Review Article Evisceration in the Modern Age Laura T. Phan1,2, Thomas N. Hwang3, Timothy J. McCulley1,2 ABSTRACT Access this article online Website: Evisceration is an ophthalmic surgery that removes the internal contents of the eye www.meajo.org followed usually by placement of an orbital implant to replace the lost ocular volume. DOI: Unlike enucleation, which involves removal of the entire eye, evisceration potentially causes 10.4103/0974-9233.92113 exposure of uveal antigens; therefore, historically there has been a concern about sympathetic Quick Response Code: ophthalmic (SO) associated with evisceration. However, critical review of the literature shows that SO occurs very rarely, if ever, as a consequence of evisceration. Its clinical applications overlap with those of enucleation in cases of penetrating ocular trauma and blind painful eyes, but it is absolutely contraindicated in the setting of suspected intraocular malignancy and may be preferred for treatment of end-stage endophthalmitis. From a technical standpoint, traditional evisceration has a limitation in the orbital implant size. Innovations with scleral modification have overcome this limitation, and accordingly, due to its simplicity, efficiency, and good cosmetic results, evisceration has once again been gaining popularity. Key words: Endophthalmitis, Enucleation, Evisceration, Intraocular Tumors INTRODUCTION While the risk of sympathetic ophthalmia continues to be a contentious issue, evisceration has gained popularity in the visceration (removal of intraocular contents) and enucleation past few decades. This is based largely on the perception E(removal of the entire eye) are competing techniques, with that evisceration provides superior functional and cosmetic fluctuating favor since their inception. Enucleation may be the results compared to enucleation. Several modified evisceration oldest operation in ophthalmology; literature from as early as techniques have been described in past decades, each proclaiming 1 2600 BC described a Chinese “god of ocularists.” Centuries improved results.7-16 later in 1817, Bear introduced evisceration when he removed the remaining intraocular contents of an eye following an expulsive This review will provide an in-depth look at evisceration by hemorrhage.2 In 1874, Noyes described evisceration for the examining the published literature that supports and refutes its management of intraocular infection.3 In 1884, Mules described association with sympathetic ophthalmia, and then describing its placing a hollow glass sphere into the eviscerated cavity.4 advantages and disadvantages in different clinical scenarios, and Because evisceration unlike enucleation disrupts the integrity finally discussing the basic technique as well as recent technical of the globe, there is a theoretical risk of exposing uveal modifications. antigens, which could incite an autoimmune reaction known as sympathetic ophthalmia (SO) in the contralateral eye. The SYMPATHETIC OPHTHALMIA first report of sympathetic ophthalmia occurring in association with evisceration was in 1887.5 Despite this, evisceration gained Sympathetic ophthalmia is a potentially devastating autoimmune popularity until 1972 when Green et al. reignited the concern condition characterized by bilateral panuveitis, where the injured of inciting sympathetic ophthalmia with a report of four alleged eye incites inflammation in the fellow (sympathizing) eye.17 Its cases.6 specific pathophysiology remains elusive. It is believed to be an 1Wilmer Eye Institute, Johns Hopkins School of Medicine, Baltimore, 2King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia, 3Department of Ophthalmology, The Permanente Medical Group, Redwood City, California Corresponding Author: Dr. Timothy J McCulley, The Wilmer Eye Institute, Johns Hopkins School of Medicine, 600 North Wolfe Street, Wilmer 110, Baltimore, MD 21287. E-mail: [email protected] 24 Middle East African Journal of Ophthalmology, Volume 19, Number 1, January - March 2012 Phan, et al.: Evisceration in the Modern Age immunologic response to exposure of previously sequestered the clinical appearance; histopathologic confirmation was not tissue.17,18 Proposed antigens include retinal S-antigen, retinoid established. binding protein, melanin associated antigen, or those from the retinal pigment epithelium.18-21 Signs and symptoms of uveitis In 2006, Freidlin et al.32 reported the first case of sympathetic have been reported to develop between 5 days22,23 and 66 years24 ophthalmia found in a soldier since World War II. This 21-year- from the time of injury. Sixty-five percent of the cases occur old man sustained shrapnel wounds while in combat and between 2 weeks and 2 month with roughly 90% present within underwent evisceration the day of injury. Within a month of a year of injury.25-27 surgery, he developed panuveitis in his remaining eye with conjunctival injection, vitreous floaters, and paracentral scotoma. Evisceration, along with a number of other intraocular He responded favorably to immunosuppressant therapy. After procedures, has been implicated as a potential cause of 6 months of oral and topical steroid treatment, best-corrected sympathetic ophthalmia.6,28 Whether or not evisceration can visual acuity was 20/20 in this “sympathetic” eye. Interestingly, incite sympathetic ophthalmia is one of the most notorious histologically proven uveal tissue was found and removed from controversies in oculoplastic surgery.29 We will present what the subconjunctiva of the eviscerated eye at the onset of his evidence exists supporting and refuting this relationship. symptoms. The authors hypothesized that this residual uveal tissue induced the inflammation. Given the failure to remove Supportive evidence all uveal tissue, this case does not support evisceration as an Evidence suggesting a causal relationship between evisceration inciting event. However, it does stress the need to perform and sympathetic ophthalmia lies almost entirely in a handful of eviscerations properly and in appropriately selected patients. case observations. This and the previous case illustrate the additional point that in at least some cases, good vision can be preserved in the Earliest reports of sympathetic ophthalmia following sympathetic eye with treatment. evisceration include 47 cases from various ophthalmologists in the United States and the United Kingdom from 1887 to There is good evidence that potentially any type of intraocular 1909.30 Unfortunately, many of these cases did not have exam surgery can incite sympathetic ophthalmia. Previous retrospective documentation of the fellow eye at the onset of injury or even studies estimated the incidence of sympathetic ophthalmia to be after the sympathetic occurrence. Therefore, definitive time anywhere from 0.02% to 0.06% for intraocular surgery,33,34,35 of onset of sympathetic ophthalmia and association to original and 0.28% to 1.9% for nonsurgical penetrating injury.35-37 injury versus evisceration were dubious to say the least. Moreover, These surveys implicated various intraocular surgeries, including these cases were often initially diagnosed as endophthalmitis or cataract extractions, glaucoma procedures, and vitrectomies neuroretinitis. None had records of intraocular histopathology but not eviscerations. In a prospective surveillance, Kilmaren studies. Little else was published until 1972 when Green et al. et al. estimated the incidence of sympathetic ophthalmia to described four cases.6 Two occurred earlier in the first half of the be 0.03 per 100,000.38 Ocular surgery, particularly retinal century (1927 and 1949) and the others in the latter half of the surgery, was the most common cause in this group, as opposed century (1968 and 1969). The first two cases provided strong to previous reports where accidental trauma overwhelmingly evidence. Slides of the scleral shells that were subsequently prevailed.6,37 Between July 1997 and September 1998, all removed demonstrated granulomatous inflammation consistent permanently employed ophthalmologists in the United Kingdom with sympathetic ophthalmia. Green et al. did not provide the were sent monthly report cards to notify any newly diagnosed number of cases performed during the study period; therefore, cases of sympathetic ophthalmia. There were 23 valid cases the relative risk of sympathetic ophthalmia following evisceration of sympathetic ophthalmia reported during this period, but could not be estimated base on this study. only 17 that were reported in last 12 consecutive months were included in Kilmarin et al.’s estimation of the incidence. Of In this past decade, there have been two case reports of alleged these patients, one underwent an enucleation for recurrent post-evisceration sympathetic ophthalmia. In 2005 Griepentrog choroidal melanoma, but none had evisceration. Of note, the et al.31 reported a case of presumed sympathetic ophthalmia patient with a history of enucleation also twice underwent pars after evisceration in a 75 year-old man, who had a blind, painful plana vitrectomy. eye after a penetrating globe injury that caused neovascular glaucoma. Notably the injury occurred 66 years prior. The Although these studies fail to establish evisceration as a definitive patient developed ciliary injection, mild cataract, vitreous cells, causation for sympathetic ophthalmia, they do stress that any and macular retinal pigment epithelium mottling and serous type of intraocular surgery may be causative. Logic follows that detachment in the fellow eye fourteen