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Achalasia cardia sub-types in children: Does it affect the response to therapy?
Anshu Srivastava1, Ujjal Poddar2, Amrita Mathias2
1Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226 014, India. avanianshu@yahoo.com.
Insights
Pediatric achalasia subtypes, Type I and II, show similar clinical features and treatment responses to pneumatic dilatation (PD). Type II exhibits higher lower esophageal sphincter pressure and a less dilated esophagus than Type I.
Area of Science:
- Pediatric Gastroenterology
- Esophageal Motility Disorders
- Achalasia Subtyping
Background:
- Achalasia subtypes influence treatment outcomes in adults, but data in children are limited.
- Understanding these differences in pediatric achalasia is crucial for tailored therapeutic approaches.
Purpose of the Study:
- To investigate clinico-laboratory distinctions between achalasia subtypes in children.
- To compare treatment response rates across achalasia subtypes in pediatric patients.
Main Methods:
- Forty-eight children diagnosed with achalasia underwent clinical evaluation, barium swallow, high-resolution manometry (HRM), and gastroscopy.
- Achalasia subtypes were classified using the Chicago classification based on HRM findings.
- Primary treatments included pneumatic dilatation (PD) or surgery, with success defined by an Eckhardt score ≤3.
Main Results:
- Achalasia Type I (n=19) and Type II (n=19) presented with similar clinical symptoms, primarily dysphagia and regurgitation.
- Type II achalasia showed significantly higher basal lower esophageal sphincter (LES) pressure and a less dilated esophagus on timed barium esophagogram (TBE) compared to Type I.
- Initial PD demonstrated comparable success rates for both Type I and Type II (86.6% vs. 92.8%), with no significant difference in the need for post-PD myotomy.
Conclusions:
- Pediatric achalasia Types I and II share similar clinical presentations and respond equally well to initial pneumatic dilatation.
- Type II achalasia is characterized by higher LES pressure and a less dilated esophagus than Type I.
- Timed barium esophagogram (TBE) is a valuable tool for assessing treatment response following pneumatic dilatation.
Background:
Achalasia sub-types affect treatment response in adults, but there is no similar data in children. We studied the differences in clinico-laboratory features and response to therapy between achalasia sub-types in children.
Methods:
Forty-eight children (boys:girls-25:23, 14 [0.9-18] years) with achalasia (clinical, barium, high-resolution manometry [HRM], gastroscopy) were evaluated. The sub-type was determined by Chicago classification at HRM. Pneumatic dilatation (PD) or surgery was the primary therapy. Success was defined as Eckhardt score of ≤ 3.
Results:
Dysphagia (95.8%) and regurgitation (93.8%) were the most common symptoms. Forty of 48 cases had an adequate HRM study: Type I (n-19), II (n-19) and III (n-2). Types I and II had similar clinical profile. Type II had higher basal lower esophageal sphincter (LES) pressure (30.5 [16.5-46] vs. 22.5 [13-43] mmHg; p = 0.007) and less dilated esophagus on timed barium esophagogram (TBE, 25 [13-57] vs. 34.5 [20-81] mm; p = 0.006) than type I. Both types had similar success (86.6% [13/15] vs. 92.8% [13/14]; p = 1) after first PD and need of post-PD myotomy (5/17 vs. 1/16; p = 0.1) in follow-up. Twenty-three cases had TBE before and after PD; 15 (65.2%) had good clearance. These subjects required myotomy (1/15 vs. 4/8; p = 0.03) and repeat PD (5/15 vs. 4/8; p = 0.08) less often than those with poor clearance on TBE.
Conclusion:
Types I and II achalasia have similar frequency and clinical profile. Type II has higher LES pressure and less dilated esophagus than Type I. Both respond equally well to initial PD. Type I required post-PD myotomy more often, though not significantly. TBE is useful for assessing therapeutic response.
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