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


CONCLUSIONS
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.
Scherer
John R
JR
Department of Medicine, Division of Gastroenterology, Feinberg School of Medicine, Northwestern University, 676 N St Clair Street, Suite 1400, Chicago, IL 60611, USA.
Kwiatek
Monika A
MA
Soper
Nathanial J
NJ
Pandolfino
John E
JE
Kahrilas
Peter James
PJ
D000328:Adult; D001037:Aphasia / Q000209:etiology / Q000628:therapy; D002637:Chest Pain / Q000209:etiology; D004931:Esophageal Achalasia / Q000503:physiopathology*; D004943:Esophagogastric Junction / Q000503:physiopathology*; D005260:Female; D018662:Fundoplication; D006551:Hernia, Hiatal / Q000503:physiopathology; D006801:Humans; D008297:Male; D008365:Manometry; D008875:Middle Aged; D010528:Peristalsis; D011183:Postoperative Complications / Q000503:physiopathology; D013577:Syndrome