Like an Osteophyte. . . or Worse? A Curious Case of Dysphagia in a Patient With Scleroderma

Significance Statement

We hereby present a rare case of severe scleroderma-induced oropharyngeal dysphagia secondary to large retropharyngeal cervical spine calcinosis. The patient’s dysphagia presentation has qualities resembling patients with cervical osteophytes. Given the patient’s history of scleroderma, it is important to appreciate the multifactorial causes for dysphagia associated with this disease entity. This severe case of cervical spine calcinosis was evident on plain film imaging, fluoroscopic-guided swallowing tests, computed topography scanning, and clinical endoscopic examination.

An 82 year-old female known for scleroderma was admitted to hospital for aspiration pneumonia. Her disease was manifested by Raynaud’s, severe calcinosis, sclerodactyly with contractures, telangiectasia, upper gastrointestinal dysmotility, and myositis, and was being treated with a daily dose of 5 mg prednisone. She had multiple hospitalizations for episodes of aspiration pneumonia. During the present admission, a modified barium swallow (MBS) was performed. The MBS demonstrated oropharyngeal dysphagia, a Penetration-Aspiration Scale score of 3 with regular liquids, and diffuse pooling (Supplemental Material). A possible posterior pharyngeal wall lesion was noted to be limiting epiglottic inversion, and an otolaryngology assessment was suggested for direct visualization of the area (Figure 1).

Figure 1.

Radiologic demonstration of severe posterior pharyngeal calcinosis during MBS (arrow pointing to finding). MBS, modified barium swallow.

The otolaryngology team performed a fiberoptic endoscopic evaluation of swallowing to assess her swallow. The patient reported a long-term history of mild-to-moderate dysphagia, with a recent acute deterioration. Her family reported being previously offered surgery for this pharyngeal calcinosis at another center, which was ultimately refused due to associated risks. On examination, the patient had significant stigmata of scleroderma, including microstomia, tightening of the facial skin, significant calcinosis cutis (Figure 2), sclerodactyly, and flexion contractures of the hands and feet. On flexible nasolaryngoscopy, an irregular, submucosal posterior pharyngeal mass was noted which was consistent with the aforementioned radiologic findings. The mass was later re-demonstrated on Computed Tomography imaging (Figure 3). Upon ingestion of purees, the patient was found to have significant pooling, residue throughout the pyriform sinuses and vallecula, and frank aspiration without evidence of protective reflexes. The swallowing test was aborted, and the recommendation was made for strict nil per os (NPO) and consideration of enteral feeding However, the patient ultimately refused. In the following days, she clinically deteriorated secondary to repeated aspiration events and passed away shortly thereafter. Written consent for this article was obtained from next of kin (legal representative).

Figure 2.

Patient’s significant calcinosis cutis noted on hands.

Figure 3.

Radiologic (CT) demonstration of posterior pharyngeal mass at the level of the hypopharynx.

Discussion

Scleroderma, or systemic sclerosis, is an autoimmune connective tissue disorder causing vasculitis and fibrous dysfunction affecting one’s skin, lungs, heart, and gastrointestinal tract.^{1 -3} The limited form is more commonly known as CREST syndrome, presenting with calcinosis cutis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias.^1,4 The diffuse form often includes early visceral and truncal involvement with poorer overall prognosis.

Approximately 90% of patients with scleroderma present with gastrointestinal involvement which most often targets the esophagus. ⁵ Activated endothelial cells in the esophagus release endothelin 1, primarily impairing smooth muscle of the lower esophagus and lower esophageal sphincter.^1,2 This esophageal promotes reflux, esophagitis, bleeding, ulcers, strictures, and fistulas. Patient symptoms typically include dysphagia, regurgitation, heartburn, nausea, vomiting, and/or bloating.^1,2,5

While most patients with scleroderma have dysphagia rooted in the esophageal phase, swallowing is a coordinated process with multiple potential impairment. ⁴ Starting with the pre-oral phase, hand dysfunction from sclerodactyly, skin thickening, and contractures lead to problems in meal preparation and transferring of food into one’s mouth. ² Oral phase obstacles include fibrosis and scarring causing microstomia/microcheilia, joint erosion, and mandibular bone resorption leading to poor temporomandibular joint opening, as well as dental cavities or loss from poor hygiene and xerostomia from a higher prevalence of concomitant Sjogren’s syndrome.^{5 -11}

Pharyngeal dysfunction arises mostly from myositis and cricopharyngeal dysfunction. The myositis leads to muscle impairment and symptoms secondary to muscle inflammation. ¹² On pharyngeal high-resolution impedance manometry, muscle contractions are weak, with possible absent hypopharyngeal constrictor activity and abnormal upper esophageal sphincter contractions leading to increased pooling, residue, penetration, and aspiration.^13,14 The cricopharyngeal muscle is often weakened and less sensitive secondary to severe and chronic reflux. Previous studies have reported that upper sphincter stenosis may occur secondary to reflux.^15,16

The above effects of scleroderma on the gastrointestinal tract and the phases of swallowing are well described. However, our patient’s large cervical spine calcinosis causing dysphagia has not yet been reported. Calcinosis, a soft-tissue calcification that presents in up to 25% of late-stage cases, usually affects extremities but rarely the spine.^{17 -20} Dystrophic calcinosis of the spine impinging posteriorly can cause neurological deficits as the mass destabilizes the spine or leads to spinal canal stenosis.^19,21 Our patient’s cervical spine calcinosis projected anteriorly, mimicking a large osteophyte. Osteophyte-induced dysphagia arises from mechanical compression, impaired laryngeal elevation, epiglottic inversion, or inflammatory edema.^{22 -25} While the other symptoms of scleroderma can affect quality of life, research has demonstrated that the gastrointestinal affects alone can be burdensome due to physical discomfort, mental, and emotional stress. ⁵ Understanding the multifactorial causes of dysphagia in this patient population can help guide management in a patient-specific manner. In this report, we present a novel finding in scleroderma which can contribute to the overall clinical picture of dysphagia.

Supplemental Material

sj-docx-1-ear-10.1177_01455613251411274 – Supplemental material for Like an Osteophyte. . . or Worse? A Curious Case of Dysphagia in a Patient With Scleroderma

Supplemental material, sj-docx-1-ear-10.1177_01455613251411274 for Like an Osteophyte. . . or Worse? A Curious Case of Dysphagia in a Patient With Scleroderma by Gabriella Le Blanc, Jennifer A. Silver and Karen M. Kost in Ear, Nose & Throat Journal