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   Table of Contents      
CASE REPORT
Year : 2019  |  Volume : 6  |  Issue : 2  |  Page : 51-54

Unilateral blindness after percutaneous injection sclerotherapy of orbital venous malformation presenting as orbital cellulitis


Department of Diagnostic and Interventional Neuroradiology, Lahore General Hospital, Lahore, Pakistan

Date of Submission30-Apr-2020
Date of Acceptance07-Aug-2020
Date of Web Publication27-Nov-2020

Correspondence Address:
Dr. Saima Ahmad
Department of Diagnostic and Interventional Neuroradiology, Pakistan Institute of Neurosciences, Lahore General Hospital, Ferozpur Road, Lahore
Pakistan
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DOI: 10.4103/bijo.bijo_1_20

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  Abstract 


This study pertains to a case of central retinal artery occlusion (CRAO) and complete unilateral blindness after intralesional injection of a sclerosing agent into orbital venous malformation (VM) presenting as orbital cellulitis. A 20-year-old female underwent a direct injection of 6 mg of bleomycin with a 23G needle into an orbital VM infiltrating the retrobulbar area and compressing the optic nerve. She was discharged without any complication after 24 h but reported back after 15 days with complete loss of vision in the right eye. Her visual acuity in the right eye was reduced only to perception of light. Fundoscopy of the right eye revealed signs of CRAO. Magnetic resonance imaging of the orbit showed a decrease in the size and enhancement of orbital VM. After 2 weeks, her visual acuity remained up to light perception. Permanent visual loss should be kept in mind as a disastrous complication of sclerosing therapy in a patient with orbital and paraorbital VM encroaching the retrobulbar area. In addition, orbital venolymphatic malformations may present as orbital cellulitis.

Keywords: Bleomycin, orbital cellulitis, sclerotherapy


How to cite this article:
Ahmad S. Unilateral blindness after percutaneous injection sclerotherapy of orbital venous malformation presenting as orbital cellulitis. Albasar Int J Ophthalmol 2019;6:51-4

How to cite this URL:
Ahmad S. Unilateral blindness after percutaneous injection sclerotherapy of orbital venous malformation presenting as orbital cellulitis. Albasar Int J Ophthalmol [serial online] 2019 [cited 2021 Sep 19];6:51-4. Available from: https://www.bijojournal.org/text.asp?2019/6/2/51/301679






  Introduction Top


Ocular complications following nonocular surgeries rarely occur, but the outcomes are devastating. Among all the surgeries carried out, the incidence of perioperative visual loss after nonocular procedures is 0.013%.[1] The main causes of sudden visual loss after nonocular surgery are central retinal artery occlusion (CRAO) and ischemic optic neuropathy. This case report presents a unique finding of such visual loss after 15 days post procedure. Sclerotherapy involves percutaneous sclerosants injected into the vascular lesions under imaging guidance. Bleomycin is a cytotoxic, antineoplastic antibiotic that acts on endothelial cells, which causes sclerosis and fibrosis.[2] CRAO and ophthalmoplegia following sclerotherapy of orbital venous malformations (VMs) are rare. In this report, we describe a patient with orbital VM who presented with recurrent attacks of inflammation and developed unilateral visual loss after a single session of percutaneous injection sclerotherapy with bleomycin.


  Case Report Top


A 20-year-old female developed diminution of vision in the right eye 15 days after intralesional injection of a sclerosing agent into the right orbital and retro-orbital VM. Initially, she presented with recurrent attacks of inflammation and was diagnosed as a case of orbital cellulitis by an ophthalmologist and was treated unsuccessfully using steroids, but no improvement was seen. Later, she was referred to a neurointerventionalist who found this to be an orbital VM by the joint decision of a team comprising interventional neuroradiologists and ophthalmologists. The treatment regimen prescribed was a direct percutaneous intralesional injection of six units of bleomycin with a butterfly needle under fluoroscopic guidance. Prior to the injection, digital subtraction angiogram was performed to see any communication with intraorbital veins. Obscuration of vision progressed after the 15th day of discharge and commenced within 24 h completely. Details are summarized in [Table 1].
Table 1: Clinical and radiological features of orbital venous malformation, treatment, and outcome

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The patient gave a history of three episodes of amaurosis fugax during this period of 2 weeks, but no other significant history of tinnitus, headache, weakness of either side of the body, loss of consciousness, or fever was reported. On examination which was carried out on the 15th day of injection sclerotherapy, the patient was conscious and oriented. Visual acuity in the left eye was normal. The perception of light was positive in the affected eye, however there was no projection of light. Rapid afferent pupillary defect was positive in the right eye. The axial proptosis had also improved from 25 to 18 mm in the right eye. There was no ptosis, and extraocular movements were intact in both eyes. Intraocular pressure was normal in both eyes [Figure 1], [Figure 2].
Figure 1: (a) A 20-year-old female with right orbital venous malformation causing ocular dystopia. (b) Magnetic resonance imaging coronal T1 and axial T2 short-tau inversion recovery images of the orbit showing a lobulated lesion occupying the intraconal compartment extending from the retrobulbar area and compressing the optic nerve, and hypointense to orbital fat on T1 and hyperintense on short-tau inversion recovery images suggesting venous malformation. (c) Digital subtraction angiogram showing abnormally dilated venous channels opacified by contrast injection through percutaneous approach by a 23G butterfly needle. (d) Magnetic resonance imaging axial T2-weighted image obtained after central retinal artery occlusion showing a marked decrease in the size of orbital venous malformation and proptosis. (e) Follow-up image after 7 months showing a complete reduction in proptosis

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Figure 2: Follow-up image after 7 months showing a complete reduction in proptosis

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Fundus examination revealed marked retinal pallor with a cherry-red spot on the right side. There were occluded arteries and small infarcts of cotton wool type, suggesting CRAO. The retina on the left was normal. In view of these findings, the patient was diagnosed to have retinal artery occlusion in the right eye. The patient was advised to lay supine and start doing ocular massage, and oxygen therapy was also administered.

A complete blood count, kidney and liver function tests, and C-reactive protein were carried out, which were found to be unremarkable. Echocardiography and carotid Doppler were done to find other sources of emboli, but turned out to be unremarkable. Magnetic resonance imaging of the brain was done, which showed a decrease in the size of orbital VM with decreased enhancement pattern, however no other findings were obvious.


  Discussion Top


Bleomycin is one of the most commonly used sclerosing agents for head-and-neck VMs. Bleomycin was first developed as an antineoplastic antibiotic in 1966. The mechanism involves damage to the endothelial cells with a nonspecific inflammatory reaction and occlusion of vessels.[3] The sclerosants induce endothelial damage within 15 min. It leads to activation of ophthalmic autoregulation process and subsequent thrombus formation. The thrombus becomes adherent to the wall of endothelium within 2–3 h after injection. The organization of fibrosis would take place in 60 days depending on the dose given.[3]

Sclerotherapy is considered a safe and effective treatment for head-and-neck and extremity VMs.[4] In orbital and paraorbital VMs, if lesion extends into the orbit and compresses the optic nerve, then the risk of developing ocular complications is high. Alireza Dehghaniet al., reported their findings of ophthalmic artery occlusion following head-and-neck VM after sclerosing therapy.[1]

Intense inflammatory signs are not common in low-flow VMs. Sudden chemical changes in patients with orbital VMs are usually related to orbital hemorrhage. There were only two cases reported by Alicia Galindo– Ferreiraet al. in 2016[5],[6] in 2001 with atypical presentation of orbital lymphatic malformation with orbital cellulitis; the third such case is being reported by us in this study. In both cases, the patients developed recurrent attacks of orbital inflammation in the presence of orbital venolymphatic malformation, however no paranasal sinus infection was observed. In our case, the patient also presented with recurrent attacks of orbital inflammation in the presence of orbital and paraorbital VM, thus orbital VM may atypically present as a orbital cellulitis.

We believe that, in our study case, the patient had the same attacks of orbital inflammation superimposed with the underlying pathology of orbital VM and hence responded well to steroid therapy during acute phases, however the proptosis remained unresolved with medical therapy.

Obstruction of the ophthalmic artery is acute with simultaneous occlusion of both the retinal and choroidal circulations and has the following features: severe visual loss with minimal or no light perception; marked ischemic retinal whitening of the macula; small-to-complete disappearance of the cherry-red spot; no recordable electroretinogram; and late disturbances of the retinal pigment epithelium. Prolonged choroidal filling in the absence of a cherry-red spot should raise a suspicion of an obstruction of the ophthalmic artery.[1] Intra-arterial thrombolysis has been considered a treatment method in CRAO. Gijon Hwanget al. reported two patients diagnosed with CRAO treated with intra-arterial thrombolysis and achieved complete recanalization.[7]

In our patient, light of perception was present, however no projection was present. On day 1 fundoscopy, bright, cherry-red spot was observed with a pale disc, however the spot disappeared in the chronic phase. Small cotton wool spots were also evident, suggesting ganglionic cell death.

In our case, the possible causes of CRAO were as follows: reflux of sclerosants into the ophthalmic artery during percutaneous injection sclerotherapy, leading to ophthalmic artery endothelial damage, thrombus formation, and occlusion. Second, it is possible that postsclerotherapy edema formation due to sclerosants would have further compressed the optic nerve and ophthalmic artery in the presence of intraconal orbital VM, and another plausible explanation of delayed ophthalmic artery occlusion in our case is possibly due to fibrosis of VM, which subsequently occurred within 60 days. The proximity of VM and endothelium of the ophthalmic artery made it more susceptible to develop fibrosis along with VM. Multiple attacks of amaurosis fugax are in line with this possible hypothesis.

A study of literature shows very few such presentations, and this is one of the few reported cases of orbital VM, which presented as orbital cellulitis. In general, sclerotherapy is considered a safe treatment for VMs; however, any physician administrating sclerosants in orbital VM should be aware of this potential but lethal complication of blindness.

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.
Dehghani A, Rezaei L, Ghanbari H, Nasrollahi K, Tavakoli M. Ophthalmic artery occlusion following facial sclerosing therapy. J Ophthalmic Vis Res 2018;13:351-4.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Pimpalwar S. Vascular malformations: Approach by an interventional radiologist. Semin Plast Surg 2014;28:91-103.  Back to cited text no. 2
    
3.
Ahmad S, Akhtar FK. Percutaneous sclerotherapy of para-orbital and orbital venous malformation: A single center, case series. Phlebology 2019;34:355-61.  Back to cited text no. 3
    
4.
Yang Y, Sun M, Ma Q, Cheng X, Ao J, Tian L, et al. Bleomycin A5 sclerotherapy for cervicofacial lymphatic malformations. J Vasc Surg 2011;53:150-5.  Back to cited text no. 4
    
5.
Mames RN, Snady-McCoy L, Guy J. Central retinal and posterior ciliary artery occlusion after particle embolization of the external carotid artery system. Ophthalmology 1991;98:527-31.  Back to cited text no. 5
    
6.
Ferreiro AG, Alkatan HM, ElKhamary SM, AlDosairi S, Cruz AA. Recurrent Orbital Inflammation Mimicking Orbital Cellulitis Associated With Orbitopalpebral Venous Lymphatic Malformation, Ophthalmic Plast Reconstr Surg, Sep/Oct 2017;33:e112-e114.  Back to cited text no. 6
    
7.
Hwang G, Woo SJ, Jung C, Park KH, Hwang JM, Kwon OK. Intra-arterial Thrombolysis for Central Retinal Artery Occlusion: Two Cases Report, J Korean Med Sci 2010;v.25(6).  Back to cited text no. 7
    


    Figures

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