case report
A 48-year-old male with a history of loud snoring and severe obstructive sleep apnea (OSA) as diagnosed by Polysomnography (Apnea/hypopnea index AHI of 58/h (normal <5/h)). He was operated by septoplasty, inferior turbinectomy, tonsillectomy, UPPP, coblation tongue base ablation at one setting one year before presentation. The patient had a tough postoperative course, severe pain, edema of the neck and edema of tongue base. The patient suffered from Dysphagia, difficult breathing, change of voice and persistent snoring and OSA after surgery.
At presentation, the patient had diffuse submental and submandibular firm neck edema, limited mouth opening, firm edema of tongue base with limited tongue movement. (Fig. 1) A flexible endoscopy was performed, revealing some retropalatal adhesions, diffuse edema of the tongue base, adhesions of lateral border of tongue base with lateral oropharyngeal walls limiting full tongue movements, but otherwise normal upper aerodigestive tract with no obstructive lesions.
Radiological evaluation included Barium swallow but showed normal barium decent through the pharynx and esophagus. CT scan of neck showed only submental and submandibular edema with mural thickening of the pharyngeal walls. Those findings were confirmed by head and neck MRI with contrast, it revealed retropalatal adhesions, bilateral symmetrical swollen pterygoid muscles “pterygoid rhabdomyolysis” as described by radiologists, causing oro- & hypo-pharyngeal luminal narrowing. (Fig. 2)
Drug induced sedation endoscopy (DISE) was performed to confirm diagnosis; and taking punch biopsy from tongue base & FNAC from submental and submandibular edema tissue while patient is sedated. Biopsy revealed fibrous tissue with amorphous eosinophilic materials, no malignancy, no lymphoproliferative process. Further staining of the tissue with Congo red stain; Diagnosis was confirmed to be “Amyloidosis of tongue base”.
Patient was referred to Hematologist to identify the ethology of Amyloidosis. Patient was investigated by complete lab work with immunological tests and bone marrow biopsy. Bone marrow biopsy revealed interstitial increase in plasma cells and lymphocytes, immunohistochemical staining (IHC) showed few scattered CD20 positivity with 50-60% CD138 positive; A picture was suggestive of Plasma cell myeloma as a causative underlying etiology of amyloidosis. Patient started chemotherapy and steroids, he showed improvement in swallowing and less edema of the tongue, however, he was put on CPAP therapy for residual OSA.