Transition of care in patients with anorectal malformations: Consensus by the ARM-net consortium

Giuliani, Stefano; Grano, Caterina; Aminoff, Dalia; Schwarzer, Nicole; Vorle, Mariette Van De; Crétolle, Célia; Haanen, Michel; Brisighelli, Giulia; Märzheuser, Stefanie; Connor, Martin J.

doi:10.1016/j.jpedsurg.2017.06.008

Cited by 37 publications

(45 citation statements)

References 59 publications

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“…Secondly, the importance of a structured, individually adapted transitional process for ARM-patients previously identified by other studies [15,17] was confirmed by this study. Participants emphasized the importance of a dynamic transitional process, grounded in the individual's needs and wishes by e.g.…”

Section: Discussionsupporting

confidence: 84%

“…Firstly, patient education and patient health literacy were described as important, which is in line with previous studies regarding the transition to adult care for patients with chronic conditions [17,[27][28][29][30]. Knowledge and condition literacy are also reported to be essential contributors to positive coping abilities when living with a chronic condition [31,32].…”

Section: Discussionsupporting

confidence: 82%

“…Since patients born with ARM might suffer from several somatic-and psychological conditions related to their congenital malformation, they depend on life-long treatment and follow-up by multiple medical specialties. There are a few studies exploring the transitional process from paediatric-to adult healthcare for patients born with ARM [15][16][17][18][19]. The transitional care is referred to as being inadequate, but the manner in which adolescents wish to make their transfer has not yet been explored.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Key components of successful transition for adolescents born with anorectal malformations–a Nordic focus group study

Tofft

Hoel

Håkansson

et al. 2020

International Journal of Adolescent Medicine and Health

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ObjectivesTransitional care for adolescents with congenital malformations, such as anorectal malformations (ARM), is described sparsely in the literature and referred to as being inadequate. In order to organize future successful healthcare structures, knowledge of patient-reported important aspects of transition is required. The aim of the study was therefore to explore the needs and expectations of transitional- and adult healthcare among adolescents and adults born with ARM.MethodsTwo tertiary paediatric surgical centres, in collaboration with two tertiary pelvic floor centres, in Sweden and Norway, conducted a qualitative study, involving adolescents and adults born with ARM in focus group discussions regarding transitional care. Discussions were analyzed by qualitative content analysis. Ethical approval was obtained.ResultsSixteen participants (10 women) with a median age of 24 (19–47) years, born with mixed subtypes of ARM were included in gender-divided focus groups. Participants emphasized a need for improved knowledge of ARM, both among patients and adult care providers. Participants identified a need for support with coping strategies regarding challenging social- and intimate situations due to impaired bowel function. Participants pin-pointed well-functioning communication between the patient and the paediatric- and adult care providers as a key factor for a successful transitional process. Further, participants emphasized the importance of easy access to specialized adult healthcare when needed, suggested to be facilitated by appointed patient navigators.ConclusionAdolescents and adults born with ARM identify improved knowledge of ARM, well-functioning communication and easy access to specialized adult care as key components of a successful transition.

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Section: Discussionsupporting

confidence: 84%

Section: Discussionsupporting

confidence: 82%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Key components of successful transition for adolescents born with anorectal malformations–a Nordic focus group study

Tofft

Hoel

Håkansson

et al. 2020

International Journal of Adolescent Medicine and Health

View full text Add to dashboard Cite

show abstract

“…It is interesting to notice that studies exploring factors affecting the transition process or providing specific transition guidelines are available for all the above mentioned groups of RD, but none of them provides data on the global number of patients potentially experiencing transition into adulthood [ 46 , 47 , 48 , 49 , 50 , 51 ]. When available, these figures are scattered, either because referred to a single Center experience and/or to a single rare disease [ 52 , 53 ].…”

Section: Discussionmentioning

confidence: 99%

The Epidemiology of Transition into Adulthood of Rare Diseases Patients: Results from a Population-Based Registry

Mazzucato

Pozza

Minichiello

et al. 2018

IJERPH

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Background: Despite the fact that a considerable number of patients diagnosed with childhood-onset rare diseases (RD) survive into adulthood, limited information is available on the epidemiology of this phenomenon, which has a considerable impact both on patients’ care and on the health services. This study describes the epidemiology of transition in a population of RD patients, using data from the Veneto Region Rare Diseases Registry (VRRDR), a web-based registry monitoring since 2002 a consistent number of RD in a defined area (4.9 million inhabitants). Methods: Longitudinal cohorts of patients born in the years 1988 to 1998 and enrolled in the VRRDR in their paediatric age were identified. Data referred to this group of patients, experiencing transition from paediatric to adult age during the years 2006–2016, are presented. Results: 2153 RD patients (44.1% females and 55.9% males) passed from childhood to adulthood in the study period, corresponding to a 3-fold increase from 2006 to 2016. The majority of these patients was affected by congenital anomalies (32.0%), by hematologic diseases (15.9%), eye disorders (12.1%) and neoplasms (7.9%). RD patients who experienced transition from paediatric age to adulthood represent the 9.2% of adult patients enrolled in the Registry at 31 December 2016. Conclusions: We described a subset of RD young adults experiencing transition into adulthood. The data reported can be considered as minimum values for estimating the size of this increasing population presenting specific transition needs. These figures are valuable for clinicians, patients and health planners. Public policy interventions are needed in order to promote dedicated care transition pathways in the broader framework of health policies devoted to RD.

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“…The possibility to group patients based on similar characteristics is of great importance to uniform follow up and make different centers' series comparable ( 4 ). Indeed, one of the problems, when dealing with adolescents and adult patients, is to understand the original anatomic defect and preceding treatments in order to correctly address the complications ( 5 – 8 ).…”

Section: Introductionmentioning

confidence: 99%

Inter- and Intraobserver Variation in the Assessment of Preoperative Colostograms in Male Anorectal Malformations: An ARM-Net Consortium Survey

et al. 2020

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Aim: Male patients with anorectal malformations (ARM) are classified according to presence and level of the recto-urinary fistula. This is traditionally established by a preoperative high-pressure distal colostogram that may be variably interpreted by different surgeons. The aim of this study was to evaluate the inter-and intraobserver variation in the assessment by pediatric surgeons of preoperative colostograms with respect to the level of the recto-urinary fistula. Materials and Methods: Sixteen pediatric surgeons from 14 European centers belonging to the ARM-Net Consortium twice scored 130 images of distal colostograms taken in sagittal projection at a median age of 66 days of life (range: 4-1,106 days). Surgeons were asked to classify the fistula in bulbar, prostatic, bladder-neck, no fistula, and "unclear anatomy" example. Their assessments were compared with the intraoperative findings (kappa) for two scoring rounds with an interval of 6 months (intraobserver variation). Agreement among the surgeons' scores (interobserver variation) was also calculated using Krippendorff's alpha. A kappa over 0.75 is considered excellent, between 0.40 and 0.75 fair to good, and below 0.40 poor. Surgeons were asked to score the images in "poor" and "good" quality and to provide their years of experience in ARM treatment. Midrio et al. Colostograms of Recto-Urinary Fistula Results: Agreement between the image-based rating of surgeons and the intraoperative findings ranges from 0.06 to 0.45 (mean 0.31). Interobserver variation is higher (Krippendorff's alpha between 0.40 and 0.45). Years of experience in ARM treatment does not seem to influence the scoring. The mean intraobserver variation between the two rounds is 0.64. Overall, the quality of the images is considered poor. Images categorized as having a good quality result in a statistically significant higher kappa (mean: 0.36 and 0.37 in the first and second round, respectively) than in the group of bad-quality images (mean: 0.25 and 0.23, respectively). Conclusions: There is poor agreement among experienced pediatric colorectal surgeons on preoperative colostograms. Techniques and analyses of images need to be improved in order to generate a homogeneous series of patients and make comparison of outcomes reliable.

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Transition of care in patients with anorectal malformations: Consensus by the ARM-net consortium

Cited by 37 publications

References 59 publications

Key components of successful transition for adolescents born with anorectal malformations–a Nordic focus group study

Key components of successful transition for adolescents born with anorectal malformations–a Nordic focus group study

The Epidemiology of Transition into Adulthood of Rare Diseases Patients: Results from a Population-Based Registry

Inter- and Intraobserver Variation in the Assessment of Preoperative Colostograms in Male Anorectal Malformations: An ARM-Net Consortium Survey

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