Hydropericardium causing sudden infant death in glycogenosis type I: osmotic injury due to percutaneous silastic catheterization

BACKGROUND: The aim of this case report and the review of the literature is to demonstrate dangers when using peripherial silastic catheters in preterm and term newborns or infants. PATIENT, METHODS AND RESULTS: We report on a female infant with glycogenosis type I a (MIM 232200) due to glucose 6-phosphatase deficiency (homozygosity for R170X) and sudden infant death at the age of 9 months due to a rare catheter complication (hydropericardium with tamponade without perforation). CONCLUSION: We believe that this fatal complication was caused by local osmotic dysbalance due to direct contacts between atrial wall and the catheter tip. There is no relation known between patients with inborn errors of metabolism complicated by metabolic derangement and higher incidences of mechanical or non-mechanical catheter complications.