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.