- Email: [email protected]
SOMATIC FUNCTION, MENTAL HEALTH AND PSYCHOSOCIAL FUNCTIONING IN 22 ADOLESCENTS WITH BLADDER EXSTROPHY AND EPISPADIAS
00225347/98/1595-1684$03.00/0
THEJOLIR”.
Vol. 159,1684-1690,May 1998 Printed in U.S.A.
OF UROUXY
Copyright 0 1998 by AMERlCAN UROLOCICAL ASSOCV\nON, INC.
SOMATIC FUNCTION, MENTAL HEALTH AND PSYCHOSOCIAL FUNCTIONING IN 22 ADOLESCENTS WITH BLADDER EXSTROPHY AND EPISPADIAS TROND H. DISETH, ROALD BJORDAL, ALEXANDER SCHULTZ, MARIANNE STANGE RAGNHILD EMBLEM
AND
From the Division of Child and Adolescent Psychiatry and Sections of Pediatric Surgery and Urology, National Hospital, Oslo, Norway
ABSTRACT
Purpose: We report the long-term somatic outcome, mental health and psychosocial adjustment in adolescents with bladder exstrophy and epispadias. Materials and Methods: A total of 22 adolescents 11to 20 years old (median age 14.5),including 19 with bladder exstrophy and 3 with epispadias, were assessed for urogenital status, stoma, renal and bowel function, anorectal physiology, mental health and psychosocial functioning by physical examinations, semistructured interviews and standardized questionnaires. The parents of 21 patients were interviewed and completed questionnaires. Information was also obtained on control groups. Results: Of the 22 patients 9 (41%) had no urinary diversion and were urinary incontinent, 6 (27%) had persistent fecal staining and anal canal pressures that were lower than the controls, 10 (59%) were dissatisfied with the penile appearance and 11(50%) met the criteria for psychiatric diagnoses. The main predictors of mental health were parental warmth and patient genital appraisal in the 11to 14-year age group, and parental warmth and urinary continence function in the 15 to 20-year age group. Psychosocial dysfunction was predicted by fecal incontinence in the younger group and worries about future sexual relationships in the older group. Conclusions: The present multimodal outcome study revealed that adolescents with bladder exstrophy and epispadias had significant physical and mental problems. Genital malformation, and urinary and fecal incontinence may have a negative impact on mental health and psychosocial functioning. Our findings emphasize the need to include psychosocial experts on health care teams to reveal the amount of distress caused by these anomalies and to offer psychosocial support. KEYWORDS:bladder exstrophy, urinary incontinence, mental health, epispadias
Bladder exstrophy and epispadias involve urinary incontinence and genital malformation. Surgical treatment includes restoration of the pelvic ring, preservation of renal function, achievement of urinary continence and improvement of genital appearance. Efforts to achieve dryness require extensive resources to treat patients with bladder exstrophy and epispadias but there is still no uniform method for urinary continence management.1 However, any therapy for an infant with bladder exstrophy and epispadias should include a basis for emotional and social gr0wth.2~3Surgical followup studies of adults with bladder exstrophy and epispadias indicate adequate psychosocial integration and normal sexual hnction.4-7 However, to our knowledge there have been no studies to assess the mental and psychosocial outcome of children and adolescents with bladder exstrophy and epispadias. Nonorganic enuresis may be associated with adverse mental or psychosocial consequences.8 However, to our knowledge there are no long-term outcome studies of psychosocial functioning in children with other disorders that include organic urinary incontinence. Other studies of children with genital malformations indicate that these anomalies may be related to mental problems and psychosocial dysfunction. Compared to controls children with hypospadias are reported to experience adequate sexual adjustment but they also have more anxiety, depression and negative genital appraisal.9-11 A study of patients with micropenis demonstrated normal gender identity and good sexual function.lZ We examined Accepted for publication October 23, 1997
somatic outcome, mental health and psychosocial adjustment in adolescents with bladder exstrophy and epispadias, and assessed parental experiences. We also explored possible associations among somatic, mental and psychosocial outcome variables. SUBJECTS AND METHODS
Patients. Our primary sample included 29 patients born from 1974 to 1984 with bladder exstrophy and epispadias who were treated at our hospital. According to the Norwegian Medical Birth Registry, this group represents about 91% of all bladder exstrophy and epispadias patients born in Norway in this period. Of the original 29 patients 2 with severe associated anomalies (anorectal anomalies and multiple handicaps) were excluded from the study and another had emigrated. Of the remaining 26 patients 17 boys and 5 girls (85%), median age 14.5 years (range 11 to 201,consented to participate. The parents of 21 patients were interviewed and completed questionnaires. Data about previous medical treatment were collected from the medical records. There were 15 boys and 4 girls with bladder exstrophy and 2 boys and 1 girl had only epispadias. Bladder closure was performed in 14 of 19 patients (74%) with exstrophy at a median of 60 weeks post partum (range 2 to 156).No patient had pelvic osteotomy associated with bladder closure. Bladder disruptions developed in 4 patients (29%). Bladder neck reconstruction (V-Y plasty or Young-Dees technique) was performed in 4 patients (1 with bladder exstrophy, 3 with
1684
MENTAL AND PSYCHOSOCIAL OUTCOME IN ADOLESC:ENTS WITH BLADDER EXSTROPHY AND EPISPADIAS
epispadias) at a median age of 6.5 years (range 4 to 7). None of these patients achieved urinary continence. Of the 13 patients (59%) who underwent urinary diversion 8 had undergone previous bladder closure, 12 conduit urinary diversion (11with sigmoid colon, 1with ileum) at a median age of 3 years (range 1to 6.5), 1continent urinary diversion at age 19 years and 1placement of an artificial sphincter at age 18 years. Surgery for genital reconstruction was performed a median of 2 times (range 1 to 5) in all 22 patients, with the iirst procedure at a median age of 3 years (range 2 weeks to 6.6 years). Inguinal herniotomy was performed at a median age of 30 days (range 1 to 270) in 7 boys and 2 girls (47%)with exstrophy. None had rectal prolapse and all had an anteriorly placed anus. Control groups. The control group for the assessment of anorectal urogenital function included 7 hospitalized boys and 7 girls, mean age 16 years (range 10 to 20), with no urogenital or anorectal dysfunction. The control group for the psychiatric and psychosocial assessments was a subsample of 17 boys and 16 girls, mean age 15 years (range 14 to 17), selected randomly from a Norwegian epidemiological study of mental health.13.14 In this control group 6% of the adolescents had chronic illnesses of eczema and epilepsy. The clinical data or data in the medical records did not suggest any difference in cognitive functioning between the patients with bladder exstrophy and epispadias and controls. Baseline demographic characteristics of the sample, hospital admissions, operations and followup are presented in table 1. We found no significant differences between the patients and controls in gender, age or sociodemographic characteristics. At followup 13 patients (59%) had urinary diversion (conduit in 12 and continent in 1).The 9 patients without urinary diversion required diapers. The patient who was emptying the bladder by clean intermittent catheterization still required diapers. Somatic assessment. Urogenital function was assessed by obtaining a history of urinary continence status and clinical examinations. Urinary Continence was graded according to the Hollowell and Ransley clas~ification.~~ The classification was applied to all patients, regardless of whether they had urinary diversion, using maximum dry intervals of excellent-no urine loss during the day and night, and dry inter-
1685
vals for more than 4 hours (group l),good-some nighttime wetting and dry intervals for more than 3 hours (group 2). poor-dry intervals for not more than 2 hours and protection required (group 3) and wet-continuous leaking (group 4). For simplicity in reporting results the terms satisfactory (groups 1 and 2) and unsatisfactory (groups 3 and 4) are used.15 Local status of the urinary stoma was assessed with regard to stomal site size and shape, and peristomal skin.16 The degree of urine leakage from the collecting system, the urostomy appliances and stoma care routines were also evaluated. Genital malformations were classified as severe or mild. Severe malformation was defined as a short, broad, upturned penis (tight dorsal chordee) with urethral orifice on the penile shaft or at the penopubicjunction, or bifid clitoris with a urethra split to a variable extent.17 Satisfaction with genital appraisal and function was assessed on a 10 cm. visual analog scale, ranging from 0 (bery satisfied with the genital appearance and function”)to 10 (bery dissatisfied”). The adolescents were interviewed regarding experiences with erection, ejaculation, masturbation, intercourse, and thoughts of establishing a close sexual relationship, family and having children in the future. Renal function was assessed by glomerular filtration rate measured by 99”technetriumpentetic acid. Glomerular filtration rate above 70 was considered normal.18 Anorectal function was assessed by clinical and physiological examination with anal canal manometry.19 Fecal continence was graded according to the Wingspread classification, rating 4 clinical states of continence as 1-clean, 2--staining, S i n termittent fecal soiling and 4-constant soiling.20 Flatus continence function was assessed by asking patients to indicate the control of flatus on a 10 cm. visual analog scale.21 Psychiatric and psychosocial assessments. The psychiatric assessment of the adolescents was based on a semistructured interview, the Child Assessment Schedule (CAS),22,23which provided a total problem score of symptom complexes analogous to psychiatric diagnoses of the American Psychiatric Association DSM-III-R.24 Standardized questionnaires, Youth Self-Report25 and Child Behavior Checklist26 provided a combined total behavior and emotional score with internalizing and externalizing subscales. Overall psychosocial functioning of the adolescents was assessed by the Children’s Global Assessment Scale (CGAS),27which was based on information from patient interview. The scores ranged from 100 (excellent function) to 0 (severe malfunction). PsychosoTABLE1. Demographic chamcteristics of adolescents with bladder cial problems of clinical significance were scores below 71. ezstrophv and eDispadias. and a population group The interviews were supplemented with questions concernPopulation Bladder Exstrophy and ing adolescent view of the present situation regarding the Epispadias (22pts.) (33 pts.) anomalies. 14.5 (11-20) 15 (14-17) Median age, (range) Parents were interviewed separately using a slightly mod17 (52) 17 (77) NO.boys (%) ified version of the standardized semistructured interview, NO.family composition (90): Parental Account of Children’s which included 22 (67) 17 (77) Both biological parents 11 (33) 5 (23) Single biological parent ratings of maternal warmth and criticism towards the index Median yrs. parental education child. A global assessment of chronic family difficulties29.30 (range): was based on all parental information available about the 14 (9-17) 12.5 (9-19) Mother previous and present family situation. Parent interviews 14 (9-17) 12 wig) Father were supplemented with questions regarding emotional and NO.community (%): 1s (55) 10 (45) Urban practical aspects of treatment procedures and medical fol15 (45) 12 (55) Rural lowup. Median hospital admissions Adolescent and parent interviews were performed by a (range): 0 (0-2) 15 (6-23) Total hospital admissions child psychiatrist with no knowledge of the surgical condition 9 (2-15) 0 (0-2) Hospital admissions before and were videotaped for testing of inter-rater reliability. This age 5 yrs. assessment of inter-rater reliability was based on 10 ran7 (1-14) 26 (14-114) Longest hospital stay (days) domly selected videotaped interviews independently scored 4 (1-13) 11 (7-18) Age at last hospital admisby another child psychiatrist. The intraclass correlation was sion 1 (0-1) 4 (3-10) No. operations 0.93 for the CAS, 0.84 for the CGAS and 0.91 for the chronic NO.urinary continence treatfamily difficulties scores. The kappa correlation coefficients ment (%): were 0.74 for parental warmth and 0.55 for parental c d i 0 12 (55) Conduit diversion 0 Continent diversion 1 (5) cism. 0 9 (41) Diauers Statistics. Due to abnormal distributions results are re-
1686 MENTAL AND PSYCHOSOCIAL OUTCOME IN ADOLESCENTS WITH BLADDER EXSTROPHY AND EPISPADIAS ported as medians (ranges) unless otherwise stated. Nonparametric statistics primarily were used.31 For assessment of differences between independent groups a chi-square test with Yaks correction or Fisher's exact test for small samples was used for categorical variables and the Mann-Whitney U-test was used for continuous variables. Dependency between sets of variables is expressed through the Spearman's rank correlation coefficient. Multiple regression analyses with a stepwise (forward) procedure were conducted to determine independent somatic and family/parental variables contributing significantly to the explained variance of adolescent mental health (CAS) and psychosocial function (CGAS).32 Careful checks of model assumptions, including investigations of residual plots, did not reveal any violation of the assumptions and 2-tailed p <0.05 was considered statistically significant. Since a considerable number of tests were performed, the most emphasis is on results with low p values. All calculations were performed using statistics software. Informed written consent was obtained from the adolescents and the parents. The study was approved by the Regional Ethics Committee for Medical Research. RESULTS
Somatic condition. Urinary, fecal and flatus continence, anal canal pressures and genital status in patients and controls are given in table 2. Of the 13 patients with urinary diversion 12 had satisfactory urinary continence (groups 1 and 2). One patient with conduit urinary diversion had fiequent urine leakage from the collecting system day and night. However, 2 patients had weekly to daily urine leakage and 2 had monthly leakage. These 5 patients reported that the urine leakage interfered with social activities. The stomal complications were not due to an obviously inadequate stoma placement, improper size or shape, or peristomal skin problems but to poor stomal care routines, improper urostomy appliances and poor stomal care followup. All 9 patients (6 with exstrophy, 3 with epispadias) without diversion were urinary incontinent (groups 3 and 4). They used a median of 4 diapers (range 1 to 10)day and night. There were 5 patients who reported that using diapers influenced the social life because of unpleasant odor, bullying or always having to bring a change of diapers. Of the 19 adolescents with bladder
exstrophy 6 (32%)had fecal staining at followup and 4 had fecal staining until late childhood. None of the patients with epispadias had fecal continence problems. With regard to fecal continence function, no significant differences in age or sex were found. Glomerular filtration rate was a median of 83 (range 42 to 108), and 5 patients (28%) had a glomerular filtration rate below the cutoff (median 57, range 42 to 68). In 5 patients with exstrophy and 1with epispadias, 3 girls and 3 boys, the genital malformations were assessed by the surgeon as mild. On the visual analog scale 10 boys (59%) indicated dissatisfaction with the genital appearance and function (median 5.0,range 4 to 9), whereas all 5 girls were satisfied (median 0, range 0 to 2). Four boys and 1 girl (23%) stated that the genital malformations had influenced the thought of establishing a close sexual relationship, 5 boys and 2 girls (32%) reported worrying about the future ability to establish a family and have children, 16 patients (73%)reported dating, 16 (94%)had erection and 10 (59%)had ejaculation. Ten boys and 2 girls (55%),median age 17.5 years (range 14 to 20), regularly masturbated. Four adolescents had had sexual intercourse, and the median age of initial intercourse was 17 years (range 16 to 17). Mental health and psychosocial function. Of the 22 adolescents 11 (50%) met the criteria for a psychiatric diagnosis according to the DSM-111-R criteria (table 3). Two adolescents received 2 diagnoses. There were significant differences in the prevalence of psychiatric diagnoses between patients and controls. Most of the psychopathology concerned emotional and internalizing problems, such as overanxiousness, sadness, depression, low self-esteem, poor body concept and withdrawal. The adolescents with obsessive-compulsive disorders had recurrent and persistent thoughts or images of the multiple operations and hospital stays, wearing diapers because of urinary incontinence, genital malformations, body or sexuality. One also had compulsions of "always having to run after women" but "when it comes to intercourse I run away." Much energy was used in hiding the anomaly and symptoms. These thoughts interfered with social activities or relationships with others, and they became isolated and withdrawn. The bladder exstrophy and epispadias group had a median score of 82.5 on the CGAS which was not significantly different from the population group score of 85. The 11 adolescents
TABLE2. Urinary, fecal and flatus continence function, anal canal pressures and genital malformations of adolescents with bladder exstrophy and epispadias and controls Bladder Exstrophy and Epispadias (22 Db.)
TABLE 3. Mental health and psychosocial functioning in adolescents with bladder exstrophy and epispadias, and population
Controls (14 pts.)
POUP
No. urinary continence function (%):*
Satisfactory, groups 1 + 2 Unsatisfactory, groups 3 + 4 No. fecal continence function
12 (55) 10 (45)
13 (93) l ( 7 , p <0.05)
(%):t 14 (100) Continent, grade 1 16 (73) 0 (0, p <0.05) Incontinent, grades 2-4 6 (27) No. flatus continence function i%):$ Continent, score 0-3 12 (86) 19 (86) Incontinent, score 4-10 3 (14) 2 (14) Median an.water anal canal pressures (range): 98 (80-105, p (0.05) Resting 70 (40-100) Squeezing 200 (90-300,p 10.05) 110 (50-200) No. genital malformations (96): 0 14 (100, p <0.05) None Mild 6 (27) 0 0 16 (73) Severe * Graded according to Hollowell and Ransley classification. f Graded according to the Wingspread classification of clean-1, stain ing-2, intermittent fecal soiline-3 - and constant fecal soiline-4. t Indicated on a 10 cm. visual analog scale.
No. DSM-111-R mental health diagnosis:24 Dysthymic disorder Simple phobia Overanxious disorder Conduct disorder Obsessive-compulsive disorder Total No. (55) Median total problem CAS scores Median Child Behavior Checklist raw scores (range)? Total behavior Internalizing Externalizing Median Youth Self-Report raw scores Total behavior Internalizing Externalizing Median Psychosocial functioning CGAS scores (ranee):"' -
Bladder Exstrophy
Population
and(22 Epispadias Db.)
(33 pts.)
2 1 3 1 4 11 (50) 21 ( 5 4 8 )
4 (12, p <0.01) 15 ( 3 4 4 )
9 2 2
(1-75) (0-14) (0-35)
29 (12-128) 7.5 (1-46) 11 (3-37) 82.5 (52-98)
7 (0-76) 4 (0-19) 2 (0-39) 22 (0-86) 5 (0-30) 7 (0-45) 85 (55-98)
MENTAL AND PSYCHOSOCIAL OUTCOME IN ADOLESCENTS WITH BLADDER EXSTROPHY AND EPISPADIAS 1687
without a psychiatric diagnosis had a median CGAS score of 92 (range 81 to 981,indicating optimal psychosocial functioning. Of the 11 adolescents who met the DSM-111-R criteria for a psychiatric diagnosis 8 had CGAS scores less than 71, implying clinically significant psychosocial problems. Regarding mental health and psychosocial functioning, we found no significant difference in sex or age between the urinary continent and incontinent adolescents or between those who had urostomies and those who required diapers. However, patients with severe genital malformations had a significantly higher prevalence of psychiatric diagnoses (chisquare = 6.77,df 1,p = 0.009),higher CAS scores (z = -2.09, p = 0.045)and lower CGAS scores (z = -2.18, p = 0.029) than patients with mild genital malformations. Furthermore, we found significantly more mental health problems (CAS) in the patients with flatus incontinence (visual analog scale 4 to 10) than in patients who were continent (z = -2.11, p = 0.035). The answers about present physical and/or psychosocial problems are presented in table 4.Only 1 adolescent reported no problems at the time of the assessments. There were 15 adolescents (68%) who reported teasing or bullying because of the incontinence, diapers, stomas, genital malformations or scars. However, 16 adolescents (72%) reported that the physical and emotional problems were not difficult to talk about to parents or close friends. The advice that they would give to parents of children with bladder exstrophy and epispadias included openness, a provision of information and a normal upbringing with a supportive attitude, especially regarding self-esteem and autonomy. Family and parental difficulties. The economy, housing, education, and physical and mental health of the family members of the bladder exstrophy and epispadias group were generally good. No or only minor chronic family difficulties were reported (median 2.0, range 0 to 5). Chronic family difficulties were primarily due to somatic and mental health problems in the parents or the siblings and/or minor family conflicts. No significant differences were found between the bladder exstrophy and epispadias, and population groups with regard to parental warmth or criticism toward the adolescents. Of the parents 57% described the first year as practically and emotionally difficult, 67% expressed worries about the future sexual orientation of the adolescent, possibilities for sexual relationships, marriage and parenthood or the impact of the malformation on self-esteem and confidence, and 19% mentioned worries about how the adolescents managed the present somatic and psychosocial situation as the most difficult. For 43% of the parents the problems of the child had influenced positively married and family life, for example, they had become emotionally closer as partners, united by the experience of having a disabled child, and 90% experienced support from family and friends. The anomaly and physical problems had never been difficult to discuss. TABLE4. Present physical and psychosocial problems reported in 22 adolescents with bladder exstrophy and epispadias
-
Total No. (No. boysf No. girls)
Womes about genital malformation 15 (132) Womes about urinary incontinence 9 (7/2) Unpleasant smelVdirty trousers 8 (711) Uncertainty with physical closeness 8 (711) and shyness in tinding a partner* Problems with self-esteemmodyimage I (710) Walking pain in hipsfpelvis 6 (U2) Womes about fecayflatus incontinence 6 (511) Stoma1 problems 5 (5/0) Abdominal pain, urinary tract infec5 (5/0) tions or stones Womes about scars 2 (ZO) No problems 1 (YO) * This question was posed to 11 patients older than 14 years.
Associations among somatic variables, parental I family variables, and mental and psychosocial outcome. The patients were classified into the 2 age groups of ll to 14 years (11)and 15 to 20 years (11).33 In the younger group more mental problems (CAS symptom score) were correlated with less parental warmth (r, = 0.68,p = 0.021)and more chronic family difficulties (r, = 0.63,p = 0.038).Poorer psychosocial function (CGAS) was correlated with fecal incontinence (r, = -0.75,p = 0.008),flatus incontinence (r, = -0.70, p = 0.016) and less parental warmth (r, = -0.66,p = 0.026).In the older group more mental problems (CAS symptom score) were correlated with urine incontinence (r, = 0.63, p = 0.0391,less parental warmth (r, = 0.67,p = 0.036)and more parental criticism (r, = 0.76,p = 0.010).Poorer psychosocial function was correlated with less parental warmth (r, = -0.73,p = 0.017)and more parental criticism (r, = -0.77, p = 0.009). To investigate further the contribution of the independent somatic and family/parental variables in predicting psychosocial (CGAS) and mental (CAS) outcome, stepwise multiple regression analyses were used. With CGAS (psychosocial function) as the dependent variable, the somatic correlates (urinary, fecal and flatus continence, and genital appraisal), gender and adolescent belief in the possibility of a close sexual relationship, and the familylparental correlates (chronic family difficulties, parental warmth and criticism) were entered as explanatory variables. The only predictor of CGAS in the younger group (11to 14 years) was fecal continence function, explaining 53% of the variance ( p = -13.7, p = 0.01).In the older group belief in the possibility of a close sexual relationship was the only predictor of CGAS,explaining 69% of the variance ( p = 24.8,p = 0.002). With CAS symptom score (mental health) as the dependent variable, somatic correlates, gender, and family and parental correlates were entered as explanatory variables. In the younger group parental warmth was the strongest predictor of CAS, explaining 64% of the variance ( p = 6.7,p = 0.0018). Adolescent satisfaction with genital appearance and function added another 18% ( p = 1.04,p = 0.011).In the older group parental warmth was the strongest predictor of CAS, explaining 68% of the variance Cp = 6.3,p = 0.002).Urinary continence added another 13% ( p = 0.8,p = 0.03). DISCUSSION
The discovery of significant persistent problems in the adolescents with bladder exstrophy and epispadias contrasts with reports of satisfactory long-term urinary control in patients with bladder exstrophy and epispadias with or without urinary diversion.4.5.15,34-37 However, the findings are consistent with multicenter followup reports of unsatisfactory urinary control in 75 to 86% of children with bladder exstrophy and epispadias without primary urinary diversion,3ss39 and with recent reports that men with bladder exstrophy and epispadias were bothered by penile appearance and function.4.5 The diverse findings are probably due to the development of surgical techniques, such as the recent aggressive functional reconstruction with primary pelvic closure, which seems to achieve better urinary continence. The inconsistency may also be due to methodological problems, such as the definition of urinary continence and sexual function or the inclusion of different age groups. Children, adolescents and adults have different developmental tasks regarding the anomalies. However, in our study the variety in somatic outcome made it possible to assess the effect of somatic risk factors on mental and psychosocial dysfunction, regardless of operative treatment procedures. The retrospective design of the study and the small number of patients may limit the generalizability and comparability of varying treatment procedure results, and we ad& . the caution in interpreting the statistical a n d p e ~However,
1688 MENTAL AND PSYCHOSOCIAL OUTCOME IN ADOLESCENTS WITH BLADDER EXSTROPHY AND EPISPADIAS present sample is representative of the Norwegian patient which indicates good global psychosocial functioning and is population from 13 to 23 years ago. All but 3 patients born in accordance with recent outcome studies of adults with However, -~ comparisons during this time were included in the study. Adolescents with bladder exstrophy and e p i ~ p a d i a s . ~ bladder exstrophy and epispadias had a 4-fold increase in with these studies are difficult due to differences in age, psychiatric disorders compared to the control group and gen- patient sampling and methods. The finding that fecal inconeral pediatric population.4o.41 This overrepresentation ex- tinence predicted psychosocial dysfunction in the younger ceeds the generally doubled risk of maladjustment in chil- bladder exstrophy and epispadias group coincides with finddren with chronic illnesses compared to healthy children42 ings in patients with fecal incontinence due to anorectal and coincides with the results of studies of Norwegian ado- anomalies and Hirschsprung‘s disease.21.48.49 Fecal staining is shameful and socially unacceptable regardless of the dislescents with anorectal anomalies.21 The high risk of psychiatric disorders among the adoles- order. However, it seems that although adolescents with cents with bladder exstrophy and epispadias is probably not bladder exstrophy and epispadias may have a high frequency only due to having a chronic physical illness but more spe- of specific emotional problems, these do not necessarily incifically related to characteristics of the anomaly, such as fluence the present social functioning within the family, at urinary incontinence and severity of genital malformation. In school or among peers to the same degree as for children with the older adolescents mental health problems were corre- anoredal anomalies.21 In contrast with fecal and flatus inlated with poor urinary continence function. In the younger continence, urinary incontinence does not involve such a foul patients dissatisfaction with genital appearance and function smell and does not disturb others. Fecal and urinary inconwas a predictor of mental health problems. Such distress was tinence may also impact families differently. Our findings not adequately expressed in the consultation with the pedi- emphasize the necessity of considering the nature and stage atric surgeon or urologist. For adolescents body image, self- of the chronic illness, symptom severity and visibility,50 and esteem, sexuality, sexual function and fertility are funda- parental and family related factors when assessing the immentally important. The adolescents in our study had more pact of chronic disabilities on mental health and psychosocial problems with body image or self-esteem, worries about the functioning. genital malformation, present or future sexual function, and the possibility of having children than had been reported in CONCLUSIONS previous studies.4-7.43-45 More negative genital appraisal Adolescents with bladder exstrophy and epispadias who was noted in our boys (59%)than in boys with hypospadias (25%) and comparison subjects (5%) without any genital underwent operations 13 to 23 years ago have significant persistent physical and mental problems. However, the suranomaly reported by Mureau et al.11 Emotional and body image development may be affected gical treatment of these patients was not what would be profoundly by genital deformity and reconstructive sur- considered standard therapy today and the results probably gery.46.47 Given the importance of early feedback and social do not reflect the status of exstrophy management today. The interactions with parents and later with peers, the earlier adaptation of all patients with bladder exstrophy and episthe genital repair, the more likely the child will achieve a padias must be considered in the context of the specific chalpsychologically healthy perspective about body image. Nor- lenges posed by the urogenital malformation, as well as in mal psychosocial and psychosexual development is more the context of the current developmental phase of the paprobable if it is anticipated and prepared for in childhood.45 tient. Mental health was determined by genital appearance, The importance of parental confidence, expectations and re- urinary continence and parental warmth. Thus, optimal sponses is confirmed by our finding that high parental treatment and followup of patients with bladder exstrophy warmth was the most important predictor of mental health and epispadias require collaboration among the pediatric and is consistent with the report of Reilly and Woodhouse.12 surgeon, adult urologist, stoma1 therapist and psychosocial They emphasize that the strongest influence for all patients expert from birth to adulthood. Professional mental health with micropenis was the “parental attitude; well informed, guidance with a developmental and family perspective open and supportive parents who explained the problems and should be offered routinely to the patient with bladder exencouraged normality produced confident and well adjusted strophy and epispadias and the parents. children.” Dr. Inger Helene Vandvik provided scientific advice, Dr. Different developmental tasks may create different levels Thore Egeland statistical support and Sonja Rosvold practiof vulnerability in childhood and adolescence, highlighting cal help. Dr. Astrid Aasland scored the videos used in assessthe need for individual psychosocial support at all stages of ing reliability. care. In early adolescence children compare themselves with peers of the same sex and are anxious about being different REFERENCES from them, as exemplified by the finding that the boys with 1. Dewan, P. A: Historical trends in the management of bladder bladder exstrophy and epispadias often stopped showering in exstrophy. Ped. Surg. Int., 10 289, 1995. public from age 12 to 13 years. Dissatisfaction with genital 2. Lattimer, J. K, Hensle, T. W., MacFarlane, M. T., Beck, L., appearance and function was the only somatic predictor of Braun, E. and Esposito, Y.: The exstrophy support team: a new mental health problems in the youngest bladder exstrophy concept in the care of the exstrophy patient. J.Urol., 121: 472, and epispadias group. In late adolescence social and sexual 1979. relationships with the opposite sex are increasingly impor3. Vandvik, I. H. and Storhaug, K.: Family-focused services for tant. For example, wearing diapers because of urine incontichildren with rare disorders, exemplified by bladder exstronence when a boy is planning to date is the type of challenge phy. Clin. Ped., 24: 97, 1985. that may lead to withdrawal and isolation. Urinary conti4. Feitz, W. F. J., Van Grunsven, E. J. K. J. E. M., Froeling, F. M. J. A. and De Vries, J. D. M.: Outcome analysis of the nence function was the only somatic predictor of mental psychosexual and socioeconomical development of adult pahealth in the older bladder exstrophy and epispadias group. tients born with bladder exstrophy. J. Urol., 152 1417, 1994. The need for specific individual patient psychological coun5. Ben-Chaim, J., Jeffs, R. D., Reiner, W. G. and Gearhart, J. P.: seling, including opportunities to discuss feelings about the The outcome of patients with classic bladder exstrophy in anomaly symptoms, self-esteem, body image, sexual matters adult life. J. Urol., 155 1251,1996. and womes about the future should be assessed during fol6. Avolio, L., Koo, H. P., Bescript, A. C., Snyder, H. M., Canning, lowup. D. A. and Duckett, J. W., Jr.: The long-term outcome in men In contrast to the high frequency of specific psychiatric with exstrophy/epispadias: sexual function and social integraproblems, we found an almost normal median CGAS score, tion. J. Urol., 156 822, 1996.
MENTAL AND PSYCHOSOCIAL OUTCOME IN ADOLESCENTS WITH BLADDER EXSTROPHY AND EPISPADIAS
1689
7. Stein, R., Hohenfellner, K., Fisch, M., Stockle, M., Beetz, R. and 130 1142,1983. Hohenfellner, R.: Social integration, sexual behavior and fer- 35. Mesrobian, H. G. J., Kelalis, P. P. and Kramer, S. A,: Long-term tility in patients with bladder exstrophy - a long-term follow followup of 103 patients with bladder exstrophy. J. Urol., 139: up. Eur. J. Ped., 155 678,1996. 719, 1988. 8. Shaffer, D.:Enuresis. In: Child and Adolescent Psychiatry. Ed- 36. Connor, J. P., Hensle, T. W., Lattimer, J. K. and Burbige, K. A,: ited by M. Rutter, E. Taylor and L. Hersov. Oxford Blackwell Long-term followup of 207 patients with bladder exstrophy: an Scientific Pub., chapt. 29,pp. 505-519, 1994. evolution in treatment. J. Urol., 142 793, 1989. 9. Robertson, M. and Walker, D.: Psychological factors in hypospa- 37. Gearhart, J. P. and Jeffs, R. D.: Bladder exstrophy: increase in dias repair. J. Urol., 113 698,1975. capacity following epispadias repair. J. Urol., 142:525,542, 10. Berg, R., Berg, G. and Svensson, J.: Penile malformation and 1989. mental health. A controlled psychiatric study of men operated 38. de la Hunt, M. N. and O'Donnell, B.: Current management of for hypospadias in childhood. Acta Psychiat. Scand., 86: 398, bladder exstrophy: a BAPS collectivereview from eight centres 1982. of 81 patients born between 1975 and 1985.J. Ped. Surg., 24: 11. Mureau, M.A. M., Slijper, F. M. E., Nijman, R. J. M., Van Der 584, 1989. Meulen, J. C., Verhulst, F. C. and Koos Slob, A.: Psychosexual 39. Kockum, C. C., Hansson, E., Stenberg, A., Svensson, J. and adjustment of children and adolescents after different types of Malmfors, G.: Bladder exstrophy in Sweden - a long-term hypospadias surgery: a norm-related study. J. Urol., 154: follow-up study. Eur. J. Ped. Surg., 6: 208, 1996. 1902, 1995. 40. Rutter. M.:Isle of Wieht revisited: twentv-five vears of child 12. Reilly, J. M. and Woodhouse, C. R. J.: Small penis and the male psychiatric epidemioiogy. J. Amer. Acad. khild Adol. Psychiasexual role. J. Urol., 142 569,1989. try, 2 8 633,1989. 13. Gjone, H. and Nevik, T. S.: Parental ratings of behaviour prob- 41. Brandenburg, N. A., Friedman, R. M. and Silver, S. E.: The lems: a twin and general population comparison. J. Child epidemiology of childhood psychiatric disorders: prevalence Psychol. Psychiatry, 36 1213,1995. findings from recent studies. J. Amer. Acad. Child Adol. Psy14. Nevik, T. S.:Are postal surveys in child psychiatry feasible? chiatry, 2 9 76, 1990. Results of an epidemiological study. Nord. J. Psychiatry, 4 9 42. Pless, I. B. and Nolan, T.: Revision, replication and neglect337, 1995. research on maladjustment in chronic illness. J. Child Psychol. 15. Hollowell, J. G. and Ransley, P. G.: Surgical management of Psychiatry, 3 2 347,1991. incontinence in bladder exstrophy. Brit. J. Urol., 68: 543,1991. 43. Woodhouse, C. R. J., Ransley, P. G. and Williams, D. I.: The 16. Nordstrem, G. M., Borglund, E. and Nyman, C. R.: Local status patient with exstrophy in adult life. Brit. J. Urol., 5 5 632, of the urinary stoma - the relation to peristomal skin compli1983. cations. Scand. J. Urol. Nephrol., 2 4 117,1990. 44. Mesrobian, H. G. J., Kelalis, P. P. and Kramer, S. A.: Long-term 17. Rickwood, A. M. K.: Exstrophic anomalies. In: Neonatal Surgery, followup of cosmetic appearance and genital function in boys 3rd ed. Edited by J. Lister and I. M. Irving. Butterworth & Co., with exstrophy: review of 53 patients. J. Urol., 136 256,1986. chapt. 51,pp. 709-717, 1990. 45. Woodhouse, C.R. J.: The sexual and reproductive consequences 18. Rootwelt, K.: Nukleaxmedisin. Oslo: Universitetsforlaget, pp. of congenital genitourinary anomalies.J. Urol., 152 645,1994. 86-87, 1995. 46. Schultz, J. R., Klykylo, W. M. and Wacksman, J.: Timing of 19. Emblem, R., Diseth, T. H., Merkrid, L., Stien, R. and Bjordal, R.: elective hypospadias repair in children. Pediatrics, 71: 342, Anal endosonography and physiology in adolescents with cor1983. rected low anorectal anomalies. J. Ped. Surg., 29 447,1994. 47. American Academy of Pediatrics: Timing of elective surgery on 20. Stephens, F. D.and Smith, E. D.: Classification, identification, the genitalia of male children with particular reference to the risks, benefits, and psychological effects of surgery and anesand assessment of surgical treatment of anorectal anomalies. Ped. Surg. Int., 1: 200,1986. thesia. Pediatrics, 97.590, 1996. 21. Diseth, T.H. and Emblem, R.: Somatic function, mental health 48. Diseth, T. H., Emblem, R. and Vandvik, I. H.: Adolescents with anorectal malformations and their families. Fam. Systems and psychosocial adjustment of adolescents with anorectal anomalies. J. Ped. Surg., 31: 638,1996. Med., 1 3 215, 1995. 22. Hodges, K.,Kline, J., Stem, L., Cytryn,L. and McKnew, D.: The 49. Diseth, T. H., Bjemland, K, Novik, T. and Emblem, R.: Bowel function, mental health and psychosocial function in adolesdevelopment of a child assessment interview for research and cents with Hirschsprung's disease. Arch. Dis. Child., 76 100, clinical use. J. Abnorm. Child Psychol., 10 173,1982. 23. Hodges, K.: Manual for the Child Assessment Schedule (CAS). 1997. 50. Garralda, M. E.: Chronic physical illness and emotional disorder Columbia: University of Missouri, 1985.Unpublished. in childhood:where the brain's not involved, there may still be 24. American Psychiatric Association: Diagnostic and Statistical problems. Brit. J. Psychiatry, 1W. 8, 1994. Manual of Mental Disorders, 3rd ed. Washington, D.C.: American Psychiatric Association, 1987. 25. Achenbach, T. M. and Edelbrock, C. S.: Manual for the Youth EDITORIAL COMMENT Self Report and Profile. Burlington, Vermont: University of The authors provide a detailed description of the psychosocial Vermont, Department of Psychiatry, 1987. 26. Achenbach, T. M.: Manual for the Child Behavior Checklist and functioning of adolescents with bladder exstrophy. However, the 1991 Profile. Burlington, Vermont University of Vermont, population in the study may not be representative of bladder exstrophy patients treated a t centers with specific expertise in this disorDepartment of Psychiatry, 1991. 27. Shaffer, D., Gould, U. S., Brasic, J., Ambrosini, P., Fisher, p., der. For example, although the authors state that surgical treatment Bird, H. and Aluwahlia, S.: Children's Global Assessment includes restoration of the pelvic ring, none of the patients studied had the benefit of pelvic osteotomy and almost none underwent early Scale (CGAS).Psychopharm. Bull., 21: 747, 1985. 28. Taylor, E.: The overactive child. In: Clinics in Developmental closure. The lack of pelvic closure contributes to poor cosmetic outcome, and fecal, flatus and urinary incontinence. Patients in this Medicine, No. 97.Oxford: Blackwell Scientific Pub., 1986. 29. Vandvik, I. H., Hgyeraal, H. M. and Fagertun, H.: Chronic fam- series were considered to have had urinary continence if they had a ily difficulties and stressful life events in recent onset juvenile successful incontinent urinary diversion. It is misleading to describe the psychosocial adaptation of patients with urinary continence arthritis. J. Rheumatol., 16 1088, 1989. 30. Bjomstad, P., Lindberg, H. and Spurkland, I.: Unge hjerter i when they all wear an appliance. This group cannot be considered to be the same as adolescents who have functional reconstruction and faresonen. (Young hearts a t risk). Oslo: Tan0 A. S., 1990. 31. Altman. D. G.:Practical Statistics for Medical Research. London: true urinary continence. Cosmetic issues are important to the adolescent. These issues go Chapman and Hall, 1991. 32. Kleinbaum, D., Kupper, L. and Muller, K: Applied Regression beyond the genital appearance. The majority of children in this Analysis and Other Multivariable Methods, 2nd ed. Boston: group had undergone urinary diversion, presumably resulting in abdominal scars and stomal appliances. While genital appearance PWS-KENT Publishing Co., 1988. 33. Mangs and Martell: 0-20 C enligt psykoanalytisk teori. Stock- only affects intimate encounters, the other surgical deformities are obvious in many social situations. No information is provided as to holm: Natur och Kultur, 1974. 34. k p o r , H. and Jeffs, R. D.: Primary bladder closure and bladder the type of genital reconstruction performed or the adquaey of this neck reconstruction in classical bladder exstrophy. J. Urol., reconstruction. Therefore, the implications about the genital malfor-
1690 MENTAL AND PSYCHOSOCIAL OUTCOME IN ADOLESCENTS WITH BLADDER EXSTROPHY AND EPISPADIAS mation and its effect on the psychosocial functioning of the adolescent are difficult to put into perspective. Psychological evaluation and followup are vital to the well-being of the child with bladder exstrophy. The psychosocial impact of bladder exstrophy will likely be minimized through early functional reconstruction with the expectation of achieving urinary continence in the majority of children, satisfactory pelvic closure with the secondary benefits of improved fecal control and cosmesis, and minimizing the
outward signs of physical deformity, such as stomas, appliances and large surgical scars. Steve Docimo Department of Urology James Buchanan Brady Urological Institute Johns Hopkins Hospital Baltimore, Maryland
THEJOLIR”.
Vol. 159,1684-1690,May 1998 Printed in U.S.A.
OF UROUXY
Copyright 0 1998 by AMERlCAN UROLOCICAL ASSOCV\nON, INC.
SOMATIC FUNCTION, MENTAL HEALTH AND PSYCHOSOCIAL FUNCTIONING IN 22 ADOLESCENTS WITH BLADDER EXSTROPHY AND EPISPADIAS TROND H. DISETH, ROALD BJORDAL, ALEXANDER SCHULTZ, MARIANNE STANGE RAGNHILD EMBLEM
AND
From the Division of Child and Adolescent Psychiatry and Sections of Pediatric Surgery and Urology, National Hospital, Oslo, Norway
ABSTRACT
Purpose: We report the long-term somatic outcome, mental health and psychosocial adjustment in adolescents with bladder exstrophy and epispadias. Materials and Methods: A total of 22 adolescents 11to 20 years old (median age 14.5),including 19 with bladder exstrophy and 3 with epispadias, were assessed for urogenital status, stoma, renal and bowel function, anorectal physiology, mental health and psychosocial functioning by physical examinations, semistructured interviews and standardized questionnaires. The parents of 21 patients were interviewed and completed questionnaires. Information was also obtained on control groups. Results: Of the 22 patients 9 (41%) had no urinary diversion and were urinary incontinent, 6 (27%) had persistent fecal staining and anal canal pressures that were lower than the controls, 10 (59%) were dissatisfied with the penile appearance and 11(50%) met the criteria for psychiatric diagnoses. The main predictors of mental health were parental warmth and patient genital appraisal in the 11to 14-year age group, and parental warmth and urinary continence function in the 15 to 20-year age group. Psychosocial dysfunction was predicted by fecal incontinence in the younger group and worries about future sexual relationships in the older group. Conclusions: The present multimodal outcome study revealed that adolescents with bladder exstrophy and epispadias had significant physical and mental problems. Genital malformation, and urinary and fecal incontinence may have a negative impact on mental health and psychosocial functioning. Our findings emphasize the need to include psychosocial experts on health care teams to reveal the amount of distress caused by these anomalies and to offer psychosocial support. KEYWORDS:bladder exstrophy, urinary incontinence, mental health, epispadias
Bladder exstrophy and epispadias involve urinary incontinence and genital malformation. Surgical treatment includes restoration of the pelvic ring, preservation of renal function, achievement of urinary continence and improvement of genital appearance. Efforts to achieve dryness require extensive resources to treat patients with bladder exstrophy and epispadias but there is still no uniform method for urinary continence management.1 However, any therapy for an infant with bladder exstrophy and epispadias should include a basis for emotional and social gr0wth.2~3Surgical followup studies of adults with bladder exstrophy and epispadias indicate adequate psychosocial integration and normal sexual hnction.4-7 However, to our knowledge there have been no studies to assess the mental and psychosocial outcome of children and adolescents with bladder exstrophy and epispadias. Nonorganic enuresis may be associated with adverse mental or psychosocial consequences.8 However, to our knowledge there are no long-term outcome studies of psychosocial functioning in children with other disorders that include organic urinary incontinence. Other studies of children with genital malformations indicate that these anomalies may be related to mental problems and psychosocial dysfunction. Compared to controls children with hypospadias are reported to experience adequate sexual adjustment but they also have more anxiety, depression and negative genital appraisal.9-11 A study of patients with micropenis demonstrated normal gender identity and good sexual function.lZ We examined Accepted for publication October 23, 1997
somatic outcome, mental health and psychosocial adjustment in adolescents with bladder exstrophy and epispadias, and assessed parental experiences. We also explored possible associations among somatic, mental and psychosocial outcome variables. SUBJECTS AND METHODS
Patients. Our primary sample included 29 patients born from 1974 to 1984 with bladder exstrophy and epispadias who were treated at our hospital. According to the Norwegian Medical Birth Registry, this group represents about 91% of all bladder exstrophy and epispadias patients born in Norway in this period. Of the original 29 patients 2 with severe associated anomalies (anorectal anomalies and multiple handicaps) were excluded from the study and another had emigrated. Of the remaining 26 patients 17 boys and 5 girls (85%), median age 14.5 years (range 11 to 201,consented to participate. The parents of 21 patients were interviewed and completed questionnaires. Data about previous medical treatment were collected from the medical records. There were 15 boys and 4 girls with bladder exstrophy and 2 boys and 1 girl had only epispadias. Bladder closure was performed in 14 of 19 patients (74%) with exstrophy at a median of 60 weeks post partum (range 2 to 156).No patient had pelvic osteotomy associated with bladder closure. Bladder disruptions developed in 4 patients (29%). Bladder neck reconstruction (V-Y plasty or Young-Dees technique) was performed in 4 patients (1 with bladder exstrophy, 3 with
1684
MENTAL AND PSYCHOSOCIAL OUTCOME IN ADOLESC:ENTS WITH BLADDER EXSTROPHY AND EPISPADIAS
epispadias) at a median age of 6.5 years (range 4 to 7). None of these patients achieved urinary continence. Of the 13 patients (59%) who underwent urinary diversion 8 had undergone previous bladder closure, 12 conduit urinary diversion (11with sigmoid colon, 1with ileum) at a median age of 3 years (range 1to 6.5), 1continent urinary diversion at age 19 years and 1placement of an artificial sphincter at age 18 years. Surgery for genital reconstruction was performed a median of 2 times (range 1 to 5) in all 22 patients, with the iirst procedure at a median age of 3 years (range 2 weeks to 6.6 years). Inguinal herniotomy was performed at a median age of 30 days (range 1 to 270) in 7 boys and 2 girls (47%)with exstrophy. None had rectal prolapse and all had an anteriorly placed anus. Control groups. The control group for the assessment of anorectal urogenital function included 7 hospitalized boys and 7 girls, mean age 16 years (range 10 to 20), with no urogenital or anorectal dysfunction. The control group for the psychiatric and psychosocial assessments was a subsample of 17 boys and 16 girls, mean age 15 years (range 14 to 17), selected randomly from a Norwegian epidemiological study of mental health.13.14 In this control group 6% of the adolescents had chronic illnesses of eczema and epilepsy. The clinical data or data in the medical records did not suggest any difference in cognitive functioning between the patients with bladder exstrophy and epispadias and controls. Baseline demographic characteristics of the sample, hospital admissions, operations and followup are presented in table 1. We found no significant differences between the patients and controls in gender, age or sociodemographic characteristics. At followup 13 patients (59%) had urinary diversion (conduit in 12 and continent in 1).The 9 patients without urinary diversion required diapers. The patient who was emptying the bladder by clean intermittent catheterization still required diapers. Somatic assessment. Urogenital function was assessed by obtaining a history of urinary continence status and clinical examinations. Urinary Continence was graded according to the Hollowell and Ransley clas~ification.~~ The classification was applied to all patients, regardless of whether they had urinary diversion, using maximum dry intervals of excellent-no urine loss during the day and night, and dry inter-
1685
vals for more than 4 hours (group l),good-some nighttime wetting and dry intervals for more than 3 hours (group 2). poor-dry intervals for not more than 2 hours and protection required (group 3) and wet-continuous leaking (group 4). For simplicity in reporting results the terms satisfactory (groups 1 and 2) and unsatisfactory (groups 3 and 4) are used.15 Local status of the urinary stoma was assessed with regard to stomal site size and shape, and peristomal skin.16 The degree of urine leakage from the collecting system, the urostomy appliances and stoma care routines were also evaluated. Genital malformations were classified as severe or mild. Severe malformation was defined as a short, broad, upturned penis (tight dorsal chordee) with urethral orifice on the penile shaft or at the penopubicjunction, or bifid clitoris with a urethra split to a variable extent.17 Satisfaction with genital appraisal and function was assessed on a 10 cm. visual analog scale, ranging from 0 (bery satisfied with the genital appearance and function”)to 10 (bery dissatisfied”). The adolescents were interviewed regarding experiences with erection, ejaculation, masturbation, intercourse, and thoughts of establishing a close sexual relationship, family and having children in the future. Renal function was assessed by glomerular filtration rate measured by 99”technetriumpentetic acid. Glomerular filtration rate above 70 was considered normal.18 Anorectal function was assessed by clinical and physiological examination with anal canal manometry.19 Fecal continence was graded according to the Wingspread classification, rating 4 clinical states of continence as 1-clean, 2--staining, S i n termittent fecal soiling and 4-constant soiling.20 Flatus continence function was assessed by asking patients to indicate the control of flatus on a 10 cm. visual analog scale.21 Psychiatric and psychosocial assessments. The psychiatric assessment of the adolescents was based on a semistructured interview, the Child Assessment Schedule (CAS),22,23which provided a total problem score of symptom complexes analogous to psychiatric diagnoses of the American Psychiatric Association DSM-III-R.24 Standardized questionnaires, Youth Self-Report25 and Child Behavior Checklist26 provided a combined total behavior and emotional score with internalizing and externalizing subscales. Overall psychosocial functioning of the adolescents was assessed by the Children’s Global Assessment Scale (CGAS),27which was based on information from patient interview. The scores ranged from 100 (excellent function) to 0 (severe malfunction). PsychosoTABLE1. Demographic chamcteristics of adolescents with bladder cial problems of clinical significance were scores below 71. ezstrophv and eDispadias. and a population group The interviews were supplemented with questions concernPopulation Bladder Exstrophy and ing adolescent view of the present situation regarding the Epispadias (22pts.) (33 pts.) anomalies. 14.5 (11-20) 15 (14-17) Median age, (range) Parents were interviewed separately using a slightly mod17 (52) 17 (77) NO.boys (%) ified version of the standardized semistructured interview, NO.family composition (90): Parental Account of Children’s which included 22 (67) 17 (77) Both biological parents 11 (33) 5 (23) Single biological parent ratings of maternal warmth and criticism towards the index Median yrs. parental education child. A global assessment of chronic family difficulties29.30 (range): was based on all parental information available about the 14 (9-17) 12.5 (9-19) Mother previous and present family situation. Parent interviews 14 (9-17) 12 wig) Father were supplemented with questions regarding emotional and NO.community (%): 1s (55) 10 (45) Urban practical aspects of treatment procedures and medical fol15 (45) 12 (55) Rural lowup. Median hospital admissions Adolescent and parent interviews were performed by a (range): 0 (0-2) 15 (6-23) Total hospital admissions child psychiatrist with no knowledge of the surgical condition 9 (2-15) 0 (0-2) Hospital admissions before and were videotaped for testing of inter-rater reliability. This age 5 yrs. assessment of inter-rater reliability was based on 10 ran7 (1-14) 26 (14-114) Longest hospital stay (days) domly selected videotaped interviews independently scored 4 (1-13) 11 (7-18) Age at last hospital admisby another child psychiatrist. The intraclass correlation was sion 1 (0-1) 4 (3-10) No. operations 0.93 for the CAS, 0.84 for the CGAS and 0.91 for the chronic NO.urinary continence treatfamily difficulties scores. The kappa correlation coefficients ment (%): were 0.74 for parental warmth and 0.55 for parental c d i 0 12 (55) Conduit diversion 0 Continent diversion 1 (5) cism. 0 9 (41) Diauers Statistics. Due to abnormal distributions results are re-
1686 MENTAL AND PSYCHOSOCIAL OUTCOME IN ADOLESCENTS WITH BLADDER EXSTROPHY AND EPISPADIAS ported as medians (ranges) unless otherwise stated. Nonparametric statistics primarily were used.31 For assessment of differences between independent groups a chi-square test with Yaks correction or Fisher's exact test for small samples was used for categorical variables and the Mann-Whitney U-test was used for continuous variables. Dependency between sets of variables is expressed through the Spearman's rank correlation coefficient. Multiple regression analyses with a stepwise (forward) procedure were conducted to determine independent somatic and family/parental variables contributing significantly to the explained variance of adolescent mental health (CAS) and psychosocial function (CGAS).32 Careful checks of model assumptions, including investigations of residual plots, did not reveal any violation of the assumptions and 2-tailed p <0.05 was considered statistically significant. Since a considerable number of tests were performed, the most emphasis is on results with low p values. All calculations were performed using statistics software. Informed written consent was obtained from the adolescents and the parents. The study was approved by the Regional Ethics Committee for Medical Research. RESULTS
Somatic condition. Urinary, fecal and flatus continence, anal canal pressures and genital status in patients and controls are given in table 2. Of the 13 patients with urinary diversion 12 had satisfactory urinary continence (groups 1 and 2). One patient with conduit urinary diversion had fiequent urine leakage from the collecting system day and night. However, 2 patients had weekly to daily urine leakage and 2 had monthly leakage. These 5 patients reported that the urine leakage interfered with social activities. The stomal complications were not due to an obviously inadequate stoma placement, improper size or shape, or peristomal skin problems but to poor stomal care routines, improper urostomy appliances and poor stomal care followup. All 9 patients (6 with exstrophy, 3 with epispadias) without diversion were urinary incontinent (groups 3 and 4). They used a median of 4 diapers (range 1 to 10)day and night. There were 5 patients who reported that using diapers influenced the social life because of unpleasant odor, bullying or always having to bring a change of diapers. Of the 19 adolescents with bladder
exstrophy 6 (32%)had fecal staining at followup and 4 had fecal staining until late childhood. None of the patients with epispadias had fecal continence problems. With regard to fecal continence function, no significant differences in age or sex were found. Glomerular filtration rate was a median of 83 (range 42 to 108), and 5 patients (28%) had a glomerular filtration rate below the cutoff (median 57, range 42 to 68). In 5 patients with exstrophy and 1with epispadias, 3 girls and 3 boys, the genital malformations were assessed by the surgeon as mild. On the visual analog scale 10 boys (59%) indicated dissatisfaction with the genital appearance and function (median 5.0,range 4 to 9), whereas all 5 girls were satisfied (median 0, range 0 to 2). Four boys and 1 girl (23%) stated that the genital malformations had influenced the thought of establishing a close sexual relationship, 5 boys and 2 girls (32%) reported worrying about the future ability to establish a family and have children, 16 patients (73%)reported dating, 16 (94%)had erection and 10 (59%)had ejaculation. Ten boys and 2 girls (55%),median age 17.5 years (range 14 to 20), regularly masturbated. Four adolescents had had sexual intercourse, and the median age of initial intercourse was 17 years (range 16 to 17). Mental health and psychosocial function. Of the 22 adolescents 11 (50%) met the criteria for a psychiatric diagnosis according to the DSM-111-R criteria (table 3). Two adolescents received 2 diagnoses. There were significant differences in the prevalence of psychiatric diagnoses between patients and controls. Most of the psychopathology concerned emotional and internalizing problems, such as overanxiousness, sadness, depression, low self-esteem, poor body concept and withdrawal. The adolescents with obsessive-compulsive disorders had recurrent and persistent thoughts or images of the multiple operations and hospital stays, wearing diapers because of urinary incontinence, genital malformations, body or sexuality. One also had compulsions of "always having to run after women" but "when it comes to intercourse I run away." Much energy was used in hiding the anomaly and symptoms. These thoughts interfered with social activities or relationships with others, and they became isolated and withdrawn. The bladder exstrophy and epispadias group had a median score of 82.5 on the CGAS which was not significantly different from the population group score of 85. The 11 adolescents
TABLE2. Urinary, fecal and flatus continence function, anal canal pressures and genital malformations of adolescents with bladder exstrophy and epispadias and controls Bladder Exstrophy and Epispadias (22 Db.)
TABLE 3. Mental health and psychosocial functioning in adolescents with bladder exstrophy and epispadias, and population
Controls (14 pts.)
POUP
No. urinary continence function (%):*
Satisfactory, groups 1 + 2 Unsatisfactory, groups 3 + 4 No. fecal continence function
12 (55) 10 (45)
13 (93) l ( 7 , p <0.05)
(%):t 14 (100) Continent, grade 1 16 (73) 0 (0, p <0.05) Incontinent, grades 2-4 6 (27) No. flatus continence function i%):$ Continent, score 0-3 12 (86) 19 (86) Incontinent, score 4-10 3 (14) 2 (14) Median an.water anal canal pressures (range): 98 (80-105, p (0.05) Resting 70 (40-100) Squeezing 200 (90-300,p 10.05) 110 (50-200) No. genital malformations (96): 0 14 (100, p <0.05) None Mild 6 (27) 0 0 16 (73) Severe * Graded according to Hollowell and Ransley classification. f Graded according to the Wingspread classification of clean-1, stain ing-2, intermittent fecal soiline-3 - and constant fecal soiline-4. t Indicated on a 10 cm. visual analog scale.
No. DSM-111-R mental health diagnosis:24 Dysthymic disorder Simple phobia Overanxious disorder Conduct disorder Obsessive-compulsive disorder Total No. (55) Median total problem CAS scores Median Child Behavior Checklist raw scores (range)? Total behavior Internalizing Externalizing Median Youth Self-Report raw scores Total behavior Internalizing Externalizing Median Psychosocial functioning CGAS scores (ranee):"' -
Bladder Exstrophy
Population
and(22 Epispadias Db.)
(33 pts.)
2 1 3 1 4 11 (50) 21 ( 5 4 8 )
4 (12, p <0.01) 15 ( 3 4 4 )
9 2 2
(1-75) (0-14) (0-35)
29 (12-128) 7.5 (1-46) 11 (3-37) 82.5 (52-98)
7 (0-76) 4 (0-19) 2 (0-39) 22 (0-86) 5 (0-30) 7 (0-45) 85 (55-98)
MENTAL AND PSYCHOSOCIAL OUTCOME IN ADOLESCENTS WITH BLADDER EXSTROPHY AND EPISPADIAS 1687
without a psychiatric diagnosis had a median CGAS score of 92 (range 81 to 981,indicating optimal psychosocial functioning. Of the 11 adolescents who met the DSM-111-R criteria for a psychiatric diagnosis 8 had CGAS scores less than 71, implying clinically significant psychosocial problems. Regarding mental health and psychosocial functioning, we found no significant difference in sex or age between the urinary continent and incontinent adolescents or between those who had urostomies and those who required diapers. However, patients with severe genital malformations had a significantly higher prevalence of psychiatric diagnoses (chisquare = 6.77,df 1,p = 0.009),higher CAS scores (z = -2.09, p = 0.045)and lower CGAS scores (z = -2.18, p = 0.029) than patients with mild genital malformations. Furthermore, we found significantly more mental health problems (CAS) in the patients with flatus incontinence (visual analog scale 4 to 10) than in patients who were continent (z = -2.11, p = 0.035). The answers about present physical and/or psychosocial problems are presented in table 4.Only 1 adolescent reported no problems at the time of the assessments. There were 15 adolescents (68%) who reported teasing or bullying because of the incontinence, diapers, stomas, genital malformations or scars. However, 16 adolescents (72%) reported that the physical and emotional problems were not difficult to talk about to parents or close friends. The advice that they would give to parents of children with bladder exstrophy and epispadias included openness, a provision of information and a normal upbringing with a supportive attitude, especially regarding self-esteem and autonomy. Family and parental difficulties. The economy, housing, education, and physical and mental health of the family members of the bladder exstrophy and epispadias group were generally good. No or only minor chronic family difficulties were reported (median 2.0, range 0 to 5). Chronic family difficulties were primarily due to somatic and mental health problems in the parents or the siblings and/or minor family conflicts. No significant differences were found between the bladder exstrophy and epispadias, and population groups with regard to parental warmth or criticism toward the adolescents. Of the parents 57% described the first year as practically and emotionally difficult, 67% expressed worries about the future sexual orientation of the adolescent, possibilities for sexual relationships, marriage and parenthood or the impact of the malformation on self-esteem and confidence, and 19% mentioned worries about how the adolescents managed the present somatic and psychosocial situation as the most difficult. For 43% of the parents the problems of the child had influenced positively married and family life, for example, they had become emotionally closer as partners, united by the experience of having a disabled child, and 90% experienced support from family and friends. The anomaly and physical problems had never been difficult to discuss. TABLE4. Present physical and psychosocial problems reported in 22 adolescents with bladder exstrophy and epispadias
-
Total No. (No. boysf No. girls)
Womes about genital malformation 15 (132) Womes about urinary incontinence 9 (7/2) Unpleasant smelVdirty trousers 8 (711) Uncertainty with physical closeness 8 (711) and shyness in tinding a partner* Problems with self-esteemmodyimage I (710) Walking pain in hipsfpelvis 6 (U2) Womes about fecayflatus incontinence 6 (511) Stoma1 problems 5 (5/0) Abdominal pain, urinary tract infec5 (5/0) tions or stones Womes about scars 2 (ZO) No problems 1 (YO) * This question was posed to 11 patients older than 14 years.
Associations among somatic variables, parental I family variables, and mental and psychosocial outcome. The patients were classified into the 2 age groups of ll to 14 years (11)and 15 to 20 years (11).33 In the younger group more mental problems (CAS symptom score) were correlated with less parental warmth (r, = 0.68,p = 0.021)and more chronic family difficulties (r, = 0.63,p = 0.038).Poorer psychosocial function (CGAS) was correlated with fecal incontinence (r, = -0.75,p = 0.008),flatus incontinence (r, = -0.70, p = 0.016) and less parental warmth (r, = -0.66,p = 0.026).In the older group more mental problems (CAS symptom score) were correlated with urine incontinence (r, = 0.63, p = 0.0391,less parental warmth (r, = 0.67,p = 0.036)and more parental criticism (r, = 0.76,p = 0.010).Poorer psychosocial function was correlated with less parental warmth (r, = -0.73,p = 0.017)and more parental criticism (r, = -0.77, p = 0.009). To investigate further the contribution of the independent somatic and family/parental variables in predicting psychosocial (CGAS) and mental (CAS) outcome, stepwise multiple regression analyses were used. With CGAS (psychosocial function) as the dependent variable, the somatic correlates (urinary, fecal and flatus continence, and genital appraisal), gender and adolescent belief in the possibility of a close sexual relationship, and the familylparental correlates (chronic family difficulties, parental warmth and criticism) were entered as explanatory variables. The only predictor of CGAS in the younger group (11to 14 years) was fecal continence function, explaining 53% of the variance ( p = -13.7, p = 0.01).In the older group belief in the possibility of a close sexual relationship was the only predictor of CGAS,explaining 69% of the variance ( p = 24.8,p = 0.002). With CAS symptom score (mental health) as the dependent variable, somatic correlates, gender, and family and parental correlates were entered as explanatory variables. In the younger group parental warmth was the strongest predictor of CAS, explaining 64% of the variance ( p = 6.7,p = 0.0018). Adolescent satisfaction with genital appearance and function added another 18% ( p = 1.04,p = 0.011).In the older group parental warmth was the strongest predictor of CAS, explaining 68% of the variance Cp = 6.3,p = 0.002).Urinary continence added another 13% ( p = 0.8,p = 0.03). DISCUSSION
The discovery of significant persistent problems in the adolescents with bladder exstrophy and epispadias contrasts with reports of satisfactory long-term urinary control in patients with bladder exstrophy and epispadias with or without urinary diversion.4.5.15,34-37 However, the findings are consistent with multicenter followup reports of unsatisfactory urinary control in 75 to 86% of children with bladder exstrophy and epispadias without primary urinary diversion,3ss39 and with recent reports that men with bladder exstrophy and epispadias were bothered by penile appearance and function.4.5 The diverse findings are probably due to the development of surgical techniques, such as the recent aggressive functional reconstruction with primary pelvic closure, which seems to achieve better urinary continence. The inconsistency may also be due to methodological problems, such as the definition of urinary continence and sexual function or the inclusion of different age groups. Children, adolescents and adults have different developmental tasks regarding the anomalies. However, in our study the variety in somatic outcome made it possible to assess the effect of somatic risk factors on mental and psychosocial dysfunction, regardless of operative treatment procedures. The retrospective design of the study and the small number of patients may limit the generalizability and comparability of varying treatment procedure results, and we ad& . the caution in interpreting the statistical a n d p e ~However,
1688 MENTAL AND PSYCHOSOCIAL OUTCOME IN ADOLESCENTS WITH BLADDER EXSTROPHY AND EPISPADIAS present sample is representative of the Norwegian patient which indicates good global psychosocial functioning and is population from 13 to 23 years ago. All but 3 patients born in accordance with recent outcome studies of adults with However, -~ comparisons during this time were included in the study. Adolescents with bladder exstrophy and e p i ~ p a d i a s . ~ bladder exstrophy and epispadias had a 4-fold increase in with these studies are difficult due to differences in age, psychiatric disorders compared to the control group and gen- patient sampling and methods. The finding that fecal inconeral pediatric population.4o.41 This overrepresentation ex- tinence predicted psychosocial dysfunction in the younger ceeds the generally doubled risk of maladjustment in chil- bladder exstrophy and epispadias group coincides with finddren with chronic illnesses compared to healthy children42 ings in patients with fecal incontinence due to anorectal and coincides with the results of studies of Norwegian ado- anomalies and Hirschsprung‘s disease.21.48.49 Fecal staining is shameful and socially unacceptable regardless of the dislescents with anorectal anomalies.21 The high risk of psychiatric disorders among the adoles- order. However, it seems that although adolescents with cents with bladder exstrophy and epispadias is probably not bladder exstrophy and epispadias may have a high frequency only due to having a chronic physical illness but more spe- of specific emotional problems, these do not necessarily incifically related to characteristics of the anomaly, such as fluence the present social functioning within the family, at urinary incontinence and severity of genital malformation. In school or among peers to the same degree as for children with the older adolescents mental health problems were corre- anoredal anomalies.21 In contrast with fecal and flatus inlated with poor urinary continence function. In the younger continence, urinary incontinence does not involve such a foul patients dissatisfaction with genital appearance and function smell and does not disturb others. Fecal and urinary inconwas a predictor of mental health problems. Such distress was tinence may also impact families differently. Our findings not adequately expressed in the consultation with the pedi- emphasize the necessity of considering the nature and stage atric surgeon or urologist. For adolescents body image, self- of the chronic illness, symptom severity and visibility,50 and esteem, sexuality, sexual function and fertility are funda- parental and family related factors when assessing the immentally important. The adolescents in our study had more pact of chronic disabilities on mental health and psychosocial problems with body image or self-esteem, worries about the functioning. genital malformation, present or future sexual function, and the possibility of having children than had been reported in CONCLUSIONS previous studies.4-7.43-45 More negative genital appraisal Adolescents with bladder exstrophy and epispadias who was noted in our boys (59%)than in boys with hypospadias (25%) and comparison subjects (5%) without any genital underwent operations 13 to 23 years ago have significant persistent physical and mental problems. However, the suranomaly reported by Mureau et al.11 Emotional and body image development may be affected gical treatment of these patients was not what would be profoundly by genital deformity and reconstructive sur- considered standard therapy today and the results probably gery.46.47 Given the importance of early feedback and social do not reflect the status of exstrophy management today. The interactions with parents and later with peers, the earlier adaptation of all patients with bladder exstrophy and episthe genital repair, the more likely the child will achieve a padias must be considered in the context of the specific chalpsychologically healthy perspective about body image. Nor- lenges posed by the urogenital malformation, as well as in mal psychosocial and psychosexual development is more the context of the current developmental phase of the paprobable if it is anticipated and prepared for in childhood.45 tient. Mental health was determined by genital appearance, The importance of parental confidence, expectations and re- urinary continence and parental warmth. Thus, optimal sponses is confirmed by our finding that high parental treatment and followup of patients with bladder exstrophy warmth was the most important predictor of mental health and epispadias require collaboration among the pediatric and is consistent with the report of Reilly and Woodhouse.12 surgeon, adult urologist, stoma1 therapist and psychosocial They emphasize that the strongest influence for all patients expert from birth to adulthood. Professional mental health with micropenis was the “parental attitude; well informed, guidance with a developmental and family perspective open and supportive parents who explained the problems and should be offered routinely to the patient with bladder exencouraged normality produced confident and well adjusted strophy and epispadias and the parents. children.” Dr. Inger Helene Vandvik provided scientific advice, Dr. Different developmental tasks may create different levels Thore Egeland statistical support and Sonja Rosvold practiof vulnerability in childhood and adolescence, highlighting cal help. Dr. Astrid Aasland scored the videos used in assessthe need for individual psychosocial support at all stages of ing reliability. care. In early adolescence children compare themselves with peers of the same sex and are anxious about being different REFERENCES from them, as exemplified by the finding that the boys with 1. Dewan, P. A: Historical trends in the management of bladder bladder exstrophy and epispadias often stopped showering in exstrophy. Ped. Surg. Int., 10 289, 1995. public from age 12 to 13 years. Dissatisfaction with genital 2. Lattimer, J. K, Hensle, T. W., MacFarlane, M. T., Beck, L., appearance and function was the only somatic predictor of Braun, E. and Esposito, Y.: The exstrophy support team: a new mental health problems in the youngest bladder exstrophy concept in the care of the exstrophy patient. J.Urol., 121: 472, and epispadias group. In late adolescence social and sexual 1979. relationships with the opposite sex are increasingly impor3. Vandvik, I. H. and Storhaug, K.: Family-focused services for tant. For example, wearing diapers because of urine incontichildren with rare disorders, exemplified by bladder exstronence when a boy is planning to date is the type of challenge phy. Clin. Ped., 24: 97, 1985. that may lead to withdrawal and isolation. Urinary conti4. Feitz, W. F. J., Van Grunsven, E. J. K. J. E. M., Froeling, F. M. J. A. and De Vries, J. D. M.: Outcome analysis of the nence function was the only somatic predictor of mental psychosexual and socioeconomical development of adult pahealth in the older bladder exstrophy and epispadias group. tients born with bladder exstrophy. J. Urol., 152 1417, 1994. The need for specific individual patient psychological coun5. Ben-Chaim, J., Jeffs, R. D., Reiner, W. G. and Gearhart, J. P.: seling, including opportunities to discuss feelings about the The outcome of patients with classic bladder exstrophy in anomaly symptoms, self-esteem, body image, sexual matters adult life. J. Urol., 155 1251,1996. and womes about the future should be assessed during fol6. Avolio, L., Koo, H. P., Bescript, A. C., Snyder, H. M., Canning, lowup. D. A. and Duckett, J. W., Jr.: The long-term outcome in men In contrast to the high frequency of specific psychiatric with exstrophy/epispadias: sexual function and social integraproblems, we found an almost normal median CGAS score, tion. J. Urol., 156 822, 1996.
MENTAL AND PSYCHOSOCIAL OUTCOME IN ADOLESCENTS WITH BLADDER EXSTROPHY AND EPISPADIAS
1689
7. Stein, R., Hohenfellner, K., Fisch, M., Stockle, M., Beetz, R. and 130 1142,1983. Hohenfellner, R.: Social integration, sexual behavior and fer- 35. Mesrobian, H. G. J., Kelalis, P. P. and Kramer, S. A,: Long-term tility in patients with bladder exstrophy - a long-term follow followup of 103 patients with bladder exstrophy. J. Urol., 139: up. Eur. J. Ped., 155 678,1996. 719, 1988. 8. Shaffer, D.:Enuresis. In: Child and Adolescent Psychiatry. Ed- 36. Connor, J. P., Hensle, T. W., Lattimer, J. K. and Burbige, K. A,: ited by M. Rutter, E. Taylor and L. Hersov. Oxford Blackwell Long-term followup of 207 patients with bladder exstrophy: an Scientific Pub., chapt. 29,pp. 505-519, 1994. evolution in treatment. J. Urol., 142 793, 1989. 9. Robertson, M. and Walker, D.: Psychological factors in hypospa- 37. Gearhart, J. P. and Jeffs, R. D.: Bladder exstrophy: increase in dias repair. J. Urol., 113 698,1975. capacity following epispadias repair. J. Urol., 142:525,542, 10. Berg, R., Berg, G. and Svensson, J.: Penile malformation and 1989. mental health. A controlled psychiatric study of men operated 38. de la Hunt, M. N. and O'Donnell, B.: Current management of for hypospadias in childhood. Acta Psychiat. Scand., 86: 398, bladder exstrophy: a BAPS collectivereview from eight centres 1982. of 81 patients born between 1975 and 1985.J. Ped. Surg., 24: 11. Mureau, M.A. M., Slijper, F. M. E., Nijman, R. J. M., Van Der 584, 1989. Meulen, J. C., Verhulst, F. C. and Koos Slob, A.: Psychosexual 39. Kockum, C. C., Hansson, E., Stenberg, A., Svensson, J. and adjustment of children and adolescents after different types of Malmfors, G.: Bladder exstrophy in Sweden - a long-term hypospadias surgery: a norm-related study. J. Urol., 154: follow-up study. Eur. J. Ped. Surg., 6: 208, 1996. 1902, 1995. 40. Rutter. M.:Isle of Wieht revisited: twentv-five vears of child 12. Reilly, J. M. and Woodhouse, C. R. J.: Small penis and the male psychiatric epidemioiogy. J. Amer. Acad. khild Adol. Psychiasexual role. J. Urol., 142 569,1989. try, 2 8 633,1989. 13. Gjone, H. and Nevik, T. S.: Parental ratings of behaviour prob- 41. Brandenburg, N. A., Friedman, R. M. and Silver, S. E.: The lems: a twin and general population comparison. J. Child epidemiology of childhood psychiatric disorders: prevalence Psychol. Psychiatry, 36 1213,1995. findings from recent studies. J. Amer. Acad. Child Adol. Psy14. Nevik, T. S.:Are postal surveys in child psychiatry feasible? chiatry, 2 9 76, 1990. Results of an epidemiological study. Nord. J. Psychiatry, 4 9 42. Pless, I. B. and Nolan, T.: Revision, replication and neglect337, 1995. research on maladjustment in chronic illness. J. Child Psychol. 15. Hollowell, J. G. and Ransley, P. G.: Surgical management of Psychiatry, 3 2 347,1991. incontinence in bladder exstrophy. Brit. J. Urol., 68: 543,1991. 43. Woodhouse, C. R. J., Ransley, P. G. and Williams, D. I.: The 16. Nordstrem, G. M., Borglund, E. and Nyman, C. R.: Local status patient with exstrophy in adult life. Brit. J. Urol., 5 5 632, of the urinary stoma - the relation to peristomal skin compli1983. cations. Scand. J. Urol. Nephrol., 2 4 117,1990. 44. Mesrobian, H. G. J., Kelalis, P. P. and Kramer, S. A.: Long-term 17. Rickwood, A. M. K.: Exstrophic anomalies. In: Neonatal Surgery, followup of cosmetic appearance and genital function in boys 3rd ed. Edited by J. Lister and I. M. Irving. Butterworth & Co., with exstrophy: review of 53 patients. J. Urol., 136 256,1986. chapt. 51,pp. 709-717, 1990. 45. Woodhouse, C.R. J.: The sexual and reproductive consequences 18. Rootwelt, K.: Nukleaxmedisin. Oslo: Universitetsforlaget, pp. of congenital genitourinary anomalies.J. Urol., 152 645,1994. 86-87, 1995. 46. Schultz, J. R., Klykylo, W. M. and Wacksman, J.: Timing of 19. Emblem, R., Diseth, T. H., Merkrid, L., Stien, R. and Bjordal, R.: elective hypospadias repair in children. Pediatrics, 71: 342, Anal endosonography and physiology in adolescents with cor1983. rected low anorectal anomalies. J. Ped. Surg., 29 447,1994. 47. American Academy of Pediatrics: Timing of elective surgery on 20. Stephens, F. D.and Smith, E. D.: Classification, identification, the genitalia of male children with particular reference to the risks, benefits, and psychological effects of surgery and anesand assessment of surgical treatment of anorectal anomalies. Ped. Surg. Int., 1: 200,1986. thesia. Pediatrics, 97.590, 1996. 21. Diseth, T.H. and Emblem, R.: Somatic function, mental health 48. Diseth, T. H., Emblem, R. and Vandvik, I. H.: Adolescents with anorectal malformations and their families. Fam. Systems and psychosocial adjustment of adolescents with anorectal anomalies. J. Ped. Surg., 31: 638,1996. Med., 1 3 215, 1995. 22. Hodges, K.,Kline, J., Stem, L., Cytryn,L. and McKnew, D.: The 49. Diseth, T. H., Bjemland, K, Novik, T. and Emblem, R.: Bowel function, mental health and psychosocial function in adolesdevelopment of a child assessment interview for research and cents with Hirschsprung's disease. Arch. Dis. Child., 76 100, clinical use. J. Abnorm. Child Psychol., 10 173,1982. 23. Hodges, K.: Manual for the Child Assessment Schedule (CAS). 1997. 50. Garralda, M. E.: Chronic physical illness and emotional disorder Columbia: University of Missouri, 1985.Unpublished. in childhood:where the brain's not involved, there may still be 24. American Psychiatric Association: Diagnostic and Statistical problems. Brit. J. Psychiatry, 1W. 8, 1994. Manual of Mental Disorders, 3rd ed. Washington, D.C.: American Psychiatric Association, 1987. 25. Achenbach, T. M. and Edelbrock, C. S.: Manual for the Youth EDITORIAL COMMENT Self Report and Profile. Burlington, Vermont: University of The authors provide a detailed description of the psychosocial Vermont, Department of Psychiatry, 1987. 26. Achenbach, T. M.: Manual for the Child Behavior Checklist and functioning of adolescents with bladder exstrophy. However, the 1991 Profile. Burlington, Vermont University of Vermont, population in the study may not be representative of bladder exstrophy patients treated a t centers with specific expertise in this disorDepartment of Psychiatry, 1991. 27. Shaffer, D., Gould, U. S., Brasic, J., Ambrosini, P., Fisher, p., der. For example, although the authors state that surgical treatment Bird, H. and Aluwahlia, S.: Children's Global Assessment includes restoration of the pelvic ring, none of the patients studied had the benefit of pelvic osteotomy and almost none underwent early Scale (CGAS).Psychopharm. Bull., 21: 747, 1985. 28. Taylor, E.: The overactive child. In: Clinics in Developmental closure. The lack of pelvic closure contributes to poor cosmetic outcome, and fecal, flatus and urinary incontinence. Patients in this Medicine, No. 97.Oxford: Blackwell Scientific Pub., 1986. 29. Vandvik, I. H., Hgyeraal, H. M. and Fagertun, H.: Chronic fam- series were considered to have had urinary continence if they had a ily difficulties and stressful life events in recent onset juvenile successful incontinent urinary diversion. It is misleading to describe the psychosocial adaptation of patients with urinary continence arthritis. J. Rheumatol., 16 1088, 1989. 30. Bjomstad, P., Lindberg, H. and Spurkland, I.: Unge hjerter i when they all wear an appliance. This group cannot be considered to be the same as adolescents who have functional reconstruction and faresonen. (Young hearts a t risk). Oslo: Tan0 A. S., 1990. 31. Altman. D. G.:Practical Statistics for Medical Research. London: true urinary continence. Cosmetic issues are important to the adolescent. These issues go Chapman and Hall, 1991. 32. Kleinbaum, D., Kupper, L. and Muller, K: Applied Regression beyond the genital appearance. The majority of children in this Analysis and Other Multivariable Methods, 2nd ed. Boston: group had undergone urinary diversion, presumably resulting in abdominal scars and stomal appliances. While genital appearance PWS-KENT Publishing Co., 1988. 33. Mangs and Martell: 0-20 C enligt psykoanalytisk teori. Stock- only affects intimate encounters, the other surgical deformities are obvious in many social situations. No information is provided as to holm: Natur och Kultur, 1974. 34. k p o r , H. and Jeffs, R. D.: Primary bladder closure and bladder the type of genital reconstruction performed or the adquaey of this neck reconstruction in classical bladder exstrophy. J. Urol., reconstruction. Therefore, the implications about the genital malfor-
1690 MENTAL AND PSYCHOSOCIAL OUTCOME IN ADOLESCENTS WITH BLADDER EXSTROPHY AND EPISPADIAS mation and its effect on the psychosocial functioning of the adolescent are difficult to put into perspective. Psychological evaluation and followup are vital to the well-being of the child with bladder exstrophy. The psychosocial impact of bladder exstrophy will likely be minimized through early functional reconstruction with the expectation of achieving urinary continence in the majority of children, satisfactory pelvic closure with the secondary benefits of improved fecal control and cosmesis, and minimizing the
outward signs of physical deformity, such as stomas, appliances and large surgical scars. Steve Docimo Department of Urology James Buchanan Brady Urological Institute Johns Hopkins Hospital Baltimore, Maryland