Macular hole in the shaken baby syndrome.
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Fluorocarbons↗
Biomedical subjects
Darius M Moshfeghi
Publications and source records attributed to Darius M Moshfeghi.
Presumed transient reactive astrocytic hyperplasia in immature retina.
PURPOSE: To describe the appearance and clinical course of small, white, endophytic calcific, peripheral lesions in developing retina of premature infants. METHODS: Retrospective review of all patients evaluated for retinopathy of prematurity (ROP) at a level I neonatal intensive care unit (NICU) in San Jose, CA, between January 1, 2003, and December 31, 2004. Patients were examined either in the NICU or the affiliated outpatient clinic. Clinical examination consisted of dilated fundus examination with 360 degree scleral depression. Lesions were identified if they were white, calcific, peripheral, and transient. Ancillary testing and examination of family members was performed as indicated. RESULTS: A total of 302 unique patients were screened for ROP. Ten lesions were identified in seven eyes. Three eyes had two lesions each. All lesions were unilateral. The size of the lesions was estimated to range between 500 and 700 microm. All lesions were located anterior to the vascularized retina, had minimal elevation on scleral depression, and demonstrated a predilection for the nasal and temporal raphe. Ultrasound findings demonstrated an elevated, hyperechoic mass with orbital shadowing. Computed tomography and magnetic resonance imaging tests did not demonstrate the lesions. Lesions involute slowly over a period of 6 months. There were no systemic findings or familial dispositions. CONCLUSION: Immature retina in premature infants may predispose to the formation of transient reactive astrocytic hyperplasia. Development of mature retinal vascularization and spontaneous resolution of lesions should alleviate concerns regarding a more malignant diagnosis.
Astrocytes↗
Intraocular surgery after treatment of germline retinoblastoma.
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Adult↗
Anaphylactoid reaction after verteporfin therapy.
PURPOSE: To report a case of anaphylactoid reaction in a woman after initial exposure to verteporfin and to alert physicians of this potentially life-threatening reaction. DESIGN: Interventional case report. METHODS: An 80-year-old woman who was found to have exudative age-related macular degeneration on clinical examination and fluorescein angiography underwent treatment with verteporfin photodynamic therapy (PDT). Thirty minutes after verteporfin PDT infusion, the patient experienced throat constriction, swelling of her hands, and severe shortness of breath. She received immediate intravenous treatment with methylprednisolone, diphenhydramine, and famotidine and was admitted for observation. RESULTS: The patient's symptoms resolved, and she experienced no long-term side effects related to PDT. Routine noninvasive pulse oximetry, however, did result in finger discoloration and superficial burn. Evaluation revealed that her symptoms were noncardiac in origin. CONCLUSION: Verteporfin can result in an anaphylactoid reaction. Treating physicians should be advised of possible photosensitizing reactions once the patient has received verteporfin PDT.
Aged, 80 and over↗
Transpupillary thermotherapy: effect of wavelength on normal primate retina.
PURPOSE: To correlate changes in primate fundus after transpupillary thermotherapy (TTT) at two wavelengths. METHODS: Twelve primate eyes were treated with TTT using a wavelength of 635 nm (n=7) or 810 nm (n=5). Laser parameters were as follows: 635 nm (spot size, 1 mm; duration, 30-8 seconds; and fluence [power over time], 20-91.4 J/cm) and 810 nm (spot size, 2 mm; duration, 60 seconds; and fluence, 96-436 J/cm). Fundus photography, fluorescein and indocyanine green angiography, and enucleation were performed at time 0 or 2 weeks after TTT for histologic analysis. RESULTS: Threshold for fundus lesions (91.4 J/cm at 635 nm and 191 J/cm at 810 nm), acute and chronic retinal damage shown by histologic analysis (79.2 J/cm at 635 nm and 96 J/cm at 810 nm), and choroidal vessel occlusion (50 J/cm at 635 nm and 96 J/cm at 810 nm) were lower at 635 nm. Disorganization of the retina and retinal pigment epithelium was seen for both wavelengths at time 0 and 2 weeks after TTT. Occlusion of the choriocapillaris and choroidal stromal vessels was noted only in specimens obtained 2 weeks after TTT. CONCLUSIONS: TTT resulted in acute and delayed damage to the neurosensory retina that persisted at 2 weeks. The 635-nm wavelength demonstrated a lower threshold fluence for visible fundus lesions, retinal damage, and choroidal vascular occlusion than the 810-nm laser.
Animals↗
Appositional suprachoroidal hemorrhage: a case-control study.
PURPOSE: To identify the risk factors, prognostic factors, and clinical outcomes of patients with perioperative appositional suprachoroidal hemorrhage (ASCH). DESIGN: Case-control study. SETTING: Tertiary referral center. METHODS: Subjects included all patients with perioperative ASCH documented by B-scan ultrasound between May 1990 and March 2001. Two or three control patients were selected for each case, matched by surgeon, procedure, and date of surgery within 1 month. Surgery was performed as necessary. main outcome measures. The odds of ASCH associated with clinical risk factors. secondary outcome measure: visual acuity. RESULTS: Thirty-seven cases with ASCH were identified. Ninety-two procedure- and surgeon-matched control subjects (2.48:1) were selected. Twenty-six cases (71%) of ASCH were related to a glaucoma operation. Risk factors for the development of ASCH included previous vitrectomy (P = .003, odds ratio of 12) and older age (P = .007, odds ratio 1.57/decade of increasing age). Hypertension was found to be protective (P = .02, odds ratio of 0.33). Factors associated with a poor visual outcome in patients with ASCH included apposition >30 days (P = .01), history of uveitis (P = .04), history of dry age-related macular degeneration (P = .05), and history of extracapsular cataract extraction (P = .05). Median pre-ASCH visual acuity was 20/100, and final median visual acuity was 20/1600. CONCLUSIONS: Risk factors for the development of ASCH include previous vitrectomy and older age. Patients with these risk factors should be informed of their greater chance of poor visual acuity and anatomic outcomes secondary to the development of ASCH.
Adolescent↗
Diagnostic approaches to severe, atypical toxoplasmosis mimicking acute retinal necrosis.
PURPOSE: To describe the means of diagnosis and clinical features of atypical toxoplasmic chorioretinitis mimicking acute retinal necrosis. DESIGN: Observational case series. PARTICIPANTS: Twenty-two patients (25 eyes) with widespread chorioretinitis resulting from toxoplasmosis examined between 1990 and 2001. TESTING: Patients were diagnosed by various techniques, including polymerase chain reaction (PCR) of aqueous and vitreous, serum and intraocular antibody determination, culture of intraocular fluid, retinal biopsy, histopathologic examination, therapeutic trial of antibiotics active against toxoplasmosis, or a combination thereof. MAIN OUTCOME MEASURES: The primary outcome measure was diagnosis of disseminated toxoplasmic chorioretinitis by any combination of tests or by empiric use of specific antibiotics. The secondary outcome measure was visual and anatomic outcome of treatment. RESULTS: Mean age was 53.5 years (range, 19-77 years), with a median of 59.5 years. There were 9 women and 13 men. Six patients were infected with HIV, and 3 patients, 1 with HIV, had bilateral disease. Mean initial vision was 20/110 (median, 20/400; range, 20/20 to no light perception [NLP]). Sixteen patients (73%) had received oral or injectable corticosteroids and 11 (50%) had received antiviral therapy before the diagnosis of toxoplasmosis. Diagnosis was made solely by clinical response to antitoxoplasmosis medications in 4 patients. Sixteen patients were diagnosed based on evaluation of intraocular fluids and tissue by antibody determinations, culture, PCR, histopathologic examination, or a combination thereof. Visual acuity improved after treatment in 7 of 25 eyes (28%). Mean final visual acuity was 20/156 (median, 20/2500; range, 20/30 to NLP). Anatomically, 18 of 23 eyes with follow-up had healed or improved chorioretinitis. Retinitis was progressive in 1 eye, 2 eyes were enucleated, and 2 were phthisical. CONCLUSIONS: Diagnosis of atypical toxoplasmic chorioretinitis that mimics viral retinitis can be accomplished by several means. Prompt diagnosis may help avoid poor visual and anatomic outcomes after prolonged initial treatment with oral prednisone or antiviral medications.
Adult↗
Submacular surgery for choroidal neovascularization following nocardial endophthalmitis.
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Adult↗
Intravitreal triamcinolone acetonide.
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Glucocorticoids↗
A novel mutation in the RDS/Peripherin gene causes adult-onset foveomacular dystrophy.
PURPOSE: To describe a novel mutation in the RDS/Peripherin gene that results in a moderately severe form of adult-onset foveomacular dystrophy. DESIGN: Observational case series. METHODS: Selected members of a family with adult-onset foveomacular dystrophy underwent complete ophthalmic evaluation, including fundus photography and fluorescein angiography, in a tertiary care referral center. The study population consisted of 12 members of a Caucasian kindred. After providing informed consent, patients donated blood for genomic DNA extraction and mutational screening using standard techniques. The main outcome measure were the presence of a RDS/Peripherin gene mutation in a patient with the disease and its absence in unaffected family members and controls. RESULTS: Eight affected family members and no unaffected family members demonstrated a single guanine base deletion at nucleotide 112 that led to premature termination at amino acid 38 of RDS/Peripherin polypeptide. This frameshift mutation results in truncation of nearly 90% of the gene product, thus probably representing a null allele. That results in a relatively severe phenotype, with choroidal neovascularization developing in two patients and geographic atrophy involving the macula in three patients. CONCLUSIONS: We describe a frameshift null mutation in the RDS/Peripherin gene associated with a relatively severe manifestation of adult-onset foveomacular dystrophy in affected family members.
Adult↗
Clinicopathologic study after submacular removal of choroidal neovascular membranes treated with verteporfin ocular photodynamic therapy.
PURPOSE: To report the clinicopathologic findings after submacular removal of choroidal neovascular membranes (CNV) treated with verteporfin ocular photodynamic therapy. DESIGN: Interventional case series. METHODS: Retrospective review of eight eyes of eight patients who underwent submacular surgery for CNV after having previously received verteporfin ocular photodynamic therapy for presumed ocular histoplasmosis (one patient), age-related macular degeneration ([AMD] three patients) pathologic myopia (two patients), punctate inner choroiditis (one patient), and idiopathic CNV (one patient). All cases had undergone ocular photodynamic therapy with verteporfin using standard protocols. Six of eight patients suffered a submacular hemorrhage after ocular photodynamic therapy, and two of eight patients refused further ocular photodynamic therapy. All patients subsequently had submacular surgery with removal of the CNV. One membrane was routinely processed, sectioned, and stained with hematoxylin and eosin. Five membranes were stained with toluidine blue for light microscopic examination. Semithin (1.0 microm) sections were cut and stained with uranyl acetate-lead citrate for transmission electron microscopy. RESULTS: Choroidal neovascular membranes were removed at 3 days (presumed ocular histoplasmosis), 29 days (punctate inner choroiditis), 63 days (AMD, pathologic myopia), 66 days (AMD), 107 days (pathologic myopia), 116 days (AMD), and 152 days (idiopathic) after verteporfin ocular photodynamic therapy. Histopathologic and ultrastructural examination showed areas of vascular occlusion at 3 days that were not seen at later time points. All specimens had patent CNV. There were signs of vascular damage with extravasated erythrocytes and fibrin, pigment clumping in cells, and inflammatory cells in all but the 3-day specimen. CONCLUSIONS: This case series presents data only from patients who refused repeat treatment with ocular photodynamic therapy or who developed submacular hemorrhage after initial photodynamic therapy. Histopathologic evaluation of CNV 3 days after verteporfin ocular photodynamic therapy showed partial vascular occlusion that was not present in later specimens. These later specimens demonstrated evidence of vascular damage. Verteporfin ocular photodynamic therapy does not appear to lead to permanent and complete occlusion of the CNV. Thus, treatments that lead to permanent closure of CNV without damage to the retinal pigment epithelium and sensory retina are still needed.
Adult↗
Acute endophthalmitis following intravitreal triamcinolone acetonide injection.
PURPOSE: To report the clinical features, causative organisms, management, and visual acuity outcomes of eight eyes of eight patients who developed acute postoperative endophthalmitis following intravitreal injection of triamcinolone acetonide (IVTA). DESIGN: Retrospective, multicenter, interventional, case series. METHODS: A retrospective, interventional, case series of all patients with acute postoperative endophthalmitis following IVTA at seven academic clinical centers between March 2001 and July 2002. RESULTS: A total of 922 IVTAs were performed. Eight eyes of eight patients with acute postoperative endophthalmitis were identified in the 6 weeks following IVTA for an incidence of 0.87% (95% confidence interval of 0.38% to 1.70%). The median time to presentation was 7.5 days (range, 1-15 days) after IVTA. The most common clinical findings were iritis (n = 8), vitritis (n = 8), hypopyon (n = 8), pain (n = 7), red eye (n = 6), and decreased vision (n = 5). The median presenting visual acuity was 20/1127 (range, 20/60 to light perception). Initial treatment consisted of vitreous tap and injection of antibiotics (n = 6) or pars plana vitrectomy and injection of intravitreal antibiotics (n = 2). Intraocular cultures yielded identification in seven patients. One demonstrated intracellular gram-positive cocci in chains with numerous polymorphonuclear cells on gram stain. The median postinfection vision was 20/400 (range, 20/40 to no light perception). Three patients ended up with no light perception visual acuity, including enucleation (n = 1) and phthisis (n = 1). CONCLUSIONS: Acute postoperative endophthalmitis following IVTA occurs rapidly and can result in severe loss of vision.
Acute Disease↗
Occult orbital neuroblastoma detected after administration of an antitumor vaccine.
A 6-year-old girl with neuroblastoma developed swelling and erythema of her right upper eyelid following administration of an interleukin-2 and lymphotactin gene-modified allogeneic neuroblastoma cell vaccine. Computed tomography demonstrated a cystic lesion in the subperiosteal space. A biopsy of the mass showed necrotic neuroblastoma with minimal associated inflammation. To our knowledge, this case represents the first description of occult orbital metastases in a patient with neuroblastoma detected after administration of an antitumor vaccine.
Cancer Vaccines↗
Transpupillary thermotherapy threshold parameters: funduscopic, angiographic, and histologic findings in pigmented and nonpigmented rabbits.
PURPOSE: To evaluate the effect of pigmentation on threshold fluence levels, needed to produce visible and angiographic lesions, of transpupillary thermotherapy (TTT) in rabbits. METHODS: Six pigmented and nine nonpigmented rabbits underwent TTT with an 810-nm diode laser coupled to a slit-lamp biomicroscope using a spot size of 2 or 3 mm. The power ranged from 80 to 200 mW with 1 to 3 minutes of laser exposure for pigmented rabbits and 750 to 1800 mW with 1 minute of exposure for albino rabbits. These parameters were also evaluated after compression of the globe using the contact lens to induce blanching of the optic nerve head. After the experiment, the eyes were enucleated under deep anesthesia, and the animals were killed immediately. RESULTS: In pigmented rabbits, the threshold fluence with the 2-mm spot size was 229 J/cm2 without compression and 153 J/cm2 with compression. With the 3-mm spot size, the threshold decreased from 200 to 150 mW as the duration of exposure lengthened (2 or 3 minutes), increasing the fluence from 170 to 382 J/cm2. In nonpigmented rabbits, the threshold fluence with the 2-mm spot size was 2,865 J/cm2 without compression and 2,674 J/cm2 with compression. With the 3-mm spot size, the threshold fluence of 1,528 J/cm2 was not affected by compression. Histopathologic studies showed transretinal damage at the lowest levels necessary to achieve angiographic evidence of a treatment lesion or a barely visible funduscopic lesion at the time of treatment. CONCLUSIONS: Nonpigmented rabbits required more than a 12-fold increase in total TTT fluence compared with pigmented rabbits with the 2-mm spot size and a ninefold increase with the 3-mm spot size. Inner and outer retinal damage was seen histopathologically at these levels.
Animals↗
Orbital inflammatory syndromes with systemic involvement may mimic metastatic disease.
Physical examination of a 9-year-old girl with a 2-month history of swelling of the left orbit demonstrated an orbital mass, blepharoptosis, and proptosis. Computed tomography revealed a 2 x 3-cm mass in the superior left orbit that expanded orbital dimensions. Radiography showed abnormalities in the parietal and frontal bones and distal right tibia. Magnetic resonance imaging demonstrated an 8.5-cm abnormality of the marrow space of the right mid-tibia. Bone marrow biopsy was unremarkable. Orbital and tibial biopsies showed a nonspecific chronic inflammation. Idiopathic inflammation that involves the orbit (orbital pseudotumor) and that has systemic manifestations may mimic more serious conditions, such as metastases from rhabdomyosarcoma or Ewing sarcoma, chronic recurrent multifocal osteomyelitis (CRMO), and SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis). Accurate diagnosis requires careful clinical and pathologic examinations.
Blepharoptosis↗
Age-related macular degeneration: evaluation and treatment.
Any patient age 50 or older with distorted vision or vision loss may have age-related macular degeneration and should be immediately referred to an ophthalmologist. Early diagnosis and treatment are essential to preserve the current level of vision. We outline risk factors, clinical signs, what happens to the retina, and what treatments are currently available, as well as recommendations about vitamin and mineral supplementation.
Age Factors↗
Mutations in the RPGR gene cause X-linked cone dystrophy.
X-linked cone dystrophy is a type of hereditary retinal degeneration characterized by a progressive dysfunction of the day vision or photopic (cone) system with preservation of night vision or scotopic (rod) function. The disease presents with a triad of photophobia, loss of color vision and reduced central vision. This phenotype is distinct from retinitis pigmentosa (RP) in which there are prominent night and peripheral vision disturbances. X-linked cone dystrophy is a genetically heterogeneous disorder, with linkage to loci on Xp11.4--Xp21.1 (COD1, OMIM 304020) and Xq27 (COD2, OMIM 303800). COD1 maps to a region that harbors the RPGR gene, mutations in which account for >70% of patients with X-linked RP. The majority of these mutations reside in one purine-rich exon, ORF15, encoding 567 amino acids with a repetitive domain rich in glutamic acid residues. We mapped two families with X-linked cone dystrophy to the COD1 locus and identified two distinct mutations in ORF15 in the RPGR gene (ORF15+1343_1344delGG and ORF15+694_708del15) leading to a frame-shift and premature termination of translation in one case and a deletion of five amino acids in another. Consistent with expression of RPGR in rods and cones, our results show that mutations in RPGR, in addition to X-linked RP, can also cause cone-specific degeneration.
Amino Acid Sequence↗
Retinoblastoma metastatic to the ovary in a patient with Waardenburg syndrome.
PURPOSE: To report a child with retinoblastoma and Waardenburg syndrome who developed ovarian metastases. DESIGN: Interventional case report. METHODS: Unilateral retinoblastoma was diagnosed in a 3-year-old girl with Waardenburg syndrome and leukocoria in the right eye. The patient had a Reese-Ellsworth Group Va tumor and underwent enucleation. Two years later, she developed metastatic disease involving the bone marrow, right humerus, both supraorbital bones, and both tibias. She was treated with chemotherapy, orbital irradiation, and bone marrow transplant but returned 7 months later with back pain and urinary retention. RESULTS: Exploratory laparotomy revealed a right ovarian mass, and the excised ovary showed metastatic retinoblastoma. The child underwent chemotherapy and remained asymptomatic for 9 months, when brain metastases were diagnosed. She died within 2 days of admission. CONCLUSION: We believe that this is the first description of a patient with retinoblastoma and Waardenburg syndrome and of an ovarian metastasis from retinoblastoma.
Bone Neoplasms↗