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Combined Hamartoma of the Retina and Retinal Pigment Epithelium at Pediatric Age: Surgical versus Conservative Approach

Ozdek, Sengul; Ucgul, Ahmet Yucel; Hartnett, M Elizabeth; Akdogan, Muberra; Sen, Parveen; Bhende, Muna; Besirli, Cagri Giray; Karacorlu, Murat; Dedania, Vaidehi; Parolini, Barbara; Mittal, Sangeet; Banker, Alay; El Rayes, Ehab; Tawfik, Mohamed; Wu, Wei-Chi; Attiku, Yamini; Hansen, Eric; Portney, David; Sarvaiya, Chintan; Sahin, Ozlem; Ozdemir, Huseyin Baran; Gurelik, Gokhan
PURPOSE/OBJECTIVE:To report outcomes of pediatric patients with combined hamartoma of the retina and the retina pigment epithelium (CHRRPE) followed up conservatively or after pars plana vitrectomy (PPV). METHODS:This retrospective multicenter study included sixty-two eyes of 59 pediatric patients with CHRRPE from 13 different international centers with an average age of 7.7±4.7 (0.3-17) years at the time of the diagnosis, and having undergone PPV or followed conservatively. At baseline and each visit, visual acuity values, optical coherence tomography (OCT) for features and central foveal thickness (CFT), and tumor location were noted. Lesions were called as zone 1, if it involves the macular and peripapillary area, and the others were called as zone 2 lesions. RESULTS:Twenty-one eyes of 20 patients in the intervention group and 41 eyes of 39 patients in the conservative group were followed for a mean of 36.2±40.4 (6-182) months. BCVA improved in 11 (68.8%) of 16 eyes in the intervention group and 4 (12.9%) of 31 eyes in the conservative group (p<0.001). The mean CFT decreased from 602.0±164.9 μm to 451.2±184.3 μm in the intervention group, while it increased from 709.5±344.2 μm to 791.0±452.1 μm in zone 1 eyes of the conservative group. Posterior location of tumor, irregular configuration of the foveal contour and ellipsoid zone defect in OCT, subretinal exudate and prominent vascular tortuosity were associated with poor visual acuity. CONCLUSIONS:Vitreoretinal surgery is safe and effective in improving vision and reducing retinal distortion in zone 1 CHRRPE in children.
PMID: 36228191
ISSN: 1539-2864
CID: 5361072

Paracentral Acute Middle Maculopathy Associated with Hypercoagulability in Pregnancy

Coulon, Sara J; Dedania, Vaidehi S
PURPOSE/OBJECTIVE:To report a case of paracentral acute middle maculopathy (PAMM) in an otherwise healthy young, multiparous woman in her second trimester of pregnancy. METHODS:Case report RESULTS:: A 38-year-old woman in her twentieth week of pregnancy presented with a four-day history of an acute paracentral scotoma in her left eye. Fundoscopic examination of the left eye was significant for a white-gray lesion inferonasal to the fovea which corresponded with spectral domain-optical coherence tomography (SD-OCT) hyperreflectivity at the outer plexiform layer-inner nuclear layer junction and optical coherence tomography angiography (OCTA) non-perfusion. A diagnosis of paracentral acute middle maculopathy was made. The patient was sent for a hypercoagulability work-up that revealed elevated factor VIII activity, which has been associated with increased risk of complications during pregnancy. CONCLUSION/CONCLUSIONS:PAMM in pregnancy may be secondary to an underlying hypercoaguable condition. We recommend systemic evaluation and referral to a high-risk pregnancy specialist if PAMM is diagnosed during pregnancy. Additionally, OCTA in PAMM may demonstrate reperfusion of the affected vessels.
PMID: 33229916
ISSN: 1937-1578
CID: 4680432

Intraocular Metastasis of Large T-cell Lymphoma Transformed from Mycosis Fungoides [Letter]

Chen, Dinah; Modi, Yasha; Goduni, Lediana; Chong, Jillian; Tsui, Edmund; Breazzano, Mark P; Dedania, Vaidehi; Marr, Brian; Sarraf, David
PURPOSE/UNASSIGNED:To describe a rare case of intraocular lymphoma that metastasized from cutaneous mycosis fungoides and transformed to large cell T cell lymphoma resulting in vitreoretinal pathology. METHODS/UNASSIGNED:Retrospective case report. RESULTS/UNASSIGNED:A 57-year-old male presented with 3 months of blurred vision in the right eye. He reported only a medical history of psoriasis. Examination revealed keratic precipitates and dense vitritis in the right eye. He was taken for a diagnostic vitrectomy. Histopathology showed that atypical lymphoid cells and flow cytometry were consistent with transformed large cell T-cell lymphoma. During follow-up, pre- and inner retinal lesions were noted throughout the posterior pole. Histopathology of the psoriatic lesions was consistent with mycosis fungoides. He was initiated on systemic and intravitreal methotrexate with improvement in vision. CONCLUSIONS/UNASSIGNED:Ocular involvement in metastatic transformed T-cell lymphoma is extremely rare but can be present with vitritis and retinal deposits. Our patient responded well to intravitreal methotrexate therapy.
PMID: 35201959
ISSN: 1744-5078
CID: 5172312

Improved Prognostic Precision in Uveal Melanoma through a Combined Score of Clinical Stage and Molecular Prognostication

Stacey, Andrew W; Dedania, Vaidehi S; Materin, Miguel; Demirci, Hakan
Introduction/UNASSIGNED:Prognosis of uveal melanoma (UM) is assessed using clinical staging or molecular testing. Two modalities often used for prognostication are the American Joint Committee on Cancer (AJCC) staging and a tumor gene expression profile (GEP), the outcomes of which are often discordant. This article discusses a total risk score created to combine the discordant information from both sources. Methods/UNASSIGNED:A retrospective case series was conducted of all patients presenting with UM over 6 years to 2 referral centers. Each tumor was classified using the AJCC and the GEP. A total risk score was calculated for each patient using results from both AJCC and GEP. Kaplan-Meier analysis of metastasis-free survival was used to compare groups. Results/UNASSIGNED:A total of 294 patients were included in the study. Kaplan-Meier estimates showed significant curve separation between individual AJCC and GEP risk groups. The combined total risk score provided an accurate estimate of prognosis that incorporated results from both AJCC and GEP. Conclusions/UNASSIGNED:Clinical staging and molecular prognostication of UM can be discordant. There is important information provided by each system that is not provided by the other. The total risk score provides a simple method to combine information from both AJCC stage and the GEP class in order to provide patients and care teams with a more complete understanding of metastatic risk.
PMID: 35356606
ISSN: 2296-4681
CID: 5191142

Endophthalmitis, Visual Outcomes, and Management Strategies in Eyes with Intraocular Foreign Bodies

Keil, Jason M; Zhao, Peter Y; Durrani, Asad F; Azzouz, Lyna; Huvard, Michael J; Dedania, Vaidehi S; Zacks, David N
Purpose/UNASSIGNED:Ocular trauma with intraocular foreign body (IOFB) can have devastating visual consequences. Management and antimicrobial strategies remain variable due to the infrequency and heterogeneity of presentation. Our goal was to identify risk factors for endophthalmitis and poor visual outcomes in cases of IOFB and investigate management strategies. Patients and Methods/UNASSIGNED:A retrospective chart review was conducted in 88 eyes of 88 patients suffering traumatic injury with IOFB at the University of Michigan between January 2000 and December 2019. Medical records were reviewed to characterize the injuries and IOFBs as well as how clinical presentation and treatment modalities were associated with outcomes. Results/UNASSIGNED:Delayed presentation (P=0.016) and organic IOFB (P=0.044) were associated with development of endophthalmitis. Retinal detachment (P=0.012), wound length greater than 5 mm (P=0.041), and poor presenting visual acuity (P=0.003) correlated with poor final visual outcome. Antibiotic prophylaxis was given to all patients, though agents and routes of delivery varied. Endophthalmitis developed in 4.9% of the eyes after initial management, with primary and secondary removal of posterior segment IOFBs associated with similar rates of endophthalmitis (P=1.000). Conclusion/UNASSIGNED:Poor presenting visual acuity and severity of injury, as measured by large wound and retinal detachment, correlate with poor visual outcome. Prompt globe closure and antimicrobial prophylaxis are critical for infection prevention. In cases where IOFB removal and globe closure cannot be performed concurrently, primary globe closure with aggressive antibiotic prophylaxis offers a reasonable alternative to prevent endophthalmitis.
PMID: 35535124
ISSN: 1177-5467
CID: 5214232


Tsui, Edmund; Leong, Belinda C S; Mehta, Nitish; Gupta, Akash; Goduni, Lediana; Cunningham, Emmett T; Freund, K Bailey; Lee, Gregory D; Dedania, Vaidehi S; Yannuzzi, Lawrence A; Modi, Yasha S
PURPOSE/OBJECTIVE:To describe the vascular anatomy and intraluminal flow characteristics of segmental retinal arteritis (SRA) using structural and angiographic optical coherence tomography (OCT). METHODS:Retrospective case series of consecutive patients presenting with SRA. All patients were evaluated at presentation with fundus photography, spectral domain OCT, and OCT angiography. One patient was imaged with dense B-scan OCT angiography. RESULTS:Three eyes of three male patients were evaluated. All examinations were consistent with reactivation of ocular toxoplasmosis with an area of active retinochoroiditis adjacent to a focal chorioretinal scar. Spectral domain OCT through areas of SRA noted on clinical examination demonstrated areas of hyperreflectivity circumscribing the affected vessel with a normoreflective lumen. Optical coherence tomography angiography and dense B-scan OCT angiography demonstrated narrowing of the intraluminal flow signal that correlated with areas of segmental hyperreflectivity on spectral domain OCT. Vascular sections proximal and distal to areas of SRA showed normal flow signal. CONCLUSION/CONCLUSIONS:Vessels with SRA demonstrated hyperreflectivity highlighting the vessel wall on spectral domain OCT. Optical coherence tomography angiography showed narrowing of the flow signal within these segments suggesting reduced lumen diameter. Coupling these finding with previous indocyanine green imaging findings in SRA, the collective data suggest the plaques are localized within the vessel wall to either the endothelium or the muscular tunica media without occlusion of the vessel lumen.
PMID: 31313702
ISSN: 1937-1578
CID: 3977882

Antimicrobial guide to posterior segment infections

Patel, Tapan P; Zacks, David N; Dedania, Vaidehi S
PURPOSE/OBJECTIVE:This review article is meant to serve as a reference guide and to assist the treating physician in making an appropriate selection and duration of an antimicrobial agent. METHODS:Literature review. RESULTS:Infections of the posterior segment require prompt medical or surgical therapy to reduce the risk of permanent vision loss. While numerous options exist to treat these infections, doses and alternative therapies, especially with contraindications for first-line therapy, are often elusive. Antimicrobial agents to treat posterior segment infections can be administered via various routes, including topical, intravitreal, intravenous, and oral. CONCLUSIONS:Although there are many excellent review articles on the management of endophthalmitis, we take the opportunity in this review to comprehensively summarize the appropriate antimicrobial regimen of both common and rare infectious etiologies of the posterior segment, using evidence from clinical trials and large case series.
PMID: 33156370
ISSN: 1435-702x
CID: 4664492

Diagnostic and Therapeutic Challenge

Wilkins, Carl S; Goduni, Lediana; Dedania, Vaidehi S; Modi, Yasha S; Johnson, Brandon; Mehta, Nitish; Weng, Christina Y
PMID: 32332425
ISSN: 1539-2864
CID: 4411532

DNA testing for inherited retinal disease (IRD): Initial experience with the SPARK/Invitae 'ID your IRD' genetic testing panel [Meeting Abstract]

Lidder, A; Modi, Y; Dedania, V S; Brodie, S E
Purpose : To evaluate the diagnostic yield and clinical impact of the SPARK/Invitae gene panel in patients with known or suspected inherited degenerative retinal disease, in comparison with traditional clinical assessments. Methods : Patients of the authors' clinical practices obtained genetic screening at no charge via the SPARK/Invitae ID your IRD genetic testing panel, which ranged from 248 genes to 293 genes. Over 16 months, tests were submitted for 87 patients and results were available for 70 patients. Clinical diagnoses prior to submitting the gene panel . By continuing to use our website, you are agreeing to included retinitis pigmentosa; Stargardt disease; Best vitelliform dystrophy; Leber our privacy policy. congenital amaurosis; choroideremia; achromatopsia; cone-rod dystrophy; congenital stationary night blindness; occult macular dystrophy; and familial dominant drusen in addition to patients with normal clinical findings and unclear diagnoses. Results : Of 70 patients, SPARK/Invitae considered the results ''Positive or Potentially Positive'' in 24 cases (34.3%), Carrier in 16 cases (22.9%) and ''Uncertain'' in 30 cases (42.9%). Uncertain results comprised patients with only Variants of Uncertain Significance. Patients categorized as a Carrier by SPARK/Invitae but who demonstrated pathogenic genetic changes correlating to the clinical diagnosis were considered to be in agreement with the clinical impression. The genetic diagnosis agreed with the clinical diagnosis in 30/70 (42.9%) total patients. Test results were consistent with the clinical impression in 13/26 (50.0%) retinitis pigmentosa cases, 6/8 (75.0%) Stargardt patients, 3/7 (42.9%) cone-rod dystrophy cases, and 2/4 (50.0%) Best vitelliform dystrophy patients. Gene testing helped elucidate diagnoses in two patients with unclear clinical impressions: one panel showed autosomal recessive achromatopsia and the other showed a carrier state for autosomal recessive retinitis pigmentosa. Of four patients with normal clinical exams, none had diagnostic results: all showed Uncertain findings. Conclusions : The SPARK/Invitae gene panel provided a genetic diagnosis consistent with the clinical impression in about 40 percent of patients. Retinitis pigmentosa and Stargardt disease were the most common clinical diagnoses and the diagnoses most often confirmed by testing. Genetic screening also assisted in clarifying unknown diagnoses for two patients
ISSN: 1552-5783
CID: 4982332

Endophthalmitis, visual outcomes, and management strategies in eyes with intraocular foreign bodies [Meeting Abstract]

Keil, J M; Zhao, P Y C; Durrani, A F; Azzouz, L; Huvard, M J; Dedania, V S; Zacks, D N
Purpose : To identify risk factors for endophthalmitis and poor visual outcomes in cases of retained intraocular foreign body (IOFB) and management strategies for these cases. Methods : A retrospective chart review was conducted in 88 eyes of 88 patients suffering traumatic injury with retained IOFB at the University of Michigan between January 2000 and December 2019. Medical records were reviewed to identify the nature of the injury, IOFB composition, and presenting characteristics of each eye as well as the surgical and antimicrobial strategies employed. Details of the injury, IOFBs, and clinical presentation were utilized to identify factors associated with clinical outcomes. Visual outcomes and development of endophthalmitis were additionally evaluated for association with treatment modalities. Results : This cohort developed endophthalmitis at a rate of 11.4% (4.5% presented with endophthalmitis, 7.1% of the remaining eyes developed endophthalmitis after initial intervention). Delayed presentation and organic IOFB were significantly associated with development of endophthalmitis. Retinal detachment, wound length greater than 5 mm, and poor presenting visual acuity were associated with poor final visual outcome. Antibiotic prophylaxis was given to all patients, though agents and routes of delivery varied. Primary and deferred removal of posterior segment IOFBs were associated with similar rates of endophthalmitis. Conclusions : Poor presenting visual acuity and severity of injury as measured by large wound and retinal detachment correlate with poor visual outcome. Prompt globe closure and antimicrobial prophylaxis is critical for infection prevention. Deferred IOFB removal may carry similar risk of endophthalmitis as primary removal
ISSN: 1552-5783
CID: 4982292