Definition, investigation and management of gastrointestinal dystonia in children and young people with neurodisability.

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.