S C L E R A L BUCKLING FOR RETINAL D E T A C H M E N T IN PATIENTS W I T H RETINOBLASTOMA S H E L D O N M.
BUZNEY, M.D.,
C H A R L E S D. J. R E G A N , M . D . ,
R O N A L D C. P R U E T T ,
M.D.,
D A V I D S. W A L T O N ,
M.D.,
AND T A Y L O R R. S M I T H , Boston,
M.D.f
Massachusetts
Three children (two girls and one boy) with bilateral retinoblastoma each developed a presumed rhegmatogenous retinal detachment in one eye. All three eyes had previously received radiation and cryotherapy. In each case the retinal detachment responded promptly to convention al surgical methods via scierai buckling in the area of treated retino blastoma and presumed retinal break. All three eyes have retained useful vision for follow-up periods of 3.5 to 12 years. Increased medical awareness, greater use of indirect ophthalmoscopy, com puter-assisted tomography, 1 and innova tive techniques 2 such as aqueous enzyme assay have aided in the early diagnosis of retinoblastoma. Aggressive treatments with radiation, chemotherapy, photocoagulation, cryodestruction, 3 and, more recently, hematoporphyrin photoradiation 4 have further contributed to manage ment of the malignancy without the need for enucleation. However, the ap plication of these methods may induce complications 5,6 that mask the presence of residual viable tumors, introducing the risk of métastases and perhaps com promising efforts to salvage useful vision.
'Died Jan. 20, 1983. Accepted for publication July 24, 1984. From the Retina Associates (Drs. Buzney and Pruett), the Eye Research Institute of Retina Founda tion (Drs. Buzney and Pruett), the Massachusetts Eye and Ear Infirmary (Drs. Buzney, Pruett, Regan, Walton, and Smith), and the Department of Ophthal mology, Harvard Medical School (Drs. Buzney, Pruett, Regan, Walton, and Smith), Boston, Massa chusetts. This study was supported in part by the Massachusetts Lions Eye Research Fund, Inc. Reprint requests to Sheldon M. Buzney, M.D., Library, Eye Research Institute, 20 Staniford St., Boston, MA 02114.
Retinal, choroidal, or vitreous hemor rhage, glial proliferation, retinal detach ment, and cataract are the chief com plications that force a decision either to continue therapy or to remove the eye. 6 We treated three cases in which the occurrence of retinal detachment pre sented such a dilemma. CASE REPORTS Case 1—This child was 1 year old when she was first examined and bilateral retinoblastoma was diag nosed. The left eye showed four large masses, one of which was centered at the macula; there was diffuse vitreous seeding. The right eye had four large masses located in the mid and far periphery; there were tumor "seeds" within the inferior vitreous. The mac ula appeared to be normal. After an examination failed to disclose métastases, the left eye was enucleated and the diagnosis of retinoblastoma was confirmed by pathologic study. The tumors in the right eye were treated with intracarotid actinomycin, methotrexate, and thiotepa, as well as with 4,400 rads of external beam irradiation. Two months after treatment, the tumors in the right eye were significantly smaller and there was complete regression of the vitreous seeds. Xenon photocoagulation was applied to the posterior masses and triple freeze-refreeze cryotherapy to the periph eral tumors. Three months later, the tumors had diminished further, but a shallow retinal detachment was noted extending posteriorly from the equator and involving the entire posterior pole. The retinal surface ap peared irregular, and there was no shifting fluid. Fine new vessels with associated retinal and prereti-
©AMERICAN JOURNAL OF OPHTHALMOLOGY 98:473-477, 1984
473
474
AMERICAN JOURNAL OF OPHTHALMOLOGY
nal hemorrhage as well as glial proliferation were noted around the posterior tumor masses. No retinal breaks were detected. Additional cryotherapy and xenon photocoagulation were applied at this time and again two months later when some of the tumors still appeared to be gelatinous. The retinal detachment remained unchanged. A second examination for mé tastases found none. Three months later, or eight months after initial treatment, the tumor masses appeared to be calcified but the retinal detachment had progressed, extend ing to the ora serrata inferiorly. Repeated examina tions by experienced retinal surgeons failed to locate a retinal break. In view of the progression of the retinal detachment during a five-month period, a scierai buckling procedure was performed using scierai dissection, diathermy within the scierai bed, and implantation of a solid silicone rubber implant beneath the areas previously treated for tumor. Subretinal fluid was drained, and analysis of the fluid by ultrafiltration failed to disclose tumor cells. At the time of surgery, the retina flattened satisfactorily and has remained attached for a follow-up period of 12 years. The patient later underwent extracapsular cataract extraction and currently has a visual acuity of 5/400. Pruett 7 reported this case 27 months after scierai buckling surgery. Case 2—Bilateral retinoblastoma was diagnosed in a 10-month-old girl. The right eye showed a large subretinal tumor at the macula and total retinal detachment. The left eye had two large tumors, with early calcification, inferior to the optic disk; the retina was attached. The right eye was enucleated. The left eye was treated with 4,000 rads of external beam irradiation, which caused the tumors to shrink considerably and calcify further. Several months after completion of radiation thera py, a gray halo was noted around the tumors. Nine months after initial treatment, the halo appeared to be gelatinous, indicating recurrent retinoblastoma. After an examination failed to discover any métasta ses, the tumor masses were treated on two occasions with xenon photocoagulation and several months later by triple-freeze cryotherapy. Later examina tions showed involution of the recurrent tumor with preretinal and vitreous hemorrhage (Fig. 1, top left). Sixteen months after the initial treatment, four months after photocoagulation, and three months after cryotherapy, an extensive retinal detachment involving the macula was found. The retinal surface was irregular, the detachment extended to the ora serrata, and there was no shifting fluid (Fig. 1, top right). Telangiectatic retinal. vessels were present inferiorly with small amounts of hemorrhage, but there was no obvious viable tumor. No retinal break was seen. Retinal detachment surgery was performed with the scierai buckle placed beneath the treated tumors, using the technique described in Case 1 (Fig. 1, bottom left). The retina was reattached. The subretinal fluid was free of tumor cells, and the retina remained attached with involuted tumor for 3.5 years. After an extracapsular cataract extraction, the visual acuity was 1/200.
OCTOBER, 1984
Case 3—A 3-week-old boy with a family history of bilateral retinoblastoma was found to have two mass es consistent with retinoblastoma in the macula of his left eye. Each mass was 2 disk diameters in size. The right eye was free of tumor. After 4,000 rads of external beam radiation therapy was applied to both eyes (to the right eye prophylactically) during a five-week period, the lesions in the left eye became considerably smaller and somewhat calcified. Four months after this treatment, a tumor measur ing 1 disk diameter was noted temporally near the ora serrata of the right eye. The lesion was treated with triple freeze-refreeze cryotherapy. One month later a small retinal hemorrhage was observed along the inferior aspect of the lesion. Twenty-one months after the radiation treatment, a new tumor was noted superonasally near the ora serrata of the left eye and was treated with triple freeze-refreeze cryotherapy. Thirty-two months after the radiation treatment, another new tumor mass was noted anterior to the equator of the left eye at the 3 o'clock position, 'together with some preretinal and vitreous hemor rhage. The small mass received triple freeze-refreeze cryotherapy. Six weeks later a temporal retinal de tachment was noted in the left eye; no definite retinal break was seen, but there was a possible hole at the superior edge of the most recently treated lesion (Fig. 2, left). A scierai buckling operation was per formed without incident (Fig. 2, right). The retina remained attached for a period of four years without tumor recurrence. The visual acuitv in this eye was 20/100. DISCUSSION
In each of our three cases the retinal tumors were treated with radiation and cryotherapy. Two patients also received xenon photocoagulation, and one of the two received systemic chemotherapy. All three showed retinal or vitreous hemor rhages at some time. No definite retinal break was observed in any of the eyes, although all three retinal detachments appeared to be rhegmatogenous and be haved as if the presumed break had been closed by scierai buckling. Although retinal tears were not ob served with certainty in our patients, the combination of atrophie retina and vitre ous traction could result in retinal breaks in some eyes treated for retinoblastoma. Radiation therapy, cryotherapy, and photocoagulation cause atrophy of the retina and choroid, 840 and both cryothera py and photocoagulation can result in
VOL. 98, NO. 4
SCLERAL BUCKLE IN RETINOBLASTOMA
475
Fig. 1 (Buzney and associates). Case 2, left eye. Fundus drawings of a 10-month-old girl with retinoblastoma treated with radiation therapy, xenon photocoagulation, and cryotherapy. Top left, Calci fied tumors (arrows) within zone of chorioretinal atrophy and anterior preretinal and vitreous hemor rhage (black). Top right, Retinal detachment extend ing from the 1 o'clock to the 9 o'clock meridians. Bottom left, Inferior scierai buckle supporting the tumors and surrounding atrophie retina.
strong vitreous adhesions at the margins of scars. 11 Posterior vitreous detachment is associated with vitreous hemorrhage 1 2 such as occurs after radiation retinopathy. 13 Similar vitreoretinal traction would be expected from fibrocellular mem branes as a result of radiation, cryotherapy, or photocoagulation. 14 ' 15 Radiationinduced vaso-occlusive disease produces large areas of ischémie retina, 16 which could be susceptible to such traction. Stress upon compromised retina by con-
tracting forces within the vitreous, per haps mediated by myofibroblasts, 15 · 17 could in this way produce retinal breaks and detachment. Retinal detachment of this type occurs in proliferative diabetic retinopathy. 18 As with diabetic retinopathy, the breaks could be small and diffi cult to detect in a scarred fundus. Chorio retinal atrophy with associated pigment migration after irradiation, photocoagulation, and cryotherapy minimizes the choroidal red reflex, which is helpful in local-
476
AMERICAN JOURNAL OF OPHTHALMOLOGY
OCTOBER, 1984
Fig. 2 (Buzney and associates). Case 3, left eye. Fundus drawings of a 34-month-old boy with retinoblastoma treated with radiation therapy and cryotherapy. Left, Treated retinoblastoma (arrows); preretinal and vitreous hemorrhage (black) with retinal detachment extends from the 2 o'clock to the 4 o'clock meridians. Right, Temporal scierai buckle supporting an area of tumor and previous retinal detachment.
izing a retinal break; gliotic changes within or beneath the retina further im pair the detection of small retinal holes. Inasmuch as our patients required exami nation under anesthesia, extensive biomicroscopic study was not possible. Patients 1 and 2 may have harbored retinal breaks within areas of hemorrhage, as was sus pected in Patient 3. Choroidal exudation sometimes ac counts for the presence of subretinal fluid in patients treated for retinoblastoma, but such fluid usually resorbs spontane ously within weeks. 19 In contrast to the findings in our patients, the fluid of cho roidal effusions is usually cloudy, shifts with a change in position of the patient's head, presents a smooth elevation of the retina, and does not extend to the ora serrata. 20 If our patients had displayed choroidal effusion rather than rhegmatogenous retinal detachment, manipulation of damaged choroid, as was done during scierai buckling with drainage of subreti nal fluid, would probably have produced
a postoperative recurrence of the effu sion. Exophytic growth of retinoblastoma can also produce a retinal detachment that is extensive, highly elevated, and different in appearance from the detach ments we report. 20 Scierai buckling is contraindicated in such cases; drainage of subretinal fluid would permit access of tumor cells to the orbit and systemic vasculature. One would expect to recover tumor cells in the subretinal fluid of reti nal detachments caused by exophytic growth; all of our specimens were nega tive. Our experience underscores one prob lem encountered in caring for patients with retinoblastoma. Repetitive, destruc tive treatments for retinoblastoma appear to produce changes at the vitreoretinal interface that can result in rhegmatogenous retinal detachment. For this reason, in multiple-mode therapeutic approach es, successive applications of therapy should perhaps be less vigorous than if
VOL. 98, NO. 4
S C L E R A L BUCKLE IN
the same treatment were applied alone. If the tumors have been destroyed and there is no evidence of metastasis, con ventional scierai buckling techniques can be effective in reattaching the retina in presumed rhegmatogenous retinal de tachment and preserving vision without untoward risk to life. This is particularly important in one-eyed children. Cytolog ie examination of subretinal fluid, al though its reliability is unproven, is nevertheless advised. In our three pa tients, at least partial sight in eyes treated for retinal detachment has been retained for follow-up periods of 3.5 to 12 years. REFERENCES 1. Harris, G. J., Williams, A. L., Reeser, F. H., and Abrams, G. W. : Intraocular evaluation by com puted tomography. Int. Ophthalmol. Clin. 22:197, 1982. 2. Piro, P. A., Jr., Abramson, D. H., Ellsworth, R. M., and Kitchin, D.: Aqueous humor lactate dehydrogenase in retinoblastoma patients. Arch. Ophthalmol. 96:1823, 1978. 3. Abramson, D. H.: Retinoblastoma. Diagnosis and management.. CA 32:130, 1982. 4. Murphree, A. L., Doiron, D. R., Gomer, C. J., and Szirth, R. C : Clinical application ofhematoporphyrin photoradiation therapy in recurrent reti noblastoma. ARVO Abstracts. Supplement to Invest. Ophthalmol. Vis. Sei. St. Louis, C. V. Mosby, 1982, p. 7. 5. Egbert, P. R., Donaldson, S. S., Moazed, K., and Rosenthal, A. R. : Visual results and ocular com plications following radiotherapy for retinoblastoma. Arch. Ophthalmol. 96:1826, 1978. 6. Abramson, D. H., and Ellsworth, R. M.: The surgical management of retinoblastoma. Ophthalmic Surg. 11:596, 1980. 7. Pruett, R. C. : Scierai buckling in retinoblasto ma. Case report. Ann. Ophthalmol. 7:1115, 1975.
RETINOBLASTOMA
477
8. Egbert, P. R., Fajardo, L. F., Donaldson, S. S., and Moazed, K.: Posterior ocular abnormali ties after irradiation for retinoblastoma. A histopathological study. Br. J. Ophthalmol. 64:660, 1980. 9. LincofF, H., Long, R., Marquardt, J., and McLean, J.: The cryosurgical adhesion. Trans. Am. Acad. Ophthalmol. Otolaryngol. 72:191, 1968. 10. Marshall, J., and Bird, A. C : A comparative histopathological study of argon and krypton laser irradiations of the human retina. Br. J. Ophthalmol. 63:657, 1979. 11. LincofF, H. A., and McLean, J. M.: Cryosur gical treatment of retinal detachment. Part II. Trans. Am. Acad. Ophthalmol. Otolaryngol. 70:202, 1966. 12. Tolentino, F. I., Schepens, C. L., and Free man, H. M.: Vitreoretinal Disorders. Diagnosis and Management. Philadelphia, W. B. Saunders, 1976, p. 444. 13. Howard, G. M.: Ocular effects of radiation and photocoagulation. Arch. Ophthalmol. 76:7, 1966. 14. Martin, H., and Reese, A. B.: Treatment of retinoblastoma (retinal glioma) surgically and by irra diation. Arch. Ophthalmol. 27:40, 1942. 15. Margo, C., Hidayat, A. A., Marshall, C. F., and Renaldo, D. P.: Cryotherapy and photocoagulation in the management of retinoblastoma. Treat ment failure and unusual complication. Ophthalmic Surg. 14:336, 1983. 16. Irvine, A. R., Alvarado, J. A., Wara, W. M., Morris, B. W., and Wood, I. S.: Radiation retinopa thy. An experimental model for the ischemicproliferative retinopathies. Trans. Am. Ophthalmol. Soc. 79:103, 1981. 17. Fastenberg, D. M., Diddie, K. R., Sorgente, N., and Ryan, S. J.: A comparison of different cellu lar inocula in an experimental model of massive periretinal proliferation. Am. J. Ophthalmol 93:559, 1982. 18. Jalkh, A., Takahashi, M., Topilow, H. W., Trempe, C. L., and McMeel, J. W.: Prognostic value of vitreous findings in diabetic retinopathy. Arch. Ophthalmol. 100:432, 1982. 19. Abramson, D. H., Ellsworth, R. M., and Rozakis, G. W. : Cryotherapy for retinoblastoma. Arch. Ophthalmol. 100:1253, 1982. 20. Schepens, C. L. : Retinal Detachment and Al lied Diseases. Philadelphia, W. B. Saunders, 1983, vol. 1, pp. 224 and 225.