Cricopharyngeal achalasia in connection with autoimmune disorders in a pre-adolescent patient: a case report

• Main Authors: Urška Vučina, Primož Kotnik, Rok Orel
• Format: Article
• Language: English
• Published: Slovenian Medical Association 2017-03-01
• Series: Zdravniški Vestnik
• Subjects: dysphagia; esophagus; cricopharyngeal achalasia; autoimmunity; dilatation
• Online Access: http://vestnik.szd.si/index.php/ZdravVest/article/view/1544

<p>Cricopharyngeal achalasia is an uncommon cause of dysphagia, especially in children. Congenital form is known in neonates and infants. In older children this disease has been reported in very rare cases and mostly in connection with neurological and muscular diseases. We present a case of a 12-year-old girl with a four-year history of dysphagia. Esophagogastroduodenoscopy, radiological contrast swallow study and esophageal manometric study confrmed the diagnosis of cricopharyngeal achalasia. Te patient was successfully treated with dilatation of the upper esophageal sphincter. An initial attempt of dilatation appears to be a safe and effective option in the management of cricopharyngeal achalasia in children, and may prevent or at least postpone the need for myotomy. Following the diagnosis of cricopharyngeal achalasia, several autoimmune conditions were diagnosed. Our case report revealed that cricopharyngeal achalasia may occur in association with some other autoimmune conditions, and that autoimmunity may also play a role in cricopharyngeal achalasia itself</p>