{
  "abstract": "Background Children and young people with severe neurodisabling conditions (CYPSND)experience severe functional gastrointestinal symptoms and dependence on artificial nutrition. ‘Gastrointestinal dystonia’ (GID) has been applied by clinicians when symptoms become debilitating and potentially life-limiting. Evidence is lacking regarding the definition and appropriate management of GID.Methods We therefore assembled a RAND appropriateness panel. We performed a systematic review, created an online survey and distributed this to a panel of 27 experts from five stakeholder groups from 13 UK specialist centres across the British Isles (gastroenterology, neurology/neurodisability, surgery, palliative care and allied health professionals). A Disagreement Index ≥1 indicated disagreement.Findings The panel rated the appropriateness of 250 statements covering the following in GID: definition, clinical evaluation, nutritional assessment/feeding strategies, investigations, medications and prescribing, surgical interventions, safeguarding, palliative care and ethics. Agreement was reached except in selected statements regarding uncommon diagnostic features. There was uncertainty in specific clinical scenarios regarding: investigation, the use of blenderised diet, certain pharmacological agents and surgical interventions. The only intervention deemed inappropriate was antireflux surgery in the context of GID and gastrointestinal dysmotility without reflux disease. The remaining statements (198) were considered appropriate.Interpretation We present a comprehensive review, agreement on the definition of GID and recommendations on management pathways agreed by a selected panel of multidisciplinary experts. Clear diagnostic criteria will enable important epidemiological work to record outcomes for this complex patient group. Identifying the associated morbidity, burden of care and mortality will help advocate for appropriate health resources and support to carers and families.",
  "authors": [
    {
      "affiliations": [
        "Paediatric Gastroenterology, Royal Hospital for Children, Glasgow, UK"
      ],
      "name": "Andrew R Barclay"
    },
    {
      "affiliations": [
        "Gastroenterology, North Bristol NHS Trust, Westbury on Trym, Bristol, UK"
      ],
      "name": "Susanna Meade"
    },
    {
      "affiliations": [
        "British Association of Paediatric Surgeons, London, London, UK"
      ],
      "name": "Catherine Richards"
    },
    {
      "affiliations": [
        "Paediatric Palliative Care, University Hospital Southampton NHS Foundation Trust, Southampton, Hampshire, UK",
        "Naomi House & Jacksplace Hospices, Winchester, UK"
      ],
      "name": "Timothy Warlow"
    },
    {
      "affiliations": [
        "Paediatric Neurosciences, Guy's and St Thomas’ NHS Foundation Trust, London, UK"
      ],
      "name": "Daniel E Lumsden"
    },
    {
      "affiliations": [
        "Paediatric Neurosciences, Evelina Children’s hospital, London, UK"
      ],
      "name": "Charlie Fairhurst"
    },
    {
      "affiliations": [
        "Paediatric Gastroenterology, Royal Hospital for Children and Young People, Edinburgh, Edinburgh, UK"
      ],
      "name": "Catherine Paxton"
    },
    {
      "affiliations": [
        "Paediatric Neurology, Royal Hospital for Children, Glasgow, Glasgow, UK"
      ],
      "name": "Katharine Forrest"
    },
    {
      "affiliations": [
        "Paediatric Neurology, Sheffield Children’s Hospital NHS Foundation Trust, Sheffield, UK"
      ],
      "name": "Santosh R Mordekar"
    },
    {
      "affiliations": [
        "Paediatric Gastroenterology, Royal Victoria Infirmary Great North Children’s Hospital, Newcastle Upon Tyne, UK"
      ],
      "name": "David Campbell"
    },
    {
      "affiliations": [
        "Paediatric Gastroenterology, Great North Children’s Hospital, Newcastle Upon Tyne, UK"
      ],
      "name": "Julian Thomas"
    },
    {
      "affiliations": [
        "Paediatric Gastroenterology, Royal Hospital for Children, Glasgow, UK"
      ],
      "name": "Michelle Brooks"
    },
    {
      "affiliations": [
        "Paediatric Surgery, Royal Hospital for Sick Children, Glasgow, UK"
      ],
      "name": "Gregor M Walker"
    },
    {
      "affiliations": [
        "Division of Neurogastroenterology & Motility, Department of Paediatric Gastroenterology, Great Ormond Street Hospital NHS Foundation Trust, London, UK"
      ],
      "name": "Osvaldo Borrelli"
    },
    {
      "affiliations": [
        "Paediatric Dietetics, University Hospital Southampton NHS Foundation Trust, Southampton, Hampshire, UK"
      ],
      "name": "Helen Wells"
    },
    {
      "affiliations": [
        "Alder Hey Children’s Hospital, Liverpool, Merseyside, UK"
      ],
      "name": "Susie Holt"
    },
    {
      "affiliations": [
        "c/o Paediatric Gastroenterology Secretary, Tallaght University Hospital, Dublin, Ireland"
      ],
      "name": "Shoana Quinn"
    },
    {
      "affiliations": [
        "Paediatric Palliative care, Birmingham Children’s Hospital NHS Foundation Trust, Birmingham, Birmingham, UK"
      ],
      "name": "Yi Fan Liang"
    },
    {
      "affiliations": [
        "Evelina London Children’s Hospital, London, UK"
      ],
      "name": "Mohamed Mutalib"
    },
    {
      "affiliations": [
        "Paediatric Gastroenterology, Leeds Teaching Hospitals NHS Trust, Leeds, UK"
      ],
      "name": "Elena Cernat"
    },
    {
      "affiliations": [
        "John Radcliffe Hospital, Oxford, Oxfordshire, UK"
      ],
      "name": "Alex C H Lee"
    },
    {
      "affiliations": [
        "Department of Paediatric Neurodisability, Belfast Health and Social Care Trust, Belfast, UK"
      ],
      "name": "Claire Teresa Lundy"
    },
    {
      "affiliations": [
        "Palliative Medicine, Children’s Health Ireland, Dublin, Ireland",
        "School of Medicine, University College Dublin, Dublin, Leinster, Ireland"
      ],
      "name": "Fiona McElligott"
    },
    {
      "affiliations": [
        "Hafan Y Mor Children’s Centre, Abertawe Bro Morgannwg University Health Board, Swansea, Neath Port Talbot, UK"
      ],
      "name": "Jo Griffiths"
    },
    {
      "affiliations": [
        "Medicine, Children’s Hospice Association Scotland, Edinburgh, Scotland, UK"
      ],
      "name": "Paul Eunson"
    },
    {
      "affiliations": [
        "Paediatric Dietetics, Birmingham Children’s Hospital NHS Foundation Trust, Birmingham, Birmingham, UK"
      ],
      "name": "Haidee Norton"
    },
    {
      "affiliations": [
        "Paediatric Gastroenterology, Birmingham Children’s Hospital NHS Foundation Trust, Birmingham, Birmingham, UK"
      ],
      "name": "Lisa Whyte"
    },
    {
      "affiliations": [
        "Gastroenterology, Guy's and St Thomas’ Hospitals NHS Trust, London, London, UK"
      ],
      "name": "Mark A Samaan"
    },
    {
      "affiliations": [
        "Department of Paediatric Gastroenterology, Birmingham Women's and Children’s NHS Foundation Trust, Birmingham, UK"
      ],
      "name": "Sue Protheroe"
    }
  ],
  "title": "Definition, investigation and management of gastrointestinal dystonia in children and young people with neurodisability",
  "uid": "4e01b198-524d-54cc-afd1-531d1f2af521"
}