MEN 2B masquerading as chronic blepharitis and euryblepharon.

This is a retrospective case description of a single male patient found to have multiple endocrine neoplasia syndrome type 2B (MEN 2B). At the age of 14, he presented for evaluation of ocular irritation and eyelid thickening. Intraoperative assessment revealed mucosal lesions on the anterior dorsum of his tongue and biopsy of multiple eyelid lesions disclosed bilateral submucosal neuromas, leading to the diagnosis of MEN 2B. MEN 2B is a potentially life-threatening syndrome due to the risk of developing medullary thyroid carcinoma (MTC) and pheochromocytoma. The patient was found to have MTC requiring thyroidectomy. We hope to highlight the clinical and histopathologic findings of MEN 2B in an effort to emphasize the importance of its identification in the absence of a family history.

Dacryoadenitis due to gonococcal conjunctivitis complicated by corneal perforation.

Adnexal and periocular involvement in Neisseria gonorrhoeae (NG) infection is rare. This report describes the case of a patient with a delayed diagnosis of gonococcal dacryoadenitis with contiguous conjunctivitis and corneal involvement. She underwent extensive inpatient laboratory and infectious workup but rapidly progressed to corneal perforation requiring emergent penetrating keratoplasty prior to a positive culture confirming the diagnosis. To date, this is the first reported case of ophthalmologic NG infection with associated conjunctivitis, dacryoadenitis, and corneal perforation.

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.

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.

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.

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.

Ocular Adnexal Reconstruction for Cutaneous Periocular Malignancies.

Although the majority of lesions present in the periocular region are benign, periocular cutaneous malignancies are certainly not uncommon and must be considered. The management of nonmelanoma cutaneous malignancies is predominately surgical with Mohs micrographic surgery or excision with frozen sections. The approach to reconstruction of the resulting defects depends on the defect location and size. When able, it is preferable to close lesions directly or with the recruitment of adjacent tissue in an effort to preserve the inherent anatomy. The eyelid's dynamic function is thus maintained, which is essential for optimal ocular surface protection. However, larger and more extensive defects will require complex reconstructions that are able to restore the necessary structural integrity to the eyelids. The authors review the various reconstruction approaches for defects of all sizes involving the periocular area including nonmarginal defects and defects of the lower and upper eyelids, as well as those of the medial canthal region.

Clinical Evaluation of Blepharoptosis: Distinguishing Age-Related Ptosis from Masquerade Conditions.

Aponeurotic ptosis accounts for the majority of acquired ptosis encountered in clinical practice. Other types of ptosis include traumatic, mechanical, neurogenic, and myogenic. In addition to true ptosis, some patients present with pseudoptosis caused by globe dystopia, globe asymmetry, ocular misalignment, or retraction of the contralateral lid. It is particularly important for the clinician to rule out neurologic causes of ptosis such as dysfunction of the third cranial nerve, Horner's syndrome, and myasthenia gravis, as these conditions can be associated with significant systemic morbidity and mortality. A thorough history and physical examination is necessary to evaluate each patient presenting with a complaint of ptosis. Correctly identifying the cause of the patient's complaint allows the ptosis surgeon to plan for appropriate surgical repair when indicated and to defer surgery when observation or additional clinical evaluation is warranted.

CENTRAL RETINAL VEIN OCCLUSION IN HEREDITARY SPHEROCYTOSIS.

PURPOSE: To investigate and identify hereditary spherocytosis as a possible risk factor for the development of a central retinal vein occlusion in a young, otherwise healthy, adult. METHOD: This study is a retrospective case description of a single female patient. RESULTS: A 31-year-old white woman with hereditary spherocytosis after splenectomy was found to have a central retinal vein occlusion. She had no atherosclerotic risk factors and a negative hypercoaguable workup. Her course was complicated by macular edema. She was treated with intravitreal ranibizumab injections with resultant improvement of her vision and resolution of her macular edema. CONCLUSION: Hereditary spherocytosis is an inherited deficiency in erythrocyte membrane proteins that should be considered in younger patients with central retinal vein occlusions because of the disease's impact on hematologic factors. Furthermore, intravitreal antivascular endothelial growth factor injections may improve macular edema in this population.