Concomitant adult onset xanthogranuloma and IgG4-related orbital disease: a rare occurrence.

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.

Abnormal Pupil Finding After an Orbital Floor Fracture in a Child.

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.

The medial transorbital approach in cranioendoscopic skull base tumor resections for locally advanced tumors.

BACKGROUND: Orbital structure preservation and avoidance of facial incisions without compromising oncological outcome are key to maintaining function and quality of life in locally advanced sinonasal tumor surgery. A transorbital approach at our institution has proven invaluable during cranioendoscopic skull base tumor resections and there are few descriptions of this in the literature. METHODS: An IRB-approved retrospective chart review was conducted at a tertiary cancer center for patients between 2020 and 2022 undergoing cranioendoscopic tumor resections utilizing a transorbital approach. Data collected included histopathology, sinus origin, disease extent, stage, operative details, length of stay, neo-adjuvant treatment and adjuvant treatment. Recurrence, survival, and complication rates were assessed. RESULTS: Four patients were identified for inclusion, including a SMARCB1-deficient carcinoma, esthesioneuroblastoma, squamous cell carcinoma and meningioma. All patients had resection of gross and microscopic disease with preservation of orbital contents. Post-operatively, one patient had mild diplopia on inferior gaze, all other patients had normal vision. Median follow-up was 9.5 months. One patient had recurrence of disease intracranially. CONCLUSIONS: The cranioendoscopic approach with a medial transorbital incision has multiple benefits. It avoids the need for a Weber-Ferguson incision with associated facial scar, allows for early intra-operative assessment for orbital invasion using tactile feedback and safe dissection of disease while protecting the globe and rectus muscles. This leads to preservation of eye function while ensuring an oncological resection. Other advantages include ligation of the anterior ethmoid artery and access for reconstruction of the medial orbital wall.

A Pilot Study of Subclinical Non-Capillary Peripapillary Perfusion Changes in Thyroid-Related Orbitopathy Detected Using Optical Coherence Tomography Angiography.

Purpose: 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.

Dedifferentiated adenoid cystic carcinoma of the lacrimal gland.

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.

Rapidly progressive streptococcus dysgalactiae corneal ulceration associated with erlotinib use in stage IV lung cancer.

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.

Transitional cell carcinoma of the lacrimal sac.

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.

Surgeon point-of-view recording: Using a high-definition head-mounted video camera in the operating room.

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.

Long-term outcomes and patterns of failure in orbital lymphoma treated with primary radiotherapy.

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.

Endoscopic and transconjunctival orbital decompression for thyroid-related orbital apex compression.

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 <.0005) and in 34.1% of those without neuropathy. Proptosis was reduced by 3.65 mm, on average. There was one case of transient intraoperative cerebrospinal fluid extravasation at the site of the optic nerve decompression, and one patient developed epistaxis. CONCLUSIONS: The study supports the treatment of thyroid-related orbital apex compression with and without compressive optic neuropathy by a combined transconjunctival and endoscopic approach. This approach offers short hospital stays, excellent visual recovery, and minimal complications in patients with thyroid-related orbital apex compressive myopathy and related compressive optic neuropathy. The beneficial effects observed in the patients with visual loss continued to improve over time and were significant (P <.001).

Ocular adnexal lymphoma staging and treatment: American Joint Committee on Cancer versus Ann Arbor.

PURPOSE: To evaluate the prognostic utility of the American Joint Committee on Cancer (AJCC) staging system for ocular adnexal lymphoma (OAL).
 METHODS: 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.
 RESULTS: 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).
 CONCLUSIONS: Regardless of stage, recurrence and disease-free survival were more closely related to treatment and histopathology rather than tumor size or site-specific location.

Mucin-producing sweat gland carcinoma of the eyelid: diagnostic and prognostic considerations.

PURPOSE: To describe the clinical and pathologic characteristics of mucin-producing sweat gland carcinoma of the eyelid and to determine whether neuroendocrine differentiation is of prognostic significance. DESIGN: Retrospective interventional case series. METHODS: Search of the New York Eye and Ear Infirmary pathology database between 1990 and 2011 identified 16 patients with mucin-producing sweat gland carcinoma. Clinical, histopathologic, and immunohistochemical analyses were performed on all identified cases. RESULTS: The patients presented with vascularized, focally cystic, nonulcerated eyelid margin lesions. Histopathologic evaluation showed that 4 lesions (25%) had a cystic, papillary, and solid growth pattern with an in situ component, 7 (44%) were pure invasive mucinous carcinomas, and 5 (31%) demonstrated both growth patterns. Immunohistochemical analysis of 15 tumors showed that pure cystic/papillary lesions had a significantly greater percentage of synaptophysin-immunoreactive cells (P = .036). There was no significant difference in the number of neuroendocrine markers expressed or in the intensity of immunostaining among the 3 different growth patterns. Re-excision for margin clearance was performed in 8 of 13 cases (61.5%). Two of 13 lesions recurred (15%); 1 of these was an in situ tumor with cystic morphology and neuroendocrine differentiation and the other was pure invasive mucinous carcinoma. None of the lesions metastasized. CONCLUSIONS: Mucin-producing sweat gland carcinoma pathologically represents a continuum, from an in situ lesion to a classic, invasive mucinous carcinoma. Immunohistochemical evidence of neuroendocrine differentiation can be observed in all lesions and does not appear to have a prognostic significance, arguing against the utility of immunohistochemical subtyping of mucinous sweat gland carcinomas.

Excision of periocular basal cell carcinoma with stereoscopic microdissection of surgical margins for frozen-section control: report of 200 cases.

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.

The lateral tarsal strip: illustrated pearls.

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.

Direct repair of canalicular lacerations.

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.