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BRIEF COMMUNICATION
Year : 2020  |  Volume : 8  |  Issue : 2  |  Page : 67-69

Unilateral localized amyloidosis of levator palpebrae superioris muscle causing blepharoptosis


Department of Orbit and Oculoplasty, Sankara Eye Hospital, Bengaluru, Karnataka, India

Date of Submission12-Jun-2019
Date of Decision07-Aug-2019
Date of Acceptance19-Nov-2019
Date of Web Publication2-Jul-2020

Correspondence Address:
Saptagirish Rambhatla
Department of Orbit and Oculoplasty, Sankara Eye Hospital, Kundalahalli Gate, Varthur Main Road, Bengaluru - 560 037, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcor.jcor_49_19

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  Abstract 


We report a rare case of unilateral localized amyloidosis of levator palpebrae superioris (LPS) muscle causing blepharoptosis. A 71-year-old male presented with a history of gradually progressing left upper eyelid ptosis. LPS resection was planned and the resected unusually thickened muscle was sent for histopathological examination which revealed amyloidosis. Investigations excluded systemic involvement. Amyloidosis should be considered in the differential diagnosis of aponeurotic ptosis.

Keywords: Amyloidosis causing ptosis, levator palpebrae superioris, localized amyloidosis


How to cite this article:
Patel A, Kumari M, Rambhatla S. Unilateral localized amyloidosis of levator palpebrae superioris muscle causing blepharoptosis. J Clin Ophthalmol Res 2020;8:67-9

How to cite this URL:
Patel A, Kumari M, Rambhatla S. Unilateral localized amyloidosis of levator palpebrae superioris muscle causing blepharoptosis. J Clin Ophthalmol Res [serial online] 2020 [cited 2020 Aug 5];8:67-9. Available from: http://www.jcor.in/text.asp?2020/8/2/67/288845



Amyloid is derived from the Latin word “amylum” meaning “starch-like” and was coined by Rudolph Virchow in 1854. Amyloidosis is accumulation of abnormal proteinaceous material (amyloid) in extracellular and/or intracellular spaces in variety of tissues.[1]

In humans, more than 20 different proteins are known to cause amyloidosis.[2] It could be hereditary or acquired. It can manifest as a systemic disease or organ-limited or as localized involvement.[3]

Amyloidosis within the orbit is rare.[4] It can affect any part of the eye, adnexa, and orbital tissues presenting as yellowish subconjunctival mass or an orbital mass or subconjunctival hemorrhage or eyelid thickening and blepharoptosis.[5]

This report describes a rare case of unilateral ptosis due to localized amyloid deposition in levator palpebrae superioris (LPS). Earlier reports were mainly of combined involvement of conjunctiva, tarsus, LPS, and adnexa.


  Case Report Top


A 71-year-old otherwise healthy male presented with a history of drooping of left upper eyelid for the last 10 years which gradually worsened. There was no history of any ocular or systemic disease in the past and no history of ocular trauma, ocular surgery, or contact lens wear. The patient had 20/20 vision in both eyes with normal intraocular pressure. Palpebral fissure height was 10 mm in the right eye and 5 mm in the left eye. Margin reflex distance 1 was 4 mm on the right and 0 mm on the left side. Eyelid crease height was 10 mm on the right side, but on the left side, multiple skin folds were present. Levator action was 15 mm on the right and 10 mm on the left side. The rest of the ocular findings including tarsal and bulbar conjunctiva were unremarkable. Diagnosis of severe involutional ptosis due to LPS dehiscence with good LPS action in the left eye was made and the left eye LPS reattachment was advised under local anesthesia [Figure 1].
Figure 1: Preoperative photograph. Blepharoptosis of the left eye

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When performing LPS reattachment, LPS was noted to be thicker than usual for that age. Hence, the resected LPS was sent for histopathological examination [Figure 2].
Figure 2: Intraoperative photograph. Left eye-thickened levator palpebrae superioris exposed showing amyloid deposition

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Histologically, muscle showed large amorphous deposits of amyloid, which after Congo red staining demonstrated apple green birefringence in polarized light confirming the diagnosis of amyloid infiltration of LPS [Figure 3] and [Figure 4].
Figure 3: Tissue section of resected levator palpebrae superioris muscle. Hematoxylin–eosin staining shows acellular eosinophilic deposits in the muscle and around vessels

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Figure 4: Apple green birefringence under polarized light with Congo red staining

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Postoperative period was uneventful except for undercorrection of ptosis. The patient underwent systemic evaluation by an internist and there was no evidence of systemic amyloidosis.


  Discussion Top


Only 4% of localized amyloidosis of the head and neck region occurs in the orbit. In 1871, localized ocular amyloidosis was reported for the first time. Macoul and Winter reported a case of ocular manifestation in systemic amyloidosis in 1968.[5] There are some reports of amyloidosis as a cause of ptosis in the literature.[3] Conjunctiva is the most commonly affected site in primary localized amyloidosis of eye.[6]

Our patient presented with unilateral ptosis for many years which was initially thought to be involutional. Ocular motility was normal. No visible or palpable mass except for the thickened LPS was noted while operating. Histopathology of the resected LPS was positive for amyloidosis.

The amyloid deposition in LPS probably led to mechanical and myogenic blepharoptosis.[3],[7] Dehiscence of levator aponeurosis might be a contributing factor.

There are only two case reports suggesting localized amyloidosis involvement of resected levator muscle. Almost all have reported combined involvement of conjunctiva, LPS, tarsus, adnexa presenting with a mass; or ptosis or proptosis. Liesegang in 1983 reported a 50-year-old female with a 2-year history of unilateral ptosis. Slit-lamp examination was unremarkable. The levator muscle was enlarged, with biopsy revealing amyloidosis. Subsequent clinical and laboratory tests yielded no further evidence of amyloid.[3]

Dithmar et al. in 2004 reported a case describing unilateral ptosis in a 32-year-old female without clinically detectable amyloid deposits within the conjunctiva or elsewhere at initial presentation. The patient underwent ptosis correction elsewhere and 7 years later presented with recurrence developing clinically evident conjunctival deposits. Resected LPS and conjunctival biopsy showed amyloidosis.[3]

An unidentified, restricted immunologic disorder could be the reason for localized amyloidosis, but the exact reason remains unknown.[8] Systemic neoplastic plasma cell disease should be excluded, even in localized amyloidosis after histopathological confirmation. The nature of amyloid material can be determined using diagnostic proteomic analysis by chromatography–electrospray tandem mass spectrometry. The amyloid types in tissue have been determined using immunohistochemistry, and the results are often difficult to interpret.

There are some descriptions of computed tomography appearance of orbital amyloidosis, especially when lacrimal gland and extraocular muscles are involved.[3],[9] Changes in adjacent bones can be seen as focal thinning or thickening with punctate calcifications.[5],[9] Significant ossification and calcification are probably due to chronicity. Documented magnetic resonance imaging reports in focal amyloidosis are very few. Hypointense signal is observed in the lesion on T2-weighted images.

Anterior approach levator advancement or resection is treatment of choice in such a condition. This spares the conjunctiva from postoperative scarring. Various treatment modalities such as conservative local excision debulking, cryotherapy, superficial cobalt therapy, and radiotherapy all were reported with partial success.[3],[5],[9]


  Conclusion Top


There are reports of rare recurrence [3],[5],[9] with persistent ocular complaints. This can be attributed to conservative local excision and persistent disease. A suspicious looking or feeling LPS aponeurosis should be subjected to histopathological examination. Amyloidosis should be considered in the differential diagnosis of aponeurotic ptosis.[3]

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Chuang E, Hori AM, Hesketh CD, Shorter J. Amyloid assembly and disassembly. J Cell Sci 2018;131. pii: jcs189928.  Back to cited text no. 1
    
2.
Buxbaum JN. The systemic amyloidoses. Curr Opin Rheumatol 2004;16:67-75.  Back to cited text no. 2
    
3.
Dithmar S, Linke RP, Kolling G, Völcker HE, Helmke B. Ptosis from localized A-lambda-amyloid deposits in the levator palpebrae muscle. Ophthalmology 2004;111:1043-7.  Back to cited text no. 3
    
4.
Murdoch IE, Sullivan TJ, Moseley I, Hawkins PN, Pepys MB, Tan SY, et al. Primary localised amyloidosis of the orbit. Br J Ophthalmol 1996;80:1083-6.  Back to cited text no. 4
    
5.
Tari AS, Eshraghi B, Hashemian H, Amoli FA. Primary localized conjunctival amyloidosis presenting with unilateral ptosis. Iran J Ophthalmol 2010;22:46-51.  Back to cited text no. 5
    
6.
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. 6
    
7.
Liesegang TJ. Amyloid infiltration of the levator palpebrae superioris muscle: Case report. Ann Ophthalmol 1983;15:610-3.  Back to cited text no. 7
    
8.
Borodic GE, Beyer-Machule CK, Millin J, Conte J, Foster CS. Immunoglobulin deposition in localized conjunctival amyloidosis. Am J Ophthalmol 1984;98:617-22.  Back to cited text no. 8
    
9.
Al Hussain H, Edward DP. Anterior orbit and adnexal amyloidosis. Middle East Afr J Ophthalmol 2013;20:193-7.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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