Cricopharyngeal bar and dermatomyositis: A cause of rapidly progressive dysphagia

Abstract

Background: Idiopathic inflammatory myopathies (IIM) are immune-mediated conditions that affect striated muscle, and are frequently associated with dysphagia. Dysphagia in these cases can be due to weakness of the muscles involved in swallowing or the presence of restrictive pharyngeal defects, such as cricopharyngeal bars. Treatment of dysphagia in IIM revolves around immunosuppressive therapies, and procedures to disrupt cricopharyngeus muscle when immunosuppressive therapies are unsuccessful. Case report: A 73-year-old female presented with rapidly progressive proximal muscle weakness and dysphagia to the point she could not swallow liquids or solids. She had a rash over the extensor surfaces of the limbs, and periorbitaledema. Her creatine kinase was elevated, and skin biopsy showed an interface inflammatory reaction; however, myositis line assay revealed no autoantibodies, and a muscle biopsy was unremarkable. She was diagnosed with dermatomyositis with life-threatening dysphagia, and was admitted to our institution and treated with corticosteroids, methotrexate and intravenous immunoglobulin. A videofluoroscopic swallowing study revealed a large esophageal protrusion at the level of C5-C6, which was thought to be consistent with a cricopharyngeal bar, with large boluses unable to pass, leading to aspiration. After 10 weeks of treatment, the cricopharyngeal bar remained present, but swallowing had improved to the point that she was successfully swallowing all consistencies. Conclusion: Dysphagia associated with IIM can be multifactorial, and can be due to the involvement of the muscles of swallowing in the inflammatory process, or due to restrictive pharyngeal defects, and determination of the cause of dysphagia can assist with management.

Retrograde Cricopharyngeal Dysfunction: A Review

“…The function of the UES is to relax during the initial phase of swallowing, prevent regurgitation of esophageal contents, and limit air from entering the stomach during inspiration [10]. Anatomic studies indicate that the CP is the principle contractile component of the upper esophageal sphincter and inserts on the lateral aspects of the cricoid cartilage [9,[11][12][13][14]. The CP is largely responsible for generating basal tone in the upper esophageal sphincter, thus preventing reflux into the airway [11,15,16].…”

Botulinum Toxin Injection for the Treatment of Upper Esophageal Sphincter Dysfunction

“…The cervical esophagus portion of the UES has a length of 1–2 cm, presenting a transitional anatomic structure between the cricopharyngeal muscle and the adjacent lower section of the esophagus and demonstrating unique histologic features and the presence of circular muscle fibers [ 38 ]. This cervical esophagus segment is located between C6 and C7 [ 38 ], at a level just below the cricopharyngeal muscle and the cricopharyngeal bar (reported at various levels, from C4 to C6) [ 39 , 40 ].…”

“…Under most conditions, the targets of UES botulinum toxin injection are the cricopharyngeus (level with the cricoid cartilage) and the upper part of the cervical esophagus (posterior to the trachea, approximately between C6 and C7 [ 38 ]). A cricopharyngeal bar viewed by a barium study is usually located between C4 and C6 [ 39 , 40 ]. The cervical esophagus in the lower neck deviates to the left and is closer to the carotid sheath and thyroid gland on the left side than on the right side [ 53 ].…”