Corinna Mae R. Carag
St. Lukeā??s Medical Center, Philippines
Posters & Accepted Abstracts: Gen Med
Achalasia is a rare disorder, particularly in Pediatrics, characterized by esophageal aperistalsis and inadequate relaxation of the lower esophageal sphincter. Its etiology remains unclear and is mostly idiopathic. This is a case of an adolescent male who presented with progressive dysphagia, occasional chest pain, and significant weight loss, and was diagnosed with Esophageal Achalasia through upper GI series, endoscopy, and manometry. The patient underwent Heller’s Myotomy, the surgical gold standard for treatment. Introduction: Achalasia of the esophagus is a very rare condition, with an estimated annual incidence of 1:100,000 cases overall, and less than 5% of which occur in children 0.11 per 100,000 pediatric patients.1 Pediatric achalasia is generally diagnosed between 7 and 15 years of age and has a slight predominance in boys.2 In the Philippines, only 3 cases have been reported in the Philippine Pediatric Society Registry for the year 2020.3 Achalasia is a pathological condition causing dysphagia, reflux, and regurgitation. The hallmarks of diagnosis include esophageal dysmotility and lack of relaxation of the lower esophageal sphincter LES. Diagnosis is suspected by the clinical history, but is often delayed in children because of the rarity of the disease. If left untreated, the sequelae can be significant, as the proximal esophageal tissue becomes more compliant as a compensatory measure and entirely non- functional by end-stage disease. It is a life-long, debilitating condition, with a significant impact on quality of life. Hence, prompt diagnosis and intervention are needed. To date, pneumatic balloon dilation and Heller’s myotomy are considered the most effective therapeutic options in children. Case Presentation: A 15-year-old male was admitted for a 1 year history of progressive dysphagia (initially to solids, and eventually progressing to liquids), with frequent regurgitation, occasional chest pain, and significant weight loss ~60 lbs. or 27kg in 1 year. The patient had no known illnesses and this was the patient’s first admission. The patient had normal vital signs for age and an unremarkable physical examination. Esophagoduodenoscopy was done for the patient revealing absence of any mechanical obstruction or inflammatory cause of the patient’s esophageal Symptoms. Gastric and distal esophageal biopsy showed unremarkable results with no active inflammation, intestinal metaplasia, atrophy, or dysplasia. Hence, an upper Gastrointestinal Series (UGIS) with barium contrast was facilitated. Results showed satisfactory swallowing mechanism, with no gross tracheal aspiration or nasopharyngeal regurgitation. However, there was narrowing at the region of the lower esophageal sphincter with lack of peristalsis, dilatation, and pooling of contrast at the distal segment of the esophagus. Findings were reflective of Achalasia (Figure 1). To further strengthen the diagnosis, an Esophageal Manometry test was done which showed incomplete relaxation of the LES in response to swallowing; high resting LES pressure, and absent esophageal peristalsis which were consistent with a Classic Achalasia according to the Chicago Classification of Esophageal Motility Disorders for manometry. A chest CT scan was also done for the patient which showed dilated intrathoracic esophagus down to the gastro- esophageal junction with air-fluid level and non- thickened walls reflective of Achalasia. The patient then underwent Heller’s Myotomy with Dor Fundoplication which the patient tolerated well with no immediate post-operative complications noted. The patient was admitted for a total of 7 days for post- operative care. The patient was initially placed on NPO for 24 hours post-operatively and feeding was slowly progressed until diet as tolerated was achieved prior to discharge. The patient had an unremarkable hospital course with no noted regurgitation or chest pain post-operatively and was discharged well.
