Heather D. Potter

Angiolymphoid Hyperplasia With Eosinophilia of the Orbit and Ocular Adnexa: Report of 5 Cases

IMPORTANCE To report the clinical and histopathologic findings of ocular adnexal angiolymphoid hyperplasia with eosinophilia, an unusual but often misdiagnosed benign disorder. OBSERVATIONS The ophthalmologic findings of angiolymphoid hyperplasia with eosinophilia with ocular adnexal involvement are variable and include eyelid swelling, ptosis, proptosis, and loss of vision. Imaging studies typically reveal a well-circumscribed mass in the orbit. The condition may resemble other diseases that involve the orbit and ocular adnexal tissue, such as lymphoma, hemangioma, sarcoidosis, and dermoid cyst. Histopathologic analysis reveals marked vascular proliferation with an accompanying inflammation composed of numerous eosinophils, lymphocytes, and plasma cells. CONCLUSIONS AND RELEVANCE Angiolymphoid hyperplasia with eosinophilia is a rare disease that can affect the ocular adnexal tissue. The clinical presentation is often nonspecific; therefore, histopathologic studies are essential for diagnosis and subsequent management of this benign condition.

Transitional cell carcinoma of the lacrimal sac presenting with bloody tears.

Transitional cell carcinomas (TCC) of the lacrimal sac are uncommon tumors that can have significant morbidity and mortality if not diagnosed and treated in a timely fashion. These tumors have variable clinical and histologic features. Bloody tears, also known as dacryohemorrhea, has been reported only once previously as a presenting sign of the tumor [1] .We describe a case of lacrimal sac TCC presenting with epiphora, dacryohemorrhea, and medial canthal mass.

Shaken Adult Syndrome: Report of 2 Cases

IMPORTANCE To establish that the intracranial and ophthalmologic findings present in victims of abusive head trauma can also be seen in shaken adults. OBSERVATIONS We report 2 cases of shaken adults with intracranial and ophthalmologic findings that resulted from repetitive acceleration-deceleration injury. These findings included intracranial hemorrhages, hemorrhages involving the optic nerve sheath, intraretinal and subretinal hemorrhages, and macular folds. CONCLUSIONS AND RELEVANCE The intracranial and ophthalmologic findings that are characteristic of abusive head trauma--subdural hemorrhages, optic nerve sheath hemorrhages, and retinal hemorrhages--are generally thought to be limited to young children and infants. Adults may also be victims of shaking abuse, and an ophthalmic examination may be beneficial when shaking is suspected.

The Significance of the Discordant Occurrence of Lens Tumors in Humans versus Other Species

PURPOSE The purpose of this study was to determine in which species and under what conditions lens tumors occur. DESIGN A review of databases of available human and veterinary ocular pathologic material and the previously reported literature. PARTICIPANTS Approximately 18 000 patients who had ocular surgical specimens submitted and studied at the University of Wisconsin School of Medicine and Public Health between 1920 and 2014 and 45 000 ocular veterinary cases from the Comparative Ocular Pathology Laboratory of Wisconsin between 1983 and 2014. METHODS Material in 2 major archived collections at the University of Wisconsin medical and veterinary schools were studied for occurrence of lens tumors. Tumor was defined as a new growth of tissue characterized by progressive, uncontrolled proliferation of cells. In addition, cases presented at 3 major eye pathologic societies (Verhoeff-Zimmerman Ophthalmic Pathology Society, Eastern Ophthalmic Pathology Society, and The Armed Forces Institute of Pathology Ophthalmic Alumni Society) from 1975 through 2014 were reviewed. Finally, a careful search of the literature was carried out. Approval from the institutional review board to carry out this study was obtained. MAIN OUTCOME MEASURES The presence of tumors of the lens. RESULTS The database search and literature review failed to find an example of a lens tumor in humans. In contrast, examples of naturally occurring lens tumors were found in cats, dogs, rabbits, and birds. In the veterinary school database, 4.5% of feline intraocular and adnexal neoplasms (234/5153) were designated as feline ocular posttraumatic sarcoma, a tumor previously demonstrated to be of lens epithelial origin. Similar tumors were seen in rabbit eyes, a bird, and in a dog. All 4 species with lens tumors had a history of either ocular trauma or protracted uveitis. The literature search also revealed cases where lens tumors were induced in zebrafish, rainbow trout, hamsters, and mice by carcinogenic agents (methylcholanthrene, thioacetamide), oncogenic viruses (SV40, HPV-16), and genetic manipulation. CONCLUSIONS Our results suggest that lens tumors do not occur in humans. In contrast, after lens capsule rupture, a lens tumor can occur in other species. We hypothesize that a genetic mechanism exists that prevents lens tumors in humans.

Postoperative visual acuity in patients with fuchs dystrophy undergoing descemet membrane-stripping automated endothelial keratoplasty: correlation with the severity of histologic changes.

OBJECTIVE To investigate a correlation between the severity of histologic changes of the Descemet membrane in patients with Fuchs endothelial dystrophy and the best-corrected visual acuity (VA) after Descemet membrane-stripping automated endothelial keratoplasty (DSAEK). METHODS In a retrospective study design, we created a histologic grading system based on common characteristics observed histologically among 92 DSAEK specimens sent to the University of Wisconsin Eye Pathology Laboratory with a clinical diagnosis of Fuchs dystrophy from 3 separate corneal surgeons. Cases were graded as mild, moderate, or severe on the basis of guttae dispersion, presence of a laminated Descemet membrane, presence of embedded guttae, and density of guttae. Regression models were built to study the relationship among preoperative VA, histologic findings, and best-corrected VA 6 months and 1 and 2 years after DSAEK. RESULTS No correlation was found between the severity of histologic changes of Descemet membrane and preoperative VA. However, a correlation was noted between the preoperative and final VA. Cases with a laminated Descemet membrane but no embedded guttae (n = 8) appeared to be less responsive to DSAEK. Otherwise, the severity of histologic changes of Descemet membrane observed in patients with Fuchs corneal dystrophy after DSAEK did not show a statistically significant correlation with final VA. CONCLUSIONS Our analysis fails to show an inverse relationship between the severity of histologic changes of the Descemet membrane and the best-corrected VA of at least 20/40 after DSAEK for Fuchs endothelial dystrophy. However, in a subset of patients with Fuchs dystrophy who develop a laminated Descemet membrane without embedded guttae, the visual recovery after DSAEK is less than expected. The laminated architecture of Descemet membrane without embedded guttae may facilitate separation between the membrane layers and, thus, incomplete removal of the recipients Descemet membrane during DSAEK, which may then limit the postoperative visual outcome.

Ciliary Body Medulloepithelioma in a 10-Year-Old Boy

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Neurofibromatosis type 1: A neuro-psycho-cutaneous syndrome?

ABSTRACT Neurofibromatosis type 1 (NF1) is an inherited disorder often associated with optic nerve gliomas, low-grade brain tumors, and readily visible signs. Though these features are frequently emphasized, the psychosocial and emotional morbidities are often overlooked. We present a patient with depressive disorder resulting in suicide in a patient with NF1.

Melkersson-Rosenthal Syndrome Presenting as Isolated Eyelid Edema.

Originally received: August 15, 2016. Final revision: September 21, 2016. Accepted: September 28, 2016. Available online: November 15, 2016. Manuscript no. 2016-87. 1 Departments of Ophthalmology and Public Health Sciences, Penn State Eye Center, Penn State College of Medicine, Hershey, Pennsylvania. 2 The Emmes Corporation, Rockville, Maryland. 3 Doheny Eye Institute, University of California, Los Angeles, California. 4 University of Wisconsin Fundus Photograph Reading Center, Madison, Wisconsin. 5 Retinal Consultants of Arizona, Phoenix, Arizona. 6 USC Roski Eye Institute, Keck School of Medicine, University of Southern California, Los Angeles, California. Presented in part at the Annual Meeting of the Association for Research in Vision and Ophthalmology (ARVO), May 1e5, 2016, Seattle, Washington. Financial Disclosure(s): Supported by National Eye Institute (National Institutes of Health, Department of Health and Human Services) grants U10EY023529, U10EY023533, and U10EY023521. Support also provided in part by Regeneron, Inc., and Allergan, Inc., through donation of investigational drug. Author Contributions: Conception and design: Scott, VanVeldhuisen, Ip, Blodi, Oden, Figueroa Analysis and interpretation: Scott, VanVeldhuisen, Ip, Blodi, Oden, Figueroa, Dugel

Angiolymphoid Hyperplasia with Eosinophilia Accompanying Vernal Conjunctivitis

Financial Disclosure(s): The author(s) have made the following disclosure(s): R.E.H.: Grants e Novartis. I.S.Y.: Grant e European Union. P.T.dJ.: Travel support e European Commission. A.E.F.: Grant and travel support e European Commission. European Commission Vth Framework, Brussels, Belgium (Contract No. QLK6-CT-1999-02094). Additional funding for cameras was provided by the Macular Disease Society (Andover, UK). M.R.: Financed by the Ministry of Education and Science, Tallinn, Estonia (Target funding no. 01921112s02). Additional funding in Alicante was received from the Fondo de Investigacion Sanitaria (Madrid, Spain) (grant nos. FIS 01/1692E, RCESP C 03/09) and Oficina de Ciencia y Tecnologia Generalitat Valenciana (Valencia, Spain) (grant no. CTGCA/2002/06). The Thomas Pocklington Trust funded conversion of dietary data to nutrients. Author Contributions: Conception and design: Hogg, Woodside, Fletcher Data collection: Hogg, Woodside, McGrath, Young, Vioque, Chakravarthy, de Jong, Rahu, Seland, Soubrane, Tomazzoli, Topouzis, Fletcher Analysis and interpretation: Hogg, Woodside, McGrath, Fletcher Obtained funding: Not applicable Overall responsibility: Hogg, Woodside, McGrath, Young, Vioque, Chakravarthy, de Jong, Rahu, Seland, Soubrane, Tomazzoli, Topouzis, Fletcher Abbreviations and Acronyms: AMD 1⁄4 age-related macular degeneration; AREDS 1⁄4 Age-Related Eye Disease Study; CAREDS 1⁄4 Carotenoids in Age-Related Eye Disease Study; EPIC 1⁄4 European Prospective Investigation into Cancer and Nutrition; EUREYE 1⁄4 European Eye; FFQ 1⁄4 food-frequency questionnaire; GA 1⁄4 geographic atrophy; MDS 1⁄4 Mediterranean Diet Score; nvAMD 1⁄4 neovascular age-related macular degeneration. Correspondence: Jayne V. Woodside, PhD, Centre for Public Health, School of Medicine, Dentistry and Biomedical Sciences, Queen’s University Belfast, Grosvenor Road, Belfast, County Antrim, Northern Ireland BT12 6BA, UK. E-mail: j.woodside@qub.ac.uk.

Bilateral Primary Mucinous Carcinoma of the Eyelid.

The aim of this study is to report a case of bilateral primary mucinous carcinoma of the eyelids. This is a case report and literature review. A 71-year-old female presented with primary mucinous carcinoma of the left upper eyelid, which was excised with Mohs surgery. One year later, she developed primary mucinous carcinoma of the right upper eyelid, which was also treated Mohs surgery. Extensive workup was negative for evidence of an unknown primary carcinoma or metastasis. Primary mucinous carcinoma of the eyelids may occur as multifocal tumors, and bilateral disease is not necessarily indicative of metastatic disease.