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Adult onset xanthogranulomatous disease is a rare orbital disease. IgG4-related disease is a systemic disease that can often manifest in the orbit. In this communication, we present the case of a patient with a xanthelasma-like lesion on the upper eyelid, and an enlarged lacrimal gland, which on biopsy was diagnosed as an orbital xanthogranuloma. Detailed serological workup showed that the patient was found to have elevated serum IgG4 levels. The orbital specimen was re-stained and found to be positive for IgG4. The patient was treated with oral steroids with partial resolution of the lesion. This is an interesting case of both histopathological adult onset xanthogranuloma (AOX) and IgG4-related orbital disease (IgG4-ROD). The early evidence suggests that the diagnosis of one of these disorders should point the physician to investigate for the presence of the other, especially if xanthogranulomatous disease is diagnosed first.

Alexander Pinhas,1,2 Jorge S Andrade Romo,1 Giselle Lynch,1,3 Davis B Zhou,1,3 Maria V Castanos Toral,1 Phillip A Tenzel,1 Oscar Otero-Marquez,1 Shoshana Yakubova,1,4 Alexander Barash,1 David Della Rocca,1 Robert Della Rocca,1 Toco YP Chui,1 Richard B Rosen,1 Harsha S Reddy1 1Department of Ophthalmology, New York Eye and Ear Infirmary of Mount Sinai, New York, NY, USA; 2Department of Ophthalmology, State University of New York Downstate Medical Center, Brooklyn, NY, USA; 3Department of Ophthalmology, Icahn School of Medicine at Mount Sinai, New York, NY, USA; 4Department of Biology, Macaulay Honors College at City University of New York Queens College, Flushing, NY, USACorrespondence: Harsha S Reddy, Department of Ophthalmology, New York Eye and Ear Infirmary of Mount Sinai, 310 E 14th St, New York, NY, 10003, USA, Tel +1 212-979-4284, Fax +1 212-966-6295, Email hreddy@nyee.eduPurpose: Hemodynamic changes surrounding the optic nerve head are known to occur in thyroid-related orbitopathy (TRO). This pilot study explores the capillary and non-capillary peripapillary perfusion changes of the retina in TRO eyes without dysthyroid optic neuropathy (DON) using optical coherence tomography angiography (OCT-A).Methods: Non-capillary and capillary peripapillary perfusion densities were calculated using single 4.5 × 4.5mm en face “RPC layer” OCT-A scans of 8 TRO patients without DON (8 eyes, mean age 40.6 years, range 23– 69 years). Results were compared to a previously published dataset of 133 healthy controls (133 eyes, mean 41.5 years, range 11– 83 years). The strength of association was measured between OCT-A perfusion densities and clinical measures of TRO.Results: Non-capillary peripapillary perfusion density in TRO eyes was found to be significantly decreased compared to healthy controls (TRO group 15.4 ± 2.9% vs controls 21.5 ± 3.1%; p < 0.0001). Capillary peripapillary perfusion densities showed no significant difference (TRO group 42.5 ± 1.8% vs controls 42.5 ± 1.5%; p = 1.0). Clinical measures of disease did not correlate well with OCT-A perfusion densities (p> 0.05).Conclusion: These findings may represent decreased blood flow and subclinical ischemia to the optic nerve. We discuss possible pathogenic mechanisms of thyroid-related vasculopathy, including vessel wall thickening due to immunologically-induced media enlargement.Keywords: thyroid-related orbitopathy, optical coherence tomography angiography, peripapillary microvasculature, thyroid-related vasculopathy

Purpose To present a unique case of streptococcus dysgalactiae keratitis with progression to corneal perforation and endophthalmitis, in the setting of epidermal growth factor receptor inhibitor (erlotinib) therapy for advanced non-small cell lung cancer. Observations An 89-year-old female with non-small cell lung cancer on erlotinib presented with corneal perforation due to infectious keratitis. Microbial cultures grew streptococcus dysgalactiae, a virulent pathogen known to affect immunocompromised patients that has not been previously described to cause infectious keratitis. Despite aggressive medical intervention, the clinical course was complicated by rapid progression to no light perception visual acuity in the setting of endophthalmitis with orbital cellulitis, necessitating evisceration. Conclusions and Importance Epidermal growth factor receptor inhibitor therapy can result in significant ocular complications including dry eyes, epithelial keratopathy, non-healing abrasions, infectious keratitis, and rarely, corneal melting and perforation. These side effects can predispose patients to aggressive infections with rare organisms, highlighting the importance of understanding the ocular side effects of systemic chemotherapeutic agents.

The purpose of this study was to evaluate the long-term outcome and patterns of failure in patients treated with primary radiotherapy (RT) for orbital lymphoma (OL). Seventy-nine patients diagnosed with stage IE OL between 1995 and 2012 were included. Fifty-nine patients (75%) had mucosa-associated lymphoid tissue lymphoma and 20 patients (25%) had follicular lymphoma subtype. The median follow-up was 49.7 months. Major tumor sites were conjunctiva (29%), orbit (47%) and lacrimal gland (24%). After treatment to a median dose of 30.6 Gy, there were a total of no local, one contralateral orbital, two regional and two distant recurrences, all outside of the treatment fields. The 10-year local relapse-free, distant metastasis-free and overall survival rates were 100%, 94.2% and 98.2%, respectively. Definitive RT to 30 Gy was shown to be highly effective for indolent OL, and this study represents one of the largest single-institution studies using primary RT for stage IE OL.

Objective: To study the utility of a commercially available small, portable ultra-high definition (HD) camera (GoPro Hero 4) for intraoperative recording. Methods: A head mount was used to fix the camera on the operating surgeon's head. Due care was taken to protect the patient's identity. The recorded video was subsequently edited and used as a teaching tool. This retrospective, noncomparative study was conducted at three tertiary eye care centers. The surgeries recorded were ptosis correction, ectropion correction, dacryocystorhinostomy, angular dermoid excision, enucleation, blepharoplasty and lid tear repair surgery (one each). The recorded videos were reviewed, edited, and checked for clarity, resolution, and reproducibility. Results: The recorded videos were found to be high quality, which allowed for zooming and visualization of the surgical anatomy clearly. Minimal distortion is a drawback that can be effectively addressed during postproduction. The camera, owing to its lightweight and small size, can be mounted on the surgeon's head, thus offering a unique surgeon point-of-view. In our experience, the results were of good quality and reproducible. Conclusions: A head-mounted ultra-HD video recording system is a cheap, high quality, and unobtrusive technique to record surgery and can be a useful teaching tool in external facial and ophthalmic plastic surgery.

To evaluate the prognostic utility of the American Joint Committee on Cancer (AJCC) staging system for ocular adnexal lymphoma (OAL). A multicenter, consecutive case series of patients with biopsy-proven conjunctival, orbit, eyelid, or lacrimal gland/sac lymphoma was performed. The electronic pathology and clinical records were reviewed for new or recurrent cases of ocular adnexal lymphoma. The main outcome measures included pathology and clinical staging (AJCC and Ann Arbor systems), treatment, and recurrence (local and systemic). Statistical analysis included demographic evaluations and the Kaplan-Meier survival probability method. Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue were the most common (n=60/83, 72%). The most common Ann Arbor clinical stages were IE (76%) followed by IIE (17%) and IIIE (7%). Pathology identified 13 cases (15%) that were upstaged to group IV (p=0.017). Similarly, AJCC clinical stages were cT1NOMO (21.7%), cT2NOMO (44.6%), cT3N0M0 (5%), and cT4NOMO (2.4%). Local control was achieved in 75% of treated patients. There were 19 local recurrences from which 14 (74%) belonged to the non-radiation treatment groups. Lower-risk groups (T1 and T2 without lymph node involvement or metastatic disease of AJCC and IE of Ann Arbor) had longer disease-free survival than the higher-risk groups (AJCC T1, T2 with nodal involvement or metastatic disease, T3, and T4 as well as Ann Arbor II, III, and IV). The overall mean follow-up was 43.3 months (range 6-274). Regardless of stage, recurrence and disease-free survival were more closely related to treatment and histopathology rather than tumor size or site-specific location.

Purpose Trauma to the pupillary fibers can occur during repair of orbital floor fracture resulting in a fixed and dilated pupil. The authors report, discuss, and propose an etiology of an abnormal pupil in a child with an orbital floor fracture before surgical repair. Methods A case report is described. Results Although the orbital fracture was repaired, anisocoria persisted 18 months after the initial trauma. Conclusions Pupillary function must be assessed before surgical repair of an orbital floor fracture as damage of the pupillary fibers can occur secondary to the orbital floor fracture.

Objective To evaluate the efficacy and safety of a combined endoscopic and transconjunctival orbital decompression in patients with thyroid-related orbitopathy with orbital apex compression. Study Design Retrospective review. Methods A sequential series of patients with thyroid-related orbitopathy presenting with orbital apex compressive myopathy with and without optic neuropathy who were undergoing combined endoscopic and transconjunctival decompression by the same surgeons from 1992 to 2001 was reviewed. Patients were regularly evaluated preoperatively and postoperatively over a 3- to 55-month period to record the effects of this approach on visual acuity, Hertel exophthalmometry, and diplopia. Complications and secondary ophthalmological procedures were reviewed. Results Between 1992 and 2001, 72 combined endoscopic and transconjunctival decompressions were performed on 41 patients with orbital apex compression. Visual acuity improved in 89.3% of the patients with compressive optic neuropathy (P

A rare variant of adenoid cystic carcinoma is the dedifferentiated sarcomatoid form, which has previously been reported in the hard and soft palate, maxillary sinus, submandibular glands, and nasal cavity. The authors report the first case of a dedifferentiated sarcomatoid adenoid cystic carcinoma occurring in the lacrimal gland, that of a 52-year-old man. The patient presented with a 4-month history of diplopia, decreased vision, and right upper eyelid swelling. Radiographic imaging showed a soft tissue mass in the extraconal compartment superolateral to the right eye. The patient subsequently underwent surgical debulking. Histologic examination of the tissue revealed classic cribiform adenoid cystic carcinoma and a sarcomatous component consisting of malignant spindle cells and fusiform cells arranged in whorls. Dedifferentiation is a well-established phenomenon in salivary gland tumors that is associated with aggressive behavior and poor prognosis; however, the exact nature of such dedifferentiated neoplasms remains unclear.

Objective To report our experience with 200 cases of basal cell carcinoma (BCC) in 192 patients treated with an enhanced frozen-section control (FSC) technique using stereoscopic microdissection of surgical margins. Methods Retrospective series of 192 patients with 200 lesions diagnosed as BCC of the periocular region. All were excised en bloc with 1-mm margins beyond the clinically apparent tumor and examined using an enhanced FSC technique with stereoscopic microdissection of the surgical margins. Results Of 200 malignant BCCs, 93.0% represented primary tumors. The overall recurrence rate was 1.0%, with a mean follow-up of 4 years. In patients with primary lesions, the overall recurrence rate was 1.1%, with a mean follow-up of 3.9 years. There were no recurrences in the secondary tumor group after a mean follow-up of 4.8 years. Of the 200 lesions, 66.0% lesions required a single en bloc resection to achieve tumor-free margins. Conclusions An enhanced FSC technique using stereoscopic microdissection of the surgical margins permits greater conservation of healthy tissue and yields cure rates comparable to those of the standard FSC technique and Mohs micrographic surgery. We believe that this enhanced FSC technique is a highly effective method for resection of periocular BCC.

Injury and disruption of the canaliculi of the lacrimal excretory system commonly occur from laceration or shearing trauma. This type of injury will likely lead to dysfunction of tear flow from the palpebral sulcus and chronic epiphora. The goal of this article is to give a step-by-step review of canalicular repair.

The lateral tarsal strip is a mainstay procedure of oculoplastic surgery. The technique is easy to conceive but difficult to master. Awareness of its many indications and its limitations along with a working knowledge of its proper execution is necessary.

Lacrimal sac tumor is a rare tumor, for which we do not know the exact incidence. Malignant tumors account for 70% of all cases. Epithelial tumors are the most common pathological type and squamous cell carcinoma is the most common of these, a transitional cell carcinoma is rare and has a poor prognosis. The authors report a rare case of transitional cell carcinoma of the lacrimal sac. A 57-year-old woman had tearing on her right eye. The pre-operative diagnosis was possible nasolacrimal duct obstruction with signs of dacryocele. Intraoperatively the authors found a lacrimal mass and a frozen section showed squamous papilloma with dysplasia. The tumor was completely removed and DCR was done. The subsequent pathological report of the right lacrimal sac was papillary transitional cell carcinoma, so medial maxillectomy and resection of the medial inferior orbit with ethmoidectomy were performed. She received radiation and has been tumor-free for 2 years.