Esophagogastric Fistula Associated with Paraesophageal Hernia in a 5-Year-Old Girl.

Esophagogastric fistula is a rare complication related to severe gastroesophageal reflux disease, previous surgery, or malignancy. We describe an unusual case of esophagogastric fistula associated with paraesophageal hernia in a 5-year-old girl. This is the first report in pediatric patients. Copyright:

INTRODUCTION

Esophagogastric fistula, which may be congenital (duplication) or acquired (gastroesophageal reflux disease, malignancy, or surgical intervention) is rare, especially in pediatric patients. To our knowledge, 18 cases have been reported thus far. In this article, we report a case of esophagogastric fistula associated with paraesophageal hernia in a 5-year-old girl.

CASE REPORT

A 5-year-old girl without a history of surgery presented to our hospital with frequent vomiting. No vomiting episodes were observed until 3 years of age. However, the frequency of vomiting episodes increased incrementally thereafter. At 4 years of age, she vomited every meal she ate. The patient was admitted to our hospital when she was 5 years old because of 10–30 vomiting episodes per day. Her height and body weight were <-2SD. Physical examination was unremarkable. Laboratory studies revealed microcytic anemia. A computed tomography (CT) scan of the thorax and abdomen showed giant hiatus hernia [Figure 1a], and an esophagogram revealed a large sliding hiatus hernia [Figure 1b]. At that time, the paraesophageal hernia was thought to be a sliding hernia. Thus, esophagogastroduodenoscopy (EGD) was not considered. We performed laparoscopic fundoplication. At surgery, the entire fundus of the stomach was incarcerated in the chest. However, we could not pull down the fundus into the abdomen. Furthermore, intestinal perforation was performed by an operative procedure. Accordingly, we converted to an open surgery and performed fundoplication by wrapping stomach to reseal the perforation. On postoperative day 13, esophagogram revealed a residual sliding hiatus hernia [Figure 1c]. However, the frequency of vomiting had reduced, and the patient was discharged on postoperative day 26. At 5 months after the surgery, EGD with esophagogram revealed double lumen esophagus [Figure 2a]; one was the primary esophagus [Figure 2b] and the other was esophagogastric fistula connected to the paraesophageal hernia [Figure 2c]. EGD was inserted into the stomach through paraesophageal hernia from esophagogastric fistula. EGD revealed that the outlet of esophagogastric fistula connected the stomach, which was not duplication. Hence, we determined that esophagogastric fistula was not congenital but acquired. However, we did not perform an EGD preoperatively and could not determine whether esophagogastric fistula was preoperative complication or postoperative complication due to intestinal perforation. The number of vomiting episodes increased, and the patient was transferred to another hospital for the treatment of esophagogastric fistula.

Figure 1

(a) Computed tomography: giant hiatus hernia was observed (white arrow). (b) Preoperative esophagography: a large sliding hiatus hernia was observed (white arrow). (c) Postoperative esophagography: residual sliding hiatus hernia was observed (white arrow). A nasogastric tube inserted before operation was passed through esophagogastric fistula and into the stomach

Figure 2

(a) Esophagogastroduodenoscopy with esophagogram finding: double lumen of esophagus was observed. One was primary esophagus (1) and another was esophagogastric fistula connecting to paraesophageal hernia (2). (b) Esophagogastroduodenoscopy: Lugol staining revealed primary esophagus. (c) Esophagogastroduodenoscopy: Lugol unstained lumen showed esophagogastric fistula connecting to paraesophageal hernia

DISCUSSION

Esophagogastric fistulas have been recognized as a rare, potential complication of gastroesophageal reflux disease including paraesophageal hernia and surgery such as fundoplication. Confirmatory studies include EGD and fluoroscopy such as esophagogram, although recent reports have shown that CT is effective as EGD or fluoroscopy.[1] In our case, preoperative CT and fluoroscopy showed the potential presence of an esophagogastric fistula. However, we were not aware that esophagogastric fistula could be a potential complication of paraesophageal hernia. As a result, we not only missed esophagogastric fistula on preoperative CT and fluoroscopy but also lacked to recognize the necessity of EGD. Thus, it is recommended that endoscopic evaluation with EGD should be done for exclusion of paraesophageal hernia before surgery.[2] If esophagogastric fistula was recognized, a biopsy should be considered to exclude duplication. Furthermore, gastroesophageal reflux is the factor of redding, ulceration, or pseudopolyp in larynx. It is recommended that laryngoscopy should be considered to evaluate the larynx before surgery.

Twelve cases of esophagogastric fistula after fundoplication have been reported. Pathogenetic causes include residual reflux disease with ulcerations and erosions, migration of the wrap, intraoperative injuries, ischemic necrosis, tissue reaction with used sutures or Teflon pledgets, and local existing malignancy or prior surgery.[3] On the other hand, esophagogastric fistula without a history of previous surgery has been reported in six cases.[1] Pathogenetic causes include a prolonged untreated reflux disease with ulcerations and erosions and local existing diseases such as esophageal tumor or Crohn's disease.[1] These reports suggest that, to reduce the risk of esophagogastric fistula, we need to evaluate for tumors and ulcers preoperatively, avoid intestinal injuries intraoperatively, and check for residual of reflux and recurrent hernia postoperatively.

There are no clear guidelines for the treatment of esophagogastric fistula. Whether preoperative or postoperative esophagogastric fistula, conservative treatment with acid suppression is performed first.[1] If conservative treatment relieves reflux symptoms, follow-up EGD for surveillance should be performed. If conservative treatment does not relieve reflux symptoms, the management is solely reliant on medication along with surgical intervention. Surgical intervention can be in the form of endoscopic therapy or operation. Endoscopic dilations or closures of small fistulas from esophagogastric junction surgeries are performed using endoscopic suturing devices, stents, clips, and Argon plasma coagulation followed by the injection of histoacryl. These procedures have been reported with varied success rates.[134] Past reports suggest that endoscopic therapy is the choice of treatment treating small fistulas. Attempted operations included partial esophagectomy, resection and primary closure, thoracotomy with transgastric division, laparoscopic gastroplasty, and laparoscopic transgastric gastroplasty using a stapler.[5]

In our case, the assumption that esophagogastric fistula associated with paraesophageal hernia was very rare in pediatric patients lead not to diagnosis esophagogastric fistula preoperatively. Hence, we report this case as a reminder to avoid such assumption in the future.

CONCLUSION

Esophagogastric fistula associated with paraesophageal hernia is very rare in pediatric patients. We suggest that EGD should be performed to check for esophagogastric fistula before treating a paraesophageal hernia in pediatric patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.