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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
Journal of Pediatric Surgery (2010) 45, 1693–1700
www.elsevier.com/locate/jpedsurg
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 Sandy Jochault-Ritz a , Mariette Mercier b , Didier Aubert a,⁎ a
Saint-Jacques University Hospital, 2, Place Saint Jacques, 25030 Besançon Cedex-France EA 3181, University of Franche-Comté, 25030 Besançon Cedex-France
b
Received 1 September 2009; revised 26 March 2010; accepted 28 March 2010
Key words: Exstrophy; Quality of life; Long-term outcome; Continence; Sexuality
Abstract Purpose: The aim of the study was to assess quality of life (QOL) of patients born with bladder exstrophy (BE) and reconstructed during early childhood in 7 French university hospitals (QUALEX study: QUAlity of Life of bladder EXstrophy). Methods: Patients from 6 to 42 years old answered self-administered Short-Form 36 (SF-36), VSP-A (Vécu et Santé Perçue de l'Adolescent), VSP-AE (Vécu et Santé Perçue de l'Enfant), AUQUIE (AUtoQUestionnaire Imagé de l'Enfant), and general questionnaires about functional and socioeconomic data. Dimension scores were compared between adults and adolescents using SF-36 and adolescents and children using VSP-AE. Scores were also compared to the general French population. Results: Among the 134 eligible patients, 36 adults, 18 adolescents, and 17 children answered the questionnaire. There was no difference between responders and nonresponders in reconstruction criteria. Continence was achieved in 77% of adults, 65% of adolescents, and 12% of children. Adolescent QOL was globally superior to adults and children. Adult QOL was globally lower than the general population except on the physical dimension. Children's QOL was also globally lower than the general population except for relations with family and school work. Adolescents' scores on SF-36 were superior to the general population but lower on half of the dimensions with VSP-AE. Conclusion: Patients presenting with reconstructed BE have impaired QOL, and functional results seem to be the most likely predictive factor of health-related QOL score. © 2010 Elsevier Inc. All rights reserved.
Bladder exstrophy (BE) is a rare but serious birth defect whose reconstruction, although now more codified, still remains the subject of controversy. Long-term results are judged in continence [1], aesthetic results, sexual function [2,3], and fertility [4]—the mains aspects that can impact on quality of life (QOL) throughout the patient's life. Many ⁎ Corresponding author. Tel.: +33 3 81 21 82 21; fax: +33 3 81 21 86 40. E-mail address: [email protected] (D. Aubert). 0022-3468/$ – see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2010.03.032
studies have been published reporting the long-term outcome of reconstruction [5-8] to assess which kind of reconstruction gives the best results in continence or sexual function [9]. Few studies have evaluated patient QOL [10,11], and only some have used health-related QOL (HRQOL) questionnaires [11-13]. The major difficulty is that because of the rarity of this malformation, most studies only have small sample sizes and, therefore, lack the power to identify factors that influence QOL.
1694 With the progress in antenatal diagnosis [14], pediatric surgeons need to have precise and reliable information based on scientific findings to adequately inform parents [15]. This information is even more essential in countries where medical pregnancy termination is accepted for fetuses presenting this malformation. The information must include functional results of surgical treatment and information about QOL in patients. The QUALEX (QUAlity of life of bladder EXstrophy) study is a French national study the primary aim of which was to assess the QOL of adults, adolescents, or children born with classical BE using validated HRQOL questionnaires. The secondary objective was to identify factors influencing patient's QOL such as functional results (urinary continence …), surgical history (type of reconstruction, number of surgical procedures, occurrence of complications …), or socioeconomic data. These preliminary results on a limited sample report characteristics of BE reconstruction and compare HRQOL between different age groups: children vs adolescents and adolescents vs adults.
1. Patients and methods 1.1. Study population and data collection Patients born with BE in France between 1962 and 2001 and underwent reconstruction before the age of 2 were included. Isolated epispadias was ruled out. Patient files were reviewed retrospectively in 7 French university hospitals (Besançon, Lille, Marseille Nord, Paris-Trousseau, Reims, Strasbourg, and Toulouse). The data collected included the number of surgical procedures, the type of surgical procedure used for reconstruction, and the type and number of surgical procedure used to achieve urinary continence. The different questionnaires were sent by post asking patients or their parents to answer questions about their life (level of education, monthly family income, marital status, number of children) and functional results (urinary continence and sexual activity) and to fill out the appropriate HRQOL questionnaires. After a month, nonresponders were contacted by telephone.
1.2. HRQOL questionnaires The HRQOL questionnaires were adapted to each age group: Short-Form 36 (SF-36) for adults; SF-36,Vécu et Santé Perçue de l'Adolescent (VSP-A), and Vécu et Santé Perçue de l'Enfant (VSP-AE) for adolescents (12-17 years old); and VSP-AE and AUto-QUestionnaire Imagé de l'Enfant for children (AUQUIE) (6-12 years old). Short-Form 36 is the French translation [16] and adaptation of the Medical Outcome Study Short Form 36 Health Survey (MOS SF-36) [17]. It is a generic health status questionnaire and can be used to assess health status
S. Jochault-Ritz et al. independent of the disease or illnesses affecting the population. It can be self-administered to adults as well as adolescents from 14 years old. It comprises 36 questions divided into 8 scales: physical functioning, role limitation related to physical health, bodily pain, general health perceptions, vitality, social functioning, emotions, and role limitations relating to mental health. The number of questions in each dimension ranges from 2 to 10, and questions have 2 to 6 answer modalities. Dimension scores were calculated by adding the answers from each dimension, followed by a linear transformation to obtain a score ranging from 0 (worst) to 100 (best). Missing data were estimated by the mean value of the other questions of the same dimension, if more than half the questions were answered. The VSP-AE questionnaire is made up of 39 items plus one open question. Eight dimension scores were computed: self-esteem; general well-being; vitality; school performance; leisure activities; relations with friends; relation with medical staff; and global VSP-AE index. VSP-A comprises 38 items and one open question. Eleven dimensions were computed: vitality; psychological well-being; relations with friends, parents, teachers, and medical staff; leisure activities; physical well-being; school work; selfesteem; and sentimental and sexual life. Each question has 5 answer modalities. Dimension scores and missing data were treated as in the SF-36 [18-20]. AUQUIE is a questionnaire that has been developed for children aged 6 to 12 years. It begins with an open independent question designed to verify the child's capacity to answer the questions. Twenty-six questions with 4 modalities of answers evaluate family and social relationships, activity, health, global functioning (sleep, appetite), and separation. A global score and dimension scores were computed by adding the answers of the questions in each dimension.
1.3. Statistical analysis The primary end point of the QUALEX study was to evaluate factors related to the surgical procedures, functional results, and everyday life that influence HRQOL scores. The secondary end point was to compare the QOL scores of adolescents and adults using SF-36 and between adolescents and children using VSP-AE. These scores were compared with those of French general population as described in the literature [21,22]. To detect a difference of 10 points on a scale of 100 between 2 mean dimension scores, with α = 5% and β = 15%, the number of patients needed in each group was 110. Comparisons between responders and nonresponders and between the 3 age groups were performed using Fisher's Exact test or χ2 test for categorical variables and t test or variance analysis for quantitative variables. Comparison of SF-36 dimension scores between adults and adolescents was performed with t test, and comparisons of VSP-AE dimensions between adolescents and children was performed using nonparametric Wilcoxon test. Bivariate analysis was
QOL after bladder exstrophy reconstruction
1695
performed to identify potential correlations between patient characteristics and functional results and each HRQOL dimension score for each age group (SF-36 for adults, VSPA for adolescents, and VSPAE for children). To take into account the multiplicity of comparisons, a P b .01 was considered significant. Data collection and statistical analyses were performed using the Statistical Analysis Software (version 9.2; SAS Institute, Cary, NC). This study received approval from the local ethics committee and was sponsored by the French national program for clinical research (Programme Hospitalier de Recherche Clinique). Informed consent was obtained from all participants (and from both children and their parents).
2. Results Between April 2008 and June 2009, 134 patients were eligible. Preliminary results are reported concerning the 71 patients who responded.
2.1. Patient characteristics Patient characteristics are shown in Table 1. No statistical difference was found for the sex repartition in the 3 groups. There was no difference in patients' father's level of education. Mothers of adolescents and children had a higher education level than the adults' mother (P = .005). Table 1
Among the adults, 56% had less than a high school degree. Thirty-two percent of adult male patients and 54% of women were married (no difference); 2 of the men and 6 of the women had children; and 1 of the men had had recourse to medically assisted procreation (sperm donor). Intermittent clean catheterization (ICC) was more frequent among adults and adolescents than among children.
2.2. Characteristics of surgical reconstruction by age group Details are shown in Table 2. There was no significant difference between the 3 groups except for mean age at urethral reconstruction for boys and mean number of surgical procedures. Mean age at reconstruction ranged from 6.5 years in the adults' group to 1.2 in the children's group (P b .0001). Mean number of surgical procedures was 12.8 for adults and 6.2 for children (P = .001). Antenatal diagnosis was not very frequent. Age at bladder closure in days was lower in the children's group. Initial bladder closure consisted mainly of simple bladder closure, complete primary repair of exstrophy was used in 4 boys and adolescents, modern staged repair of exstrophy in 1 adolescent and 5 children, and ureterosigmoidostomy was performed in 3 adults. Associated osteotomy was performed in less than half of cases, more often for girls (65.5% vs 38.5%) and when surgery was performed after the third day of life. Post surgical immobilization (mostly external fixator or traction) was more frequent if bladder closure occurred after the third
Patient characteristics
n Age at inclusion, mean (SD) Sex Male Female Paternal educational level (≥HSD) Maternal educational level (≥HSD) Parents marital status Single Married or in couple Separated or divorced Widowed Educational level (≥HSD) Married (yes) Children (yes) Working (yes) Family monthly income b3000€ ≥3000€ Missing data Intermittent catheterization (yes) HSD indicates high school diploma. ⁎ P = .005.
Adults, n (%)
Adolescents, n (%)
Children, n (%)
36 26.3 (7.7)
18 15.3 (1.9)
17 7.7 (1.6)
23 (63.9) 13 (36.1) 8 (25) 9 (25.7)
9 9 7 10
(50.0) (50.0) (38.9) (55.6)
1 (2.8) 24 (68.6) 5 (14.3) 5 (14.3) 16 (44.4) 14 (40.0) 8 (22.2) 19 (57.6)
1 13 2 1
(5.9) (76.5) (11.7) (5.9)
1 (5.9) 14 (82.3) 2 (11.8) 0
20 (55.6) 3 (8.3) 13 (36.1) 9 (26.4)
9 5 3 5
(52.9) (29.4) (17.6) (29.5)
10 3 4 2
9 8 6 7
(52.9) (47.1) (40.0) (70.6) ⁎
(58.8) (17.6) (23.5) (11.8)
1696
S. Jochault-Ritz et al.
day of life. Surgical procedure to restore continence was necessary for 53 patients (75%). No difference was found in the number of surgical procedures necessary to achieve continence between the 3 groups. Continent urinary diversion was done mostly in adults and adolescents; incontinent external urinary diversion was done only in the adult group.
2.3. Characteristics of reconstruction in responders and nonresponders No difference was observed between the 2 groups (Table 3) in sex ratio, age group, associated malformation, type of initial surgical procedure, associated osteotomy, reference center, age at bladder closure, total number of surgical procedures, and number of surgical procedures needed to achieve continence. Associate malformation and ICC differed in the 3 age groups: when present, most of adults did not respond, whereas most of children and adolescents did respond (data not shown). Associated conditions included cloacae, anteposed or imperforated anus, uterine, vaginal or pyeloureteral duplicity, and caryotypic or vertebral anomalies.
2.4. Functional results for reconstructed BE epispadias complex Day and night continence were coded as follows: no leaking or occasional leaking, and frequent to continuous
Table 2
leaking. Seventy-seven percent of adults and 65% of the adolescents had satisfactory continence but in only 12% of the children (P b .0001). No difference was found according to sex or number of surgical procedures necessary to achieve continence. There was also no difference according to the type of surgery used to achieve continence. Seventy-eight percent of adults and 53% of adolescents when concerned had sexual interest, 72% and 53% had sexual activity, and 72% of adults had pleasure in it. There was no difference in group or sex. Details are shown in Table 4.
2.5. Comparison of HRQOL between groups 2.5.1. Adolescents vs adults Adolescents have superior QOL than adults in all SF-36 dimensions, from 8 to 24 points (Table 5). Only physical functioning (Δ = −10.6; P = .02) and role limitation because of mental health (Δ = −24.4; P = .04) were statistically different. 2.5.2. Children vs adolescents Children's scores are superior to that of adolescent's on all VSP-AE dimensions except self-esteem, vitality, and relations with friends (Table 6). Relations with medical staff were significantly better for children (Δ = 46.6; P = .0002). However, relations with friends were significantly better for adolescents and superior to children's. (Δ = 23.7; P = .01).
Characteristics of surgical reconstruction by age group Adults (n = 36), n (%)
Antenatal diagnosis (yes) Associated malformation (yes) Age at bladder closure (d) Mean (SD) Median Initial surgery Simple bladder closure Complete primary repair of exstrophy Modern staged repair of exstrophy Ureterosigmoidostomy Others Associated osteotomy (yes) Postoperative immobilization (yes) Complication of the 1st surgery (yes) Mean age at urethral reconstruction, mean (SD) Surgical procedure for continence (yes) No. of surgical procedures needed to achieve continence, mean (SD) ICC (yes) Mean no. of surgical procedures, mean (SD) Continent urinary diversion (yes) Incontinent external urinary diversion (yes) Bladder augmentation (yes) Surgery for vesicourinary reflux (yes)
0 (0) 1 (3.2) 91.1 (178.2) 6.5 24 0 4 3 5 19 18 15 6.5 28 1.5 9 12.8 10 4 19 27
(66.7) (11.1) (8.3) (13.9) (55.9) (56.2) (48.4) (3.8) (82.3) (1.7) (26.4) (7.6) (31.2) (12.5) (57.6) (79.4)
Adolescents (n = 18), n (%) 1 (6.2) 7 (43.7)
Children (n = 17), n (%) 3 (12.5) 4 (28.6)
121.5 (283.6) 3
1.6 (1.5) 1
10 (55.6) 3 (16.7) 1 (5.6) 0 4 (22.2) 7 (41.2) 11 (64.7) 6 (40.0) 1.4 (0.9) 12 (66.7) 1.0 (1.3) 5 (29.5) 8.1 (4.3) 4 (22.2) 0 8 (44.4) 14 (77.8)
9 (52.9) 1 (5.8) 5 (29.4) 0 2 (11.7) 8 (47.1) 9 (52.9) 9 (52.9) 1.2 (1.0) 13 (76.5) 1.1 (0.9) 2 (11.8) 6.2 (3) 1 (5.9) 0 3 (17.6) 12 (70.6)
QOL after bladder exstrophy reconstruction Table 3
1697
Characteristics of reconstruction for responders and nonresponders
Characteristics
Responders, n (%)
n Sex Male Female Age group Adults Adolescents Children Associated malformation (yes) Initial surgery Bladder closure Complete primary repair of exstrophy Modern staged repair of exstrophy Ureterosigmoidostomy Others Associated osteotomy (yes) Age at Bladder closure (d), mean (SD) No. of surgical procedures, mean (SD) No. of surgical procedures for continence, mean (SD)
71 (51.8)
66 (48.2)
41 (57.7) 30 (42.3)
46 (73.0) 17 (27.0)
36 (50.7) 18 (25.3) 17 (24.0) 12 (19.7)
41 (65.1) 13 (20.6) 9 (14.3) 14 (24.1)
43 (60.6) 4 (5.6) 10 (14.1) 3 (4.2) 11 (15.5) 34 (50.0) 76 (192) 9.9 (6.7) 1.2 (1.4)
39 (63.9) 5 (8.2) 7 (11.5) 1 (1.6) 9 (14.8) 34 (57.6) 67 (126) 11.2 (8.6) 1.4 (1.7)
2.5.3. Factors influencing HRQOL dimension scores Because the analysis was performed on a limited sample, only bivariate analyses were carried out between SF-36 dimensions and VSP-AE dimensions and patient characteristics and functional results. For adults, daytime continence is significantly related to role limitation related to mental health (RE). Sexual interest is related to vitality. Pleasure in sexual activity relates strongly to vitality and mental health. For adolescents, continence is significantly related to psychological well-being. Table 4
Nonresponders, n (%)
P .07
.23
.66 .89
.47 .75 .39 .59
For the children, the global number of surgical procedure is significantly related to relations with family (P = .003). Daytime continence is related to self-esteem, nighttime continence with relations with medical staff, and mother's educational level with QOL related with leisure activities.
3. Discussion In this retrospective multicenter study, we found that after reconstructive surgery for BE, most of the adults and
Functional results of reconstructed BE
Voiding control Daytime continence No or little leaking Frequent to continuous leaking Nighttime continence No or little leaking Frequent to continuous leaking Sexual interest None/few Some/much Missing data/inappropriate Sexual activity Yes No Missing data/inappropriate Pleasure in sexual activity None/few Some/much Missing data/inappropriate
Adults, n = 36, n (%)
Adolescents, n = 17, n (%)
Children, n = 17, n (%)
P
20 (55.6)
10 (62.5)
11 (64.7)
.8 b.0001
27 (77.1) 8 (22.9)
11 (64.7) 6 (35.3)
2 (12.5) 14 (87.5)
26 (76.5) 8 (23.5)
10 (66.7) 5 (33.3)
1 (5.9) 16 (94.1)
6 (16.7) 28 (77.8) 2 (5.6)
1 (5.9) 9 (52.9) 7 (41.2)
26 (72.2) 10 (27.8) 2 (5.6)
5 (29.4) 5 (29.4) 7 (41.1)
5 (13.9) 26 (72.2) 4 (11.1)
0 3 (17.6) 14 (82.3)
b.0001
.22
.13
.83
1698 Table 5
S. Jochault-Ritz et al. SF-36 dimension score for adults and adolescents
Physical functioning Role physical Bodily pain General health Vitality Social functioning Role emotional Mental health
Adults (n = 36), mA (SD)
Adolescents (n = 16), mT (SD)
Δ (mA − mT)
P
French general population
84.1 (24.9) 77.8 (35.7) 71.9 (27.5) 62.1 (25.0) 53.6 (20.7) 70.8 (30.3) 66.7 (42.0) 61.7 (21.9)
94.7 (5.9) 85.9 (28.8) 81.9 (20.9) 71.7 (20.1) 61.6 (18.9) 78.9 (22.7) 91.1 (19.8) 72.7 (16.8)
−10.6 −8.2 −10.1 −9.5 −7.9 −8.1 −24.4 −11.1
.02 .43 .20 .18 .19 .34 .04 .08
84.4 (21.2) 81.2 (32.2) 73.4 (23.7) 69.1 (18.6) 59.9 (18.0) 81.5 (21.4) 82.1 (32.1) 68.5 (17.6)
mA indicates mean for adults; mT, mean for adolescents.
adolescents have satisfactory continence, whereas few of the children have. Adults and adolescents, when concerned, are interested in sexuality and 72% of them have sexual activity, and 72% have pleasure in it. Quality of life of adolescents is superior to adults on SF-36 dimension score but is superior to children in only 3 dimensions. It is alarming that only 2 of 17 children have little or no leaking in our study. Reports from the literature indicate continence rates ranging from 63% for Mollard et al [23] and 70% for Baird et al [6] to 75% for Baka-Jakubiak [24]. However, the criteria for continence were different (dry interval of 3 hours). It was also difficult to know the mean age of patients at inclusion, and therefore, comparison with our findings was not possible. The rates of continence observed among adults and adolescents observed in our study (78% and 65%) are coherent with literature. Lower continence results in children could be explained by a possible bias in response with parents of more affected children feeling more concerned by research on the subject, but this is not the case in the adolescent group. A second possibility is that continence is achieved mostly during adolescence, at least, in our study. Intermittent clean catheterization, either transurethral or through a stoma, was performed by only 25% of adults and adolescents in our study. Catti et al [10] reported 22% of women with ICC in his study. Only 2 of the children performed ICC, but this is not surprising as the mean age to start catheterization is 12 years in our study. Table 6
The results observed in sexual activity among adults are also coherent with the literature. Baird et al [13] report 54% of adults sexually active, Catti et al [10] reported 76% sexually active women, and Ebert et al [25] reported 78% sexually active men. Pleasure in sexual activity was not always evaluated. Missing data were only 5.6% for this question, showing patient's interest and willingness to answer questions about sexuality. In contrast, the question about monthly income had 36% missing data, confirming our impression that patients are very much concerned with sexual issues and that this should be a part of the information given to parents before or at birth and to adolescents even before this topic arises. Socioeconomic data in our study were difficult to compare with the literature because of the difference in educational and economic systems. We did not find any study reporting parent's educational level. In our study, we found that the level of education of the mothers of adolescents and children were superior to that of the adults' mothers. This raises the question of either an increase in women's educational level or a bias in response, with women with a higher level of education feeling more involved with research on their child's pathologic condition. Adolescents' scores on SF-36 were not only higher than the adults' scores but were also higher in all dimensions than the general French population [21]. Interestingly, adults were globally similar to the general population on physical
VSP-AE dimensions and global score for adolescents and children
Family Self-esteem Vitality Friends Global well-being Hobbies Scholar work Relation with medical staff Global score
Adolescents (n = 18), mT (SD)
Children (n = 11), mC (SD)
Δ (mT − mC)
P
French general population
68.2 (17.2) 83.5 (11.5) 80.4 (15.1) 63.1 (28.4) 28.4 (19.8) 45.4 (40.0) 57.9 (17.0) 22.0 (33.2) 54.4 (10.7)
72.8 71.6 70.0 39.3 37.6 55.8 72.8 68.6 61.1
−4.6 12.0 10.4 23.7 −9.2 −10.4 −14.8 −46.6 −6.6
.40 .08 .15 .01 .24 .48 .07 .0002 .13
63.9 (22.1) 73.9 (21.1) 77.7 (18.1) 48.7 (26.6) 71.4 (17.9) 66.7 (20.4) 70.4 (24.2)
mT indicates mean for adolescent; mC, mean for children.
(12.1) (21.3) (20.2) (19.2) (20.3) (37.0) (22.2) (24.0) (10.3)
67.5 (12.9)
QOL after bladder exstrophy reconstruction dimensions or pain but lower (by about 6 points) on the other dimensions, with the greatest difference on social functioning and RE. It could be because adolescents are still optimistic about future life, whereas adults have had to cope with the difficulty of confronting others, the greatest challenge being to find a partner in life. Also, higher QOL in the adolescent groups when estimated with SF-36 reflects SF-36's propriety to affect better QOL to younger people. It could also be because SF-36 is not adapted to score QOL for adolescents, although it can be used in adolescents as young as 14 years. Giron et al [12] tried to assess QOL in 21 young adult patients in Brazil using the SF-36. As in our study, sex did not relate with HRQOL, but bad functional results were related to low QOL. They did notice that the lowest score was obtained in the social domain, whereas in our study, the lowest scores were observed in vitality and general health. The Lille study [26] in 25 patients from the north and west of France found results closer to ours, namely, lower dimension scores on limitations in physical activities because of health problems and on general health perception. Although patients from the Lille area were also included in our study, our results are different, but this could be explained by responding (or sampling) bias. For the differences between children and adolescents, results are more contrasted. Children have higher QOL on all dimensions except self-esteem, vitality, and relations with friends. The difference observed between children and adolescents in relations with medical staff could be a reflection of adolescence being a period of transition and conflict against authority. The difference in relations with friends could be because of the poor continence observed in the children's group. When compared to the French global population [22], BE has an impact on vitality, hobbies, and more important, on global well-being, with a difference of 35 points for children and 48 points for adolescents. Children and adolescents seem to compensate with higher scores on relation with family and in school work for children than the general population. The literature reports similar results of lower QOL in children after major urinary tract reconstruction, independently of age, sex, diagnostic procedure, and reconstruction results [27]. It is hard to tell whether the adolescents' score, which was higher than the global population on self-esteem, vitality, and relations with friends, is because of an evolution of QOL between infancy and adolescence, mainly in continence. It could also be because of the questionnaire being ill adapted for adolescents, as it was conceived for children from 8 to 10. However, Dodson et al [11] and Gearhart et al [14] also report high adolescent scores in self-esteem and family involvement, but the HRQOL questionnaire used was very different from VSP-AE. Lemelle et al [28], using VSP-A to evaluate QOL of adolescents born with spina bifida, whose surgical aims are similar to BE, report scores very much similar to the control age group. Continence as a factor influencing QOL is significantly related to influence on role limitation related to mental
1699 health for adults, psychological well-being for adolescents, and self-esteem for children. As a comparison, people having genital herpes virus have a lower score on RE and also on mental health [21]. Another factor influencing HRQOL is maternal level of education, which is significantly related to leisure activities for children. This could be explained by their mother's desire for them to live a normal life. Sexual interest and pleasure in sexual activity relate strongly with vitality and mental health on SF-36 dimension. This preliminary study has several limitations. The rate of response was expected to be higher (75% expected), as BE is a rare malformation and many patients and their parents participate in active patient associations. Not all centers could be included, explaining the lack of power. Most of the centers doing BE reconstruction surgery are included in our study, and thus, we hope to that the sample size will be higher for final analysis.
4. Conclusion Although incomplete, this preliminary study shows promising results confirming the clinical impression that functional results are strong determinants of QOL. The major drawback is that in view of the small sample size, the results are very sensitive to responding or sampling bias. A larger sample size is therefore necessary, ideally from more University Hospitals in France. Further studies could be done prospectively and focus on evaluating HRQOL at important time-points in patients' lives, such as entry into the primary and secondary school and early adulthood.
Acknowledgments The authors are indebted to all the physicians and secretaries who participated in this study for their time and help in identifying the eligible patients. We would like to sincerely acknowledge Nina Huss for invaluable technical assistance and Fiona Ecarnot for editorial assistance.
Appendix A. Physicians and centers participating in the multicenter trial: Pierre Alessandrini, PHD, Service de Chirurgie Infantile, AP-HM (Hôpital Nord), Marseille Georges Audry, PHD, Service de Chirurgie Infantile, APHP (Hôpital Trousseau), Paris François Becmeur, PHD, Hôpital Mère-Enfant, Hôpital Hautepierre, Strasgourg Rémi Besson, PHD, Hôpital Jeanne de Flandres, Lille
1700 Gérard Leford, PHD, Marie-Laurence Poli-Mérol, PHD, Service de Chirurgie Pédiatrique, Hôpital Américain, Reims Jacques Moscovici, PHD, Pôle de Pédiatrie Chirurgie Viscérale, Hôpital des Enfants, Toulouse Michel Schmitt, PHD, Jean Louis Lemelle, MD, CHU Nancy Hôpital d'Enfant, Vandoeuvre les Nancy
References [1] Mouriquand PD, Bubanj T, Feyaerts A, et al. Long-term results of bladder neck reconstruction for incontinence in children with classical bladder exstrophy or incontinent epispadias. BJU Int 2003;92: 997-1001 [discussion 1002]. [2] Woodhouse CR. Sexual function in boys born with exstrophy, myelomeningocele, and micropenis. Urology 1998;52:3-11. [3] Avolio L, Koo HP, Bescript AC, et al. The long-term outcome in men with exstrophy/epispadias: sexual function and social integration. J Urol 1996;156:822-5. [4] Stein R, Hohenfellner K, Fisch M, et al. Social integration, sexual behavior and fertility in patients with bladder exstrophy—a long-term follow up. Eur J Pediatr 1996;155:678-83. [5] Gobet R. Alternative management of bladder exstrophy. Curr Opin Urol 2009;19:424-6. [6] Baird AD, Nelson CP, Gearhart JP. Modern staged repair of bladder exstrophy: a contemporary series. J Pediatr Urol 2007;3:311-5. [7] Shnorhavorian M, Grady RW, Andersen A, et al. Long-term follow-up of complete primary repair of exstrophy: the Seattle experience. J Urol 2008;180:1615-9 [discussion 1619-1620]. [8] Woodhouse CR, North AC, Gearhart JP. Standing the test of time: long-term outcome of reconstruction of the exstrophy bladder. World J Urol 2006;24:244-9. [9] Hanna MK, Williams DI. Genital function in males with vesical exstrophy and epispadias. Br J Urol 1972;44:169-74. [10] Catti M, Paccalin C, Rudigoz RC, et al. Quality of life for adult women born with bladder and cloacal exstrophy: a long-term follow up. J Pediatr Urol 2006;2:16-22. [11] Dodson JL, Furth SL, Hsiao CJ, et al. Health related quality of life in adolescents with abnormal bladder function: an assessment using the Child Health and Illness Profile-Adolescent Edition. J Urol 2008;180: 1846-51 [discussion 1851]. [12] Giron AM, Passerotti CC, Machado M, et al. Quality of life predictors in young adult patients treated with bladder exstrophy. J Urol 2008;179:96.
S. Jochault-Ritz et al. [13] Baird AD, Sanders C, Woolfenden A, et al. Coping with bladder exstrophy: diverse results from early attempts at functional urinary tract surgery. BJU Int 2004;93:1303-8. [14] Gearhart JP, Ben-Chaim J, Jeffs RD, et al. Criteria for the prenatal diagnosis of classic bladder exstrophy. Obstet Gynecol 1995;85: 961-4. [15] Cacciari A, Pilu GL, Mordenti M, et al. Prenatal diagnosis of bladder exstrophy: what counseling? J Urol 1999;161:259-61 [discussion 262]. [16] Leplege A, Ecosse E, Verdier A, et al. The French SF-36 Health Survey: translation, cultural adaptation and preliminary psychometric evaluation. J Clin Epidemiol 1998;51:1013-23. [17] Ware Jr JE, Sherbourne CD. The MOS 36-item short-form health survey (SF-36). I. Conceptual framework and item selection. Med Care 1992;30:473-83. [18] Simeoni MC, Auquier P, Antoniotti S, et al. Validation of a French health-related quality of life instrument for adolescents: the VSP-A. Qual Life Res 2000;9:393-403. [19] Sapin C, Antoniotti S, Simeoni MC, et al. Shortening the VSP-A: preliminary development of the VSP-A12, a 12-item short-form. Qual Life Res 2004;13:235-41. [20] Sapin C, Simeoni MC, El Khammar M, et al. Reliability and validity of the VSP-A, a health-related quality of life instrument for ill and healthy adolescents. J Adolesc Health 2005;36:327-36. [21] Leplege A, Ecosse E, Pouchot J, et al. Le Questionnaire MOS SF-36. 1srt ed. Paris: Editions Estem; 1993. [22] Auquier P, Clément A, Sapin C, et al. Development, validation of HRQL measurement in children: VSP-Ae. Qual Life Res 2001;10:268. [23] Mollard P, Mouriquand PD, Buttin X. Urinary continence after reconstruction of classical bladder exstrophy (73 cases). Br J Urol 1994;73:298-302. [24] Baka-Jakubiak M. Combined bladder neck, urethral and penile reconstruction in boys with the exstrophy-epispadias complex. BJU Int 2000;86:513-8. [25] Ebert AK, Bals-Pratsch M, Seifert B, et al. Genital and reproductive function in males after functional reconstruction of the exstrophyepispadias complex—long-term results. Urology 2008;72:566-9 [discussion 569-570]. [26] Wittmeyer V, Aubry E, Liard-Zmuda A, et al. Quality of life of adult patients with a bladder exstrophy-epispadias complex. J Pediatr Urol 2009;5:S94-5. [27] Bogris S, Reilly D, Johal N, et al. Health-related quality of life (HRQOL) in children after major urinary tract reconstruction. J Pediatr Urol 2009;5:S42. [28] Lemelle JL, Guillemin F, Aubert D, et al. Quality of life and continence in patients with spina bifida. Qual Life Res 2006;15: 1481-92.
www.elsevier.com/locate/jpedsurg
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 Sandy Jochault-Ritz a , Mariette Mercier b , Didier Aubert a,⁎ a
Saint-Jacques University Hospital, 2, Place Saint Jacques, 25030 Besançon Cedex-France EA 3181, University of Franche-Comté, 25030 Besançon Cedex-France
b
Received 1 September 2009; revised 26 March 2010; accepted 28 March 2010
Key words: Exstrophy; Quality of life; Long-term outcome; Continence; Sexuality
Abstract Purpose: The aim of the study was to assess quality of life (QOL) of patients born with bladder exstrophy (BE) and reconstructed during early childhood in 7 French university hospitals (QUALEX study: QUAlity of Life of bladder EXstrophy). Methods: Patients from 6 to 42 years old answered self-administered Short-Form 36 (SF-36), VSP-A (Vécu et Santé Perçue de l'Adolescent), VSP-AE (Vécu et Santé Perçue de l'Enfant), AUQUIE (AUtoQUestionnaire Imagé de l'Enfant), and general questionnaires about functional and socioeconomic data. Dimension scores were compared between adults and adolescents using SF-36 and adolescents and children using VSP-AE. Scores were also compared to the general French population. Results: Among the 134 eligible patients, 36 adults, 18 adolescents, and 17 children answered the questionnaire. There was no difference between responders and nonresponders in reconstruction criteria. Continence was achieved in 77% of adults, 65% of adolescents, and 12% of children. Adolescent QOL was globally superior to adults and children. Adult QOL was globally lower than the general population except on the physical dimension. Children's QOL was also globally lower than the general population except for relations with family and school work. Adolescents' scores on SF-36 were superior to the general population but lower on half of the dimensions with VSP-AE. Conclusion: Patients presenting with reconstructed BE have impaired QOL, and functional results seem to be the most likely predictive factor of health-related QOL score. © 2010 Elsevier Inc. All rights reserved.
Bladder exstrophy (BE) is a rare but serious birth defect whose reconstruction, although now more codified, still remains the subject of controversy. Long-term results are judged in continence [1], aesthetic results, sexual function [2,3], and fertility [4]—the mains aspects that can impact on quality of life (QOL) throughout the patient's life. Many ⁎ Corresponding author. Tel.: +33 3 81 21 82 21; fax: +33 3 81 21 86 40. E-mail address: [email protected] (D. Aubert). 0022-3468/$ – see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2010.03.032
studies have been published reporting the long-term outcome of reconstruction [5-8] to assess which kind of reconstruction gives the best results in continence or sexual function [9]. Few studies have evaluated patient QOL [10,11], and only some have used health-related QOL (HRQOL) questionnaires [11-13]. The major difficulty is that because of the rarity of this malformation, most studies only have small sample sizes and, therefore, lack the power to identify factors that influence QOL.
1694 With the progress in antenatal diagnosis [14], pediatric surgeons need to have precise and reliable information based on scientific findings to adequately inform parents [15]. This information is even more essential in countries where medical pregnancy termination is accepted for fetuses presenting this malformation. The information must include functional results of surgical treatment and information about QOL in patients. The QUALEX (QUAlity of life of bladder EXstrophy) study is a French national study the primary aim of which was to assess the QOL of adults, adolescents, or children born with classical BE using validated HRQOL questionnaires. The secondary objective was to identify factors influencing patient's QOL such as functional results (urinary continence …), surgical history (type of reconstruction, number of surgical procedures, occurrence of complications …), or socioeconomic data. These preliminary results on a limited sample report characteristics of BE reconstruction and compare HRQOL between different age groups: children vs adolescents and adolescents vs adults.
1. Patients and methods 1.1. Study population and data collection Patients born with BE in France between 1962 and 2001 and underwent reconstruction before the age of 2 were included. Isolated epispadias was ruled out. Patient files were reviewed retrospectively in 7 French university hospitals (Besançon, Lille, Marseille Nord, Paris-Trousseau, Reims, Strasbourg, and Toulouse). The data collected included the number of surgical procedures, the type of surgical procedure used for reconstruction, and the type and number of surgical procedure used to achieve urinary continence. The different questionnaires were sent by post asking patients or their parents to answer questions about their life (level of education, monthly family income, marital status, number of children) and functional results (urinary continence and sexual activity) and to fill out the appropriate HRQOL questionnaires. After a month, nonresponders were contacted by telephone.
1.2. HRQOL questionnaires The HRQOL questionnaires were adapted to each age group: Short-Form 36 (SF-36) for adults; SF-36,Vécu et Santé Perçue de l'Adolescent (VSP-A), and Vécu et Santé Perçue de l'Enfant (VSP-AE) for adolescents (12-17 years old); and VSP-AE and AUto-QUestionnaire Imagé de l'Enfant for children (AUQUIE) (6-12 years old). Short-Form 36 is the French translation [16] and adaptation of the Medical Outcome Study Short Form 36 Health Survey (MOS SF-36) [17]. It is a generic health status questionnaire and can be used to assess health status
S. Jochault-Ritz et al. independent of the disease or illnesses affecting the population. It can be self-administered to adults as well as adolescents from 14 years old. It comprises 36 questions divided into 8 scales: physical functioning, role limitation related to physical health, bodily pain, general health perceptions, vitality, social functioning, emotions, and role limitations relating to mental health. The number of questions in each dimension ranges from 2 to 10, and questions have 2 to 6 answer modalities. Dimension scores were calculated by adding the answers from each dimension, followed by a linear transformation to obtain a score ranging from 0 (worst) to 100 (best). Missing data were estimated by the mean value of the other questions of the same dimension, if more than half the questions were answered. The VSP-AE questionnaire is made up of 39 items plus one open question. Eight dimension scores were computed: self-esteem; general well-being; vitality; school performance; leisure activities; relations with friends; relation with medical staff; and global VSP-AE index. VSP-A comprises 38 items and one open question. Eleven dimensions were computed: vitality; psychological well-being; relations with friends, parents, teachers, and medical staff; leisure activities; physical well-being; school work; selfesteem; and sentimental and sexual life. Each question has 5 answer modalities. Dimension scores and missing data were treated as in the SF-36 [18-20]. AUQUIE is a questionnaire that has been developed for children aged 6 to 12 years. It begins with an open independent question designed to verify the child's capacity to answer the questions. Twenty-six questions with 4 modalities of answers evaluate family and social relationships, activity, health, global functioning (sleep, appetite), and separation. A global score and dimension scores were computed by adding the answers of the questions in each dimension.
1.3. Statistical analysis The primary end point of the QUALEX study was to evaluate factors related to the surgical procedures, functional results, and everyday life that influence HRQOL scores. The secondary end point was to compare the QOL scores of adolescents and adults using SF-36 and between adolescents and children using VSP-AE. These scores were compared with those of French general population as described in the literature [21,22]. To detect a difference of 10 points on a scale of 100 between 2 mean dimension scores, with α = 5% and β = 15%, the number of patients needed in each group was 110. Comparisons between responders and nonresponders and between the 3 age groups were performed using Fisher's Exact test or χ2 test for categorical variables and t test or variance analysis for quantitative variables. Comparison of SF-36 dimension scores between adults and adolescents was performed with t test, and comparisons of VSP-AE dimensions between adolescents and children was performed using nonparametric Wilcoxon test. Bivariate analysis was
QOL after bladder exstrophy reconstruction
1695
performed to identify potential correlations between patient characteristics and functional results and each HRQOL dimension score for each age group (SF-36 for adults, VSPA for adolescents, and VSPAE for children). To take into account the multiplicity of comparisons, a P b .01 was considered significant. Data collection and statistical analyses were performed using the Statistical Analysis Software (version 9.2; SAS Institute, Cary, NC). This study received approval from the local ethics committee and was sponsored by the French national program for clinical research (Programme Hospitalier de Recherche Clinique). Informed consent was obtained from all participants (and from both children and their parents).
2. Results Between April 2008 and June 2009, 134 patients were eligible. Preliminary results are reported concerning the 71 patients who responded.
2.1. Patient characteristics Patient characteristics are shown in Table 1. No statistical difference was found for the sex repartition in the 3 groups. There was no difference in patients' father's level of education. Mothers of adolescents and children had a higher education level than the adults' mother (P = .005). Table 1
Among the adults, 56% had less than a high school degree. Thirty-two percent of adult male patients and 54% of women were married (no difference); 2 of the men and 6 of the women had children; and 1 of the men had had recourse to medically assisted procreation (sperm donor). Intermittent clean catheterization (ICC) was more frequent among adults and adolescents than among children.
2.2. Characteristics of surgical reconstruction by age group Details are shown in Table 2. There was no significant difference between the 3 groups except for mean age at urethral reconstruction for boys and mean number of surgical procedures. Mean age at reconstruction ranged from 6.5 years in the adults' group to 1.2 in the children's group (P b .0001). Mean number of surgical procedures was 12.8 for adults and 6.2 for children (P = .001). Antenatal diagnosis was not very frequent. Age at bladder closure in days was lower in the children's group. Initial bladder closure consisted mainly of simple bladder closure, complete primary repair of exstrophy was used in 4 boys and adolescents, modern staged repair of exstrophy in 1 adolescent and 5 children, and ureterosigmoidostomy was performed in 3 adults. Associated osteotomy was performed in less than half of cases, more often for girls (65.5% vs 38.5%) and when surgery was performed after the third day of life. Post surgical immobilization (mostly external fixator or traction) was more frequent if bladder closure occurred after the third
Patient characteristics
n Age at inclusion, mean (SD) Sex Male Female Paternal educational level (≥HSD) Maternal educational level (≥HSD) Parents marital status Single Married or in couple Separated or divorced Widowed Educational level (≥HSD) Married (yes) Children (yes) Working (yes) Family monthly income b3000€ ≥3000€ Missing data Intermittent catheterization (yes) HSD indicates high school diploma. ⁎ P = .005.
Adults, n (%)
Adolescents, n (%)
Children, n (%)
36 26.3 (7.7)
18 15.3 (1.9)
17 7.7 (1.6)
23 (63.9) 13 (36.1) 8 (25) 9 (25.7)
9 9 7 10
(50.0) (50.0) (38.9) (55.6)
1 (2.8) 24 (68.6) 5 (14.3) 5 (14.3) 16 (44.4) 14 (40.0) 8 (22.2) 19 (57.6)
1 13 2 1
(5.9) (76.5) (11.7) (5.9)
1 (5.9) 14 (82.3) 2 (11.8) 0
20 (55.6) 3 (8.3) 13 (36.1) 9 (26.4)
9 5 3 5
(52.9) (29.4) (17.6) (29.5)
10 3 4 2
9 8 6 7
(52.9) (47.1) (40.0) (70.6) ⁎
(58.8) (17.6) (23.5) (11.8)
1696
S. Jochault-Ritz et al.
day of life. Surgical procedure to restore continence was necessary for 53 patients (75%). No difference was found in the number of surgical procedures necessary to achieve continence between the 3 groups. Continent urinary diversion was done mostly in adults and adolescents; incontinent external urinary diversion was done only in the adult group.
2.3. Characteristics of reconstruction in responders and nonresponders No difference was observed between the 2 groups (Table 3) in sex ratio, age group, associated malformation, type of initial surgical procedure, associated osteotomy, reference center, age at bladder closure, total number of surgical procedures, and number of surgical procedures needed to achieve continence. Associate malformation and ICC differed in the 3 age groups: when present, most of adults did not respond, whereas most of children and adolescents did respond (data not shown). Associated conditions included cloacae, anteposed or imperforated anus, uterine, vaginal or pyeloureteral duplicity, and caryotypic or vertebral anomalies.
2.4. Functional results for reconstructed BE epispadias complex Day and night continence were coded as follows: no leaking or occasional leaking, and frequent to continuous
Table 2
leaking. Seventy-seven percent of adults and 65% of the adolescents had satisfactory continence but in only 12% of the children (P b .0001). No difference was found according to sex or number of surgical procedures necessary to achieve continence. There was also no difference according to the type of surgery used to achieve continence. Seventy-eight percent of adults and 53% of adolescents when concerned had sexual interest, 72% and 53% had sexual activity, and 72% of adults had pleasure in it. There was no difference in group or sex. Details are shown in Table 4.
2.5. Comparison of HRQOL between groups 2.5.1. Adolescents vs adults Adolescents have superior QOL than adults in all SF-36 dimensions, from 8 to 24 points (Table 5). Only physical functioning (Δ = −10.6; P = .02) and role limitation because of mental health (Δ = −24.4; P = .04) were statistically different. 2.5.2. Children vs adolescents Children's scores are superior to that of adolescent's on all VSP-AE dimensions except self-esteem, vitality, and relations with friends (Table 6). Relations with medical staff were significantly better for children (Δ = 46.6; P = .0002). However, relations with friends were significantly better for adolescents and superior to children's. (Δ = 23.7; P = .01).
Characteristics of surgical reconstruction by age group Adults (n = 36), n (%)
Antenatal diagnosis (yes) Associated malformation (yes) Age at bladder closure (d) Mean (SD) Median Initial surgery Simple bladder closure Complete primary repair of exstrophy Modern staged repair of exstrophy Ureterosigmoidostomy Others Associated osteotomy (yes) Postoperative immobilization (yes) Complication of the 1st surgery (yes) Mean age at urethral reconstruction, mean (SD) Surgical procedure for continence (yes) No. of surgical procedures needed to achieve continence, mean (SD) ICC (yes) Mean no. of surgical procedures, mean (SD) Continent urinary diversion (yes) Incontinent external urinary diversion (yes) Bladder augmentation (yes) Surgery for vesicourinary reflux (yes)
0 (0) 1 (3.2) 91.1 (178.2) 6.5 24 0 4 3 5 19 18 15 6.5 28 1.5 9 12.8 10 4 19 27
(66.7) (11.1) (8.3) (13.9) (55.9) (56.2) (48.4) (3.8) (82.3) (1.7) (26.4) (7.6) (31.2) (12.5) (57.6) (79.4)
Adolescents (n = 18), n (%) 1 (6.2) 7 (43.7)
Children (n = 17), n (%) 3 (12.5) 4 (28.6)
121.5 (283.6) 3
1.6 (1.5) 1
10 (55.6) 3 (16.7) 1 (5.6) 0 4 (22.2) 7 (41.2) 11 (64.7) 6 (40.0) 1.4 (0.9) 12 (66.7) 1.0 (1.3) 5 (29.5) 8.1 (4.3) 4 (22.2) 0 8 (44.4) 14 (77.8)
9 (52.9) 1 (5.8) 5 (29.4) 0 2 (11.7) 8 (47.1) 9 (52.9) 9 (52.9) 1.2 (1.0) 13 (76.5) 1.1 (0.9) 2 (11.8) 6.2 (3) 1 (5.9) 0 3 (17.6) 12 (70.6)
QOL after bladder exstrophy reconstruction Table 3
1697
Characteristics of reconstruction for responders and nonresponders
Characteristics
Responders, n (%)
n Sex Male Female Age group Adults Adolescents Children Associated malformation (yes) Initial surgery Bladder closure Complete primary repair of exstrophy Modern staged repair of exstrophy Ureterosigmoidostomy Others Associated osteotomy (yes) Age at Bladder closure (d), mean (SD) No. of surgical procedures, mean (SD) No. of surgical procedures for continence, mean (SD)
71 (51.8)
66 (48.2)
41 (57.7) 30 (42.3)
46 (73.0) 17 (27.0)
36 (50.7) 18 (25.3) 17 (24.0) 12 (19.7)
41 (65.1) 13 (20.6) 9 (14.3) 14 (24.1)
43 (60.6) 4 (5.6) 10 (14.1) 3 (4.2) 11 (15.5) 34 (50.0) 76 (192) 9.9 (6.7) 1.2 (1.4)
39 (63.9) 5 (8.2) 7 (11.5) 1 (1.6) 9 (14.8) 34 (57.6) 67 (126) 11.2 (8.6) 1.4 (1.7)
2.5.3. Factors influencing HRQOL dimension scores Because the analysis was performed on a limited sample, only bivariate analyses were carried out between SF-36 dimensions and VSP-AE dimensions and patient characteristics and functional results. For adults, daytime continence is significantly related to role limitation related to mental health (RE). Sexual interest is related to vitality. Pleasure in sexual activity relates strongly to vitality and mental health. For adolescents, continence is significantly related to psychological well-being. Table 4
Nonresponders, n (%)
P .07
.23
.66 .89
.47 .75 .39 .59
For the children, the global number of surgical procedure is significantly related to relations with family (P = .003). Daytime continence is related to self-esteem, nighttime continence with relations with medical staff, and mother's educational level with QOL related with leisure activities.
3. Discussion In this retrospective multicenter study, we found that after reconstructive surgery for BE, most of the adults and
Functional results of reconstructed BE
Voiding control Daytime continence No or little leaking Frequent to continuous leaking Nighttime continence No or little leaking Frequent to continuous leaking Sexual interest None/few Some/much Missing data/inappropriate Sexual activity Yes No Missing data/inappropriate Pleasure in sexual activity None/few Some/much Missing data/inappropriate
Adults, n = 36, n (%)
Adolescents, n = 17, n (%)
Children, n = 17, n (%)
P
20 (55.6)
10 (62.5)
11 (64.7)
.8 b.0001
27 (77.1) 8 (22.9)
11 (64.7) 6 (35.3)
2 (12.5) 14 (87.5)
26 (76.5) 8 (23.5)
10 (66.7) 5 (33.3)
1 (5.9) 16 (94.1)
6 (16.7) 28 (77.8) 2 (5.6)
1 (5.9) 9 (52.9) 7 (41.2)
26 (72.2) 10 (27.8) 2 (5.6)
5 (29.4) 5 (29.4) 7 (41.1)
5 (13.9) 26 (72.2) 4 (11.1)
0 3 (17.6) 14 (82.3)
b.0001
.22
.13
.83
1698 Table 5
S. Jochault-Ritz et al. SF-36 dimension score for adults and adolescents
Physical functioning Role physical Bodily pain General health Vitality Social functioning Role emotional Mental health
Adults (n = 36), mA (SD)
Adolescents (n = 16), mT (SD)
Δ (mA − mT)
P
French general population
84.1 (24.9) 77.8 (35.7) 71.9 (27.5) 62.1 (25.0) 53.6 (20.7) 70.8 (30.3) 66.7 (42.0) 61.7 (21.9)
94.7 (5.9) 85.9 (28.8) 81.9 (20.9) 71.7 (20.1) 61.6 (18.9) 78.9 (22.7) 91.1 (19.8) 72.7 (16.8)
−10.6 −8.2 −10.1 −9.5 −7.9 −8.1 −24.4 −11.1
.02 .43 .20 .18 .19 .34 .04 .08
84.4 (21.2) 81.2 (32.2) 73.4 (23.7) 69.1 (18.6) 59.9 (18.0) 81.5 (21.4) 82.1 (32.1) 68.5 (17.6)
mA indicates mean for adults; mT, mean for adolescents.
adolescents have satisfactory continence, whereas few of the children have. Adults and adolescents, when concerned, are interested in sexuality and 72% of them have sexual activity, and 72% have pleasure in it. Quality of life of adolescents is superior to adults on SF-36 dimension score but is superior to children in only 3 dimensions. It is alarming that only 2 of 17 children have little or no leaking in our study. Reports from the literature indicate continence rates ranging from 63% for Mollard et al [23] and 70% for Baird et al [6] to 75% for Baka-Jakubiak [24]. However, the criteria for continence were different (dry interval of 3 hours). It was also difficult to know the mean age of patients at inclusion, and therefore, comparison with our findings was not possible. The rates of continence observed among adults and adolescents observed in our study (78% and 65%) are coherent with literature. Lower continence results in children could be explained by a possible bias in response with parents of more affected children feeling more concerned by research on the subject, but this is not the case in the adolescent group. A second possibility is that continence is achieved mostly during adolescence, at least, in our study. Intermittent clean catheterization, either transurethral or through a stoma, was performed by only 25% of adults and adolescents in our study. Catti et al [10] reported 22% of women with ICC in his study. Only 2 of the children performed ICC, but this is not surprising as the mean age to start catheterization is 12 years in our study. Table 6
The results observed in sexual activity among adults are also coherent with the literature. Baird et al [13] report 54% of adults sexually active, Catti et al [10] reported 76% sexually active women, and Ebert et al [25] reported 78% sexually active men. Pleasure in sexual activity was not always evaluated. Missing data were only 5.6% for this question, showing patient's interest and willingness to answer questions about sexuality. In contrast, the question about monthly income had 36% missing data, confirming our impression that patients are very much concerned with sexual issues and that this should be a part of the information given to parents before or at birth and to adolescents even before this topic arises. Socioeconomic data in our study were difficult to compare with the literature because of the difference in educational and economic systems. We did not find any study reporting parent's educational level. In our study, we found that the level of education of the mothers of adolescents and children were superior to that of the adults' mothers. This raises the question of either an increase in women's educational level or a bias in response, with women with a higher level of education feeling more involved with research on their child's pathologic condition. Adolescents' scores on SF-36 were not only higher than the adults' scores but were also higher in all dimensions than the general French population [21]. Interestingly, adults were globally similar to the general population on physical
VSP-AE dimensions and global score for adolescents and children
Family Self-esteem Vitality Friends Global well-being Hobbies Scholar work Relation with medical staff Global score
Adolescents (n = 18), mT (SD)
Children (n = 11), mC (SD)
Δ (mT − mC)
P
French general population
68.2 (17.2) 83.5 (11.5) 80.4 (15.1) 63.1 (28.4) 28.4 (19.8) 45.4 (40.0) 57.9 (17.0) 22.0 (33.2) 54.4 (10.7)
72.8 71.6 70.0 39.3 37.6 55.8 72.8 68.6 61.1
−4.6 12.0 10.4 23.7 −9.2 −10.4 −14.8 −46.6 −6.6
.40 .08 .15 .01 .24 .48 .07 .0002 .13
63.9 (22.1) 73.9 (21.1) 77.7 (18.1) 48.7 (26.6) 71.4 (17.9) 66.7 (20.4) 70.4 (24.2)
mT indicates mean for adolescent; mC, mean for children.
(12.1) (21.3) (20.2) (19.2) (20.3) (37.0) (22.2) (24.0) (10.3)
67.5 (12.9)
QOL after bladder exstrophy reconstruction dimensions or pain but lower (by about 6 points) on the other dimensions, with the greatest difference on social functioning and RE. It could be because adolescents are still optimistic about future life, whereas adults have had to cope with the difficulty of confronting others, the greatest challenge being to find a partner in life. Also, higher QOL in the adolescent groups when estimated with SF-36 reflects SF-36's propriety to affect better QOL to younger people. It could also be because SF-36 is not adapted to score QOL for adolescents, although it can be used in adolescents as young as 14 years. Giron et al [12] tried to assess QOL in 21 young adult patients in Brazil using the SF-36. As in our study, sex did not relate with HRQOL, but bad functional results were related to low QOL. They did notice that the lowest score was obtained in the social domain, whereas in our study, the lowest scores were observed in vitality and general health. The Lille study [26] in 25 patients from the north and west of France found results closer to ours, namely, lower dimension scores on limitations in physical activities because of health problems and on general health perception. Although patients from the Lille area were also included in our study, our results are different, but this could be explained by responding (or sampling) bias. For the differences between children and adolescents, results are more contrasted. Children have higher QOL on all dimensions except self-esteem, vitality, and relations with friends. The difference observed between children and adolescents in relations with medical staff could be a reflection of adolescence being a period of transition and conflict against authority. The difference in relations with friends could be because of the poor continence observed in the children's group. When compared to the French global population [22], BE has an impact on vitality, hobbies, and more important, on global well-being, with a difference of 35 points for children and 48 points for adolescents. Children and adolescents seem to compensate with higher scores on relation with family and in school work for children than the general population. The literature reports similar results of lower QOL in children after major urinary tract reconstruction, independently of age, sex, diagnostic procedure, and reconstruction results [27]. It is hard to tell whether the adolescents' score, which was higher than the global population on self-esteem, vitality, and relations with friends, is because of an evolution of QOL between infancy and adolescence, mainly in continence. It could also be because of the questionnaire being ill adapted for adolescents, as it was conceived for children from 8 to 10. However, Dodson et al [11] and Gearhart et al [14] also report high adolescent scores in self-esteem and family involvement, but the HRQOL questionnaire used was very different from VSP-AE. Lemelle et al [28], using VSP-A to evaluate QOL of adolescents born with spina bifida, whose surgical aims are similar to BE, report scores very much similar to the control age group. Continence as a factor influencing QOL is significantly related to influence on role limitation related to mental
1699 health for adults, psychological well-being for adolescents, and self-esteem for children. As a comparison, people having genital herpes virus have a lower score on RE and also on mental health [21]. Another factor influencing HRQOL is maternal level of education, which is significantly related to leisure activities for children. This could be explained by their mother's desire for them to live a normal life. Sexual interest and pleasure in sexual activity relate strongly with vitality and mental health on SF-36 dimension. This preliminary study has several limitations. The rate of response was expected to be higher (75% expected), as BE is a rare malformation and many patients and their parents participate in active patient associations. Not all centers could be included, explaining the lack of power. Most of the centers doing BE reconstruction surgery are included in our study, and thus, we hope to that the sample size will be higher for final analysis.
4. Conclusion Although incomplete, this preliminary study shows promising results confirming the clinical impression that functional results are strong determinants of QOL. The major drawback is that in view of the small sample size, the results are very sensitive to responding or sampling bias. A larger sample size is therefore necessary, ideally from more University Hospitals in France. Further studies could be done prospectively and focus on evaluating HRQOL at important time-points in patients' lives, such as entry into the primary and secondary school and early adulthood.
Acknowledgments The authors are indebted to all the physicians and secretaries who participated in this study for their time and help in identifying the eligible patients. We would like to sincerely acknowledge Nina Huss for invaluable technical assistance and Fiona Ecarnot for editorial assistance.
Appendix A. Physicians and centers participating in the multicenter trial: Pierre Alessandrini, PHD, Service de Chirurgie Infantile, AP-HM (Hôpital Nord), Marseille Georges Audry, PHD, Service de Chirurgie Infantile, APHP (Hôpital Trousseau), Paris François Becmeur, PHD, Hôpital Mère-Enfant, Hôpital Hautepierre, Strasgourg Rémi Besson, PHD, Hôpital Jeanne de Flandres, Lille
1700 Gérard Leford, PHD, Marie-Laurence Poli-Mérol, PHD, Service de Chirurgie Pédiatrique, Hôpital Américain, Reims Jacques Moscovici, PHD, Pôle de Pédiatrie Chirurgie Viscérale, Hôpital des Enfants, Toulouse Michel Schmitt, PHD, Jean Louis Lemelle, MD, CHU Nancy Hôpital d'Enfant, Vandoeuvre les Nancy
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