Periocular Microcystic Adnexal Carcinoma: A Case Report and a Major Review.

PURPOSE: To describe a patient with periocular microcystic adnexal carcinoma (MAC) and to review the clinical presentation, systemic work-up, histopathologic features, and outcome of all previously reported periocular MAC. METHODS: A major literature review. PubMed/MEDLINE and Google Scholar databases were searched for all well-documented cases of periocular MAC. RESULTS: The final analysis yielded 93 patients with MAC, 48 (52%) females, 39 (42%) males, and 6 with sex not specified (6%) with an average age of 56 years (range 3 days-95 years). Most tumors were localized to the eyebrow (26/93, 28%) and lower eyelid (20/93, 22%). Of patients with known information, MAC most commonly presented as a nodule (37/68, 54%) or plaque (20/68, 29%) with poorly-defined margins (20/51, 39%) and distortion of eyelid margin (13/51, 25%). Orbital involvement at any point of the disease course was seen in 20 of 93 (22%) patients. An accurate histopathologic diagnosis on initial biopsy was made in 25 of 70 (36%) cases. Initial management included surgical excision (47/93, 51%), Mohs micrographic surgery (17/93, 18%), and excision with frozen section control of margins (8/93, 9%). Aggressive or recurrent MAC was managed with multimodal therapies, including adjuvant radiation (10/34, 29%). The average follow-up after the last treatment was 3 years (median 2, range 0.2-20 years). In total, 33 of 86 (38%) tumors recurred, and 6 of 87 (7%) metastasized. Disease-related mortality occurred in 3 of 79 (4%) of patients. CONCLUSIONS: Periocular MAC is frequently misdiagnosed on initial biopsy and has a tendency for recurrence and locally aggressive behavior, highlighting the importance of accurate timely diagnosis, and appropriate management.

Management of Orbital Masses: Outcomes of Endoscopic and Combined Approaches With No Orbital Reconstruction.

INTRODUCTION: The endoscopic endonasal approach to management of orbital pathology has expanded. Due to the rarity of these conditions, most reports in the literature consist of small case reports. We report a series from a single institution with a focus on outcomes. METHODS: A retrospective chart review was carried out between 2010 and 2018. RESULTS: Twenty-four patients were identified (average age 58 years, 15 males, 9 females). Average follow-up was 14.9 months. Most common etiologies included cavernous hemangioma (7), metastases (6), idiopathic orbital inflammatory syndrome (6), orbital hematoma/clot (2), and schwannoma (1). Most common presenting symptoms were decreased visual acuity (8), proptosis (8), diplopia (7), and incidental findings (2). All patients underwent endoscopic medial wall orbital decompressions. Sixteen involved a combined open approach by an ophthalmologist. Pathology was either biopsied (15), resected (6), or could not be identified (3). No intraoperative complications were noted. No patients underwent orbital reconstruction of the medial wall. Six patients developed postoperative sinusitis successfully managed with antibiotics. One patient developed epistaxis managed conservatively. In 5 patients, Sino-Nasal Outcome Test-22 scores increased immediately postop and then decreased, whereas scores only decreased in 6 patients. Six patients noted reduced proptosis. There were no new cases of diplopia or worsening visual acuity. CONCLUSIONS: A combined endoscopic endonasal and external approach can be useful for managing orbital lesions. Patients tolerated the procedure well with improvement in ocular symptoms and minimal sinonasal complications. Reconstruction of the medial wall may not be warranted to prevent postoperative diplopia.

Combined Endoscopic and External Resection of an Inverted Papilloma of the Lacrimal Sac: A Case Report and Review of the Literature.

BACKGROUND: Inverted papillomas (IPs) are benign, locally aggressive neoplasms with a high recurrence rate that most commonly arise from the lateral nasal wall. Rarely, IP can originate from the lacrimal sac and/or nasolacrimal duct (NLD) system. A 58-year-old man presented with chronic epiphora and an enlarging mass inferior to his left medial canthal tendon (MCT) for 2 years. OBJECTIVE: The patients' clinical presentation and surgical management are described. A literature review on IP of the lacrimal sac and NLD system was conducted. METHODS: Case report. RESULTS: We present a case of an IP that arose primarily from the lacrimal sac and grew below the MCT rather than extending above it. This is one of few published case reports detailing the use of a combined endoscopic and external approach for the resection of an IP of the lacrimal sac. CONCLUSION: Combined endoscopic and external resection is a viable approach for complete removal of extensive IP of the lacrimal sac and NLD system. IP arising from the lacrimal sac may grow below the MCT or extend above it as previously reported.

Endoscopic Management of Lacrimal System Dysgenesis and Dacryocystoceles in Fraser Syndrome: A Case Report and Literature Review.

BACKGROUND: Fraser syndrome is an autosomal recessive disorder characterized primarily by syndactyly, cryptophthalmos, urinary tract anomalies, ambiguous genitalia, and laryngeal anomalies. A 28-year-old man with Fraser syndrome presented with cryptophthalmos, microphthalmia, lacrimal system dysgenesis, and chronic sinusitis. OBJECTIVE: The patients' clinical condition and surgical treatment are described. A literature review was conducted, and articles relevant to the case are presented. METHODS: Case report. RESULTS: To our knowledge, this is the first published case report of endonasal management of dacryocystoceles in a Fraser syndrome patient. The patient was treated via endoscopic endonasal marsupialization and drainage. CONCLUSION: Fraser syndrome patients may initially present to many different specialties as the spectrum of clinical manifestations is broad. Physicians treating these patients should take a collaborative approach to surgical and medical management.

Orbital T-cell lymphoma versus lupus panniculitits: a T-cell-mediated spectrum of orbital lymphoproliferative disease.

This is a case description of a single male patient found to have T-cell-mediated inflammation and lymphoproliferation of the orbit. Chronic T-cell-mediated inflammatory disease can pose a diagnostic challenge particularly in its differentiation from a neoplastic process. The histopathology in this case demonstrated features of both lupus erythematosus panniculitis and features of orbital T-cell lymphoma. While both are rare, lupus erythematosus panniculitis of the orbit is even more exceptional; this patient's indolent, chronic relapsing course distinguished itself from the typical aggression of orbital T-cell lymphoma. We believe this rare case may actually represent an example of a newly described disease spectrum that incorporates lupus erythematosus panniculitis as well as subcutaneous panniculitis-like T-cell lymphoma.

Basaloid adenocarcinoma of the lacrimal sac: A rare entity and approach to management.

Presented is a rare case of basaloid adenocarcinoma of the lacrimal sac. Low grade features of this malignancy permitted a conservative surgical approach to maximize functional and cosmetic outcomes. This is retrospective case description of a single 77-year-old-female patient who underwent a dacryocystorhinostomy with silicone tubes at an outside hospital for epiphora, but was referred to the Wills Eye Hospital Oculoplastics Department for a lacrimal sac biopsy showing low-grade carcinoma with basaloid features. She was treated with complete excision of the mass, common canaliculus and distal canaliculi, and the nasolacrimal duct. The associated maxillary, lacrimal, and ethmoidal bones were similarly excised. Frozen sections were sent of the surrounding periosteum, orbital fat, nasal and sinus mucosa, and soft tissues, and all were negative. Reconstruction of the lacrimal system was performed with silicone tube intubation and the defect was closed with large glabellar and mid-facial flaps. One year post-operatively she maintained a normal ocular examination, and her eyelids and silicone tubes were well positioned. Systemic evaluation remained unrevealing. Basaloid adenocarcinoma of the lacrimal sac is relatively rare, and we highlight the role of a more conservative surgical intervention given the high rates of survival and low incidence of progression to metastatic disease when treating this rare malignancy.

A case of concurrent silent sinus syndrome, thyroid eye disease, idiopathic orbital inflammatory syndrome, and dacryoadenitis.

This is a retrospective case description of a single female patient found to have concomitant bilateral silent sinus syndrome, bilateral thyroid eye disease, unilateral dacryoadenitis, and idiopathic orbital inflammation that presented as progressive unilateral right-sided proptosis. The spectrum of inflammatory orbital diseases can make discerning between different entities challenging, but more unique in this case was the simultaneous presentation with bilateral silent sinus syndrome, a rare entity in its own right. Identifying each of these concurrent disease processes is important to establishing a multidisciplinary treatment approach to address all the patient's orbital and peri-orbital pathology. We hope to highlight the clinical and radiographic findings unique to each of these entities and share our approach to treatment in this complex case.

Paranasal sinus lymphoma: Retrospective review with focus on clinical features, histopathology, prognosis, and relationship to systemic lymphoma.

BACKGROUND: Paranasal sinus lymphoma is a rare clinical entity. METHODS: We conducted a retrospective case series of 68 patients with biopsy-confirmed paranasal sinus lymphoma with attention on systemic disease association. RESULTS: Of 63 patients with paranasal sinus lymphoma, 35 (56%) had systemic involvement. Four patient groups were identified: (1) primary paranasal sinus lymphoma (44%); (2) systemic disease occurring concurrently with paranasal sinus lymphoma (25%); (3) paranasal sinus lymphoma with relapse of preexisting systemic lymphoma (22%); and (4) progression to systemic disease after primary paranasal sinus lymphoma (8%). Most of the patients with systemic disease were diagnosed at 50 + years and had positive smoking histories. There was a trend toward disease activity in the neighboring ocular location. For patients with preexisting systemic lymphoma, the mean time to paranasal sinus lymphoma was 65 months. When systemic lymphoma developed after localized paranasal sinus lymphoma, mean time to progression was 23 months. Diffuse large B cell lymphoma was the most common paranasal sinus lymphoma. CONCLUSION: There is a risk of systemic involvement during the disease course of paranasal sinus lymphoma. Biopsy is the preferred first management step and should precede debulking or mass resection in nonemergent cases. © 2017 Wiley Periodicals, Inc. Head Neck 39: 1065-1070, 2017.

Solitary Extramedullary Plasmacytoma of the Orbital Apex and Dura Masquerading as Idiopathic Inflammation.

This is a retrospective case description of a single female patient found to have a primary solitary extramedullary plasmacytoma of the orbital apex and temporal/parietal dura. These monoclonal plasma cell neoplasms account for fewer than 5% of plasma cell neoplasms. Although most commonly found in the head and neck, orbital and dural solitary extramedullary plasmacytomas are extremely uncommon. The authors present a case of an extramedullary plasmacytoma involving both of these structures in a middle-age otherwise healthy female to highlight this rare entity and elucidate current treatment modalities and future considerations.

Giant Ocular Horn Occurring in a 10-Year-Old Female.

Cutaneous horns uncommonly involve the periocular region. Involvement of the ocular surface is particularly rare. The authors present a patient who underwent a perinatal buccal mucosal graft for corneal perforation due to congenital corneal ectasia, most likely resulting from Peters anomaly. She developed a giant ocular horn 10 years later.

A rare case of orbital hodgkin lymphoma demonstrating therapeutic response to a novel systemic medication.

Orbital Hodgkin lymphoma is infrequent, representing less than 1% of adult orbital malignancies. It is typically seen in the setting of terminally advanced systemic disease. This case highlights orbital, head, and neck disease in a systemically stable individual treated with the novel anti-CD30 monoclonal antibody brentuximab vedotin.

Goat horn-induced intracranial emphysema and orbital injury.

This is a case description of a male patient found to have orbital and intracranial emphysema, specifically with air in his cavernous sinuses bilaterally following penetrating trauma to the medial orbit from a goat's horn. There were no orbital or skull base fractures. Although the presence of traumatic intracranial emphysema is not uncommon, it is typically the result of direct communication of the cranial vault with the paranasal sinuses in the setting of associated fracture or, alternatively, from direct penetration and inoculation. We present a rare case of orbital emphysema with traumatic intracranial emphysema without these previously described associations and postulate a mech``anism behind its development.

The Effectiveness of a Mobile Clinic in Improving Follow-up Eye Care for At-Risk Children.

PURPOSE: To investigate the potential of a mobile ophthalmic unit in the schoolyard to improve the follow-up rate for children who have failed an optometric in-school screening program. Previously, the optometric program made referrals to the center and only 53% of students attended the desired ophthalmology consultation. METHODS: This was a cohort study of students conducted in elementary school lots in socioeconomically disadvantaged communities. The mobile ophthalmic unit visited schools where students with parental consent who needed examination had been identified by an in-school optometric vision care program. RESULTS: A total of 132 students were referred by the optometric program, of whom 95 (72%) had complete signed consent forms. Eighty-two patients (62%, confidence interval: 54% to 70%) were successfully seen by the mobile unit. Compared to the historical rate of successful completion of ophthalmology consultation (53%), a statistically significant improvement in follow-up was noted (P = .036). On a Likert scale of 1 to 5, the mean school nurse satisfaction rating was 4.8. CONCLUSIONS: The findings demonstrate the potential impact of mobile eye clinics at schools in connecting children with ophthalmic care. [J Pediatr Ophthalmol Strabismus. 2016;53(6):344-348.].

Acute Charles Bonnet Syndrome following Hughes procedure.

A 69-year-old male experienced monocular formed visual hallucinations after occlusion of the right eye following resection of eyelid basal cell carcinoma and reconstruction with a Hughes procedure (tarsoconjunctival flap). His symptoms included recurrent, well-defined, organized, complex, formed images of small children playing in the snow. These visual phenomena occurred only in the occluded eye, began several hours after surgery, and recurred intermittently several times daily for 4 days, lasting several minutes with each occurrence. The patient retained insight into the false nature of the images throughout the duration of his symptoms, and the hallucinations resolved spontaneously while the flap was still in place. To our knowledge, this is the first reported case of Charles Bonnet Syndrome (CBS) following a Hughes procedure in a patient with normal visual acuity in the non-occluded fellow eye. Unlike other reported cases of acute onset CBS following transient monocular occlusion, hallucinations in the occluded eye remitted prior to restoration of vision in the occluded eye. Ophthalmologists should be aware of the potential for CBS following even transient monocular occlusion and should consider warning patients about its potential to occur.

Unilateral Prostaglandin-Associated Periorbitopathy: A Syndrome Involving Upper Eyelid Retraction Distinguishable From the Aging Sunken Eyelid.

PURPOSE: To study the effects of prostaglandin analogue drops on the eyelids and adnexa in unilaterally treated subjects with the intention of qualifying, quantifying, and categorizing the characteristics of prostaglandin-associated periorbitopathy (PAP). METHODS: Patients using prostaglandin analogue drops in only 1 eye for at least 1 year were evaluated by masked examiners. Orbital and eyelid measurements were obtained for each patient, and adnexal photographs were taken. PAP was divided into 3 grades based on the presence and severity of fat atrophy and the existence and depth of superior sulcus deformity. Statistical analysis was performed comparing data between treated and untreated eyes. RESULTS: Thirty-three patients meeting eligibility criteria were enrolled, with equal numbers of subjects using latanoprost, travoprost, and bimatoprost. Treated eyes had a statistically significant increase in lagophthalmos (0.62 mm, p < 0.001), superior sulcus deformity/PAP grade (0.72, p < 0.001), and eyelid redness (1.08, p < 0.001). Treated eyes had significantly greater marginal reflex distance 1 measurements (0.89 mm, p = 0.02), highest with bimatoprost and moderate PAP. Treated eyes had relatively greater enophthalmos than untreated eyes. Very few patients noticed or complained about eyelid changes. CONCLUSION: Prostaglandin analogue drops cause adnexal changes and orbital fat atrophy leading to eyelid redness, superior sulcus deformity, higher eyelid crease, and enophthalmos. In contrast to previous studies showing ptosis in PAP, relative upper eyelid retraction was seen in most of our treated eyes. Our novel PAP grading scale may help objectify and categorize this syndrome. Awareness of these signs is critical, as the eyelids and eyes may be affected even in the absence of patient recognition.

Endogenous Serratia marcescens endophthalmitis.

PURPOSE: The purpose of this study was to describe a rare case of endogenous endophthalmitis associated with dental disease secondary to Serratia marcescens in an HIV-negative individual. METHODS: Retrospective case report. RESULTS: A 50-year-old white man with a history of intravenous drug use presented with pain and decreased vision in his right eye. Slit-lamp examination showed a hazy cornea, hypopyon with fibrin in the anterior chamber, and elevated intraocular pressure. B-scan ultrasound showed vitritis and choroidal thickening. Computed tomography showed gingival inflammation and lucencies of several teeth. Blood and urine cultures were negative, and HIV testing was negative. Echocardiography was negative for vegetations. Intravitreal culture revealed S. marcescens. Despite intravitreal and systemic antibiotics, the patient's clinical situation rapidly deteriorated, and the eye was eviscerated. The patient underwent dental extraction and was subsequently discharged in stable condition. CONCLUSION: The first case of endogenous endophthalmitis secondary to S. marcescens in an otherwise healthy, HIV-negative, intravenous drug user in association with severe dental disease is reported. Serratia may be found in oral biofilm, and this mechanism should be considered in cases where other etiologies have been ruled out.

Contrast-enhanced ultrasound-guided Sentinel lymph node biopsy of the ocular conjunctiva.

OBJECTIVES/HYPOTHESIS: Sentinel lymph node biopsy (SLNB) has been utilized for cutaneous melanoma and other malignancies arising from the eye and ocular adnexa. Currently, SLNB requires blue dyes and/or radiopharmaceuticals; both of which have significant shortcomings. This study sought to evaluate the feasibility of SLNB with the use of real-time, contrast-enhanced ultrasound (CEUS) as an alternative technique for tumors arising in the conjunctiva. STUDY DESIGN: Prospective feasibility study in a porcine model. METHODS: Twelve experiments were performed on six non-tumor-bearing Yorkshire swine. An ultrasound contrast agent, Sonazoid (GE Healthcare, Oslo, Norway), (99m) technetium ((99m) Tc), and methylene blue (MB) (Covidien, Mansfield, MA) were injected in the ocular conjunctiva. Sentinel lymph nodes (SLNs) were localized with CEUS and findings were compared to that of MB and (99m) Tc. Fisher exact test was used. RESULTS: Contrast-enhanced SLNs were identified within an average of 6.2 minutes from time of injection of Sonazoid. A total of 17 SLNs were identified by at least one of the three techniques. Correlation between Sonazoid and (99m) Tc was 94.1% (16/17 SLNs). Correlation between (99m) Tc and MB was 88.2% (15/17). One SLN that was positive for (99m) Tc but negative for Sonazoid and was considered to be a false positive (1/17); findings were similar for MB (1/17). Differences between the three techniques were not significant (P = .886). CONCLUSIONS: CEUS-guided injection of conjunctiva for SLNB is technically feasible and correlates well with standard detection techniques. This technique shows promise for rapid, real-time, intraoperative imaging for SLNB, using a widely available imaging modality and avoiding the need for radiopharmaceuticals. LEVEL OF EVIDENCE: NA

The frequency of granulomatous lacrimal gland inflammation as a cause of lacrimal gland enlargement in patients without a diagnosis of systemic sarcoidosis.

PURPOSE: To determine the rates of orbital sarcoidosis in patients with clinical lacrimal gland enlargement and no history of sarcoidosis. METHODS: This was a retrospective study of patients from the Orbital Surgery Service at Wills Eye Institute who underwent lacrimal gland incisional biopsy from 1994-2009 for lacrimal gland enlargement. Patients were excluded if they had a history of sarcoidosis, other autoimmunities, or lacrimal gland enlargement thought to be malignant in etiology. Subject charts were reviewed for pathology results, as well as clinical and demographic data. Rates of sarcoidosis in a patient population presenting with isolated lacrimal gland enlargement were thereby analyzed. RESULTS: 75 patients fulfilling study criteria had lacrimal gland biopsies. 67 (89)% had benign lacrimal gland pathology and 8 (11)% had malignant lacrimal gland pathology. Sarcoidosis was found histologically in 15 (20%). The most frequent pathologic finding was non-specific chronic non-granulomatous inflammation, seen in 32 (43%) studies specimens. Age and gender were not statistically different between sarcoid positive and sarcoid negative patients. Patients with a positive biopsy were more likely to be African American. 26 (35%) of the studied patients had ACE levels obtained. The average ACE level was statistically greater in patients with sarcoidosis-positive biopsies than in those with negative biopsies. DISCUSSION: In patients with clinical lacrimal gland enlargement, incisional biopsy led to a diagnosis of sarcoidosis in 20% of patients. An elevated ACE level may help predict which patients have a greater diagnostic yield with biopsy.

Diesel fuel injury to the orbit.

A 48-year-old man was seen in the Wills Eye Institute emergency room for pain and decreased vision after having a diesel engine fuel cylinder explosion in his face. Clinical examination showed proptosis, decreased extraocular motility, and increased intraocular pressure; CT scan imaging revealed hypodense areas within the orbit. His orbital signs rapidly worsened despite aggressive antibiotics, steroids, and surgical intervention. Two orbitotomies revealed diesel fuel infiltration of the intraconal and extraconal spaces that proved impossible to evacuate completely. The patient ended up losing vision from an intense chemical cellulitis with subsequent orbital compartment syndrome and optic neuropathy. This case highlights the severity of orbital chemical injuries and their variable prognoses despite aggressive management.

Update on advanced imaging options for thyroid-associated orbitopathy.

Thyroid-associated orbitopathy (TAO) is a diverse spectrum of signs and symptoms that appears to have immunologic and pathologic causative factors as diverse as its clinical presentations. Lymphocytes, hormones, and cytokines affect orbital fibroblasts and other similar cells, which exert their effects on orbital tissues, including the extraocular muscles, orbital fat, and optic nerve. This complicated inflammatory cascade and the myriad of clinical findings that result contributes to the active phase of TAO. The distinction between the active and inactive phases of TAO is an important one, as the proper treatment will depend on the disease phase and degree thereof. Several clinical grading scales and scores have been established to help qualify and quantify the disease severity. Aiding clinical exam and acumen, proper and reproducible imaging of the orbit and ocular adnexa is incredibly important to the management of TAO. Orbital ultrasound, computed tomography, magnetic resonance imaging, and scintigraphy each have unique abilities, including quantifying orbital changes, assessing disease activity, correlating orbital findings with clinical changes, guiding appropriate treatment, and monitoring therapeutic responses. Further, study ease, accessibility, cost, sensitivity, specificity, reproducibility, and risks are all important considerations in picking the right test with which to diagnose and follow TAO. This analysis will provide a review of orbital imaging for TAO, including the mechanism of each imaging technique as well as their rationales, advantages, disadvantages, and utilities.