Esophageal achalasia presenting as recurrent pneumonia in children: A case series

Abstract

Esophageal achalasia (EA) is a rare primary esophageal motility disorder that is considered a rare etiology of dysphagia among infants and children. The proposed primary pathophysiology is related to the loss of ganglion cells in the distal esophageal sphincters, particularly in the Auerbachian muscle layer, which then leads to the dysmotility and failure of lower esophageal sphincter relaxation. Dysphagia, vomiting, poor weight gain, cough, and recurrent aspiration pneumonia are the most common presenting complaints. Herein, we report 3 cases of EA who presented with chronic cough and recurrent aspiration pneumonia. This study reviewed 3 pediatric patients with typical symptoms of EA. All the patients were admitted and referred to the pediatric pulmonology service for evaluation of recurrent pneumonia and suspected aspiration syndrome. All patients underwent a barium esophagogram as a part of the aerodigestive workup of recurrent vomiting, dysphagia, and aspiration pneumonia. Additionally, all the patients underwent workup for other associated congenital anomalies, which included echocardiography, brain magnetic resonance imaging, and an abdominal ultrasound. All patients had EA and presented with recurrent pneumonia. All patients had isolated EA, and none had any evidence of Allgrove syndrome. Pneumatic balloon dilatation was performed for all patients at the same time as the upper gastrointestinal endoscopy. Later, all the patients underwent a laparoscopic Heller myotomy and had no postoperative complications, and their symptoms resolved. EA is a rare condition in children, yet it can be a serious and life-threatening condition if left untreated. Our cases emphasize the significance of considering achalasia in children who experience esophageal dysphagia and recurrent pneumonia. Several pediatric cases have been reported in which respiratory involvement was the primary manifestation of achalasia. These cases highlight the importance of considering gastrointestinal disorders, particularly EA, in the differential diagnosis of children who experience recurrent pneumonia. Early diagnosis and treatment with laparoscopic Heller myotomy can lead to good outcomes for children with achalasia.

Figure 1.

(A) Barium esophagogram and upper gastrointestinal (UGI) endoscopy in case 1. (A and B; barium esophagogram) Note the severe dilatation of esophagus with narrowing at the gastroesophageal junction of the lower segment—a characteristic image of “bird’s beak” (white arrow). (C and D; UGI endoscopy) UGI endoscopy revealing stenosis of the esophagogastric junction with retained food content.

Figure 2.

Chest radiography (CXR), CT chest findings, and barium esophagogram in case 2. (A and B; anteroposterior (AP) and lateral CXRs) AP CXR shows dilated esophagus with air (arrow) behind the trachea in the posterior mediastinum “air esophagogram” sign; lateral CXR of the patient shows dilated esophagus projecting beyond shadows of the heart (arrow). (C) CT scan of the chest shows a massively dilated esophagus (arrow). (D) Barium esophagogram showing dilatation of esophagus with narrowing at the gastroesophageal junction of the lower segment. T = tracheal shadow.

Figure 3.

Chest radiography (CXR) and barium esophagogram in case 3. (A) CXR of the patient shows consolidations involving the left lower lobe (arrow) with bilateral peribronchial thickening indicative of chronic aspiration syndrome. (B) Barium esophagogram showing diffuse dilatation of esophagus with narrowing at the gastroesophageal junction of the lower segment—a characteristic image of “bird’s beak” (arrow)—and presence of food particles.