Clinical features and long-term outcomes of lower esophageal sphincter-dependent and lower esophageal sphincter-independent jackhammer esophagus

Background The most recent Chicago Classification expanded the criteria for diagnosis of jackhammer esophagus (JHE) to include the distal contractile integral (DCI) of the lower esophageal sphincter (LES). The clinical impact of the manometric inclusion of LES hypercontractility remains unclear. We aimed to analyze the clinical features and long-term outcomes of measured LES-dependent (LD-JHE) and LES-independent (LI-JHE) jackhammer esophagus. Methods Patients meeting diagnostic criteria for JHE were identified at two academic medical centers. High-resolution esophageal manometry data were re-analyzed with inclusion and exclusion of the LES DCI. LD-JHE was defined by falling outside JHE diagnostic criteria with exclusion of the LES. A telephone survey was conducted for follow-up utilizing the impact dysphagia (IDQ-10) questionnaire. Key Results Eighty-one patients met study inclusion criteria, with 12 (14.8%) classified as LD-JHE. LD-JHE patients had a significantly lower mean DCI and fewer swallows with DCI >8000 mm Hg-s-cm. Basal LES pressure was higher in patients with dysphagia to solids than those with dysphagia to solids and liquids. Clinical and manometric parameters were otherwise similar between groups. Sixty-six patients had clinical or phone follow-up at a median of 46.6 months. Forty-one patients (62.1%) received therapies directed at JHE. There was no difference in symptom improvement for treated vs untreated patients or for JHE subtype. Conclusions and Inferences Our findings suggest that LD-JHE and LI-JHE are clinically indistinguishable and thus support existing diagnostic criteria. Furthermore, our long-term follow-up data suggest that JHE, irrespective of LES involvement, may improve without treatment. Further study is needed to clarify which patients merit therapeutic intervention.