Background: People with severe recessive dystrophic epidermolysis bullosa (RDEB) have reduced oral intake as a result of oropharyngeal blistering, esophageal stricture, and constipation. Their nutritional requirements are increased by the need for constant wound repair and inflammation. No studies have been published on long-term outcomes in patients in whom gastrostomy tubes (G-tubes) were placed in childhood and who have now reached adulthood.
Methods: Twelve adults with RDEB who had used G-tubes in situ for a mean of 14.5 years (range: 10-19 years) were studied. The following growth parameters were recorded: current body mass index (BMI); height centile pre-gastrostomy; best height centile post-gastrostomy; height centile reached by the age of 18 years; and pubertal status. Nutrient intake via the tube and leakage were noted. Data on BMI and height centile reached by the age of 18 years in five young adults with severe RDEB who had declined tube placement were recorded.
Results: The mean ± SD BMI of patients fed by G-tube at adulthood was 20.2 ± 3.9 kg/m2. Eight subjects had increased, three had maintained, and one had decreased their BMI centile position. Three patients had increased, six had maintained, and three had decreased their height centile position; the latter three all increased their height centile initially. They were compared with five adults with severe RDEB who had declined G-tube placement. Four had reached a height within the normal centile range. In two, this was lower than their best height centile. None had reached a normal BMI (mean: 15.4 kg/m2).
Conclusions: By increasing nutritional intake, G-tube feeding led to an improvement in BMI and helped maintain or improve linear growth.
© 2015 The International Society of Dermatology.