Quantified outcome for patients with bladder exstrophy

Journal of Pediatric Urology (2013) 9, 298e302

Quantified outcome for patients with bladder exstrophy Ravi Prakash Kanojia*, Sumit Agarwal, Kirti K.J. Rathod, Monika Bawa, Prema Menon, Jai K. Mahajan, Ram Samujh, K.L.N. Rao Department of Pediatric Surgery, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India Received 1 November 2011; accepted 2 April 2012 Available online 16 May 2012

KEYWORDS Bladder exstrophy; Bladder augmentation; Continence; Health-related quality of life; Psychosocial outcome

Abstract Aim: Outcomes of bladder exstrophy patients were studied in numerical terms using scoring systems for continence and health-related quality of life (HRQOL), along with renal function, in short-term follow up. Patients and methods: Patients who had undergone bladder-preserving exstrophy repairs, either staged or as a single procedure, and those who had been managed by bowel augmentation were included. Continence was assessed according to five variables (max. score 15) and HRQOL assessment was by means of a structured modified questionnaire scored on a Likertscale model (max. score 150). Results: A total of 39 patients were followed. Mean age was 8.3 years and mean follow up duration 3 years. Mean HRQOL score was 107.55 (83e133, SD
19.31). Mean continence score was 8.73 (6e11, SD
1.544). Conclusions: Evaluation of bladder exstrophy outcomes should not be done merely by reporting the length of dry intervals. If performed in numerical terms as outlined in this series, patients across centres will be comparable over a common assessment protocol. Continence score achieved in this series was low in comparison to the literature due to the strict evaluation protocol. Crown Copyright ª 2012 Published by Elsevier Ltd on behalf of Journal of Pediatric Urology Company. All rights reserved.

Introduction Bladder exstrophy is a difficult condition to manage. Even after multiple attempts at repair, the level of continence

achieved is variable. The outcome of bladder exstrophy is measured in terms of continence, upper tract deterioration, health-related quality of life (HRQOL), satisfaction with genitalia reconstruction, sexual function and fertility

* Corresponding author. Block 3A, Room No 3103, Advanced Pediatric Center, Post Graduate Institute of Medical Education and Research (PGIMER), Sector 12, Chandigarh 160012, India. Tel.: þ91 9914208331 (mobile). E-mail address: [email protected] (R.P. Kanojia). 1477-5131/$36 Crown Copyright ª 2012 Published by Elsevier Ltd on behalf of Journal of Pediatric Urology Company. All rights reserved. doi:10.1016/j.jpurol.2012.04.006

Quantified outcome for bladder exstrophy

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[1]. Several studies have provided results of exstrophy treatment with regard to all or some of these factors across various age groups [2,3]. There are few studies published that have combined an HRQOL survey with continence and renal function. Lack of a common evaluation protocol makes it difficult to compare these series of patients. The importance of standardization for evaluation of continence and HRQOL in conditions like exstrophy has never been stressed in the literature. Different surgeons across the world report outcomes that are not comparable due to different variables being used and the subjective nature of evaluation. The aim of this paper was to present an objectivized standardized format for evaluation of outcomes of bladder exstrophy. The same format is used for patients of mutually non-exclusive groups (bladder-preserving exstrophy repairs and augmentationbased repairs) to report the results. The described protocol can be applied to any other group of patients, for comparison across series.

Patients and methods This was a bidirectional study (5-year retrospective study conducted from July 2004 to June 2009 and 1-year prospective study from July 2009 to June 2010) involving two mutually non-exclusive groups of patients. Group I had undergone bladder-preserving exstrophy repairs either as a staged or single-stage procedure. Group II were patients who had been managed by bowel augmentation. Patients

Figure 1

who had undergone repair outside our institute, those with less than 1-year of follow up, and those with incontinent epispadias were excluded. The protocol followed for the management of bladder exstrophy was that patients presenting early with a pliable non-polypoidal bladder plate were subjected to bladderpreserving repair either as staged or single-stage surgery. Late on, when a subset of these patients demonstrated incontinence with minimal bladder growth, they were subjected to bladder augmentation. At the beginning of the study, a scheme was developed under which the patients were evaluated. The various components of the scheme are depicted in Fig. 1. As can be seen, this is a three-dimensional system with two objectivized arms measuring HRQOL and continence. The third arm measures renal status in terms of dilatation, scars, reflux and pyelonephritis. HRQOL or psychosocial assessment was done by means of a structured questionnaire as provided by KIDSCREEN-52 [4]. The questionnaire was modified to suit the study population. The modified questionnaire was not validated as there was no control group. The questions cover six dimensions representing various aspects of physical and psychosocial well being. These are: physical well being and participation in daily life activities, school environment and performance, participation in group activities, social support and relationship with peers, self perception and satisfaction with genitalia reconstruction, and psychological well being. The response was recorded on a Likert-scale model ‒ never, seldom, sometimes, often, always ‒ represented by a 1e5

Scheme of assessment.

300 Table 1

R.P. Kanojia et al. Continence assessment scheme based on five variables with scoring.

Continence

3

2

1

Ability to micturate Dry interval duration

Via natural orifice >2.5 h during day, >4 h during night Always None

Occasional catheterization 1.5e2.5 h during day, 3e4 h during night Occasional Occasional (<4/month), needs fluid restriction Occasionally

Always needs catheterization <1.5 h during day, <3 h during night Never Multiple times (>4/month)

Ability to sense bladder filling Nocturia

Ability to withhold micturition Always Score: 5‒7 poor (incontinent), 8‒11 average, >11 good (continent).

Never

point scoring system, the maximum score being 150 (<100 Z poor, 101‒120 Z average, >121 Z good). The responses to the questionnaire were taken from the parents if the patient was too young to answer. HRQOL for the study groups was not compared with the general population. The continence assessment was objectivized as per Fig. 1 and Table 1. This continence scoring system includes five possible components: route of bladder emptying, duration of dry interval, sensation of bladder filling, nocturia, and ability to withhold micturition. If applied to a normal human being, whether child or adult, they will always score maximum. The status of the upper tract was assessed for dilatation, pyelonephritic changes and reflux. These were determined by serum creatinine, renal ultrasound, micturating cysto-urethrogram and dimercaptosuccinic acid (DMSA) renal scan. Ancillary data such as metabolic complications and salvage procedures required were also recorded. The study was approved by the institute’s ethics committee.

range of 8e11 (average); none of the patients in group I had a score above 10. In group II, 16 patients had a score between 8 and 10, 1 had a score above 10 and 1 had a score below 7. The data shows that 42% (9/21) of group I patients were incontinent even after complete repair, and 38% (8/21) had scored a poor quality of life. Small capacity bladder was the cause of incontinence in half of these patients. Regarding the group II patients, 88% (16/18) had average continence (i.e. score 8e11). Only one patient was incontinent and is under evaluation. The colo-cystoplasty patients fared better and had a higher mean continence score than the ileo-cystoplasty patients, and 88% (16/18) of all the group II patients had a good quality of life with a score of >100/ 150. With regard to renal function (Table 3), serum creatinine was normal in both groups, and hydronephrosis consequent to VUR was comparable in both groups.

Results and observations

In this study, we have tried to determine the quality of life along with continence for bladder exstrophy patients in numeric terms during the short-term follow up. The study also outlines a standard protocol for the follow up evaluation of these patients, which may provide a benchmark for the comparison of subsequent studies. Objectification of the parameters measured is necessary in order to get uniformity across studies. We measured continence and HRQOL status on predefined scales, with each patient achieving specific scores after assessment so making them comparable with the other patients in the study. KIDSCREEN-52 [4] was formulated by a European

A total of 39 patients (8 females) were evaluated. Group I had 21 patients (7 females) and group II had 18 patients (1 female). The patients’ characteristics are given in Table 2. The HRQOL, continence and renal status assessment data are given in Table 3. The type of augmentation done for group II patients was colo-cystoplasty in 13 (4 with Mitrofanoff), ileo-cystoplasty in 3 and gastro-cystoplasty in 2. There were no cases of metabolic derangement associated with augmentation. In group I, 9 patients had a continence score within the range of 5e7 (poor) and 12 within the

Table 2

Discussion

Patient and treatment characteristics.

Mean age at assessment Mean duration of follow up Mean age at completion of all surgeries/augmentation Reason for augmentation Initial operative procedure

All patients (n Z 39)

Group I (n Z 21)

Group II (n Z 18)

8.3 years 3.03 years 5.1 years

5.7 years 2.7 years 3 years

11.52 years 4.13 years 7.3 years

e

Small capacity bladder with incontinence Total primary repair in 8, staged repair in 10

Bladder closure in 10, total primary repair in 11

Quantified outcome for bladder exstrophy Table 3

301

Assessment scores for various outcome variables. All patients (n Z 39)

HRQOL score (max. Z 150) Continence score (max. Z 15) Renal status Serum creatinine

Group I (n Z 21)

107.55 (83e133, SD
19.31) 102.9 (range 83e133, SD
11.18) 8.73 (6e11, SD
1.544) 7.76 (7e9, SD
0.76)

0.585 (0.2e1.4, SD
0.0292) Hydronephrosis 7 Vesicoureteric reflux (VUR) 27 Scars on DMSA 7

group as a generic health-related quality of life questionnaire for children and adolescents. The questionnaire originally measures HRQOL in 10 dimensions. This was modified to suit the study population, to produce a score which tell us about the actual psychosocial status of the patient. Similarly, the continence score was objectivized by measuring it from five aspects over a point scale. So, the best of the exstrophy patients should have a psychosocial score of 120 and above out of 150, and a continence score of 11 and above out of 15. A few other authors have studied the HRQOL outcome using similar scales. Ebert et al. [5] used a tailor-made questionnaire and assessed the response on a Likertscale. This series also assessed continence and genital function. The continence rate was reported as 83% in their non-referred patients. The same group reported a similar series [1] of 17 adult male patients of whom 16 were continent. Hammouda and Kotb [2] published their early experience with 33 cases and reported 72.7% continence. A more comprehensive HRQOL study was published by Jochault-Ritz et al. [6]. They reported that 12% of children, 65% of adolescents and 77% of adults were continent, and also that adolescent quality of life was better than in the other age groups. They concluded that functional results directly affect the HRQOL score. Diseth et al. [7] performed a systematic overview of exstrophy and epispadias outcome studies, and reported that only 10 studies dealt with the mental and psychosocial aspect. They concluded that the findings had clinical implications. It should be noted that these studies measured outcomes across age groups. The somatic and mental problems that may be experienced by a toddler, an adolescent and an adult are completely different and therefore not comparable. The continence scores reported in the present study are significantly on the lower side as compared to those reported in the literature. We believe that the reason for this discrepancy is that the other authors measured continence solely according to duration of dry interval [3,8e10] (day and night), whereas we based our assessment on all the five parameters given in Table 1. According to these criteria, a patient who was dry for >2.5 h during the day but was only able to achieve this by clean intermittent catheterization would score lower than a similar patient who could void per urethra. Very few reports [1,11] have measured continence beyond the dry interval criterion. We suspect that if all the continent

Group II (n Z 18) 112.2 (range 88e127, SD
10.19) 9.7 (6e13, SD
1.56)

0.57 (0.2e0.8, SD
0.13) 0.6 (0.4e1.4, SD
0.21) 4 (1 bilateral) 11 (7 bilateral) 3

3 (2 bilateral) Bilateral grade II Z 7, I Z 4, III Z 5 5 (2 bilateral)

patients from the above-mentioned studies were subjected to reassessment on the given 5-point scale, or assessed by questionnaire as done by von Gontard and Neve ´us [12], they would score lower than reported. This statement does require validation in a separate study. According to the strict continence assessment scoring system, the bladder-preserving group showed a 58% continence rate with poor bladder growth as the main cause. Since most of the patients did not present in the neonatal age group, the mean age of completion of all surgeries was 3 years. Such late reconstruction means that the bladder undergoes inflammatory damage which later hinders growth even after repair and giving outlet resistance. Several of our patients in the bladder augmentation group in early follow up were able to sense bladder filling and empty per urethra by abdominal pressure. These patients therefore had a satisfying outcome in terms of both HRQOL and continence. Poor continence directly affects quality of life, as can be quantified by the provided questionnairebased score. The desired scores for continence and psychosocial outcomes are 11e15 and 121‒150, respectively. The present study provides mean scores for the two outcome measures in both groups I and II that fall short of the desired range (see Table 3). To conclude, this study provides a short-term follow up of our results with bladder exstrophy patients from a tertiary care centre. The patients who do not achieve continence do better after augmentation as reflected by better continence and psychosocial scores. The continence rate is not comparable to the literature because of delayed presentation leading to poor bladder growth and a stricter assessment methodology. Through this evaluation, we have tried to develop a format for assessing bladder exstrophy patients at our institute, to give us a quantification of patient outcome in numeric terms. If this is applied across future studies, it will make comparison between studies possible.

Source of support None.

Conflict of interest None.

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Funding None.

[6]

Acknowledgement None.

[7]

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