A Neonatal Case of Paraesophageal Mixed Hiatal Hernia Detected by "Coiled-Up Sign"

Paraesophageal mixed hiatal hernia is a rare entity in neonates, that occasionally induces near-complete esophageal or gastric obstruction, and its diagnosis is sometimes difficult. Furthermore, its management remains controversial. This case provides new insights into the diagnosis and treatment strategies of neonatal paraesophageal mixed hiatal hernias.

The case was a 4-day-old female infant who had frequent emesis after feeding. Plain radiography showed a "coiled-up sign" of an orogastric tube at the level of the diaphragm. CT indicated that the upper half of the stomach protruded into the mediastinal space. In the upper gastrointestinal study, the abdominal esophagus and cardia were compressed and bent, causing the contrast material to stagnate. Thus, she was diagnosed with paraesophageal mixed hiatal hernia. The enteral tube was advanced to the level of the jejunum by insufflating the stomach and returning it to the abdominal cavity under fluoroscopy. After feeding via an enteral tube during the neonatal period, the patient underwent radical surgery at 37 days old. We performed laparoscopic procedures involving pulling the stomach down, approximating the widened hiatus, and Toupet fundoplication to prevent postoperative gastroesophageal reflux.

The "coiled-up sign" of the gastric tube at the level of the diaphragm in neonates suspected with upper gastrointestinal obstruction or stenosis should raise suspicion of a paraesophageal mixed hiatal hernia and an upper gastrointestinal contrast study is useful for the diagnosis. Insertion of a feeding tube may allow for elective radical surgery while avoiding life-threatening complications.