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Special Issue Editor

Prof. Dr. Stefanie Märzheuser

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Guest Editor

Klinik für Kinderchirurgie, Chirurgische Klinik und Poliklinik, Universität Rostock Uniklinikum und Medizinische Fakultät, Rostock, Germany
Interests: pediatric surgery; colorectal surgery

Special Issue Information

Dear Colleagues,

Hirschsprung Disease (HSCR) is a rare condition affecting the motility of the enteric system and resulting in obstruction of the bowel. Its most severe form, Total Colonic Aganglionosis (TCA), occurs in 2–5 % of all children with HSCR.

Patients should be managed by specialized medical personnel familiar with the disease.

Competence in the management of Hirschsprung’s Disease is not only defined by an ability to perform complex surgery; it is also based on diagnostic processes, handling complications and addressing long term effects of the malformation.

Therapeutic strategies must be adjusted in line with current scientific knowledge and should increase the quality of life in patients as well as maintain continuity through transition of care.

At the OASIS Symposium, clinicians, researchers and patients (representatives) from all over Europe will work together on a holistic approach to care.

There will be a particular focus on:

Patients and Family needs before and after surgery;
Patient support groups for Hirschsprung’s Disease in Europe as well as ideas for cross-collaboration;
Diagnostic pathways;
Surgical Strategies in Hirschsprung’ Disease and total colonic aganglionosis;
Optimal bowel management;
Holistic Continence Score;
Nutrition and Microbioma;
Quality of life aspects; longitudinal follow up;
Transition of care.

The Symposium will be open to everyone. We want clinicians (doctors and allied health professionals), scientists and patient associations to come together to share their clinical experiences and discuss together how to achieve the best level of care for this patient group and their families.

More information please contact us: [email protected] (https://forms.gle/CdHppxTobEY9ShiXA).

This Special Issue will also focus on other articles or reviews related to Hirschsprung’s Disease (OASIS).

Prof. Dr. Stefanie Märzheuser
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Children is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2400 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

Hirschsprung’s disease
total colonic aganglionosis
self-help-group
quality of life

Published Papers (3 papers)

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Research

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11 pages, 1106 KiB

Open AccessArticle

Bowel Management in Hirschsprung Disease—Pre-, Peri- and Postoperative Care for Primary Pull-Through

by Judith Lindert, Felix Schulze and Stefanie Märzheuser

Children 2024, 11(5), 588; https://doi.org/10.3390/children11050588 - 13 May 2024

Viewed by 556

Abstract

(1) Background: Bowel management contributes throughout the pathway of care for children with Hirschsprung. Preoperative bowel management prepares the child and family for the pull-through surgery. Perioperative bowel management supports early recovery and tailored bowel management in the follow-up supports the achievement of social continence. (2) Methods: We conducted a cross-sectional assessment of our institutional bowel management program to illustrate the pre-, peri- and postoperative bowel management strategies. (3) Results: A total of 31 children underwent primary pull-through, 23 without a stoma and 8 with a stoma, at a median age of 9 months. All children without a stoma were prepared for surgery by using rectal irrigations. Children with a stoma were prepared for surgery with a transfer of stoma effluent. Transanal irrigation supported early recovery. (4) Conclusions: Bowel management is a key pillar of the management of children with Hirschsprung disease. Incorporating bowel management in the pathway of care facilitates primary pull-through and supports perioperative recovery. Full article

(This article belongs to the Special Issue Selected Papers from the OASIS (Holistic Care in Hirschsprung’s Disease) Meeting)

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12 pages, 664 KiB

Open AccessArticle

Children with Hirschsprung’s Disease Report Dietary Effects on Gastrointestinal Complaints More Frequently than Controls

by Lovisa Telborn, Christina Granéli, Irene Axelsson and Pernilla Stenström

Children 2023, 10(9), 1543; https://doi.org/10.3390/children10091543 - 12 Sep 2023

Viewed by 1012

Abstract

Hirschspung’s disease (HD) is a congenital gastrointestinal (GI) disorder frequently accompanied by GI complaints. Despite the lack of evidence regarding whether diet affects GI symptoms, advice on dietary changes is common. The aim was to investigate self-reported dietary effects on GI symptoms, comparing children with HD with healthy children. This was an observational, cross-sectional, self-reported case-control study using the validated Diet and Bowel Function questionnaire. All children with HD aged 1–18 years were surgically treated during 2003–2021 at a national HD center, and their parents were invited to participate. Healthy children served as controls. The data were presented as median (range) and n (%). 71/85 children with HD (6 years (1–17); 76% boys) and 265/300 controls (9 years (1–18); 52% boys) participated. Dietary effects on GI symptoms were reported more frequently by children with HD than controls (55/71 [77%] vs. 137/265 [52%], p ≤ 0.001), as were dietary adjustments to improve GI symptoms (49/71 [69%] vs. 84/265 [32%], p ≤ 0.001), and social limitations due to dietary adjustments (20/48 [42%] vs. 22/121 [18%], p = 0.002). Of 90 food items, children with HD reported that more of the items induced GI symptoms compared to controls (7 (0–66) vs. 2 (0–34), p = 0.001). Diet-induced GI symptoms and dietary adjustments’ impact on daily life are reported more frequently by children with HD than controls. Moreover, the number and types of food items causing GI symptoms differ. The results indicate the need for disease-specific dietary advice to improve support for families of children with HD. Full article

(This article belongs to the Special Issue Selected Papers from the OASIS (Holistic Care in Hirschsprung’s Disease) Meeting)

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Other

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5 pages, 795 KiB

Open AccessOpinion

Hirschsprung Disease: The Role of the Clinical Nurse Specialist

by Julie-Ann Milbery and Joe Curry

Children 2024, 11(5), 587; https://doi.org/10.3390/children11050587 - 13 May 2024

Viewed by 492

Abstract

Hirschsprung disease is a life-long condition that can have a significant impact on both children and their families. This article explores the role of the clinical nurse specialist and the support they can provide from initial diagnosis through the patient’s surgical journey and right through to transition into adult services. Through the provision of education, training, signposting of social and psychological support, and linking in with community-based services, the clinical nurse specialist can help the child and family to limit that impact of the disease. Full article

(This article belongs to the Special Issue Selected Papers from the OASIS (Holistic Care in Hirschsprung’s Disease) Meeting)

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