S4411 Where the Jackhammer Meets the Corkscrew: A Case of Manometric Hypercontractile Esophagus and Esophagogastric Junction Outflow Obstruction With Endoscopic Features of Distal Esophageal Spasm

Introduction: Esophageal peristaltic disorders with normal esophagogastric junction relaxation, as defined by the Chicago Classification v4.0, include hypercontractile (Jackhammer esophagus, HE) and distal esophageal spasm (DES). These are distinguished by the presence of ≥20% hypercontractile versus premature (DES) swallows. Esophagogastric junction outflow obstruction (EGJOO) is characterized by preserved peristalsis but impaired lower esophageal sphincter (LES) relaxation. We present a unique case of HE with concomitant EGJOO on high-resolution manometry (HRM), exhibiting endoscopic features typical of DES. Case Description/Methods: A 70-year-old woman with a history of Parkinson’s disease and diabetes mellitus presented with a 3-month history of intermittent solid food dysphagia and heartburn. Initial barium esophagram revealed tertiary contractions in the mid to distal esophagus. Esophagogastroduodenoscopy (EGD) with pH monitoring showed no structural abnormalities and acid exposure was not correlated with symptoms. A subsequent EGD with HRM revealed elevated LES pressures, 40% hypercontractile swallows, and abnormal LES relaxation. To further evaluate her esophageal dysmotility, a repeat EGD with EndoFLIP™ panometry was performed, demonstrating esophageal spasticity and multiple extra peristaltic contractions producing a classic “corkscrew”. The patient was diagnosed with HE with EGJOO and initiated on calcium channel blockers. She was referred for per-oral endoscopic myotomy for further management. Discussion: Esophageal motility disorders often present with overlapping symptoms including chest pain, regurgitation and dysphagia. While DES typically produces a “corkscrew” appearance on both barium esophagram and EGD, these findings are uncommon in HE. Interestingly, our patient’s barium study was non-specific, but endoscopic imaging revealed a striking corkscrew morphology, more characteristic of DES. HE with EGJOO is increasingly recognized as a distinct but rare manometric pattern with an unclear pathophysiologic basis. HRM and EndoFLIP™ remain essential tools in confirming diagnosis and guiding therapy. This case highlights the diagnostic complexity and clinical overlap between HE, DES and EGJOO, emphasizing the importance of integrating clinical, endoscopic, and manometric findings to tailor management effectively.

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Publication Details

Journal
The American Journal of Gastroenterology
Published
2025-10-01
DOI
https://doi.org/10.14309/01.ajg.0001145104.35745.33
Primary Topic
Gastroesophageal reflux and treatments
Type
article
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article

S4411 Where the Jackhammer Meets the Corkscrew: A Case of Manometric Hypercontractile Esophagus and Esophagogastric Junction Outflow Obstruction With Endoscopic Features of Distal Esophageal Spasm

Mariam Naveed, Irteza Inayat, Nihal I. Khan, Ernesto Robalino Gonzaga
The American Journal of Gastroenterology
Gastroesophageal reflux and treatments
article

S4411 Where the Jackhammer Meets the Corkscrew: A Case of Manometric Hypercontractile Esophagus and Esophagogastric Junction Outflow Obstruction With Endoscopic Features of Distal Esophageal Spasm

Mariam Naveed, Irteza Inayat, Nihal I. Khan, Ernesto Robalino Gonzaga
article en

Abstract

Introduction: Esophageal peristaltic disorders with normal esophagogastric junction relaxation, as defined by the Chicago Classification v4.0, include hypercontractile (Jackhammer esophagus, HE) and distal esophageal spasm (DES). These are distinguished by the presence of ≥20% hypercontractile versus premature (DES) swallows. Esophagogastric junction outflow obstruction (EGJOO) is characterized by preserved peristalsis but impaired lower esophageal sphincter (LES) relaxation. We present a unique case of HE with concomitant EGJOO on high-resolution manometry (HRM), exhibiting endoscopic features typical of DES. Case Description/Methods: A 70-year-old woman with a history of Parkinson’s disease and diabetes mellitus presented with a 3-month history of intermittent solid food dysphagia and heartburn. Initial barium esophagram revealed tertiary contractions in the mid to distal esophagus. Esophagogastroduodenoscopy (EGD) with pH monitoring showed no structural abnormalities and acid exposure was not correlated with symptoms. A subsequent EGD with HRM revealed elevated LES pressures, 40% hypercontractile swallows, and abnormal LES relaxation. To further evaluate her esophageal dysmotility, a repeat EGD with EndoFLIP™ panometry was performed, demonstrating esophageal spasticity and multiple extra peristaltic contractions producing a classic “corkscrew”. The patient was diagnosed with HE with EGJOO and initiated on calcium channel blockers. She was referred for per-oral endoscopic myotomy for further management. Discussion: Esophageal motility disorders often present with overlapping symptoms including chest pain, regurgitation and dysphagia. While DES typically produces a “corkscrew” appearance on both barium esophagram and EGD, these findings are uncommon in HE. Interestingly, our patient’s barium study was non-specific, but endoscopic imaging revealed a striking corkscrew morphology, more characteristic of DES. HE with EGJOO is increasingly recognized as a distinct but rare manometric pattern with an unclear pathophysiologic basis. HRM and EndoFLIP™ remain essential tools in confirming diagnosis and guiding therapy. This case highlights the diagnostic complexity and clinical overlap between HE, DES and EGJOO, emphasizing the importance of integrating clinical, endoscopic, and manometric findings to tailor management effectively.

The American Journal of GastroenterologyVol. 120(10S2)
Openalex Percentile: Top 53%
Gastroesophageal reflux and treatments
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