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Short and long term quality of life after reconstruction of bladder exstrophy in infancy: preliminary results of the QUALEX Study

Sandy Jochault-Ritz¹, Mariette Mercier², Didier Aubert¹
¹Saint-Jacques University Hospital,

²EA 3181, University of Franche-Comté,
Besançon, France

 

 

Correspondence

Prof Didier Aubert
Department of Pediatric Surgery
Saint-Jacques University Hospital
2, Place Saint Jacques, 25030 Besançon Cedex -France
Tel: + 33 3 81 21 82 21; Fax: + 33 3 81 21 86 40
E-mail: daubert@chu-besançon.fr

 

Abstract

Objective: To assess the quality of life of patients born with bladder exstrophy (BE) and reconstructed during early childhood in 7 French University Hospital (QUALEX study).

Material and Method: Patients from 6-42 year old answered to SF-36, VSP-A, VSP-AE AUQIE and general questionnaires about functional and socioeconomic data. Dimension scores were compared between adults and adolescents using SF-36 and 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 non-responders in terms of 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 quality of life and functional results seem to be the most likely predictive factor of health related quality of life questionnaires, HRQOL score.

Key words: bladder, exstrophy, quality of life, long term outcome, continence, sexuality

 

 

Introduction

Bladder exstrophy (BE) is a rare but serious birth defect whose reconstruction, though now more codified, still remains the subject of controversy. Long term results are judged in term of continence [1], esthetic 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 studies have been published reporting the long term outcome of reconstruction [5-8] to assess which kind of reconstruction gives the best results in term of continence or sexual function [9]. Few studies have evaluated patient QOL [10, 11] and only some have used health related quality of life questionnaires (HRQOL) [11-13]. The major difficulty is that due to the rarity of this malformation, most studies only have small sample sizes, and therefore lack the power to identify factors that influence quality of life.

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 quality of life in patients.

The QUALEX study is a French national study whose primary aim is to assess the quality of life of adults, adolescents or children born with classical bladder exstrophy using validated health-related quality of life questionnaires. The secondary objective is to identify factors influencing patient’s QOL such as functional results (urinary continence and sexual activity), surgical history (type of reconstruction, number of surgical procedures, occurrence of complications) or socio-economic 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.

Patient and method

Study population and data collection

Patients born with BE in France between 1962 and 2001 and reconstructed before the age of two were included. Isolated epispadias was excluded. Patient files were reviewed retrospectively in seven French University Hospital (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, 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 ), functional results (urinary continence and sexual activity) and to fill out the appropriate health related HRQOL questionnaires. After a month, nonresponders were contacted by phone.

HRQOL questionnaires

The HRQOL questionnaires were adapted to each age group: SF36 for Adults, SF36, VSPA and VSPAE (Vécu et Santé Perçue de l’Adolescent et de l’Enfant) for adolescents (12 to 17 years-old), VSPAE and AUQUIE (Auto-Questionnaire Imagé de l’Enfant) children (6 to 12 years-old). SF36 is the French translation [16] and adaptation of the MOS 36-item Short Form Health Survey [17]. It is a generic health status questionnaire and can be used to assess health status 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, parents, teachers, 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, self-esteem and sentimental and sexual life. Each question has 5 answer modalities. Dimension scores and missing data were treated as in the SF36 [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.

Statistical analysis

The primary endpoint of the QUALEX study was to evaluate factors related to the surgical procedures, functional results and every-day life that influence HRQOL scores. The secondary endpoint was to compare the QOL scores of adolescents and adults using SF36, 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 two mean dimension scores, with α=5% and ß=15%, the number of patients needed in each group was 110. Comparisons between responders and non-responders and between the three age groups were performed using Fisher’s exact test or Chi 2 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 non-parametric Wilcoxon test. Bivariate analysis was performed to identify potential correlations between patient characteristics and functional results and each HRQOL dimension score for each age group (SF36 for adults, VSPA for adolescents and VSPAE for children). To take into account the multiplicity of comparisons, a p-value of < 0.01 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, PHRC). Informed consent was obtained from all participants (and from both children and their parents).

Results

Between April 2008 and June 2009, 134 patients were eligible. Preliminary results are reported concerning the 71 patients who responded.

Patient characteristics:

Patient characteristics are shown in Table 1. No statistical difference was found for the gender repartition in the three 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=0.005).

Table 1: Patient characteristics

 

Adults n (%)

Adolescents n (%)

Children n (%)

Number

36

18

17

Age at inclusion mean (sd)

26.3 (7.7)

15.3 (1.9)

7.7 (1.6)

Gender
Men
Women

 23 (63.9)

13 (36.1)

9 (50.0)

9 (50.0)

9 (52.9)

8 (47.1)

Paternal educational level
<HSD*
>=HSD

 

 

24 (75)

8 (25)

 

 

11 (61.1)

7 (38.9)

 

 

9 (60.0)

6 (40.0)

Maternal educational level
<HSD
>=HSD

 

 

26 (74.3)

9 (25.7)

 

 

8 (44.4)

10 (55.6)

 

 

5 (29.4)

7 (70.6)

Parents marital status
single
married or in couple
separated or divorced
widowed

 

1 (2.8)

24 (68.6)

5 (14.3)

5 (14.3)

 

1 (5.9)

13 (76.5)

2 (11.7)

1 (5.9)

 

1 (5.9)

14 (82.3)

2 (11.8)

0

Educational level
<HSD
>=HSD

 

20 (55.6)

16 (44.4)

 

 

 

Marital status
Yes
No

 

14 (40.0)

21 (60.0)

 

 

Children
Yes
No

 

8 (22.2)

28 (77.8)

 

 

Working
Yes
No

 

19 (57.6)

14 (42.4)

 

 

Family monthly income
<3000€
>=3000€
Missing data

 

20 (55.6)

3 (8.3)

13 (36.1)

 

9 (52.9)

5 (29.4)

3 (17.6)

 

10 (58.8)

3 (17.6)

4 (23.5)

Intermittent catheterisation
Yes
No

 

 

9 (26.4)

25 (73.6)

 

 

5 (29.5)

12 (70.5)

 

 

2 (11.8)

15 (88.2)

Sd: standard deviation; *HSD high school diploma 

 

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), two of the men and six of the women had children; one of the men had had recourse to medically assisted procreation (sperm donor). Intermittent clean catheterisation was more frequent among adults and adolescents than among children.

Characteristics of surgical reconstruction by age group

Details are shown in Table 2. There was no significant difference between the three 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 group (p< 0.0001). Mean number of surgical procedures was 12.8 for adults and 6.2 for children (p=0.001). 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 (CPRE) was used in four boys and adolescents, modern staged repair of exstrophy (MSRE) in one adolescent and five children, ureterosigmoidostomy was performed in three adults.

Table 2: characteristics of surgical reconstruction by age group

 

Adults n (%)

Adolescents n (%)

Children n (%)

Number

36

18

17

Antenatal Diagnosis
Yes
No

 

0 (0)

34 (100)

 

1 (6.2)

15 (93.8)

 

3 (12.5)

14 (87.5)

Associated malformation
Yes
No

 

1 (3.2)

30 (96.8)

 

7 (43.7)

9 (56.2)

 

4 (28.6)

10 (71.4)

Age at bladder closure in days
mean (sd)

 

91.1 (178.2)

6.5

 

121.5 (283.6)

3

 

 

1.6 (1.5)

1

Initial surgery
Simple bladder closure
CPRE*
MSRE**
USS***
Others

 

24 (66.7)

0

4 (11.1)

3 (8.3)

5 (13.9)

 

10 (55.6)

3 (16.7)

1 (5.6)

0

4 (22.2)

 

9 (52.9)

1 (5.8)

5 (29.4)

0

2 (11.7)

Associated osteotomy
Yes
No

 

19 (55.9)

15 (44.1)

 

 

7 (41.2)

10 (58.8)

 

 

8 (47.1)

9 (52.9)

Postoperative immobilization
Yes
No

 

 

18 (56.2)

14 (43.8)

 

 

11 (64.7)

6 (35.3)

 

 

9 (52.9)

8 (47.1)

Complication of the 1st surgery
Yes
No

 

 

15 (48.4)

16 (51.6)

 

 

6 (40.0)

9 (60.0)

 

 

9 (52.9)

8 (47.1)

Mean age at urethral reconstruction m (sd)

 

6.5 (3.8)

 

1.4 (0.9)

 

1.2 (1.0)

Surgical procedure for continence
Yes
No

 

 

28 (82.3)

6 (17.4)

 

 

12 (66.7)

6 (33.3)

 

 

13 (76.5)

4 (23.5)

Number of surgical procedures needed
to achieve continence, m (sd)

 

 

 

1.5 (1.7)

 

 

 

1.0 (1.3)

 

 

 

1.1 (0.9)

Intermittent clean catheterisation
Yes
No

 

 

9 (26.4)

25 (73.6)

 

 

5 (29.5)

12 (70.5)

 

 

2 (11.8)

15 (88.2)

Mean number of surgical procedures,
m (sd)

 

 

12.8 (7.6)

 

 

8.1 (4.3)

 

 

6.2 (3)

Continent urinary diversion
Yes
No

 

 

10 (31.2)

22 (68.8)

 

 

4 (22.2)

14 (77.8)

 

 

1 (5.9)

16 (94.1)

Incontinent external urinary diversion
Yes
No

 

 

4 (12.5)

28 (87.5)

 

 

0

18 (100)

 

 

0

17 (100)

Bladder augmentation
Yes
No

 

19 (57.6)

14 (42.4)

 

8 (44.4)

10 (55.6)

 

3 (17.6)

14 (82.4)

Surgery for vesicourinary reflux
Yes
No

 

 

27 (79.4)

7 (20.6)

 

 

14 (77.8)

4 (22.2)

 

 

12 (70.6)

5 (29.4)

*CPRE: complete primary repair of bladder exstrophy
**MSRE: modern staged repair of bladder exstrophy
***USS: ureteroigmoidostomy 

 

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 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 three groups. Continent urinary diversion was done mostly in adults and adolescents; incontinent external urinary diversion was done only in the adult group.

Characteristics of reconstruction in responders and non-responders No difference was observed between the two groups (Table 3) in terms of sex ratio, age group, associated malformation, type of initial surgical procedure, associated osteotomy, reference center, age at bladder closure, total number of surgical procedures, number of surgical procedures needed to achieve continence. Associate malformation and intermittent clean catheterisation differed in the three age groups: when present, a majority of adults did not respond, while a majority of children and adolescents did respond (data not shown).

Table 3: Characteristics of reconstruction for responders and non responders

Characteristics

Responders

N (%)

Non responders

N (%)

P Value

Number

71 (51.8)

66 (48.2)

Gender

Men

Woman

41 (57.7)

30 (42.3)

46 (73.0)

17 (27.0)

0.07

Age Group

Adults

Adolescents

Children

36 (50.7)

18 (25.3)

17 (24.0)

41 (65.1)

13 (20.6)

9 (14.3)

0.23

Associated malformation

Yes

No

12 (19.7)

49 (80.3)

14 (24.1)

44 (75.7)

0.66

Initial Surgery

Bladder closure

CPRE*

MSRE**

USS***

Others

43 (60.6)

4 (5.6)

10 (14.1)

3 (4.2)

11 (15.5)

39 (63.9)

5 (8.2)

7 (11.5)

1 (1.6)

9 (14.8)

0.89

Associated osteotomy

Yes

No

34 (50.0)

34 (50.0)

34 (57.6)

25 (42.4)

0.47

Age at Bladder closure (days)

Mean (sd)

76 (192)

67 (126)

0.75

Number of surgical procedures

Mean (sd)

9.9 (6.7)

11.2(8.6)

0.39

Number of surgical procedures for continence

Mean (sd)

1.2 (1.4)

1.4 (1.7)

0.59

*CPRE: complete primary repair of bladder exstrophy
**MSRE: modern staged repair of bladder exstrophy
***USS: ureterosigmoidostomy 

 

Functional results for reconstructed BEEC Day and night continence were coded as follows: no leaking or occasional leaking, and frequent to continuous leaking. 77% of adults and 65% of the adolescents had satisfactory continence, but only 12% of the children (p0.0001). No difference was found according to gender, or number of surgical procedures necessary to achieve continence. 78% 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 terms of group or gender. Details are shown in Table 4.

Table 4: Functional results of reconstructed BE 

 

Aults

(n=36)

N (%)

Adolescents

(n=17)

n(%)

Children

N=17

N (%)

P value

Voiding control

20 (55.6)

10 (62.5)

11 (64.7)

0.8

Daytime continence

No or little leaking

Frequent to continuous leaking

27 (77.1)

8 (22.9)

11 (64.7)

6 (35.3)

2 (12.5)

14 (87.5)

<.0001

Nightime continence

No or little leaking

Frequent to continuous leaking

26 (76.5)

8 (23.5)

10 (66.7)

5 (33.3)

1 (5.9)

16 (94.1)

<.0001

Sexual interest

None/few

Some/much

Missing data/inappropriate

6 (16.7)

28 (77.8)

2 (5.6)

1 (5.9)

9 (52.9)

7 (41.2)

0.22

Sexual activity

Yes

No

Missing data/inappropriate

26 (72.2)

10 (27.8)

2 (5.6)

5 (29.4)

5 (29.4)

7 (41.1)

0.13

Pleasure in sexual activity

None/few

Some/much

Missing data/inappropriate

5 (13.9)

26 (72.2)

4 (11.1)

0

3 (17.6)

14 (82.3)

0.83

Comparison of HRQOL between groups

Adolescents vs adults

Adolescents have superior quality of life than adults in all SF-36s dimensions, from 8 to 24 points (Table 5). Only physical functioning (Δ=-10.6, p=0.02) and role limitation due to mental health (Δ=-24.4, p=0.04) were statistically different.

Table 5: SF-36 dimension score for adults and adolescents

 

Aults

(n=36)

mA (sd)

Adolescents

(n=16)

mT(sd)

Δ

(mA-mT)

P value

French general population

Physical functioning

84.1 (24.9)

94.7 (5.9)

-10.6

0.02

84.4 (21.2)

Role physical

77.8 (35.70)

85.9 (28.8)

-8.2

0.43

81.2 (32.2)

Bodily pain

71.9 (27.5)

81.9 (20.9)

-10.1

0.20

73.4 (23.7)

General health

62.1 (25.0)

71.7 (20.1)

-9.5

0.18

69.1 918.6)

Vitality

53.6 (20.7)

61.6 (18.9)

-7.9

0.19

59.9 (18.0)

Social functioning

70.8 (30.3)

78.9 (22.7)

-8.1

0.34

81.5 (21.4)

Role emotional

66.7 (42.0)

91.1 (19.8)

-24.4

0.04

82.1 (32.1)

Mental health

61.7 (21.9)

72.7 (16.8)

-11.1

0.08

68.5 (17.6)

mA : mean for adults, mT mean for adolescents 

 

Children vs adolescents

Children’s scores are superior to adolescents on all VSPAEs dimensions except self esteem, vitality and relations with friends (Table 6). Relations with medical staff were significantly better for children (Δ=46.6, p=0.0002). However, relations with friends were significantly better for adolescents and superior to children’s. (Δ=23.7, p=0.01).

Table 6: VSP-AE dimensions and global score for adolescents and children 

 

Adolescents

(n=18)

mT (sd)

Children

(n=11)

mC(sd)

Δ

(mT-mC)

P value

French general population

Family

68.2 (17.2)

72.8 (12.1)

-4.6

0.40

63.9 (22.1)

Self-esteem

83.5 (11.5)

71.6 (21.3)

12.0

0.08

73.9 (21.1)

Vitality

80.4 (15.1)

70.0 (20.2)

10.4

0.15

77.7 (18.1)

Friends

63.1 (28.4)

39.3 (19.2)

23.7

0.01

48.7 (26.6)

Global well being

28.4 (19.8)

37.6 (20.3)

-9.2

0.24

71.4 (17.9)

Hobbies

45.4 (40.0)

55.8(37.0)

-10.4

0.48

66.7 (20.4)

Scholar work

57.9 (17.0)

72.8 (22.2)

-14.8

0.07

70.4 (24.2)

Relation with medical staff

22.0 (33.2)

68.6 (24.0)

-46..6

0.0002

Global score

54.4 (10.7)

61.1 (10.3)

-6.6

0.13

67.5 (12.9)

 mT : mean for adolescent, mC : mean for children

 

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-mental health. 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. For the children, the global number of surgical procedure is significantly related to relations with family (p=0.003). 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.

Discussion

In this retrospective multicenter study, we found that after reconstructive surgery for BE, most of the adults and 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 three dimensions. It is alarming that only 2 out of 17 children have little or no leaking in our study. Reports from the literature indicate continence rates ranging from 63% for Mollard [23], 70% for Gearhart [6] to 75% for Baka-Jakubiak [24]. However, the criteria for continence were different (dry interval of three 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 catheterisation (ICC), either transurethral or through a stoma, was performed by only 25% of adults and adolescents in our study. Catti reported 22% of women with ICC in his study [10]. Only two of the children performed ICC but this is not surprising as the mean age to start catheterisation is 12 years in our study. The results observed in terms of sexual activity among adults are also coherent with the literature.

Baird et al [13] report 54% of adults sexually active, Catti [10] 76% sexualy active women and Ebert 78% sexually active men [25]. Pleasure in sexual activity was not always evaluated. Missing data was 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. Socio-economic data in our study were difficult to compare with the literature, due to 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 pathology. 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 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 due to adolescents being still optimistic about future life, while adults have had to cope with the difficulty of confronting others, the greatest challenge being to find a partner in life. It could also be due to the fact that 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 tried to assess quality of life in 21 young adult patients in Brazil using the SF-36 [12]. As in our study, gender did not relate with HRQOL, but bad functional results were related to low quality of life. They did notice that the lowest score was obtained in the social domain, while 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 due to 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 importantly, 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, gender, 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 due to an evolution of quality of life between infancy and adolescence, mainly in terms of continence. It could also be due to the questionnaire being ill adapted for adolescents, as it was conceived for children from 8 to 10. However, Dodson and Gearhart [11] also report high adolescent scores in self esteem and family involvement but the HR QOL questionnaire used was very different from VSP-AE. Lemelle [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 quality of life is significantly related to influence on role limitation related to mental health for adults, psychological well-being for adolescents and self esteem for children. As a comparison, people suffering from 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.

Conclusion

Though incomplete, this preliminary study shows promising results confirming the clinical impression that functional results are strong determinants of quality of life. 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.

 

Appendix:

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, Strasbourg. Rémi Besson, PhD, Hôpital Jeanne de Flandres, Lille 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

 

Acknowledgements:

The authors are indebted to all the physicians and secretaries who participated in this study for their time and help in identifying the patients eligible. We would like to sincerely acknowledge Nina Huss for invaluable technical assistance and Fiona Ecarnot for editorial assistance.

 

 

 

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