Surgical Management of Hiatal Hernia in Children with Asplenia Syndrome.

Purpose  Patients with asplenia syndrome (AS) are likely to have upper gastrointestinal tract malformations such as hiatal hernia. This report discusses the treatment of such conditions. Methods  Seventy-five patients with AS underwent initial palliation in our institution between 1997 and 2013. Of these, 10 patients had hiatal hernia. Of the patients with hiatal hernia, 6 had brachyesophagus and 7 had microgastria. Results  Of the 10 patients with hiatal hernia, 9 underwent surgery in infancy (7 before Glenn operation, 2 after Glenn operation). Two underwent typical Toupet fundoplication, and the other 7 underwent atypical repair including reduction of the stomach. Two patients with atypical repair showed recurrence of hernia and required reoperation. Three patients required reoperation due to duodenal obstruction. Duodenal obstruction occurred due to preduodenal portal vein or abnormal vessels compressing the duodenum. Obstructive symptoms were not seen in any cases preoperatively. Conclusions  In patients with hiatal hernia, typical fundoplication is often difficult because most have concomitant brachyesophagus, microgastria, and hypoplasia of the esophageal hiatus. However, we should at least reduce the stomach to the abdominal cavity as early as possible to increase thoracic cavity volume and allow good feeding. Increasing the volume of the thoracic cavity thus makes Glenn and Fontan circulations more stable. Duodenal obstruction secondary to vascular anomalies is also common, so the anatomy in the area near the duodenum should be evaluated pre- and intraoperatively.