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   Table of Contents      
Year : 2019  |  Volume : 67  |  Issue : 12  |  Page : 1929

Acquired progressive unilateral blepharoptosis

1 Oculoplasty, Orbit and Ocular Oncology Services, HORUS Specialty Eye Care, Bengaluru; Oculoplasty, Orbit and Ocular Oncology Services, Amardeep Eye Care Centre, Kollam, Kerala, India
2 Oculoplasty, Orbit and Ocular Oncology Services, HORUS Specialty Eye Care, Bengaluru, India

Date of Web Publication22-Nov-2019

Correspondence Address:
Dr. Fairooz P Manjandavida
Oculoplasty, Orbit and Ocular Oncology, HORUS Specialty Eye Care, Bengaluru - 560 078, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1988_19

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How to cite this article:
Manjandavida FP, Chahar S. Acquired progressive unilateral blepharoptosis. Indian J Ophthalmol 2019;67:1929

How to cite this URL:
Manjandavida FP, Chahar S. Acquired progressive unilateral blepharoptosis. Indian J Ophthalmol [serial online] 2019 [cited 2020 Aug 10];67:1929. Available from: http://www.ijo.in/text.asp?2019/67/12/1929/271372

  Case Report Top

A 37-year-old healthy lady was referred for left eye (OS) upper lid ptosis correction with history of gradually progressive upper lid drooping for 2 years and, an episode of subconjunctival hemorrhage. There was no history of trauma, allergy, diurnal variation or generalized weakness. Best-corrected visual acuity was 20/20 in both eyes (OU). External ocular examination revealed significant blepharoptosis in OS [Figure 1]a. Eversion of the upper lid revealed a yellowish-pink sub-conjunctival lesion throughout the length of the upper edge of the tarsus [Figure 1]b. Anterior segment evaluation of right eye (OD) showed a yellowish-pink subconjunctival mass near the caruncle, extending into medial bulbar conjunctiva and inferior fornix [Figure 1]c and [Figure 1]d. Fundus evaluation was unremarkable.
Figure 1: (a) Showing significant blepharoptosis of left eye (OS) in a 37-years-old lady. (b) On eyelid eversion there is yellowish pink sub-conjunctival lesion visible in the upper border of the tarsus extending into the upper fornix. (c and d) Similar appearing lesions are seen in the right eye (OD) involving the caruncle, medial bulbar and inferior forniceal conjunctiva

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  What Is Your Next Step? Top

  1. Observe with regular follow-up
  2. Blepharoptosis correction OS
  3. Incision biopsy OU
  4. Excision biopsy and cryotherapy OU.

  Findings Top

In the presence of yellowish-pink subconjunctival lesion with an episode of subconjunctival hemorrhage, our clinical suspicion was conjunctival amyloidosis. Complete excision of the lesion was performed in OD followed by cryotherapy. Careful debulking to preserve LPS function and cryotherapy was done in OS. Histopathological examination revealed a positive Congo red staining of the specimen with apple green birefringence in polarized light, which is pathognomic of amyloidosis. Systemic evaluation was within normal limits.

  Diagnosis: Top
Bilateral Primary Localized Conjunctival Amyloidosis.

  Correct Answer: Top

  Discussion Top

This case highlights the importance of bilateral detailed ocular examination in all cases of blepharoptosis. Upper lid eversion in a droopy eyelid aided in the clinical diagnosis.

Amyloidosis results from the localized (one organ) or systemic (multiple organ) deposition of amyloid fibrils in the extracellular spaces. Three major types include: primary (AL: amyloid light chain), secondary (AA-amyloid A), and heredofamilial (ATTR- amyloid transthyretin).[1] Ocular adnexal amyloidosis accounts for 4% of the localized cases. Most common sites involved are eyelids, orbit and conjunctiva presenting as conjunctival mass, ptosis or subconjunctival hemorrhage.[2] Misdiagnosis as ocular surface malignancy is common.[1] Excision or debulking with cryotherapy, depending on extent of the amyloid deposit is the treatment of choice. Systemic amyloidosis can involve heart, liver, kidney, nerves and gastrointestinal tract, causing vital organ dysfunction and mortality. Ocular findings can be the initial manifestation of life threatening systemic involvement in 10% that should be ruled out in all cases.[1],[3]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Demirci H, Shields CL, Eagle RC Jr, Shields JA. Conjunctival amyloidosis: Report of six cases and review of the literature. Surv Ophthalmol 2006;51:419-33.  Back to cited text no. 1
Eneh AA, Farmer J, Kratky V. Primary localized orbital amyloid: Case report and literature review; 2004-2015. Can J Ophthalmol 2016;51:e131-6.  Back to cited text no. 2
Leibovitch I, Selva D, Goldberg RA, Sullivan TJ, Saeed P, Davis G, et al. Periocular and orbital amyloidosis: Clinical characteristics, management, and outcome. Ophthalmology 2006;113:1657-64.  Back to cited text no. 3


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