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Revista chilena de pediatría

Print version ISSN 0370-4106

Rev. chil. pediatr. vol.90 no.1 Santiago  2019  Epub Jan 21, 2019

http://dx.doi.org/10.32641/rchped.v90i1.884 

CLINICAL CASE

Per oral endoscopic myotomy in a pediatric patient with achalasia

Ricardo Mejía1  2 

Josefina Sáez3 

Francisco Aranda4 

Juan Carlos Pattillo5 

José Fernando Vuletin5 

Daniela Gattini6 

María Francisca Jaime6 

Allan Sharp1 

1 Gastrointestinal Surgery Department, Division of Surgery, Faculty of Medicine, Pontificia Universidad Católica de Chile, Chile.

2 Gastrointestinal Surgery, Dr Sótero del Río Hospital, Pontificia Universidad Católica de Chile, Chile.

3 General Surgery Resident, Faculty of Medicine, Pontificia Universidad Católica de Chile, Chile.

4 Intern of Medicine, Faculty of Medicine, Pontificia Universidad Católica de Chile, Chile.

5 Pediatric Surgery, Division of Surgery, Faculty of Medicine, Pontificia Universidad Católica de Chile, Chile.

6 Gastroenterology, Hepatology and Pediatric Nutrition Department, Division of Pediatrics, Faculty of Medicine Pontificia Universidad Católica de chile, Chile.

Abstract:

Introduction:

Achalasia is the most common primary motor disorder of the esophagus. Its reported incidence is low, even more in pediatric patients. Laparoscopic Heller myotomy is the current stan dard of treatment. During the last years, per-oral endoscopic myotomy (POEM) has been positioned as a safe and effective therapeutic alternative as the Heller procedure for esophageal achalasia.

Ob jective:

To describe the POEM technique and report the first pediatric case in our country.

Clinical Case:

11-year-old patient, previously healthy, who presented with progressive dysphagia for solids and liquids and weight loss. The study concluded a type II achalasia. The patient underwent a POEM and had a postoperative course without incidents. One year after the intervention, symptomatic, endoscopic and manometric resolution have been documented.

Conclusions:

The described case is the first POEM in a pediatric patient in our country. Esophageal achalasia is uncommon in pediatrics and POEM has demonstrated clinical success and safety comparable to laparoscopic Heller myotomy in short and medium term. Long-term follow-up will determine its definitive role in the treatment of pediatric patients with esophageal achalasia.

Keywords: Esophageal achalasia; POEM; Heller myotomy; therapeutic endoscopy

Introduction

Achalasia is the most common primary esophageal motor disorder. It is a rare disease characterized by the absence of esophageal peristalsis and incomplete or ab sent lower esophageal sphincter relaxation1.

Its reported incidence is low, in pediatric patients it is estimated annually at 0.11 per 100,000 inhabitants, without predilection by race or gender2 and the avera ge age at diagnosis is 10 years3.

The most common symptoms are dysphagia, re gurgitation, retrosternal pain, and vomiting after fe eding4. It can be associated with weight loss, failure to thrive4, and even appear atypically with cough or recurrent pulmonary infections secondary to aspiration5.

Partly because of the limited number of pediatric patients with achalasia, the standard of treatment has been controversial3. The traditional management in cludes endoscopic balloon dilation and laparoscopic Heller myotomy with or without partial fundoplication6. The botulinum toxin injection, use of calcium channel blockers or long-acting nitrates are less effecti ve therapies in the long term and are probably conside red as a bridge to surgery or palliation in those patients that definitive surgical management is contraindica ted7.

In 2007, Pasricha described the endoscopic myo tomy technique in a porcine model8, and in 2010, Inoue reported the first results in humans9. Since then, the POEM (peroral endoscopic myotomy) has been installed as a new therapeutic alternative in the acha lasia management7. The accumulated experience in adults has shown results that position it as a safe tech nique and as effective as Heller’s myotomy. The pro cedure has been gradually introduced in the pediatric population, with initial results7.

The objective of this report is to describe the first case, in our country, of a pediatric patient with achala sia undergoing POEM and his follow-up to date.

Clinical case

11-year-old male patient with history of allergic rhinitis and atopic dermatitis, eutrophic and with good psychomotor development. He consulted on an outpatient health center due to two-month progressi ve dysphagia for solids and liquids, without vomiting, heartburn or regurgitation. He has lost four kg in the last four months, without abdominal pain.

As part of the diagnostic study, an upper endoscopy was performed which was within normal limits and the esophageal biopsy was reported without alterations.

An esophageal contrast study was requested, which identified findings compatible with esophageal achala sia (Figure 1). It was complemented with a high-reso lution esophageal manometry, whose report described an integrated relaxation pressure median of 36 mmHg (VN < 15 mmHg), panesophageal pressurization phe nomena after multiple swallows and esophageal ape- ristalsis, consistent with type II achalasia according to the modified Chicago classification (Figure 2).

Figure 1 Preoperative esophageal contrast study. A short segment stenosis with the characteristic morphology of a bird's beak, dilation of the esophagus in its middle third and filiform passage of the contrast to the gastric chamber is evident at the gastroesophageal junction. 

Figure 2 High resolution esophageal manometry. The absence of relaxation of the gastroesophageal junction (demarcated area) and panpresuri- zation phenomena (arrows) can be identified in protocolized swallows. 

Once the diagnosis was confirmed, the case was discussed in a multidisciplinary team and it was de cided, after agreement with the parents, to resolve it by endoscopy using POEM. The preoperative Eckardt score10 was 10 points (dysphagia 3 points, retrosternal pain 3 points, regurgitation 3 points, and weight loss 1 point).

The procedure was carried out in the operating room, under general anesthesia, and in the supine po sition. A diagnostic endoscope with a hood, a CO2 in sufflator, and an ERBE® electrosurgical unit was used. The initial endoscopy showed food content in the esophagus, which was aspirated prior to initiating the intervention. The gastroesophageal junction (GEJ) was 38 centimeters from the dental arch.

An elevation of the mucosa was performed with a Voluven® and indigo carmine solution, then a longitu dinal mucotomy was performed in the anterior face of the esophagus between the two and three o’clock posi tions (Figures 3A and 3B), approximately 11 centime ters proximal to the GEJ. Subsequently, a submucosal tunnel (Figure 3C) was created using a submucosal dis section technique and an endoscopic Flush Knife BT® of 2.5 mm, extending 41 centimeters to the dental arch. The distal extension to the GEJ was verified through endoscopic retroflexion in the gastric area to observe the subendocardial submucosa staining, added to the endoscopic measurement, identification of the oblique muscle fibers of the stomach and the vessels of the gas tric submucosa.

Figure 3 POEM technique. (A) Elevation of the mucosa. (B) Longitudinal mucotomy. (C) Creation of the submucosal tunnel. (D) Myotomy of the internal circular fibers of the esophagus. (E) Mucotomy closure with clips. 

The myotomy of the internal circular fibers of the esophageal muscle was performed with a triangle tip electrosurgical knife (TT Knife®), from 30 to 41 cen timeters from the dental arch (Figure 3D). Hemostasis was then assured and the tunnel was instilled with a gentamicin solution. The mucotomy was closed with endoscopic clips (Figure 3E) and the final stage ensured easy passage of the instrument through the GEJ.

Complete fasting was kept until an esophageal stu dy with water-soluble contrast was performed within the first 24 hours, which ruled out filtration (Figure 4). The patient was then fed with a liquid diet, with good tolerance, and was discharged on the second day, with indication of proton pump inhibitor (PPI) in two daily doses.

Figure 4 Postoperative esophageal contrast study clips are ob served in the distal third of the esophagus, with contrast passage to the stomach. The area of obstruction previously described in the gastroesophageal junction is no longer visible and no extra luminal contrast, suggestive of filtration, is identified. 

The patient was controlled one week after the in tervention, observing the resolution of symptoms and good diet tolerance. He started eating mash and it was indicated that other foods should be progressively in corporated.

Three months after the surgery, he was controlled with an upper endoscopy, which showed a good passa ge of the instrument through the GEJ, absence of poor esophageal emptying or evidence of gastroesophageal reflux (GER). The high-resolution esophageal mano metry showed no esophageal pressurization phenome na and an integrated relaxation pressure median of 15 mmHg. The patient remains asymptomatic, with no GER symptoms or PPI requirement. The upper en doscopy performed after one year does not show any changes compared to the previous one. His Eckardt score at follow-up is one (occasional dysphagia).

Discussion

Esophageal achalasia is a rare diagnosis in pediatrics11. Available treatments include pharmacological, endoscopic, and surgical alternatives, the most com monly used is the endoscopic balloon dilation and la paroscopic Heller’s myotomy. Between the latter two, the superiority of Heller’s myotomy is recognized be cause it is related to lower recurrence rates than ba lloon dilation2. Currently, optimal management in pe diatric patients has not been defined and the literature still lacks standardized follow-up protocols4.

POEM has emerged as a competitive therapeutic alternative in the treatment of achalasia over the last decade because it combines the benefits of an endos copic and, therefore, minimally invasive procedure with the surgical myotomy efficacy2. Some authors consider in its favor that it avoids excessive dissection of the esophageal hiatus, scars and allows reintegration into activity in a few days12,13, in addition to planning the length of myotomy according to the manometric and endoscopic study2. The available follow-up stu dies report clinical success rates (Eckardt < 3) higher than 90% and improvement in the pressure profile of esophageal manometry13-15. In recent years, series have been published in pediatric patients, with good results in the resolution of symptoms, registering up to 100% at 24.6 months of the procedure, without serious perioperative complications (Clavien >III) or medium- term follow-up11.

The technique has a good safety profile; capnoperi- toneum and submucosal emphysema are unavoidable consequences and can be easily managed with intra procedure if they are clinically significant9,16.

The available data are not yet sufficient to establish recommendations for the technique application accor ding to weight and age limits2. The youngest patient re ported corresponds to the case published by Maselli et al., a three-year-old patient with trisomy 21 and severe malnutrition secondary to esophageal achalasia treated with POEM with success17.

It is important to recognize the learning curve as sociated with the procedure execution, which has been established in about 20 cases18; the best results are ob tained in centers with experience in therapeutic endoscopy11.

Currently, it is considered that all patients with achalasia can be treated with POEM, and it has even been established as a therapeutic alternative in other esophageal motor disorders such as diffuse esophageal spasm, nutcracker esophagus, and hypercontractile esophagus (jackhammer)( 11 ). It would be the treatment of choice in type III achalasia because it allows myoto mies of longer length and in patients with symptoma tic relapse after Heller’s myotomy or after a first POEM because it provides the alternative of varying the ap proach avoiding fibrosis zones2.

While laparoscopic Heller’s myotomy is currently the treatment of choice in children, POEM is increa singly used as a therapeutic alternative. It has promi sing results from the point of view of effectiveness and safety in pediatrics, and this report would constitute, to our knowledge, the first case made in the country. The results of the technique must be supported in mul ti-center studies of greater volume and with long-term follow-up, in order to constitute it as the alternative of choice in this population.

Ethical Responsibilities

Human Beings and animals protection: Disclosure the authors state that the procedures were followed ac cording to the Declaration of Helsinki and the World Medical Association regarding human experimenta tion developed for the medical community.

Data confidentiality: The authors state that they have followed the protocols of their Center and Local regu lations on the publication of patient data.

Rights to privacy and informed consent: The authors have obtained the informed consent of the patients and/or subjects referred to in the article. This docu ment is in the possession of the correspondence author.

Financial Disclosure: Authors state that no economic support has been asso ciated with the present study.

Conflicts of Interest: Authors declare no conflict of interest regarding the present study.

Referencias:

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Received: September 20, 2018; Accepted: November 15, 2018

Correspondence: ricardo Mejía Martínez. E-mail: rjmejiam@gmail.com.

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