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Diffuse esophageal spasm in children. John M Rosen, Teri Lavenbarg, Jose Cocjin, Paul E Hyman, University of Kansas Medical Center, Kansas City, KS, USA.
Diffuse esophageal spasm (DES) causes chest pain and/or dysphagia in adults but is rarely reported in children. We defined DES as simultaneous contractions associated with >10% of swallows and mean simultaneous contraction amplitude > 30 mmHg (Castell, 2004). We reviewed charts of 278 subjects 0-18 years of age after esophageal manometry. Results included normal motility 61%, nonspecific esophageal motility disorder 20%, DES 13% (n=36), and achalasia 4%. Of 24 subjects < 5 years, chief complaints were food refusal (14), vomiting (3), choking (3), retching (1), and no symptoms (3). Of 12 subjects > 5 years, chief complaints were chest pain (4), dysphagia (3), vomiting (2), food refusal (2), and retching (1). Food refusal was more common in infants and toddlers than in children and teens (p<0.05). Esophageal manometry was the only motility test in 14 subjects; 22 subjects were evaluated with antroduodenal (15) or colonic manometry (3) or both (4). Of these 22 subjects, 9 were diagnosed with DES only, and 13 were diagnosed with generalized motility disorders. Prior to manometry, 25 subjects (69%) had surgery for symptoms: 20 gastrostomies, 13 fundoplications, 3 pyloroplasties, 1 Collis gastroplasty, and 1 gastrojejunostomy. Comorbid medical conditions, often multiple, were found in 33 subjects: idiopathic developmental delay (7), heart malformation (7), Down?s Syndrome (6), cerebral palsy (4), seizures (2), and neuropathic pseudoobstruction (2). Mitochondrial myopathy, chromosome 3p25 deletion, chromosome X q26-28 duplication, mixed connective tissue disease, holoprosencephaly, Rett Syndrome, Charcot-Marie-Tooth disease, Ehlers-Danlos Syndrome, Hirschsprung?s disease, and celiac disease were each found in one subject. Follow up treatment modalities and effects were variable. We conclude that DES in infants and children is not rare. DES should be considered when children present with food refusal, dysphagia, chest pain, or unexplained vomiting. DES in infants and children rarely presents without comorbidity. We speculate that early diagnosis and treatment may prevent unnecessary surgery in some cases.
Abstract draft for presentation at DDW
