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Leukocoria Caused by Occult Penetrating Trauma in a Child
FIGURE. (Top) Right eye. (Bottom) Left eye. Bilateral macular ischemia (arrows) associated with peripheral vasculitis (*) in the left eye (bottom).
showed bilateral ischemic maculae associated with peripheral retinal vasculitis and peripheral ischemia (Figure). Visual field testing showed a bilateral central scotoma. We diagnosed Behcet disease because three of the major criteria of the disease1 were present in our patient: erythema nodosum-like eruptions, eye le sions, and recurrent oral aphthous ulcerations. All signs were associated with biologic inflammatory signs, and HLA typing showed that HLA B51 antigen was present. Corticosteroids were initiated (intravenous pulse of 1 g of methylprednisolone per day for 3 days and then 1 mg/kg per day taken orally for 3 months). Visual acuity recovered within 1 week with corticosteroids, VOL.124,
No. 1
and macular ischemia resolved on angiography. Six months later, best-corrected visual acuity was 20/50 and remained stable while the patient continued taking colchicine (1 mg per day) for up to 2 years. Visual loss is the most serious observed ocular complication of Behcet disease. Vision in Behcet disease is usually lost within an average of 3.3 years after onset of eye symptoms, and the frequency of ocular manifestations in patients with Behcet disease ranges from 70% to 85%.2 Although the most com mon ocular feature is uveitis, necrotizing retinal vascular lesions often lead to vision loss. Retinal features have been reported in up to 50% of cases.3 In our patient, bilateral occlusive retinal vasculitis leads to peripheral vasculitis and macular ischemia. Bilateral ocular involvement is very common; howev er, macular ischemia remains rare.4 Permanent loss of visual acuity in Behcet disease may be related to retinal vasculitis, cystoid macular edema, and optic atrophy. Colchicine is ineffective in treating retinal occlusive vasculitis, but corticosteroids have a benefi cial effect on acute manifestations of inflammation. Corticosteroids, however, seem to be ineffective in treating late sequelae when blindness has occurred.5 In Behcet disease, vascular damage may lead to severe visual impairment caused by bilateral macular ischemia. Early treatment with corticosteroids may be beneficial when vascular involvement may potentially lead to blindness. REFERENCES 1. International Behcet's study group. Criteria for diagnosis of Behcet's disease. Lancet 1990;335:1078-1080. 2. Michelson JB, Chisari FV. Behcet's disease. Surv Ophthalmol 1982;26:190-203. 3. Atmaca LS. Fundus changes associated with Behcet's disease. Graefes Arch Clin Exp Ophthalmol 1989;227:340-344. 4. Palimeris G, Theodossiades B, Constantinidou B, Chimonidou E. La macula dans le syndrome d'Adamantiades-Behcet. J Fr Ophtalmol 1989;12:887-890. 5. Nussenblatt RB, Whitcup SM, Palestine AG. Uveitis: funda mentals and clinical practice. 2nd ed. St Louis: CV Mosby, 1996:334-353.
Leukocoria Caused by Occult Penetrating Trauma in a Child Adolfo Guemes, MD, Kenneth W. Wright, MD, Michael Humayun, MD, Robert E. Foster, MD, and Hilel Lewis, MD
BRIEF REPORTS
117
PURPOSE: To report a child with leukocoria caused by occult penetrating trauma. METHODS: Case report. The clinical findings and surgical repair of acquired leukocoria of the right eye in a 2-year-old boy are presented. RESULTS: In the right eye, slit-lamp examination disclosed a retrolenticular cyclitic membrane and moderately severe (3 + ) cells and flare anterior chamber reaction. The eye was hypotonous, and B scan showed that the membrane was associated with a retinal detachment. Surgery was performed to repair the retinal detachment and to remove the retrolenticular membrane. Two months before ini tial examination, the patient had been attacked by a rooster. CONCLUSION: Occult penetrating trauma should be considered in the differential diagnosis of pediatric leukocoria.
C
OMMON CAUSES OF PEDIATRIC LEUKOCORIA INclude congenital cataract, retinoblastoma, persis tent hyperplastic primary vitreous, retinopathy of prematurity, toxocariasis, Coats disease, and other less common conditions.1 We examined a child with right eye leukocoria associated with fibrous ingrowth resulting from occult penetrating trauma caused by a rooster attack. A 2-year-old boy was referred for evaluation of a white pupillary reflex in his right eye first observed by his parents 3 days earlier. His ocular and medical history were unremarkable except for a rooster attack 2 months previously that resulted in several small facial abrasions. His parents did not notice any direct ocular injury, and the child was asymptomatic. Initial ophthalmic examination disclosed a visual acuity of light perception in the right eye, and he was able to fix and follow with his left eye. Pupillary testing demonstrated a right relative afferent pupillary defect. External examination showed a healed right upper eyelid scar temporally. Slit-lamp examination of the right eye disclosed mild conjunctival hyperemia without visible conjunctival laceration, chemosis, or
Accepted for publication Feb 28, 1997. Division of Ophthalmology, The Cleveland Clinic Foundation. Inquiries to Kenneth W. Wright, MD, American Eye Institute, 8635 W Third St, Los Angeles, CA 90048; fax: (310) 652-4353.
118
FIGURE. Slit-lamp photograph of the right eye showing ciliary body traction associated with the white fibrous retrolenticular membrane. The membrane emanated from a single focus under the scarred lateral rectus muscle.
hemorrhage. The anterior chamber was shallow and contained moderately severe (3 + ) cells and flare. Intraocular pressure measured RE, 2 mm Hg and LE, 12 mm Hg. Posterior segment examination of the right eye was difficult because of a dense white retrolenticular membrane (Figure), although the na sal retina was attached. B-scan ultrasonographic examination showed an immobile highly reflective membrane extending circumferentially from the midperiphery to behind the lens. No intraocular tumor or calcification was detected. Laboratory evaluation in cluding complete blood cell count, angiotensin-converting enzyme, enzyme-linked immunosorbent assay for toxocariasis, antinuclear antibody, and chest x-ray were normal. Five days later, the patient was taken to the operating room because of worsening hypotony and the new finding of a retinal detachment. Lateral rectus muscle scarring was detected, although no scleral wound was found. Lensectomy was performed, followed by dissection of a dense white retrolenticular membrane that emanated from a focal point under the lateral rectus muscle. The ciliary body was
AMERICAN JOURNAL OF OPHTHALMOLOGY
JULY 1997
disinserted at the 3-o'clock position temporally asso ciated with traction from the retrolenticular cyclitic membrane, and a macula-involving retinal detach ment associated with a giant retinal tear was found. After scleral buckling, pars plana vitrectomy, retinopexy, and silicone oil tamponade, the retina was reattached. Serial sections of the cyclitic membrane showed mild inflammatory cells and fibrovascular tissue. No Toxocara or intraocular foreign body was found. Postoperatively, visual acuity in the right eye mea sured light perception. Intraocular pressure normal ized to 14 mm Hg, and the retina has remained completely reattached with 4 months of follow-up, except for a shallow inferior tractional detachment outside of the macula associated with an epiretinal membrane. Children may not complain of reduced vision, particularly if the fellow eye has normal vision. Occult penetrating trauma is usually not considered in the differential diagnosis of pediatric leukocoria, although penetrating trauma is not uncommon in children.2,3 In this case, fibrous ingrowth resulting from penetrat ing trauma was not considered until lateral rectus muscle scarring and fibrous ingrowth were detected intraoperatively. Most likely, penetrating trauma from the sharp beak of the rooster resulted in fibrous ingrowth and subsequent ciliary body traction caus ing hypotony and retinal traction causing the giant tear. Occult penetrating trauma should be considered in the differential diagnosis of pediatric leukocoria. REFERENCES 1. Howard GM, Ellsworth RM. Differential diagnosis of retinoblastoma: a statistical survey of 500 children. Relative frequen cy of the lesions which simulate retinoblastoma. A m J Ophthalmol 1965;60:610-618. 2. Alfaro Virgil D, Chaudry NA, Walonker AF, Runyan T, Saito Y, Ligget PE. Penetrating eye injuries in young children. Retina 1994;14:201-205. 3. Sternberg P, De Juan E, Michels RG. Penetrating ocular injuries in young patients: initial injuries and visual results. Retina 1984;4:5-8.
Choroidal Osteoma in an Infant Yuki Kida, MD, Yuzo Shibuya, MD, Masami Oguni, MD, Atsuko Fujitani, MD, Tatsuo Kodama, MD, Kozo Yamada, MD, and Tomoichi Setogawa, MD VOL.124, No. 1
PURPOSE: To report an 8-month-old infant with choroidal osteoma. METHOD: Case report. RESULTS: The patient, who had bilateral yellow stippling at the posterior pole, was followed up for 8 years. Both fundi developed creamy, irregular scalloped lesions. Computed tomography showed a bony plate at the posterior pole bilaterally. CONCLUSION: We believe that this is the young est patient reported to have choroidal osteoma.
C
HOROIDAL OSTEOMA IS A RARE, BENIGN CHOROI-
dal tumor that occurs typically in young girls.1 We examined an 8-month-old infant with the initial manifestations of bilateral choroidal osteoma and followed up the patient for 8 years. A healthy 8-month-old infant had conjunctival hyperemia bilaterally. She was the product of a normal pregnancy, labor, and delivery, and her medi cal history was unremarkable. There was no history of trauma. At the initial examination, external and biomicroscopic findings of both eyes were within normal limits, except for the conjunctiva. Fundus examination of both eyes disclosed irregular juxtapapillary lesions. The lesions appeared as yellow mottling (Figure 1). We diagnosed conjunctivitis. Conjunctival injection disappeared after 1 week. The patient was followed up for 8 years. During this period, no orbital pseudotumor, uveitis, or posterior scleritis developed. The fundi became yellow-white and irregularly scalloped (Figure 2). At age 8 years, her visual acuity was 20/15 in both eyes. B-scan ultrasonography demonstrated high reflectivity at the posterior pole. This intense reflectivity was main tained with reduction of sensitivity. Computed to mography showed a bony plate at the posterior pole of both eyes. Laboratory test results showed normal serum calcium, phosphorus, alkaline phosphatase, and calcitonin levels. Our patient exhibited the characteristic findings of choroidal osteoma. Choroidal osteoma is an ossified tumor first described in 1978 by Gass and associates.1 Choroidal osteomas were reported previously in about Accepted for publication Feb 24, 1997. Department of Ophthalmology, Shimane Medical University, Izumo. Inquiries to Yuki Kida, MD, Department of Ophthalmology, Shimane Medical University, Izumo, Shimane 693, Japan; fax: 81-853-25-0464; e-mail: [email protected]
BRIEF REPORTS
119
showed bilateral ischemic maculae associated with peripheral retinal vasculitis and peripheral ischemia (Figure). Visual field testing showed a bilateral central scotoma. We diagnosed Behcet disease because three of the major criteria of the disease1 were present in our patient: erythema nodosum-like eruptions, eye le sions, and recurrent oral aphthous ulcerations. All signs were associated with biologic inflammatory signs, and HLA typing showed that HLA B51 antigen was present. Corticosteroids were initiated (intravenous pulse of 1 g of methylprednisolone per day for 3 days and then 1 mg/kg per day taken orally for 3 months). Visual acuity recovered within 1 week with corticosteroids, VOL.124,
No. 1
and macular ischemia resolved on angiography. Six months later, best-corrected visual acuity was 20/50 and remained stable while the patient continued taking colchicine (1 mg per day) for up to 2 years. Visual loss is the most serious observed ocular complication of Behcet disease. Vision in Behcet disease is usually lost within an average of 3.3 years after onset of eye symptoms, and the frequency of ocular manifestations in patients with Behcet disease ranges from 70% to 85%.2 Although the most com mon ocular feature is uveitis, necrotizing retinal vascular lesions often lead to vision loss. Retinal features have been reported in up to 50% of cases.3 In our patient, bilateral occlusive retinal vasculitis leads to peripheral vasculitis and macular ischemia. Bilateral ocular involvement is very common; howev er, macular ischemia remains rare.4 Permanent loss of visual acuity in Behcet disease may be related to retinal vasculitis, cystoid macular edema, and optic atrophy. Colchicine is ineffective in treating retinal occlusive vasculitis, but corticosteroids have a benefi cial effect on acute manifestations of inflammation. Corticosteroids, however, seem to be ineffective in treating late sequelae when blindness has occurred.5 In Behcet disease, vascular damage may lead to severe visual impairment caused by bilateral macular ischemia. Early treatment with corticosteroids may be beneficial when vascular involvement may potentially lead to blindness. REFERENCES 1. International Behcet's study group. Criteria for diagnosis of Behcet's disease. Lancet 1990;335:1078-1080. 2. Michelson JB, Chisari FV. Behcet's disease. Surv Ophthalmol 1982;26:190-203. 3. Atmaca LS. Fundus changes associated with Behcet's disease. Graefes Arch Clin Exp Ophthalmol 1989;227:340-344. 4. Palimeris G, Theodossiades B, Constantinidou B, Chimonidou E. La macula dans le syndrome d'Adamantiades-Behcet. J Fr Ophtalmol 1989;12:887-890. 5. Nussenblatt RB, Whitcup SM, Palestine AG. Uveitis: funda mentals and clinical practice. 2nd ed. St Louis: CV Mosby, 1996:334-353.
Leukocoria Caused by Occult Penetrating Trauma in a Child Adolfo Guemes, MD, Kenneth W. Wright, MD, Michael Humayun, MD, Robert E. Foster, MD, and Hilel Lewis, MD
BRIEF REPORTS
117
PURPOSE: To report a child with leukocoria caused by occult penetrating trauma. METHODS: Case report. The clinical findings and surgical repair of acquired leukocoria of the right eye in a 2-year-old boy are presented. RESULTS: In the right eye, slit-lamp examination disclosed a retrolenticular cyclitic membrane and moderately severe (3 + ) cells and flare anterior chamber reaction. The eye was hypotonous, and B scan showed that the membrane was associated with a retinal detachment. Surgery was performed to repair the retinal detachment and to remove the retrolenticular membrane. Two months before ini tial examination, the patient had been attacked by a rooster. CONCLUSION: Occult penetrating trauma should be considered in the differential diagnosis of pediatric leukocoria.
C
OMMON CAUSES OF PEDIATRIC LEUKOCORIA INclude congenital cataract, retinoblastoma, persis tent hyperplastic primary vitreous, retinopathy of prematurity, toxocariasis, Coats disease, and other less common conditions.1 We examined a child with right eye leukocoria associated with fibrous ingrowth resulting from occult penetrating trauma caused by a rooster attack. A 2-year-old boy was referred for evaluation of a white pupillary reflex in his right eye first observed by his parents 3 days earlier. His ocular and medical history were unremarkable except for a rooster attack 2 months previously that resulted in several small facial abrasions. His parents did not notice any direct ocular injury, and the child was asymptomatic. Initial ophthalmic examination disclosed a visual acuity of light perception in the right eye, and he was able to fix and follow with his left eye. Pupillary testing demonstrated a right relative afferent pupillary defect. External examination showed a healed right upper eyelid scar temporally. Slit-lamp examination of the right eye disclosed mild conjunctival hyperemia without visible conjunctival laceration, chemosis, or
Accepted for publication Feb 28, 1997. Division of Ophthalmology, The Cleveland Clinic Foundation. Inquiries to Kenneth W. Wright, MD, American Eye Institute, 8635 W Third St, Los Angeles, CA 90048; fax: (310) 652-4353.
118
FIGURE. Slit-lamp photograph of the right eye showing ciliary body traction associated with the white fibrous retrolenticular membrane. The membrane emanated from a single focus under the scarred lateral rectus muscle.
hemorrhage. The anterior chamber was shallow and contained moderately severe (3 + ) cells and flare. Intraocular pressure measured RE, 2 mm Hg and LE, 12 mm Hg. Posterior segment examination of the right eye was difficult because of a dense white retrolenticular membrane (Figure), although the na sal retina was attached. B-scan ultrasonographic examination showed an immobile highly reflective membrane extending circumferentially from the midperiphery to behind the lens. No intraocular tumor or calcification was detected. Laboratory evaluation in cluding complete blood cell count, angiotensin-converting enzyme, enzyme-linked immunosorbent assay for toxocariasis, antinuclear antibody, and chest x-ray were normal. Five days later, the patient was taken to the operating room because of worsening hypotony and the new finding of a retinal detachment. Lateral rectus muscle scarring was detected, although no scleral wound was found. Lensectomy was performed, followed by dissection of a dense white retrolenticular membrane that emanated from a focal point under the lateral rectus muscle. The ciliary body was
AMERICAN JOURNAL OF OPHTHALMOLOGY
JULY 1997
disinserted at the 3-o'clock position temporally asso ciated with traction from the retrolenticular cyclitic membrane, and a macula-involving retinal detach ment associated with a giant retinal tear was found. After scleral buckling, pars plana vitrectomy, retinopexy, and silicone oil tamponade, the retina was reattached. Serial sections of the cyclitic membrane showed mild inflammatory cells and fibrovascular tissue. No Toxocara or intraocular foreign body was found. Postoperatively, visual acuity in the right eye mea sured light perception. Intraocular pressure normal ized to 14 mm Hg, and the retina has remained completely reattached with 4 months of follow-up, except for a shallow inferior tractional detachment outside of the macula associated with an epiretinal membrane. Children may not complain of reduced vision, particularly if the fellow eye has normal vision. Occult penetrating trauma is usually not considered in the differential diagnosis of pediatric leukocoria, although penetrating trauma is not uncommon in children.2,3 In this case, fibrous ingrowth resulting from penetrat ing trauma was not considered until lateral rectus muscle scarring and fibrous ingrowth were detected intraoperatively. Most likely, penetrating trauma from the sharp beak of the rooster resulted in fibrous ingrowth and subsequent ciliary body traction caus ing hypotony and retinal traction causing the giant tear. Occult penetrating trauma should be considered in the differential diagnosis of pediatric leukocoria. REFERENCES 1. Howard GM, Ellsworth RM. Differential diagnosis of retinoblastoma: a statistical survey of 500 children. Relative frequen cy of the lesions which simulate retinoblastoma. A m J Ophthalmol 1965;60:610-618. 2. Alfaro Virgil D, Chaudry NA, Walonker AF, Runyan T, Saito Y, Ligget PE. Penetrating eye injuries in young children. Retina 1994;14:201-205. 3. Sternberg P, De Juan E, Michels RG. Penetrating ocular injuries in young patients: initial injuries and visual results. Retina 1984;4:5-8.
Choroidal Osteoma in an Infant Yuki Kida, MD, Yuzo Shibuya, MD, Masami Oguni, MD, Atsuko Fujitani, MD, Tatsuo Kodama, MD, Kozo Yamada, MD, and Tomoichi Setogawa, MD VOL.124, No. 1
PURPOSE: To report an 8-month-old infant with choroidal osteoma. METHOD: Case report. RESULTS: The patient, who had bilateral yellow stippling at the posterior pole, was followed up for 8 years. Both fundi developed creamy, irregular scalloped lesions. Computed tomography showed a bony plate at the posterior pole bilaterally. CONCLUSION: We believe that this is the young est patient reported to have choroidal osteoma.
C
HOROIDAL OSTEOMA IS A RARE, BENIGN CHOROI-
dal tumor that occurs typically in young girls.1 We examined an 8-month-old infant with the initial manifestations of bilateral choroidal osteoma and followed up the patient for 8 years. A healthy 8-month-old infant had conjunctival hyperemia bilaterally. She was the product of a normal pregnancy, labor, and delivery, and her medi cal history was unremarkable. There was no history of trauma. At the initial examination, external and biomicroscopic findings of both eyes were within normal limits, except for the conjunctiva. Fundus examination of both eyes disclosed irregular juxtapapillary lesions. The lesions appeared as yellow mottling (Figure 1). We diagnosed conjunctivitis. Conjunctival injection disappeared after 1 week. The patient was followed up for 8 years. During this period, no orbital pseudotumor, uveitis, or posterior scleritis developed. The fundi became yellow-white and irregularly scalloped (Figure 2). At age 8 years, her visual acuity was 20/15 in both eyes. B-scan ultrasonography demonstrated high reflectivity at the posterior pole. This intense reflectivity was main tained with reduction of sensitivity. Computed to mography showed a bony plate at the posterior pole of both eyes. Laboratory test results showed normal serum calcium, phosphorus, alkaline phosphatase, and calcitonin levels. Our patient exhibited the characteristic findings of choroidal osteoma. Choroidal osteoma is an ossified tumor first described in 1978 by Gass and associates.1 Choroidal osteomas were reported previously in about Accepted for publication Feb 24, 1997. Department of Ophthalmology, Shimane Medical University, Izumo. Inquiries to Yuki Kida, MD, Department of Ophthalmology, Shimane Medical University, Izumo, Shimane 693, Japan; fax: 81-853-25-0464; e-mail: [email protected]
BRIEF REPORTS
119