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1.
Ophthalmic Plast Reconstr Surg ; 39(5): e158-e160, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37195834

RESUMO

A 58-year-old female with a 3-year history of adult-onset asthma, bilateral blepharoptosis, dry eye, and yellow-orange xanthelasma-like plaques extensively involving both upper eyelids presented with a diagnosis of adult-onset asthma with periocular xanthogranuloma (AAPOX) and systemic IgG4-related disease. Over the next 8 years, she received 10 intralesional triamcinolone injections (40-80 mg) in the right upper eyelid, 7 intralesional triamcinolone injections (30-60 mg) in the left upper eyelid, underwent right anterior orbitotomy twice followed by 4 doses of rituximab (1000 mg intravenous infusion) without regression of the AAPOX. The patient was then treated with 2 monthly doses of Truxima (1000 mg intravenous infusion), a biosimilar to rituximab. At the most recent follow-up, 13 months later, the xanthelasma-like plaques and orbital infiltration had markedly improved. To the best of the authors' knowledge, this is the first report of Truxima being used to treat AAPOX with systemic IgG4-related disease and to generate a sustained clinical response.


Assuntos
Asma , Doença Relacionada a Imunoglobulina G4 , Neoplasias de Tecidos Moles , Xantomatose , Feminino , Adulto , Humanos , Pessoa de Meia-Idade , Rituximab/uso terapêutico , Granuloma/diagnóstico , Xantomatose/complicações , Xantomatose/diagnóstico , Xantomatose/tratamento farmacológico , Asma/complicações , Asma/diagnóstico , Asma/tratamento farmacológico , Triancinolona
2.
Ophthalmic Plast Reconstr Surg ; 39(1): 49-57, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36598390

RESUMO

PURPOSE: To compare the clinical features at presentation and treatment outcomes of conjunctival melanoma by absence/presence of orbital invasion. METHODS: A retrospective review of patients with conjunctival melanoma managed at a single tertiary referral center from April 18, 1974, to September 9, 2019. RESULTS: Of 430 patients with conjunctival melanoma, 21 (5%) had orbital invasion at presentation. A comparison between the 2 groups (orbital invasion absent vs. present) revealed that the orbital invasion group had a higher frequency of prior eyelid incisional biopsy (5% vs. 24%, P = 0.006), greater tumor basal diameter (12.2 vs. 17.3, P = 0.009), greater tumor thickness (2.4 vs. 7.0, P < 0.001), more quadrants involved (1.8 vs. 2.5, P = 0.002), and more clock hours involved (4.4 vs. 5.8, P = 0.037). In addition, those with orbital invasion were more likely to undergo exenteration as primary treatment (1% vs. 24%, P < 0.001). Multivariate relative risk regression analysis revealed that variables predictive of orbital invasion included greater tumor thickness (P < 0.001) and greater involvement of the fornix (P = 0.031) and tarsus (P = 0.033). Outcomes revealed orbital invasion group with greater 5-year/10-year distant metastatic rate (16%/21% vs. 63%/63%, P = 0.005), and greater melanoma-related death rate (7%/13% vs. 38%/53%, P = 0.001). CONCLUSIONS: Conjunctival melanoma with orbital invasion at presentation demonstrate larger, more extensive tumors involving the fornix or tarsus, and with greater rate of melanoma-related metastasis and death.


Assuntos
Neoplasias Ósseas , Neoplasias da Túnica Conjuntiva , Melanoma , Humanos , Recidiva Local de Neoplasia , Neoplasias da Túnica Conjuntiva/patologia , Melanoma/patologia , Resultado do Tratamento , Estudos Retrospectivos
3.
Ophthalmic Plast Reconstr Surg ; 39(6): e173-e176, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37279029

RESUMO

A 53-year-old Caucasian male presented with an inflamed-appearing limbal nodule in his OD, clinically compatible with nodular episcleritis, that was unresponsive to topical corticosteroid therapy. Excisional biopsy of the lesion was performed and histopathological examination revealed foci of necrotizing vasculitis and granulomatous inflammation in a background of intense actinic elastosis. Infectious stains for organisms were negative. A comprehensive systemic evaluation for vasculitides was negative. Three years later, the patient returned with a clinically and histopathologically identical lesion in his OS. Systemic evaluation was noncontributory again, and a diagnosis of bilateral conjunctival actinic granuloma with necrobiotic vasculitic pattern was made.


Assuntos
Dermatopatias , Vasculite , Humanos , Masculino , Pessoa de Meia-Idade , Granuloma/diagnóstico , Granuloma/patologia , Diagnóstico Diferencial , Biópsia
4.
Ophthalmology ; 129(6): 679-693, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-35085662

RESUMO

PURPOSE: To evaluate targetable mutations and molecular genetic pathways in conjunctival melanoma with clinical correlation. DESIGN: Observational case series. PARTICIPANTS: Patients with conjunctival melanoma. MAIN OUTCOME MEASURES: Mutational profile of the tumor by next-generation sequencing (NGS), alternative lengthening of telomeres (ALT) by fluorescence in situ hybridization (FISH), and ATRX immunohistochemistry. Outcomes at 2 years and 5 years of tumor-related metastasis and death were recorded. RESULTS: Of the 101 patients, mean age at presentation was 60 years, 52% were male, and 88% were White. The NGS panels initially targeted BRAF only (n = 6, 6%), BRAF/NRAS (n = 17, 17%), and BRAF/NRAS/NF1 (n = 10, 10%). Sixty-eight tumors were tested with the expanded 592-gene panel. Next-generation sequencing identified high-frequency mutations in NF1 (29/74, 39%), BRAF (31/101, 31%), NRAS (25/95, 26%), and ATRX (17/68, 25%). Of those with an ATRX mutation, 12 (71%) had an additional NF1 mutation. A subset analysis of 21 melanomas showed that the ATRX mutation was associated with loss of ATRX protein expression and ALT. Loss of ATRX expression and ALT were present in both intraepithelial and invasive tumors, suggesting that an ATRX mutation is an early event in conjunctival melanoma progression. The NF1 and ATRX mutations were associated with tarsal (vs. nontarsal) tumors (NF1: 28% vs. 9%, P = 0.035, ATRX: 41% vs. 14%, P = 0.021) and orbital (vs. nonorbital) tumors (ATRX: 24% vs. 2%, P = 0.007). ATRXMUT (vs. ATRXWT) tumors were associated with a lower 2-year rate of metastasis (0% vs. 24%, P = 0.005). NRASMUT (vs. NRASWT) tumors were associated with a greater 2-year rate of metastasis (28% vs. 14%, P = 0.07) and death (16% vs. 4%, P = 0.04), with a 5-fold increased risk of death (relative risk, 5.45 [95% confidence interval, 1.11-26.71], P = 0.039). CONCLUSIONS: This study confirms the high frequency of previously documented BRAF and NRAS mutations and recently reported ATRX and NF1 mutations in conjunctival melanoma. An NRAS mutation implied increased risk for metastasis and death. Loss of ATRX and ALT may be early events in conjunctival melanoma development.


Assuntos
Neoplasias da Túnica Conjuntiva , Melanoma , Neoplasias Cutâneas , Neoplasias da Túnica Conjuntiva/genética , Neoplasias da Túnica Conjuntiva/patologia , Análise Mutacional de DNA , Feminino , Humanos , Hibridização in Situ Fluorescente , Masculino , Melanoma/genética , Melanoma/patologia , Mutação , Proteínas Proto-Oncogênicas B-raf/genética , Neoplasias Cutâneas/patologia
5.
Ophthalmic Plast Reconstr Surg ; 38(6): e180-e183, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35793663

RESUMO

Myxomas are a heterogeneous group of mesenchymal tumors. Soft tissue myxomas are divided into myocardial, intramuscular, juxta-articular, superficial, aggressive, and nerve sheath myxomas. Although benign, myxomas have site-specific biologic behavior and syndromic associations, which can influence prognosis and management. In addition, myxomas need to be distinguished from malignant neoplasms, such as myxofibrosarcomas, low-grade fibromyxoid sarcomas, myxoid liposarcomas, and peripheral nerve sheath tumors. While myxomas can occur throughout the body, these tumors arise more commonly in the thigh, shoulder, buttocks, and upper extremity, and less often in the head and neck. Rarely, myxomas can arise in a periocular location, typically in the conjunctiva and eyelid skin. In this case report, we present a patient with recurrent intramuscular myxoma of the eyelid and discuss the differential diagnosis and syndromic associations of this neoplasm. To our knowledge, there have been no prior reports of intramuscular myxoma of the eyelid and orbit.


Assuntos
Fibrossarcoma , Mixoma , Neoplasias de Bainha Neural , Adulto , Humanos , Mixoma/diagnóstico , Mixoma/cirurgia , Mixoma/patologia , Fibrossarcoma/diagnóstico , Diagnóstico Diferencial , Neoplasias de Bainha Neural/diagnóstico , Pálpebras/patologia
6.
Orbit ; 40(5): 423-430, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32811269

RESUMO

Lacrimal drainage apparatus melanoma is a rare entity that may arise primarily or, more commonly, as secondary involvement from melanoma originating elsewhere. Conjunctival melanoma may involve the lacrimal drainage apparatus (LDA) via spread along the canalicular epithelium, separate in situ processes, or direct invasion. Only seven cases exist in the literature where conjunctival melanoma remotely recurred in the LDA. We report three additional patients, two with invasive conjunctival melanoma and one with primary acquired melanosis (PAM) with severe atypia/melanoma in situ, who developed LDA melanoma at 5, 8, and 16 years after initial treatment of conjunctival melanoma. This report confirms the ability of conjunctival melanoma to give rise to spatially and temporally remote LDA melanoma despite adequate local treatment, and reviews the proposed mechanisms and associated characteristics of LDA recurrence in conjunctival melanoma.


Assuntos
Neoplasias da Túnica Conjuntiva , Aparelho Lacrimal , Melanoma , Melanose , Túnica Conjuntiva , Neoplasias da Túnica Conjuntiva/terapia , Humanos , Melanoma/terapia
7.
Mod Pathol ; 33(7): 1242-1255, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-31932683

RESUMO

In 2018, the consensus meeting for the WHO Classification of Tumours of the Eye decided that conjunctival mucoepidermoid carcinoma should be reclassified as adenosquamous carcinoma, as this represented a better morphological fit. To examine the applicability of this terminology, we studied the clinical, histopathological, immunohistochemical and molecular pathology of 14 cases that were originally diagnosed as conjunctival mucoepidermoid carcinoma. There were 7 (50%) females and 7 (50%) males. The median age was 64 years. The left eye was affected in 8 and the right eye in 6 patients. In-situ carcinoma was present in 11/14 (79%) cases and comprised in-situ squamous cell carcinoma (SCC) and conjunctival intraepithelial neoplasia with mucinous differentiation (CIN-Muc). Invasive carcinoma was present in 11/14 (79%) cases. Group 1 (1/11 cases, 9%) comprised invasive SCC only. Group 2 (6/11 cases, 55%) comprised SCC with mucinous differentiation, manifesting as scattered intracellular mucin, occasionally together with intercellular mucin, with no evidence of true glandular differentiation. Group 3 (3/11 cases. 27%) comprised true adenosquamous carcinoma. Group 4 (1/11 cases, 9%) comprised pure adenocarcinoma. Thirteen of 14 cases (93%) underwent FISH for MAML2 translocation and none were rearranged. Two cases harboured high-risk HPV (type 16 and 18). The combined findings confirm that all lesions in our study were not mucoepidermoid carcinoma, but represented predominantly SCC with mucinous differentiation and adenosquamous carcinoma. We, therefore, recommend future revision of the WHO classification to include SCC with mucinous differentiation alongside adenosquamous carcinoma.


Assuntos
Carcinoma Adenoescamoso/patologia , Carcinoma Mucoepidermoide/patologia , Carcinoma de Células Escamosas/patologia , Neoplasias da Túnica Conjuntiva/classificação , Neoplasias da Túnica Conjuntiva/patologia , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/análise , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Organização Mundial da Saúde
8.
Ophthalmic Plast Reconstr Surg ; 36(5): 490-496, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32134771

RESUMO

PURPOSE: To determine the association of Fitzpatrick skin type (FST) with conjunctival melanoma. METHODS: Retrospective case series of 540 patients with conjunctival melanoma to assess clinical features and outcomes per FST. RESULTS: The FST was Type I (n = 126, 23%), II (n = 337, 62%), III (n = 56, 10%), IV (n = 8, 2%), V (n = 12, 2%), and VI (n = 1, <1%). A comparison (FST I vs. II vs. III, IV, V, and VI) revealed Types I and II associated with older mean patient age (63.9 vs. 60.7 vs. 51.1 years, p < 0.001), greater percentage of female patients (68% vs. 44% vs. 42%, p < 0.001), lower frequency of complexion associated melanosis (1% vs. 2% vs. 13%, p < 0.001), smaller tumor thickness (2.1 vs. 2.8 vs. 3.6 mm, p = 0.01), and less eyelid involvement (13% vs. 13% vs. 28%, p = 0.02). Kaplan-Meier estimates for 5-year risk showed no difference by Types for visual acuity loss ≥3 lines, local tumor recurrence, exenteration, metastasis, or death. CONCLUSION AND RELEVANCE: Most patients with conjunctival melanoma show FST I or II, and this demonstrated no association with 5-year rate of vision loss, tumor recurrence, exenteration, metastasis, or death.


Assuntos
Neoplasias da Túnica Conjuntiva , Melanoma , Melanose , Neoplasias da Túnica Conjuntiva/epidemiologia , Neoplasias da Túnica Conjuntiva/terapia , Feminino , Humanos , Melanoma/diagnóstico , Melanoma/epidemiologia , Melanoma/terapia , Recidiva Local de Neoplasia , Estudos Retrospectivos
9.
Orbit ; 39(2): 139-142, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31021283

RESUMO

Purpose: To report a case of delayed implant infection with Cutibacterium acnes (C. acnes, previously known as Propionibacterium acnes) 30 years after silicone sheet orbital floor implant.Methods: Case report with orbital imaging.Results: A 61-year-old male with a history of traumatic orbital floor fracture right eye (OD) repaired using a silicone sheet orbital floor implant 30 years prior, presented with 6 months of painless blepharoptosis and diplopia OD. On examination, there was 3 mm right upper eyelid blepharoptosis and hyperglobus. There was no globe proptosis, dysmotility, or compression and no cutaneous erythema, hyperthermia, discharge, or tenderness to palpation. Orbital magnetic resonance imaging (MRI) revealed a cystic mass in the inferior orbit in the region of the floor implant, measuring 25 mm in diameter and 10 mm in thickness. By MRI, T1-weighted images revealed a hypointense signal within the mass and T2-weighted images showed hyperintense signal with a flat hypointensity centrally representing the floor implant. Microbiologic cultures grew C. acnes.Conclusions: C. acnes can manifest several decades after placement of an orbital prosthetic implant, leading to delayed infection.


Assuntos
Blefaroptose/microbiologia , Diplopia/microbiologia , Infecções por Bactérias Gram-Positivas/microbiologia , Implantes Orbitários/microbiologia , Propionibacterium acnes , Infecções Relacionadas à Prótese/microbiologia , Blefaroptose/cirurgia , Remoção de Dispositivo , Diplopia/cirurgia , Infecções por Bactérias Gram-Positivas/cirurgia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Fraturas Orbitárias/cirurgia , Infecções Relacionadas à Prótese/cirurgia , Silicones
10.
Ophthalmic Plast Reconstr Surg ; 35(3): e76-e80, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30908464

RESUMO

Atypical lipomatous tumor/well-differentiated liposarcoma is a common neoplasm of the superficial and deep soft tissues of the extremities, trunk, and retroperitoneum. Atypical lipomatous tumor/well-differentiated liposarcoma is very rare in the orbit, with only 19 previously reported cases. The authors describe a 22-year-old woman who presented with an 8-month history of diplopia and was found to have an orbital mass on MRI. The excised tumor initially was interpreted as spindle cell/pleomorphic lipoma based on its morphologic and immunohistochemical features. Nine years later, the patient returned with a recurrence that required surgical debulking. Histopathologic and molecular cytogenetic evaluation of both primary and recurrent lesions disclosed Atypical lipomatous tumor/well-differentiated liposarcoma. This case highlights the diagnostic challenges and the importance of molecular genetic studies in evaluation of fatty orbital tumors.


Assuntos
Lipoma/diagnóstico , Lipossarcoma/diagnóstico , Órbita/diagnóstico por imagem , Neoplasias Orbitárias/diagnóstico , Biomarcadores Tumorais/metabolismo , Biópsia , Diagnóstico Diferencial , Feminino , Humanos , Imuno-Histoquímica , Lipossarcoma/metabolismo , Proteínas S100/metabolismo , Adulto Jovem
11.
Retina ; 38(10): 2010-2022, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29528980

RESUMO

PURPOSE: To analyze the clinical features and rate of metastatic disease in eyes with large (≥10 mm thickness) uveal melanoma. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: There were 1,311 consecutive patients. METHODS: Retrospective medical chart review. MAIN OUTCOME MEASURES: Clinical features and rate of metastatic melanoma. RESULTS: Of 1,311 patients with large melanoma, the mean age was 59 years (median 60, range 6-98 years) and 95% were white. Mean tumor basal dimension was 17 mm (median 17, range 7-25 mm), and mean tumor thickness was 12 mm (median 12, range 10-24 mm). Mean distance to the foveola was 6 mm (median 6, range 0-19 mm) and to optic nerve was 6 mm (median 5, range 0-19 mm). Of all eyes, using Kaplan-Meier analysis, metastasis occurred in 11, 30, 45, and 52% at 1, 3, 5, and 7 years, respectively. According to tumor thickness (10.0-11.0, 11.1-12.0, 12.1-13.0, 13.1-14.0, 14.1-15.0, 15.1-16.0, and >16.0 mm), metastasis at 1 year was found in 7, 12, 13, 15, 18, 22, and 20%; metastasis at 3 years was 24, 27, 37, 35, 51, 69, and 57%; metastasis at 5 years was 38%, 42%, 56%, 48%, 61%, not available, and 66%; and metastasis at 7 years was 47%, 47%, 61%, 57%, 61%, not available, and 66%. Clinical features associated with fewer metastatic events included Bruch membrane rupture (7-year metastasis at 48%, P = 0.018) and macular location (7-year metastasis at 32%, P = 0.014), whereas those with worse outcome included extraocular extension (7-year metastasis at 79%, P < 0.001). There was no significant difference in rate of melanoma-related metastasis for patients treated with plaque radiotherapy versus enucleation. CONCLUSION: Large uveal melanoma demonstrates 7-year rate of metastasis at 52%, with generalized increasing risk per 1-mm or 2-mm thickness increments. Extraocular extension was associated with greater metastatic rate, whereas Bruch membrane rupture and macular location demonstrated lower rate.


Assuntos
Melanoma/patologia , Metástase Neoplásica/patologia , Neoplasias Uveais/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Adulto Jovem
13.
Ophthalmic Plast Reconstr Surg ; 34(2): 162-167, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-28353472

RESUMO

PURPOSE: To evaluate the relationship between radiographic and histopathologic features of orbital schwannoma. METHODS: Retrospective review of 15 patients with orbital schwannoma managed at an ocular oncology service. RESULTS: The mean patient age at the time of presentation was 42 years old (median 40, range 15-64 years). The orbital schwannoma was found incidentally (n = 2) or with symptoms of proptosis (n = 2), blurred vision (n = 3), pain (n = 3), eyelid swelling (n = 2), diplopia (n = 2), or headache (n = 1). The mean duration of symptoms was 15 months (median, 9; range 1-60 months). The tumor occupied the superior (n = 11) or inferior (n = 4) orbit. Antero-posterior tumor location involved the anterior (n = 2), middle (n = 3), posterior (n = 4), or entire (n = 6) orbit. MRI was performed in 12 patients (80%) and CT was the only form of imaging in 3 patients (20%). The T1-weighted MRI (n = 11) showed the mass as isointense (n = 10) or hyperintense (n = 1) to the extraocular muscles). On T2-weighted MRI (n = 10), the mass demonstrated hyperintensity (n = 9) or hypointensity (n = 1). Histopathologic assessment demonstrated Antoni A (n = 12) and Antoni B (n = 12) patterns. Antoni A pattern correlated with hyperintensity on T1-weighted MRI and hypointensity on T2-weighted MRI. Antoni B pattern correlated with hypointensity on T1-weighted MRI and hyperintensity on T2-weighted MRI. As Antoni B approached >50% of the solid mass (n = 8), both T1- and T2-weighted MRI images were more likely to be heterogeneous (n = 7, 88% and n = 6, 75%, respectively). CONCLUSION: Orbital schwannomas are nearly always benign, well-encapsulated slowly progressive tumors. Due to the biphasic distribution of Antoni A and Antoni B pattern, the appearance on MRI has a variable degree of heterogeneity. Antoni A pattern correlated with hyperintensity and Antoni B pattern correlated with hypointensity on T1-weighted MRI.


Assuntos
Neurilemoma/patologia , Neoplasias Orbitárias/patologia , Adolescente , Adulto , Exoftalmia/patologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neurilemoma/diagnóstico por imagem , Músculos Oculomotores/patologia , Neoplasias Orbitárias/diagnóstico por imagem , Estudos Retrospectivos , Adulto Jovem
14.
Ophthalmology ; 123(2): 378-384, 2016 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-26522706

RESUMO

PURPOSE: Diffuse anterior retinoblastoma is an exquisitely rare variant of retinoblastoma in which the tumor resides in the anterior segment of the eye, without apparent retinal involvement. Previously published cases have been managed with enucleation. We describe globe salvage and visual preservation in 3 consecutive cases using chemotherapy and radiotherapy. DESIGN: Retrospective case series. PARTICIPANTS: Three children with diffuse anterior retinoblastoma. METHODS: Plaque radiotherapy plus intravenous chemotherapy. MAIN OUTCOME MEASURES: Globe and vision preservation. RESULTS: The mean patient age at presentation elsewhere was 5.7 years (median, 7; range, 3-7 years). There were 2 white female patients and 1 African American male patient. The initial observation by parents/caregiver was reduced vision (n = 1), red eye (n = 1), or cloudy eye (n = 1), and the initial finding by physician was iris tumor (n = 2) or hyphema (n = 1). Referring diagnosis was iris melanoma (n = 1), infectious endotheliitis (n = 1), and possible tumor (nonspecified) (n = 1). At our evaluation, visual acuity was 20/50 to 20/60 (n = 2) and fix no follow (n = 1). In all cases, the opposite eye was normal. Mean intraocular pressure was 20 mm Hg (median, 16; range, 15-30 mmHg). Our examination revealed solid iris tumor (n = 3), ciliary body involvement (n = 2), and anterior chamber seeding (n = 3). In no case was there choroidal or retinal tumor, vitreous seed or subretinal seed, or extrascleral extension. Clear corneal fine-needle aspiration biopsy confirmed the diagnosis as retinoblastoma in each case. Treatment included plaque radiotherapy (n = 3) plus additional systemic chemotherapy (n = 2). At mean follow-up of 35 months (median, 34; range, 20-51 months), there has been no recurrence, extrascleral extension, enucleation, metastasis, or death. In all 3 cases, cataract surgery was necessary at a mean interval of 16 months after complete and stable regression of retinoblastoma. CONCLUSIONS: The rare diffuse anterior form of retinoblastoma can be managed with globe-salvaging alternatives and with visual preservation in selected cases.


Assuntos
Câmara Anterior/patologia , Corpo Ciliar/patologia , Neoplasias da Íris/patologia , Inoculação de Neoplasia , Retinoblastoma/patologia , Neoplasias Uveais/patologia , Antineoplásicos/uso terapêutico , Biópsia por Agulha Fina , Braquiterapia , Criança , Pré-Escolar , Terapia Combinada , Feminino , Humanos , Neoplasias da Íris/fisiopatologia , Neoplasias da Íris/terapia , Masculino , Retinoblastoma/fisiopatologia , Retinoblastoma/terapia , Estudos Retrospectivos , Neoplasias Uveais/fisiopatologia , Neoplasias Uveais/terapia , Acuidade Visual/fisiologia
15.
Curr Opin Ophthalmol ; 27(5): 413-5, 2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-27219273

RESUMO

PURPOSE OF REVIEW: The purpose of this article is to review the recent published literature in 2015 on lymphatic malformation and provide updated information on the disease. Specifically, this article will describe recent advances in identifying and managing lymphatic malformations. RECENT FINDINGS: All articles discussed in this article emphasize the difficulty in treating lymphatic malformations. Most patients have long complicated histories with multiple recurrences. Studies are trying to identify which lesions will be successful treated by certain therapeutic modality. Treatment includes nonsurgical and surgical therapies. SUMMARY: Lymphatic malformations are difficult lesions to treat because they do not respect tissue planes. The use of sclerosing agents shows great promise for treating macrocysts. More knowledge is needed in the development of the disease process. Hopefully, antilymphangiogenic agent can provide targeted treatment.


Assuntos
Linfangioma/diagnóstico , Neoplasias Orbitárias/diagnóstico , Neoplasias Orbitárias/terapia , Humanos , Linfangioma/terapia , Anormalidades Linfáticas/diagnóstico , Anormalidades Linfáticas/terapia , Soluções Esclerosantes/uso terapêutico , Escleroterapia
16.
Ophthalmic Plast Reconstr Surg ; 32(5): 371-3, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26325381

RESUMO

PURPOSE: To evaluate the efficacy of topical 5% imiquimod cream in the treatment of periocular melanoma in situ (lentigo maligna). DESIGN: Retrospective case series. SUBJECTS: There were 12 patients in this series, and the mean patient age was 77 years. The anatomical locations were the lower eyelid (n=5), upper and lower eyelid (n=4), lower eyelid including the eyelid margin (n=1), brow (n=1), and the medial canthus (n=1). Topical 5% imiquimod cream was used as a primary treatment (n=6) or as an adjunctive therapy following local excision (n=2), cryotherapy (n=2), or excisional biopsy with cryotherapy (n=2). METHODS: Twelve patients with periocular melanoma in situ were treated with topical 5% imiquimod cream daily for a mean treatment period of 3.9 months. The clinical features of the patients and the responses to treatment were evaluated in a retrospective case series. MAIN OUTCOME MEASURES: Histologic clearance of atypical melanocytes. RESULTS: Eleven patients achieved complete histologic clearance of atypical melanocytes on post-treatment biopsy. One patient could not tolerate local irritation from imiquimod and stopped in the first month of therapy with residual disease. The median follow-up time was 1.5 years. Side effects included redness (n=12), discomfort (n=6), swelling (n=4), ectropion (n=1), and conjunctival chemosis (n=1). The patients experienced no systemic side effects from the treatment. CONCLUSIONS: Topical 5% imiquimod cream is an effective option as primary or adjunct therapy in the treatment of periocular melanoma in situ.


Assuntos
Aminoquinolinas/administração & dosagem , Neoplasias Palpebrais/tratamento farmacológico , Melanoma/tratamento farmacológico , Administração Tópica , Idoso , Antineoplásicos/administração & dosagem , Biópsia , Neoplasias Palpebrais/diagnóstico , Pálpebras/diagnóstico por imagem , Feminino , Humanos , Imiquimode , Masculino , Melanoma/diagnóstico , Estudos Retrospectivos , Creme para a Pele/administração & dosagem , Neoplasias Cutâneas , Resultado do Tratamento , Melanoma Maligno Cutâneo
17.
Ophthalmic Plast Reconstr Surg ; 32(4): 270-4, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27337659

RESUMO

PURPOSE: Pediatric idiopathic orbital inflammation (IOI) is a rare entity with little known about the clinical presentation and natural history. The authors report the demographics, clinical presentations, radiographic and histopathologic characteristics, and treatment outcome of 30 children with IOI. METHODS: Retrospective chart review of 30 patients 18 years and younger diagnosed with IOI and statistical analysis using analysis of variance and Fisher's exact test. This study was reviewed and approved by the Institutional Review Board of SUNY Downstate Medical Center. RESULTS: There were 9 males (30%) and 21 females (70%) with pediatric IOI who presented at a median age of 11 years (range 2-18 years). Primary IOI was found in 19 patients (63%) and recurrent IOI in 11 patients (37%). Overall, 26 patients (87%) had unilateral IOI while 4 patients (13%) had bilateral disease at presentation. There were 12 patients (40%) with systemic constitutional signs. The most common ophthalmic findings included periorbital edema (n = 20, 67%) and blepharoptosis (n = 17, 57%). All patients had orbital radiography with common findings of dacryoadenitis (n = 12, 40%), orbital mass (n = 12, 40%), or myositis (n=10, 33%). The presence of a radiographic orbital mass was significantly related to the clinical presence of blepharoptosis (p = 0.03). The most common treatment was oral glucocorticoids in 24 patients (80%). Over mean follow up of 19 months (range 6-64 months), females were more likely to display recurrent disease (p = 0.01). CONCLUSIONS: Idiopathic orbital inflammation is an uncommon but important cause of acute orbital syndrome in children, manifesting as a bilateral condition in 13% and with constitutional symptoms in 40%. Posttreatment recurrence is found in 37% of cases.


Assuntos
Glucocorticoides/administração & dosagem , Órbita/diagnóstico por imagem , Pseudotumor Orbitário/diagnóstico , Adolescente , Biópsia , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Seguimentos , Humanos , Injeções Intravenosas , Masculino , Pseudotumor Orbitário/tratamento farmacológico , Estudos Retrospectivos , Fatores de Tempo , Tomografia Computadorizada por Raios X , Resultado do Tratamento
18.
Orbit ; 35(5): 292-4, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27467709

RESUMO

A 69-year-old male experienced monocular formed visual hallucinations after occlusion of the right eye following resection of eyelid basal cell carcinoma and reconstruction with a Hughes procedure (tarsoconjunctival flap). His symptoms included recurrent, well-defined, organized, complex, formed images of small children playing in the snow. These visual phenomena occurred only in the occluded eye, began several hours after surgery, and recurred intermittently several times daily for 4 days, lasting several minutes with each occurrence. The patient retained insight into the false nature of the images throughout the duration of his symptoms, and the hallucinations resolved spontaneously while the flap was still in place. To our knowledge, this is the first reported case of Charles Bonnet Syndrome (CBS) following a Hughes procedure in a patient with normal visual acuity in the non-occluded fellow eye. Unlike other reported cases of acute onset CBS following transient monocular occlusion, hallucinations in the occluded eye remitted prior to restoration of vision in the occluded eye. Ophthalmologists should be aware of the potential for CBS following even transient monocular occlusion and should consider warning patients about its potential to occur.


Assuntos
Carcinoma Basocelular/cirurgia , Neoplasias Palpebrais/cirurgia , Alucinações/etiologia , Procedimentos Cirúrgicos Oftalmológicos/efeitos adversos , Neoplasias Cutâneas/cirurgia , Doença Aguda , Idoso , Carcinoma Basocelular/patologia , Neoplasias Palpebrais/patologia , Alucinações/fisiopatologia , Humanos , Masculino , Neoplasias Cutâneas/patologia , Retalhos Cirúrgicos , Acuidade Visual
19.
Ophthalmic Plast Reconstr Surg ; 31(2): 108-14, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-24896774

RESUMO

PURPOSE: To evaluate the features and long-term outcomes of hydroxyapatite (HA) orbital implant following enucleation in pediatric patients. METHODS: A retrospective review was performed on clinical records of patients ≤18 years of age , managed with enucleation and HA implant placement. Recorded outcomes included implant-related complications, implant and prosthesis motility, and patient cosmetic satisfaction. RESULTS: There were 525 children (531 sockets) with HA implant following enucleation. The mean age at HA implant placement was 3 years (median, 2 years; range, 4 days to 17 years). Indications for enucleation included retinoblastoma (n = 457, 86%), uveal melanoma (n = 17, 3%), medulloepithelioma (n = 8, 2%), and others (n = 49, 9%). The HA implant was uncoated (n = 370, 70%) or polymer coated (n = 161, 30%). For the 370 uncoated implants, wrapping was provided with scleral (n = 346, 94%) or bovine pericardium (n = 1, <1%), and 23 patients (6%) had no wrapping. The extraocular muscles were attached to the implant/polymer coat/wrap (n = 531, 100%), by suturing 4 rectus muscles (n = 349, 66%) or all 6 muscles (n = 182, 34%). Over mean follow up of 60 months, in 477 orbital implants, complications included conjunctival thinning (n = 10, 2%), implant infection (n = 6, 1%), implant exposure (n = 13, 3%), and implant migration/extrusion (n = 0, 0%). Motility for implant was judged as excellent (n = 245, 78%) and small-angle prosthesis motility was excellent (n = 196, 59%). Patient/family satisfaction with cosmetic outcome was rated as excellent in 471 implants (99%). CONCLUSIONS: Following enucleation in children, long-term outcomes of the HA orbital implant are excellent with favorable patient cosmetic satisfaction and rare complication.


Assuntos
Materiais Biocompatíveis , Durapatita , Enucleação Ocular , Órbita/cirurgia , Implantes Orbitários , Adolescente , Criança , Pré-Escolar , Neoplasias Oculares/cirurgia , Olho Artificial , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Satisfação do Paciente , Complicações Pós-Operatórias , Implantação de Prótese , Estudos Retrospectivos
20.
Ophthalmology ; 121(7): 1453-60, 2014 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24656794

RESUMO

OBJECTIVE: To analyze our 5-year experience of intra-arterial chemotherapy (IAC) for retinoblastoma as primary or secondary therapy. DESIGN: Retrospective interventional case series. PARTICIPANTS: A total of 70 eyes of 67 patients. INTERVENTION: Ophthalmic artery chemotherapy infusion under fluoroscopic guidance was performed using melphalan (3, 5, or 7.5 mg) in every case, with additional topotecan (1 mg) and/or carboplatin (30 or 50 mg) as necessary. MAIN OUTCOME MEASURES: Tumor control and treatment complications. RESULTS: The mean patient age at IAC was 30 months. The treatment was primary in 36 eyes and secondary in 34 eyes. Those primary therapy eyes were classified according to the International Classification of Retinoblastoma (ICRB) as group A (n = 0), B (n = 1), C (n = 4), D (n = 17), or E (n = 14). The secondary therapy eyes had failed previous intravenous chemotherapy (n = 34) in every case. Each eye received a mean of 3 IAC sessions per eye (median, 3; range, 1-7 sessions). After IAC with a mean follow-up of 19 months, globe salvage was achieved in 72% of primary-treated cases and in 62% of secondary-treated cases. Specifically, primary therapy achieved globe salvage for group B (100%), group C (100%), group D (94%), and group E (36%). Of all 70 eyes, complete regression was achieved for solid tumor in 48 of 51 eyes (94%), subretinal seeds in 40 of 42 eyes (95%), and vitreous seeds in 34 of 39 eyes (87%). After each catheterization (n = 198), the main complications included transient eyelid edema (5%), blepharoptosis (5%), and forehead hyperemia (2%). More lasting complications included vitreous hemorrhage (2%), branch retinal artery obstruction (1%), ophthalmic artery spasm with reperfusion (2%), ophthalmic artery obstruction (2%), partial choroidal ischemia (2%), and optic neuropathy (<1%). Over the past 3 years, the combined incidence of ophthalmic, retinal, and choroidal vascular ischemia was reduced to 1%. There was no patient with stroke, seizure, neurologic impairment, limb ischemia, secondary leukemia, metastasis, or death. CONCLUSIONS: Five-year experience with IAC indicates that this technique is remarkably effective for the management of retinoblastoma as both a primary and a secondary treatment.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Neoplasias da Retina/tratamento farmacológico , Retinoblastoma/tratamento farmacológico , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Carboplatina/administração & dosagem , Criança , Pré-Escolar , Feminino , Angiofluoresceinografia , Seguimentos , Humanos , Lactente , Infusões Intra-Arteriais , Classificação Internacional de Doenças , Masculino , Melfalan/administração & dosagem , Microscopia Acústica , Artéria Oftálmica , Neoplasias da Retina/classificação , Neoplasias da Retina/diagnóstico , Retinoblastoma/classificação , Retinoblastoma/diagnóstico , Estudos Retrospectivos , Topotecan/administração & dosagem , Resultado do Tratamento , Adulto Jovem
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