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Maria M Choudhary, Rao Chundury, Priyanka Kumar, Julian Perry; Amyloidosis Masquerading as Unilateral Ptosis. Invest. Ophthalmol. Vis. Sci. 2016;57(12):679.
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© ARVO (1962-2015); The Authors (2016-present)
We report 2 cases of amyloidosis masquerading as unilateral ptosis. To the author’s knowledge these are the first 2 cases in which a diagnosis of amyloidosis was made from isolated unilateral ptosis without any other clinical signs.
Case 1 is a 34 year old woman with a history of monocular strabismus surgery of the left eye combined with left upper blepharoplasty and external ptosis repair 4 years prior to her presentation for persistent droopy left upper eyelid (Figure 1). MRD was 4mm on the right and 2mm on the left. Levator function was 12mm on both sides and there were no conjunctival abnormalities on slit lamp examination. During a modified conjunctivo-mullerectomy ptosis repair, the posterior superior palpebral conjunctival tissue was found to be necrotic and friable upon double eversion. The tissue was biopsied and demonstrated amyloid deposition with positive congo red staining (Figure 2). Systemic work up was negative. Case 2 is a 74-year old man with a history of right upper eyelid ptosis with MRD of 2mm on the right and 3.5mm on the left. Just as in case 1 the patient had no other periorbital or conjunctival abnormalities. During double eversion for right conjunctivo-mullerectomy a friable mass was noted deep within the superior conjunctival fornix. Biopsy revealed amyloid dispersed throughout the tissue, with a positive Thioflavin stain. Systemic work up was negative for other systematic disease.
Only 4% of head and neck amyloid involve the orbit and the vast majority of periorbital cases involve the conjunctiva and eyelid. Periorbital signs commonly seen include conjunctival deposits or soft tissue nodular deposits. Classically, eyelid amyloid implies systemic disease, while conjunctival amyloid suggests no systemic involvement. Ptosis can be a presenting sign of amyloidosis but it is almost always associated with other signs or symptoms. Myogenic infiltration of the levator muscle has been reported in one patient with amyloid who underwent a levator resection. Our patients underwent mullerectomy hence no levator specimens were available for review.
These are the first two cases to describe amyloidosis masquerading as isolated ptosis without any other signs. Eyelid eversion during pre-op evaluation and careful examination during double eversion at the time of mullerectomy surgery may help in detecting any unusual entities such as amyloidosis.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.
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