Cricopharyngeal achalasia (CPA) is an infrequently encountered but important diagnosis in pediatric dysphagia. This disorder is characterized by difficulty in feeding, regurgitation of feeds, and recurrent aspiration episodes. In this review, we discuss the current understanding of the pathophysiology of the disease and the recent developments in the diagnosis and therapeutic management of CPA.
Because of the rarity of the disease, the literature reporting the treatment of CPA is limited to small case series. Although open surgical treatments including cricopharyngeal myotomy have been reported in the past, recent studies advocate less-invasive endoscopic approaches, including balloon dilation, endoscopic cricopharyngeal myotomy, and botulinum toxin injections.
When CPA is suspected as a cause of dysphagia in a child, the diagnosis can be confirmed with videofluoroscopic swallow studies that demonstrate narrowing at the region of the cricopharyngeus muscle. Treatment should be initiated for children who are unable to feed orally. Current options for treatment include botulinum toxin injections, endoscopic balloon dilation, and open or endoscopic cricopharyngeal myotomy. All techniques have shown success in the treatment of the disease. Further studies comparing treatment modalities are needed before a clear recommendation can be made.
aDepartment of Otolaryngology - Head and Neck Surgery, CHOC Children's Hospital of Orange County, University of California, Irvine School of Medicine, Orange, California
bDivision of Pediatric Otolaryngology, Department of Otolaryngology - Head and Neck Surgery, Stanford School of Medicine, Stanford, California, USA
Correspondence to Anna H. Messner, MD, Department of Otolaryngology - Head and Neck Surgery, Stanford School of Medicine, 801 Welch Road, Stanford, CA 94304, USA. Tel: +1 650 725 6500; fax: +1 650 725 8502; e-mail: Amessner@ohns.stanford.edu