined as having regular normal patients, our procedure of choice has been interval use of toilet, voluntary control of the appendicovesicostomy with or without bladder urinary stream and regular normal regular augmentation as these patients have insufficient underclothes without diapers. anal sphincter tone. In this paper we report data obtained from children who underwent urinary Results diversion over a period of 12 years at IGlimanjaro During the period under review, a total of 15 Christian Medical Centre (ICCMC). children underwent continent urinary diversion for non-malignant conditions. There were eight Patients and methods cases of exstrophy epispadia complex, 4 of Continent urinary diversion for non-malignant incontinent epispadias, 2 of neurogenid condtions conditions at I,cars\~-ith a mc;m ;isc of -5.4\.c;ll.s classical ureterosigmoidostomy,a midline and a mean follow up of 3.2 pears (range of 1 to laparotomy incision was used, the colon was 13 years). Sigma-rectum pouch (Mainz pouch 11) was to sigma rectum pouch. The two paraplegic performed in 7 cases, 6 had classical patients who were done appen&covesicostomy ureterosigmoidostom~iwhile two patients had were fully continent, doing intermittent appendicovesicostom~~.Post-operatively, two catheterisation through their stomas. No stoma patients developed superficial wound sepsis. No complications had developed. other early postoperative complications were recorded. Discussion In most developed countries, the standard Long-term complication treatment for exstrophj~epispadia complex is primary bladder closure during the neonatal UPPER URINARY TRACTS: period with subsequent bladder neck All patients were followed up with intravenous reconstruction at the age of 2 to 5 years. Often urography and more recently by renal ultrasound. additional procedures are required to achieve hlild unilateral, non-progressive hydronephrosis continence. Despite these multiple procedures developed in three patients, none requiring however, continence rate vary, ranging between sursical intervention. All patients had their serum 32 and 80%. Primary bladder closure and creatinine and blood urea nitrogen within normal subsequent bladder neck reconstruction is not range. No cases of pyelonephritis or urolithiasis alwaos successful. The reason for the failure of were recorded. primary bladder closure is the fact that creation of a functional sphincter mechanism is ultimately One female clild with incontinent epispadias who impossible. To achieve complete continence, had undergone ureterosigmoidostom~~developed further procedures beyond Young - Dees- stenosis at the ureteral implantation site. Tlis cMd Leadbetter bladder neck reconstruction are often had a solitary Iudney; she was undiverted and necessary. These additional procedures include simultaneously underwent Marshal Marchet intestinal bladder augmentation, a pubovaginal bladder neck reconstruction six months after the sling or artificial sphincter implantation". In those primary diversion. cases where outlet resistance is high, clean intermittent self-catheterisation is required. This METABOLIC COMPLICATION: is an additional risk procedure especially for boys Patients had regular determinations of serum in whom it has been associated with increased electrolytes (Na+, I<+, C1-) and recently C02 rate of epididymitis, urethral stricture and even levels. All patients were metabolically well perforation of the augmented bladder5. Another compensated with none requiring sodium hypothesis for failure of primary bladder closure bicarbonate supplements. may be insufficiency of the detrusor muscle itself. Hollowell et al" demonstrated urine leakage due CONTINENCE: to involuntary detrusor contractions in 10 out of All seven children who had sigma rectum pouch 18 patients with poor continence. were fully continent. Among the six who underwent ureterosigmoidostom~~,one had Normal bladder function during filling and nighttime soiling requiring diapers. This was a contractile voiding was demonstrated in only 8% female child who had exstrophy epispadias of their patients. They therefore concluded that complex and was being considered for conversion < < evolution to completely normal lower urinary tract function after bladder neck reconstruction children with bladder esstrophy epispad is unrealistic espectation for the majority of comples. children with esstrophy and severe epispadias." References In our series, metabolic complications were 1. Koo H P, Arollo L, Duckett J \V. Long-term results of ureterosigmoidostomy in children with bladder esstrophy. minimal and so were changes in the upper urinary 2. Stein R, Fisch XI. Stockle hl, Hohenfellner R. Urinary diversion in tracts. Continence rate after sigma rectum pouch bladder esstrophy and incontinent epispadias: 25 years experience, J Urol 1995; 154:1177. was 100% while after ureterosigmoidostomy, it 3. Geahart JP, Jeff RD. Esstrophy of the bladder, epispadias and other was 83%; findings which were comparable with bladder anomalies. In \\'Ash PC, Retik AB, Stamey TA, Vaughan ED Jr , eds. In Campbell's 6th edn. Philadelphia: \Y!B. Saunders the continence rates of SO - 100% reported in Co., 1992; 1701.2: 1772-1 821. literature from elsewhere. Since 1992, we have 4. Ansell JS. Sursical treatment of esstrophy of the bladder with been performing sigma rectum pouch in emphasis on neonatal primary closure: personal esperience with 28 consecutive cases treated at the Unil-ersity of \\ishington hospitals preference o the classical ureterosigmoidostomy. ft-om 1962 to 1997 techniques and results. 1979; 121:650. For the paraplegcs with insufficient anal sphincter 5. \Yryndaele J J, maes d,. Clean intermittent catheterisation: a 12 !.ears foUo\r up. J Urol 1990; 143: 906. unction, our procedure of choice has been 6. hlasrobian H, Icelais PP, [Gamer S A,. Long term follon.up of 103 appendecovesicostomy. The overall continence patients with bladder esstrophy. J Urol 1988; 139:719. 7. Stockle AI, Becht E, Voges G, Riedmiller H, Hohenfellner R. rate among our 15 children was 93%. Ureterosi~moidostomyan outdated approach to bladder essnophy? J Urol 1990; 143:770. In conclusion, we have presented our 12 years' 8. Coffey R C. Ph!.siologic implantation of the severed or common bile duct into the intestine. J Am hled Assoc 191 1; 56: esperience with primary urinary diversion in 397. children with esstrophy epispadias comples. A 9. Ashken hI H. Reriav of continence mechanism; InHohenfellner R, \\kmmack R, eds Continent urinary diversion 1st etln: continence rate of more than 90% mas achieved Churchill Livingstone, 1992:63-83. with good preservation of upper tracts as well as 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 adequate metabolic compensation. Our treatment. J Urol 1989; 142:793. experience has therefore been most favourable 11. Hollo\vell J G, Hill P D, Duffy P G, Ransley P G. Evaluation and and we conclude that continent urinary diversion treatment of incontinence after neck reconstruction in exstropliy and epispadias. BrJ Urol 1993; 71:743. is a viable alternative treatment method for