- Email: [email protected]
Choice of Procedure
Choice of Procedure Enucleation, Evisceration, or Prosthetic Fitting Over Globes RICHARD K. DORTZBACH, MD, JOHN J. WOOG, MD
Abstract: Enucleation is more likely to be associated with certain intraoperative and postoperative complications but remains the procedure of choice in cases where detailed histopathologic examination of the globe is required, in many cases of intraocular neoplasm, and in selected cases of ocular trauma with visual loss. In many patients, evisceration results in enhanced cosmesis compared to enucleation and is a technically simpler and faster operation. Evisceration may be indicated in patients with blind and unsightly or painful eyes and in selected instances of ocular trauma following discussion of the risk of sympathetic ophthalmia with the patient. It is contraindicated in patients with possible intraocular malignancy. In appropriate cases, a cosmetic shell or contact lens may constitute an alternative to enucleation or evisceration, and may provide superior cosmesis. Management of patients requiring these procedures should be tailored to the particular clinical situation with consideration of the wishes of the well-informed patient. [Key words: contact lens, enucleation, evisceration, eye surgery, prosthetic eye, scleral shell.] Ophthalmology 92:1249-1255,1985
Various ocular disorders necessitate therapeutic intervention, not to preserve or restore vision, but rather to minimize the risk to life, minimize the risk to vision in the fellow eye, maximize patient comfort, and/or enhance cosmesis. Therapeutic efforts in such cases might ideally share the following objectives: (1) preservation of life, (2) preservation of vision in the fellow eye, (3) limitation of operative complexity and risk, (4) limitation of perioperative morbidity and immediate and late operative complications, and (5) achievement of the optimal cosmetic result. With these considerations in mind we will review the merits, disadvantages, and indications for the surgical procedures of enucleation and evisceration, and for the use of cosmetic scleral shells and contact lenses. From the Department of Ophthalmology, University of Wisconsin Medical School, Madison. Presented at the Eighty-ninth Annual Meeting of the American Academy of Ophthalmology, Atlanta, Georgia, November 11-15. 1984.
ENUCLEATION Enucleation consists of removal of the entire globe and a portion of the optic nerve. The bulbar conjunctiva and Tenon's capsule are retained. Enucleation has traditionally been considered the procedure of choice in three major clinical settings: INTRAOCULAR MALIGNANCY
In the past, this procedure has been considered definitive therapy for primary intraocular malignancies. 1- 4 At present, however, differences in opinion exist concerning optimal management of intraocular choroidal malignant melanoma5 •6 and retinoblastoma. 7 PREVENTION OF SYMPATHETIC OPHTHALMIA
Enucleation allows the most complete removal of uveal melanocytic tissue, which is thought to serve as the stimulus inciting development of this devastating disorder. 8
Supported in part by a grant from Research to Prevent Blindness. Inc. Dr. Woog is a 1984-1985 Heed Foundation Ophthalmic Fellow.
HISTOPATHOLOGY
Reprint requests to Richard K. Dortzbach. MD. Clinical Science Center, Department of Ophthalmology, 600 Highland Avenue, Madison, WI 53792.
Enucleation permits thorough histopathologic examination of the intact globe and a section of optic nerve for 1249
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Fig 2. Inferior migration of the spherical implant is evident. Fig 1. Areas of dehiscence of conjunctiva and Tenon's capsule are noted over this extruding mesh·covered implant.
diagnostic purposes and for determination of intraneural or extrascleral extension of intraocular malignancies. Both enucleation and evisceration provide effective and immediate relief of intractable ocular pain, although patient discomfort in the immediate postoperative period is less following the former procedure. 2 In addition, enucleation may be performed in cases of phthisis bulbi when the phthiscial eye cannot tolerate a cosmetic scleral shell, and when the small globe prohibits placement of an implant large enough to give a good cosmetic result if an evisceration were to be done. These advantages of enucleation, however, must be balanced against the disadvantages and complications of the procedure, which include the following: Alterations in orbital anatomy.9,IO Enucleation (and, to a much lesser extent, evisceration) induces multiple changes in orbital anatomy and physiology, including: (I) decreased orbital blood flow, as reflected by orbital thermographic asymmetry following surgery; (2) orbital fat atrophy and decreased orbital soft tissue volume, (3) disturbance of the levator palpebrae superioris-Whitnall's ligament complex; (4) retardation of growth of the bony orbit, following enucleation in infancy or childhood, II and (5) prosthesis-induced interference with upper and lower eyelid position. Operative complications. The following intraoperative and postoperative complications may occur with both enucleation and evisceration, but some are more likely with enucleation. Intraoperative complications. Removal of wrong eye. This complication can be prevented by rechecking the chart and operative permit and reexamining the patient (with ophthalmoscopy, if possible) in the operating room.12 Hemorrhage. Post-enucleation hemorrhage may be avoided by crushing the optic nerve prior to nerve transection, application of gauze packing and firm pressure following removal of the globe, and the use of electrocautery, Surgicel®, Oxycel®, and Gelfoam® with topical thrombin as necessary.2,3,9 Perforation of the globe. Management of this compli1250
cation includes removal of the ruptured globe and resection of adjacent orbital tissue with submission of this tissue for histopathologic examination, if an intraocular tumor is proven or suspected. 12 Injury to orbital structures. Careful surgical technique minimizes trauma to normal orbital tissues, including conjunctiva, Tenon's capsule, and the levator palpebrae superioris. Postoperative complications. Wound dehiscence and implant extrusion (Fig 1). These complications may be avoided by the choice of an implant of appropriate size, by complete closure of Tenon's capsule without excess tension, and by meticulous hemostasis. Placement of the implant in the muscle cone behind posterior Tenon's capsule and encasement of the implant in donor sclera or autogenous fascia may be of value in the prevention of subsequent extrusion. 9 The use of scleral patch grafting, as advocated by Helveston,13 may avert implant extrusion when wound dehiscence is limited and of short duration. When wound dehiscence is long standing, however, the implant cavity is lined by conjunctival epithelium; appropriate therapy consists of removal of the implant and the epithelial lining, preferably with swabbing the cavity with absolute alcohol, and placement of a new implant in the muscle cone behind posterior Tenon's capsule. A dermis-fat graft may be used when a limited amount of conjunctiva remains in the socket. Implant migration (Fig 2). According to several authors,2,4 implant migration occurs most commonly following use of a spherical implant placed in the muscle cone with imbrication of the extraocular muscles over the implant. The characteristic direction of migration is superotemporal or inferotemporal. Infection. Postoperative infection may be managed by administration of appropriate local and systemic antibiotics, and often necessitates implant removal. Ptosis (Fig 3). Ptosis following enucleation may be related to levator dehiscence or third nerve palsy induced by excessive manipUlation of the superior rectus muscle during dissection of the superior fornix or orbital apex, and may be prevented by careful attention to surgical technique. Allen ls believes that ptosis and implant mi-
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Fig 3. Leji, ptosis following enucleation may result from third nerve damage or levator disinsertion, as in this patient. Right, following repair of levator disinsertion.
Fig 4. Lefi, use of an intraorbital implant of sufficient size may promote a good cosmetic result, as in this patient. Right, intraorbital volume loss may lead to a deep superior sulcus following enucleation.
gration constitute a distinct syndrome following imbrication of the rectus muscles over spherical intraorbital implants, and advises against this practice. Surgical management of post-enucleation ptosis may include levator repair, minimal ptosis surgery, or eyelid suspension procedures. One author2 feels that levator resection may jeopardize the superior fornix and should thus be avoided in enucleation patients. Enophthalmos associated with orbital volume loss following enucleation may produce an apparent ptosis (pseudoptosis) of the upper eyelid, as noted below. Shallowing of the fornices and socket contracture. Thorough dissection of conjunctiva from Tenon's capsule during enucleation has been recommended to ensure adequate forniceal depth in the postoperative period. 9 As noted above, care must be exercised in the dissection of the superior fornix to minimize the possibility of an iatrogenic ptosis. Furthermore, excessive dissection may cause increased fibrosis and subsequent socket contracture. Postoperative socket contracture may be managed with a variety of techniques, including the use of full thickness mucosal grafts,2 orbital implants composed of silicone or other alloplastic materials,16 and dermis-fat grafting. 17 Deep superior sulcus syndrome (Fig 4). Removal of the globe results in a loss of intraorbital volume of approximately 7 ml. Orbital fat atrophy following surgical trauma may add a further volume deficit of 2-3 ml, and may be a source of ongoing volume 10SS.10 Implantation of an 18 mm diameter sphere provides approximately 3 ml of volume replacement, and a prosthesis may provide an ad-
ditional 2 to 3 ml; enucleation with use of an intraorbital implant may thus be associated with an orbital volume loss of 3 to 5 ml, which may result in the clinical appearance of a deep superior sulcus. In an attempt to replace this volume loss and achieve optimal cosmesis, a large, and therefore heavy artificial eye may then be fitted; under the weight of this prosthesis, however, the lower eyelid will stretch, resulting in inferior displacement of the prosthesis and socket, secondary ptosis of the upper eyelid, enophthalmos and recurrence of the deep superior sulcus. Treatment involves surgical shortening of the lower eyelid horizontally, and the artificial eye may then be replaced with a larger prosthesis, with temporary improvement in appearance until continued sagging oflower eyelid tissues results in progressive enophthalmos and deepening of the superior sulcus. This "post-enucleation socket syndrome,,10 may be prevented and/or treated by the introduction of intraorbital or subperiosteal implants of adequate volume. 2,9,18 Orbital cysts. Conjunctival inclusion cysts may arise following enucleation in otherwise uncomplicated cases, 19 and may develop in up to 20% of cases in which scleral patch grafting is used to manage orbital implant extrusion. These cysts may be painful, and when large, may interfere with the fitting and wearing of a prosthesis. Effective therapeutic approaches include cyst marsupialization, excision, and cyst aspiration with absolute alcohol injection.3,20 Despite these potential complications, enucleation is the procedure of choice in instances of ocular trauma with visual loss in which severe scleral damage prohibits evis1251
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ORBITAL INFECflON
Some authors 3.4.9 feel that enucleation in cases of endophthalmitis may result in the spread of infection to orbital tissues and the meninges with possible intracranial extension; they believe that evisceration is thus the procedure of choice in patients with endophthalmitis. Other investigators,8 however, believe that the risks of intraorbital and intracranial extension are minimal in view of current intensive antibiotic management of endophthalmitis. Fig 5. Good cosmetic result following evisceration in the left eye. (Courtesy of Dori Hosek.)
ceration, in cases where complete histopathologic examination of the globe is desired, and in many cases where the presence of an intraocular malignancy is proven or suspected. 1-7 Some authors21 ,22 feel that enucleation may favorably influence the course of sympathetic ophthalmia performed within two weeks of the onset of symptoms in the sympathizing eye. Additional indications for enucleation may include the presence of a blind painful eye in which pain cannot be controlled with other modalities (including retrobulbar alcohol injection and cyclocryotherapy) and the presence of extensive corneal or bulbar staphylomata (in which cases evisceration cannot be considered).
EVISCERATION Evisceration is a procedure wherein the intraocular contents are removed and the sclera, Tenon's capsule, conjunctiva, and optic nerve are preserved. The cornea may be retained or excised. Advantages of evisceration include the following: PRESERVATION OF ORBITAL ANATOMY
Evisceration entails less disruption of orbital anatomy and physiology than does enucleation, with fewer attendant complications. The relationships of the eyelid tissues and the extraocular muscles to the scleral wall and the fornices, in particular, are relatively undisturbed. ENHANCED COSMESIS (Fig 5)
Orbital fat atrophy is less pronounced following evisceration, and retention of the scleral or corneosclerallayers provides an additional 0.5 ml of orbital volume compared with enucleation. The prospects for development of significant postoperative volume loss, enophthalmos, and a deep superior sulcus are thus minimized. The relative preservation of orbital anatomy noted above results in better motility of the socket (which may be transmitted to the prosthesis) and a lower incidence of postoperative ptosis of the upper eyelid and inferior displacement of the lower eyelid and prosthesis. There is a lower incidence, as well, of implant migration and extrusion and of socket contracture. 4 ,9 1252
RELIEF OF PAIN
Enucleation and evisceration are equally effective in relieving intractable pain associated with intraocular inflammation. 4 OPERA TIVE CONSIDERATIONS
Evisceration is technically simpler and faster than enucleation and may be the indicated procedure in patients at poor risk for prolonged general anesthesia or even a lengthy procedure under local anesthesia. 4 As with enucleation, the advantages of evisceration must be weighed carefully against the disadvantages of the procedure: HISTOPATHOLOGY
Evisceration specimens are unsuitable for detailed histopathologic examination. Pathologic study of enucleated eyes, on the other hand, may provide not only a specific anatomic diagnosis but also important information concerning the pathogenesis of visual loss in a variety of ocular disorders. 8 OPERATIVE COMPLICATIONS
Intraoperative complications. Evisceration performed in the setting of opaque media and an unsuspected intraocular tumor may result in orbital and systemic dissemination of malignancy. One study8 has cited a IO to 16% incidence of unsuspected intraocular malignancies in eyes with opaque media; this study may not, however, apply to patients examined with currently available diagnostic modalities, including indirect ophthalmoscopy and ultrasound. Postoperative complications. Sympathetic ophthalmia. Green and co-workers8 reported four cases of sympathetic ophthalmia following evisceration. In three of the four cases, operative notes described in detail efforts to remove all uveal tissue from the scleral shell, and histopathologic examination of the scleral shell in all four cases revealed no evidence of intact uveal tissue on the inner surface of the shell. Uveal melanocytes and associated granulomatous inflammatory reactions were noted in all cases, however, at the optic nerve head, in perineural sclera, and in scleral emissary canals containing nerves and blood vessels-areas where, as Green has pointed out, uveal melanocytes are normally present and not accessible for removal with standard evisceration techniques.
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Fig 6. A prosthetic scleral shell may afford superior cosmesis.
Fig 7. Anterior (left) and posterior (right) surfaces of a cosmetic scleral shell (Courtesy of Sue Alexander, CASO.)
Postoperative discomfort. Postoperative reaction and pain may be more pronounced following evisceration. 3 As noted above, patients with visual loss following endophthalmitis may be suitable candidates for evisceration. This procedure may also be indicated in patients with blind, painful, and unsightly eyes secondary to glaucoma, uveitis, or corneal scarring and in selected cases of ocular trauma where the scleral coat is preserved. Eyes with opaque media should be studied with ultrasonography in addition to clinical examination prior to evisceration.
COSMETIC SCLERAL SHELLS AND CONTACT LENSES Cosmetic scleral shells and hard and soft corneoscleral lenses constitute a satisfactory and, in many cases, preferable alternative to enucleation or evisceration. Both cosmetic scleral shells and contact lenses generally provide good-to-excellent prosthetic motility and often afford a cosmetic result superior to that obtained with enucleation or evisceration (Figs 6-8), while avoiding the possible op-
erative and postoperative complications of these procedures. SCLERAL SHELLS
Cosmetic shell prostheses are most often indicated in the setting of phthisis bulbi or microphthalmia with no useful vision. Limitations to the use of scleral shells include corneal irritation and difficulty in fitting lenses secondary to irregularities in contour of the ocular surface, although shells usually can be fit to vault over an irregular corneal surface. The fitting process may be prolonged, and careful patient follow-up is required,z3 as after enucleation or evisceration. In addition, in the case of eyes not small enough the thickness of the scleral shell (0.51.0 mm) may impart a somewhat proptotic appearance to the prosthesis. 23 HARD AND SOFT CONTACf LENSES (Figs 9-11)
Both hard and soft lenses may be employed in the cosmetic rehabilitation of the unsightly eye of normal size, 1253
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Fig 8. Prosthetic motility following evisceration (middle row) is generally intermediate between that of enucleation (top row) and cosmetic scleral shell (bottom row). (Middle row photographs courtesy ofDori Hosek.)
Fig 9. Patient with bullous keratopathy (left) with cosmetic soft contact lens in place (right). (Courtesy of Narcissus Medical Foundation.)
Fig 10. Patient with iris coloboma (left) with cosmetic soft contact lens in place (center). Iris detail may be hand-painted on lens to match that of normal eye (right).
and may be used in eyes with useful vision, unlike cosmetic shell prostheses. Prosthetic soft contact lenses are available either as corneal lenses or corneoscleral lenses with transparent haptics. Soft lenses may be more comfortable than shell prostheses and are preferred by some authors for routine usage except in cases of marked surface irregularity or strabismus. Prosthetic soft lenses are somewhat thicker than other types of soft lenses, but oxygen transmission usually is adequate due to the corneal neovascularization often present in these severely traumatized or diseased eyes. 24,25 Problems associated with cosmetic lenses include difficulty in obtaining a color match with the good eye (Fig 11), flaking oflens paint, and lens damage and loss, particularly with soft contact lenses. 1254
COMMENT Enucleation results in more profound disruption of orbital anatomy and physiology and is more likely to be associated with a number of complications (including ptosis, implant migration, implant extrusion, socket contracture, and the deep superior sulcus syndrome) that may compromise the cosmetic result. Despite these potential complications, enucleation is the procedure of choice in cases where detailed histopathologic examination of the globe is required, in many instances where an intraocular tumor is proven or suspected, and in selected cases of ocular trauma with visual loss. Evisceration results in less
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REFERENCES
Fig 11. Lens decentration and inexact color match may occur with cosmetic soft contact lenses.
disruption of orbital anatomy and, in many patients, enhanced cosmesis compared to enucleation, and is a technically simpler and faster operation. This procedure is contraindicated in patients with possible intraocular malignancy. Evisceration may be indicated in patients with visual loss following endophthalmitis, in patients with blind and unsightly eyes when intraocular malignancy has been excluded, and in selected patients sustaining ocular trauma with visual loss. Enucleation may be the procedure of choice in patients sustaining ocular trauma where severe scleral disruption prohibits evisceration. Evisceration may be an acceptable alternative procedure in cases of ocular trauma when the integrity of the scleral coat is preserved if the patient expresses a preference for this procedure following careful discussion of the relative benefits and risks of enucleation and evisceration (with particular attention to the risk, though minimal, of sympathetic ophthalmia following evisceration). If the patient sustaining ocular trauma with visual loss does not express a strong preference for either procedure, we tend to favor enucleation. When tolerated, a cosmetic scleral shell (in the case of phthisis bulbi or microphthalmos with no useful vision) or cosmetic contact lens (in the case of an unsightly globe of normal size with or without vision) may constitute an alternative to enucleation or evisceration, and may provide a superior cosmetic result. In many cases, the choice between enucleation and evisceration is not clearly defined; the choice of procedure in these cases must be made on an individualized basis following careful consideration of the relative merits and disadvantages of each procedure in conjunction with the wishes of the well-informed patient.
ACKNOWLEDGMENTS Sue Alexander, CASO, Dori Hosek, and Narcissus Medical Foundation provided several of the Figures. Lorraine Boyle assisted in the preparation of the manuscript. Barry Ulsrud assisted in photography.
1. Callahan MA, Callahan A. Ophthalmic Plastic and Orbital Surgery. Birmingham: Aesculapius, 1979; 42-54. 2. Soli DB. Enucleation and evisceration. In: Duane TO, ed. Clinical Ophthalmology. Philadelphia: Harper & Row, 1982; Volume 5, Chapter 17. 3' Fox SA. Ophthalmic Plastic Surgery, 4th ed. New York: Grune &Stratton, 1970; 475-501. 4. Baylis H, Shorr N. Evisceration, enucleation, and exenteration. In: McCord CD Jr, ed. Oculoplastic Surgery. New York: Raven Press, 1981; 313-26. 5. Zimmerman LE, McLean IW. An evaluation of enucleation in the management of uveal melanomas. Am J Ophthalmol1979; 87:741-60. 6. Manschot WA, van Peperzeel HA. Choroidal melanoma; enucleation or observation? A new approach. Arch Ophthalmol1980; 98:71-7. 7. Abramson DH, Marks RF, Ellsworth RM, et al. The management of unilateral retinoblastoma without primary enucleation. Arch Ophthalmol 1982; 100:1249-52. 8. Green WR, Maumenee AE, Sanders TE, Smith ME. Sympathetic uveitis following evisceration. Trans Am Acad Ophthalmol Otolaryngol1972; 76:625-44. 9. Soli DB. The anophthalmic socket. Ophthalmology 1982; 89:407-23. 10. Tyers AG, Collin JRO. Orbital implants and post enucleation socket syndrome. Trans Ophthalmol Soc UK 1982; 102:90-2. 11. Kennedy RE. The effect of early enucleation on the orbit in animals and humans. Am J Ophthalmol1965; 60:277-306. 12. American Academy of Ophthalmology. Basic and Clinical Science Course; Section 9: Orbit, Eyelids and Lacrimal System. San Francisco: American Academy of Ophthalmology, 1981; 69. 13. Helveston EM. A scleral patch for exposed implants. Trans Am Acad Ophthalmol Otolaryngol 1970; 74: 1307 -10. 14. Dryden R, Leibsohn J. Postenucleation orbital implant extrusion. Arch Ophthalmol1978; 96:2064-5. 15. Allen L. The argument against imbricating the rectus muscles over spherical orbital implants after enucleation. Ophthalmology 1983; 90: 1116-20. 16. Dortzbach RK, Callahan A. Advances in socket reconstruction. Am J Ophthalmol 1970; 70:800-13. 17. Smith B, Petrelli R. Dermis-fat graft as a moveable implant within the muscle cone. Am J Ophthalmol1978; 85:62-6. 18. Dortzbach RK, Alexander SC, Sutula FC, Hawes MJ. Correction of facial contour deformities with prefabricated sculptured implants. Ophthalmology 1981; 88:908-16. 19. Allison SC, Wergeland FL Jr. Giant conjunctival cyst of the orbit. Ann Ophthalmol1973; 5:199-202. 20. Hornblass A, Bosniak S. Orbital cysts following enucleation: the use of absolute alcohol. Ophthalmic Surg 1981; 12:123-6. 21. Lubin JR, Albert OM, Weinstein M. Sixty-five years of sympathetic ophthalmia; a clincopathologic review of 105 cases (1913-1978). Ophthalmology 1980; 87:109-21. 22. Reynard M, Riffenburgh RS, Maes EF. Effect of corticosteroid treatment and enucleation on the visual prognosis of sympathetic ophthalmia. Am J Ophthalmol1983; 96:290-4. 23. Marriott PJ. Impression scleral lens fitting for special and pathological conditions. In: Stone J, Phillips AJ, eds. Contact Lenses; A Textbook for Practitioner and Student, 2d ed. London: Butterworths, 1981; 602. 24. Stone J. Special types of contact lenses and their uses. In: Stone J, Phillips AJ, eds. Contact Lenses; A Textbook for Practitioner and Student, 2d ed. London: Butterworths, 1981; 666. 25. Kersley HJ. Coloured soft contact lenses and the unsightly eye. Trans Ophthalmol Soc UK 1982; 102:101-3.
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Abstract: Enucleation is more likely to be associated with certain intraoperative and postoperative complications but remains the procedure of choice in cases where detailed histopathologic examination of the globe is required, in many cases of intraocular neoplasm, and in selected cases of ocular trauma with visual loss. In many patients, evisceration results in enhanced cosmesis compared to enucleation and is a technically simpler and faster operation. Evisceration may be indicated in patients with blind and unsightly or painful eyes and in selected instances of ocular trauma following discussion of the risk of sympathetic ophthalmia with the patient. It is contraindicated in patients with possible intraocular malignancy. In appropriate cases, a cosmetic shell or contact lens may constitute an alternative to enucleation or evisceration, and may provide superior cosmesis. Management of patients requiring these procedures should be tailored to the particular clinical situation with consideration of the wishes of the well-informed patient. [Key words: contact lens, enucleation, evisceration, eye surgery, prosthetic eye, scleral shell.] Ophthalmology 92:1249-1255,1985
Various ocular disorders necessitate therapeutic intervention, not to preserve or restore vision, but rather to minimize the risk to life, minimize the risk to vision in the fellow eye, maximize patient comfort, and/or enhance cosmesis. Therapeutic efforts in such cases might ideally share the following objectives: (1) preservation of life, (2) preservation of vision in the fellow eye, (3) limitation of operative complexity and risk, (4) limitation of perioperative morbidity and immediate and late operative complications, and (5) achievement of the optimal cosmetic result. With these considerations in mind we will review the merits, disadvantages, and indications for the surgical procedures of enucleation and evisceration, and for the use of cosmetic scleral shells and contact lenses. From the Department of Ophthalmology, University of Wisconsin Medical School, Madison. Presented at the Eighty-ninth Annual Meeting of the American Academy of Ophthalmology, Atlanta, Georgia, November 11-15. 1984.
ENUCLEATION Enucleation consists of removal of the entire globe and a portion of the optic nerve. The bulbar conjunctiva and Tenon's capsule are retained. Enucleation has traditionally been considered the procedure of choice in three major clinical settings: INTRAOCULAR MALIGNANCY
In the past, this procedure has been considered definitive therapy for primary intraocular malignancies. 1- 4 At present, however, differences in opinion exist concerning optimal management of intraocular choroidal malignant melanoma5 •6 and retinoblastoma. 7 PREVENTION OF SYMPATHETIC OPHTHALMIA
Enucleation allows the most complete removal of uveal melanocytic tissue, which is thought to serve as the stimulus inciting development of this devastating disorder. 8
Supported in part by a grant from Research to Prevent Blindness. Inc. Dr. Woog is a 1984-1985 Heed Foundation Ophthalmic Fellow.
HISTOPATHOLOGY
Reprint requests to Richard K. Dortzbach. MD. Clinical Science Center, Department of Ophthalmology, 600 Highland Avenue, Madison, WI 53792.
Enucleation permits thorough histopathologic examination of the intact globe and a section of optic nerve for 1249
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Fig 2. Inferior migration of the spherical implant is evident. Fig 1. Areas of dehiscence of conjunctiva and Tenon's capsule are noted over this extruding mesh·covered implant.
diagnostic purposes and for determination of intraneural or extrascleral extension of intraocular malignancies. Both enucleation and evisceration provide effective and immediate relief of intractable ocular pain, although patient discomfort in the immediate postoperative period is less following the former procedure. 2 In addition, enucleation may be performed in cases of phthisis bulbi when the phthiscial eye cannot tolerate a cosmetic scleral shell, and when the small globe prohibits placement of an implant large enough to give a good cosmetic result if an evisceration were to be done. These advantages of enucleation, however, must be balanced against the disadvantages and complications of the procedure, which include the following: Alterations in orbital anatomy.9,IO Enucleation (and, to a much lesser extent, evisceration) induces multiple changes in orbital anatomy and physiology, including: (I) decreased orbital blood flow, as reflected by orbital thermographic asymmetry following surgery; (2) orbital fat atrophy and decreased orbital soft tissue volume, (3) disturbance of the levator palpebrae superioris-Whitnall's ligament complex; (4) retardation of growth of the bony orbit, following enucleation in infancy or childhood, II and (5) prosthesis-induced interference with upper and lower eyelid position. Operative complications. The following intraoperative and postoperative complications may occur with both enucleation and evisceration, but some are more likely with enucleation. Intraoperative complications. Removal of wrong eye. This complication can be prevented by rechecking the chart and operative permit and reexamining the patient (with ophthalmoscopy, if possible) in the operating room.12 Hemorrhage. Post-enucleation hemorrhage may be avoided by crushing the optic nerve prior to nerve transection, application of gauze packing and firm pressure following removal of the globe, and the use of electrocautery, Surgicel®, Oxycel®, and Gelfoam® with topical thrombin as necessary.2,3,9 Perforation of the globe. Management of this compli1250
cation includes removal of the ruptured globe and resection of adjacent orbital tissue with submission of this tissue for histopathologic examination, if an intraocular tumor is proven or suspected. 12 Injury to orbital structures. Careful surgical technique minimizes trauma to normal orbital tissues, including conjunctiva, Tenon's capsule, and the levator palpebrae superioris. Postoperative complications. Wound dehiscence and implant extrusion (Fig 1). These complications may be avoided by the choice of an implant of appropriate size, by complete closure of Tenon's capsule without excess tension, and by meticulous hemostasis. Placement of the implant in the muscle cone behind posterior Tenon's capsule and encasement of the implant in donor sclera or autogenous fascia may be of value in the prevention of subsequent extrusion. 9 The use of scleral patch grafting, as advocated by Helveston,13 may avert implant extrusion when wound dehiscence is limited and of short duration. When wound dehiscence is long standing, however, the implant cavity is lined by conjunctival epithelium; appropriate therapy consists of removal of the implant and the epithelial lining, preferably with swabbing the cavity with absolute alcohol, and placement of a new implant in the muscle cone behind posterior Tenon's capsule. A dermis-fat graft may be used when a limited amount of conjunctiva remains in the socket. Implant migration (Fig 2). According to several authors,2,4 implant migration occurs most commonly following use of a spherical implant placed in the muscle cone with imbrication of the extraocular muscles over the implant. The characteristic direction of migration is superotemporal or inferotemporal. Infection. Postoperative infection may be managed by administration of appropriate local and systemic antibiotics, and often necessitates implant removal. Ptosis (Fig 3). Ptosis following enucleation may be related to levator dehiscence or third nerve palsy induced by excessive manipUlation of the superior rectus muscle during dissection of the superior fornix or orbital apex, and may be prevented by careful attention to surgical technique. Allen ls believes that ptosis and implant mi-
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Fig 3. Leji, ptosis following enucleation may result from third nerve damage or levator disinsertion, as in this patient. Right, following repair of levator disinsertion.
Fig 4. Lefi, use of an intraorbital implant of sufficient size may promote a good cosmetic result, as in this patient. Right, intraorbital volume loss may lead to a deep superior sulcus following enucleation.
gration constitute a distinct syndrome following imbrication of the rectus muscles over spherical intraorbital implants, and advises against this practice. Surgical management of post-enucleation ptosis may include levator repair, minimal ptosis surgery, or eyelid suspension procedures. One author2 feels that levator resection may jeopardize the superior fornix and should thus be avoided in enucleation patients. Enophthalmos associated with orbital volume loss following enucleation may produce an apparent ptosis (pseudoptosis) of the upper eyelid, as noted below. Shallowing of the fornices and socket contracture. Thorough dissection of conjunctiva from Tenon's capsule during enucleation has been recommended to ensure adequate forniceal depth in the postoperative period. 9 As noted above, care must be exercised in the dissection of the superior fornix to minimize the possibility of an iatrogenic ptosis. Furthermore, excessive dissection may cause increased fibrosis and subsequent socket contracture. Postoperative socket contracture may be managed with a variety of techniques, including the use of full thickness mucosal grafts,2 orbital implants composed of silicone or other alloplastic materials,16 and dermis-fat grafting. 17 Deep superior sulcus syndrome (Fig 4). Removal of the globe results in a loss of intraorbital volume of approximately 7 ml. Orbital fat atrophy following surgical trauma may add a further volume deficit of 2-3 ml, and may be a source of ongoing volume 10SS.10 Implantation of an 18 mm diameter sphere provides approximately 3 ml of volume replacement, and a prosthesis may provide an ad-
ditional 2 to 3 ml; enucleation with use of an intraorbital implant may thus be associated with an orbital volume loss of 3 to 5 ml, which may result in the clinical appearance of a deep superior sulcus. In an attempt to replace this volume loss and achieve optimal cosmesis, a large, and therefore heavy artificial eye may then be fitted; under the weight of this prosthesis, however, the lower eyelid will stretch, resulting in inferior displacement of the prosthesis and socket, secondary ptosis of the upper eyelid, enophthalmos and recurrence of the deep superior sulcus. Treatment involves surgical shortening of the lower eyelid horizontally, and the artificial eye may then be replaced with a larger prosthesis, with temporary improvement in appearance until continued sagging oflower eyelid tissues results in progressive enophthalmos and deepening of the superior sulcus. This "post-enucleation socket syndrome,,10 may be prevented and/or treated by the introduction of intraorbital or subperiosteal implants of adequate volume. 2,9,18 Orbital cysts. Conjunctival inclusion cysts may arise following enucleation in otherwise uncomplicated cases, 19 and may develop in up to 20% of cases in which scleral patch grafting is used to manage orbital implant extrusion. These cysts may be painful, and when large, may interfere with the fitting and wearing of a prosthesis. Effective therapeutic approaches include cyst marsupialization, excision, and cyst aspiration with absolute alcohol injection.3,20 Despite these potential complications, enucleation is the procedure of choice in instances of ocular trauma with visual loss in which severe scleral damage prohibits evis1251
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ORBITAL INFECflON
Some authors 3.4.9 feel that enucleation in cases of endophthalmitis may result in the spread of infection to orbital tissues and the meninges with possible intracranial extension; they believe that evisceration is thus the procedure of choice in patients with endophthalmitis. Other investigators,8 however, believe that the risks of intraorbital and intracranial extension are minimal in view of current intensive antibiotic management of endophthalmitis. Fig 5. Good cosmetic result following evisceration in the left eye. (Courtesy of Dori Hosek.)
ceration, in cases where complete histopathologic examination of the globe is desired, and in many cases where the presence of an intraocular malignancy is proven or suspected. 1-7 Some authors21 ,22 feel that enucleation may favorably influence the course of sympathetic ophthalmia performed within two weeks of the onset of symptoms in the sympathizing eye. Additional indications for enucleation may include the presence of a blind painful eye in which pain cannot be controlled with other modalities (including retrobulbar alcohol injection and cyclocryotherapy) and the presence of extensive corneal or bulbar staphylomata (in which cases evisceration cannot be considered).
EVISCERATION Evisceration is a procedure wherein the intraocular contents are removed and the sclera, Tenon's capsule, conjunctiva, and optic nerve are preserved. The cornea may be retained or excised. Advantages of evisceration include the following: PRESERVATION OF ORBITAL ANATOMY
Evisceration entails less disruption of orbital anatomy and physiology than does enucleation, with fewer attendant complications. The relationships of the eyelid tissues and the extraocular muscles to the scleral wall and the fornices, in particular, are relatively undisturbed. ENHANCED COSMESIS (Fig 5)
Orbital fat atrophy is less pronounced following evisceration, and retention of the scleral or corneosclerallayers provides an additional 0.5 ml of orbital volume compared with enucleation. The prospects for development of significant postoperative volume loss, enophthalmos, and a deep superior sulcus are thus minimized. The relative preservation of orbital anatomy noted above results in better motility of the socket (which may be transmitted to the prosthesis) and a lower incidence of postoperative ptosis of the upper eyelid and inferior displacement of the lower eyelid and prosthesis. There is a lower incidence, as well, of implant migration and extrusion and of socket contracture. 4 ,9 1252
RELIEF OF PAIN
Enucleation and evisceration are equally effective in relieving intractable pain associated with intraocular inflammation. 4 OPERA TIVE CONSIDERATIONS
Evisceration is technically simpler and faster than enucleation and may be the indicated procedure in patients at poor risk for prolonged general anesthesia or even a lengthy procedure under local anesthesia. 4 As with enucleation, the advantages of evisceration must be weighed carefully against the disadvantages of the procedure: HISTOPATHOLOGY
Evisceration specimens are unsuitable for detailed histopathologic examination. Pathologic study of enucleated eyes, on the other hand, may provide not only a specific anatomic diagnosis but also important information concerning the pathogenesis of visual loss in a variety of ocular disorders. 8 OPERATIVE COMPLICATIONS
Intraoperative complications. Evisceration performed in the setting of opaque media and an unsuspected intraocular tumor may result in orbital and systemic dissemination of malignancy. One study8 has cited a IO to 16% incidence of unsuspected intraocular malignancies in eyes with opaque media; this study may not, however, apply to patients examined with currently available diagnostic modalities, including indirect ophthalmoscopy and ultrasound. Postoperative complications. Sympathetic ophthalmia. Green and co-workers8 reported four cases of sympathetic ophthalmia following evisceration. In three of the four cases, operative notes described in detail efforts to remove all uveal tissue from the scleral shell, and histopathologic examination of the scleral shell in all four cases revealed no evidence of intact uveal tissue on the inner surface of the shell. Uveal melanocytes and associated granulomatous inflammatory reactions were noted in all cases, however, at the optic nerve head, in perineural sclera, and in scleral emissary canals containing nerves and blood vessels-areas where, as Green has pointed out, uveal melanocytes are normally present and not accessible for removal with standard evisceration techniques.
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Fig 6. A prosthetic scleral shell may afford superior cosmesis.
Fig 7. Anterior (left) and posterior (right) surfaces of a cosmetic scleral shell (Courtesy of Sue Alexander, CASO.)
Postoperative discomfort. Postoperative reaction and pain may be more pronounced following evisceration. 3 As noted above, patients with visual loss following endophthalmitis may be suitable candidates for evisceration. This procedure may also be indicated in patients with blind, painful, and unsightly eyes secondary to glaucoma, uveitis, or corneal scarring and in selected cases of ocular trauma where the scleral coat is preserved. Eyes with opaque media should be studied with ultrasonography in addition to clinical examination prior to evisceration.
COSMETIC SCLERAL SHELLS AND CONTACT LENSES Cosmetic scleral shells and hard and soft corneoscleral lenses constitute a satisfactory and, in many cases, preferable alternative to enucleation or evisceration. Both cosmetic scleral shells and contact lenses generally provide good-to-excellent prosthetic motility and often afford a cosmetic result superior to that obtained with enucleation or evisceration (Figs 6-8), while avoiding the possible op-
erative and postoperative complications of these procedures. SCLERAL SHELLS
Cosmetic shell prostheses are most often indicated in the setting of phthisis bulbi or microphthalmia with no useful vision. Limitations to the use of scleral shells include corneal irritation and difficulty in fitting lenses secondary to irregularities in contour of the ocular surface, although shells usually can be fit to vault over an irregular corneal surface. The fitting process may be prolonged, and careful patient follow-up is required,z3 as after enucleation or evisceration. In addition, in the case of eyes not small enough the thickness of the scleral shell (0.51.0 mm) may impart a somewhat proptotic appearance to the prosthesis. 23 HARD AND SOFT CONTACf LENSES (Figs 9-11)
Both hard and soft lenses may be employed in the cosmetic rehabilitation of the unsightly eye of normal size, 1253
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Fig 8. Prosthetic motility following evisceration (middle row) is generally intermediate between that of enucleation (top row) and cosmetic scleral shell (bottom row). (Middle row photographs courtesy ofDori Hosek.)
Fig 9. Patient with bullous keratopathy (left) with cosmetic soft contact lens in place (right). (Courtesy of Narcissus Medical Foundation.)
Fig 10. Patient with iris coloboma (left) with cosmetic soft contact lens in place (center). Iris detail may be hand-painted on lens to match that of normal eye (right).
and may be used in eyes with useful vision, unlike cosmetic shell prostheses. Prosthetic soft contact lenses are available either as corneal lenses or corneoscleral lenses with transparent haptics. Soft lenses may be more comfortable than shell prostheses and are preferred by some authors for routine usage except in cases of marked surface irregularity or strabismus. Prosthetic soft lenses are somewhat thicker than other types of soft lenses, but oxygen transmission usually is adequate due to the corneal neovascularization often present in these severely traumatized or diseased eyes. 24,25 Problems associated with cosmetic lenses include difficulty in obtaining a color match with the good eye (Fig 11), flaking oflens paint, and lens damage and loss, particularly with soft contact lenses. 1254
COMMENT Enucleation results in more profound disruption of orbital anatomy and physiology and is more likely to be associated with a number of complications (including ptosis, implant migration, implant extrusion, socket contracture, and the deep superior sulcus syndrome) that may compromise the cosmetic result. Despite these potential complications, enucleation is the procedure of choice in cases where detailed histopathologic examination of the globe is required, in many instances where an intraocular tumor is proven or suspected, and in selected cases of ocular trauma with visual loss. Evisceration results in less
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REFERENCES
Fig 11. Lens decentration and inexact color match may occur with cosmetic soft contact lenses.
disruption of orbital anatomy and, in many patients, enhanced cosmesis compared to enucleation, and is a technically simpler and faster operation. This procedure is contraindicated in patients with possible intraocular malignancy. Evisceration may be indicated in patients with visual loss following endophthalmitis, in patients with blind and unsightly eyes when intraocular malignancy has been excluded, and in selected patients sustaining ocular trauma with visual loss. Enucleation may be the procedure of choice in patients sustaining ocular trauma where severe scleral disruption prohibits evisceration. Evisceration may be an acceptable alternative procedure in cases of ocular trauma when the integrity of the scleral coat is preserved if the patient expresses a preference for this procedure following careful discussion of the relative benefits and risks of enucleation and evisceration (with particular attention to the risk, though minimal, of sympathetic ophthalmia following evisceration). If the patient sustaining ocular trauma with visual loss does not express a strong preference for either procedure, we tend to favor enucleation. When tolerated, a cosmetic scleral shell (in the case of phthisis bulbi or microphthalmos with no useful vision) or cosmetic contact lens (in the case of an unsightly globe of normal size with or without vision) may constitute an alternative to enucleation or evisceration, and may provide a superior cosmetic result. In many cases, the choice between enucleation and evisceration is not clearly defined; the choice of procedure in these cases must be made on an individualized basis following careful consideration of the relative merits and disadvantages of each procedure in conjunction with the wishes of the well-informed patient.
ACKNOWLEDGMENTS Sue Alexander, CASO, Dori Hosek, and Narcissus Medical Foundation provided several of the Figures. Lorraine Boyle assisted in the preparation of the manuscript. Barry Ulsrud assisted in photography.
1. Callahan MA, Callahan A. Ophthalmic Plastic and Orbital Surgery. Birmingham: Aesculapius, 1979; 42-54. 2. Soli DB. Enucleation and evisceration. In: Duane TO, ed. Clinical Ophthalmology. Philadelphia: Harper & Row, 1982; Volume 5, Chapter 17. 3' Fox SA. Ophthalmic Plastic Surgery, 4th ed. New York: Grune &Stratton, 1970; 475-501. 4. Baylis H, Shorr N. Evisceration, enucleation, and exenteration. In: McCord CD Jr, ed. Oculoplastic Surgery. New York: Raven Press, 1981; 313-26. 5. Zimmerman LE, McLean IW. An evaluation of enucleation in the management of uveal melanomas. Am J Ophthalmol1979; 87:741-60. 6. Manschot WA, van Peperzeel HA. Choroidal melanoma; enucleation or observation? A new approach. Arch Ophthalmol1980; 98:71-7. 7. Abramson DH, Marks RF, Ellsworth RM, et al. The management of unilateral retinoblastoma without primary enucleation. Arch Ophthalmol 1982; 100:1249-52. 8. Green WR, Maumenee AE, Sanders TE, Smith ME. Sympathetic uveitis following evisceration. Trans Am Acad Ophthalmol Otolaryngol1972; 76:625-44. 9. Soli DB. The anophthalmic socket. Ophthalmology 1982; 89:407-23. 10. Tyers AG, Collin JRO. Orbital implants and post enucleation socket syndrome. Trans Ophthalmol Soc UK 1982; 102:90-2. 11. Kennedy RE. The effect of early enucleation on the orbit in animals and humans. Am J Ophthalmol1965; 60:277-306. 12. American Academy of Ophthalmology. Basic and Clinical Science Course; Section 9: Orbit, Eyelids and Lacrimal System. San Francisco: American Academy of Ophthalmology, 1981; 69. 13. Helveston EM. A scleral patch for exposed implants. Trans Am Acad Ophthalmol Otolaryngol 1970; 74: 1307 -10. 14. Dryden R, Leibsohn J. Postenucleation orbital implant extrusion. Arch Ophthalmol1978; 96:2064-5. 15. Allen L. The argument against imbricating the rectus muscles over spherical orbital implants after enucleation. Ophthalmology 1983; 90: 1116-20. 16. Dortzbach RK, Callahan A. Advances in socket reconstruction. Am J Ophthalmol 1970; 70:800-13. 17. Smith B, Petrelli R. Dermis-fat graft as a moveable implant within the muscle cone. Am J Ophthalmol1978; 85:62-6. 18. Dortzbach RK, Alexander SC, Sutula FC, Hawes MJ. Correction of facial contour deformities with prefabricated sculptured implants. Ophthalmology 1981; 88:908-16. 19. Allison SC, Wergeland FL Jr. Giant conjunctival cyst of the orbit. Ann Ophthalmol1973; 5:199-202. 20. Hornblass A, Bosniak S. Orbital cysts following enucleation: the use of absolute alcohol. Ophthalmic Surg 1981; 12:123-6. 21. Lubin JR, Albert OM, Weinstein M. Sixty-five years of sympathetic ophthalmia; a clincopathologic review of 105 cases (1913-1978). Ophthalmology 1980; 87:109-21. 22. Reynard M, Riffenburgh RS, Maes EF. Effect of corticosteroid treatment and enucleation on the visual prognosis of sympathetic ophthalmia. Am J Ophthalmol1983; 96:290-4. 23. Marriott PJ. Impression scleral lens fitting for special and pathological conditions. In: Stone J, Phillips AJ, eds. Contact Lenses; A Textbook for Practitioner and Student, 2d ed. London: Butterworths, 1981; 602. 24. Stone J. Special types of contact lenses and their uses. In: Stone J, Phillips AJ, eds. Contact Lenses; A Textbook for Practitioner and Student, 2d ed. London: Butterworths, 1981; 666. 25. Kersley HJ. Coloured soft contact lenses and the unsightly eye. Trans Ophthalmol Soc UK 1982; 102:101-3.
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