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Key Points

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  1. Esophageal achalasia has profound impact on a child's growth and development with many children presenting with failure to thrive.

  2. Symptoms include dysphagia, regurgitation of undigested food, and nocturnal wheezing in older children; and respiratory difficulties such as wheezing, chronic coughing, bronchitis, pneumonitis, and recurrent pneumonia in younger children.

  3. Classic radiographic findings of a barium esophagogram are dilated esophagus with a “bird's beak” narrowing of the lower esophageal sphincter (LES).

  4. Esophageal manometry is the gold standard study to confirm the diagnosis of achalasia. The hallmark manometric findings are increased LES resting pressure, absence of peristalsis, and failure of LES to relax to swallowing.

  5. The primary treatment of choice for children with achalasia is surgical, specifically the modified Heller esophagomyotomy (EM).

  6. Either a laparoscopic or thoracoscopic approach can be used with similar success. Currently, the preferred approach is a laparoscopic Heller EM with concomitant fundoplication.

  7. Long-term outcomes show around 90% relief of dysphagia with rapid resumption of normal diet and growth with diminution of respiratory symptoms.

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Introduction

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Achalasia is an esophageal motility disorder characterized by the absence of peristalsis and failure of the lower esophageal sphincter (LES) to relax in response to swallowing. Its incidence in the United States and Europe is 0.2 to 1 per 100,000 per year, with children comprising only 5% of the patient population. The impact of achalasia on the growth and development of children is, however, profound.

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Pathophysiology

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Swallowing is a complex coordinated set of neuronal impulses originating in the swallowing center of the medulla. These impulses first act on the striated pharyngeal musculature, propelling a food bolus into the upper esophagus; a peristaltic wave then moves it down the esophagus. The LES, which is a high-pressure zone at the gastroesophageal junction, acts as a valve to prevent the reflux of gastric contents into the esophagus. It relaxes when reached by the peristaltic wave transporting the food bolus into the stomach. In contrast to this normal process, achalasia is characterized by obstruction at the gastroesophageal junction and abnormal peristalsis in the body of the esophagus. Manometric techniques have clearly defined a combination of hypertensive sphincter resting pressures and incomplete relaxation in response to a swallow. Additionally, contractions in the lower two thirds of the body of the esophagus are irregular or absent.

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Although peristalsis appears to be under vagal control, the innervation of the esophagus is not well understood. The LES has intrinsic myogenic tone, which is modulated by neural and hormonal mechanisms. The vagus nerve sends both excitatory and inhibitory nerve fibers to the LES. However, their degree of influence on LES tone is unclear. Interestingly, the LES continues to relax on swallowing, suggesting a degree of autonomy. Hormones and gut peptides found to increase LES pressure are gastrin, motilin, substance P, and bombesin; those that decrease this pressure are secretin, gastric inhibitory peptide, neuropeptide Y, and vasoactive intestinal peptide.

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