HIATUS PARAESOPHAGEAL HERNIAS IN NEONATAL MARFAN-SYNDROME/

We report on an infant with neonatal Marfan syndrome (NMS) and hiatus/ paraesophageal hernia who presented to a university hospital with an u nusual early complication of this connective tissue disorder. An abnor mal course of the nasogastric tube was noted on the first day of life by a radiograph of the chest and abdomen performed for bloody gastric drainage. The question of esophageal perforation was raised. Subsequen t contrast study demonstrated a large hiatus/paraesophageal hernia wit h pronounced gastroesophageal reflux (GER). A part of the hernia was p ositioned posterior and to the right of the gastroesophageal junction (GEJ), presumably the location of the nasogastric tube as noted on the initial films. Although characterized by cardiac/aortic abnormalities , NMS can be a difficult diagnosis and should be considered in any inf ant with hiatus/paraesophageal hernia with or without GER. (C) 1997 Wi ley-Liss, Inc.