Catarina Duarte, Raquel Bento, Paulo Martins, Paulo Borges Dinis, Ana Rita Santos,. António Marques Pereira, Rosa Roque Farinha. Conclusion. Amyloidosis ...
Laryngeal Amyloidosis: a rare cause of dysphonia in adolescent age Catarina Duarte, Raquel Bento, Paulo Martins, Paulo Borges Dinis, Ana Rita Santos, António Marques Pereira, Rosa Roque Farinha
Amyloidosis seems to be a chronic disease of unknown cause
surgical debridement.
characterized by the abnormal accumulation of insoluble protein
follow-up is recommended.
A multidisciplinary approach and a long term
deposits in tissues and organs, most commonly in the kidney, heart, liver, nervous system and gastrointestinal tract. Amyloid deposition progressively leads to tissue and organ dysfunction and can be either
Case Report
systemic or localized (organ-specific), with a number of cases
A previous healthy and non-smoker 17-year-old male complaints with
apparently occurring secondary to other underlying chronic
persistent hoarseness for the last 3 years, with no other symptoms
inflammatory conditions. Localized disease in the upper respiratory
associated. The ENT exam revealed a mass in the right Morgagni
tract most commonly affects the larynx, with the vocal cords and
ventricle (Figure 1). A contrast enhanced Computed Tomography scan
ventricles being the most affected sites. It is relatively rare and usually
and a Magnetic Ressonance Imaging of the patient’s neck showed a
revealed by hoarseness. Its pathophysiology is poorly understood.
right supraglotic mass in the (Figure 3,4 and 5). The subsequent biopsy showed evidence of amyloidosis which was later confirmed by
Whilst there is no general consensus regarding optimal treatment, localized disease may benefit from surgery to improve organ function and alleviate symptoms, whenever amyloid deposits are accessible to
immunohistochemical studies. All the other studies where normal, including the minor salivary glands biopsy. Therefore, diagnosis of localized amyloidosis was established.
Fig. 3
Fig. 1
Fig. 4
Fig. 5
Conclusion Amyloidosis, either in its localized or systemic form, is relatively rare in Otolaryngology. However, it should not be forgotten in the differential diagnosis of any head and neck lesion even in the adolescent age, as this case proves. Although most of the lesions in this area represent localized disease, it is mandatory to exclude the systemic form.
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