CONGENITAL CRICOPHARYNGEAL ACHALASIA - THE NECESSITY OF GASTROSTOMY AS AN INITIAL APPROACH

Congenital cricopharyngeal achalasia (CCA) is a transient disorder of the swallowing mechanism. Although the clinical picture of this rare e ntity has been clearly described, the treatment remains controversial. Four cases of CCA are presented. Gastrostomy was performed in two pat ients initially or following short-term conservative treatment includi ng gavage feeding and continuous semiupright positioning, because of d istressing respiratory problems. Two other patients were initially tre ated conservatively for 4 weeks and 6 months, respectively; the latter had undergone cricopharyngeal myotomy elsewhere. Gastrostomy was also eventually performed in these patients because they failed to thrive and respiratory symptoms and feeding difficulties persisted despite co nservative treatment. All of our patients gained weight satisfactorily and respiratory symptoms subsided following gastrostomy. One patient died 4 months after the gastrostomy due to neurological problems. We r ecommend performing gastrostomy initially after the diagnosis of CCA i s established in order to ensure satisfactory feeding during the spont aneous recovery period of this disorder, which can be unexpectedly pro longed.