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A review of achalasia in 33 children
Sunny Zaheed Hussain1, Ronald Thomas, Vasundhara Tolia
1Department of Pediatrics, Children's Hospital of Michigan, 3901 Beaubien, Detroit, Michigan 48201, USA.
Insights
Esophageal achalasia in children is diagnosed using barium studies and manometry. Surgical intervention is common, but botulinum toxin (botox) injections offer a less invasive treatment option for this rare condition.
Area of Science:
- Pediatric Gastroenterology
- Gastrointestinal Motility Disorders
Background:
- Esophageal achalasia is a rare esophageal motility disorder characterized by impaired relaxation of the lower esophageal sphincter and loss of peristalsis.
- Diagnosis and management in pediatric populations require specialized approaches due to the rarity and varied presentations.
Purpose of the Study:
- To review the diagnostic methods and management strategies for esophageal achalasia in a pediatric cohort.
- To evaluate the long-term outcomes and complications associated with different treatment modalities.
Main Methods:
- Retrospective chart review of 33 children diagnosed with esophageal achalasia over a 25-year period.
- Diagnostic tools included barium contrast studies, esophageal manometry, and upper endoscopy.
- Management strategies encompassed surgical myotomy and botulinum toxin (botox) injections.
Main Results:
- Dysphagia and vomiting were the most frequent symptoms; weight loss, chest pain, and recurrent pneumonia also occurred.
- Barium studies and manometry were primary diagnostic modalities.
- Surgical myotomy was the predominant treatment, with seven patients receiving botox injections as initial therapy. Post-surgical complications included GERD and residual achalasia, with the latter responding to botox.
Conclusions:
- Esophageal achalasia in children presents with diverse symptoms and requires a combination of diagnostic imaging and functional studies.
- While surgery remains a primary treatment, botulinum toxin (botox) injection is a viable alternative, particularly for residual disease.
- Careful surgical technique, including antireflux procedures, may mitigate post-operative complications.
Abstract:
We reviewed our experience in the diagnosis and management of esophageal achalasia in 33 children over a 25-year period at a single center by a retrospective chart review of all patients diagnosed with achalasia between December 1, 1975 and January 30, 2001. There were 33 cases ranging from 5 months to 16 years of age at the time of presentation (17 boys and 16 girls). Although dysphagia and vomiting were the commonest presenting symptoms, weight loss, chest pain, coughing, and recurrent pneumonia also occurred in many patients. Barium contrast study of the esophagus was the initial diagnostic modality followed by esophageal manometry. An upper endoscopy was also performed in 78.7% of cases. Management was predominantly surgical; however, seven recently diagnosed patients opted for botulinum toxin (botox) injection as the first line of treatment. The follow-up duration varied from 10 months to 10 years (mean 4.71 +/- 3.2 years). Postsurgical complications included gastroesophageal reflux disease in five patients who had not received a simultaneous antireflux procedure and "residual achalasia" in two patients, who both responded to a single botox injection.