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Outcome in adults with anorectal malformations in relation to modern classification – Which patients do we need to follow beyond childhood?
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Women's and Children's Health, Paediatric Surgery.
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Surgical Sciences, Colorectal Surgery.
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Surgical Sciences, Colorectal Surgery.
Department of Pediatric Surgery, Karolinska University Hospital and Karolinska Institutet, Stockholm, Sweden.
2017 (English)In: Journal of Pediatric Surgery, ISSN 0022-3468, E-ISSN 1531-5037, Vol. 52, no 3, 463-468 p.Article in journal (Refereed) Published
Abstract [en]

Background/purpose

Knowledge about the functional outcome in adults with anorectal malformations is essential to organize structured transition to adult care for this patient group. The aim of this study was to investigate the functional outcome and quality of life in adults with anorectal malformations characterized according to the Krickenbeck classification.

Methods

Of 256 patients diagnosed with anorectal malformations at our institution in 1961–1993, 203 patients could be traced and were invited to participate in the study. One hundred and thirty-six patients replied (67%) and were compared with one hundred and thirty-six population based sex and age-matched controls. Patients and controls were evaluated with both a validated questionnaire as well as a study-specific questionnaire to assess bowel function. SF-36 was used for quality of life. Outcome in nine incontinence-related parameters, 10 constipation-related, 6 urogenital function-related, and 13 quality of life parameters were assessed in the patients and compared to the outcome of controls as well as to the type of anorectal malformations according to the Krickenbeck classification.

Results

The ARM-patients had an inferior outcome (P < 0.05) for all incontinence parameters, 8 of 10 parameters for constipation, 2 of 6 for urogenital function and 7 of 13 quality of life parameters. Patients with rectobulbar and vestibular fistulas had the worst statistical outcome but patients with cloaca and rectoprostatic/bladder-neck fistula had worse outcome in absolute numbers. Forty-four patients (32%) reported incontinence of stool at least once a week and 16 (12%) had a permanent colostomy.

Conclusions

The functional outcome and quality of life in adults with anorectal malformations are closely related to the type of malformation. A large proportion of the patients have persistent fecal incontinence, constipation and sexual problems that have a negative influence on their quality of life. Structured multidisciplinary follow-up of adults with anorectal malformations by pediatric and colorectal surgeons, as well as urologists and gynecologists is therefore advocated.

Place, publisher, year, edition, pages
2017. Vol. 52, no 3, 463-468 p.
Keyword [en]
Anorectal malformations, Long term outcome, Functional outcome, Sexual outcome, Quality of life, Transition to adult care
National Category
Surgery
Research subject
Pediatric Surgery
Identifiers
URN: urn:nbn:se:uu:diva-241240DOI: 10.1016/j.jpedsurg.2016.10.051ISI: 000397964100019PubMedID: 27894765OAI: oai:DiVA.org:uu-241240DiVA: diva2:777878
Available from: 2015-01-09 Created: 2015-01-09 Last updated: 2017-04-26Bibliographically approved
In thesis
1. Anorectal Malformations: Long-term outcome and aspects of secondary treatment
Open this publication in new window or tab >>Anorectal Malformations: Long-term outcome and aspects of secondary treatment
2015 (English)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

Faecal incontinence (FI) is defined as the inability to control bowel movements. The causes of FI are many and diverse. One of the more uncommon reasons for FI is Anorectal Malformations (ARMs). An ARM is a congenital anomaly that affects somewhere between 1/2500 and 1/5000 live born babies. Many ARM patients have persistent FI. Several different procedures have been utilised to address this issue. This thesis aims to evaluate (1) the long-term outcome in adulthood of ARMs in relation to the modern Krickenbeck classification, and (2) scope for treating FI with transanal injection with dextranomer in non-animal stabilised hyaluronic acid (NASHA/Dx), in patients both with and without ARMs.

All patients treated for ARMs in Uppsala up to 1993 were invited to participate in a questionnaire study of quality of life and function. The study included 136 patients and compared them with 136 age- and sex-matched controls. The Krickenbeck classification was found to predict functional outcome, and ARM patients had more problems with incontinence and obstipation, as well as inferior Quality of Life (QoL), compared with controls. 

Thirty-six patients with FI, owing to causes other than ARMs, were treated with transanal submucous injection of NASHA/Dx. The patients were monitored for two years after treatment. Significant reductions in both their incontinence score and the number of their incontinence episodes were achieved.  A significant improvement in QoL was observed in patients who had at least a 75% reduction in incontinence episodes. No serious complications occurred.

A prospective study of transanal injection of NASHA/Dx was conducted on seven patients with persistent FI after ARMs. After six months a significant reduction in the number of incontinence episodes was obtained. A significant improvement in QoL was also found. No serious complications occurred.

In conclusion, adult patients with ARMs have inferior outcome of anorectal function and QoL compared with controls. NASHA/Dx is effective and appears to be safe in treating FI in general. This effect seems to be the same in selected patients with persistent FI after ARMs.

Place, publisher, year, edition, pages
Uppsala: Acta Universitatis Upsaliensis, 2015. 109 p.
Series
Digital Comprehensive Summaries of Uppsala Dissertations from the Faculty of Medicine, ISSN 1651-6206 ; 1065
Keyword
anorectal malformation, faecal incontinence, long-term outcome, Krickenbeck, NASHA/Dx
National Category
Surgery
Identifiers
urn:nbn:se:uu:diva-241243 (URN)978-91-554-9140-6 (ISBN)
Public defence
2015-02-27, Rosénsalen, Entrance 95/96, ground floor, Uppsala University Children’s Hospital, Uppsala, 13:15 (English)
Opponent
Supervisors
Available from: 2015-02-05 Created: 2015-01-09 Last updated: 2015-03-09

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Danielson, JohanKarlbom, UrbanGraf, Wilhelm

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