GI Issues
Question
An 8-year-old boy with CdLS had his G-tube removed in August of last year. He was admitted to the hospital for dehydration in February with symptoms of gagging and high temperature. He was hydrated but received no other treatment at that time. Now he is refusing liquids and showing symptoms of gagging, but no fever. Mother may seek hospital treatment again. Should she have his Nissen checked for loosening or are there other procedures indicated?
Answer of our experts
This may be reflux again - or possibly something else. He may need to have his Nissen checked again and perhaps be put on an empiric trial of medication. Most importantly he should be checked by his pediatric gastroenterologist to sort things out
CD/TK 7-13-10
Approved byClinical Advisory Board (CAB)
Answer is checked and valid for
Recommendation(s)
Reflux
R32
Consider always gastro-oesophageal reflux disease (GORD) in any individual with CdLS owing to its frequency and wide variability in presentation, which includes challenging behaviour.
R33
Modification of nutrition and proton pump inhibitors (PPI) are the first-line treatments of GORD. Anti-reflux medications need to be used to their maximum dosage. Surgical interventions for GORD should be limited to those individuals with CdLS in whom nutritional and medical treatments have been unsuccessful or airway safety is at risk.
R34
If GORD symptoms persist, endoscopy should be strongly considered whilst an individual with CdLS is still in paediatric care.
R35
Surveillance for Barrett’s Oesophagus needs to be discussed with and decided together with the family, balancing the potential gain in health and burden for the individual with CdLS.
