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

Urška Vučina; Primož Kotnik; Rok Orel

doi:10.6016/ZdravVestn.1544

Urška Vučina University Children’s Hospital, University Medical Centre Ljubljana, Slovenia
Primož Kotnik Department of Endocrinology, Diabetes and Metabolism, University Children’s Hospital, University Medical Centre Ljubljana, Slovenia
Rok Orel Department of Gastroenterology, Hepatology and Nutrition, University Children’s Hospital, University Medical Centre Ljubljana, Slovenia

DOI: https://doi.org/10.6016/ZdravVestn.1544

Keywords: dysphagia, esophagus, cricopharyngeal achalasia, autoimmunity, dilatation

Abstract

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

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Author Biographies

Urška Vučina, University Children’s Hospital, University Medical Centre Ljubljana, Slovenia

University Children’s Hospital, University Clinical Centre Ljubljana, Slovenia

Urška Vučina, dr. med.

Primož Kotnik, Department of Endocrinology, Diabetes and Metabolism, University Children’s Hospital, University Medical Centre Ljubljana, Slovenia

Department of Endocrinology, Diabetes and Metabolism, University Children’s Hospital, University Clinical Centre Ljubljana, Slovenia

doc. dr. Primož Kotnik, dr. med.

Rok Orel, Department of Gastroenterology, Hepatology and Nutrition, University Children’s Hospital, University Medical Centre Ljubljana, Slovenia

Department of Gastroenterology, Hepatology and Nutrition, University Children’s Hospital, University Clinical Centre Ljubljana, Slovenia

izr. prof. dr. Rok Orel, dr. med.

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