Surgical treatment of a rare case of bilateral ptosis due to localized ocular amyloidosis

Abstract

We describe a rare case of a 31-year old woman with bilateral ptosis due to localized amyloidosis. She referred a nine-year history of ptosis and surgical treatment with frontalis suspension three years previously. Following complete ophthalmological examination and evaluation of the ptosis we carried out tarsal and fornix biopsy, which revealed accumulation of a weakly eosinophilic amyloid positive substance. We performed surgical correction using the levator aponeurosis-Müller's muscle complex re-adaptation technique and amyloid substance debulking in all the palpebral layers in the left eye. The material obtained was stained with hematoxylin-eosin, Congo Red, PAS and alpha-actin, which confirmed amyloid deposition. Successively, the right eye was operated in the same manner and entropion was managed by dissection and removal of amyloid from subconjunctival layers. Five years following surgery, the corrective procedure for ptosis was still effective. Surgical treatment of ptosis is very complex and requires precise indications. Appropriate management depends on the etiopathogenesis, accurate diagnosis, and clinical findings.

Retinal and choroidal circulation determined by optical coherence tomography angiography in patient with amyloidosis

“…The cryopyrine-associated periodic syndrome (CAPS) is a hereditary autoinflammatory syndrome with fever, exanthema, arthritis, aseptic meningitis and optic neuritis. The chronic inflammation causes amyloid deposits in multiple organs including the eye 1–5. A case report showed amyloidosis is associated with chorioretinopathy and a thicker hyporeflective choriocapillaris band at the area of choriocapillaris in optical coherence tomography (OCT).…”

Bilateral Amyloidosis of Three Eyelids. A Case Report

“…Infiltrace amyloidu bývají v bulbární spojivce eventuálně i v oblasti fornixu [18,19,21], v tarzální spojivce [17,21] dolního víčka, ale i v semilunální plice [24]. Významné a rozsáhlé postižení horní tarzální spojivky může vyvolat ptózu [1,23,24,25], do diferenciální diagnózy v této lokalizaci také patří vernální katar [26]. Léčebně je doporučovaná excize [18,19,23] eventuálně doplněná o kryoterapii [20,23] a lokálně kortikosteroidy k utlumení symptomů chemózy [20,21].…”

“…Úspěšně byla chirurgicky řešena stenóza slzných cest [22] i ektropia [17]. Operační řešení ptóz u mladších pacientů ve věku 23 let [26] a 31 let [25], bylo kosmeticky i funkčně úspěšné, a to i po pěti letech u jediné oboustranné formy. Další čtyři oboustranná postižení byla popsána u ektropia víček [1], při stenóze slzných cest [21] a dvakrát u bulbárních spojivek v dolních fornixech [20,21] z celkové počtu 19 nemocných z jedenácti uvedených studií v letech 2002 až 2018.…”