Abstract

Introduction: Abnormal oral behaviour is frequently associated with neuromuscular disease, and its underlying mechanisms may be very heterogeneous. The aim of the present retrospective study was: 1) to seek a relation between clinical symptoms and oesophageal manometric abnormalities in two groups of patients, with congenital neurological pathology and acquired neurological pathology, respectively; 2) to better characterize different manometric profiles. Methods: Twenty two children with congenital pathology (Group 1. Mean age: 3.5 years; range: 2 months-18.5 years) and eight children with acquired pathology (Group 2. Mean age: 7.5 years; range: 5months- 14 years) underwent oesophageal manometry (OM) because of oral behaviour dysfonction. Oral behaviour abnormalities were classified in 3 grades: grade A: mild; grade B: important; grade C: severe. Other investigations were also performed: long-term pH-metry, UGI endoscopy, UGI series, ENT examination, and auditive evoked potentials (AEP). Results: Group 1. Oral behaviour abnormalities were: grade A (n=6), grade B (n=13), and grade C (n=3). OM results were as follows: normal or isolated lower oesophageal sphincter abnormalities (n=2), achalasia (n=11), dyskinesia (n=4). Other investigations showed: gastrooesophageal reflux (GER) (n=11/16), oesophagitis (n=10/16), UGI series abnormalities (n=4/9), abnormal ENT examination (n=10/11), and AEP central dysfonction (n=4/13). Group 2. Oral behaviour abnormalities were as follows: grade A (n=3), grade B (n=3), and grade C (n=2). OM results were: normal (n=1), upper oesophageal sphincter abnormalities (n=3), achalasia (n= 2), dyskinesia (n=1), and incomplete study (n=1). Other investigations showed: GER (n= 3/5), oesophagitis(n= 2/4), UGI series abnormalities (n=2/3), abnormal ENT examination (n=5/5), and AEP central dysfonction (n=4/4). Follow-up showed the occurrence of appropriate autonomous feeding in 11/22 patients from Group 1 and in 3/8 patients from Group 2, and the occurrence of respiratory complications in 4/22 patients from Group 1 and in 5/8 patients from Group 2. Four children of Group died. Conclusion: This study did not show any significant difference in symptom severity between the two groups of patients. Partial or mild achalasic profiles were only seen in congenital encephalopathy while upper oesophageal sphincter abnormalities were more frequent in the acquired pathology group. Manometric abnormalities could be linked to GER and/or oesophagitis, or could be the single explanation for oral behaviour dysfunction.

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