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CASE REPORT
Year : 2013  |  Volume : 1  |  Issue : 1  |  Page : 48-50

Presentation of conjunctival amyloidosis as bilateral ptosis and cicatricial entropion


Sankara Eye Care Institutions, Sivanandapuram, Coimbatore, India

Date of Web Publication3-Jun-2013

Correspondence Address:
Kumar Sambhav
Sankara Eye Centre, Sathy Road, Sivanandapuram, Coimbatore
India
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DOI: 10.4103/1658-631X.112934

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  Abstract 

Primary localized amyloidosis of the conjunctiva is a rare occurrence. This case report describes a 35-year-old male, who presented with drooping of both upper eyelids and a pricking sensation in the left eye. Examination of the conjunctiva showed granular swellings. The biopsy revealed amyloid deposition. Systemic amyloidosis was ruled out on further investigation. To our knowledge, this is the first report of amyloidosis of the conjunctiva and tarsus, presenting as ptosis with entropion, from India.

Keywords: Conjunctival amyloidosis, entropion, ptosis


How to cite this article:
Sambhav K, Bhandari V, Ajith H. Presentation of conjunctival amyloidosis as bilateral ptosis and cicatricial entropion. Saudi J Med Med Sci 2013;1:48-50

How to cite this URL:
Sambhav K, Bhandari V, Ajith H. Presentation of conjunctival amyloidosis as bilateral ptosis and cicatricial entropion. Saudi J Med Med Sci [serial online] 2013 [cited 2017 May 23];1:48-50. Available from: http://www.sjmms.net/text.asp?2013/1/1/48/112934


  Introduction Top


Amyloidosis constitutes of a spectrum of disorders characterized by the deposition of protein in the tissues of single or multiple organs. In this disorder, normally soluble proteins become insoluble and are deposited in the extracellular space of various organs or tissues, disrupting normal function. The insoluble fibrous protein aggregates that develop in amyloidosis are known as amyloids. They result from a change in the secondary structure of the protein, which causes the protein to take on a particular aggregated insoluble form, similar to the beta-pleated sheet. Symptoms vary widely depending upon where the amyloid deposits accumulate in the body. The deposition of amyloid fibrils causes toxicity of cells and dysfunction of organs. Amyloidosis maybe inherited or acquired. There are numerous ocular manifestations of systemic and localized amyloidosis. Amyloidosis affects the eyelids, conjunctiva, lacrimal gland, rectus muscles, vitreous humor, and anterior chamber angle. [1]

Conjunctival amyloidosis is thought to be the manifestation of a local immunologic disorder, and affected patients rarely have systemic amyloidosis. [2] This is a rare case report of conjunctival amyloidosis presenting as bilateral ptosis and entropion of the left lower lid.


  Case Report Top


A 35-year-old male presented to us with progressive drooping of the upper lids in both eyes since three years. It was associated with discomfort and a pricking sensation in the left eye. Detailed history did not reveal any preceding ocular trauma or other chronic ophthalmic complaint. On examination, the best corrected visual acuity was 20/20 both eyes. The patient had bilateral ptosis with a palpebral aperture height of 6 mm on both sides. The upper lids on either side were bulky with thickened tarsal plate; the levator function was 10 mm on both sides. The left lower lid had entropion of grade 3. There was the presence of multiple, firm, waxy, polypoid subconjunctival lesions [Figure 1]. Biopsy of the conjunctival and tarsal plates revealed acellular, eosinophilic, hyaline material beneath the epithelium. On Congo red staining, the material stained brick red color and showed a green birefringence with polarized light [Figure 2]. Examination of the extraocular muscles, eyeball, retrobulbar area, and optic nerve was normal.
Figure 1: Multiple subconjunctival lesions

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Figure 2: Histopathological section stained with Congo red and viewed under polarized light showing the apple green birefringence

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The patient underwent further diagnostic evaluation. The workup included complete blood count, urine analysis, urine protein electrophoresis, and ultrasound of the abdomen. All reports were within normal limits, ruling out systemic disease. The patient was referred to a physician to rule out any other systemic feature of amyloidosis, after which a diagnosis of isolated conjunctival amyloidosis with tarsal involvement was made.

Patient was treated with modified Wheeler's operation for entropion of the left lower lid. The entropion was corrected and patient was relieved of his symptoms. The patient was reluctant to undergo ptosis surgery as ptosis did not involve the visual axis.


  Discussion Top


Amyloidosis of the conjunctiva is a rare entity. Various ocular structures maybe involved in systemic as well as localized amyloidosis. [1],[3] Adnexal involvement that includes skin deposition is a part of a systemic disorder, until proved otherwise. The involvement that spares the skin is probably a localized disease. [1] Conjunctival amyloidosis is loosely defined in the literature and seems to include various combinations of amyloidosis involving the conjunctiva, tarsal plate, orbicularis muscle, levator muscle, and Tenon's capsule.

The common presenting features of amyloidosis include conjunctival mass, ptosis, swelling of lid, and subconjunctival hemorrhage. [2],[4],[5] The clinical picture is often confused with a neoplasm or granuloma, when the disease affects the conjunctiva, lids, or orbit. [4] Primary localized conjunctival amyloidosis presents in middle age and is often bilateral, and the lesions are typically described as solitary or multiple, firm, rubbery, well-vascularized, waxy-appearing, painless, fusiform or polypoid subconjunctival elevations. [5] The eyelids are diffusely thickened due to infiltration of the muscular and subcutaneous layers. [5] Biopsy of the tissue in amyloidosis shows eosinophilic deposits with hematoxylin and eosin staining. Amyloid takes up brick red color with Congo red staining and shows apple green birefringence with polarized light which is pathognomonic of the disease. [6]

There has been little literary discussion on the management options and operative approaches for ptosis and entropion due to amyloidosis. [5] Small localized lesions causing ptosis have been treated by en bloc resection, cryotherapy, and superficial cobalt therapy. [3],[5] Advanced amyloid deposits can be treated by debulking the mass, preserving the anatomical planes of the eyelid and thus preserving normal eyelid tissues and preventing hemorrhagic sequelae. [5] One of the characteristic features of amyloidosis is its tendency to bleed which was not seen in our case.

 
  References Top

1.Daniel MA, Federick AJ, Dmitri TA, Evangelos SG, Susan MP, Nancy LR. Principles and Practice of ophthalmology. 2 nd ed. Philedelphia: W. B Saunders; 2000. p. 4636-53.  Back to cited text no. 1
    
2.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. 2
    
3.Rodrigues G, Sanghvi V, Lala M. Conjunctival amyloidosis of both eyelids. Indian J Ophthalmol 2001;49:116-7.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
4.Smith ME, Zimmerman LE. Amyloidosis of the eyelid and conjunctiva. Arch Ophthalmol 1966;75:42-51.  Back to cited text no. 4
    
5.Hubbard AD, Brown A, Bonshek RE, Leatherbarrow B. Surgical management of primary localised conjunctival amyloidosis causing ptosis. Br J Ophthalmol 1995;79:707.  Back to cited text no. 5
    
6.Bennhold H. Eine specifische amyloidfärbung mit kongorot, Münchener Medizinische Wochenschrift, 1922;44:1537.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2]



 

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