A 20-Year Series of Orbital Exenteration Peter S. Levin, M.D., and Jonathan ]. Dutton, M.D.
We reviewed patient records of 99 consecutive orbital exenterations performed between 1969 and 1988. Patients ranged in age from 2 to 86 years (mean, 55.9 years). Classification of cases on histopathologic criteria showed 32 exenterations were performed for squamous cell carcinoma originating in the paranasal sinus (13), skin (12), conjunctiva (six), and lacrimal sac (one). Orbital exenteration was performed for treatment of other epithelial malignancy in basal cell carcinoma (eight), sebaceous carcinoma (six), adenoid cystic carcinoma (five), undifferentiated carcinoma (four), adenocarcinoma (two), intraepithelial carcinoma of the conjunctiva (two), benign mixed tumor (one), and transitional cell carcinoma (one). Exenterations were performed for melanoma of the conjunctiva (ten), nasosinus (three), skin (two), orbit (two), and choroid (one). Exenterations were also performed as treatment for mucormycosis (five), meningioma (three), fibrosarcoma (two), rhabdomyosarcoma (two), hemangiopericytoma (two), orbital cellulitis (one), fibrous histiocytoma (one), schwannoma (one), lymphangioma (one), benign lymphoepithelial lesion (one), and undifferentiated malignancy (one). ORBITAL EXENTERATION entails removal of the eye together with its extraocular muscles and other soft tissues of the orbit. The procedure is usually performed with reluctance because it generally represents the failure of alternative treatments. We reviewed conditions leading to orbital exenteration in 99 patients at our institution between 1969 and 1988.
Accepted for publication March 28, 1991; revised manuscript received Aug. 8, 1991. From the Department of Ophthalmology, Duke University School of Medicine, Durham, North Carolina. Reprint requests to Peter S. Levin, M.D., Department of Ophthalmology, Stanford Medical Center, Stanford, CA 94305.
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Patients and Methods Since 1969, a computerized database of surgical procedures and discharge diagnoses has been maintained at our institution. A database search of the years 1969 to 1989 disclosed 93 cases of orbital exenteration. A search of files in the eye pathology laboratory yielded an additional six cases. This study would not have identified cases that were not entered into the medical records system or found in eye pathology files. All patient charts were examined to determine the initial clinical features and diagnoses. Follow-up was inconsistent because some patients underwent operations at our institution but received postoperative care at other facilities and were, therefore, lost to our follow-up. In several cases, a death certificate was available, but did not specify whether death was tumor-related.
Results We identified 99 cases (60 males and 39 females) of orbital exenteration. Patients ranged in age from 2 to 86 years (mean, 55.9 years). The indications for an operation were as follows: (1) treatment of life-threatening neoplasm in 88 (89%), (2) treatment of life-threatening infection in six (6%), and (3) treatment of pain or deformity in five (5%). Cases were also classified on confirmed histopathologic criteria as epithelial neoplasms (61), melanoma (18), infectious processes (six), mesenchymal tumors (five), nerve-sheath tumors (four), vascular lesions (three), lymphoproliferative disorders (one), or undifferentiated malignancy (one). Thirty-two of the 61 epithelial neoplasms were exenterated as part of the treatment for squamous carcinoma originating in the paranasal sinus, conjunctiva, skin, or lacrimal sac. In the 13 patients with squamous cell carcinoma
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originating in the paranasal sinus, combined orbital and sinus exenteration was performed. Seven of these patients died from several days to 2Jh years after exenteration (median, nine months). Three survivors were disease-free at 2Jh, seven, and 14 years after exenteration. Three patients were lost to follow-up examination. Six conjunctival squamous cell lesions initially were manifest three months to five years (median, 1Jh years) before exenteration. Three patients died at eight months, two years, and three years after exenteration, whereas three others were free of recurrence at two, three, and six years after the operation. In 12 patients, squamous cell carcinoma originated from a cutaneous site and extended to the orbit. Four patients had initial eyelid involvement and eight others had initial involvement of the cheek, brow, nose, temple, forehead, or multiple periorbital sites. Six known fatalities occurred at one to five years (median, two years) after exenteration. The other six patients were alive at one month to seven years (median, 4Jh months) after the operation. One patient had a squamous cell carcinoma of the lacrimal sac. He died ten years after the operation from cerebrovascular disease. In eight patients, exenteration was performed for orbital extension of eyelid basal cell carcinoma. Five of these patients had initial lesions in the medial canthus. Six patients had previously undergone an operation or radiotherapy. The basal cell nevus syndrome was diagnosed in one patient. Five patients showed no recurrence at 12 months. In one patient, tumor recurred within one year. Two patients were lost to follow-up. Six patients aged 26 to 62 years (median, 58 years) had sebaceous carcinoma. In five patients, eyelid abnormalities had been present for two to 12 years (median, four years) before exenteration. Four patients died at 1Jh to five years after exenteration (median, three years). A fifth patient was alive and well two years postoperatively. No clinical information is available on the 26-year-old patient. Orbital exenteration was performed for 15 other cases of epithelial malignancy. Exenteration was performed for adenoid cystic carcinoma (five), undifferentiated carcinoma (four), adenocarcinoma (two), intraepithelial carcinoma of the conjunctiva (two), benign mixed tumor of the lacrimal gland (one), and transitional cell carcinoma of the lacrimal sac (one). Orbital exenteration was performed for melanomas of the conjunctiva (ten), nasal sinus
(three), orbit (two), choroid with extrascleral extension (one), skin (one), and nevus of Ota with cutaneous malignancy (one). Clinical histories were available in nine of the ten cases of conjunctival melanoma. Abnormal conjunctival pigmentation had been present for two months to 12 years (median, six years) before exenteration. Three patients were alive and well one, two, and 12 years after exenteration. Six patients died two months to ten years after exenteration (median, 1Jh years). Exenterations were also performed as treatment for infection in six patients. All five patients with mucormycosis had diabetes mellitus, and three patients had diabetic ketoacidosis at initial examination. Two of these patients died in the perioperative period. In one patient without evidence of zygomycosis, orbital exenteration was performed in the presence of pansinusitis, panophthalmitis, and central retinal vein occlusion. Acute orbital inflammation and necrosis but no organism was found on laboratory study. Three patients with meningiomas and one patient with an orbital schwannoma progressed to exenteration. All of these patients had progressive proptosis with corneal exposure and compromise. Two cases were of sphenoid wing meningiomas diagnosed 13 and 14 years before extensive orbital extension warranted orbital exenteration. One case was of an orbital meningioma of four years' duration. The patient with the schwannoma had had proptosis for 37 years before orbital exenteration. All patients did well except for one of the patients with a sphenoid wing meningioma who had partial excision of a nasal tumor four years after orbital exenteration and was doing poorly nine years after orbital exenteration. Mesenchymal tumors accounted for five orbital exenterations. Two patients had rhabdomyosarcoma. Chemotherapy and radiation therapy had failed for one patient and the patient died within one year. The other patient received exenteration succeeded by chemotherapy and radiation therapy and was alive 13 years later. Two patients had fibrosarcoma; one had had a previous history of retinoblastoma treated with radiation therapy. Both patients died within two years of exenteration. A patient with an aggressive orbitosinus fibrous histiocytoma was alive and well ten years later. Of three vascular lesions accounting for exenteration, two were hemangiopericytomas. An 18-year-old patient died four months after exenteration. A 26-year-old patient who had
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shown rapid progression of an orbitosinus hemangiopericytoma was alive and well 15 years later. One patient received exenteration as treatment for orbital lymphangioma that had caused severe recurrent orbital bleeding, pain, and a visual acuity of hand motion. One patient received orbital exenteration because of a lacrimal fossa mass that pathologic examination disclosed as a benign Iymphoepithelial lesion (Mikuliczs syndrome). No follow-up was available. One patient had an undifferentiated neoplasm of the maxillary sinus with carcinomatous and sarcomatous elements. This patient was alive and well 14 years later. Four of the 99 patients previously described on the basis of histopathologic criteria had clinically evident lacrimal gland fossa tumors. Two patients had exenteration for adenoid cystic carcinoma. One of these had had eight months of proptosis at the time of initial examination. A recurrence two years later was treated with radiation, and an additional recurrence with lung metastases six years later was treated with chemotherapy. This patient died from the disease 11 years after orbital exenteration. A second patient with a four-week history of orbital disease died of regional and metastatic disease one year after the operation. The one patient with benign mixed tumor of the lacrimal gland had had an incomplete excision with rupture of the pseudocapsule of the tumor 23 years earlier. She had severe proptosis at initial examination and was alive and well 17 years after orbital exenteration. The fourth lacrimal gland fossa mass was in the patient with the benign lymphoepithelial lesion.
Discussion Orbital exenteration causes blindness and deformity. This study shows the following three indications for orbital exenteration performed from 1969 to 1988: (1) eradication of presumed life-threatening malignancy, 89 %; (2) eradication of presumed life-threatening infection, 6%; and (3) alleviation of intractable pain or deformity, 5%. Our study is consistent with other large studies l -5 that showed that orbital exenteration is most frequently performed for the treatment of life-threatening malignancy. Orbital exenteration was performed for presumed life-threaten-
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ing epithelial neoplasm in 61 patients. Our experience was similar to that of other investigators in showing the predominance of epithelial malignancy as the indication for exenteration (Table). Seven of eight exenterations performed for basal cell carcinoma in our study were performed for recurrent lesions. This is consistent with other reports suggesting that recurrent basal cell carcinomas have a greater chance of recurring than do primary tumors.v' Although published studies report that only 10% to 24% of periocular basal cell carcinomas develop at the medial canthus," three of eight basal cell carcinomas ultimately requiring exenteration at our institution originated in the medial canthus and five of eight exenteration specimens had medial orbital involvement on histopathologic examination. This high recurrence rate for medial canthal basal cell carcinomas has been reported.' Adequate treatment of the medial canthus is difficult because of the extensive vessels and nerves that extend from the medial canthus back into the orbit and the presence of the nasolacrimal drainage system. Current guidelines for excision of medial canthal basal cell carcinomav" were confirmed by the experience at our institution. Medial canthal tumors may spread into the orbit and, therefore, medial canthal basal cell carcinomas warrant the most effective primary modalities of treatment. Secondary orbital involvement from paranasal sinus malignancy has been estimated to be 60%.1l,12 In our study, squamous cell carcinoma of the paranasal sinuses accounted for 12 orbital exenterations and sinus melanoma accounted for another three. In three of these cases, final pathologic reports indicated no orbital invasion by tumor, which emphasizes that orbital involvement may be difficult to assess. The indications for orbital exenteration because of neoplasm of the paranasal sinuses may diminish as surgeons supplement clinical observations with the high-resolution orbital and sinus imaging techniques currently available. Our experience with lacrimal gland fossa tumors is consistent with the reported literature. Only one patient with adenoid cystic carcinoma of the lacrimal gland is reported to be alive and well without recurrent tumor 20 years after the operation." Our experience with two patients is consistent with this poor prognosis. Although one of our patients lived for 11 years after exenteration, she had recurrent disease within two years of exenteration and had metastatic disease six years after the operation. The case of
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TABLE A COMPARISON OF ORBITAL EXENTERATION AT SIX INSTITUTIONS' UNIVERSITY OF CALIFORNIA
UNIVERSITY
AT
OF
MAYO
OF
DUKE
WILMER INSTITUTE
SAN FRANCISCO
KANSAS
CLINIC
---
NAPLES
UNIVERSITY
1927-1953
1940-1971
1951-1964
1967-1986
1976-1986
1969-1988
(N
Squamous cell carcinoma Skin Sinus Conjunctiva Tear sac Basal cell carcinoma Sebaceous carcinoma Other epithelial tumors Malignant melanoma Conjunctiva Sinus Choroid Other Rhabdomyosarcoma Infectious Other
= 48)
4 3 1 0 0 11 0 6 12 3 0 9 0 2 1 12
(N
= 48)
6 2 0 3 1 14 1 8 8 5 0 2 1 5 0 6
(N
= 31)
3 1 2 0 0 11 0 5 7
UNIVERSITY
(N
= 102)
33 NAt NA NA NA
21 6 9 16
NA
NA
NA
NA
NA NA
NA
2 0 3
2 1 14
8
(N
~
39)
7 6 1 0 0 9 0 10 8 1 0 7 0 2 0 3
(N
= 99)
32 12 13 6 1 8 6 15 18 10 3 1 4 2 6 12
'At Wilmer, University of California at San Francisco, and University of Naples only, exenterations performed by ophthalmology departments were surveyed; at University of Kansas, Mayo Clinic. and Duke University. all exenterations were reported. regardless of the department performing the operation. tNA indicates no data available.
our patient with benign mixed tumor of the lacrimal gland leading to exenteration attests to the recurrence of locally aggressive, but histopathologically benign tumor when subtotal initial excision is performed.'! Current treatment trends have diminished the life-preserving role of orbital exenteration in certain specific neoplastic diseases. In our study, exenteration was performed in ten patients with conjunctival melanomas who had preexisting pigmentary abnormality for up to 12 years. With the success of earlier aggressive excision and cryotherapy of affected conjunctival tissues, orbital exenteration is now reserved for cases of deep orbital involvement." Previously, exenteration was the accepted primary treatment for orbital rhabdomyosarcoma. In the three published studies ending before 1971,3-5 nine of the 127 orbital exenterations were for rhabdomyosarcoma, whereas in the three studies (including ours) from 1967 to 1989,1,2 rhabdomyosarcoma accounted for six of 239. In our study, only two exenterations were performed for rhabdomyosarcoma and one of these represented the failure of previous nonsurgical therapy. Today, exenteration for rhabdomyosarco-
rna is reserved for tumors unresponsive to other therapies." Orbital exenteration does not affect the ultimate prognosis for life in cases of extrascleral extension of intraocular melanoma.F:" Local resection of melanoma and adjacent Tenon capsule is the treatment of choice for extrascleral extension of choroidal melanoma. Local conjunctival excision may be preferable to exenteration in the management of superficial squamous cell carcinoma of the conjunctiva without demonstrable orbital involvement.P The second category of disease leading to orbital exenteration in our study was lifethreatening infection with mucormycosis leading to five exenterations and orbital cellulitis leading to one exenteration. Rhino-orbital phycomycosis including cases from our institution has previously been reviewed.vf Although orbital exenteration is consistent with the treatment principles of wide debridement of involved tissues, effective treatment of selected patients can be provided without a radical operation.P-" Severe deformity and disability led to orbital exenteration in five patients (5%). Three patients had progressive meningioma with blind-
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ness, proptosis, and exposure keratopathy. One had a schwannoma with marked proptosis and corneal ulceration. The fifth patient had a lymphangioma of the orbit with a visual acuity of hand motion and painful hemorrhages that required recurrent hospitalization. Although not life-prolonging, these procedures were indicated because of severe deformity or pain. Although orbital exenteration remains a viable alternative in such patients, some of them can be treated effectively today with combined intracranial and orbital operations" to excise intraorbital tumor without sacrificing the eye, and with improved cosmesis. Other conditions that are not life-threatening but which may lead to exenteration are severe orbital contracture." severe unremitting orbital inflammatory processes.! and neurofibromatosis with orbital deformity and visual Ioss." An improved spectrum of reconstructive and prosthetic options is now available that makes orbital exenteration a more acceptable procedure than it was in the past." Local reconstructive techniques, primarily split-thickness skin grafting and healing by granulation, result in rapid rehabilitation and allow detection of recurrent disease. Regional reconstructive techniques, such as temporalis muscle transfer procedures":" and galeofrontalis fascial flaps," as well as distant reconstructive techniques, such as free latissimus dorsi myocutaneous free flaps," allow for partial or total obliteration of the exenteration cavity and may provide considerable benefits for the patient who has undergone orbital exenteration. Such reconstructive options may be more acceptable to patients who require exenteration for control of disease.
References 1. Bartley, G. B., Garrity, J. A., Waller, R. R., Henderson, J. W., and Ilstrup, D.M.: Orbital exenteration at the Mayo Clinic. 1967-1986. Ophthalmology 96:468, 1989. 2. de Conciliis, c., and Bonavolonta, G.: Incidence and treatment of dural exposure and CSF leak during orbital exenteration. Ophthalmic Plast. Reconstr. Surg. 3:61, 1987. 3. Naquin, H. A.: Exenteration of the orbit. Arch. Ophthalmol. 51 :850, 1954. 4. Rathbun, J. E., Beard, c.. and Quickert, M. H.: Evaluation of 48 cases of orbital exenteration. Am. J. Ophthalmol. 72: 191, 1971. 5. Simons, J. N., Robinson, D. W., and Masters, F. W.: Malignant tumors of the orbit and periorbital
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structures treated by exenteration. Plast. Reconstr. Surg. 37:100, 1966. 6. Menn, H., Robins, P., Kept, A. W., and Bart, R. S.: The recurrent basal cell carcinoma. Arch. Dermatol. 103:628, 1971. 7. Robins, P., Rodriguez-Sains, R., Rabinovitz, H., and Rigel, D.: Mohs surgery for periocular basal cell carcinomas. J. Dermatol. Surg. Oncol. 11: 1203, 1985. 8. Henkind, P., and Friedman, A.: Cancer of the lids and ocular adnexa. In Andrade, R., Gumport, S. L., Popkin, G. L., and Reese, R. D. (eds.): Cancer of the Skin, vol. 2. Philadelphia, W. B. Saunders Company, 1976, p. 1351. 9. Doxanas, M. T., Green, W. R., and Iliff, C. E.: Factors in the successful surgical management of basal cell carcinoma of the eyelids. Am. J. Ophthalmol. 91:726, 1981. 10. Mohs, F. E.: Micrographic surgery for the microscopically controlled excision of eyelid cancers. Arch. Ophthalmol. 104:901, 1986. 11. Johnson, L. N., Krohel, G. B., Yeon, E. B., and Parnes, S. M.: Sinus tumors invading the orbit. Ophthalmology 91:209, 1984. 12. Conley, J., and Baker, D. c.: Management of the eye socket in cancer of the paranasal sinuses. Arch. Otolaryngol. 105:702, 1979. 13. Henderson, J. W.: Adenoid cystic carcinoma of the lacrimal gland, is there a cure? Trans. Am. Ophthalmol. Soc. 85:312, 1987. 14. Wright, J. E., Stewart, W. B., and Krohel, G. B.: Clinical presentation and management of lacrimal gland tumors. Br. J. Ophthalmol. 63:600, 1979. 15. Iakobiec, F. A., Brownstein, S., Albert, W., Schwarz, F., and Anderson, R.: The role of cryotherapy in the management of conjunctival melanoma. Ophthalmology 89:502, 1982. 16. Wharam, M., Beltangady, M., Hays, D., Heyn, R., Ragab, A., Soule, E., Tefft, M., and Maurer, H.: Localized orbital rhabdomyosarcoma. Ophthalmology 94:251,1987. 17. Kersten, R. c.. Tse, D. T., Anderson, R. L., and Blodi, F. c.: The role of orbital exenteration in choroidal melanoma with extrasc1eral extension. Ophthalmology 92:436, 1985. 18. Pach, J. M., Robertson, D. M., Taney, B. S., Martin, J. A., Cambell, R. J., and O'Brien, P. c. Prognostic factors in choroidal and ciliary body melanomas with extrasc1eral extension. Am. J. Ophthalmol. 101:325, 1986. 19. Rini, F. J., [akobiec, F. A., Hornblass, A., Beckerman, B. L., and Anderson, R. L.: The treatment of advanced choroidal melanoma with massive orbital extension. Am. J. Ophthalmol. 104:634, 1987. 20. Iliff, W. J., Marback, R., and Green, W. R.: Invasive squamous cell carcinoma of the conjunctiva. Arch. Ophthalmol. 93:119,1975. 21. Schwartz, J. N., Donnelly, E. H., and Klintworth, G. K.: Ocular and orbital phycomycosis. Surv. Ophthalmol. 22:3, 1977. 22. Gamba, J. L., Woodruff, W. W., Djang, W. T., and Yeates, A. E.: Craniofacial mucormycosis. Assessment with CT. Radiology 160:207, 1986.
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23. Kohn, R., and Hepler, R.: Management of limited rhino-orbital mucormycosis without exenteration. Ophthalmology 92:1440, 1985. 24. Lee, H. W., and Liao, H. R.: Treatment of rhino-orbital mucormycosis without exenteration. Trans. Ophthalmol. Soc. Repub. China 26:585,1987. 25. Maroon, J. c.. and Kennerdell, J. S.: Surgical approaches to the orbit. Indications and techniques. J. Neurosurg. 60:1226, 1984. 26. Small, R. G., and LaFuente, H.: Exenteration of the orbit in selected cases of several orbital contracture. Ophthalmology 90:236, 1983. 27. Jackson, I. T., Laws, E. R., Jr., and Martin, R. D.: The surgical management of orbital neurofibromatosis. Plast. Reconstr. Surg. 71:751, 1983. 28. Levin, P. S., Ellis, D. S., Stewart, W. B., and Toth, B. A.: Orbital exenteration. The reconstructive ladder. Ophthalmic Plast. Reconstr. Surg. 7:84, 1991.
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29. Holmes, A. D., and Marshall, K. A.: Uses of the tempora lis muscle flap in blanking out the orbits. Plast. Reconstr. Surg. 63:336, 1979. 30. Naquin, H. A.: Orbital reconstruction. Utilizing tempora lis muscle. Am. J. Ophthalmol. 41:519, 1956. 31. Shagets, F. W., Panje, W. R., and Shore, J. W.: Use of temporalis muscle flaps in complicated defects of the head and face. Arch. Otolaryngol. Head Neck Surg. 112:60, 1986. 32. Brent, B., Upton, J., Acland, R. D., Shaw, W. W., Finseth, F. J., Rogers, c.. Pearl, R. M., and Hentz, V. R.: Experience with the temporoparietal fascial flap. Plast. Reconstr. Surg. 76:177, 1985. 33. Donahue, P. J., Liston, S. L., Falconer, D. P., and Manlove, J. c.: Reconstruction of orbital exenteration cavities. The use of the latissimus dorsi myocutaneous free flap. Arch. Ophthalmol. 107:1681,1989.
OPHTHALMIC MINIATURE
He was a touch nearsighted, and the mesh of the window screen seemed more distinct to him than what lay beyond it. What lay beyond it-homehad the blocky, blurred appearance of something worked in needlepoint, each tiny square in the screen filled with a square of color. Not only was there a needlepoint house but also a needlepoint car out front, a needlepoint swing on the porch, a needlepoint bicycle in the yard. Anne Tyler, Saint Maybe New York, Alfred A. Knopf, 1991, p. 182
We reviewed patient records of 99 consecutive orbital exenterations performed between 1969 and 1988. Patients ranged in age from 2 to 86 years (mean, 55.9 years). Classification of cases on histopathologic criteria showed 32 exenterations were performed for squamous cell carcinoma originating in the paranasal sinus (13), skin (12), conjunctiva (six), and lacrimal sac (one). Orbital exenteration was performed for treatment of other epithelial malignancy in basal cell carcinoma (eight), sebaceous carcinoma (six), adenoid cystic carcinoma (five), undifferentiated carcinoma (four), adenocarcinoma (two), intraepithelial carcinoma of the conjunctiva (two), benign mixed tumor (one), and transitional cell carcinoma (one). Exenterations were performed for melanoma of the conjunctiva (ten), nasosinus (three), skin (two), orbit (two), and choroid (one). Exenterations were also performed as treatment for mucormycosis (five), meningioma (three), fibrosarcoma (two), rhabdomyosarcoma (two), hemangiopericytoma (two), orbital cellulitis (one), fibrous histiocytoma (one), schwannoma (one), lymphangioma (one), benign lymphoepithelial lesion (one), and undifferentiated malignancy (one). ORBITAL EXENTERATION entails removal of the eye together with its extraocular muscles and other soft tissues of the orbit. The procedure is usually performed with reluctance because it generally represents the failure of alternative treatments. We reviewed conditions leading to orbital exenteration in 99 patients at our institution between 1969 and 1988.
Accepted for publication March 28, 1991; revised manuscript received Aug. 8, 1991. From the Department of Ophthalmology, Duke University School of Medicine, Durham, North Carolina. Reprint requests to Peter S. Levin, M.D., Department of Ophthalmology, Stanford Medical Center, Stanford, CA 94305.
496
Patients and Methods Since 1969, a computerized database of surgical procedures and discharge diagnoses has been maintained at our institution. A database search of the years 1969 to 1989 disclosed 93 cases of orbital exenteration. A search of files in the eye pathology laboratory yielded an additional six cases. This study would not have identified cases that were not entered into the medical records system or found in eye pathology files. All patient charts were examined to determine the initial clinical features and diagnoses. Follow-up was inconsistent because some patients underwent operations at our institution but received postoperative care at other facilities and were, therefore, lost to our follow-up. In several cases, a death certificate was available, but did not specify whether death was tumor-related.
Results We identified 99 cases (60 males and 39 females) of orbital exenteration. Patients ranged in age from 2 to 86 years (mean, 55.9 years). The indications for an operation were as follows: (1) treatment of life-threatening neoplasm in 88 (89%), (2) treatment of life-threatening infection in six (6%), and (3) treatment of pain or deformity in five (5%). Cases were also classified on confirmed histopathologic criteria as epithelial neoplasms (61), melanoma (18), infectious processes (six), mesenchymal tumors (five), nerve-sheath tumors (four), vascular lesions (three), lymphoproliferative disorders (one), or undifferentiated malignancy (one). Thirty-two of the 61 epithelial neoplasms were exenterated as part of the treatment for squamous carcinoma originating in the paranasal sinus, conjunctiva, skin, or lacrimal sac. In the 13 patients with squamous cell carcinoma
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originating in the paranasal sinus, combined orbital and sinus exenteration was performed. Seven of these patients died from several days to 2Jh years after exenteration (median, nine months). Three survivors were disease-free at 2Jh, seven, and 14 years after exenteration. Three patients were lost to follow-up examination. Six conjunctival squamous cell lesions initially were manifest three months to five years (median, 1Jh years) before exenteration. Three patients died at eight months, two years, and three years after exenteration, whereas three others were free of recurrence at two, three, and six years after the operation. In 12 patients, squamous cell carcinoma originated from a cutaneous site and extended to the orbit. Four patients had initial eyelid involvement and eight others had initial involvement of the cheek, brow, nose, temple, forehead, or multiple periorbital sites. Six known fatalities occurred at one to five years (median, two years) after exenteration. The other six patients were alive at one month to seven years (median, 4Jh months) after the operation. One patient had a squamous cell carcinoma of the lacrimal sac. He died ten years after the operation from cerebrovascular disease. In eight patients, exenteration was performed for orbital extension of eyelid basal cell carcinoma. Five of these patients had initial lesions in the medial canthus. Six patients had previously undergone an operation or radiotherapy. The basal cell nevus syndrome was diagnosed in one patient. Five patients showed no recurrence at 12 months. In one patient, tumor recurred within one year. Two patients were lost to follow-up. Six patients aged 26 to 62 years (median, 58 years) had sebaceous carcinoma. In five patients, eyelid abnormalities had been present for two to 12 years (median, four years) before exenteration. Four patients died at 1Jh to five years after exenteration (median, three years). A fifth patient was alive and well two years postoperatively. No clinical information is available on the 26-year-old patient. Orbital exenteration was performed for 15 other cases of epithelial malignancy. Exenteration was performed for adenoid cystic carcinoma (five), undifferentiated carcinoma (four), adenocarcinoma (two), intraepithelial carcinoma of the conjunctiva (two), benign mixed tumor of the lacrimal gland (one), and transitional cell carcinoma of the lacrimal sac (one). Orbital exenteration was performed for melanomas of the conjunctiva (ten), nasal sinus
(three), orbit (two), choroid with extrascleral extension (one), skin (one), and nevus of Ota with cutaneous malignancy (one). Clinical histories were available in nine of the ten cases of conjunctival melanoma. Abnormal conjunctival pigmentation had been present for two months to 12 years (median, six years) before exenteration. Three patients were alive and well one, two, and 12 years after exenteration. Six patients died two months to ten years after exenteration (median, 1Jh years). Exenterations were also performed as treatment for infection in six patients. All five patients with mucormycosis had diabetes mellitus, and three patients had diabetic ketoacidosis at initial examination. Two of these patients died in the perioperative period. In one patient without evidence of zygomycosis, orbital exenteration was performed in the presence of pansinusitis, panophthalmitis, and central retinal vein occlusion. Acute orbital inflammation and necrosis but no organism was found on laboratory study. Three patients with meningiomas and one patient with an orbital schwannoma progressed to exenteration. All of these patients had progressive proptosis with corneal exposure and compromise. Two cases were of sphenoid wing meningiomas diagnosed 13 and 14 years before extensive orbital extension warranted orbital exenteration. One case was of an orbital meningioma of four years' duration. The patient with the schwannoma had had proptosis for 37 years before orbital exenteration. All patients did well except for one of the patients with a sphenoid wing meningioma who had partial excision of a nasal tumor four years after orbital exenteration and was doing poorly nine years after orbital exenteration. Mesenchymal tumors accounted for five orbital exenterations. Two patients had rhabdomyosarcoma. Chemotherapy and radiation therapy had failed for one patient and the patient died within one year. The other patient received exenteration succeeded by chemotherapy and radiation therapy and was alive 13 years later. Two patients had fibrosarcoma; one had had a previous history of retinoblastoma treated with radiation therapy. Both patients died within two years of exenteration. A patient with an aggressive orbitosinus fibrous histiocytoma was alive and well ten years later. Of three vascular lesions accounting for exenteration, two were hemangiopericytomas. An 18-year-old patient died four months after exenteration. A 26-year-old patient who had
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shown rapid progression of an orbitosinus hemangiopericytoma was alive and well 15 years later. One patient received exenteration as treatment for orbital lymphangioma that had caused severe recurrent orbital bleeding, pain, and a visual acuity of hand motion. One patient received orbital exenteration because of a lacrimal fossa mass that pathologic examination disclosed as a benign Iymphoepithelial lesion (Mikuliczs syndrome). No follow-up was available. One patient had an undifferentiated neoplasm of the maxillary sinus with carcinomatous and sarcomatous elements. This patient was alive and well 14 years later. Four of the 99 patients previously described on the basis of histopathologic criteria had clinically evident lacrimal gland fossa tumors. Two patients had exenteration for adenoid cystic carcinoma. One of these had had eight months of proptosis at the time of initial examination. A recurrence two years later was treated with radiation, and an additional recurrence with lung metastases six years later was treated with chemotherapy. This patient died from the disease 11 years after orbital exenteration. A second patient with a four-week history of orbital disease died of regional and metastatic disease one year after the operation. The one patient with benign mixed tumor of the lacrimal gland had had an incomplete excision with rupture of the pseudocapsule of the tumor 23 years earlier. She had severe proptosis at initial examination and was alive and well 17 years after orbital exenteration. The fourth lacrimal gland fossa mass was in the patient with the benign lymphoepithelial lesion.
Discussion Orbital exenteration causes blindness and deformity. This study shows the following three indications for orbital exenteration performed from 1969 to 1988: (1) eradication of presumed life-threatening malignancy, 89 %; (2) eradication of presumed life-threatening infection, 6%; and (3) alleviation of intractable pain or deformity, 5%. Our study is consistent with other large studies l -5 that showed that orbital exenteration is most frequently performed for the treatment of life-threatening malignancy. Orbital exenteration was performed for presumed life-threaten-
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ing epithelial neoplasm in 61 patients. Our experience was similar to that of other investigators in showing the predominance of epithelial malignancy as the indication for exenteration (Table). Seven of eight exenterations performed for basal cell carcinoma in our study were performed for recurrent lesions. This is consistent with other reports suggesting that recurrent basal cell carcinomas have a greater chance of recurring than do primary tumors.v' Although published studies report that only 10% to 24% of periocular basal cell carcinomas develop at the medial canthus," three of eight basal cell carcinomas ultimately requiring exenteration at our institution originated in the medial canthus and five of eight exenteration specimens had medial orbital involvement on histopathologic examination. This high recurrence rate for medial canthal basal cell carcinomas has been reported.' Adequate treatment of the medial canthus is difficult because of the extensive vessels and nerves that extend from the medial canthus back into the orbit and the presence of the nasolacrimal drainage system. Current guidelines for excision of medial canthal basal cell carcinomav" were confirmed by the experience at our institution. Medial canthal tumors may spread into the orbit and, therefore, medial canthal basal cell carcinomas warrant the most effective primary modalities of treatment. Secondary orbital involvement from paranasal sinus malignancy has been estimated to be 60%.1l,12 In our study, squamous cell carcinoma of the paranasal sinuses accounted for 12 orbital exenterations and sinus melanoma accounted for another three. In three of these cases, final pathologic reports indicated no orbital invasion by tumor, which emphasizes that orbital involvement may be difficult to assess. The indications for orbital exenteration because of neoplasm of the paranasal sinuses may diminish as surgeons supplement clinical observations with the high-resolution orbital and sinus imaging techniques currently available. Our experience with lacrimal gland fossa tumors is consistent with the reported literature. Only one patient with adenoid cystic carcinoma of the lacrimal gland is reported to be alive and well without recurrent tumor 20 years after the operation." Our experience with two patients is consistent with this poor prognosis. Although one of our patients lived for 11 years after exenteration, she had recurrent disease within two years of exenteration and had metastatic disease six years after the operation. The case of
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TABLE A COMPARISON OF ORBITAL EXENTERATION AT SIX INSTITUTIONS' UNIVERSITY OF CALIFORNIA
UNIVERSITY
AT
OF
MAYO
OF
DUKE
WILMER INSTITUTE
SAN FRANCISCO
KANSAS
CLINIC
---
NAPLES
UNIVERSITY
1927-1953
1940-1971
1951-1964
1967-1986
1976-1986
1969-1988
(N
Squamous cell carcinoma Skin Sinus Conjunctiva Tear sac Basal cell carcinoma Sebaceous carcinoma Other epithelial tumors Malignant melanoma Conjunctiva Sinus Choroid Other Rhabdomyosarcoma Infectious Other
= 48)
4 3 1 0 0 11 0 6 12 3 0 9 0 2 1 12
(N
= 48)
6 2 0 3 1 14 1 8 8 5 0 2 1 5 0 6
(N
= 31)
3 1 2 0 0 11 0 5 7
UNIVERSITY
(N
= 102)
33 NAt NA NA NA
21 6 9 16
NA
NA
NA
NA
NA NA
NA
2 0 3
2 1 14
8
(N
~
39)
7 6 1 0 0 9 0 10 8 1 0 7 0 2 0 3
(N
= 99)
32 12 13 6 1 8 6 15 18 10 3 1 4 2 6 12
'At Wilmer, University of California at San Francisco, and University of Naples only, exenterations performed by ophthalmology departments were surveyed; at University of Kansas, Mayo Clinic. and Duke University. all exenterations were reported. regardless of the department performing the operation. tNA indicates no data available.
our patient with benign mixed tumor of the lacrimal gland leading to exenteration attests to the recurrence of locally aggressive, but histopathologically benign tumor when subtotal initial excision is performed.'! Current treatment trends have diminished the life-preserving role of orbital exenteration in certain specific neoplastic diseases. In our study, exenteration was performed in ten patients with conjunctival melanomas who had preexisting pigmentary abnormality for up to 12 years. With the success of earlier aggressive excision and cryotherapy of affected conjunctival tissues, orbital exenteration is now reserved for cases of deep orbital involvement." Previously, exenteration was the accepted primary treatment for orbital rhabdomyosarcoma. In the three published studies ending before 1971,3-5 nine of the 127 orbital exenterations were for rhabdomyosarcoma, whereas in the three studies (including ours) from 1967 to 1989,1,2 rhabdomyosarcoma accounted for six of 239. In our study, only two exenterations were performed for rhabdomyosarcoma and one of these represented the failure of previous nonsurgical therapy. Today, exenteration for rhabdomyosarco-
rna is reserved for tumors unresponsive to other therapies." Orbital exenteration does not affect the ultimate prognosis for life in cases of extrascleral extension of intraocular melanoma.F:" Local resection of melanoma and adjacent Tenon capsule is the treatment of choice for extrascleral extension of choroidal melanoma. Local conjunctival excision may be preferable to exenteration in the management of superficial squamous cell carcinoma of the conjunctiva without demonstrable orbital involvement.P The second category of disease leading to orbital exenteration in our study was lifethreatening infection with mucormycosis leading to five exenterations and orbital cellulitis leading to one exenteration. Rhino-orbital phycomycosis including cases from our institution has previously been reviewed.vf Although orbital exenteration is consistent with the treatment principles of wide debridement of involved tissues, effective treatment of selected patients can be provided without a radical operation.P-" Severe deformity and disability led to orbital exenteration in five patients (5%). Three patients had progressive meningioma with blind-
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ness, proptosis, and exposure keratopathy. One had a schwannoma with marked proptosis and corneal ulceration. The fifth patient had a lymphangioma of the orbit with a visual acuity of hand motion and painful hemorrhages that required recurrent hospitalization. Although not life-prolonging, these procedures were indicated because of severe deformity or pain. Although orbital exenteration remains a viable alternative in such patients, some of them can be treated effectively today with combined intracranial and orbital operations" to excise intraorbital tumor without sacrificing the eye, and with improved cosmesis. Other conditions that are not life-threatening but which may lead to exenteration are severe orbital contracture." severe unremitting orbital inflammatory processes.! and neurofibromatosis with orbital deformity and visual Ioss." An improved spectrum of reconstructive and prosthetic options is now available that makes orbital exenteration a more acceptable procedure than it was in the past." Local reconstructive techniques, primarily split-thickness skin grafting and healing by granulation, result in rapid rehabilitation and allow detection of recurrent disease. Regional reconstructive techniques, such as temporalis muscle transfer procedures":" and galeofrontalis fascial flaps," as well as distant reconstructive techniques, such as free latissimus dorsi myocutaneous free flaps," allow for partial or total obliteration of the exenteration cavity and may provide considerable benefits for the patient who has undergone orbital exenteration. Such reconstructive options may be more acceptable to patients who require exenteration for control of disease.
References 1. Bartley, G. B., Garrity, J. A., Waller, R. R., Henderson, J. W., and Ilstrup, D.M.: Orbital exenteration at the Mayo Clinic. 1967-1986. Ophthalmology 96:468, 1989. 2. de Conciliis, c., and Bonavolonta, G.: Incidence and treatment of dural exposure and CSF leak during orbital exenteration. Ophthalmic Plast. Reconstr. Surg. 3:61, 1987. 3. Naquin, H. A.: Exenteration of the orbit. Arch. Ophthalmol. 51 :850, 1954. 4. Rathbun, J. E., Beard, c.. and Quickert, M. H.: Evaluation of 48 cases of orbital exenteration. Am. J. Ophthalmol. 72: 191, 1971. 5. Simons, J. N., Robinson, D. W., and Masters, F. W.: Malignant tumors of the orbit and periorbital
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structures treated by exenteration. Plast. Reconstr. Surg. 37:100, 1966. 6. Menn, H., Robins, P., Kept, A. W., and Bart, R. S.: The recurrent basal cell carcinoma. Arch. Dermatol. 103:628, 1971. 7. Robins, P., Rodriguez-Sains, R., Rabinovitz, H., and Rigel, D.: Mohs surgery for periocular basal cell carcinomas. J. Dermatol. Surg. Oncol. 11: 1203, 1985. 8. Henkind, P., and Friedman, A.: Cancer of the lids and ocular adnexa. In Andrade, R., Gumport, S. L., Popkin, G. L., and Reese, R. D. (eds.): Cancer of the Skin, vol. 2. Philadelphia, W. B. Saunders Company, 1976, p. 1351. 9. Doxanas, M. T., Green, W. R., and Iliff, C. E.: Factors in the successful surgical management of basal cell carcinoma of the eyelids. Am. J. Ophthalmol. 91:726, 1981. 10. Mohs, F. E.: Micrographic surgery for the microscopically controlled excision of eyelid cancers. Arch. Ophthalmol. 104:901, 1986. 11. Johnson, L. N., Krohel, G. B., Yeon, E. B., and Parnes, S. M.: Sinus tumors invading the orbit. Ophthalmology 91:209, 1984. 12. Conley, J., and Baker, D. c.: Management of the eye socket in cancer of the paranasal sinuses. Arch. Otolaryngol. 105:702, 1979. 13. Henderson, J. W.: Adenoid cystic carcinoma of the lacrimal gland, is there a cure? Trans. Am. Ophthalmol. Soc. 85:312, 1987. 14. Wright, J. E., Stewart, W. B., and Krohel, G. B.: Clinical presentation and management of lacrimal gland tumors. Br. J. Ophthalmol. 63:600, 1979. 15. Iakobiec, F. A., Brownstein, S., Albert, W., Schwarz, F., and Anderson, R.: The role of cryotherapy in the management of conjunctival melanoma. Ophthalmology 89:502, 1982. 16. Wharam, M., Beltangady, M., Hays, D., Heyn, R., Ragab, A., Soule, E., Tefft, M., and Maurer, H.: Localized orbital rhabdomyosarcoma. Ophthalmology 94:251,1987. 17. Kersten, R. c.. Tse, D. T., Anderson, R. L., and Blodi, F. c.: The role of orbital exenteration in choroidal melanoma with extrasc1eral extension. Ophthalmology 92:436, 1985. 18. Pach, J. M., Robertson, D. M., Taney, B. S., Martin, J. A., Cambell, R. J., and O'Brien, P. c. Prognostic factors in choroidal and ciliary body melanomas with extrasc1eral extension. Am. J. Ophthalmol. 101:325, 1986. 19. Rini, F. J., [akobiec, F. A., Hornblass, A., Beckerman, B. L., and Anderson, R. L.: The treatment of advanced choroidal melanoma with massive orbital extension. Am. J. Ophthalmol. 104:634, 1987. 20. Iliff, W. J., Marback, R., and Green, W. R.: Invasive squamous cell carcinoma of the conjunctiva. Arch. Ophthalmol. 93:119,1975. 21. Schwartz, J. N., Donnelly, E. H., and Klintworth, G. K.: Ocular and orbital phycomycosis. Surv. Ophthalmol. 22:3, 1977. 22. Gamba, J. L., Woodruff, W. W., Djang, W. T., and Yeates, A. E.: Craniofacial mucormycosis. Assessment with CT. Radiology 160:207, 1986.
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23. Kohn, R., and Hepler, R.: Management of limited rhino-orbital mucormycosis without exenteration. Ophthalmology 92:1440, 1985. 24. Lee, H. W., and Liao, H. R.: Treatment of rhino-orbital mucormycosis without exenteration. Trans. Ophthalmol. Soc. Repub. China 26:585,1987. 25. Maroon, J. c.. and Kennerdell, J. S.: Surgical approaches to the orbit. Indications and techniques. J. Neurosurg. 60:1226, 1984. 26. Small, R. G., and LaFuente, H.: Exenteration of the orbit in selected cases of several orbital contracture. Ophthalmology 90:236, 1983. 27. Jackson, I. T., Laws, E. R., Jr., and Martin, R. D.: The surgical management of orbital neurofibromatosis. Plast. Reconstr. Surg. 71:751, 1983. 28. Levin, P. S., Ellis, D. S., Stewart, W. B., and Toth, B. A.: Orbital exenteration. The reconstructive ladder. Ophthalmic Plast. Reconstr. Surg. 7:84, 1991.
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29. Holmes, A. D., and Marshall, K. A.: Uses of the tempora lis muscle flap in blanking out the orbits. Plast. Reconstr. Surg. 63:336, 1979. 30. Naquin, H. A.: Orbital reconstruction. Utilizing tempora lis muscle. Am. J. Ophthalmol. 41:519, 1956. 31. Shagets, F. W., Panje, W. R., and Shore, J. W.: Use of temporalis muscle flaps in complicated defects of the head and face. Arch. Otolaryngol. Head Neck Surg. 112:60, 1986. 32. Brent, B., Upton, J., Acland, R. D., Shaw, W. W., Finseth, F. J., Rogers, c.. Pearl, R. M., and Hentz, V. R.: Experience with the temporoparietal fascial flap. Plast. Reconstr. Surg. 76:177, 1985. 33. Donahue, P. J., Liston, S. L., Falconer, D. P., and Manlove, J. c.: Reconstruction of orbital exenteration cavities. The use of the latissimus dorsi myocutaneous free flap. Arch. Ophthalmol. 107:1681,1989.
OPHTHALMIC MINIATURE
He was a touch nearsighted, and the mesh of the window screen seemed more distinct to him than what lay beyond it. What lay beyond it-homehad the blocky, blurred appearance of something worked in needlepoint, each tiny square in the screen filled with a square of color. Not only was there a needlepoint house but also a needlepoint car out front, a needlepoint swing on the porch, a needlepoint bicycle in the yard. Anne Tyler, Saint Maybe New York, Alfred A. Knopf, 1991, p. 182
