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where the more common simple lipoma is not frequently encountered. This patient’s case and this review of the literature suggest that appropriately aggressive surgical excision is recommended to reduce the risk of recurrence.

REFERENCES 1. Daniel CS, Beaconsfield M, Rose GE, et al. Pleomorphic lipoma of the orbit: a case series and review of literature. Ophthalmology 2003;110:101–5. 2. Bartley GB, Yeatts RP, Garrity JA, et al. Spindle cell lipoma of the orbit. Am J Ophthalmol 1985;100:605–9. 3. Kim MH, Sa HS, Woo K, et al. Fibrolipoma of the orbit. Ophthal Plast Reconstr Surg 2011;27:e16–8. 4. Shah NB, Chang WY, White VA, et al. Orbital lipoma: 2 cases and review of literature. Ophthal Plast Reconstr Surg 2007;23:202–5. 5. Bujalska IJ, Durrani OM, Abbott J, et al. Characterisation of 11beta-hydroxysteroid dehydrogenase 1 in human orbital adipose tissue: a comparison with subcutaneous and omental fat. J Endocrinol 2007;192:279–88. 6. Langenberg T, Kahana A, Wszalek JA, et al. The eye organizes neural crest cell migration. Dev Dyn 2008;237:1645–52. 7. Bowen JT. Multiple subcutaneous hemangiomas, together with multiple lipomas, occurring in enormous numbers in an otherwise healthy, muscular subject. Am J Med Sci. 1912;114:189–92. 8. Howard WR, Helwig EB. Angiolipoma. Arch Dermatol 1960;82:924–31. 9. Feinfield RE, Hesse RJ, Scharfenberg JC. Orbital angiolipoma. Arch Ophthalmol 1988;106:1093–5. 10. Arenaz Búa J, Luáces R, Lorenzo Franco F, et al. Angiolipoma in head and neck: report of two cases and review of the literature. Int J Oral Maxillofac Surg 2010;39:610–5. 11. Sah K, Kadam A, Sunita J, et al. Non-infiltrating angiolipoma of the upper lip: A rare entity. J Oral Maxillofac Pathol 2012;16:103–6. 12. Hoeft S, Luettges J, Werner JA. Infiltrating angiolipoma of the M. temporalis. Auris Nasus Larynx 2000;27:265–9. 13. Reibel JF, Greene WM. Liposarcoma arising in the pharynx nine years after fibrolipoma excision. Otolaryngol Head Neck Surg 1995;112:599–602.

Isolated Zygomycetes Endophthalmitis: A Case Report Juliet Idiga, B.S.*†, Daniel B. Rootman, M.D.†, Aaron Nagiel, M.D., Ph.D.†, and Robert A. Goldberg, M.D.† Abstract: Mucormycosis is a rare fungal infection typically limited to immunocompromised patients or patients with diabetes.1,2 Clinical manifestation varies, with rhino-orbitalcerebral mucormycosis as the most common presentation.3 The authors present here a 56-year-old man who complained of isolated OD pain with no evidence of cerebral or sinus inflammation on imaging. Enucleation was eventually performed, and histopathology of the globe demonstrated characteristics of zygomycetes infection. Intraocular mucormycosis is rare and when reported is related to contiguous spread of disease or surgical inoculation. After

*Department of Opthalmology, Columbia University, College of Physicians and Surgeons, New York, New York; and †Department of Opthalmology, Jules Stein Eye Institute, University of California, Los Angeles, California, U.S.A. Accepted for publication January 23, 2014. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Juliet Idiga, b.s., Columbia University, College of Physicians and Surgeons, New York, NY 10032. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000187

Case Reports

thorough literature review, the authors believe this to be the first reported case of isolated intraocular mucormycosis in a patient with no prior history of ocular surgery.

M

ucormycosis is a rare, yet life-threatening fungal infection caused by organisms belonging to the Zygomycete class of fungi.1 The fungal infection is typically limited to patients who are immunocompromised or have poorly controlled diabetes mellitus.1,2 Given its rarity, epidemiologic data regarding the disease burden have been difficult to assess. Nevertheless, an active population-based surveillance performed in the United States between 1992 and 1993 reported that mucormycosis developed in 1.7 persons per million-population year.3 The spectrum of mucormycosis varies and depends on its anatomical location. The 3 most common clinical manifestations include rhino-orbital-cerebral (39%), pulmonary (24%), and cutaneous (19%) mucormycosis.4 The typical pattern for spread is continuous, and because of angioinvasion, infection leads to extensive necrosis which is difficult to treat with systemic therapy. This tissue disruption in already compromised persons results in poor clinical outcomes overall, with mortality rates for orbital mucormycosis ranging up to 62%.5 Intraocular mucormycosis is rare and when reported is related to contiguous spread of disease or surgical inoculation.6,7 The authors report a case of a man with isolated intraocular Zygomycete endophthalmitis. This case report is HIPAA compliant as no names or other PHI are reported.

CASE REPORT A 56-year-old man with a medical history of type II diabetes mellitus presented to our institution with chronic mild OD pain. His ocular history was significant for proliferative diabetic retinopathy in OU, with neovascularization of the iris on the OD, but no history of intraocular surgery or trauma. On examination, his OD had no light perception vision, an intraocular pressure of 6, a shallow anterior chamber, and neovascularization of the iris. He was prescribed 1% atropine and Pred-Forte drops and discharged with instructions to follow up with the oculoplastics team to discuss the possibility of evisceration versus enucleation OD. One month after his initial presentation, the patient returned to the hospital due to worsening OD pain. He presented with a red painful eye, inflamed orbit, and an intraocular pressure of 60 mm Hg in that eye. He was treated with 500 mg Diamox (2×), Travoprost, cosopt, and brimonidine tartrate; however, the intraocular pressure remain elevated and the pain did not abate. Retrobulbar anesthesia followed by transscleral cyclophotocoagulation OD was then performed successfully with no complications. He was discharged on 250 mg Diamox per os 4 times a day. At follow up 2 days later, the patient was still complaining of worsening pain and an intraocular pressure of 55 mm Hg. At this time, an orbital process such as cellulitis or vascular fistula was considered and a CT scan of the orbits was performed (Fig. 1). This demonstrated diffuse inflammation focused anteriorly around a thickened sclera; however, the sinuses appeared normal with no thickening or fluid collection. Examination and nasal endoscopy by the ear, nose, and throat service revealed a grossly normal sinonasal mucosa with no evidence of invasive fungal sinusitis. Blood and urine culture showed no growth. Laboratory tests demonstrated blood glucose ranging between 200 and 500, and white blood cells were within normal limits. Given the severity of the

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Case Reports

FIG. 1.  Axial CT of the orbits. Note proptotic OD with anteriorly thickened sclera surrounded by diffuse inflammation. FIG. 3.  Axial MRI T1 with contrast status postenucleation demonstrating nonspecific diffuse right orbital inflammation.

posteriorly. These biopsies were negative for fungus on permanent section. Given an unremarkable workup and rising creatinine, amphotericin B was tapered off and the patient was discharged. He was doing well at his last follow up 3 months later.

DISCUSSION

FIG. 2.  Histologic section of the globe stained with periodic acid–Schiff stain illustrates fungi with nonseptate hyphae branched at right angles surrounded by polymorphonuclear leukocytes.

patient’s pain, the OD was enucleated with subsequent relief of pain, and the patient was discharged shortly after. Histopathology of the globe revealed intraocular zygomycetes with mixed inflammatory cells (predominantly polymorphonuclear leukocytes; Fig. 2). Microbiology performed on the vitreous specimen was negative for growth. The patient, though asymptomatic, was readmitted for treatment with intravenous amphotericin B. Repeat blood culture and laboratory test results were negative for an infectious process. MRI of the orbits, sinuses, and brain was significant for diffuse enhancement of orbital soft tissues consistent with nonspecific orbital inflammation (Fig. 3). Biopsy of the bilateral middle turbinates by the ear, nose, and throat service showed no evidence of zygomycetes. Random orbital biopsies of the orbit were performed in 4 quadrants both anteriorly and

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Although mucormycosis is rare, the zygomycete fungus is ubiquitous in nature and is often found in the soil or decaying matter.1,4 Rhino-orbital-cerebral mucormycosis occurs after inhalation of airborne spores in a typically immunocompromised host and takes hold in the paranasal sinuses.1 Orbital involvement is the result of direct extension of the fungus from the nasal cavity.1,6,7 Given the highly vasotropic nature of the fungus, infiltration and subsequent thrombosis of blood vessels is common and leads to necrosis of neighboring tissue.1 The patient presented with poorly controlled diabetes, orbital inflammatory features, and decreased motility: these can be features of orbital mucormycosis. However, the imaging and clinical picture was most consistent with endophthalmitis, and he was treated with enucleation surgery. The discovery of zygomycetes on histopathology was unexpected. Subsequent evaluation for any evidence of sinus or orbital mucormycosis was negative, including sinus and orbital biopsies. Thus, a diagnosis of mucormycosis endophthalmitis must be entertained. There is little in the literature regarding isolated intraocular mucormycosis. A case in 1951 described nonseptate fungi in the retina of an otherwise healthy boy suggestive of mucormycosis; however, this finding has since been disputed by others.4,8 Because zygomycetes are ubiquitous in the environment, contamination of the specimen should be suspected as a possible etiology. However, the presence of a pervasive inflammatory infiltrate on histopathology is suggestive of an active infection rather than contamination.9 Contaminated eye drops could also be a possible source of infection; however, such an occurrence is rare and is more likely to occur with the use of preservative-free drops. Moreover,

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fungal infection outside the globe would have been expected as well, which was not the case with this patient. Direct inoculation during administration of retrobulbar anesthetics is another possibility. This is also unlikely because accidental penetration into the globe would have been evident clinically during the injection and the subsequent procedure. Hematogenous spread of mucormycosis has been reported, suggesting a possible etiology for the patient.10 Dissemination most commonly originates from the lungs; however, the patient had no signs of pulmonary involvement.11 Moreover, dissemination is highly unlikely in the absence of significant immunosuppression or neutropenia.10 Finally, the patient’s multiple blood cultures did not grow zygomycetes, and there was no evidence of infection elsewhere in the body. A few cases of scleral perforation following transscleral cyclophotocoagulation have been reported in the literature.12,13 As such, introduction of the fungi via a microperforation following this procedure can be considered in the patient, although such an etiology would be quite rare. Direct extension through the conjunctiva or anterior sclera is another possibility. While we were not able to find evidence of scleral involvement in the tissue sample, sampling error could account for this. The available evidence in this case does not satisfactorily answer the question as to the mechanism of intraocular spread. It appears as though in very rare cases mucormycosis can present as an isolated endophthalmitis. As this is a highly morbid condition, we would further recommend an extensive local and systemic survey for infected tissue in such cases.

REFERENCES 1. Spellberg B, Edwards J Jr, Ibrahim A. Novel perspectives on mucormycosis: pathophysiology, presentation, and management. Clin Microbiol Rev 2005;18:556–69. 2. Chakrabarti A, Das A, Mandal J, et al. The rising trend of invasive zygomycosis in patients with uncontrolled diabetes mellitus. Med Mycol 2006;44:335–42. 3. Mucormycosis Statistics. Centers for Disease Control and Prevention. January 5, 2012. Available at: http://www.cdc.gov/ fungal/diseases/mucormycosis/statistics.html. Accessed July 26, 2013. 4. Schwartz JN, Donnelly EH, Klintworth GK. Ocular and orbital phycomycosis. Surv Ophthalmol 1977;22:3–28. 5. Roden MM, Zaoutis TE, Buchanan WL, et al. Epidemiology and outcome of zygomycosis: a review of 929 reported cases. Clin Infect Dis 2005;41:634–53. 6. Orgel IK, Cohen KL. Postoperative zygomycetes endophthalmitis. Ophthalmic Surg 1989;20:584–7. 7. Hosseini SM, Borghei P. Rhinocerebral mucormycosis: pathways of spread. Eur Arch Otorhinolaryngol 2005;262:932–8. 8. Wadsworth JA. Ocular mucormycosis. Report of a case. Am J Ophthalmol 1951;34:405–409. 9. Xu N, Lei X, Liu L. Tracking neutrophil intraluminal crawling, transendothelial migration and chemotaxis in tissue by intravital video microscopy. J Vis Exp 2011;e3296. 10. Ingram CW, Sennes J, Cooper JN, Perfect JR. Disseminated Zygomycosis: Report of Four Cases and Review. Rvw Infect Dis 1989;11:741–54. 11. Tomita T, Ho H, Allen M, et al. Zygomycosis involving lungs, heart and brain, superimposed on pulmonary edema. Pathol Int 2005;55:202–5. 12. Palmer DJ, Cohen J, Torczynski E, et al. Transscleral diode laser cyclophotocoagulation on autopsy eyes with abnormally thinned sclera. Ophthalmic Surg Lasers 1997;28:495–500. 13. Sabri K, Vernon SA. Scleral perforation following trans-scleral cyclodiode. Br J Ophthalmol 1999;83:502–3.

Case Reports

Orbital Involvement by NUT Midline Carcinoma Jill N. D’Souza, M.D.*, Gregory Notz, D.O.†, Ronald N. Bogdasarian, B.A.‡, David M. Cognetti, M.D.*, Joseph M. Curry, M.D.*, Marc R. Rosen, M.D.*, Madalina Tuluc, M.D., Ph.D.§, James J. Evans, M.D.║, and Jurij R. Bilyk, M.D.¶ Abstract: A 32-year-old female presented with a sino-orbital lesion that proved to be a NUT midline carcinoma. This is only the third case of orbital involvement by this aggressive lesion. The clinical, radiographic, and histopathologic features of NUT midline carcinoma are discussed, as well as its management options.

N

UT midline carcinoma (NMC) is a rare variant of squamous cell carcinoma (SCC) defined by a specific genetic abnormality: chromosomal translocation involving t(15;19).1,2 The tumor, by definition, often involves midline structures including the mediastinum, oropharynx, and paranasal sinuses. To date, 64 cases of NMC with NUT rearrangement have been reported, with only 2 cases involving the orbit.2,3 The authors present a sino-orbital case of NMC in a 32-year-old, 27-week gravid female. This report is Health Insurance Portability and Accountability Act complaint.

CASE REPORT A 32-year-old, 27-week gravid female presented to her family doctor with nasal congestion and rhinorrhea for 6 months. She denied fevers, purulent rhinorrhea, facial pain, or vision changes. She recalled episodes of epistaxis 6 weeks prior to presentation, along with increasing sinus pressure that did not improve with a course of oral ampicillin/clavulinic acid. Swelling around the right eye occurred 2 to 3 weeks prior to presentation. Her medical history was significant for hypothyroidism. There was no history of prior tobacco use. The patient was referred to an otolaryngologist. CT of the midface demonstrated a right nasal mass extending in the adjacent paranasal sinuses (Fig. 1). Extension in the orbit was present inferiorly and medially with tumor adjacent to the optic nerve, displacing or invading the intraconal contents. Biopsy of the intranasal mass showed a malignant epithelial neoplasm with squamous features showing direct juxtaposition of basaloid, immature and undifferentiated cells to more mature, almost “benign” looking squamous cells. A panel of immunohistochemistry stains showed positive staining for markers of squamous differentiation (high molecular weight keratin, p63, Ventana Medical Systems, Tucson, Arizona, U.S.A. ), for p16, focal (Ventana Medical Systems), and for NUT antibody (Cell Signaling Technology, Danvers, Massachusetts, U.S.A.). Histologic features corroborated with this immunoprofile established the diagnostic of NMC. CD34, a marker positive in some NUT midline carcinomas, was negative. She was referred for further management. Initial ophthalmic examination *Department of Otolaryngology/Head Neck Surgery, Thomas Jefferson University Hospital, Philadelphia; †Department of Ophthalmology, Geisinger Health System, Danville; ‡The Commonwealth Medical College, Scranton; §Department of Pathology; ║Department of Neurosurgery, Thomas Jefferson University Hospital; and ¶Skull Base Division, Neuro-Ophthalmology Service, Wills Eye Hospital, Philadelphia, Pennsylvania, U.S.A. Accepted for publication March 4, 2014. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Jurij R. Bilyk, m.d., Wills Eye Institute, 840 Walnut Street, Philadelphia, PA 19107. E-mail: jrbilyk@ aol.com DOI: 10.1097/IOP.0000000000000179

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Isolated Zygomycetes Endophthalmitis: A Case Report.

Mucormycosis is a rare fungal infection typically limited to immunocompromised patients or patients with diabetes., Clinical manifestation varies, wit...
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