Congenital paraesophageal hiatal hernia.

Abstract

A healthy newborn baby with a left cleft lip and palate presented to our institution at 18 hours of life because of persistent emesis after feeds. Initial evaluation with chest radiography and a contrast swallow at the outside facility demonstrated an intrathoracic stomach (A, arrows outline the stomach with retained contrast). A repeat contrast swallow, performed at our institution to help better define the anatomy of the esophagus, stomach, and duodenum revealed a congenital paraesophageal hiatal hernia with an intrathoracic stomach without any evidence of gastric volvulus (B, arrow outlines gastroesophageal junction). The stomach appeared to be herniating through a posterior midline defect in the diaphragm. At laparotomy, a large paraesophageal hiatal hernia with an intrathoracic stomach and hernia sac was identified. The hernia sac was resected, the stomach was returned to its intraabdominal position, and closure of the large hiatal hernia was accomplished by reapproximating the crura using interrupted nonabsorbable sutures. On the second postoperative day, oral feeds were initiated and the baby was advanced to full feeds by the fourth postoperative day. Congenital paraesophageal hiatal hernia is a rare condition in neonates and infants. Clinical presentation is nonspecific and usually consists of persistent emesis or pulmonary infections. Diagnostic workup usually includes chest radiograph with confirmation by a barium swallow study. Once the diagnosis is established, prompt surgical repair is mandatory to avoid potentially lethal complications, such as gastric volvulus and perforation. Operative repair consists of resecting the hernia sac, reduction of the stomach, and repair of the hiatal defect with reapproximation of the crura. Because this is such a rare entity, the addition of a fundoplication, gastropexy, or both remains controversial.