To study the effects of subretinal fluid shift on the visual function, we recorded 30-Hz flicker ERG using a contact lens elec¬ trode with a built-in light-emitting diode,3 which provided constant flicker illumination under the following positions: supine, lateral (right down), and sitting. The visual field was also measured with a Forster perimeter un¬ der a photopic condition (500 lux) at each po¬ sition (test target, 10 mm in diameter). The Figure shows the fundus photo¬ graphs, flicker ERGs, and visual fields of the left eye. In the sitting position (top) retinal detachment localized in the lower quadrant and the superior visual field was lost. In the lateral position (center) the subretinal fluid shifted to the nasal quadrant, causing a de¬ fect in lateral visual field. In the supine po¬ sition (bottom), although the entire retina was detached, the visual field was preserved in the central portion. In all three positions, the flicker ERG showed a similar small response (23 µ ) with normal peak time. The right eye showed a normal visual field and normal flicker ERG (80 µ ).
Comment.—Unlike
cases
of
rheg-
matogenous retinal detachment, our pa¬
tient showed almost constant ERG am¬ plitude regardless of detached area, and visual field was preserved even when the entire retina was detached. These results cannot be explained simply as retinal functional impairment caused by separation of the sensory retina and the retinal pigment epithelium. We there¬ fore propose the following hypotheses.1 The damage to the photoreceptor cells was not as severe as that noted in rhegmatogenous retinal detachment, and it was uniform regardless of the area of the retinal detachment.2 Stiles-Crawford ef¬ fect or similar orientation problems con¬ tributed to the field defects in addition to retinal functional damage by chronic sep¬ aration or lack of nutrients. These hy¬ potheses allow the following explanation of our data. With a partially detached ret¬ ina, both retinal dysfunction, induced by retinal detachment, and a defocusing or Stiles-Crawford effect, induced by high elevation of the detached retina, caused the visual field defect. When the entire retina was detached, however, the eleva¬ tion was lower; the central retina, having the highest cone density, was able to pro¬ duce a measurable visual field. For the same reason, the flicker ERG showed a detectable response under all positions. A recent study has demonstrated that the ERG can be recorded from the detached macula in central serous retinopathy .4 Se¬ rous fluid could retain nutrients and avoid the possible deleterious effects of vitre¬ ous material in the subretinal space. Masayuki Horiguchi, MD Yozo Miyake, MD
Nagoya, Japan
Reprint requests to the Department of Ophthalmol¬ Nagoya University School of Medicine, 65
ogy,
appeared massively swollen by a dense white (Fig 1). The central retinal
Tsuruma-cho, Showa-ku, Nagoya 466, Japan (Dr Horiguchi).
tumor infiltrate
1. Harrington DO, Drake MV. The visual field. In: Text and Atlas of Clinical Perimetry. St Louis, Mo: CV Mosby Co; 1990:177-178. 2. Rendahl I. The electroretinogram in detachment of the retina. Arch Ophthalmol. 1957;57:566\x=req-\ 576. 3. Miyake Y, Yagasaki K, Horiguchi M. Electroretinographic monitor of retinal function during eye surgery. Arch Ophthalmol. 1991;109:1123-1126. 4. Miyake Y, Shiroyama N, Ota I, Horiguchi M. Local macular electroretinographic responses in idiopathic central serous retinopathy. Am J Ophthalmol. 1988;106:546-550.
was occluded and the retina was edematous. There was hemorrhage in the deep and superficial layers of the retina as well as within the vitreous cavity. The blood column within the retinal veins appeared abnormally dark. A subsequent lumbar puncture confirmed the presence of blasts in the cerebrospinal fluid. In Trweighted coronal magnetic reso¬ nance images with gadolinium, presumed tumor metastasis appeared as a ring of bright signal surrounding the optic nerve
artery
Magnetic Resonance Imaging of Leukemic Invasion of the Optic Nerve As treatment regimens for acute leukemia continue to improve, patients are relapsing more frequently with tumor involvement of the central nervous system.1 The central nervous system provides a sanctuary for leukemic cells because of the poor penetration of chemotherapeutic agents across the blood-brain barrier. Consequently, with prolonged patient survival, late invasion of the optic nerve by neoplastic cells is no longer a rare clinical manifestation of leukemia. Computed tomography has been a rather insensitive method for detecting abnormalities in patients with leukemic infiltration of the optic nerve. Although in some patients it has shown a thickened optic nerve shadow or enhancement of the perioptic meninges after contrast administration, in other patients it has revealed nothing.2,3 We provide the first description of the magnetic resonance findings in a patient with presumed leukemic invasion of the optic nerve.
Fig 1.—Fundus of the left eye shows en¬ gorgement of the optic disc by infiltrating tu¬ mor. There is extensive inal edema.
hemorrhage and ret¬
Report of a Case.\p=m-\A58-year-old man developed fever, lethargy, and gingival bleeding in January 1991. The white blood cell count was 165X107L (165 000 cells per mm3), and the peripheral smear showed sheets of blasts. A diagnosis was made of acute myelocytic leukemia. Complete remission was induced with cytarabine hydrochloride and mitoxantrone hydrochloride. Three months later, the patient developed diplopia from a right oculomotor nerve palsy. Visual acuity was 20/20 in each eye and the optic discs were
normal. A lumbar puncture showed blasts in the cerebrospinal fluid. The results of mag¬ netic resonance imaging of the brain and or¬ bits, including detailed views of the oculo¬ motor nerves and the optic nerves, appeared normal with gadolinium administration. The patient declined intrathecal chemotherapy and cranial irradiation. He was treated in¬ travenously with cytarabine hydrochloride and etoposide. The blasts disappeared from the cerebrospinal fluid and the right oculo¬ motor nerve palsy slowly resolved. In July 1991, the patient complained of vi¬ sion loss in the left eye. The optic disc
Downloaded From: http://archopht.jamanetwork.com/ by a Monash University Library User on 06/20/2015
Fig 2.—Top, Coronal magnetic resonance image shows a ring of gadolinium enhance¬ ment around the left optic nerve (arrows). Note atrophy of the right medial rectus and right superior rectus muscles from the old oculomotor nerve palsy. Bottom, In axial section, the leukemic infiltrate appears
as
bright signal within the subarachnoid space and leptomeninges of the left optic nerve (ar¬ rows).
(Fig 2, top). The gadolinium enhancement silhouetted against a dark background by using a fat-suppression technique that eliminates the normal bright signal of orbital fat. In axial views, the nerve was lined with a cuff of bright signal within a distended op¬ was
tic
sheath that extended from the globe to the optic canal (Fig 2, bottom). The patient was treated immediately with radia¬ tion of the left globe and orbit. He died 2 weeks later in blast crisis. nerve
Comment.—With leukemic invasion of the optic nerve, tumor cells are found within the leptomeninges, subarachnoid space, and along the pial septa of the nerve parenchyma.4 This neoplastic infiltrate accounts for the gadolinium enhancement along the optic nerve sheath seen in the magnetic resonance images of our patient. In a few patients, early diagnosis followed by immediate radiation of the optic nerve has sal¬ vaged vision.5 If magnetic resonance imaging proves more sensitive than computed tomography for detection of perioptic métastases, it may lead to earlier recognition of this visionthreatening complication of leukemia. Jonathan C. Horton, MD, PhD Edwin G. Garcia, MD Eleanor K. Becker, MD San Francisco, Calif 1. Shaw RK, Moore EW, Freireich EJ, Thomas LB. Meningeal leukemia. Neurology. 1960;10:
823-833. 2. De Gersem R, Laey JJ. Optic nerve infiltration in leukemia. Bull Soc Beige Ophtalmol. 1968; 227:65-74. 3. Nikaido H, Mishima H, Ono H, Choshi K, Dohy H. Leukemic involvement of the optic nerve. Am J Ophthalmol. 1988;105:294-298. 4. Kincaid MC, Green WR. Ocular and orbital involvement in leukemia. Surv Ophthalmol. 1983; 27:211-232. 5. Ridgeway EW,Jaffe N, Walton DS. Leukemic ophthalmopathy in children. Cancer. 1976;38:1744\x=req-\ 1749.
Familial Posterior Lenticonus and Microcornea Most
cases
of
posterior
lenticonus
are
unilateral, isolated, and not associated with other ocular or systemic abnormalities. Familial posterior lenticonus has been documented in a total of five fam-
ilies in three previous reports.1-3 We present a mother and son with bilateral posterior lenticonus and microcornea as well as high myopia in the mother.
Report of Cases.\p=m-\Themother was aged 32 years when we first examined her. She had congenital cataracts and underwent four surgical procedures in the right eye, the first at age 3 years, but did not undergo surgery in the left eye. She developed glaucoma in the right eye. Except for her 6-year-old son with bilateral congenital cataracts, no other family members were known to be affected. She had mitral valve prolapse and pectus excavatum but no other findings suggestive of, or diagnostic of, the Marfan syndrome. Visual acuity was 2/200 OD with +5.75 sphere and 8/200 OS. Intraocular pressures were 28 mm Hg OD and 13 mm Hg OS. Cor¬ neal diameters measured 8.5 mm in each eye with a corneal pannus in both eyes. The patient had an exotropia of 40 prism diopters (D) and pendular nystagmus. The right eye was aphakic, with some residual cortex and vitreous strands to the wound. The left eye had a posterior lenticonus with surrounding posterior subcapsular opacifica-
tion. There was a right macular scar that was presumed to be secondary to chronic cystoid macular edema and high myopia and a myo¬ pic crescent at the disc. The left fundus could not be visualized. The patient underwent left cataract extraction with anterior vitrectomy. She developed postoperative glau¬ coma that was controlled medically. She also developed cystoid macular edema that re¬ solved spontaneously. Four years after sur¬ gery visual acuity was 20/200 OS with +7.75 + 1.75X35. The patient's son was examined at age 6 years. Visual acuity was 20/40 OD and 20/100 OS. Intraocular pressure was 14 mm Hg OD and 16 mm Hg OS. Corneal diameters mea¬ sured 10.5 mm horizontally and 9 mm verti¬ cally in each eye. There was a left exotropia. A posterior lenticonus was present in each eye and was more prominent on the left, with surrounding opacified posterior cortex. The retina was normal in both eyes. The patient underwent cataract extraction and anterior vitrectomy in the left eye and was fitted with a contact lens measuring 15.00 D. Patching of the right eye was initiated to treat left amblyopia. Visual acuity 6 years after surgery was 20/30 OD and 20/40 OS.
Comment.—Posterior lenticonus is estimated to be present in one to four per 100000 patients in a general oph-
Downloaded From: http://archopht.jamanetwork.com/ by a Monash University Library User on 06/20/2015
thalmological practice.1 The etiology of this abnormality is unclear; vitreous in¬ flammation, overgrowth of posterior lens fibers, uneven pull on the zonules during embryogenesis, and hyaloid ar¬ tery traction have all been proposed as possible pathogenetic mechanisms. It is possible that posterior lenticonus re¬ sults from thinning and weakness of the central part of the posterior capsule. If this thinning or its underlying cause is genetically determined, then familial instances could be explained. The association of familial posterior
lenticonus and microcornea has not been previously reported, to our knowl¬ edge. The development of aphakic glau¬ coma in the mother may be related to the microcornea and an anteriorsegment dysgenesis that leads to the development of microcornea, posterior lenticonus, cataract, and glaucoma. The familial occurrence of posterior lentico¬ nus should be considered in bilateral cases and in the presence of associated ocular malformations, such as microcornea in the present report. The pres¬ ence of pectus excavatum and mitral valve prolapse in the mother suggests that she had a mild connective tissue disorder, but both abnormalities are relatively common in the general popu¬ lation. Children of patients with bilat¬ eral posterior lenticonus and associated ocular malformations should be screened early in life for lenticonus. This allows prompt initiation of appro¬ priate therapy and a better visual
outcome.4
Jamal H. Bleik, MD Elias I. Traboulsi, MD Irene H. Maumenee, MD Baltimore, Md This study was supported in part by the Krieble and Walter Edel Funds. Jerry Crum, MD, Roanoke, Va, referred the family for treatment. 1. Butler TH. Posterior lenticonus. Arch Ophthalmol. 1930;3:425-436. 2. Howitt D, Hornblass A. Posterior lenticonus. Am J Ophthalmol. 1968;66:1133-1136. 3. Pollard ZF. Familial bilateral posterior lenticonus. Arch Ophthalmol. 1983;101:1238-1240. 4. Cheng KP, Hiles DA, Biglan AW, Pettapiece MC. Management of posterior lenticonus. J Pediatr Ophthalmol Strabismus. 1991;28:143-149.
Comment.—Unlike
cases
of
rheg-
matogenous retinal detachment, our pa¬
tient showed almost constant ERG am¬ plitude regardless of detached area, and visual field was preserved even when the entire retina was detached. These results cannot be explained simply as retinal functional impairment caused by separation of the sensory retina and the retinal pigment epithelium. We there¬ fore propose the following hypotheses.1 The damage to the photoreceptor cells was not as severe as that noted in rhegmatogenous retinal detachment, and it was uniform regardless of the area of the retinal detachment.2 Stiles-Crawford ef¬ fect or similar orientation problems con¬ tributed to the field defects in addition to retinal functional damage by chronic sep¬ aration or lack of nutrients. These hy¬ potheses allow the following explanation of our data. With a partially detached ret¬ ina, both retinal dysfunction, induced by retinal detachment, and a defocusing or Stiles-Crawford effect, induced by high elevation of the detached retina, caused the visual field defect. When the entire retina was detached, however, the eleva¬ tion was lower; the central retina, having the highest cone density, was able to pro¬ duce a measurable visual field. For the same reason, the flicker ERG showed a detectable response under all positions. A recent study has demonstrated that the ERG can be recorded from the detached macula in central serous retinopathy .4 Se¬ rous fluid could retain nutrients and avoid the possible deleterious effects of vitre¬ ous material in the subretinal space. Masayuki Horiguchi, MD Yozo Miyake, MD
Nagoya, Japan
Reprint requests to the Department of Ophthalmol¬ Nagoya University School of Medicine, 65
ogy,
appeared massively swollen by a dense white (Fig 1). The central retinal
Tsuruma-cho, Showa-ku, Nagoya 466, Japan (Dr Horiguchi).
tumor infiltrate
1. Harrington DO, Drake MV. The visual field. In: Text and Atlas of Clinical Perimetry. St Louis, Mo: CV Mosby Co; 1990:177-178. 2. Rendahl I. The electroretinogram in detachment of the retina. Arch Ophthalmol. 1957;57:566\x=req-\ 576. 3. Miyake Y, Yagasaki K, Horiguchi M. Electroretinographic monitor of retinal function during eye surgery. Arch Ophthalmol. 1991;109:1123-1126. 4. Miyake Y, Shiroyama N, Ota I, Horiguchi M. Local macular electroretinographic responses in idiopathic central serous retinopathy. Am J Ophthalmol. 1988;106:546-550.
was occluded and the retina was edematous. There was hemorrhage in the deep and superficial layers of the retina as well as within the vitreous cavity. The blood column within the retinal veins appeared abnormally dark. A subsequent lumbar puncture confirmed the presence of blasts in the cerebrospinal fluid. In Trweighted coronal magnetic reso¬ nance images with gadolinium, presumed tumor metastasis appeared as a ring of bright signal surrounding the optic nerve
artery
Magnetic Resonance Imaging of Leukemic Invasion of the Optic Nerve As treatment regimens for acute leukemia continue to improve, patients are relapsing more frequently with tumor involvement of the central nervous system.1 The central nervous system provides a sanctuary for leukemic cells because of the poor penetration of chemotherapeutic agents across the blood-brain barrier. Consequently, with prolonged patient survival, late invasion of the optic nerve by neoplastic cells is no longer a rare clinical manifestation of leukemia. Computed tomography has been a rather insensitive method for detecting abnormalities in patients with leukemic infiltration of the optic nerve. Although in some patients it has shown a thickened optic nerve shadow or enhancement of the perioptic meninges after contrast administration, in other patients it has revealed nothing.2,3 We provide the first description of the magnetic resonance findings in a patient with presumed leukemic invasion of the optic nerve.
Fig 1.—Fundus of the left eye shows en¬ gorgement of the optic disc by infiltrating tu¬ mor. There is extensive inal edema.
hemorrhage and ret¬
Report of a Case.\p=m-\A58-year-old man developed fever, lethargy, and gingival bleeding in January 1991. The white blood cell count was 165X107L (165 000 cells per mm3), and the peripheral smear showed sheets of blasts. A diagnosis was made of acute myelocytic leukemia. Complete remission was induced with cytarabine hydrochloride and mitoxantrone hydrochloride. Three months later, the patient developed diplopia from a right oculomotor nerve palsy. Visual acuity was 20/20 in each eye and the optic discs were
normal. A lumbar puncture showed blasts in the cerebrospinal fluid. The results of mag¬ netic resonance imaging of the brain and or¬ bits, including detailed views of the oculo¬ motor nerves and the optic nerves, appeared normal with gadolinium administration. The patient declined intrathecal chemotherapy and cranial irradiation. He was treated in¬ travenously with cytarabine hydrochloride and etoposide. The blasts disappeared from the cerebrospinal fluid and the right oculo¬ motor nerve palsy slowly resolved. In July 1991, the patient complained of vi¬ sion loss in the left eye. The optic disc
Downloaded From: http://archopht.jamanetwork.com/ by a Monash University Library User on 06/20/2015
Fig 2.—Top, Coronal magnetic resonance image shows a ring of gadolinium enhance¬ ment around the left optic nerve (arrows). Note atrophy of the right medial rectus and right superior rectus muscles from the old oculomotor nerve palsy. Bottom, In axial section, the leukemic infiltrate appears
as
bright signal within the subarachnoid space and leptomeninges of the left optic nerve (ar¬ rows).
(Fig 2, top). The gadolinium enhancement silhouetted against a dark background by using a fat-suppression technique that eliminates the normal bright signal of orbital fat. In axial views, the nerve was lined with a cuff of bright signal within a distended op¬ was
tic
sheath that extended from the globe to the optic canal (Fig 2, bottom). The patient was treated immediately with radia¬ tion of the left globe and orbit. He died 2 weeks later in blast crisis. nerve
Comment.—With leukemic invasion of the optic nerve, tumor cells are found within the leptomeninges, subarachnoid space, and along the pial septa of the nerve parenchyma.4 This neoplastic infiltrate accounts for the gadolinium enhancement along the optic nerve sheath seen in the magnetic resonance images of our patient. In a few patients, early diagnosis followed by immediate radiation of the optic nerve has sal¬ vaged vision.5 If magnetic resonance imaging proves more sensitive than computed tomography for detection of perioptic métastases, it may lead to earlier recognition of this visionthreatening complication of leukemia. Jonathan C. Horton, MD, PhD Edwin G. Garcia, MD Eleanor K. Becker, MD San Francisco, Calif 1. Shaw RK, Moore EW, Freireich EJ, Thomas LB. Meningeal leukemia. Neurology. 1960;10:
823-833. 2. De Gersem R, Laey JJ. Optic nerve infiltration in leukemia. Bull Soc Beige Ophtalmol. 1968; 227:65-74. 3. Nikaido H, Mishima H, Ono H, Choshi K, Dohy H. Leukemic involvement of the optic nerve. Am J Ophthalmol. 1988;105:294-298. 4. Kincaid MC, Green WR. Ocular and orbital involvement in leukemia. Surv Ophthalmol. 1983; 27:211-232. 5. Ridgeway EW,Jaffe N, Walton DS. Leukemic ophthalmopathy in children. Cancer. 1976;38:1744\x=req-\ 1749.
Familial Posterior Lenticonus and Microcornea Most
cases
of
posterior
lenticonus
are
unilateral, isolated, and not associated with other ocular or systemic abnormalities. Familial posterior lenticonus has been documented in a total of five fam-
ilies in three previous reports.1-3 We present a mother and son with bilateral posterior lenticonus and microcornea as well as high myopia in the mother.
Report of Cases.\p=m-\Themother was aged 32 years when we first examined her. She had congenital cataracts and underwent four surgical procedures in the right eye, the first at age 3 years, but did not undergo surgery in the left eye. She developed glaucoma in the right eye. Except for her 6-year-old son with bilateral congenital cataracts, no other family members were known to be affected. She had mitral valve prolapse and pectus excavatum but no other findings suggestive of, or diagnostic of, the Marfan syndrome. Visual acuity was 2/200 OD with +5.75 sphere and 8/200 OS. Intraocular pressures were 28 mm Hg OD and 13 mm Hg OS. Cor¬ neal diameters measured 8.5 mm in each eye with a corneal pannus in both eyes. The patient had an exotropia of 40 prism diopters (D) and pendular nystagmus. The right eye was aphakic, with some residual cortex and vitreous strands to the wound. The left eye had a posterior lenticonus with surrounding posterior subcapsular opacifica-
tion. There was a right macular scar that was presumed to be secondary to chronic cystoid macular edema and high myopia and a myo¬ pic crescent at the disc. The left fundus could not be visualized. The patient underwent left cataract extraction with anterior vitrectomy. She developed postoperative glau¬ coma that was controlled medically. She also developed cystoid macular edema that re¬ solved spontaneously. Four years after sur¬ gery visual acuity was 20/200 OS with +7.75 + 1.75X35. The patient's son was examined at age 6 years. Visual acuity was 20/40 OD and 20/100 OS. Intraocular pressure was 14 mm Hg OD and 16 mm Hg OS. Corneal diameters mea¬ sured 10.5 mm horizontally and 9 mm verti¬ cally in each eye. There was a left exotropia. A posterior lenticonus was present in each eye and was more prominent on the left, with surrounding opacified posterior cortex. The retina was normal in both eyes. The patient underwent cataract extraction and anterior vitrectomy in the left eye and was fitted with a contact lens measuring 15.00 D. Patching of the right eye was initiated to treat left amblyopia. Visual acuity 6 years after surgery was 20/30 OD and 20/40 OS.
Comment.—Posterior lenticonus is estimated to be present in one to four per 100000 patients in a general oph-
Downloaded From: http://archopht.jamanetwork.com/ by a Monash University Library User on 06/20/2015
thalmological practice.1 The etiology of this abnormality is unclear; vitreous in¬ flammation, overgrowth of posterior lens fibers, uneven pull on the zonules during embryogenesis, and hyaloid ar¬ tery traction have all been proposed as possible pathogenetic mechanisms. It is possible that posterior lenticonus re¬ sults from thinning and weakness of the central part of the posterior capsule. If this thinning or its underlying cause is genetically determined, then familial instances could be explained. The association of familial posterior
lenticonus and microcornea has not been previously reported, to our knowl¬ edge. The development of aphakic glau¬ coma in the mother may be related to the microcornea and an anteriorsegment dysgenesis that leads to the development of microcornea, posterior lenticonus, cataract, and glaucoma. The familial occurrence of posterior lentico¬ nus should be considered in bilateral cases and in the presence of associated ocular malformations, such as microcornea in the present report. The pres¬ ence of pectus excavatum and mitral valve prolapse in the mother suggests that she had a mild connective tissue disorder, but both abnormalities are relatively common in the general popu¬ lation. Children of patients with bilat¬ eral posterior lenticonus and associated ocular malformations should be screened early in life for lenticonus. This allows prompt initiation of appro¬ priate therapy and a better visual
outcome.4
Jamal H. Bleik, MD Elias I. Traboulsi, MD Irene H. Maumenee, MD Baltimore, Md This study was supported in part by the Krieble and Walter Edel Funds. Jerry Crum, MD, Roanoke, Va, referred the family for treatment. 1. Butler TH. Posterior lenticonus. Arch Ophthalmol. 1930;3:425-436. 2. Howitt D, Hornblass A. Posterior lenticonus. Am J Ophthalmol. 1968;66:1133-1136. 3. Pollard ZF. Familial bilateral posterior lenticonus. Arch Ophthalmol. 1983;101:1238-1240. 4. Cheng KP, Hiles DA, Biglan AW, Pettapiece MC. Management of posterior lenticonus. J Pediatr Ophthalmol Strabismus. 1991;28:143-149.
