Treatment for swallowing difficulties (dysphagia) in chronic muscle disease

The management of dysphagia (difficulty in swallowing), a common complication of long-term progressive muscle disease in children and adults, is currently unclear.

Our objective was to determine the most appropriate intervention for dysphagia in people with chronic, untreatable, non-inflammatory muscle disease.

We searched the Cochrane Neuromuscular Disease Group trials register, Cochrane Central Register of Controlled Trials (Cochrane Library Issue 2, 2003), MEDLINE (from January 1966 to Week 2 June 2003), EMBASE (from January 1980 to Week 2 June 2003), AMED (from January 1985 to Week 2 June 2003), LILACS (from January 1982 to June 21 2003) and CINAHL (from January 1982 to Week 2 June 2003) and contacted authors of published studies and other experts.

We included randomised and quasi-randomised controlled trials of adults and children with chronic untreatable non-inflammatory muscle disease. The interventions under review included dietary modification, swallowing manoeuvres, a range of surgical interventions and enteral feeding. Our primary outcome was stabilisation of previously documented progressive weight loss not attributable to any other cause or weight gain of at least 5 kg in adults or increase in weight to at least the 10th centile in children, maintained for at least six months following the intervention. Secondary outcomes were: reduction in laryngeal penetration of bolus and/or aspiration observed on videofluoroscopy (modified barium swallow), reduction in chest infections attributable to aspiration over a six-month period, improvement in quality of life using a validated rating scale, the proportion of subjects who refused the intervention, the proportion of carers who refused the intervention, and serious adverse events related to the intervention within the first twelve months after intervention.

We identified no randomised controlled trials. We identified seven case series reporting the results of surgical intervention for moderate to severe dysphagia, and one reporting on the outcome of feeding advice and enteral feeding in children with a congenital myopathy.

No studies were found that fulfilled the inclusion criteria. Therefore it was not possible to determine the benefit or otherwise of surgical intervention (cricopharyngeal myotomy or upper oesophageal dilatation) for oculopharyngeal muscular dystrophy or other chronic progressive muscle diseases, and dietary advice or enteral feeding for children with congenital myopathy, compared with no intervention or an alternative intervention.

There are no trials that have adequately evaluated treatments in the management of dysphagia for chronic muscle disease. It is therefore not possible to decide on the most appropriate treatment for a given individual based on current evidence.