Dysphagia is a frequent complication in patients that undergo fundoplication, and according to some authors, can present in up to 76% of cases, normally resolving within the first postoperative weeks. Dysphagia after said procedure is reported to present in adults at two years in 7.5% of cases and at five years in 5%.1 In the pediatric patient, severe persistent dysphagia can occur in 5.3% of cases.2 According to Sobrino-Cossio et al.,1 post-fundoplication dysphagia may be due to an undiagnosed pre-existing condition, such as insufficiently effective esophageal motility, esophageal aperistalsis, etcetera, or to a structural or functional alteration, such as the currently well-described esophagogastric junction outflow obstruction (EGJOO).

EGJOO is a motility disorder that generally presents as a consequence of fundoplication, and according to the Chicago v3.0 classification (CC V3.0), is defined as an elevated integrated relaxation pressure (IRP) with a conserved peristalsis pattern, thus not meeting the criteria for achalasia.3 Some authors refer to it as an early or incomplete manifestation of achalasia4–6 and others have associated it with mechanical disorders, such as eosinophilic esophagitis, hiatal hernia, stricture, Schatzki rings, tumors, or structural alterations derived from fundoplication or bariatric surgery. Therefore, those diagnostic possibilities should always be ruled out, and not be classified as a functional problem.7–11

Clinical presentation is varied and dependent on the cause. Dysphagia is the most common symptom in up to 57% of cases.8 Diagnosis is made through manometry (preferably high-resolution manometry). Likewise, imaging studies, such as esophagram, can be useful, as well as upper gastrointestinal endoscopy and transendoscopic ultrasound.7,8

Different studies report that up to 52% of patients have spontaneous symptom resolution, with no need for specific treatment.4

At present, there are no studies reporting the progression and treatment of pediatric patients with EJGOO. The aim of our study was to objectively describe the clinical and manometric characteristics, as well as the possibility of treatment, in children with that disorder.

Of the total of 213 HREMs carried out within the period of September 2015 to September 2019 at the Physiology and Motility Unit of the Gastroenterology Service of the Instituto Nacional de Pediatría, four fit the diagnosis of EGJOO (CC v3.0), with a mean IRP of 24 (24.25 ± 3.90 mmHg) (Table 1) (Fig. 1A-D). Within that time frame, 143 fundoplications were performed by the pediatric surgery service. Nissen fundoplication was performed in three patients and one patient underwent Toupet fundoplication. All the fundoplications were open surgeries.

Figure 1.

Esophageal manometry of the patients with esophagogastric junction outflow obstruction.

Patients with a history of post-fundoplication dysphagia. High-resolution manometry was consistent with esopgagogastric junction outflow obstruction. Mean relaxation pressure was 24 mmHg, peristalsis was normal in all cases.

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Three of the study patients had a history of chronic pneumopathy and regurgitation symptoms. Given the persistence of the respiratory and digestive symptomatology, they underwent fundoplication. The fourth study patient underwent fundoplication during left diaphragmatic hernia correction. Mean patient age at diagnosis was 8 ± 3.3 years, and 3 of the patients were males.

The four patients presented with dysphagia after the surgical procedure, at a mean of 2 weeks (2 ± 1.2 weeks). Therefore, the first diagnostic test carried out was an esophagram, which revealed stricture of the esophagogastric junction (EGJ) in all four cases (Fig. 2A-D). The patients then underwent endoscopy. In two patients, passage of the endoscope was difficult, there was angulation at the EGJ in one patient, and endoscopy was normal in one patient.

Figure 2.

Esophagram of the patients after fundoplication.

A) Case 1, overdistension of the esophageal lumen upon the passage of barium. The esophagogastric junction (EGJ) is seen under the diaphragm; B) Case 2, narrowness of the EGJ; C) Case 3, the esophagus is slightly overdistended in its three thirds, with thinning of the EGJ; D) Case 4, esophagus with conserved caliber in the first two thirds, but greatly augmented in the distal third, blunt tip, and even a rounded image, and reduced caliber of the EGJ, probably overefficiency of the fundoplication.

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The primary symptom complaints were dysphagia, chest pain, and vomiting (Fig. 3). Initial treatment was dilation in the four patients. Balloon dilation was performed by the surgery service and endoscopic dilation (Savary-Miller) by the gastroenterology service in one patient, and in the remaining three cases, dilation was exclusively carried out with the Savary-Miller probe. None of the patients had improvement at 6 months after treatment. In the first patient, two series of dilations were carried out, followed by fundoplication repair with a Heller myotomy, with no improvement. In the second, third, and fourth patients, two, one, and four series of dilations were performed, respectively.

Figure 3.

Associated esophageal symptoms in patients diagnosed with esophagogastric junction outflow obstruction (EGJOO).

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Given the persistence of the dysphagia, watchful management with prokinetics was decided upon, monitoring patient weight and height. Three of the patients had spontaneous symptom improvement but one patient remained symptomatic (Table 2). Mean favorable esophageal symptomatology response was 31 months (mean: 31 ± 8.48 months). Manometry was performed at a mean of 19 post-fundoplication months (mean: 19.45 ± 20 months). Control manometry was only possible in the patient that underwent fundoplication repair, and it showed LES relaxation, one year from the onset of clinical manifestations.

Since its description in 1956, fundoplication has been a therapeutic option in the management of medical treatment-refractory reflux.10 Given its complexity, secondary postoperative effects, such as dysphagia, can be a problem. Transitory dysphagia occurs within the first weeks in up to 76% of the cases, and severe or recurrent dysphagia can occur in up to 5.3% of pediatric cases.1,2 At our center, we found that 2.8% of patients developed post-fundoplication dysphagia but we do not know if all the patients with that symptomatology were referred to the motility unit for their evaluation. In our study, symptoms presented within the first two weeks after the procedure and persisted for more than four weeks. Studies on adults have reported that transitory dysphagia can occur up to the first six weeks after the procedure and is considered persistent, if it lasts for a longer period of time.12

EGJOO is a relatively new diagnosis,4 and according to the Chicago classification, is defined by an elevated IRP and conserved esophageal peristalsis.3,13–15 In an adult population, Wilshire et al.12 found that up to 64% of patients with post-fundoplication dysphagia had evidence of EGJOO that was demonstrated manometrically by elevated IRP. Very little is known about the pathology in pediatric patients and the published studies are limited to adult populations. Different authors have reported a prevalence of EGJOO that ranges from 3.3-14%, figures very close to ours (2%).16–18

In the present study, we identified four patients with EGJOO secondary to fundoplication, diagnosed utilizing HREM and finding elevated IRP (mean: 24.25 ± 3.90 mmHg). Mean patient age was eight years, with a male to female ratio of 3:1, unlike the adult population, in which two-thirds of the patients are women.13,19

As far as we could tell from our review of the literature, the most frequent manifestation is dysphagia (57-96%),8,20 usually associated with obstructive cases (42%), such as hiatal hernia, esophageal stricture, cancer, and having undergone surgery. Surgery is the associated event found in half of the patients with EGJOO.15

In our study, 75% of the patients had undergone Nissen fundoplication, but previous studies make no mention of an association between the type of fundoplication performed and the presence of the motility disorder.20–22

All the patients underwent an imaging study (esophagram) and upper gastrointestinal endoscopy, in the search for gastrointestinal symptom etiology. All cases presented with stricture at the level of the EGJ (one of the endoscopies was reported as normal), which is why dilation was performed as the initial treatment, but the response was unfavorable, given that the dysphagia persisted in the four patients. The success rates reported in the literature are varied. Okeke et al.8 found a favorable response in 12%, whereas Richter and Clayton reported response in 67% of the cases after pneumatic balloon dilation.11

The patient that underwent fundoplication repair plus Heller myotomy did not improve after the intervention, unlike that reported by Scherer et al. Those authors found symptom improvement in 75% of the patients that underwent fundoplication repair and response in 100%20,22 of the patients that had Heller myotomy. Peroral endoscopic myotomy (POEM) has recently been utilized in esophageal motility disorders, including EGJOO, with remission rates of 60%.23

On the other hand, 33-73% of patients have been described to have symptom resolution with no need for any type of intervention.4,13,16 Even though different procedures were carried out on our patients, no improvement was seen after six months of follow-up. Therefore, endoscopic or surgical management was not continued and 75% of the patients had spontaneous resolution at a mean of 31 months after EGJOO diagnosis.

The limitations of our study were the small number of cases and the lack of treatment homogeneity, given that it was dependent on the treating physician. Nevertheless, because there are very few reports in the international literature, we considered showing the experience at our hospital to be important.

EGJOO is a manometric finding that is described very little in the pediatric population and should be suspected in all patients that present with post-fundoplication dysphagia. Unfortunately, treatment continues to be a challenge. Even though half of the cases resolve with no need for any intervention, there are still important limitations to defining optimum treatment of this motility disorder.

There was no source of funding.

The authors have no conflicts of interest to declare.