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11 pages, 240 KiB

Open AccessArticle

Bowel Function Score in Long-Term Follow-Up for Children with Hirschsprung Disease: OASIS-Holistic Care in Hirschsprung Disease Network Position Paper

by Judith Lindert, Anders Telle Hoel, Eberhard Schmiedeke, Joe I. Curry, Stavros Loukogeorgakis and Eva Amerstorfer

Children 2024, 11(11), 1284; https://doi.org/10.3390/children11111284 - 24 Oct 2024

Viewed by 451

Abstract

Introduction: The assessment of bowel function in patients with Hirschsprung disease (HD) remains controversial, as several different bowel function scores are used in the literature and are therefore not suitable for reliable comparison. Conference Section: The OASIS-Holistic Care in Hirschsprung Disease Network Group [...] Read more.

Introduction: The assessment of bowel function in patients with Hirschsprung disease (HD) remains controversial, as several different bowel function scores are used in the literature and are therefore not suitable for reliable comparison. Conference Section: The OASIS-Holistic Care in Hirschsprung Disease Network Group addressed this issue and evaluated the most commonly used scores for their utility in HD patients. Scoping Review and Expert Group Consensus were performed. It was agreed that the ideal score for patients with Hirschsprung disease should be a comprehensive, validated score that allows reliable assessment of bowel function and continence, depicts changes according to management and natural history, and quantifies baseline parameters. This score should also enable comparisons of bowel function in patients born with HD worldwide. Concluding Remarks: The OASIS-Holistic Care in Hirschsprung Disease Network Group agreed that this ideal score does not yet exist and is therefore a future goal of the group. Meanwhile, clinicians should use a score for comparable, standardized, objective assessment, and the Rintala Score is suggested. Future developments may also lead to easily accessible patient-reported outcome measures and semi-automated dashboards that allow automated data extraction from electronic health records. Full article

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

13 pages, 656 KiB

Open AccessArticle

Influence of Diet on Bowel Function and Abdominal Symptoms in Children and Adolescents with Hirschsprung Disease—A Multinational Patient-Reported Outcome Survey

by Judith Lindert, Hannah Day, Marta de Andres Crespo, Eva Amerstorfer, Sabine Alexander, Manouk Backes, Carlotta de Filippo, Andrzej Golebiewski, Paola Midrio, Mazeena Mohideen, Anna Modrzyk, Anette Lemli, Roxana Rassouli-Kirchmeier, Marijke Pfaff-Jongman, Karolina Staszkiewicz, Lovisa Telborn, Pernilla Stenström, Karolin Holström, Martina Kohl, Joe Curry, Stavros Loukogeorgakis and Joseph R Davidson Show full author list Hide full author list

Children 2024, 11(9), 1118; https://doi.org/10.3390/children11091118 - 12 Sep 2024

Cited by 1 | Viewed by 1026

Abstract

Introduction: This study aimed to understand the influence of diet and nutrition items on gastrointestinal symptoms in patients with Hirschsprung Disease (HD). Method: An online questionnaire was created to obtain patient-reported outcomes using the multinational Holistic Care in Hirschsprung Disease Network. This was [...] Read more.

Introduction: This study aimed to understand the influence of diet and nutrition items on gastrointestinal symptoms in patients with Hirschsprung Disease (HD). Method: An online questionnaire was created to obtain patient-reported outcomes using the multinational Holistic Care in Hirschsprung Disease Network. This was distributed in Dutch, English, German, Italian, Polish, and Swedish via patient associations. Information on demographics, the extension of disease, current diet, and the influence of food ingredients on bowel function were obtained. Results: In total, 563 questionnaires were answered by parents or patients themselves. The length of the aganglionic segment was short in 33%, long in 45%, total colonic aganglionosis (TCA) in 11%, and involved the small intestine in 10%. Overall, 90% reported following a mixed diet, and 31% reported taking probiotics, with twice as many patients taking probiotics in the TCA group compared to standard HD. Mealtimes and behaviours around eating were affected by 61%, while 77% had established food items that worsened symptoms, and of these, 80% stated that they had worked these items out themselves. A high-fibre diet was followed by 24% and 18% a low-fibre diet. Symptoms were reported, particularly from dairy in 30%, fruits in 39%, pulses in 54%, and sugar in 48%. Conclusions: This first multinational survey on diet and bowel function in HD reports an association between certain dietary items with gastrointestinal symptoms. This study can support an improved understanding of the interaction between food items and bowel function in children with HD. We suggest a multidisciplinary approach to balance dietary exclusions and support adequate growth, preventing nutrition deficiencies and enhancing quality of life. 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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10 pages, 246 KiB

Open AccessArticle

Transanal Endorectal Pull-Through for Hirschsprung’s Disease: Complications and Lessons from Our Practice and the Literature

by Andrzej Gołębiewski, Stefan Anzelewicz, Daria Sosińska and Monika Osajca-Kanyion

Children 2024, 11(9), 1059; https://doi.org/10.3390/children11091059 - 29 Aug 2024

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Abstract

Background/Objectives: Hirschsprung’s disease (HD) is a congenital disorder characterised by the absence of ganglion cells in the distal bowel, resulting in functional obstruction. The transanal endorectal pull-through (TEPT) procedure, a minimally invasive approach, aims to treat HD by removing the aganglionic segment. This [...] Read more.

Background/Objectives: Hirschsprung’s disease (HD) is a congenital disorder characterised by the absence of ganglion cells in the distal bowel, resulting in functional obstruction. The transanal endorectal pull-through (TEPT) procedure, a minimally invasive approach, aims to treat HD by removing the aganglionic segment. This study assessed the feasibility, safety, and efficacy of single-stage TEPT in paediatric patients, focusing on postoperative complications, bowel function, and quality of life. Methods: A retrospective cohort study was conducted on 150 children who underwent single-stage TEPT from January 2005 to December 2023 at the Medical University of Gdansk. Data were collected from medical records, including demographics, preoperative assessments, surgical details, postoperative management, and follow-up outcomes. Statistical analyses were performed using Microsoft Excel 365 and the programming language Python 3.12. The mean age at surgery was 13 months, with a male-to-female ratio of 2.75:1. The mean operative time was 129 min, and the mean hospital stay was seven days. Results: Postoperative complications included anastomotic leak (4%), wound infections (15%), and enterocolitis (26%). Redo surgeries were required in 18% of cases due to persistent constipation and obstructive symptoms. This article includes a comprehensive review of the literature. Conclusions: TEPT demonstrates a favourable safety profile and efficacy in treating HD, though significant concerns include complications such as enterocolitis and the need for additional surgeries. Surgical expertise and thorough preoperative and postoperative management are crucial to optimising patient outcomes. Full article

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

9 pages, 1031 KiB

Open AccessArticle

The Diagnostic Pathway of Hirschsprung’s Disease in Paediatric Patients: A Single-Centre Experience

by Annita Budzanowski, Niamh Geoghegan, Alexander Macdonald and Muhammad Choudhry

Children 2024, 11(8), 970; https://doi.org/10.3390/children11080970 - 12 Aug 2024

Viewed by 717

Abstract

Background: The presenting symptoms of patients with Hirschsprung’s disease (HD) are a failure to pass meconium, abdominal distension, and bilious vomiting. The gold standard diagnosis is a rectal biopsy to confirm aganglionosis. The aim of our study was to describe the diagnostic pathway [...] Read more.

Background: The presenting symptoms of patients with Hirschsprung’s disease (HD) are a failure to pass meconium, abdominal distension, and bilious vomiting. The gold standard diagnosis is a rectal biopsy to confirm aganglionosis. The aim of our study was to describe the diagnostic pathway of Hirschsprung’s disease at our institution and document the indication for a rectal biopsy. Methods: We have performed a prospective collection of all patients who underwent a rectal biopsy to exclude HD from December 2022 until September 2023 including. The following data were collected: patient’s age, presenting symptoms, type of biopsy, failure rate, complications, and histopathological results. Results: We identified 33 patients who underwent 34 rectal biopsies at 0.6 years of age. A total of 17 patients had a rectal suction biopsy (RSB), and 17 patients underwent a partial thickness under general anaesthesia (GA). 1/17 (6%) patients had an inconclusive RSB and subsequently underwent a biopsy under GA. Constipation and chronic abdominal distension plus vomiting were the most common presenting symptoms throughout all ages. Five patients (15%) had a rectal biopsy that was positive for HD. Conclusion: A protocolised approach to the assessment of infants and children with suspected HD ensures the appropriate utilisation of invasive procedures such as biopsy. 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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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

Cited by 4 | Viewed by 1589

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 [...] Read more.

(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

Cited by 2 | Viewed by 1457

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 [...] Read more.

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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5 pages, 188 KiB

Open AccessOpinion

Obstacles to an Effective Transition to Adult Services for Patients with Hirschsprung Disease

by Joseph R. Davidson and Joe Curry

Children 2024, 11(10), 1237; https://doi.org/10.3390/children11101237 - 14 Oct 2024

Viewed by 543

Abstract

A growing number of patients with Hirschsprung disease are reaching adulthood, of whom a significant minority will require ongoing input from healthcare providers. In order to ensure patients receive the best care possible, it is essential to transition patients appropriately to adult services. [...] Read more.

A growing number of patients with Hirschsprung disease are reaching adulthood, of whom a significant minority will require ongoing input from healthcare providers. In order to ensure patients receive the best care possible, it is essential to transition patients appropriately to adult services. This article describes the unmet need and some of the obstacles to this process and explores potential solutions, drawing on model examples for transitional care. Full article

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

6 pages, 183 KiB

Open AccessOpinion

The Role of Patient Organisations—Patients’ and Parents’ Views and Experience of Hirschsprung’s Disease

by Sabine Alexander and Annette Lemli

Children 2024, 11(8), 1006; https://doi.org/10.3390/children11081006 - 16 Aug 2024

Viewed by 630

Abstract

In many countries, patient organisations offer advice and the exchange of experiences to Hirschsprung’s disease patients and their families. Professional treatment by experienced health care providers and the availability of life-long multidisciplinary follow-up care are essential. However, outside the clinic, patients and their [...] Read more.

In many countries, patient organisations offer advice and the exchange of experiences to Hirschsprung’s disease patients and their families. Professional treatment by experienced health care providers and the availability of life-long multidisciplinary follow-up care are essential. However, outside the clinic, patients and their families have to manage life on a day-to-day basis at home, which often brings up uncertainties and questions: Parents go through different stages during the diagnosis and treatment of their child, the affected children themselves go through many different stages of development, and even through the course of adulthood, new questions regarding the chronic disease may arise. Patient organisations can support the patients and their families at all stages of life by listening, offering information in an understandable way, connecting people, and sharing others’ experiences. This enables families and patients to develop a better understanding of the rare disease and promotes their management strategies and confidence. The holistic approach of patient organisations aims to complement the medical treatment. Therefore, the referral of all patients and their families to patient organisations should be part of the medical advice in the treatment of Hirschsprung’s disease. Full article

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

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 963

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 [...] Read more.

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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