Relationships between Stigma, Social Support, and Distress in Caregivers of Chinese Children with Imperforate Anus: A Multicenter Cross-Sectional Study.

PURPOSE: This study was conducted to explore the relationships between stigma, social support, and distress in caregivers of children with imperforate anus (IA) and determine whether social support mediates the relationship between stigma and distress. DESIGN AND METHODS: This cross-sectional study was conducted in three tertiary children's hospitals in Eastern China. Primary caregivers completed the Social Support Scale and the Chinese versions of the Parent Stigma Scale and Kessler Psychological Distress Scale, and provided their demographic information. The children's demographic and clinical data were also collected. The hypothesized relations were explored using structural equation modeling via the bootstrap method. RESULTS: A total of 229 caregivers were enrolled. Distress was positively associated with stigma (r = 0.396, P < 0.01) and negatively associated with social support (r = -0.413, P < 0.01) in all dimensions (r = 0.314-0.346, P < 0.01). Stigma was also negatively correlated with social support (r = 0.280, P < 0.01). Furthermore, social support could partially mediate the relationship between stigma and distress (b = 0.135; 95% confidence interval: 0.072, 0.233). CONCLUSIONS: Stigma can increase caregivers' distress, while social support can reduce it. Stigma can also negatively influence caregivers' social support. Therefore, stigma should be mitigated to enhance caregivers' social support and decrease their distress. PRACTICE IMPLICATIONS: The study findings may aid in the identification of the psychological status of caregivers of children with IA, and also inform targeted intervention programs.

A child with imperforate anus for twelve years: a case report of neglect and exploitation.

We report a unique and rare case of child neglect and exploitation and similar case was not found. An infant, born with an imperforate anus, underwent surgery for a temporary colostomy on the day of birth. Surgery for reversal of colostomy with reconstruction was planned at one month of age. However, the guardian did not ensure that the child attended for this procedure. Subsequently, at the age of 12 years, the child was presented to the emergency unit with an irreducible prolapsed colostomy and a life threatening acute abdomen. Emergency laparotomy and colostomy reversal were performed with anal reconstruction. The child had features of gross neglect and abuse in the form of deprivation of essential surgical care, vaccination, and education. He was used for the purpose of begging for the financial benefit of his guardian and others. This is a case of neglect and exploitation of a child with imperforate anus and it emphasizes the failures of the health system to manage and follow-up children with congenital defects born to families with a poor socio-economic background.

Cloacal malformation patients report similar quality of life as female patients with less complex anorectal malformations.

PURPOSE: Cloacal malformations are the most complex type of anorectal malformation in females. This study aimed to report quality of life (QoL) of patients with a cloacal malformation for the first time in literature. MATERIALS AND METHODS: Female patients with an anorectal malformation participating in a follow-up program for congenital malformation survivors aged 5 or older were eligible for this study. QoL was assessed with the PedsQL™ 4.0 inventory. Scores of patients with a cloacal malformation (CM) were compared with those of female patients with rectoperineal or rectovestibular fistulas (RP/RV) and with reference data. RESULTS: A total of 59 patients (67% response rate; 13 patients with cloacal malformation) were included, QoL was assessed by patient self-report at median age of 12years (8-13), and by parent proxy-report at median age of 8years (5-12). There were no differences between groups regarding the presence of associated anomalies, with also no differences regarding anomalies in the urinary tract (CM vs. RP/RV=31% vs. 15%, p=0.237). Scores of the cloacal malformations group were similar to those of the comparison group, except the proxy-reported scores on school functioning (60.0 vs. 80.0, p=0.003). Proxy-reported scores of cloacal malformation patients were significantly lower than reference values on total QoL-score, psychosocial health, and emotional and school performance. Patients (irrespective of type of ARM) who suffered from fecal soiling reported significantly lower scores with regard to psychosocial health (71.7 vs. 81.7, p=0.034) and its subscale school performance (65.0 vs. 80.0, p<0.001). QoL-scores reported by cloacal malformation patients did not differ significantly from the reference values of the healthy population. Parents of cloacal malformation patients reported significantly lower total QoL, emotional and school performances, as well as a lower general psychosocial health for their children relative to reference data of healthy children. CONCLUSION: Patients with cloacal malformations and females with less complex anorectal malformations report similar QoL. Parents of cloacal malformation patients report more problems on several psychosocial domains relative to the healthy reference group. To monitor these matters, long-term follow-up protocols should contain multidisciplinary treatment including periodical assessment of psychosocial well-being.

Transition from childhood to adolescence: Quality of life changes 6 years later in patients born with anorectal malformations.

BACKGROUND/PURPOSE: Disease-specific quality of life (QoL) may be more or less relevant when children enter preadolescence/adolescence. Few attentions have been given to development and transition periods. Aim of the present longitudinal study is to evaluate ARM specific changes in QoL and the stability of QoL over 6 years. METHODS: Questionnaires were sent to families of the AIMAR Association (in 2007 and in 2013/2014). They included the Hirschsprung's Disease/ARM QoL Questionnaire (HAQL, [1]). Rank correlations and within group comparisons for the HAQL subscales were conducted analyzing the scores of time 1 vs time 2. Gender effects were tested. RESULTS: 134 parents answered the questionnaires at time 1 and 73 at time 2. Results of the repeated Analyses of Variance indicated improvements in the continence subscales ("Presence of Diarrhea," "Fecal Continence"). QoL significantly worsened in "Social and Emotional Functioning" and in "Body Image" areas. The analysis of stability of change indicates that QoL scores remain stable, with the exception of the Body Image area. CONCLUSIONS: Despite improvements in the continence areas, parents reported worse levels of QoL in the psychosocial areas, as their child grow. As patients grow, they might have more difficulties in daily and social activities, and feel more frequently ashamed and more dissatisfied with their body.

Children With Anorectal Malformations, Hirschsprung Disease, and Their Siblings: Proxy Reports and Self-Reports.

OBJECTIVES: The aim of the present study was to compare parent proxy reports with that of self-reports of children with anorectal malformations (ARMs) or Hirschsprung disease (HD) and healthy siblings and thereafter was examine whether these comparisons differed between patients and their siblings. METHODS: Parents (n = 98) of either children with ARM (n = 44) or HD (n = 54) and a healthy sibling (n = 98) recruited from the 6 Dutch pediatric surgical centers and from the ARM and HD patient societies were included in this cross-sectional multilevel study. Agreement between child self-reports and parent proxy reports was compared through mean differences and through (intraclass) correlations. We conducted multilevel analyses to take dependencies between assessments within families into account. RESULTS: All of the children (children with ARM or HD and their siblings) reported more pain and symptoms than their parents reported. We also found that only children with ARM or HD reported less positive emotions than their parents. Furthermore, higher correlations were found between parent proxy reports and patient-self reports than between parent proxy reports and sibling self-reports on cognitive functioning and social interaction. CONCLUSIONS: Parents tend to overestimate the physical functioning of both their ill and healthy children, and overestimate the emotional functioning of only their children with ARM or HD. Furthermore, children with ARM or HD and parents agree more on health-related quality of life domains than healthy children and parents.

Feelings of depression in people with ARM: the role of critical incidents and perceived difficulties in close and sexual relationships.

PURPOSE: Managing a chronic disease may be a difficult task which may lead patients to experience psychological distress and depression. Some studies showed that, in ARM patients, fecal incontinence (FI) is related to symptoms of depression while others studies did not. No studies investigated this relationship in adults. Since fear of having FI episodes, negative feelings associated with these episodes, and difficulties in close and sexual relationships are often reported by patients as important consequences of ARM, we were interested in investigating whether these aspects contribute in explaining feelings of depression. METHODS: Questionnaires were sent to 160 adult members of the Italian Association for Anorectal Malformation. A new scale measuring the fear of having FI episodes, negative feelings associated with these episodes and difficulties in close/sexual relationship was developed. Depressive feelings and FI were also measured. RESULTS: Seventy-two adults answered the questionnaires. Regression analyses showed that, in males, depressive feelings were predicted by difficulties in close and sexual relationships (B = 0.46; P < 0.01), while, in females, they were predicted by the fear of having FI episodes (B = 0.53; P < 0.05) and by negative feelings (B = 0.58; P < 0.01). CONCLUSIONS: Interventions aimed to prevent depression in ARM patients should consider gender and should be targeted on different aspects.

Adolescents with anorectal malformation: physical outcome, sexual health and quality of life.

BACKGROUND: The necessity of referring adolescents with anorectal malformation (ARM) from pediatric units to adult care is unclear. The issue requires knowledge about the health of the adolescent. OBJECTIVE: To examine the physical outcome, sexual health and quality of life (QoL) in adolescents with ARM. METHODS: At medical counseling, 24 adolescents with ARM, 15-21 years of age, answered questionnaires about physical outcome according to the Krickenbeck follow-up and QoL according to SF 36 and gastrointestinal quality of life (Giqli). Matched control groups were used; 15 adolescents participated in deep interviews about sexual health and body imaging. RESULTS: Fecal soiling, constipation and gas incontinence were much higher for ARM patients compared with controls (p<0.05). QoL regarding large bowel function was lower for both genders compared with controls (p<0.05). Females scored lower in physically related QoL (p<0.05). Social and sexual adaption to the symptoms was obvious in the deep interviews. CONCLUSION: Adolescents with ARM have considerable intestinal symptoms, which influence QoL and require adaption in intimate situations. A referral to adult care seems to be important, and continuous cooperation between the pediatric surgeon and adult care is suggested.

Exploring quality of life in Italian patients with rare disease: a computer-aided content analysis of illness stories.

The present study used a narrative-based approach to identify common themes that characterized the illness experience and quality of life of patients affected by rare disease (RD). Textual data were comprised of illness stories written by 32 adult Italian patients (eight men and 24 women), with the following RD diagnoses: amyotrophic lateral sclerosis (n = 12), anorectal atresia (n = 4), Poland syndrome (n = 4), and idiopathic pulmonary hypertension (n = 12). Computer-aided content analysis was performed to detect the main themes (cluster analysis) and latent factors (correspondence analysis) emerging in patients' narratives, and to test their association with gender and diagnosis. Four thematic domains were detected in the textual corpus, which are respectively referred to as: hopelessness (12.74%), need for autonomy (38.43%), search for normalcy (11.89%), and expectations of recovery (36.94%). Three latent factors explained the overall data variance: the relationship with social and medical healthcare providers (F1), adjustment processes to disease and social limitations (F2), and self-beliefs and coping (F3). Some differences were revealed with respect to patient gender and diagnosis. Illness stories highlight the significant relationship of RD patients with healthcare services and their need for a holistic approach because of the lack of effective treatment. Physical limitation and emotional distress do not necessarily seem to overlap for adjustment and quality of life (QoL). Overall, the perception of illness chronicity is likely to affect patients' self-beliefs and coping with more than their feeling of abnormalcy, that is the less salient theme.

Anxiety and quality of life of parents with children diagnosed with an anorectal malformation or Hirschsprung disease.

AIM: The aim of this study was to investigate the difference in anxiety and quality of life (QOL) between mothers and fathers of children with anorectal malformations (ARM) or Hirschsprung disease (HD). In addition, anxiety of parents of newborns was compared with parents of children in the age-group of 10 to 13 years. METHODS: Parents of 44 patients with ARM or HD completed the World Health Organization Quality of Life (WHOQOL)-BREF (26 items) and the Spielberger State-Trait Anxiety Inventory (STAI-trait 10 items, STAI-state 6 items). They were divided into two groups according to their children's age (0-4 years and 10-13 years). RESULTS: Overall mothers scored significantly worse than fathers on state anxiety (p = 0.005). Trait anxiety was not significantly different between mothers and fathers. The psychological domain of the WHOQOL-BREF was significantly different between parents (p = 0.016), with mothers scoring worse. Dividing the group in newborns and school going children mothers of newborns showed significantly more state anxiety compared with fathers of newborns (p = 0.016). In the group of older children, both anxiety and QOL were not significantly different between fathers and mothers. Comparing mothers of newborns with mothers of older children, the first group scored higher on state anxiety, but this difference was not significant (p = 0.138; 95% confidence interval, 0.535-3.717). CONCLUSIONS: Mothers of newborns have a higher level of anxiety than fathers of newborns with ARM or HD. When children with ARM or HD become older, this difference in anxiety between parents is not significant anymore. This may be explained by coping strategies.

Quality of life and anxiety in Turkish patients with anorectal malformation.

AIM: The aim of this study was to investigate quality of life (QoL) and anxiety level in Turkish children with anorectal malformation as well as the anxiety level of their mothers and the support group effects on anxiety. METHODS: A total of 87 children and their parents were included. They were grouped according to children's age: <8 years (group 1), 8-12 years (group 2) and >12 years (group 3). The anxiety of all mothers and of children in group 3 was assessed by Spielberger's State-Trait Anxiety Index-2. The QoL of children in groups 2 and 3 was evaluated with the Ped-QL 4.0 test by self-report and proxy report. The QoL results in groups 2 and 3 were compared with age-matched controls. RESULTS: Mothers in groups 1 and 3 were more anxious than were those in group 2. Adolescents in group 3 had poorer QoL compared with controls by self- and proxy reports. A significant difference was observed in QoL between the children who did and those who did not soil. The anxiety level was significantly lower in parents who attended more than one meeting. CONCLUSIONS: High anxiety and poor QoL levels in adolescence may have been related to the growing importance of body image. The higher anxiety levels of mothers in group 1 could be explained by an encounter with a baby who was different from the imagined baby and the newness of illness. QoL may have been perceived as being worse than what it was for psychological reasons.

The challenge of measuring quality of life in children with Hirschsprung's disease or anorectal malformation.

PURPOSE: The aim of the present study was to assess, after adaptation to French, the only specific quality of life (QoL) instrument for children with Hirschsprung's disease or anorectal malformation, the Hirschsprung's disease/Anorectal Malformation Quality of Life questionnaire (HAQL), in order to get a standardized QoL evaluation instrument that could further be used to help health care improvement. METHODS: The study was conducted in three teaching hospitals, including the French reference center for anorectal and pelvic malformations. After adaptation to French, QoL questionnaires were sent to the children and proxies. The questionnaire was mailed to 280 families. Psychometrics properties of the questionnaires (validity and reliability) were analysed from 120 proxy and 96 child questionnaires. RESULTS: The HAQL with the original structure was not acceptable. Exploratory steps led to a clinically pertinent structure that had acceptable fit and good validity and reliability properties. The final structure pools physical symptoms (continence, discomfort) and psychosocial dimensions (general well-being, social and emotional functioning) of QoL. CONCLUSION: The final structure, despite the disadvantage of being a new structure, allows assessment of QoL in this population and has the advantage of being shorter and validated on the clinical postoperative questionnaire from the Krickenbeck international consensus.

Long-term follow-up of paediatric anorectal anomalies and the role and efficacy of parent support groups for such conditions.

BACKGROUND/PURPOSE: There are a multitude of parent support groups for most life situations and medical conditions. The aim of this study was to discover defining characteristics and to evaluate the structure and effectiveness of parent support groups in paediatric anorectal anomalies. METHODS: Over 200 non-profit organisations and parent support groups were sent questionnaires to determine their effectiveness. Many of these were excluded as they were deemed not relevant to the final study. A final 20 groups were included for review, questions were based around origins, activities, education and evaluation, organisational structure and their affiliations with the health care team. RESULTS: Most groups had similar origins, usually an educated parent who had a child born with the condition and a zest for further knowledge and understanding and a desire to share this with others in a similar situation. Only 20% received government funding; few had paid staff and half had good relations with the local medical and paramedical fraternity. The majority had a team of long-term enthusiastic volunteers who remained with the group to support others long after their own child was no longer a concern. CONCLUSIONS: Some support groups offer a valuable service to families alongside the medical and paramedical fraternity. These groups provide families with the day-to-day lived experiences through social media, networking and meetings. Some provide more formal educational conferences designed to bring families, the medical and paramedical fraternity together to learn valuable lessons from each other.

Quality of life in children and adolescents with anorectal malformation.

PURPOSE: Quality of life (QOL) is an important endpoint in health outcomes research, especially in pediatric chronic conditions. While some studies suggest that patients with ARM report a lower level of physical and psychosocial QOL, as compared to healthy peers, not all research shows this consistently. The aim of this study was to compare the QOL of Italian children and adolescents with ARM to the QOL of a large control sample of healthy peers. METHODS: Parents of 109 children with ARM and 336 schoolchildren, completed the Pediatric Quality of Life Inventory. Analysis of variances were used to compare the groups. RESULTS: Compared to healthy peers, children with ARM showed lower emotional functioning (F = 8,41; p = .004), social functioning (F = 4,9; p = .027) and school functioning (F = 14,7; p = .000). In the adolescent group, females had a worse QOL in the physical functioning domain (F = 5,01; p = .03) than males, independent of group membership. CONCLUSIONS: While children with ARM show a more impaired QOL compared to their peers, this difference does not emerge in the adolescent group. Our results are consistent with previous findings which hypothesize that while patients with ARM face more difficulties during childhood, by adolescence they may have learned to overcome these difficulties.

Does mothers' perception of social support mediate the relationship between fecal incontinence and quality of life of the child?

PURPOSE: Fecal incontinence is a common problem in children and adolescents with anorectal malformation (ARM) and may negatively impact psycho-social well-being. Mothers' perception of social support has been proved to contribute to children's quality of life (QOL). Considering ARM studies, the role of family and social resources have received little attention. The aim of the present study was to analyze whether mothers' perception of social support mediates the impact of child fecal incontinence on his/her QOL. METHODS: One hundred and nine mothers with a child born with ARM (aged 6-15 years old; mean age = 11 years) completed questionnaires including the fecal incontinence subscale of the Hirschsprung's Disease/Anorectal Malformation QOL Questionnaire, the Pediatric QOL Inventory, and a social relationship questionnaire. Structural equation models were used to explore the relations hypothesized. RESULTS: No differences were found in the QOL scores across gender. The hypothesized model fits the data well; mothers' perception of social support partially mediated the relationship between fecal incontinence and QOL. CONCLUSIONS: An important direction for pediatric surgeons and their interdisciplinary teams may be to develop strategies to strengthen mothers' social relationships.

Clinical and psychosocial functioning in adolescents and young adults with anorectal malformations and chronic idiopathic constipation.

BACKGROUND: Faecal incontinence (FI) and constipation occur following corrective surgery for anorectal malformations (ARMs) and in children or adults with chronic constipation without a structural birth anomaly (chronic idiopathic constipation, CIC). Such symptoms may have profound effects on quality of life (QoL). This study systematically determined the burden of FI and constipation in these patients in adolescence and early adulthood, and their effect on QoL and psychosocial functioning in comparison with controls. METHODS: Patients with ARMs or CIC were compared with age- and sex-matched controls who had undergone appendicectomy more than 1 year previously and had no ongoing gastrointestinal symptoms. Constipation and FI were evaluated using validated Knowles-Eccersley-Scott Symptom (KESS) and Vaizey scores respectively. Standardized QoL and psychometric tests were performed in all groups. RESULTS: The study included 49 patients with ARMs (30 male, aged 11-28 years), 45 with CIC (32 male, aged 11-30 years) and 39 controls (21 male, aged 11-30 years). The frequency of severe constipation among patients with ARMs was approximately half that seen in the CIC group (19 of 49 versus 31 of 45); however, frequencies of incontinence were similar (22 of 49 versus 21 of 45) (P < 0·001 versus controls for both symptoms). Physical and mental well-being were significantly reduced in both ARM and CIC groups compared with controls (P = 0·001 and P = 0·015 respectively), with generally worse scores among patients with CIC. Both were predicted by gastrointestinal symptom burden (P < 0·001). There were no statistically significant differences in state or trait psychiatric morbidity between groups. CONCLUSION: FI and constipation are major determinants of poor QoL in adolescents and young adults with ARMs and in those with CIC.

The impact of anorectal malformations on anorectal function and social integration in adulthood: report from a national database.

AIM: The impact of anorectal malformation (ARM) on bowel function and social, educational and occupational end-points was investigated in adult patients entered on a national database. METHOD: Data from a national database of adult patients operated on between 1962 and 1999 for ARM were analysed. The database Malformations Ano-rectales et Pelviennes rares (MAREP) was part of a common information system, CEMARA, on rare congenital disorders. A self-administered questionnaire regarding bowel function, academic qualifications, employment and family status was mailed to patients. The type of ARM, subsequent follow-up and management including surgical interventions were retrospectively retrieved from medical records. RESULTS: Of 210 adult patients on the registry since 2008, 68 were included in this study. Only three (8.5%) had had regular follow-up. All reported some disturbance in bowel function. The fertility rate of 1.5 children per woman did not differ from the general population. CONCLUSION: Anorectal malformation ARM often leads to suboptimal bowel function in adulthood. This has an impact on social integration.

Sexual function in adults with anorectal malformation: psychosocial adaptation. German Network for Congenital Uro-REctal Malformations (CURE-Net).

PURPOSE: The aim of the German Network for Congenital Uro-REctal Malformations (CURE-Net) is to collect data of affected patients with anorectal malformation (ARM) to investigate molecular causes, clinical implications and psychosocial outcome. The current issue was to examine sexual function and to explore psychosocial adaptation in adults with ARM. METHODS: This qualitative study using narrative inquiry is part of a larger multi-center study of clinical queries and quality of life in patients with ARM. The guided interview focused on analysis of sexual function. RESULTS: 55 adult patients with ARM (23 females, 32 males, median age 23 years, range from 18 to 56 years) were investigated via standardized case-report forms comprising interview, analysis of medical data and personal questionnaires. In the female patients, 8 (35 %) of them lived alone and 15 (65 %) had sexual intercourse. In the male patients, the majority of 20 (69 %) patients lived alone and 13 (45 %) had sexual intercourse. 6 of the females got pregnant, 5 got 2 or more children. 3 of the men induced 2 or more pregnancies and fathered children. CONCLUSION: Besides reconstructing the ARM, another main goal is the preservation of sexual function. According to our data, there seems to be a close relationship between psychosocial development and sexual activity.

Assessing health-related quality-of-life changes in informal caregivers: an evaluation in parents of children with major congenital anomalies.

PURPOSE: Relatively few attempts to measure the effects on the health-related quality of life (HRQoL) of informal caregivers within the context of economic evaluations have been reported. This paper is an exploratory attempt to find suitable methods to assess caregivers' HRQoL, using a population of parents of children with major congenital anomalies. METHODS: A total of 306 parents of children born with either congenital anorectal malformations (ARM) or congenital diaphragmatic hernia were surveyed. They rated their current HRQoL on the EQ-VAS. After that, they rated their HRQoL again on the assumption that someone would take over their caregiving activities completely and free of charge. Finally, the parents classified their HRQoL on the EQ-5D. The caregivers' scores on the EQ-VAS and the EQ-5D were compared with scores elicited in the general population. RESULTS: Most parents indicated that their HRQoL would not change if someone else took on their caregiving activities. Some methodological issues may have influenced this outcome, such as difficulties in self-assessing HRQoL changes due to caregiving, process utility, protest answers, and difficulties in understanding the hypothetical question. The HRQoL of the parents was relatively low compared with population statistics, especially in the parents of children with ARM and in mothers. This can be illustrated by the difference between the mean EQ-5D score of the mothers aged 25-34 years of the children with ARM and that of the general population (0.83 vs. 0.93; P = 0.002). CONCLUSIONS: Significant HRQoL differences exist between parents caring for children with congenital anomalies and the general population. It would be useful to further improve our understanding of the HRQoL impact of informal caregiving, separating 'caregiving effects' from 'family effects', and distinguishing parent-child relationships from other caregiving situations. This study underlines the importance of considering caregivers, also in the context of economic evaluations. It indicates that general HRQoL measures, as used in patients, may be able to detect HRQoL effects in caregivers, which facilitates the incorporation in common economic evaluations of HRQoL effects in carers. Analysts and policy makers should be aware that if HRQoL improvement is an important aim, they should register HRQoL changes not only in patients but also in their caregivers.

Medical predictors of psychological anxieties in VATER patients.

BACKGROUND/PURPOSE: Following a recent classification of the VATER Association provided by the CURE-Net consortium (submitted), we investigate medical predictors of psychological stress and anxieties in this particular condition. METHODS: We developed a new set of questionnaires measuring psychological adjustment and quality of life outcome in conditions associated with anorectal and/or urogenital malformation (one self- report form to be completed by patients 7-17 years of age, two parent report forms with one relating to patients with an age range of 0-6 years, resp. 7-17 years of age). The questionnaire "Malformation-related Stress and Anxieties" comprises 26 items belonging to five subscales (I. Functional and cosmetic impairment, II. Intimacy and relationship, III. Social inclusion, IV. Psychological functioning, V. Family functioning). Every item can be responded to with respect to both actual, present problems already experienced as well as to future anxieties anticipating future development and adjustment (a perspective which especially applies in younger patients). Internal consistencies of the scales are good, resp. very good (Cronbach's α = .85 concerning present sources of anxiety scale, resp., .94 concerning future anxieties scale). The items are supplied with a Likert-type 5-point scale. We administered the questionnaire in N = 17 children and adolescents suffering from VATER via parental (proxy) report. RESULTS: As most medical risk factors affected nearly the entire sample, statistical analysis excluded investigation of differential impact on psychological stress experience and anxieties in subjects exposed versus not exposed. Special attention, therefore, was paid to those medical parameters with the best statistical power to differentiate between individuals of high versus low psychological outcome. Medical predictors differentiating between individuals with high versus low adjustment comprise post-operative infections of the urinary tract (t[15] = -3.78, p = .09), wound infections (t[15] = -3.04, p < .01), stoma complications (t[15] = -2.11, p = .08) (e.g., prolapsed (t[13] = -2.37, p = .05), other treatment complications (t[15] = -2.59, p < .05) and presence of a megacolon (t[13] = -2.44, p = .06). DISCUSSION AND CONCLUSIONS: From the perspective of stress psychology, the findings may indicate that particular medical characteristics of a malformation may operate via two different pathways: (a) pathway of severity of a particular medical risk factor: the presence of a megacolon, for example, may restrict quality of life and successful adjustment via multiple and long term functional impairments associated and (b) pathway of subjective predictability and controllability of treatment course. In accordance with theoretical models from stress psychology, the psychological impact of complicating factors such as wound-healing infections is not operating via severity of impairment, but via implicit messages they convey, indicating a low predictability and controllability of course of disease and treatment. As a result, they may increase intensity of worry and anxieties upon further difficulties still to come during future development. As a conclusion, psychological counseling may not only address concrete functional impairments and stressors, but also basic feelings of insecurity, controllability and self-efficacy.