Journal of Neurogastroenterology and Motility 2020; 26(1): 164-166  https://doi.org/10.5056/jnm19156
Progression of Jackhammer Esophagus to Type III Achalasia and Improvement After Extended Myotomy
Pablo Vázquez García,1* Constanza Ciriza de los Ríos,1 Fernando Canga Rodríguez-Valcárcel,1 and Diego Hernández García-Gallardo2
1 Digestive Motility Unit, Department of Gastroenterology and Hepatology, 12 de Octubre University Hospital, Madrid, Spain; and 2 Esophagus Unit, Department of General and Digestive Surgery, 12 de Octubre University Hospital, Madrid, Spain
Published online: January 30, 2020.
© The Korean Society of Neurogastroenterology and Motility. All rights reserved.

cc This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Introduction

Jackhammer esophagus is manometrically defined as at least 2 swallows with distal contractile integral ≥ 8000 mmHg∙sec∙cm Achalasia is an uncommon primary esophageal motor disorder of unknown etiology characterized by incomplete or absent relaxation of the lower esophageal sphincter and loss of esophageal peristalsis.1 According to Chicago classification version 3.02 there are 3 types of achalasia. Type III or spastic achalasia is characterized by premature contractions with distal contractile integral > 450 mmHg∙sec∙cm with > 20% of swallows. Early recognition of the disease is improved with high-resolution manometry (HRM).

Since its introduction by Inoue et al,3 peroral endoscopic myotomy (POEM) has become a first-line established treatment for achalasia when an expert operator is available.4 It is especially useful in type III achalasia, which is associated with the worst outcome with all the classical treatments.5

Case Report

A 66-year-old patient who presented with a history of daily intermittent esophagus dysphagia for solids and liquids over 15 months associated with food regurgitation, retrosternal pain and weight loss. This clinical condition had only a partial response to medical therapy. Gastroscopy with esophageal biopsies, barium esophagogram and 24-hour pH monitoring did not show any significant pathological findings. Solid state HRM (Manoscan, Medtronic Inc, Minneapolis, MN, USA) with impedance showed a hypercontractile esophagus and impaired relaxation in some of the swallows. Viscous bolus clearance was also impaired (Fig. 1A).

After botulinum toxin injection the retrosternal pain improved but the dysphagia got worse. A new HRM showed a hypertonic lower esophageal sphincter with a lack of relaxation (integrated relaxation pressure > 15 mmHg) with panesophageal pressurization associated and hypercontractile contractions. These changes suggested a progression to a spastic achalasia (Fig. 1B). A thoracic computerized tomography was normal. Surgical myotomy was proposed to the patient as POEM was not available. The myotomy was prolonged according to the HRM findings. A 16 cm Heller myotomy was performed associated to an anti-reflux technique (Toupet).

The symptoms improved, especially the dysphagia. Postsurgical HRM with impedance showed the disappearing of the previous hypercontractile waves preserving normal peristalsis until the upper limit of the myotomy. Also was observed a better relaxation of the gastroesophageal junction, with an integrated relaxation pressure at the high limit of normal because of the anti-reflux surgery. However, an incomplete bolus clearance was observed in all the swallows (Fig. 2).

Discussion

In the literature, there are multiple case reports6,7 and a few prospective studies that have demonstrated progression from certain esophageal motility disorders to achalasia, including jackhammer esophagus. According to the study of Huang et al8 patients with concomitant jackhammer esophagus and impaired EGJ relaxation are more likely to develop achalasia, especially if they present with intermittent bolus clearance in the impedance exam and with dysphagia as the presenting symptom. If these patterns are found, as in our case, more aggressive treatment may be warranted to avoid deterioration of esophageal function. However, more studies are needed to confirm these findings. Spastic disorders of the esophagus seem to have a good response to longer miotomy.9

The underlying mechanism behind this progression is still unknown, but there are some hypotheses that suggest that all esophageal motility disorders represent a spectrum6 rather than a unique and stable disorder, where patients may progress over time from one to another. Therefore, it is very important to follow these patients who were diagnosed with non-achalasia motility disorders to assess for progression or a shift to a different esophageal motility disorder.

It is also important to exclude opioid and ethanol consumption as they can favor the development of esophageal motility disorders such as achalasia type III,10 but our patient refused the consumption of any of these substances.

Figures
Fig. 1. (A) High-resolution manometry (HRM) with impedance initially performed on the patient that shows 2 swallows. Mean distal contractile integral (DCI) is 8882.6 mmHg·sec·cm (> 8000 mmHg·sec·cm) and integrated relaxation pressure (IRP) is 14.8 mmHg (< 15 mmHg). The study is consistent with jackhammer esophagus. Liquid bolus clearance was complete but viscous was incomplete. (B) HRM after treatment with botulinum toxin. DCI persists over 8000 mmHg·sec·cm but IRP is now 33.6 mmHg (> 15 mmHg) and panpressurization appears. These new findings suggest a progression from the previous motor disorder to type III achalasia.

Fig. 2. High-resolution manometry performed after extended myotomy. No hypercontractile waves are observed (distal contractile integral < 8000 mmHg·sec·cm), with absence of peristalsis in the myotomy zone that has a length of 16 cm. Integrated relaxation pressure is 14.8 mmHg and bolus clearance is incomplete related to the absent peristalsis below the myotomy.

Footnotes

Financial support: None.

Conflicts of interest: None.

Author contributions: All authors were involved in the clinical management of the patient. Pablo Vázquez prepared the manuscript; and Constanza Ciriza, Fernando Canga, and Diego Hernández supervised and made contributions to the final manuscript.

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