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CASE REPORT Table of Contents  
Ahead of print publication
Venous malformation of the conjunctiva and orbit


 Department of Ophthalmology, Jawaharlal Postgraduate Medical Education and Research, Puducherry, India

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Date of Submission18-May-2021
Date of Decision19-Jul-2021
Date of Acceptance19-Jul-2021
Date of Web Publication24-May-2022
 

   Abstract 


A 28-year-old male presented with a large conjunctival lobulated, nonpulsatile, red vascular lesion involving a large part of the temporal quadrant of the left globe. There was no proptosis or globe displacement, but the left eye abduction was limited. T2-weighted magnetic resonance imaging scan of the brain and orbit revealed an extensive contrast-enhancing lobulated lesion in the left half of the face involving the upper lip, cheek, oral cavity, extraconal space of left orbit, and nasal cavity. He underwent surgical excision of the conjunctival lesion with amniotic membrane reconstruction.

Keywords: Conjunctiva, orbit, venous malformation


How to cite this URL:
Devaraj A, Gera P, Swathi S, Kasturi N. Venous malformation of the conjunctiva and orbit. Indian J Pathol Microbiol [Epub ahead of print] [cited 2022 Dec 9]. Available from: https://www.ijpmonline.org/preprintarticle.asp?id=345873





   Case Top


A 28-year-old male presented with a painless fleshy growth in the left eye and cheek region since birth. The swelling in the left eye increased in size, recently, hindering lid closure. He underwent treatment for the cheek lesion with oral steroids, sclerotherapy, and local excision with reconstruction. Ophthalmological examination revealed the best-corrected visual acuity of 6/6 in both eyes. A 3 × 2 cm soft, lobulated mass was seen in the left upper eyelid. Multiple fleshy vascular masses were noted in the bulbar conjunctiva of the left eye of different sizes, largest measuring 2 × 1.5 cm, causing lagophthalmos, and mechanical abduction restriction [Figure 1]a and [Figure 1]b. There was no exposure keratitis, and intraocular pressure measured 11 mm Hg in the left eye. Anterior segment examination in the right eye and dilated fundus examination in both eyes were within normal limits. There was no palpable periauricular or cervical lymphadenopathy. Contrast-enhanced magnetic resonance (MR) imaging of the brain, orbit, and face showed large homogeneously enhancing lobulated extensive vascular lesion in the left half of the face, including upper lip, nasal ala, cheek, buccal space, masticator space, buccal mucosa, the roof of the oral cavity, oropharynx, nasopharynx, left parapharyngeal space, infratemporal fossa, frontal scalp, intra and extraconal space of left orbit, and left nasal cavity associated with multiple phleboliths suggestive of venous malformation [Figure 2]a and [Figure 2]b. Post-valsalva shows an increase in the size of the left orbital lesion. Time-resolved imaging of contrast kinetics–MR angiogram showed intense pooling of contrast in the early venous phase in the oral cavity and parts of frontal and cheek lesions.
Figure 1: (a and b) Clinical photograph of the left eye showing a vascular mass in the temporal bulbar conjunctiva and (c) following excision

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Figure 2: (a and b) MRI scan coronal and axial image showing a lobulated extensive vascular lesion in the left half of the face extending into the intraconal and extraconal spaces of left orbit

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Considering the extensive nature of the lesion and involvement, and as total excision is not possible, a decision was made to perform primary excision of the conjunctival lesion with an amniotic membrane graft [Figure 1]c. The excised specimen sent for histopathological examination showed a vascular lesion composed of multiple ectatic thin-walled vessels filled with RBCs and fibrin. These vessels are situated in the superficial stroma extending in to the deep stroma. The overlying epithelium is thinned out. Features are consistent with vascular malformations – venous hemangioma [Figure 3].
Figure 3: Histopathology image showing hematoxylin–eosin stained (×400) vascular lesion composed of multiple ectatic thin-walled vessels filled with RBCs and fibrin

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   Discussion Top


Vascular anomalies of the eyelid and orbit can be broadly divided into malformations, shunts, and neoplasms.[1] Vascular malformations are developmental anomalies of blood vessels caused by errors of vasculogenesis during embryogenesis. Since malformations occur because of an event in time, they may be purely venous, arterial, lymphatic, or capillary or consist of any combination, and they may occur in multiple sites.[2]

Venous vascular malformations (VVMs) are either segmental irregular dilatations of the venous out-flow system or a tangled mass of abnormal venous channels with or without dysmorphic out-flow channels. Distensible VVM like orbital varices has large systemic venous connections and respond to changes in venous pressure.[1],[2] Patients complain of proptosis and pain that increase with activities such as straining, bending forward, or Valsalva maneuver. Nondistensible VVM includes little or small functioning systemic venous connections and are characterized more by stagnant blood flow, which leads to thrombosis or hemorrhage. Vascular anomalies of the conjunctiva are rare and account for 4% of the nonmelanocytic tumors.[3] Histopathologically, they are composed of poorly defined blood-filled channels of variable diameter with mature, flattened endothelial cells and attenuated muscle in which thrombi, calcifications, and fibrovascular invasion can be seen. The absence of an internal elastic membrane along with scant, disorganized smooth muscle identifies the venous origin. It must be differentiated from a venous angioma which is an acquired lesion consisting of the proliferation of venous channels with prominent muscular walls.[4]

The indications for treatment are usually related to pain, cosmesis, or progressive expansion. The enlargement is probably due to extravasation of blood or spontaneous thrombosis followed by an organization with focal intravascular endothelial hyperplasia or proliferation.[4] The vascularity of these lesions to be reduced prior to surgical excision. It includes retrograde venous catheterization and embolization with platinum microcoils and intraoperative venography and controlled glue embolization followed by excision of the glued mass.[5],[6] The embolization can be done with a mixture of n-butyl-2-cyanoacrylate, lipoidal, and tantalum powder producing a radio-opaque cast of the malformation, which can then be excised in a relatively blood-free manner. Bleomycin sclerotherapy has been reported to be a safe and effective treatment for eyelid and orbital venous malformations.[7] Surgical excision is indicated in extreme orbital pressure with a functional deficit and cosmetic disfigurement.


   Conclusion Top


Venous malformation of the conjunctiva and orbit is extremely rare. It should be considered in the differential diagnosis of vascular lesions of the conjunctiva.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Shields JA, Mashayekhi A, Kligman BE, Kunz WB, Criss J, Eagle RC Jr, et al. Vascular tumors of the conjunctiva in 140 cases. Ophthalmology 2011;118:1747-53.  Back to cited text no. 1
    
2.
Hamza E, Fadoua A. Conjunctival venous malformations: A case report. Pan Afr Med J 2015;22:129.  Back to cited text no. 2
    
3.
Shields CL, Demirci H, Karatza E, Shields JA. Clinical survey of 1643 melanocytic and nonmelanocytic conjunctival tumors. Ophthalmology 2004;111:1747-54.  Back to cited text no. 3
    
4.
Jakobiec FA, Werdich XQ, Chodosh J, Freitag SK. An analysis of conjunctival and periocular venous malformations: Clinicopathologic and immunohistochemical features with a comparison of racemose and cirsoid lesions. Surv Ophthalmol 2014;59:236-44.  Back to cited text no. 4
    
5.
Lacey B, Rootman J, Marotta TR. Distensible venous malformations of the orbit: Clinical and hemodynamic features and a new technique of management. Ophthalmology 1999;106:1197-209.  Back to cited text no. 5
    
6.
Hage AN, Chick JF, Srinivasa RN, Bundy JJ, Chauhan NR, Acord M, et al. Treatment of venous malformations: The data, where we are, and how it is done. Tech Vasc Interv Radiol 2018;21:45-54.  Back to cited text no. 6
    
7.
Shigematsu T, Sorscher M, Dier EC, Berenstein A. Bleomycin sclerotherapy for eyelid venous malformations as an alternative to surgery or laser therapy. J Neurointerv Surg 2019;11:57-61.  Back to cited text no. 7
    

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Correspondence Address:
Nirupama Kasturi,
Department of Ophthalmology, Jawaharlal Postgraduate Medical Education and Research, Puducherry - 605006
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_490_21



    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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    -  Gera P
    -  Swathi S
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