Achalasia is a rare disorder affecting esophageal motility. Although this condition has been described for more than 300 years, the etiology is still not well understood. Abnormalities in myenteric ganglion cells as well as neurotransmitters have been proposed to be the underlying pathophysiology but yet to be confirmed. Histologically, achalasia shares similarity with Chagas’ disease caused by Trypanosoma cruzi. Affected esophagus becomes aperistalic with pressurized body. The lower esophageal sphincters do not to relax in patients who suffer from achalasia. Clinically, affected children will present with progressive dysphagia and vomiting. Some will develop complications such as pulmonary infection or failure to thrive. Radiological features of achalasia in a contrast swallow are a proximal dilated esophagus with a smooth tapering of the gastro-esophageal junction on swallowing. While endoscopy will reveal a dilated proximal esophagus with narrowing at the lower esophageal sphincter, the diagnosis can be confirmed with esophageal manometry which typically shows the failure of relaxation of LOS on swallowing and absence of peristalsis in the esophageal body. Medical therapy provides limited and short-term symptomatic relief. Balloon dilatation of achalasic segments can be performed under endoscopic guidance for long-term relief but its effectiveness has been shown to be inferior to that of definitive surgery. The mainstream surgical procedure is Heller’s cardiomyotomy which can be done by open or a minimally invasive surgical approach in recent years. Trans-esophageal endoscopic myotomy represents the latest surgical advancement in the management of this condition.
KeywordsAchalasia Esophagus Dysphagia Esophageal manometry Heller myotomy Trans-esophageal endoscopic myotomy
This chapter has been adapted by permission from Springer Nature: Tam P.K.H. (2006) Achalasia. In: Puri P., Höllwarth M.E. (eds) Pediatric Surgery. Springer Surgery Atlas Series. Springer, Berlin, Heidelberg.
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