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Subretinal Aspiration Biopsy of Ocular Lymphoma
and taking in sufficient amounts of fluid, can reduce occurrences of acute mountain sickness and highaltitude retinopathy. People who have previously experienced mountain sickness are at higher risk for complications associated with high altitude. When hemoconcentration is present in patients with highaltitude retinopathy, hemodilution therapy is recom mended. Because high-altitude exposure impairs the microcirculation, prophylactic hemodilution after de scent may prevent high-altitude complications. REFERENCES 1. Zink RA, Schaffert W, Messmer K, Brendel W. Hemodilu tion: practical experience in high altitude expeditions. In: Brendel W, Zink RA, editors. High altitude physiology and medicine. New York: Springer Verlag, 1982:291-297. 2. Lubin JR, Rennie D, Hackett P, Albert DM. High altitude retinal hemorrhage: a clinical and pathological case report. A n n Ophthalmol 1982;14:1071-1076. 3. Wiedman M. High altitude retinal hemorrhage. Arch Oph thalmol 1975;93:401-403. 4- Rennie D, Morrissey J. Retinal changes in Himalayan climb ers. Arch Ophthalmol 1975;93:395-400. 5. Shults WT, Swan KC. High altitude retinopathy in mountain climbers. Arch Ophthalmol 1975;93:404-408.
Subretinal Aspiration Biopsy of Ocular Lymphoma Thomas A. Ciulla, MD, Richard D. Pesavento, MD, and Sonia Yoo, MD PURPOSE: Large-cell lymphoma can be difficult to diagnose because vitreous biopsies may fail to disclose neoplastic cells. METHODS: We report one such case in which diagnosis was confirmed by subretinal aspiration of yellow-white infiltrates using a pars plana ap proach. RESULTS: A 67-year-old woman with recurrent bilateral uveitis was diagnosed with large-cell lym phoma by subretinal aspiration of yellow-white infiltrates after two vitreous biopsies and a concur rent retinal biopsy failed to confirm the diagnosis. CONCLUSION: When the suspicion of intraocular lymphoma remains high despite previous negative vitreous biopsies, retinal biopsy and aspiration
420
biopsy of subretinal lesions may enhance the diag nostic yield.
A
LTHOUGH
OPHTHALMOLOGISTS
HAVE
BECOME
more knowledgeable about large-cell lymphoma, it can be difficult to diagnose because vitreous biop sies may fail to disclose malignant cells.1'3 We report one such case in which diagnosis was confirmed by subretinal aspiration of the yellow-white infiltrates using a pars plana approach after two vitreous biopsies and a concurrent retinal biopsy failed to confirm the diagnosis. A 67-year-old woman with recurrent bilateral uvei tis had undergone a noncontributory vitreous biopsy (cytologic examination of the vitreous cytospin) in the right eye 1 year before initial examination, after she had received numerous courses of corticosteroids. After cataract extraction in her right eye 4 months before initial examination, inflammation increased and new yellow-white subretinal infiltrates in the inferior periphery developed. A second vitreous biop sy (cytologic examination of the vitreous cytospin) 2 months before initial examination had disclosed only inflammatory cells. Systemic examination was nor mal. One month before initial examination, she developed white retinitis in the temporal periphery of the right eye. Neurologic examination, lumbar punc ture, and magnetic resonance imaging of the brain were all normal. At initial examination, best-corrected visual acuity was RE, 20/40 and LE, 20/25. In her right eye, the patient had mild anterior segment inflammation, moderate vitreous cells, normal disk, and normal fovea. In the inferior periphery, there was vascular nonperfusion, a region of white retinitis, and numer ous yellow-white subretinal infiltrates (Figure 1). The left eye was normal, with no uveitis. In the right eye, we performed pars plana vitrectomy and vitreous biopsy, during which a retinal biopsy was obtained inferiorly over a subretinal infilAccepted for publication Oct 7, 1996. Department of Ophthalmology, Indiana University School of Medi cine (T.A.C.); Ophthalmic Consultants of Boston (R.D.P.); and Depart ment of Ophthalmology, Massachusetts Eye and Ear Infirmary and Harvard Medical School (R.D.P., S.Y.). Supported in part by the Heed Ophthalmic Foundation. Inquiries to Richard D. Pesavento, MD, Ophthalmic Consultants of Boston, 50 Staniford St, Boston, MA 02114; fax: (617) 723-7028.
AMERICAN JOURNAL OF OPHTHALMOLOGY
MARCH
1997
Figure 1. On ophthalmoscopic examination, numerous yellow-white subretinal infiltrates, some of which are confluent (arrow), are visible inferiorly.
trate. The chosen retinal biopsy site was pretreated with transvitreal diathermy at its margins, sectioned with intraocular scissors, and recovered from the eye through a sclerotomy port. The exposed yellow-white lesion was aspirated directly from the surface of Bruch's membrane with a soft-tipped flute needle connected to a tuberculin syringe. The margins of the retinotomy were then photocoagulated. O n histologic examination, the retinal biopsy dis closed no evidence of tumor or sarcoidosis. The aspirate of the subretinal infiltrates, however, showed high-grade malignant lymphoma on cytologic smear (Figure 2). The vitreous biopsy had been misprocessed and was noncontributory. O n follow-up 3 months after completing a course of intravenous cytarabine and external-beam radiation, visual acuity measured 20/40; there were no postoperative compli cations, and the uveitis, as well as the subretinal lesions, had completely resolved. Large-cell lymphoma is a highly malignant intraoc ular neoplasm that carries a particularly poor progno-
jfc
Figure 2. Cytologic smear of the aspirate of the subretinal infiltrates shows atypical lymphocytes with pleomorphic nuclei (arrow) and prominent nucleoli consistent with high-grade malignant lymphoma (hematoxylin and eosin).
VOL.123, No. 3
BRIEF REPORTS
421
sis, with a median survival of 20 months from the time of diagnosis.4 Vitreous biopsies, however, may not disclose the malignant cells because they may not spill over into the vitreous cavity.1'3 Authors have described transscleral biopsy of subretinal lesions in cases in which vitreous biopsies were noncontributory.5 This approach, however, is technically difficult and can lead to choroidal hemorrhage. In this report, aspiration biopsy of subretinal le sions, using a pars plana approach, confirmed the diagnosis. This approach can provide concurrent vitreous, retinal, and subretinal tissue for histologic examination. When the suspicion of intraocular lymphoma remains high despite previous negative vitreous biopsies, retinal biopsy and aspiration biopsy of subretinal lesions using a pars plana approach may yield pathologic confirmation of the diagnosis. REFERENCES 1. Blumenkranz MS, Ward T, Murphy S, Mieler W, Williams GA, Long J. Applications and limitations of vitreoretinal biopsy techniques in intraocular large cell lymphoma. Retina 1992;12(3 Suppl):S64-S70. 2. Char DH, Ljung BM, Miller T, Phillips T. Primary intraocular lymphoma (ocular reticulum cell sarcoma) diagnosis and management. Ophthalmology 1988;95:625-630. 3. Michels RG, Knox DL, Erozan YS, Green WR. Intraocular reticulum cell sarcoma: diagnosis by pars plana vitrectomy. Arch Ophthalmol 1975;93:1331-1335. 4. Freeman LN, Schachat AP, Knox DL, Michels RG, Green WR. Clinical features, laboratory investigations, and survival in ocular reticulum cell sarcoma. Ophthalmology 1987; 94:1631-1639. 5. Kirmani MH, Thomas EL, Rao NA, Laborde RP. Intraocular reticulum cell sarcoma: diagnosis by choroidal biopsy. Br J Ophthalmol 1987;71:748-752.
Bilateral Optic Disk Edema With Macular Exudates as the Manifesting Sign of a Cerebral Arteriovenous Malformation
an unruptured frontal lobe arteriovenous malfor mation who had decreased visual acuity, bilateral optic disk edema, and bilateral macular exudates. RESULTS: The arteriovenous malformation was treated with partial embolization. A ventriculoperitoneal shunt was placed, but the patient's visual function did not improve. CONCLUSIONS: Although optic disk edema with macular exudates may be caused by neuroretinitis, ophthalmologists should consider increased intracranial pressure as a cause of these ophthalmoscopic findings, especially when atypical features are present, such as bilaterality, lack of vitreous cells, lack of infectious risk factors, absent sponta neous venous pulsations, or no significant visual recovery.
C
EREBRAL ARTERIOVENOUS
MALFORMATIONS
ARE
congenital vascular lesions characterized by the absence of a capillary bed. Patients with cerebral arteriovenous malformations may experience head aches, seizures, or focal neurologic deficit because of intracranial or subarachnoid hemorrhage.1 Papilledema, however, is an uncommon initial sign of arterio venous malformations and, when present, it is usually associated with subarachnoid hemorrhage or hydro cephalus.2 In December 1995, an 11-year-old girl with a 3-month history of daily headaches and difficulty reading was examined. An ophthalmologist found a best-corrected visual acuity of RE, 20/20 and LE, 20/100; bilateral optic disk edema; and bilateral macular exudates. A diagnosis of neuroretinitis was made. She was treated empirically with rifampin and intravenous methylprednisolone acetate. Lyme titer and syphilis serology were negative. A computed tomographic brain scan and subsequent cerebral arteriography showed a right frontal arteriovenous malformation. There was no evidence of subarach noid hemorrhage or hydrocephalus. A lumbar punc-
Alan Verm, BA, and Andrew G. Lee, MD PURPOSE: To report that an unruptured arterio venous malformation without hydrocephalus may manifest with bilateral optic disk edema and macu lar exudates. METHODS: We examined an 11-year-old girl with 422
Accepted for publication Oct 17, 1996. Baylor College of Medicine (A.V.) and Departments of Ophthalmolo gy, Neurology, and Neurosurgery, Baylor College of Medicine (A.G.L.). This work was supported in part by a grant from Research to Prevent Blindness, Inc, New York, New York (A.G.L.). Inquiries to Andrew G. Lee, MD, Departments of Ophthalmology, Neurology, and Neurosurgery, 6501 Fannin St, NC-200, Baylor College of Medicine, Houston, TX 77030; fax: (713) 798-4364; e-mail: alee® bcm.tmc.edu
AMERICAN JOURNAL OF OPHTHALMOLOGY
MARCH
1997
Subretinal Aspiration Biopsy of Ocular Lymphoma Thomas A. Ciulla, MD, Richard D. Pesavento, MD, and Sonia Yoo, MD PURPOSE: Large-cell lymphoma can be difficult to diagnose because vitreous biopsies may fail to disclose neoplastic cells. METHODS: We report one such case in which diagnosis was confirmed by subretinal aspiration of yellow-white infiltrates using a pars plana ap proach. RESULTS: A 67-year-old woman with recurrent bilateral uveitis was diagnosed with large-cell lym phoma by subretinal aspiration of yellow-white infiltrates after two vitreous biopsies and a concur rent retinal biopsy failed to confirm the diagnosis. CONCLUSION: When the suspicion of intraocular lymphoma remains high despite previous negative vitreous biopsies, retinal biopsy and aspiration
420
biopsy of subretinal lesions may enhance the diag nostic yield.
A
LTHOUGH
OPHTHALMOLOGISTS
HAVE
BECOME
more knowledgeable about large-cell lymphoma, it can be difficult to diagnose because vitreous biop sies may fail to disclose malignant cells.1'3 We report one such case in which diagnosis was confirmed by subretinal aspiration of the yellow-white infiltrates using a pars plana approach after two vitreous biopsies and a concurrent retinal biopsy failed to confirm the diagnosis. A 67-year-old woman with recurrent bilateral uvei tis had undergone a noncontributory vitreous biopsy (cytologic examination of the vitreous cytospin) in the right eye 1 year before initial examination, after she had received numerous courses of corticosteroids. After cataract extraction in her right eye 4 months before initial examination, inflammation increased and new yellow-white subretinal infiltrates in the inferior periphery developed. A second vitreous biop sy (cytologic examination of the vitreous cytospin) 2 months before initial examination had disclosed only inflammatory cells. Systemic examination was nor mal. One month before initial examination, she developed white retinitis in the temporal periphery of the right eye. Neurologic examination, lumbar punc ture, and magnetic resonance imaging of the brain were all normal. At initial examination, best-corrected visual acuity was RE, 20/40 and LE, 20/25. In her right eye, the patient had mild anterior segment inflammation, moderate vitreous cells, normal disk, and normal fovea. In the inferior periphery, there was vascular nonperfusion, a region of white retinitis, and numer ous yellow-white subretinal infiltrates (Figure 1). The left eye was normal, with no uveitis. In the right eye, we performed pars plana vitrectomy and vitreous biopsy, during which a retinal biopsy was obtained inferiorly over a subretinal infilAccepted for publication Oct 7, 1996. Department of Ophthalmology, Indiana University School of Medi cine (T.A.C.); Ophthalmic Consultants of Boston (R.D.P.); and Depart ment of Ophthalmology, Massachusetts Eye and Ear Infirmary and Harvard Medical School (R.D.P., S.Y.). Supported in part by the Heed Ophthalmic Foundation. Inquiries to Richard D. Pesavento, MD, Ophthalmic Consultants of Boston, 50 Staniford St, Boston, MA 02114; fax: (617) 723-7028.
AMERICAN JOURNAL OF OPHTHALMOLOGY
MARCH
1997
Figure 1. On ophthalmoscopic examination, numerous yellow-white subretinal infiltrates, some of which are confluent (arrow), are visible inferiorly.
trate. The chosen retinal biopsy site was pretreated with transvitreal diathermy at its margins, sectioned with intraocular scissors, and recovered from the eye through a sclerotomy port. The exposed yellow-white lesion was aspirated directly from the surface of Bruch's membrane with a soft-tipped flute needle connected to a tuberculin syringe. The margins of the retinotomy were then photocoagulated. O n histologic examination, the retinal biopsy dis closed no evidence of tumor or sarcoidosis. The aspirate of the subretinal infiltrates, however, showed high-grade malignant lymphoma on cytologic smear (Figure 2). The vitreous biopsy had been misprocessed and was noncontributory. O n follow-up 3 months after completing a course of intravenous cytarabine and external-beam radiation, visual acuity measured 20/40; there were no postoperative compli cations, and the uveitis, as well as the subretinal lesions, had completely resolved. Large-cell lymphoma is a highly malignant intraoc ular neoplasm that carries a particularly poor progno-
jfc
Figure 2. Cytologic smear of the aspirate of the subretinal infiltrates shows atypical lymphocytes with pleomorphic nuclei (arrow) and prominent nucleoli consistent with high-grade malignant lymphoma (hematoxylin and eosin).
VOL.123, No. 3
BRIEF REPORTS
421
sis, with a median survival of 20 months from the time of diagnosis.4 Vitreous biopsies, however, may not disclose the malignant cells because they may not spill over into the vitreous cavity.1'3 Authors have described transscleral biopsy of subretinal lesions in cases in which vitreous biopsies were noncontributory.5 This approach, however, is technically difficult and can lead to choroidal hemorrhage. In this report, aspiration biopsy of subretinal le sions, using a pars plana approach, confirmed the diagnosis. This approach can provide concurrent vitreous, retinal, and subretinal tissue for histologic examination. When the suspicion of intraocular lymphoma remains high despite previous negative vitreous biopsies, retinal biopsy and aspiration biopsy of subretinal lesions using a pars plana approach may yield pathologic confirmation of the diagnosis. REFERENCES 1. Blumenkranz MS, Ward T, Murphy S, Mieler W, Williams GA, Long J. Applications and limitations of vitreoretinal biopsy techniques in intraocular large cell lymphoma. Retina 1992;12(3 Suppl):S64-S70. 2. Char DH, Ljung BM, Miller T, Phillips T. Primary intraocular lymphoma (ocular reticulum cell sarcoma) diagnosis and management. Ophthalmology 1988;95:625-630. 3. Michels RG, Knox DL, Erozan YS, Green WR. Intraocular reticulum cell sarcoma: diagnosis by pars plana vitrectomy. Arch Ophthalmol 1975;93:1331-1335. 4. Freeman LN, Schachat AP, Knox DL, Michels RG, Green WR. Clinical features, laboratory investigations, and survival in ocular reticulum cell sarcoma. Ophthalmology 1987; 94:1631-1639. 5. Kirmani MH, Thomas EL, Rao NA, Laborde RP. Intraocular reticulum cell sarcoma: diagnosis by choroidal biopsy. Br J Ophthalmol 1987;71:748-752.
Bilateral Optic Disk Edema With Macular Exudates as the Manifesting Sign of a Cerebral Arteriovenous Malformation
an unruptured frontal lobe arteriovenous malfor mation who had decreased visual acuity, bilateral optic disk edema, and bilateral macular exudates. RESULTS: The arteriovenous malformation was treated with partial embolization. A ventriculoperitoneal shunt was placed, but the patient's visual function did not improve. CONCLUSIONS: Although optic disk edema with macular exudates may be caused by neuroretinitis, ophthalmologists should consider increased intracranial pressure as a cause of these ophthalmoscopic findings, especially when atypical features are present, such as bilaterality, lack of vitreous cells, lack of infectious risk factors, absent sponta neous venous pulsations, or no significant visual recovery.
C
EREBRAL ARTERIOVENOUS
MALFORMATIONS
ARE
congenital vascular lesions characterized by the absence of a capillary bed. Patients with cerebral arteriovenous malformations may experience head aches, seizures, or focal neurologic deficit because of intracranial or subarachnoid hemorrhage.1 Papilledema, however, is an uncommon initial sign of arterio venous malformations and, when present, it is usually associated with subarachnoid hemorrhage or hydro cephalus.2 In December 1995, an 11-year-old girl with a 3-month history of daily headaches and difficulty reading was examined. An ophthalmologist found a best-corrected visual acuity of RE, 20/20 and LE, 20/100; bilateral optic disk edema; and bilateral macular exudates. A diagnosis of neuroretinitis was made. She was treated empirically with rifampin and intravenous methylprednisolone acetate. Lyme titer and syphilis serology were negative. A computed tomographic brain scan and subsequent cerebral arteriography showed a right frontal arteriovenous malformation. There was no evidence of subarach noid hemorrhage or hydrocephalus. A lumbar punc-
Alan Verm, BA, and Andrew G. Lee, MD PURPOSE: To report that an unruptured arterio venous malformation without hydrocephalus may manifest with bilateral optic disk edema and macu lar exudates. METHODS: We examined an 11-year-old girl with 422
Accepted for publication Oct 17, 1996. Baylor College of Medicine (A.V.) and Departments of Ophthalmolo gy, Neurology, and Neurosurgery, Baylor College of Medicine (A.G.L.). This work was supported in part by a grant from Research to Prevent Blindness, Inc, New York, New York (A.G.L.). Inquiries to Andrew G. Lee, MD, Departments of Ophthalmology, Neurology, and Neurosurgery, 6501 Fannin St, NC-200, Baylor College of Medicine, Houston, TX 77030; fax: (713) 798-4364; e-mail: alee® bcm.tmc.edu
AMERICAN JOURNAL OF OPHTHALMOLOGY
MARCH
1997