Functional esophagogastric junction obstruction with intact peristalsis: a heterogeneous syndrome sometimes akin to achalasia

Abstract

Background: Some patients with suspected achalasia are found on manometry to have preserved peristalsis, thereby excluding that diagnosis. This study evaluated a series of such patients with functional esophagogastric junction (EGJ) obstruction.

Methods: Among 1,000 consecutive high-resolution manometry studies, 16 patients had functional EGJ obstruction characterized by impaired EGJ relaxation and intact peristalsis. Eight patients with post-fundoplication dysphagia and similarly impaired EGJ relaxation were studied as a comparator group with mechanical obstruction. Intrabolus pressure (IBP) was measured 1 cm proximal to the EGJ. Sixty-eight normal controls were used to define normal IBP. Patients' clinical features were evaluated.

Results: Functional EGJ obstruction patients presented with dysphagia (96%) and/or chest pain (42%). IBP was significantly elevated in idiopathic and post-fundoplication dysphagia patients versus controls. Among the idiopathic EGJ obstruction group treated with pneumatic dilation, BoTox(TM), or Heller myotomy, only the three treated with Heller myotomy responded well. Among the post-fundoplication dysphagia patients, three of four responded well to redo operations.

Conclusion: Functional EGJ obstruction is characterized by pressure topography metrics demonstrating EGJ outflow obstruction of magnitude comparable to that seen with post-fundoplication dysphagia. Affected patients experience dysphagia and/or chest pain. In some cases, functional EGJ obstruction may represent an incomplete achalasia syndrome.

Figure 1

Representative example of a patient with functional EGJ obstruction. Max-IBP (yellow) is the greatest IBP obtained for a contiguous or non-contiguous 3-s period within the same 10-s temporal boundary used to calculate the IRP (white). This patient later responded favorably to Heller myotomy. The IRP is calculated with ManoView™. Pressure is referenced to atmospheric with the 30 mmHg isobaric contour highlighted in black.

Figure 2

High resolution esophageal pressure topography study consistent with classic achalasia exhibiting aperistalsis and impaired EGJ relaxation. Compare this study to that in Fig. 1 consistent with functional EGJ obstruction exhibiting intact peristalsis and impaired EGJ relaxation. The IRP is calculated with ManoView™ in both examples. In this example, pressure is referenced to intragastric pressure and the 15 mmHg isobaric contour is highlighted in black.

Figure 3

Comparison of IRP, max-IBP, and max-IBP (worst 3) among subject groups. All of these manometric measures are significantly elevated in functional EGJ obstruction compared with controls. Among them, max-IBP (worst 3) best discriminated functional EGJ obstruction from controls.

Figure 4

High-resolution esophageal pressure topography (top) and landscape (bottom) plots of a hiatus hernia patient with functional EGJ obstruction attributable to the CD (left) and another hiatus hernia patient with EGJ functional obstruction attributable to the LES (right). In each case, the corresponding IRP_CD and IRP_LES values are shown.