Esophageal achalasia of unknown etiology in infants
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Esophageal achalasia of unknown etiology in infants Vijay D Upadhyaya, AN Gangopadhyaya, DK Gupta, SP Sharma, Vijayendra Kumar, SC Gopal Varanasi, India
Methods: In this retrospective study, the data were obtained from records of the Department of Pediatric Surgery IMS, BHU Varanasi, India. The patients were diagnosed by clinical examination and barium study. Any other associated anomalies were noted in these patients. The patients underwent esophagocardiomyotomy with antireflux procedure via the abdominal route. Results: In the present series, 7 patients survived. The follow-up study after operation showed remarkable relief of symptoms with satisfactory weight gain. No post-operative death occurred in the patients. Achalasia associated with alacrimia was noted in one infant who was lost to follow up. One infant was initially diagnosed as having gastric volvulus, but exploration revealed achalasia cardia. Conclusions: Esophageal achalasia is a rare disease in children and its origin is generally indeterminable. Achalasia cardia should be emphasized in the differential diagnosis of an infant presenting with signs and symptoms of esophageal obstruction. World J Pediatr 2008;4(1):63-65 Key words: esophageal achalasia; esophagomyotomy; etiology; gastroesophageal reflux; infants
Author Affiliations: Department of Pediatric Surgery, IMS, BHU, Varanasi, India (Upadhyaya VD, Gangopadhyaya AN, Gupta DK, Sharma SP, Kumar V, Gopal SC) Corresponding Author: Vijai Upadhyaya, MD, Senior Resident Pediatric Surgery, Department of Pediatric Surgery, IMS, BHU, Varanasi, 221005, U. P. India (Tel: 91-11-0542-9257; Email: [email protected])
©2008, World J Pediatr. All rights reserved.
Introduction
T
homas Willis first described achalasia in 1674. He successfully treated a patient by dilating the lower esophageal sphincter (LES) with corktipped whalebone. Esophageal achalasia of unknown etiology is a rare disease, affecting 1:100 000 of the general population and just 4% to 5% of these cases are described in children,[1] and less than 1% cases in infants. Achalasia involves failure of the LES in conjunction with reduced motility in esophagus itself. The passage of food via the esophagogastric junction is extremely difficult without true organic stenosis or extrinsic compression. Symptoms of achalasia vary from regurgitation to recurrent aspiration pneumonia. Older children present with symptoms of progressive dysphagia. For these reasons, the disease is often confused with gastroesophageal reflux disease (GERD), delaying correct diagnosis and treatment.[2,3] We present 8 infants with achalasia cardia, aged from 5 months to 9 months (meadian, 5.9 months).
Clinical summary
Background: Achalasia cardia is an uncommon disease in children particularly in infants. We present 8 cases of achalasia who were encountered over a 12-year period. In infantile achalasia, respiratory symptoms predominate and vomiting may easily be mistaken for gastroesophageal reflux (GER). Vomiting of uncurdled milk is characteristic of achalasia.
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