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JAMA ophthalmology. 02/2013; 131(2):249-51.
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ABSTRACT: PURPOSE:: To describe the atypical presentation of fungal infection and necrotizing scleritis, the potential role of histopathology in the diagnosis, and surgical excision in the management. METHODS:: Retrospective interventional case series. RESULTS:: Two patients presented with a pigmented conjunctival mass, one resembling necrotizing scleritis with uveal prolapse and the other resembling a pigmented ocular surface tumor, both after excision of nasal pterygium, 12 and 50 years previously, respectively. The pigmented lesion was 2 × 1.5 mm in each case, both situated on the bulbar surface 2 mm from the nasal limbus. After surgical excision, each lesion histopathologically displayed fungal filaments (pigmented dematiaceous fungi). CONCLUSIONS:: Pigmented mycetoma (dematiaceous fungi) can simulate uveal tissue prolapse, pigmented foreign body, or pigmented epibulbar tumors, particularly melanoma. Surgical excision of the entire lesion is effective for management.
Cornea 06/2012; · 1.73 Impact Factor
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ABSTRACT: To describe the clinical features and histopathology of epibulbar osseous choristoma in a series of patients.
Noncomparative case series with chart review of 8 patients.
At presentation, the mean age of the patients was 19 years (median, 19 years; range, 0.8-38 years), with 6 women and 2 men (6 Caucasian, 1 Hispanic, and 1 African American). There were no related systemic syndromes, and visual acuity was unaffected in all cases. The choristoma was superotemporal (n = 8, 100%), with epicenter located in the fornix (n = 8, 100%), deep to Tenon fascia (n = 8, 100%), and of yellow (n = 2, 25%), white (n = 2, 25%), or pink (n = 4, 50%) color. The mean basal dimension was 9 mm (median, 10 mm; range, 3.5-14 mm), and mean thickness was 4 mm (median, 4 mm; range, 2-5 mm). Four cases were managed with observation and 4 with surgical excision, revealing tumor base adherent to the episclera (n = 2, 50%) or loose within Tenon fascia (n = 2, 50%). There were no cases to demonstrate intrascleral involvement, growth, or recurrence.
Epibulbar osseous choristoma is a congenital lesion of mature bone located superotemporally in the fornix at the level of Tenon fascia or episclera in young patients.
Cornea 04/2012; 31(7):756-60. · 1.73 Impact Factor
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ABSTRACT: To describe the clinical, imaging, and pathology features of oncocytic lesions in the ocular adnexa.
Retrospective, noncomparative, interventional case series.
Fifteen oncocytic neoplasms (n = 15 patients) were classified as oncocytoma in 14 (93%) and oncocytic hyperplasia in 1 (7%). The mean patient age was 66 years (median = 66, range = 44-82), and 9 (60%) were male. The tumor involved the caruncle (n = 13, 86%), bulbar conjunctiva (n = 1, 7%), and plica semilunaris (n = 1, 7%). All cases were unifocal and most often presented as a painless mass (n = 12, 80%), evolving over a median 6 months (mean = 12, range = 1-48 months). The mean tumor base was 4.1 mm (median = 4.0, range = 1-10), and the tumor was well-circumscribed (n = 14), dark blue (n = 7), and cystic (n = 10). Ultrasound biomicroscopy and anterior segment optical coherence tomography disclosed mixed solid and cystic components. Complete surgical excision was curative without recurrence after a mean follow up of 39 months. Histopathology disclosed columnar cells with copious quantities of intensely eosinophilic cytoplasm in the lining epithelium (oncocytic hyperplasia) and in some cases with cystadenomatous proliferation (oncocytoma).
Oncocytic lesions of the ocular adnexae are typically well-circumscribed, cystic lesions that most often involve the caruncle. Surgical excision can be curative. Epithelial cells with copious quantities of intensely eosinophilic cytoplasm characterize oncocytic transformation histopathologically.
Ophthalmic plastic and reconstructive surgery 09/2011; 28(1):14-21. · 0.69 Impact Factor
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ABSTRACT: To describe the demographics, characteristics, and treatment of giant fornix syndrome, a rare cause of chronic purulent conjunctivitis in the elderly.
Retrospective chart review of five patients with giant fornix syndrome evaluated by the Cornea Service, Oculoplastics and Orbital Surgery Service and the Department of Pathology at the Wills Eye Institute.
The median age of the 5 female patients was 75 years (mean 80, range 70-95). The median duration of eye symptoms before presentation was 2 years (mean 2.4, range 1-4). Before referral, the chronic conjunctivitis was treated with topical antibiotics in all 5 cases and with additional dacryocystorhinostomy in one case. The right eye was affected in 2 cases, and the left eye was affected in the other 3 cases. Floppy eyelids were present in 2 cases. The superior fornix was involved in 4 cases, and the inferior fornix was involved in one case. Pseudomembranes and superficial punctate keratitis (SPK) were seen in 3 cases. Diagnosis of giant fornix syndrome was made in all 5 cases. Conjunctival culture grew methicillin-resistant Staphylococcus aureus (MRSA), Pseudomonas aeruginosa, and S. aureus in singular cases. Case 1 was treated with topical moxifloxacin, Case 2 was treated with topical vancomycin and repair of the upper eyelid, Case 3 was treated with topical besifloxacin, and Case 4 was treated with dacryocystorhinostomy and topical vancomycin. Case 5 was treated with reconstruction of the left upper eyelid. The median duration of follow up was 4 months (mean 21.6, range 1-84).
Giant fornix syndrome can lead to chronic relapsing conjunctivitis in the elderly. Deep conjunctival fornices in affected patients can be a site for prolonged sequestration of bacteria causing recurrent infections. Removing the infected debris from the superior fornix and reconstruction of the upper eyelid may prevent the recurrent chronic persistent infection.
Ophthalmic plastic and reconstructive surgery 08/2011; 28(1):4-6. · 0.69 Impact Factor
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ABSTRACT: To report clinical and histopathologic features of vascular tumors of the conjunctiva.
Retrospective, noninterventional case series.
A total of 140 patients.
None.
Tumor diagnosis, anatomic location, clinical features, management, and histopathology.
There were 140 vascular tumors of the conjunctiva with 93% benign and 7% malignant. The specific diagnoses included lymphangioma/lymphangiectasis (n = 54, 36%), pyogenic granuloma (n = 31, 22%), capillary hemangioma (n = 20, 14%), Kaposi's sarcoma (n = 10, 7%), acquired sessile hemangioma (n = 10, 7%), racemose hemangioma (n = 7, 5%), varix (n = 4, 3%), cavernous hemangioma (n = 3, 2%), and glomangioma (n = 1, <1%). The lesions were unilateral in 89% and discovered at median age of 41 years. Bilateral lesions included lymphangiectasia, Kaposi's sarcoma, and racemose hemangioma. All tumors were typically found in adults with the exception of capillary hemangioma (<1 year) and glomangioma (16 years). The median tumor diameter was 6 mm, with the largest median diameter (15 mm) with Kaposi's sarcoma and (12 mm) glomangioma and capillary hemangioma and the smallest median diameter (3 mm) with cavernous hemangioma. The most common anatomic sites included bulbar conjunctiva, which was extralimbal in 80 patients (57%), limbal in 19 patients (14%), tarsal conjunctiva in 27 patients (19%), and forniceal conjunctival in 24 patients (17%). Clinical features included cystic component in 24% of patients (seen with lymphangioma, glomangioma, and varix), well-defined margins in 64% of patients, and feeder vessels in 39% of patients.
A variety of vascular tumors can occur in both children and adults at various anatomic locations in the conjunctiva. They tend to occur unilaterally on the bulbar conjunctiva, and the majority of tumors are benign.
Ophthalmology 07/2011; 118(9):1747-53. · 5.45 Impact Factor
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ABSTRACT: To report a case series of eyes with posterior polymorphous corneal dystrophy and steep nonkeratoconic corneas.
Retrospective, descriptive, nonrandomized case series.
Thirty-five eyes of 18 patients (14 cases from 6 families and 4 isolated cases) with diffuse posterior polymorphous corneal dystrophy had mean topographic simulated keratometry readings of 52.21 diopters (D), with a range of 46.47 D to 59.86 D and an SD of 3.69 D, with no slit-lamp or topographic findings suggestive of keratoconus.
The patients in this series demonstrate diffuse posterior polymorphous corneal dystrophy and nonkeratoconic steep corneas.
Cornea 07/2011; 30(10):1120-4. · 1.73 Impact Factor
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ABSTRACT: To describe histopathologic observations in eyes enucleated after intra-arterial chemotherapy (IAC) for retinoblastoma (Rb).
Retrospective histopathologic analysis of 8 eyes.
The eyes were enucleated for tumor viability (n = 4), neovascular glaucoma (n = 2), anaphylactic reaction from IAC (n = 1), and persistent retinal detachment with poor visualization of the tumor (n = 1). Of the 2 eyes judged clinically with complete tumor regression and the 5 with viable tumor, the findings were confirmed on histopathology. The Rb response ranged from minimal (n = 1) to moderate (n = 1) to extensive (n = 4) to complete regression (n = 2). Viable vitreous seeds (n = 4 eyes), invasion into the optic nerve (n = 3), reaching the lamina cribrosa in 2 cases, and invasion into the choroid (n = 1) were observed. Histopathologic evidence of ischemic atrophy involving the outer retina and choroid was found in 4 eyes. One eye treated at another center with IAC and enucleated by our team for recurrence was observed to have extensive choroidal and outer retinal atrophy. This case showed orbital vascular occlusion and subendothelial smooth muscle hyperplasia. Intravascular birefringent foreign material was observed in 5 cases within occluded vessels, stimulating a granulomatous inflammatory response. The foreign material comprised cellulose fibers (n = 3), synthetic fabric fibers (n = 1), or unknown composition (n = 2). Thrombosed blood vessels were identified in 5 eyes and involved ciliary arteries in the retrobulbar orbit (n = 5), scleral emissarial canals (n = 1), small choroidal vessels (n = 1), and central retinal artery (n = 1).
Retinoblastoma can be controlled with IAC, but histopathology of enucleated eyes reveals that ocular complications including thromboembolic events can occur.
Archives of ophthalmology 07/2011; 129(11):1416-21. · 3.86 Impact Factor
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Oman Journal of Ophthalmology 05/2011; 4(2):90-1.
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Albrecht von Graæes Archiv für Ophthalmologie 04/2011; 249(8):1259-61. · 2.17 Impact Factor
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ABSTRACT: To describe the clinical characteristics, time of presentation, risk factors, treatment, outcomes, and prognostic factors on a recent series of Acanthamoeba keratitis (AK) treated at our institution.
Retrospective case series of 59 patients diagnosed with AK from January 1, 2004 to December 31, 2008. Of these 59 patients, 51 had complete follow-up data and were analyzed using univariate and multivariate logistic regression analyses performed with "failure" defined as requiring a penetrating keratoplasty (PKP) and/or having (1) best-corrected visual acuity (BCVA) < 20/100 or (2) BCVA < 20/25 at the last follow-up. A single multivariate model incorporating age, sex, steroid use before diagnosis, time to diagnosis, initial visual acuity (VA), stromal involvement, and diagnostic method was performed.
Symptom onset was greatest in the summer and lowest in the winter. With failure defined as requiring PKP and/or final BCVA < 20/100, univariate analysis suggests that age > 50 years, female sex, initial VA < 20/50, stromal involvement, and patients with a confirmed tissue diagnosis had a significant risk for failure; however, none of these variables were significant using multivariate analysis. Univariate analysis, with failure defined as requiring PKP and/or final BCVA < 20/25, showed stromal involvement and initial VA < 20/50 were significant for failure-only initial VA < 20/50 was significant using multivariate analysis.
Symptom onset for AK is greatest in the summer. Patients with confirmed tissue diagnosis and female patients may have a higher risk for failure, but a larger prospective population-based study is required to confirm this. Failure is likely associated with patients who present with stromal involvement and patients presenting with an initial BCVA worse than 20/50.
Cornea 10/2010; 30(4):435-41. · 1.73 Impact Factor
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ABSTRACT: To report the clinical and histopathological findings of 3 cases of late-onset deep infectious keratitis after Descemet stripping endothelial keratoplasty (DSEK) with vent incisions.
From a retrospective review of 150 consecutive patients who underwent uncomplicated DSEK with vent incisions, 3 patients developed late-onset deep infectious keratitis.
In case 1, the patient suffered a Pseudomonas corneal ulcer at the nasal vent incision after a dacryocystorhinostomy with stent, 16 months after DSEK. In case 2, a Streptococcus pneumoniae infection developed at the inferior vent incision from a spastic entropion 3 months after surgery. In case 3, an Enterococcus faecalis corneal ulcer presented as a deep stromal abscess in the nasal vent incision 7 weeks after DSEK. All cases required full-thickness penetrating keratoplasties. Visual acuities at the last follow-up were counting fingers (case 1), 20/80 (case 2), and 20/400 (case 3).
Vent incisions in DSEK may allow bacterial keratitis to penetrate deeply leading to aggressive keratolysis. One must be cautious in using vent incisions in patients with increased bacterial flora and patients with poor ocular surface healing from systemic, local, or mechanical conditions. If vent incisions are performed, a midperipheral oblique incision, parallel to the limbus, with meticulous detail to wound construction is recommended.
Cornea 10/2010; 30(2):229-32. · 1.73 Impact Factor
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ABSTRACT: We report a ciliary body melanoma that apparently arose from a melanocytoma in a 15-year-old black teenager. The eye was enucleated, and metastatic evaluation remained negative at 5 years' follow-up. This unusual case, confirmed histopathologically, reveals that young patients with melanocytoma can have malignant transformation at an early age.
Journal of AAPOS: the official publication of the American Association for Pediatric Ophthalmology and Strabismus / American Association for Pediatric Ophthalmology and Strabismus 04/2010; 14(2):178-80. · 1.07 Impact Factor
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ABSTRACT: To report a series of 5 patients with soft contact lens (SCL) related fungal keratitis caused by unusual organisms diagnosed at the Wills Eye Institute, Cornea Service in 2008.
Chart review of patients with SCL related Alternaria and Paecilomyces keratitis diagnosed in 2008 was performed. Causes of these fungal infections were compared to previous years.
During a period from 1999 to 2007, among 64 patients with fungal keratitis, Alternaria was isolated from one case after corneal foreign body removal in 2004, and Paecilomyces was not isolated in any of these patients. In 2008, however, 5 patients with SCL-related atypical fungal keratitis were diagnosed. Alternaria was isolated from 2 cases and Paecilomyces from 3 cases. All patients wore Acuvue SCL (Johnson & Johnson Vision Care): Acuvue Oasys, Acuvue Advance, and Acuvue 2 (1 case each) and Acuvue unspecified (2 cases). A total of 2 patients with Alternaria and 1 patient with Paecilomyces used ReNu or a generic-brand solution manufactured by Bausch & Lomb; 2 with Paecilomyces keratitis used Opti-free Replenish solution. A total of 3 patients wore lenses for daily wear and 2 wore them occasionally for overnight wear. The patients were treated with either voriconazole drops alone or combined with voriconazole 200 mg pills or Natamycin 5% drops. Three patients responded well to treatment. One patient with Paecilomyces keratitis developed a corneal perforation managed with tissue adhesive. One Paecilomyces keratitis patient required an emergency penetrating keratoplasty because of a perforated corneal ulcer present at the initial examination.
We report 2 cases of Alternaria and 3 cases of Paecilomyces keratitis in patients wearing frequent replacement lenses and using multipurpose solutions diagnosed during 2008. We want to raise awareness of fungal keratitis caused by unusual organisms associated with SCL wear.
Cornea 03/2010; 29(5):564-8. · 1.73 Impact Factor
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ABSTRACT: There is an increasing need to obtain fresh tumor tissue and normal tissue for genetic studies and research following enucleation of an eye for uveal melanoma, retinoblastoma, and other lesions. A concern about doing this is that the globe will often collapse and lead to difficulties with gross and histopathologic interpretation. The technique reported herein is based on the creation of an opening in the sclera using an 8-mm corneal trephine followed by removal of tumor tissue. This method provides adequate tissue, preserves the spherical configuration of the eye, and allows for excellent histopathologic sections.
Archives of ophthalmology 02/2010; 128(2):241-3. · 3.86 Impact Factor
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ABSTRACT: To report a clinicopathologic correlation of an adenocarcinoma that arose from solitary congenital hypertrophy of the retinal pigment epithelium (CHRPE).
Case report with clinicopathologic correlation.
A 56-year-old black woman was referred for an asymptomatic fundus lesion in her left eye. It had features typical of CHRPE, but there was a small elevated nodule within the flat component, and the diagnosis was adenoma of the retinal pigment epithelium (RPE) arising from CHRPE. The lesion was observed periodically. The nodule slowly enlarged, and increasing amounts of lipoproteinaceous exudation accumulated in the adjacent retina. Thirteen years after the initial diagnosis, the patient returned with severe visual loss and no view of the fundus resulting from cataract and posterior synechia. Ultrasonography revealed a total retinal detachment and a pedunculated tumor measuring 7.5 mm in thickness. The eye was enucleated and studied histopathologically.
Clinical evaluation and correlation of clinical findings with histopathologic results of the enucleated eye.
Histopathologically, the mass was composed of a proliferation of atypical RPE cells with a marked infiltration of benign plasma cells. Typical features of CHRPE were present at the base of the tumor. The final diagnosis was adenocarcinoma arising from CHRPE.
Congenital hypertrophy of the retinal pigment epithelium, once considered to be a benign and stationary lesion, may spawn a malignant neoplasm.
Ophthalmology 10/2009; 116(11):2213-6. · 5.45 Impact Factor
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ABSTRACT: To determine the primary sites, clinical features, treatment, and outcome of 20 patients with cancer metastatic to the eyelids.
Retrospective review of medical records.
The primary tumors included skin melanoma (4 [20%]), uveal melanoma (4 [20%]), breast carcinoma and conjunctival melanoma (3 [15%] each), renal cell carcinoma (2 [10%]), and medullary thyroid carcinoma, prostate carcinoma, lung carcinoma, and salivary gland carcinoma (1 [5%] each). Eyelid metastasis was the first sign of systemic cancer in 3 patients (15%). The most common clinical finding at the initial examination was a solitary nodule in 12 patients (60%), a flat pigmented lesion and diffuse eyelid swelling in 3 patients each (15%), and multiple nodules and epiphora in 1 patient (5%) each. Ten patients (50%) had concomitant ocular site metastasis. Primary treatment included excision alone in 6 patients (30%), external beam radiotherapy in 7 (35%), systemic chemotherapy in 4 (20%), and observation in 3 (15%). The metastatic tumors regressed in 10 patients (50%), remained stable in 7 (35%), and showed progression in 3 (15%). After a mean follow-up of 16 months, 9 patients (45%) were alive and 11 (55%) had died of systemic metastatic disease.
Eyelid metastasis can display a variety of clinical features and should be considered in patients with known systemic cancer. These patients usually have multiple metastatic sites, ocular and nonocular. The systemic prognosis is poor.
Archives of ophthalmology 09/2009; 127(8):999-1005. · 3.86 Impact Factor
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ABSTRACT: We conducted this study to determine whether occurrence of primary uveal melanoma in the setting of familial atypical mole and melanoma (fam-m) syndrome (an autosomal dominant cutaneous preneoplastic syndrome) follows a pattern of a hereditary cancer predisposition syndrome. A retrospective review of 4600 consecutive patients with primary uveal melanoma revealed eight patients with biopsy-proven fam-m syndrome. The clinical profile of these patients was studied and their kindreds analyzed. In patients with fam-m syndrome, the uveal melanoma occurred at a relatively young age (mean 40 years; range 10-52 years). The diagnosis of fam-m syndrome preceded or followed the diagnosis of uveal melanoma by as much as 10 years. None of the patients had an associated nonmelanocytic malignancy. Three of the eight patients had a positive family history of melanoma (cutaneous melanoma (2) and uveal melanoma (1). The authors conclude that the occurrence of primary uveal melanoma in the setting of fam-m syndrome does not follow a clear pattern of a hereditary cancer predisposition syndrome.
07/2009; 16(2):53-61.
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ABSTRACT: To report the concomitant presentation of keratoconus and corneal dystrophies at Wills Eye Hospital for the 10-year period from January 1, 1997, to December 31, 2006.
Patients with concomitant keratoconus and corneal dystrophies were identified using a computer database. Complete ophthalmologic examination, keratometry, pachymetry, and computerized videokeratography were performed in all patients. When present, cornea guttata were confirmed by clinical examination and specular microscopy. Histopathologic examination with special stains of excised corneal buttons was performed.
Fifty-one patients manifested typical signs and topographic evidence of keratoconus associated with another corneal dystrophy. Fuchs dystrophy was the most common association accounting for 27 cases (52.9%), followed by anterior basement membrane dystrophy with 13 cases (25.5%) and posterior polymorphous dystrophy with 7 cases (13.8%). A bilateral combination of Fuchs dystrophy and anterior basement membrane dystrophy with keratoconus was seen in 3 cases (5.8%). Finally, there was 1 bilateral case (2%) of granular dystrophy. Histopathologic studies in cases that underwent penetrating keratoplasty confirmed the clinical diagnoses.
To our knowledge, this is the largest report of such a concurrence in the English literature and could lead to further studies on the possible pathophysiologic or genetic links between these entities, although a chance association cannot be excluded.
Cornea 03/2009; 28(2):127-35. · 1.73 Impact Factor
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ABSTRACT: To describe the histopathologic findings and relevant clinical details of 3 patients undergoing penetrating keratoplasty (PK) after failure of posterior lamellar endothelial keratoplasty (EK).
Retrospective clinicopathologic case series. Patients 1 and 2 underwent EK for pseudophakic bullous keratopathy. Patient 3 underwent EK for persistent corneal edema secondary to a Descemet membrane (DM) detachment after cataract extraction. Patient 1 had persistent diffuse corneal edema and broad, long-standing iridocorneal adhesions that precluded repeat EK. Patient 2 had high intraocular pressure and severe anterior chamber inflammation 1 day postoperatively with subsequent noncorneal clearing and elected PK over repeat EK. Progressive corneal edema with resultant poor visual acuity after EK was the reason for PK in patient 3.
Histopathologic examination disclosed thickened, edematous corneas with attenuated endothelium consistent with graft failure caused by endothelial decompensation in all 3 cases. Although various degrees of posterior lamellar graft detachment were observed in each instance, significant parts of each graft remained adherent to the host stroma or to segments of residual host DM. The wounds in the adherent areas, although discernible, were relatively inconspicuous, resembling those seen at the flap-stromal interface after laser in situ keratomileusis. The donor graft endothelium was atrophic in all cases, and a delicate retrocorneal fibrous membrane was present in 2 cases. Most of the graft in cases 1 and 2 remained adherent, with small areas of peripheral detachment. In contrast, the graft in case 3 adhered peripherally but had separated from the stroma centrally, forming a thin cleft.
Histopathology suggests endothelial decompensation, incomplete graft adherence, and the formation of retrocorneal fibrous membranes as possible etiologies for EK failure. The adherence of endothelial grafts to residual host DM suggests that it may not be necessary to remove optically clear DM before endothelial graft placement. The inconspicuous nature of the EK interface suggests that it may not play a large role in image degradation, although more study is needed.
Cornea 10/2008; 27(8):900-4. · 1.73 Impact Factor
