Percutaneous endoscopic gastrostomy helped to normalise feeding problems and gastrointestinal symptoms in Silver-Russell syndrome.

Abstract

Aim: This study evaluated feeding problems and gastrointestinal symptoms in children with Silver-Russell syndrome (SRS), which is a rare epigenetic disorder. It also compared the symptoms experienced during different feeding methods, including percutaneous endoscopic gastrostomy (PEG).

Methods: The national expert team for children with SRS at Queen Silvia Children's Hospital, Gothenburg, studied 46 referrals (63% male) who were born with SRS in Sweden from 1984 to 2018. Patient data were extracted from the Paediatric National Growth Hormone Registry.

Results: The medical records covered a median of 68% of the time of the patients' childhood, with a median follow-up of 9 years. Their symptoms were most prevalent during infancy and decreased when they were toddlers. Feeding problems and gastrointestinal symptoms were reported in 91% of the 46 patients, with vomiting in 57% and constipation in 46%. There were 19 children who relied on enteral feeding for their nutrition and 13 of those received PEG. Their body mass index (BMI) increased significantly 2 years after PEG started (p = 0.005).

Conclusion: Feeding problems and gastrointestinal symptoms were very common in children with SRS, but partly disappeared during childhood. Providing treatment, such as PEG, normalised the BMIs of children with SRS and reduced their symptoms.