Quality of life and functional outcome in Swedish children with low anorectal malformations: a follow-up study.

PURPOSE: The aim was to investigate the quality of life and bowel function in children with low anorectal malformations (ARM). ADDITIONAL AIM: To evaluate the Swedish version the Hirschsprung's Disease/Anorectal Malformation Quality of life Questionnaire (HAQL). METHODS: Forty-four children and their parents were invited to complete the HAQL and the Bowel Function Score (BFS). Healthy children participated as controls and completed the HAQL. RESULTS: Seventeen children and 18 mothers completed the HAQL. The children reported impaired function in the physical symptom (PH) fecal continence (FC) and laxative diet (LD) domains compared to controls. Compared with their mothers, they reported impaired physical function and more symptoms in the emotional functioning (EMF) and PH domains. 27 families completed the BFS; 63% reported normal bowel function, 33% moderate outcome and one patient, comprising 4%, poor outcome. Evaluation of the HAQL, FC, EMF and PH domains showed no obvious conflicts. CONCLUSIONS: The children did not differ much regarding their QoL, even though they appeared to have impaired bowel function and worse emotional functioning compared to controls. The mothers underestimated their children's physical symptoms and overestimated their emotional functioning. Evaluated domains in the HAQL appear to work as intended, but the questionnaire needs further development.

Impact of Low Anorectal Malformation on Parenting Stress: A Mixed-Method Study.

The purpose of this study was to investigate parenting stress among parents of children with low ARM. STUDY AIMS: 1) Compare parenting stress among parents of children with low ARM, with parents of healthy children using questionnaires. 2) Identify subscales within the questionnaire which needed to be further explored. 3) Use semi-structured interviews with parents of children with low ARM, to explore parenting stress and to explain, expand and or support the quantitative findings. DESIGN AND METHODS: An explanatory sequential mixed methods design was used in this follow up study. The parents completed the Swedish Parenthood Stress Questionnaire (SPSQ), semi-structured interviews were conducted. RESULTS: Fifteen mothers and 13 fathers of children with low ARM age 8-18, returned completed questionnaires. A control group of 17 mothers and 6 fathers of healthy children age 8-18 that had visited the hospital for a minor procedure was recruited for comparison purposes. There were no significant differences found between index group and controls except in the subscale Incompetence, where parents of children with low ARM reported lower levels of stress compared to controls. Nine semi-structured interviews were conducted with parents of children with low ARM. Qualitative content analysis was used and revealed three themes - Communication between parents, Expectations of parenthood, and Challenges concerning parenthood. CONCLUSIONS: Parents of children with low ARM did not report high levels of stress. When interviewed, they told about earlier experiences of emotional stress, feelings of guilt, and chaos at the time the child was born and during infancy.

Translation and cultural adaptation of the Hirschsprung's Disease/Anorectal Malformation Quality of life Questionnaire (HAQL) into Swedish.

PURPOSE: Children with anorectal malformation or Hirschsprung's Disease (HD) often have functional problems with constipation or incontinence. The Hirschsprung's Disease/Anorectal malformation Quality of life Questionnaire (HAQL) developed in the Netherlands is a disease-specific instrument measuring the quality of life (QoL) of children and adolescents with fecal incontinence. HAQL includes several domains with questions concerning diet, laxatives, constipation, diarrhea, urine and fecal incontinence, in addition to social and emotional functioning, body image, and physical symptoms. The purpose of the study was to translate and culturally adapt the HAQL questionnaire into Swedish. METHOD: The translation was carried out according to accepted translation guidelines and a backward/forward translation method was used. RESULTS: The translation correlated well with the original. All in all the Swedish and the Dutch versions agreed well. The Swedish translators chose to use a more simplified language in the questionnaires intended for the children, but used another choice of words in the proxy version and the adolescents' version. CONCLUSIONS: The translation of the HAQL instrument into Swedish gives us a disease-specific QoL instrument for children and adolescents born with HD and anorectal malformations (ARM). The translated and culturally adapted HAQL instrument is included in a survey regarding children and adolescents born with ARM.

Cosmetic and functional outcome after stoma site skin closure in children.

PURPOSE: To evaluate the functional and cosmetic outcome of stoma closure in children after straight closure (SC) versus purse-string closure (PSC). METHODS: The patients (n = 33, age 16-159 months), operated at a median age of 6 months (1-121 months) between 2007 and 2009 in our hospital, were studied to evaluate whether the proposed superiority of the PSC technique is applicable in children. The patients were operated with SC or PSC. The most common causes of the temporary stomas were necrotizing enterocolitis, Hirschsprung's disease and anal atresia. A validated scoring-system questionnaire (patient and observer scar assessment scale),POSAS was sent to the parents containing questions concerning pain, itchiness, colour, stiffness, thickness and irregularity of the scar completed by a visual analogue scale to evaluate an overall opinion. RESULTS: 25 families (SC; n = 12, PSC;n = 13) participated. The differences between the two groups are largest, although not statistically significant, for discoloration, stiffness, thickness and irregularity, with better scores in the PSC group. There was a better total POSAS score in the PSC group whilst the VAS shows very modest differences. CONCLUSION: Our study indicates advantages of the PSC technique after stoma closure with better cosmetic and functional outcome. To be able to show statistically significant differences between PSC and SC a larger study would be useful.