INTRODUCTION

The jackhammer esophagus is a rare hypercontractile disorder clinically characterized by the presence of recurrent retroesternal pain or dysphagia ¹. The diagnosis is based on high-resolution manometry (HRM) defined by distal contractile integrals (DCI) greater than 8,000 mmHg/s/cm in at least 20% of the evaluated swallows. Despite the physiopathological differences with regard to achalasia, similar therapeutic options have been suggested ^2,3. Peroral endoscopic myotomy (POEM) of the spastic esophagus segments has been described ^4,5,6. Here, we report a pediatric patient with jackhammer esophagus that was treated endoscopically.

CASE REPORT

A 14-year-old female patient, 155 centimeters in height, 43 kg in weight (percentile 25), referred to our clinic due to retroesternal pain associated with ingestion and a 5-year evolution of dysphagia. Both gastroesophageal and eosinophilic esophagitis were ruled out; the former via a 24-hour pH-impedance test and the latter, by endoscopic biopsies of the three esophageal thirds after treatment with proton pump inhibitors.

A HRM was performed and increased esophageal contractions were observed in each of the ten liquid and solid swallows studied. Peristalsis was preserved, with an average DCI of 27,000 mmHg/s/cm and a proper relaxation of the lower esophageal sphincter (LES) (Fig. 1).

Fig. 1 HRM: increased esophageal contraction. DCI 23,000 mmHg/s/cm. 

An endoscopic therapeutic attempt was made via the helical injection of botulinum toxin in the esophageal body. However, there was no response. Inoue and other authors suggest performing a POEM of the spastic segments of the esophagus as a therapeutic option in patients with jackhammer esophagus ^7,8. Thus, a POEM of these segments was performed after an antibiotic prophylaxis that was previously determined by the HRM whilst taking care of the LES. There were no complications during the procedure. A liquid diet was started 24 hours later and the patient was discharged on the third day (Fig. 2).

Fig. 2 POEM. A. Mucosal incision to initiate submucosal tunneling. B. Panoramic view of the submucosal tunnel with a completed longitudinal myotomy. C. Mucosal incision closure with clips. 

Four months later, the patient was asymptomatic. A control HRM showed the absence of contractions in the smooth muscle with an average DCI of 1,180 mmHg/s/cm (Fig. 3).

Fig. 3 HRM: no contraction of the smooth muscle after endoscopic treatment. 

DISCUSSION

The manometric criteria for the hypercontractile esophagus varied over time. It started with the "nutcracker esophagus", defined via conventional manometry by a distal wave amplitude of ≥ 180 mmHg with preserved peristalsis. Afterwards, this value was increased to 220 mmHg in order to improve the diagnostic specificity.

With the introduction of HRM, a new classification of the motor disorders and new metric units have been incorporated (Chicago International Classification). The DCI allows for a better measurement of the activity of the smooth muscle and the vigor of the contraction. The Chicago International Classification (version 3.0) ⁹ defines jackhammer esophagus as the presence of hypertensive waves with more than 8,000 mmHg/s/cm in at least 20% of the evaluated swallows. However, this characteristic is not exclusive and can be found in patients with gastroesophageal reflux, outflow obstruction and primary hyper-contractility. The diagnostic criteria for Jackhammer esophagus are the same for the pediatric population ¹⁰.

Here, we describe the successful endoscopic treatment of a pediatric patient with jackhammer esophagus. It is necessary to perform prospective and randomized studies to confirm these findings.