Pediatric Achalasia: A Guide to Diagnosis and Approach to Surgery

Pediatric achalasia disorder is a rare esophageal disorder which results in debilitating symptoms and may even retard childhood growth and development. Often misdiagnosed, pediatric achalsia, orginating as a neuropathic condition, is considered rare and difficult to diagnose. Although the disorder affects individuals at any age, approximately 10% of all achalasia cases are found in children. With advances in technology and the availability of surgery, children suffering from this painful condition may find relief through proper diagnostic testing and surgical treatment resulting in an improvement in nutrition and improved childhood development.

In recent years, pediatric achalasia has been treated through a surgical process called the Heller operation, also known as the anterior esophageal myotomy. Balloon dilation surgery, originally suggested in 1971 by Dr. Van Trapp, is no longer a recommended procedure. Instead, the esophageal myotomy surgical procedure is often used and attributes its success in its ability to decrease pressure in the lower sphincter of the esophagus of children through completed a laparoscopic abdominal procedure. In children with achalasia, food is unable to pass through to the stomach due to a deficiency or defect in the valve which separates the stomach and esophagus. This defect prevents the valve from relaxing properly and to allow food to pass into the stomach for digestion. The condition is also compounded by a further complication in which the nerve endings, within the esophagus, lie dormant or are understimulated. Research has shown pediatric achalasia may be a co-morbid condition with cancer, parasite infection, general stomach smooth muscle deterioration or even a genetic disorder. Although not a guaranteed cure for positively diagnosed condition of pediatric achalasia, the Heller surgical procedure does offer the patient a more improved life by reducing inflammation, bad breath, coughing, symptoms of heartburn relating to smooth muscle pain, correcting nutritional deficiencies involving weight loss, anemia and relieving symptoms and co-morbid disorders such as regurgitation of food and improves child development through improved nutrition.

To diagnose pediatric achalasia a simple test called esphogeal manometry can be performed however most physicians will begin with an upper GI study to rule out other causes for the child’s symptoms. When esphogeal manometry is ordered, the physician will probe the esophagus using a catheter, to measure esophagus pressures and contractions. The test will examine the action of the lower esophagus sphincter which is the main culprit responsible for the deficiency found in children suffering from pediatrica achalasia. The test can be performed on individuals of any age, including newborn children.

As with any diagnostic procedure, the esophageal manometry does come with a few complications. However the complications are rare and may not outweigh the benefits of completing the study. When performing the procedure, your pediatrician will monitor for excessive saliva as this may increase the likelihood of aspiration which could potentially lead to a disorder of the lungs including aspiration pneumonia. In both the esophageal manometry diagnostic test, as well as the Heller surgical procedure, your pediatrician may elect to perform an anti-reflux procedure in combination to prevent post-treatment complications including gastric reflux disorder.

As stated earlier, the esophageal manometry diagnostic study and the Heller surgical procedure provide some risks to the pediatric patient, however these risks do not outweigh the benefits of the procedures and the complications which arise without treatment including delayed childhood growth and dedvelopment attributed to nutritional deficiencies. When considering a diagnostic procedure, consult your pediatrician regarding the side effects. Although not guaranteed to result in a true diagnosis of pediatric achalasia, the esophageal manometry proves to be the best technology available and, potentially, leads to surgical discussions with your pediatrician.

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