- Email: [email protected]
Continence and quality of life with the modified Heitz-Boyer-Hovelacque rectal bladder for children with urinary incontinence following bladder exstrophy
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MODEL
Journal of Pediatric Urology (2016) xx, 1.e1e1.e6
Continence and quality of life with the modified Heitz-Boyer-Hovelacque rectal bladder for children with urinary incontinence following bladder exstrophy a
Division of Pediatric Surgery, Department of Medical, Surgical and Neurological Sciences, University of Siena, Siena, Italy
b
Neonatal and Paediatric Reconstructive Surgeon, Manchester, UK Correspondence to: R. Angotti, Division of Pediatric Surgery, Department of Medical, Surgical and Neurological Sciences, University of Siena, Viale Bracci 16, 53100 Italy [email protected] (R. Angotti) Keywords Bladder exstrophy; Urinary incontinence; Rectal bladder; Heitz-Boyer-Hovelacque technique Received 29 July 2015 Accepted 27 December 2015 Available online xxx
M. Messina a, F. Molinaro a, F. Ferrara a, R. Angotti a, A.L. Bulotta a, G. Di Maggio a, A. Bianchi b Summary Background Bladder exstrophy and epispadias are severe congenital anomalies associated with an open bladder and urinary sphincter. Despite modern reconstruction, there is a significant incidence of residual or recurrent urinary incontinence that impacts on quality of life (QoL) and self-esteem, which in turn limits social interaction (Figure). The present study involved 14 patients, mainly from a Middle Eastern country, and reported the early findings with a modification of the Heitz-Boyer-Hovelacque rectal bladder technique for both urinary and faecal control. Study design Fourteen children, with a median age of 8.1 years, with poor quality of life and low self-esteem because of urinary incontinence and small polypoidal open bladders of 5e15 ml volume, mostly after bladder exstrophy surgery, were managed with a modification of the Heitz-Boyer-Hovelacque rectal bladder technique keeping an intact anal sphincter. The retrorectal pulled-through colon was anastomosed to the posterior wall of the rectum just above the external sphincter complex, thereby avoiding any possible injury to the anal sphincter. All patients had a normal colon and a competent anal sphincter without lumbosacral spinal or nerve anomalies. Results Ten children had a 5- to 10-year follow-up, one child had a 15-year follow-up, and three others, that were
Figure
also continent, were excluded because of a <5-year follow-up. There were no postoperative complications, and all were dry and odour-free by day within 2e4 weeks of surgery. Two children still had minor urinary loss at night. There were no UTIs and renal function remained unimpaired. Eleven years after surgery, one child underwent excision of a pedunculated benign inflammatory polyp from the tip of the left ureter because of recurrent torsion and bleeding, there was no recurrence at the 2-year follow-up. None of the rectal or ureteric biopsies from any of the children showed metaplasia or neoplasia; however, in view of the potential long-term risks, all children were placed on a lifelong ‘proctoscopy and biopsy’ protocol. Discussion The ability to be dry and odour-free, and to wear normal clothing had a striking impact on QoL and psychological well-being of the children and their families. This was reflected in their positive overall approach, voluntary school attendance, and enthusiastic participation in communal events. All agreed that their improved genital appearance markedly contributed to their better body image and increased self-esteem. Conclusion These significant benefits, at a crucial time in the child’s life, outweigh the potential risk of long-term neoplasia. Therefore, the Heitz-Boyer-Hovelacque rectal bladder technique is recommended with longterm proctoscopic follow-up.
Female (A) and male (B) anatomy after failed reconstruction.
http://dx.doi.org/10.1016/j.jpurol.2015.12.010 1477-5131/ª 2016 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Messina M, et al., Continence and quality of life with the modified Heitz-Boyer-Hovelacque rectal bladder for children with urinary incontinence following bladder exstrophy, Journal of Pediatric Urology (2016), http://dx.doi.org/ 10.1016/j.jpurol.2015.12.010
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MODEL
1.e2
Introduction Exstrophy-epispadias is a severe congenital anomaly that presents with major disruption of the infraumbilical abdominal wall, a wide diastasis of the pubic symphysis, a variable-volume open bladder and sphincter, and a short open urethra passing; in the male, this is along the dorsum of a foreshortened epispadiac penis [1e3]. The lumbosacral spine and nerves, the anus and posterior pelvic floor are often anatomically and functionally normal. Despite modern reconstructive techniques, residual or recurrent uncontrollable urinary incontinence and odour, the need for diapers, the inability to wear normal clothing, the poor genital aesthetics, and the limitations to social interaction have a significant impact on quality of life (QoL), body image and self-esteem. To the psychologically vulnerable child/adolescent and family, procedures that offer the possibility of being dry and odour-free rank as highly as genital aesthetics and function. In this, as yet small, study of 14 children, the early impressions with a modification of the Heitz-Boyer-Hovelacque rectal bladder technique [4] for anal control of both urinary and faecal streams, avoiding any risk to the anal sphincter, are reported.
Material and methods Thirteen patients (six males, seven females) with a median age of 8.1 years (range: 6e13 years) from a Middle Eastern country, and one European male child with a 15-year follow-up who had a rectal bladder at 7 years of age at another institution, were referred because of residual or recurrent urinary incontinence following unoperated or failed bladder exstrophy surgery (seven patients had two failed attempts). Three children with a follow-up of <5 years, but presently doing well, were excluded. The remaining 10 patients (four males, six females) with a 5- to 10-year follow-up were managed between 2004 and 2009. There had not been any pelvic osteotomies and all children had a wide pubic symphyseal diastasis, averaging 7.3 cm (range: 5e10 cm). All had a normal lumbosacral spine and a competent anal sphincter. All male patients had epispadias, two had bilateral undescended testes, and one had a unilateral indirect inguinal hernia. Females had a wide mons pubis, divergent labia, a bifid clitoris, and a short vagina; two also had vaginal stenosis. All patients were originally assessed in their own country and informed consent was obtained with the help of an acceptable interpreter, even prior to travel to the present institution. On admission, a detailed review, a full clinical examination, and a preoperative work-up were undertaken. These included: a blood film, blood group, serum urea, serum electrolytes and creatinine, electrocardiogram, abdominal and renal ultrasound, chest X-ray, lumbosacral spine and pelvic X-ray, and magnetic resonance imaging of the pelvis; a barium enema and anorectal manometry confirmed normal anatomy and function. An independent psychologist undertook a pre-operative psychological and quality of life review using the Short Form Health Survey 12 (SF-12) [5,6] that measures eight concepts of health status (i.e. physical functioning, role limitations due to physical health problems, bodily pain,
M. Messina et al. general health, vitality (energy/fatigue), social functioning, role limitations due to emotional problems, and mental health (psychological distress and psychological well-being). The first four health concepts indicate physical health status and the others are an index of mental health. The assessment was repeated postoperatively just prior to discharge and again 1 year after surgery. The mean age for rectal bladder reconstruction was 9.1 years (range: 7e14 years). No pelvic osteotomies were performed. All had significant lower abdominal and perivesical scarring from previous surgery. The open bladders were of 5e15 ml volume and filled with mucosal polyps. Six patients had excision of the polyps and closure of the native bladder, and four patients had resection of their rigid and small-volume bladder, retaining the bladder neck and prostatic urethra for the three males, in continuity with the tubularized urethral plate. At the same procedure, girls also had a single-stage genital reconstruction (not the subject of this paper). Boys had the first of a two-stage reconstruction consisting of penile lengthening by corpora cavernosa release from the pubic rami, Ransley corporal rotation [7] and glanulo-meatoplasty, and an intercorporal transfer of the urethra to a midline penoscrotal meatus. The second-stage urethroplasty with transfer of the urethral meatus to the glans was undertaken 12 months later. Prior to discharge from hospital, all patients had a renal ultrasound, a DTPA-Tc99 scan, and a proctoscopy under general anaesthesia. Baseline biopsies for neoplasia were taken
Table 1
Postoperative management.
Exam
When
Blood pressure
Daily during hospitalisation, every 15 days during the first two months, every 3 months during the first year and then every 6 months Every 15 days during the first two months, every 3 months during the first year and then every 6 months According to the needs of the individual patient Every 15 days during the first two months, every 3 months during the first year and then every 6 months At 15e21 day after surgery and then every year
Blood sample evaluation with renal function and blood gases Teaching and learning of sphincter control Ultrasound
Endoscopy with URETERORECTAL ANASTOMOSIS evaluation and Colorectal anastomosis evaluation and dilatation Renal scintigraphy with DTPA TC99 Histological examination of the rectal bladder biopsies
At 15e21 day after surgery and then according to the condition of anastomosis At 6e12 months after surgery, to be repeated in case of stenosis Every year
Please cite this article in press as: Messina M, et al., Continence and quality of life with the modified Heitz-Boyer-Hovelacque rectal bladder for children with urinary incontinence following bladder exstrophy, Journal of Pediatric Urology (2016), http://dx.doi.org/ 10.1016/j.jpurol.2015.12.010
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Heitz-Boyer-Hovelacque rectal bladder for children from the tip of each ureter, from the rectal mucosa adjacent to the ureteric orifices and at a distant rectal site (Table 1).
Operative technique All patients underwent rigorous pre-operative colonic preparation. All surgery was undertaken by the two senior surgeons (MM, AB) working together and assisted by experienced surgical staff. Under general anaesthesia, with the patient in the lithotomy position to allow access to the anus and the lower abdominal wall, the abdomen and peritoneum were opened, any infraumbilical midline abdominal scar was excised and any bladder that was over-sewn or excised was detached. The sigmoid colon and rectum were identified and, in accordance with patient size and age, 200e300 ml of warm 0.9% saline was infused through a transanal Foley catheter (24 F), with the balloon inflated to occlude the anus, to comfortably distend the rectum that was then divided and over-sewn in the form of a Hartman’s procedure. The retrorectal space was developed by blunt dissection and a large Hegar dilator was passed down to the level of the pelvic floor. Working through the anus, a full-thickness incision was made
1.e3 onto the Hegar dilator for one-half of the posterior rectal circumference just above the external anal sphincter complex that was kept intact. The sigmoid colon was passed through the retrorectal space and a liberal end-to-side colorectal anastomosis was performed (Fig. 1A and B). Through the abdominal incision, each ureter was divided close to the bladder, cannulated with an 8-F feeding tube, and anastomosed to the ipsilateral side of the rectal bladder at about 10e15 cm from the anal margin, with the stents draining into the rectal lumen. No antireflux mechanism was constructed. The abdominal wall was closed, burying the bladder beneath available layers, leaving a 10-F Jackson-Pratt catheter (Teleflex) draining the pelvis. An aesthetic umbilicoplasty and genital reconstruction (not described in this paper) was undertaken.
Results and postoperative follow-up Within a lifetime monitoring protocol, all patients had an annual review (Table 1) that averaged 7.7 years (range: 5e15 years). The protocol included: a clinical review, a psychological assessment, a physical examination, and
Figure 1 (A) and (B) show a colorectal anastomosis: (A) is a schematic drawing; (B) is the endoscopic image of a patient. (C) and (D) show ureteric orifices: (C) is a schematic drawing; (D) is the endoscopic image of a patient.
Please cite this article in press as: Messina M, et al., Continence and quality of life with the modified Heitz-Boyer-Hovelacque rectal bladder for children with urinary incontinence following bladder exstrophy, Journal of Pediatric Urology (2016), http://dx.doi.org/ 10.1016/j.jpurol.2015.12.010
MODEL
(143e145) (4.1e4.7) (105e107) (0.9e1.3) 144 4.4 106 1.1 (139e141) (4.2e4.3) (103e105) (0.6e0.8) 140 4.1 104 0.7 (136e141) (4.1e4.4) (102e105) (0.5e0.8) 138 4.2 103 0.6 (137e145) (4.1e4.3) (105e110) (0.7e0.8) 141 4.2 108.25 0.75 (137e143) (3.8e4.3) (98e110) (0.5e0.9) 139.71 4.1 101.85 0.71 (138e151) (3.9e4.8) (103e118) (0.4e0.8) 142.85 4.3 108.28 0.6
7.385 (7.38e7.39) 25.0 (24e26) 7.382 (7.37e7.39) 25.30 (24.2e26.3) 7.39 (7.37e7.39) 27.75 (26e30) 7.39 (7.37e7.41) 25.71 (23e29) 7.38e7.46 22e29
pH Bicarbonates (mmol/l) Naþ (mEq/l) Kþ (mEq/l) Cl (mEq/l) Creatinine (mg/dl)
3 months: 10 patients (Range) Normal range Biochemical datum
Renal function and acid/base balance. Table 2
Exstrophy-epispadias remains a challenge that is best managed from birth by interested paediatric surgical/urological teams. However, despite improved modern reconstructive techniques, patients often present at a later age, often following surgery, with residual or recurrent urinary incontinence and major lower abdominal and genital distortion that stigmatizes them socially, and markedly reduces their quality of life, their self-esteem, and their educational, job, and life opportunities. In some Middle Eastern countries, surgical expertise, health service provision, and financial, socio-cultural and religious norms often limit their choice of management techniques, with permanent abdominal stomas and self-catheterization being rejected as unhygienic and unacceptable. In addition, in view of their socio-economic circumstances and their total urinary incontinence following bladder surgeries, these children and families only have this one opportunity for definitive ‘life-changing’ surgery for urinary continence. In response to such a background in the present cohort, and considering that all the children had a normal competent anal sphincter, ureteric diversion with a Heitz-Boyer-Hovelacque rectal bladder technique was chosen [4], with a retrorectal colonic pull through and colorectal anastomosis above the external sphincter complex, retaining an intact anus and avoiding possible
12 months: 10 patients (Range)
Discussion
7.401 (7.38e7.41) 25.14 (23e28)
60 months: 10 patients (Range)
84 months: 7 patients (Range)
108 months: 4 patients (Range)
120 months: 2 patients (Range)
basic blood and renal function studies. All patients were clinically well and symptomless, showing normal growth. There were no UTIs. All were continent and dry by day, emptying the rectal bladder every 3e6 h, but two patients (20%) reported accidental loss of urine at night. Blood analyses and acid/base balance were within the normal range (Table 2), with no change in renal function, no outflow tract obstruction and no renal parenchymal scars noted on DTPATc99 scans. Proctoscopy showed a mild intermix of urine and faeces, and the rectal mucosa appeared macroscopically normal throughout. The posterior colorectal anastomosis (Fig. 1A and B) and the ureteric orifices (Fig. 1C and D) were easily identifiable. There was a ‘florid appearance’ of each ureteric orifice that histologically showed mild inflammation and submucosal fibrosis (Fig. 2). Rectal biopsies from close to the ureteric junction and from distant sites were histologically normal. The pre-operative SF-12 psychological and quality of life review highlighted the stigma and social isolation of both the child and family, disrupted education for the child, and the force of the cultural and religious norms that did not accept any form of abdominal stoma or appliance. One year postoperatively, nine children and their accompanying parents repeated the SF-12 assessment, with one refusal by the parent of a 7-year-old male child. The survey showed that the QoL of these patients was improved in terms of social life, friends and family relationships. Body image was an important determinant of QoL for females, but of lesser value for males. When asked specifically, 78% of the patients (all the females and two males) stated that their physical (including genital) appearance had improved and contributed significantly to their increased self-esteem and positive outlook.
7.417 (7.40e7.44) 27.0 (26e28)
M. Messina et al.
135e150 3.5e5.0 95e110 0.5e1.2
+ 1.e4
Please cite this article in press as: Messina M, et al., Continence and quality of life with the modified Heitz-Boyer-Hovelacque rectal bladder for children with urinary incontinence following bladder exstrophy, Journal of Pediatric Urology (2016), http://dx.doi.org/ 10.1016/j.jpurol.2015.12.010
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Heitz-Boyer-Hovelacque rectal bladder for children
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Figure 2 A ‘florid appearance’ of one ureteric orifice in a patient: (A) 10 years after and (B) 15 years after surgery; (C) shows the histological exam that confirmed mild inflammation and submucosal fibrosis.
weakening of the anal sphincter. This limited, but did not eliminate, the admixture of faeces and urine that is considered to be possibly responsible for neoplastic change. The statement in a publication by Malone et al. [8] is encouraging: whereas early urinary diversions to the colon were associated with an increase of sialomucin tumour markers [9e11] and an increased risk of adenocarcinoma, this incidence was not demonstrated in rectal bladders [12] such that ‘rectal bladder procedures do not carry this risk’. However, conscious of the 6e29% incidence of adenocarcinoma at the ureterocolic anastomosis [10e14] at 20e26 years after ureterosigmoidostomy, it was considered prudent to set up life-long monitoring with annual proctoscopy and biopsies. The current experience of this procedure suggests that the modified Heitz-Boyer-Hovelacque rectal bladder technique [4] is a straightforward surgical procedure without short-term complications, and any injury or weakening of the anal sphincter. The rapidity with which these children developed anal control of the rectal bladder was impressive e all the children became dry and odour-free by day within 2e3 weeks; two still had minor urine loss at night. The ability to use normal clothes without bulky protective absorbents was a major boost to the children’s desire to conform to peer fashion and activities. Over the 5- to 15-year follow-up there have been no major longer-term complications e even when the small polypoidal bladders were retained. None of the children had UTIs, renal parenchymal scarring or metabolic disorders related to urine absorption. The lower-pressure ample rectum emptied freely and ureteric reflux did not seem to be a problem, suggesting that antireflux mechanisms are unnecessary and could possibly be detrimental by obstructing free ureteric drainage. At proctoscopy, there was definite, but minimal, intermixing of urine with faeces, with the urine often being clear. Biopsies from the ‘florid’ ureters revealed minor inflammatory changes. However, 11 years after rectal bladder construction, one child required excision of a large inflammatory polyp from the tip of the left ureter because of bleeding from recurrent torsion; there was no recurrence 2 years after excision. None of the rectal and ureteric biopsies from any of the children showed any metaplasia or neoplasia; however, in this respect, the follow-up period was relatively short and longterm follow up is mandatory.
The SF-12 [5] is a reliable instrument that it applicable to children [6], and valid across age, gender and disease. The assessment was performed pre-operatively by an independent psychologist, and was repeated postoperatively at discharge and again 1 year after surgery. The major alteration in QoL and psychological uplift for both child and family, in mood and approach with a positivity and willingness to become involved and to participate in peerrelated activities, was particularly gratifying. Indeed, 78% of the study cohort (all the females and two males) felt that the operation had significantly altered their appearance and body image for the better. By providing continence and freedom from odour without obvious appliances, and by facilitating normal dress, the rectal bladder, despite the potential risk of neoplasia, offers the children valuable years of good-quality life and opportunity at a crucial time in their psychological development.
Conclusion Small patient numbers and only annual ‘hands-on’ access to the children and their families limited the present study; however, relevant medical and nursing personnel followed them locally. The modified Heitz-Boyer-Hovelacque rectal bladder technique is a most valuable asset in the management of unresolved urinary incontinence for children born with exstrophy-epispadias who have a competent anal sphincter, for those where other forms of management have been unsuccessful, or where socio-cultural-religious norms and economic opportunity do not allow otherwise more conventional approaches.
Conflict of interest All authors declare that there are no conflicts of interest. All authors disclose any financial and personal relationships with other people or organisations that could inappropriately influence (bias) their work.
Funding source All authors declare that this study had no sponsor.
Please cite this article in press as: Messina M, et al., Continence and quality of life with the modified Heitz-Boyer-Hovelacque rectal bladder for children with urinary incontinence following bladder exstrophy, Journal of Pediatric Urology (2016), http://dx.doi.org/ 10.1016/j.jpurol.2015.12.010
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Ethical approval This study did not require ethical approval.
References [1] Gearhart JP, Mathews R. Exstrophy e epispadias complex. In: Wein AJ, et al., editors. Campbell-Walsh Urology; 2011. p. 3497e553. Philadelphia. [2] Purves JT, Gearhart JP. The bladder exstrophy-epispadiascloacal exstrophy complex. In: Gearhart JP, Rink RC, Mouriquand PDE, editors. Pediatric Urology; 2010. p. 386e415. Philadelphia. [3] Eber AK, Reutter H, Ludwing M, Rosch WH. The exstrophyepispadias complex. Orphanet J Rare Dis 2009;4:23. [4] Tacciuoli M, Laurenti C, Racheli T. Sixteen years’ experience with the Heitz Boyer-Hovelacque procedure for exstrophy of the bladder. Br J Urol 1977;49:385e90. [5] Ware Jr J, Kosinski M, Keller SD. A 12-Item Short-Form Health Survey: construction of scales and preliminary tests of reliability and validity. Med Care 1996;34:220e33. [6] Jochault-Ritz S, Mercier M, Aubert D. Short and long-term quality of life after reconstruction of bladder exstrophy in infancy: preliminary results of the QUALEX (QUAlity of Life of bladder EXstrophy) study. J Pediatr Surg 2010;45:1693e700.
M. Messina et al. [7] Ransley PG, Duffy PG. Bladder exstrophy closure and epispadias repair. In: Spitz L, Coran AG, editors. Rob & Smith’s operative surgery: Pediatric surgery. London: Chapman & Hall; 1995. p. 745e59. [8] Simon J. Ectopia vesical operation for directing the orifices of the ureters into the rectum; temporary success, subsequent death, autopsy. Lancet 1982;2:568e70. [9] Giordano S, Taskinen S, Sankila A, Ala-Opas M. Adenocarcinoma in isolated rectal bladder after treatment of bladder exstrophy. Scand J Urol Nephrol 2008;42:392e4. [10] Azimuddin K, Khubchandani IT, Stasik JJ, Rosen L, Riether RD. Neoplasia after ureterosigmoidostomy. Dis Colon Rectum 1999;42:1632e8. [11] Pahernik S, Beetz R, Schede J, Stein R, Thu ¨roff JW. Rectosigmoid pouch (Mainz Pouch II) in children. J Urol 2006;175: 284e7. [12] Nitkunan T, Leaver R, Patel HR, Woodhouse CR. Modified ureterosigmoidostomy (Mainz II): a long-term follow-up. BJU Int 2004;93:1043e7. [13] Bastian PJ, Albers P, Haferkamp A, Schumacher S, Mu ¨ller SC. Modified ureterosigmoidostomy (Mainz Pouch II) in different age groups and with different techniques of ureteric implantation. BJU Int 2004;94:345e9. [14] Wagstaff KE, Woodhouse CR, Rose GA, Duffy PG, Ransley PG. Blood and urine analysis in patients with intestinal bladders. Br J Urol 1991;68:311e6.
Please cite this article in press as: Messina M, et al., Continence and quality of life with the modified Heitz-Boyer-Hovelacque rectal bladder for children with urinary incontinence following bladder exstrophy, Journal of Pediatric Urology (2016), http://dx.doi.org/ 10.1016/j.jpurol.2015.12.010
MODEL
Journal of Pediatric Urology (2016) xx, 1.e1e1.e6
Continence and quality of life with the modified Heitz-Boyer-Hovelacque rectal bladder for children with urinary incontinence following bladder exstrophy a
Division of Pediatric Surgery, Department of Medical, Surgical and Neurological Sciences, University of Siena, Siena, Italy
b
Neonatal and Paediatric Reconstructive Surgeon, Manchester, UK Correspondence to: R. Angotti, Division of Pediatric Surgery, Department of Medical, Surgical and Neurological Sciences, University of Siena, Viale Bracci 16, 53100 Italy [email protected] (R. Angotti) Keywords Bladder exstrophy; Urinary incontinence; Rectal bladder; Heitz-Boyer-Hovelacque technique Received 29 July 2015 Accepted 27 December 2015 Available online xxx
M. Messina a, F. Molinaro a, F. Ferrara a, R. Angotti a, A.L. Bulotta a, G. Di Maggio a, A. Bianchi b Summary Background Bladder exstrophy and epispadias are severe congenital anomalies associated with an open bladder and urinary sphincter. Despite modern reconstruction, there is a significant incidence of residual or recurrent urinary incontinence that impacts on quality of life (QoL) and self-esteem, which in turn limits social interaction (Figure). The present study involved 14 patients, mainly from a Middle Eastern country, and reported the early findings with a modification of the Heitz-Boyer-Hovelacque rectal bladder technique for both urinary and faecal control. Study design Fourteen children, with a median age of 8.1 years, with poor quality of life and low self-esteem because of urinary incontinence and small polypoidal open bladders of 5e15 ml volume, mostly after bladder exstrophy surgery, were managed with a modification of the Heitz-Boyer-Hovelacque rectal bladder technique keeping an intact anal sphincter. The retrorectal pulled-through colon was anastomosed to the posterior wall of the rectum just above the external sphincter complex, thereby avoiding any possible injury to the anal sphincter. All patients had a normal colon and a competent anal sphincter without lumbosacral spinal or nerve anomalies. Results Ten children had a 5- to 10-year follow-up, one child had a 15-year follow-up, and three others, that were
Figure
also continent, were excluded because of a <5-year follow-up. There were no postoperative complications, and all were dry and odour-free by day within 2e4 weeks of surgery. Two children still had minor urinary loss at night. There were no UTIs and renal function remained unimpaired. Eleven years after surgery, one child underwent excision of a pedunculated benign inflammatory polyp from the tip of the left ureter because of recurrent torsion and bleeding, there was no recurrence at the 2-year follow-up. None of the rectal or ureteric biopsies from any of the children showed metaplasia or neoplasia; however, in view of the potential long-term risks, all children were placed on a lifelong ‘proctoscopy and biopsy’ protocol. Discussion The ability to be dry and odour-free, and to wear normal clothing had a striking impact on QoL and psychological well-being of the children and their families. This was reflected in their positive overall approach, voluntary school attendance, and enthusiastic participation in communal events. All agreed that their improved genital appearance markedly contributed to their better body image and increased self-esteem. Conclusion These significant benefits, at a crucial time in the child’s life, outweigh the potential risk of long-term neoplasia. Therefore, the Heitz-Boyer-Hovelacque rectal bladder technique is recommended with longterm proctoscopic follow-up.
Female (A) and male (B) anatomy after failed reconstruction.
http://dx.doi.org/10.1016/j.jpurol.2015.12.010 1477-5131/ª 2016 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Messina M, et al., Continence and quality of life with the modified Heitz-Boyer-Hovelacque rectal bladder for children with urinary incontinence following bladder exstrophy, Journal of Pediatric Urology (2016), http://dx.doi.org/ 10.1016/j.jpurol.2015.12.010
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MODEL
1.e2
Introduction Exstrophy-epispadias is a severe congenital anomaly that presents with major disruption of the infraumbilical abdominal wall, a wide diastasis of the pubic symphysis, a variable-volume open bladder and sphincter, and a short open urethra passing; in the male, this is along the dorsum of a foreshortened epispadiac penis [1e3]. The lumbosacral spine and nerves, the anus and posterior pelvic floor are often anatomically and functionally normal. Despite modern reconstructive techniques, residual or recurrent uncontrollable urinary incontinence and odour, the need for diapers, the inability to wear normal clothing, the poor genital aesthetics, and the limitations to social interaction have a significant impact on quality of life (QoL), body image and self-esteem. To the psychologically vulnerable child/adolescent and family, procedures that offer the possibility of being dry and odour-free rank as highly as genital aesthetics and function. In this, as yet small, study of 14 children, the early impressions with a modification of the Heitz-Boyer-Hovelacque rectal bladder technique [4] for anal control of both urinary and faecal streams, avoiding any risk to the anal sphincter, are reported.
Material and methods Thirteen patients (six males, seven females) with a median age of 8.1 years (range: 6e13 years) from a Middle Eastern country, and one European male child with a 15-year follow-up who had a rectal bladder at 7 years of age at another institution, were referred because of residual or recurrent urinary incontinence following unoperated or failed bladder exstrophy surgery (seven patients had two failed attempts). Three children with a follow-up of <5 years, but presently doing well, were excluded. The remaining 10 patients (four males, six females) with a 5- to 10-year follow-up were managed between 2004 and 2009. There had not been any pelvic osteotomies and all children had a wide pubic symphyseal diastasis, averaging 7.3 cm (range: 5e10 cm). All had a normal lumbosacral spine and a competent anal sphincter. All male patients had epispadias, two had bilateral undescended testes, and one had a unilateral indirect inguinal hernia. Females had a wide mons pubis, divergent labia, a bifid clitoris, and a short vagina; two also had vaginal stenosis. All patients were originally assessed in their own country and informed consent was obtained with the help of an acceptable interpreter, even prior to travel to the present institution. On admission, a detailed review, a full clinical examination, and a preoperative work-up were undertaken. These included: a blood film, blood group, serum urea, serum electrolytes and creatinine, electrocardiogram, abdominal and renal ultrasound, chest X-ray, lumbosacral spine and pelvic X-ray, and magnetic resonance imaging of the pelvis; a barium enema and anorectal manometry confirmed normal anatomy and function. An independent psychologist undertook a pre-operative psychological and quality of life review using the Short Form Health Survey 12 (SF-12) [5,6] that measures eight concepts of health status (i.e. physical functioning, role limitations due to physical health problems, bodily pain,
M. Messina et al. general health, vitality (energy/fatigue), social functioning, role limitations due to emotional problems, and mental health (psychological distress and psychological well-being). The first four health concepts indicate physical health status and the others are an index of mental health. The assessment was repeated postoperatively just prior to discharge and again 1 year after surgery. The mean age for rectal bladder reconstruction was 9.1 years (range: 7e14 years). No pelvic osteotomies were performed. All had significant lower abdominal and perivesical scarring from previous surgery. The open bladders were of 5e15 ml volume and filled with mucosal polyps. Six patients had excision of the polyps and closure of the native bladder, and four patients had resection of their rigid and small-volume bladder, retaining the bladder neck and prostatic urethra for the three males, in continuity with the tubularized urethral plate. At the same procedure, girls also had a single-stage genital reconstruction (not the subject of this paper). Boys had the first of a two-stage reconstruction consisting of penile lengthening by corpora cavernosa release from the pubic rami, Ransley corporal rotation [7] and glanulo-meatoplasty, and an intercorporal transfer of the urethra to a midline penoscrotal meatus. The second-stage urethroplasty with transfer of the urethral meatus to the glans was undertaken 12 months later. Prior to discharge from hospital, all patients had a renal ultrasound, a DTPA-Tc99 scan, and a proctoscopy under general anaesthesia. Baseline biopsies for neoplasia were taken
Table 1
Postoperative management.
Exam
When
Blood pressure
Daily during hospitalisation, every 15 days during the first two months, every 3 months during the first year and then every 6 months Every 15 days during the first two months, every 3 months during the first year and then every 6 months According to the needs of the individual patient Every 15 days during the first two months, every 3 months during the first year and then every 6 months At 15e21 day after surgery and then every year
Blood sample evaluation with renal function and blood gases Teaching and learning of sphincter control Ultrasound
Endoscopy with URETERORECTAL ANASTOMOSIS evaluation and Colorectal anastomosis evaluation and dilatation Renal scintigraphy with DTPA TC99 Histological examination of the rectal bladder biopsies
At 15e21 day after surgery and then according to the condition of anastomosis At 6e12 months after surgery, to be repeated in case of stenosis Every year
Please cite this article in press as: Messina M, et al., Continence and quality of life with the modified Heitz-Boyer-Hovelacque rectal bladder for children with urinary incontinence following bladder exstrophy, Journal of Pediatric Urology (2016), http://dx.doi.org/ 10.1016/j.jpurol.2015.12.010
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MODEL
Heitz-Boyer-Hovelacque rectal bladder for children from the tip of each ureter, from the rectal mucosa adjacent to the ureteric orifices and at a distant rectal site (Table 1).
Operative technique All patients underwent rigorous pre-operative colonic preparation. All surgery was undertaken by the two senior surgeons (MM, AB) working together and assisted by experienced surgical staff. Under general anaesthesia, with the patient in the lithotomy position to allow access to the anus and the lower abdominal wall, the abdomen and peritoneum were opened, any infraumbilical midline abdominal scar was excised and any bladder that was over-sewn or excised was detached. The sigmoid colon and rectum were identified and, in accordance with patient size and age, 200e300 ml of warm 0.9% saline was infused through a transanal Foley catheter (24 F), with the balloon inflated to occlude the anus, to comfortably distend the rectum that was then divided and over-sewn in the form of a Hartman’s procedure. The retrorectal space was developed by blunt dissection and a large Hegar dilator was passed down to the level of the pelvic floor. Working through the anus, a full-thickness incision was made
1.e3 onto the Hegar dilator for one-half of the posterior rectal circumference just above the external anal sphincter complex that was kept intact. The sigmoid colon was passed through the retrorectal space and a liberal end-to-side colorectal anastomosis was performed (Fig. 1A and B). Through the abdominal incision, each ureter was divided close to the bladder, cannulated with an 8-F feeding tube, and anastomosed to the ipsilateral side of the rectal bladder at about 10e15 cm from the anal margin, with the stents draining into the rectal lumen. No antireflux mechanism was constructed. The abdominal wall was closed, burying the bladder beneath available layers, leaving a 10-F Jackson-Pratt catheter (Teleflex) draining the pelvis. An aesthetic umbilicoplasty and genital reconstruction (not described in this paper) was undertaken.
Results and postoperative follow-up Within a lifetime monitoring protocol, all patients had an annual review (Table 1) that averaged 7.7 years (range: 5e15 years). The protocol included: a clinical review, a psychological assessment, a physical examination, and
Figure 1 (A) and (B) show a colorectal anastomosis: (A) is a schematic drawing; (B) is the endoscopic image of a patient. (C) and (D) show ureteric orifices: (C) is a schematic drawing; (D) is the endoscopic image of a patient.
Please cite this article in press as: Messina M, et al., Continence and quality of life with the modified Heitz-Boyer-Hovelacque rectal bladder for children with urinary incontinence following bladder exstrophy, Journal of Pediatric Urology (2016), http://dx.doi.org/ 10.1016/j.jpurol.2015.12.010
MODEL
(143e145) (4.1e4.7) (105e107) (0.9e1.3) 144 4.4 106 1.1 (139e141) (4.2e4.3) (103e105) (0.6e0.8) 140 4.1 104 0.7 (136e141) (4.1e4.4) (102e105) (0.5e0.8) 138 4.2 103 0.6 (137e145) (4.1e4.3) (105e110) (0.7e0.8) 141 4.2 108.25 0.75 (137e143) (3.8e4.3) (98e110) (0.5e0.9) 139.71 4.1 101.85 0.71 (138e151) (3.9e4.8) (103e118) (0.4e0.8) 142.85 4.3 108.28 0.6
7.385 (7.38e7.39) 25.0 (24e26) 7.382 (7.37e7.39) 25.30 (24.2e26.3) 7.39 (7.37e7.39) 27.75 (26e30) 7.39 (7.37e7.41) 25.71 (23e29) 7.38e7.46 22e29
pH Bicarbonates (mmol/l) Naþ (mEq/l) Kþ (mEq/l) Cl (mEq/l) Creatinine (mg/dl)
3 months: 10 patients (Range) Normal range Biochemical datum
Renal function and acid/base balance. Table 2
Exstrophy-epispadias remains a challenge that is best managed from birth by interested paediatric surgical/urological teams. However, despite improved modern reconstructive techniques, patients often present at a later age, often following surgery, with residual or recurrent urinary incontinence and major lower abdominal and genital distortion that stigmatizes them socially, and markedly reduces their quality of life, their self-esteem, and their educational, job, and life opportunities. In some Middle Eastern countries, surgical expertise, health service provision, and financial, socio-cultural and religious norms often limit their choice of management techniques, with permanent abdominal stomas and self-catheterization being rejected as unhygienic and unacceptable. In addition, in view of their socio-economic circumstances and their total urinary incontinence following bladder surgeries, these children and families only have this one opportunity for definitive ‘life-changing’ surgery for urinary continence. In response to such a background in the present cohort, and considering that all the children had a normal competent anal sphincter, ureteric diversion with a Heitz-Boyer-Hovelacque rectal bladder technique was chosen [4], with a retrorectal colonic pull through and colorectal anastomosis above the external sphincter complex, retaining an intact anus and avoiding possible
12 months: 10 patients (Range)
Discussion
7.401 (7.38e7.41) 25.14 (23e28)
60 months: 10 patients (Range)
84 months: 7 patients (Range)
108 months: 4 patients (Range)
120 months: 2 patients (Range)
basic blood and renal function studies. All patients were clinically well and symptomless, showing normal growth. There were no UTIs. All were continent and dry by day, emptying the rectal bladder every 3e6 h, but two patients (20%) reported accidental loss of urine at night. Blood analyses and acid/base balance were within the normal range (Table 2), with no change in renal function, no outflow tract obstruction and no renal parenchymal scars noted on DTPATc99 scans. Proctoscopy showed a mild intermix of urine and faeces, and the rectal mucosa appeared macroscopically normal throughout. The posterior colorectal anastomosis (Fig. 1A and B) and the ureteric orifices (Fig. 1C and D) were easily identifiable. There was a ‘florid appearance’ of each ureteric orifice that histologically showed mild inflammation and submucosal fibrosis (Fig. 2). Rectal biopsies from close to the ureteric junction and from distant sites were histologically normal. The pre-operative SF-12 psychological and quality of life review highlighted the stigma and social isolation of both the child and family, disrupted education for the child, and the force of the cultural and religious norms that did not accept any form of abdominal stoma or appliance. One year postoperatively, nine children and their accompanying parents repeated the SF-12 assessment, with one refusal by the parent of a 7-year-old male child. The survey showed that the QoL of these patients was improved in terms of social life, friends and family relationships. Body image was an important determinant of QoL for females, but of lesser value for males. When asked specifically, 78% of the patients (all the females and two males) stated that their physical (including genital) appearance had improved and contributed significantly to their increased self-esteem and positive outlook.
7.417 (7.40e7.44) 27.0 (26e28)
M. Messina et al.
135e150 3.5e5.0 95e110 0.5e1.2
+ 1.e4
Please cite this article in press as: Messina M, et al., Continence and quality of life with the modified Heitz-Boyer-Hovelacque rectal bladder for children with urinary incontinence following bladder exstrophy, Journal of Pediatric Urology (2016), http://dx.doi.org/ 10.1016/j.jpurol.2015.12.010
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Heitz-Boyer-Hovelacque rectal bladder for children
1.e5
Figure 2 A ‘florid appearance’ of one ureteric orifice in a patient: (A) 10 years after and (B) 15 years after surgery; (C) shows the histological exam that confirmed mild inflammation and submucosal fibrosis.
weakening of the anal sphincter. This limited, but did not eliminate, the admixture of faeces and urine that is considered to be possibly responsible for neoplastic change. The statement in a publication by Malone et al. [8] is encouraging: whereas early urinary diversions to the colon were associated with an increase of sialomucin tumour markers [9e11] and an increased risk of adenocarcinoma, this incidence was not demonstrated in rectal bladders [12] such that ‘rectal bladder procedures do not carry this risk’. However, conscious of the 6e29% incidence of adenocarcinoma at the ureterocolic anastomosis [10e14] at 20e26 years after ureterosigmoidostomy, it was considered prudent to set up life-long monitoring with annual proctoscopy and biopsies. The current experience of this procedure suggests that the modified Heitz-Boyer-Hovelacque rectal bladder technique [4] is a straightforward surgical procedure without short-term complications, and any injury or weakening of the anal sphincter. The rapidity with which these children developed anal control of the rectal bladder was impressive e all the children became dry and odour-free by day within 2e3 weeks; two still had minor urine loss at night. The ability to use normal clothes without bulky protective absorbents was a major boost to the children’s desire to conform to peer fashion and activities. Over the 5- to 15-year follow-up there have been no major longer-term complications e even when the small polypoidal bladders were retained. None of the children had UTIs, renal parenchymal scarring or metabolic disorders related to urine absorption. The lower-pressure ample rectum emptied freely and ureteric reflux did not seem to be a problem, suggesting that antireflux mechanisms are unnecessary and could possibly be detrimental by obstructing free ureteric drainage. At proctoscopy, there was definite, but minimal, intermixing of urine with faeces, with the urine often being clear. Biopsies from the ‘florid’ ureters revealed minor inflammatory changes. However, 11 years after rectal bladder construction, one child required excision of a large inflammatory polyp from the tip of the left ureter because of bleeding from recurrent torsion; there was no recurrence 2 years after excision. None of the rectal and ureteric biopsies from any of the children showed any metaplasia or neoplasia; however, in this respect, the follow-up period was relatively short and longterm follow up is mandatory.
The SF-12 [5] is a reliable instrument that it applicable to children [6], and valid across age, gender and disease. The assessment was performed pre-operatively by an independent psychologist, and was repeated postoperatively at discharge and again 1 year after surgery. The major alteration in QoL and psychological uplift for both child and family, in mood and approach with a positivity and willingness to become involved and to participate in peerrelated activities, was particularly gratifying. Indeed, 78% of the study cohort (all the females and two males) felt that the operation had significantly altered their appearance and body image for the better. By providing continence and freedom from odour without obvious appliances, and by facilitating normal dress, the rectal bladder, despite the potential risk of neoplasia, offers the children valuable years of good-quality life and opportunity at a crucial time in their psychological development.
Conclusion Small patient numbers and only annual ‘hands-on’ access to the children and their families limited the present study; however, relevant medical and nursing personnel followed them locally. The modified Heitz-Boyer-Hovelacque rectal bladder technique is a most valuable asset in the management of unresolved urinary incontinence for children born with exstrophy-epispadias who have a competent anal sphincter, for those where other forms of management have been unsuccessful, or where socio-cultural-religious norms and economic opportunity do not allow otherwise more conventional approaches.
Conflict of interest All authors declare that there are no conflicts of interest. All authors disclose any financial and personal relationships with other people or organisations that could inappropriately influence (bias) their work.
Funding source All authors declare that this study had no sponsor.
Please cite this article in press as: Messina M, et al., Continence and quality of life with the modified Heitz-Boyer-Hovelacque rectal bladder for children with urinary incontinence following bladder exstrophy, Journal of Pediatric Urology (2016), http://dx.doi.org/ 10.1016/j.jpurol.2015.12.010
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1.e6
Ethical approval This study did not require ethical approval.
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Please cite this article in press as: Messina M, et al., Continence and quality of life with the modified Heitz-Boyer-Hovelacque rectal bladder for children with urinary incontinence following bladder exstrophy, Journal of Pediatric Urology (2016), http://dx.doi.org/ 10.1016/j.jpurol.2015.12.010