A rare manifestation of cricopharyngeal myopathy presenting with dysphagia in sarcoidosis

Abstract

Sarcoidosis is a systemic inflammatory granulomatous disease that affects multiple organs in the body; however, dysphagia is a relatively rare manifestation at early stages. Dysphagia in sarcoidosis is attributed to many mechanisms, such as mediastinal lymphadenopathy, esophageal or laryngeal involvement, cranial neuropathy, and brainstem infiltration. In this article, we report an extremely rare case with sarcoidosis who presented with dysphagia due to isolated cricopharyngeal myopathy. The 75-year-old woman presented with slowly progressive swallowing difficulty and videofluorography showed insufficient opening of the upper esophageal sphincter. On presentation, she had no cranial nerve or central nervous system impairments. A cricopharyngeal myotomy was performed, and histopathological study revealed a significant inflammatory change with non-necrotizing granulomas within the muscle tissue. We concluded that this was a very rare case of sarcoidosis presenting with localized cricopharyngeal myopathy. Postoperatively, a contracture of the esophageal entrance was successfully released and the dysphagia was alleviated.

Dysphagia, an uncommon initial presentation of sarcoidosis

“…Videofluorography showed marked stenosis posteriorly at the level of pharyngoesophageal junction. She subsequently underwent cricopharyngeal myotomy with histology of the tissue revealing non-caseating granulomas [ 9 ]. Initial videofluoroscopic study of our patient revealed indentation of the hypopharynx in the region of the cricopharyngeal muscle indicating potential myopathic granulomatous infiltration.…”

“…Direct involvement of the enteric nervous plexus can cause dysphagia, and the clinical picture can mimic that of achalasia. Previous studies have reported that the nervous system is involved in approximately 5% of cases, and in postmortem examination, the rates are as high as 15–27% [ 10 ]. Histopathological studies often show diffuse inflammatory infiltration of the myenteric plexus with complete demyelination, loss of axons, and active degeneration [ 11 ].…”

Esophageal Sarcoidosis: A Review of Cases and an Update

“…Histological examination of the cricopharyngeus muscle contained numerous noncaseating granulomas. Similarly, Nishikubo et al [39] describe the case of a woman who underwent cricopharyngeal myotomy due to dysphagia. The muscle on pathological examination was found to be fibrotic and hypertrophic with microscopic evidence of noncaseating granulomas.…”

“…Wasfi et al [36] reported the rare case of a patient who presented with worsening dysphagia as a result of both extrinsic compression by enlarged mediastinal nodes and due to neuromuscular dysfunction due to direct infiltration. Previous studies have reported that the nervous system is involved in approximately 5% of cases, and in postmortem examination, the rates are as high as 15–27% [39]. Sarcoidosis is also known to affect the cranial nerves, with the facial and optic nerves most commonly involved, followed by the collective involvement of cranial nerves IX, X, XI [60].…”

Autoimmune Neurogenic Dysphagia

“…The most common cranial neuropathy in sarcoidosis is the one affecting the facial nerve (VII), but other nerves including the IX and X can be affected, resulting in dysphagia. Of note, involvement of other organs, such as the esophagus [ 82 , 83 ] and the swallowing muscle apparatus [ 84 ], can also cause dysphagia. Diagnosis is aided by increased circulating ACE levels, chest CT showing hilar lymphadenopathy, brain MRI showing leptomeningeal enhancement, PET-CT showing sites of active inflammation, lumbar puncture revealing high protein and oligoclonal bands, and ultimately biopsy of accessible tissue showing non-caseating granulomas.…”