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Journal of Pediatric Urology (2015) xx, 1.e1e1.e6

Efficacy and safety of continent anal urinary diversion for complicated bladder exstrophy in children by using modified Duhamel’s procedure a

Al Azher University, Department of Pediatric Surgery, Cairo, Egypt

b

National Institute of Urology, Cairo, Egypt

Mohamed A. Baky Fahmy a, Alae A. Al Shenawy b, Sameh M. Shehata c Summary Background

c

Alexandria University, Department of Pediatric Surgery, Egypt Correspondence to: M.A. Baky Fahmy, Al Azher University, Cairo, Egypt [email protected] (M.A. Baky Fahmy) Keywords Duhamel’s pull-through; Bladder exstrophy; Urinary diversion Received 1 November 2014 Accepted 19 February 2015 Available online xxx

A high proportion of children with bladder exstrophy will continue to suffer from urinary incontinence and a miserable life even after a well-performed staged reconstruction in specialized centers. Most of those children usually have a normal anal sphincter allowing construction of a neobladder from the rectum, so they are continent without an abdominal stoma, and do not require frequent catheterization, which greatly contribute to a favorable body image.

Objective In this study a modified Duhamel’s rectal pouch done for 19 children, with implication of suitable stapler adopted to construct a rectal bladder with a non-refluxing urterorectostomy, there is a theoretical advantage in our procedure of avoiding a mix of urine and feces. All patients were followed for up to 6 years (2e8 years) for efficacy, safety, subsequent renal complications, and surveillance for any rectal neoplastic changes in this new diversion.

Study design Assessment of electrolytes, acid base balance, and renal function were carried out regularly and all data were analyzed using the SPSS 9.0.1 statistical package and

compared using a paired t test; data were considered significant if p < 0.05. Proctoscopy was performed 6 monthly in the first year then annually thereafter, and at any time if there was any rectal bleeding.

Results In this group of patients, follow-up revealed no neoplastic changes in the rectal bladder, deterioration in renal function, or major electrolytes disturbance. They can hold up to 400 mL (350e550 mL) of urine and all are continent during the daytime with an emptying frequency of 3e5 h; three patients had infrequent (4 episodes/month) nocturnal enuresis; and four cases developed pyelonephritis controlled with medical treatment.

Conclusion The continent rectal bladder created by using the principles of the Duhamel pull-through is feasible, easy to perform, successful in the immediate short term with low complications after 6 years of follow-up and appropriately accepted by the children and their families with marked improvement in quality of life regarding continence; longer-term follow-up is requested to rule out rectal neoplastic changes. A comparative review of the complications, patient’s acceptance, and longer-term follow-up with other wellknown procedures, such as Mainz II, is required.

Figure Diagram showing the created rectal pouch with its uppermost part as rectal bladder above the feacal stream. http://dx.doi.org/10.1016/j.jpurol.2015.02.018 1477-5131/ª 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

Please cite this article in press as: Baky Fahmy MA, et al., Efficacy and safety of continent anal urinary diversion for complicated bladder exstrophy in children by using modified Duhamel’s procedure, Journal of Pediatric Urology (2015), http://dx.doi.org/10.1016/ j.jpurol.2015.02.018

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Introduction Surgical reconstruction of the exstrophic bladder with local tissues is usually undertaken primarily in one stage or as a staged procedure. However, many children will end up with a bladder of small capacity, and several patients remain incontinent even after a well-performed staged reconstruction in specialized centers [1,2]. In Egypt, the incidence of residual incontinence is high, reaching 40%, and most of these children remain diaper dependent and looking for consideration of internal or external diversion [3]. Ureterosigmoidostomy suffers from significant inherent complications, including metabolic derangements, pyelonephritis, reduced growth, and particularly delayed carcinogenesis [4]. The expectations associated with urinary diversion have changed from simple diversion without protecting the upper urinary tract to anatomic and functional reconstruction of the urinary tract that is almost equal to the natural preoperative state. Progress in this field was achieved in three different ways: subsequent incontinent cutaneous diversion (conduit), continent cutaneous diversion (pouch), and continent diversion followed by the intact urethra (neobladder, orthotopic reconstruction) [2]. In the past 15 years, the neobladder has become the standard procedure conducted in many urologic centers, when initially it had been merely an experimental surgical method. In giving advice to patients who have to have urinary diversion, surgeons must decide on the method that offers the greatest safety and the lowest rates of short- and long-term complications; what suits patients’ lifestyles best, and which method thus interrupts their quality of life least [5]. Children with bladder exstrophy usually have a normal anal sphincter allowing construction of a neobladder from the rectum, so continence without an abdominal stoma or frequent catheterization greatly contribute to a favorable body image; especially if done early, the child could rapidly learn anal control for both systems, noticing little difference from the usual means of eliminating urine. We reported our initial experience with ureteric diversion to the rectum and bowel reconstruction with a modified Duhamel pull-through technique in 2007 with 11 patients [6]. Followup for quality of continence, subsequent renal complications, and surveillance for any rectal neoplastic changes of those cases with an additional eight children operated during the last 6 years will be discussed.

Materials and methods Seventeen otherwise normal boys and two girls with mean age of 5.5 years (4e9 years) had no gross anomalies other than bladder exstrophy and epispadias who had been operated on several times (3e6, mean 3) as neonates or subsequently at older age were included in this study. Five children had incomplete abdominal wall dehiscence and bladder prolapse, and 14 had a small contracted bladder without a bladder neck. Ten had a different grade of epispadias and all were incontinent for urine with total dependence on diapers; none of them had previous diversion or augmentation. All patients had normal renal function and continent anal sphincter. The parents and eventually the patients were made fully aware of the

M.A. Baky Fahmy et al. required long-term specialized care and scheduled followup. blood urea nitrogen (BUN), creatinine clearance, renal ultrasound, and renal nuclear scan were assessed; only one child had a grade 2 vesicoureteric reflux, and another two had grade 1. Anal sphincter function was studied using a water-soluble contrast enema; subsequently, anal sphincter profilometry at rest and during stress (straining and coughing), anorectal manometry, and anal muscle EMG using the anal plug and surface electrode were carried out; all cases were selected to have an anal sphincter resting pressure of 50 mmHg or more (50e86 mmHg), and maximum squeezing pressure 160 mmHg (150e390 mmHg), which is normal compared with age-matched controls [7]. Only those who had normal findings were selected for diversion.

Duhamel pull-through with ureterorectostomy Bowel preparation was carried out 3 days preoperatively; metronidazole and a broad-spectrum antibiotic were given during the operation. The child was placed in the Lloyd Davis position and the abdomen was entered through a midline incision, sometimes through the previous scar. After mobilization of the sigmoid colon, the rectum was drawn up into the abdomen with stay sutures and a path was developed posteriorly by blunt dissection down to the pelvic floor. The sigmoid colon was divided above the rectum, from 20 to 30 cm of the rectum above the peritoneal reflection to be used as a urinary reservoir. The proximal colon passed behind the rectum; it was anastomosed to the posterior wall of the anal canal above the dentate line and internal sphincter from the anus using a circular stapler size 21 or 23 according to age. An appropriate GIA linear autostapler was passed through the anal canal anastomosing and dividing the colorectal septum and creating a common neorectum with a longer rectal spur than the one created for the Duhamel procedure used for treating cases of Hirschsprung’s disease (Fig. 1). The two ureters were detached from the bladder and using the Goodwin technique [8] implanted in a submucosal tunnel on the lateral rectal wall below the upper edge of the rectum. Transanastomotic ureteric catheters were passed into the rectal lumen and were accessible through the anus. The proximal rectum was closed in two layers, and the abdomen was closed, leaving a pelvic drain. The residual urinary bladder was left in situ and excised subsequently at the time of genital reconstruction. Caudal blocks were performed with 1 mg/kg of clonidine with ropivacaine for postoperative analgesia. Oral feeding had resumed by the fourth day (3e6 days) and the ureteric splints were removed on the tenth day. The child was discharged home at postoperative day 13 (range 12e15 days). Routine alkalinizing with sodium bicarbonate was started orally, in doses according to the patient’s pH. Intensive toilet training to help the child to control loose motions started at the third postoperative week, for a period of 1e2 months. Follow-up had been scheduled every 3 months for 1 year, then every 6 months; serum electrolytes, pH, acidebase balance, blood urea nitrogen (BUN), creatinine, and creatinine clearance were analyzed. Data were entered and analyzed using the SPSS 9.0.1 statistical

Please cite this article in press as: Baky Fahmy MA, et al., Efficacy and safety of continent anal urinary diversion for complicated bladder exstrophy in children by using modified Duhamel’s procedure, Journal of Pediatric Urology (2015), http://dx.doi.org/10.1016/ j.jpurol.2015.02.018

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Continent anal urinary diversion by using modified Duhamel’s procedure

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with suspicious mucosa. The families were aware that the child would need further life-time follow-up with proctoscopy or colonoscopy, and if the child’s motion contained blood.

Results

Figure 1 Diagram explaining the anastomosis between the sigmoid colon and the rectum with implanted ureters in the rectal reservoir.

package (SPSS, Chicago, IL, USA), and compared using the paired t test; data were considered significant if p < 0.05 (Table 1). Renal ultrasound was performed during a visit and for 12 of 19 patients a renal DMSA isotope scan was performed at 6 months. The rectal pouch capacity was assessed at 3 months postoperatively by proctography with a soluble contrast medium. At each visit, particular attention was given to the frequency of urinary evacuation and nocturnal bedwetting. The children and their mothers were questioned about the frequency of defection, bedwetting, and nature of the motion to assess continence status. Proctoscopy was carried out 6 monthly in the first year, and then annually for 17 out of 19 patients; the examination was carried out on a day case basis, with an overnight enema. Biopsy was indicated in three patients

Table 1

The mean operative time was 3 h (2e4 h). Blood loss was minimal, and the hemoglobin level and hematocrit value were maintained within the normal range postoperatively. The 3-monthly renal ultrasound demonstrated a normal scan without scarring or dilation. Blood gases, serum electrolytes, BUN, and creatinine clearance were estimated twice during the stay in the hospital, and at the 3-monthly follow-up were within normal ranges (Table 1). Three patients developed acidosis during the early postoperative period; in two of them the acidebase balance was corrected with intravenous fluid, but the third one was corrected with oral sodium bicarbonate. Soluble contrast proctography revealed an average rectal pouch capacity of 400 mL (from 300 to 550 mL) at 3 months; no reflux of contrast to the ureters was detected in 17 cases; two cases had a ureteric reflux on one side: one was corrected surgically by subserosal retunneling of the distal ureter in the rectal wall without recurrence. By the end of the third month the children were able to retain motions for 3 h, which had increased to 4 h by the eighteenth month, and 4.5 h by the second year (4e4.5). Initially, nocturnal enuresis occurred some seven times per month (6e8 months) in nine patients, but decreased to three times per month by the second year (2e4 time) in only three patients; the other 16 patients were dry at night after 2 years (Table 2). Three children had perianal skin excoriation and dermatitis, which was managed with local emollients; two had diarrhea for 10e15 days postoperatively, which was self-limiting. Another child required a constipating agent for 2 weeks to help control his rectal content. Four children had a urinary tract infection with pyelonephritis 3 weeks after surgery, and one of them required hospitalization and antibiotic injections without recurrence. The follow-up proctoscopy after 6 years for three of 19 patients at for 2 years for 16 of 19 patients revealed no suspicious neoplastic changes in the rectal wall. Three cases showed elevated mucosa around the

Biochemical data of 19 patients followed for 18 months; data presented as median (minimumemaximum).

Biochemical data

Normal values (n Z 19)

3 months

6 months

12 months

pH Bicarbonate mmol/L Sodium mmol/L Chloride mmol/L Potassium mmol/L Creatinine mmol/L Creatinine clearance mL/min BUN mmol/L

7.38e7.48 22e29 135e150 98e106 3.5e5 60e125 97e137 1.8e7.1

7.37 23.5 139 103 3.9 89 101 4.8

7.40 24.5 141 103 4 80 103 4.1

7.41 24 141 102 4 76 112 2.6

(7.35e7.4) (23e25) (138e142) (102e105) (3.7e4.1) (80e95) (95e113) (2.2e7)

(7.36e7.41) (23e26)a (140e142)a (102e105) (4e4.2)a (72e85)a (97e112) (1.9e5.9)a

(7.39e7.42)a (22e26) (140e143)a (100e104)a (3.9e4.2)a (70e82)a (102e119)a (2e3.1)a

18 months 7.41 24.6 141 101 4 72 116 2.2

(7.4e7.42)a (24.5e23)a (141e140)a (101e100)a (4e4)a (69.5e68)a (118e110)a (2e2.3)a

BUN Z blood urea nitrogen. a Significant difference in relation to the results of the third month, where p < 0.05.

Please cite this article in press as: Baky Fahmy MA, et al., Efficacy and safety of continent anal urinary diversion for complicated bladder exstrophy in children by using modified Duhamel’s procedure, Journal of Pediatric Urology (2015), http://dx.doi.org/10.1016/ j.jpurol.2015.02.018

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M.A. Baky Fahmy et al.

Table 2

Rectal evacuation and bed witting of all cases; data presented as median (minimumemaximum).

Bowel evacuation Frequency to evacuate the rectum/hours Bed wetting at night per month No. of patients (n Z 19) a

3 months 3 (2.5e4) 7 (6e8) 9

6 months 3 (3e4) 7 (6e8) 6

12 months

18 months

a

a

4 (3e4.3) 6 (5e7)a 4

4 (4e4.3) 4 (4e6)a 3

24 month 4.5 (4.5e5)a 3 (2e4)a 3

Significant difference in relation to the results of third month, where p < 0.05.

ureteric orifice; biopsy and histopathology revealed no metaplasia or anaplasia, and only one case showed inflammatory changes with predominant lymphocytic cells. This child also had reflux.

Discussion Management of bladder exstrophy aims to obtain a secure abdominal wall closure, achieving urinary continence with preservation of renal function, and adequate cosmetic and functional genital reconstruction. Shaw et al. [9] have reported excellent outcomes from the Modern Staged Repair of Bladder Exstrophy MSRE in 2004, achieving continence in 85% of children, but their continence was not volitional voiding continence, and further analysis shows that only 23% achieved this successful goal. In developing countries where early surgical reconstruction is not done routinely and with limited technical resources, there is a high incidence of bladder reconstruction failure for children with bladder exstrophy; many, either primarily or following failed surgery, want to be considered for diversion. Also, social and culture concerns often do not favor an external stoma and add to the need for an internal continent urinary diversion [10]. In cases of final reconstruction failure, urinary diversion may be considered; the use of internal urinary diversions in the bladder exstrophy population in not new and the first experiences were reported in 1852 by Simon [11] when he attempted to divert urine via the bowel by creating a fistula between the ureters and the rectum in a patient with bladder exstrophy. More than 60 modifications of ureterosigmoidostomy had been published; these concentrate on preventing renal deterioration from ascending infection, by creating a non-refluxing uretero-intestinal anastomosis; these techniques have a high incidence of unexplained metabolic disturbances and led to the abandonment of ureterosigmoidostomy in favor of ileal conduit urinary diversion in the mid-20th century. In the following years, improved understanding of the pathophysiology of hyperchloremic metabolic acidosis, the development of alkalinizing drugs, and Goodwin’s technique of ureteric implantation solved many of the previous problems of ureterosigmoidostomy and reawakened interest in this procedure [12]. Currently, continent diversion should be preferably chosen in spite of the fact that there is argument over the usefulness of the non-continent diversion in cases of reconstruction in young children. In this case, the priority is to preserve renal function, achieving maximum survival rates and trying to, if possible, facilitate conversion to a continent ostomy of abdominal catheterization [13]. Most

surgeons are quite rightly concerned about the possibility of neoplastic change within intestinal segments transposed to the lower urinary tract, and this fear arises from the occurrence of latent adenocarcinoma arising from urterocolic anastomosis in approximately 10% of patients who underwent ureterosigmoidostomy in childhood. An update of previous reviews and details of work published since 1990 on epidemiological, experimental, and clinical studies, and consideration of the collected evidence confirms the increased risk of colonic neoplasia following mixing of the fecal and urinary stream by ureterosigmoidostomy or its more recent variants. In contrast, the occurrence of tumors within transposed intestinal segments appears more likely related to the underlying urinary tract disease for which the surgery was performed rather than exposure of the intestinal mucosa to urine [14]. Various innovative surgical techniques have been advocated for separating the fecal and urinary streams while still employing ureterosigmoidostomy principles. These operations can generally be considered together as rectal bladder urinary diversions. In each of these operations, the ureters are transplanted to the rectal stump, and the proximal sigmoid colon is managed by terminal colostomy, or more commonly by bringing the sigmoid colon to the anal sphincter, using the sphincter for both bowel and urinary control. The principal concern is the calamitous complication of postoperative urinary and fecal incontinence, presumably occurring as a consequence of damage to the anal sphincter during the operative dissection [15]. The DuhameleMartin pull-through technique with its subsequent modifications is a well-known procedure, adopted in pediatric surgery centers from late sixties, for the surgical correction of Hirschsprung’s disease [16]. In this study a modified Duhamel technique using a suitable stapler was used to construct a rectal bladder with a non-refluxing urterorectostomy; there is a theoretical advantage in our procedure of avoiding a mix of urine and feces [6] (Fig. 2). This constructed new bladder is unlikely to keep the urine away from feces physically, but the “separation” may be due to the timing of fecal entrance into the rectum, rather than due to any mechanical reconstruction as a result of the surgical technique, and this could explain the nonexistence of any neoplastic changes after more than 200 proctoscopy examinations during 9 years of surveillance; however, a longer-term follow-up is required to prove that the procedure is thoroughly safe. A dry genital region will enable the surgeon to reconstruct any epispadias the child may have, remedy the phallus, and revise abdominal scars, which may help achieve an acceptable sexual life. The lack of hyperchloremic acidosis during the follow-up period should be interpreted with caution; this could be due to

Please cite this article in press as: Baky Fahmy MA, et al., Efficacy and safety of continent anal urinary diversion for complicated bladder exstrophy in children by using modified Duhamel’s procedure, Journal of Pediatric Urology (2015), http://dx.doi.org/10.1016/ j.jpurol.2015.02.018

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Limitation of the study A comparative review of the complications with other wellknown procedures such as the Mainz II, and the patient’s acceptance and longer-term follow-up are required.

Conflict of interest None.

Funding None.

Ethical approval This paper approved by the ethical committee at Al Azhar University.

References Figure 2 Postoperative proctography showing the capacity of rectal pouch.

lack of absorption of electrolytes in the rectum. Experimental studies comparing the absorption potential of the rectum and sigmoid colon for electrolytes are in favor of rectal diversion over the ureterosigmoidostomy, which could possibly avoid hyperchloremic acidosis [17]. Most of the present patients are receiving alkalinizing drugs, because of our policy of early alkaline substitution according to the acidebase status results, to prevent clinical metabolic acidosis and potential bone demineralization in the long term. This technique is a simple, reliable, and viable alternative for continent urinary diversion in selected patients; this procedure is associated with minimal pelvic dissection and achieves a reasonable rectal reservoir. The period of follow-up is too short to consider that there is no risk of neoplasia, as neoplasia in ureterosigmoidostomy can be detected after 20 years of surgery [17].

Conclusion Follow-up of the 19 patients for 6 years (2e8 years) showed that there is no deterioration of the upper renal tract, no significant electrolyte or acidebase disturbance, and no neoplastic changes in the rectal bladder. The continent rectal bladder created by using the principles of the Duhamel pull-through is feasible, easy to perform, successful in the immediate short term, with low complications after 6 years of follow-up and appropriately accepted by the children and their families with marked improvement in the quality of life regarding continence, but longerterm follow-up is needed to rule out rectal neoplastic changes.

[1] Chan DY, Jeffs RD, Gearhart JP. Determinants of continence in the bladder exstrophy population: predictors of success. Urology 2001;57:774e7. [2] Dickson AP. The management of bladder exstrophy: the Manchester experience. J Pediatr Surg 2014;49:244e50. [3] Hammouda HM, Kotb H. Complete primary repair of bladder exstrophy: initial experience with 33 cases. J Urol 2004;172: 1441e4. [4] Koo HP, Avolio L, Duckett Jr JW. Long term results of ureterosigmoidostomy in children with bladder exstrophy. J Urol 1996;156:2037e40. [5] Abrams P, Grant A, Khoury S. Evidence-based medicine: overview of the main steps for developing and grading guideline recommendations. In: Abrams P, Cardozo L, Khoury S, et al., editors. Incontinence, vol. 1. Plymouth: Health Publications Ltd; 2004. p. 10e1. [6] Fahmy MA, Mansour A, Mazy A. Ureterorectostomy as a continent urinary diversion for complicated bladder exstrophy in children by using a modified Duhamel procedure. Inter J Surg 2007;5:394e8. [7] Cali RL, Blatchford GJ, Perry RE, Pitsch RM, Thorson AG, Christensen MA. Normal variation in anorectal manometry. Dis Colon Rectum 1992;35:1161e4. [8] Goodwin WE, Harris AP, Kaufman JJ, Beal JM. Open transcolonic ureterointestinal anastomosis: new approach. Surg Gynaecol Obstet 1953;97:295e300. [9] Shaw MB, Rink RC, Kaefer M, Cain MP, Casale AJ. Continence and classic bladder exstrophy treated with staged repair. J Urol 2004;172:1450e3. [10] Shoukry AI, Ziada AM, Morsi HA, Habib EI, Aref A, Badawy HA, et al. Outcome of complete primary bladder exstrophy repair: single-center experience. J Pediatr Urol 2009:496e9. [11] Simon J. Ectopia vesicae (absence of the anterior wall of the bladder and pubic abdominal parities): operation for directing the orifices of the ureters into the rectum; temporary success; subsequent death; autopsy. Lancet 1852;ii:568. [12] Pierre K, Borer J, Phelps A, Chow JS. Bladder exstrophy: current management and postoperative imaging. Pediatr Radiol 2014;44:768e86. [13] Hensle TW, Chang DT. Reconstructive surgery for children with pelvis rhabdomyosarcoma. Urol Clin North Am 2000;27: 489e502.

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1.e6 [14] Pickard R. Tumour formation within intestinal segments transposed to the urinary tract. World J Urol 2004;22:227e34. [15] Kanojia RP, Rao KN, Menon P, Agarwal S, Bawa M, Mahajan JK, et al. Recto sigmoid bladder reservoir for patients with exstrophy: three dimensional evaluation for outcome analysis. J Pediatr Urol 2015;10(6):1176e80.

M.A. Baky Fahmy et al. [16] Duhamel BA. New operation for the treatment of Hirschsprung’s disease. Arch Dis Child 1960;35:38e9. [17] Bastian PJ, Albers P, Haferkamp A, Schumacher S, Muller SC. Modified ureterosigmoidostomy (Mainz Pouch II) in different age groups and with different techniques of ureteric implantation. BJU Int 2004;94:345e9.

Please cite this article in press as: Baky Fahmy MA, et al., Efficacy and safety of continent anal urinary diversion for complicated bladder exstrophy in children by using modified Duhamel’s procedure, Journal of Pediatric Urology (2015), http://dx.doi.org/10.1016/ j.jpurol.2015.02.018

Efficacy and safety of continent anal urinary diversion for complicated bladder exstrophy in children by using modified Duhamel's procedure.

A high proportion of children with bladder exstrophy will continue to suffer from urinary incontinence and a miserable life even after a well-performe...
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