Achalasia of the esophagus in children is a rare disease. In our patient, parents noted a wet pillow in the morning and other typical clinical symptoms of achalasia, such as obstructive bronchitis, recurrent pneumonia, and loss of body weight. It must be kept in mind that similar to Hirschsprung disease, the deficient neurons in achalasia cannot be cured. The aim of treatment is to improve symptoms, to facilitate adequate emptying of the distal esophagus, and to avoid progressive dilation of the proximal part of the esophagus.
Currently, no generally accepted guidelines for the treatment of achalasia of the esophagus in children are available. According to the literature, the operation most frequently conducted is myotomy of the cardia according to Heller, supplemented by one of the variants of fundoplication.[9–13]
However, LHM has evolved as the preferred treatment of esophageal achalasia in children and adults.[6,13] In a review of the literature in patients under 18 years of age, Pandian et al noted that the pediatric LHM experience comprises only retrospective studies suggesting that LHM is safe and effective. Unfortunately, long-term studies of achalasia in children are extremely rare.
Conservative methods of treatment in adult patients are not applicable in children (e.g., administration of calcium-channel blockers) or have only a temporary effect. Endoscopic interventions, that is, balloon dilation of the cardia or injection of botulinum toxin (Botox), can result in scarring of the distal esophagus and occurrence of complications.[9,13] In children, endoscopic pneumatic dilatation (EPD) has been applied for many years with varying rates of success. EPD may result in recurrences of esophageal obstruction necessitating repeated EPD.[14,15] In children suffering from esophageal achalasia, previous EPD and Botox injections are considered risk factors for perforation when performing LHM.[16,17] Mucosal perforation detected during LHM should be repaired immediately using absorbable sutures. In contrast, it seems not very reliable to discern between a full thickness tear and a partial tear of the esophageal wall during EPD and therefore, esophageal perforations may remain undetected, resulting in severe mediastinitis. Young age at first symptoms and classic type of esophageal achalasia are prognostic parameters for the need of repeated treatment when applying EPD. The overall recurrence rate after EPD is 33%.
The necessity to add partial fundoplication to LHM in children is discussed controversially. The rate of gastroesophageal reflux after LHM appears low.[16,19] Anterior partial fundoplication according to Dor is the preferred type of fundoplication in children undergoing LHM.[13,17,20–23] We opted for laparoscopic myotomy according to Heller combined with Dor fundoplication, which helped to eliminate the girl's symptoms of dysphagia and aspiration pneumonia and improved her quality of life.
The innovative treatment approach of peroral endoscopic myotomy (POEM) for achalasia is performed by experienced endoscopists and represents a new, scarless treatment option for esophageal achalasia. Comparing the outcome between a group of children treated by POEM with a group of children treated by LHM, Caldaro et al noted shorter operative time, longer distance of myotomy, and earlier tolerance of oral feeding in the first group.
We describe a rare disease in a child aged 11-years with a long history of symptoms caused by late presentation of achalasia of the esophagus. Achalasia was not diagnosed at the outpatient stage of treatment, which led to a delay in establishing the correct diagnosis and complications, such as obstructive bronchitis, recurrent (aspiration) pneumonia, recurrent vomiting, loss of body weight, pancreatitis, and recurrent headache.
In hindsight, Botox injection and (repeated) EPD would have been an alternative option in our patient, who presented in a poor pulmonal and physical condition initially. However, after treatment of bronchopulmonary symptoms, LHM was carried out without significant complications and with a good short-term outcome. Whether the addition of Dor fundoplication to LHM helped to achieve the good outcome in our patient remains uncertain. Minimally invasive laparoscopic operation combined with bronchoscopic interventions helped to reduce surgical trauma and facilitated shortening of the postoperative rehabilitation period in this child. The cosmetic result of the laparoscopic intervention was better than that after open procedures.
Esophageal achalasia is a rare disease in children. Delayed diagnosis of achalasia can cause recurrent aspiration pneumonia, bronchiectasis, poor weight gain, pancreatitis, and headache. Laparoscopic myotomy of the cardia according to Heller, supplemented by one of the variants of fundoplication, is feasible and advisable in children. Laparoscopic techniques reduce surgical trauma, shorten the postoperative period of rehabilitation, and improve the cosmetic outcome.
Conceptualization: Johannes Michael Mayr, Alexandr Evgen’evich Mashkov, Dmitrii Anatolèvich Pykchteev.
Data curation: Dmitrii Anatolèvich Pykchteev, Andrei Viktorovich Bobylev.
Investigation: Alexandr Viktorovic Sigarchev, Andrei Viktorovich Bobylev.
Methodology: Alexandr Evgen’evich Mashkov, Dmitrii Anatolèvich Pykchteev, Alexandr Viktorovic Sigarchev, Andrei Viktorovich Bobylev.
Project administration: Alexandr Viktorovic Sigarchev.
Supervision: Johannes Michael Mayr, Alexandr Evgen’evich Mashkov.
Validation: Andrei Viktorovich Bobylev.
Visualization: Dmitrii Anatolèvich Pykchteev, Alexandr Viktorovic Sigarchev.
Writing – original draft: Alexandr Viktorovic Sigarchev.
Writing – review & editing: Johannes Michael Mayr, Alexandr Evgen’evich Mashkov, Andrei Viktorovich Bobylev.
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Keywords:Copyright © 2018 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.
aspiration; child; esophageal achalasia; laparoscopic Heller myotomy; obstructive bronchitis; pneumonia