Faculty Bibliography

Inflammatory orbital lesions include a broad list of diagnoses, many of them with overlapping clinical and radiographic features. They often present a diagnostic conundrum, even to the most experienced orbital specialist, thus placing considerable weight on surgical biopsy and histopathological analysis. However, histopathological diagnosis is also inherently challenging due to the rarity of these lesions and the overlaps in histologic appearance among distinct disease entities. We herein present the case of an adolescent male with a subacutely progressive orbital mass that generated a significant diagnostic dilemma. Early orbital biopsy was consistent with a benign fibro-inflammatory lesion, but corticosteroid therapy was ineffective in halting disease progression. After an initial substantial surgical debulking, histopathological analysis revealed several key features consistent with IgG4-related disease (IgG4-RD), a systemic fibro-inflammatory process typically accompanied by multifocal tumor-like lesions. Surprisingly, within months, there was clear evidence of clinical and radiographic disease progression despite second-line rituximab treatment, prompting a second surgical debulking. This final specimen displayed distinctive features of Rosai-Dorfman disease (RDD), a systemic inflammatory disease characterized by uncontrolled histiocytic proliferation. Interestingly, certain features of this re-excision specimen were still reminiscent of IgG4-RD, which not only reflects the difficulty in differentiating RDD from IgG4-RD in select cases, but also illustrates that these diagnoses may exist along a spectrum that likely reflects a common underlying pathogenetic mechanism. This case emphasizes the importance of surgical biopsy or resection and histopathological analysis in diagnosing-and, ultimately, treating-rare, systemic inflammatory diseases involving the orbit, and, furthermore, highlights the shared histopathological features between RDD and IgG4-RD.

A 72-year-old woman who presented with right-sided epiphora and conjunctivitis underwent a probing and irrigation procedure with normal results. She improved with antibiotic-steroid drops. A swelling in the medial canthal region completely resolved. One year later, she returned with symptoms of dacryocystitis. An external dacryocystorhinostomy was performed. Characteristic dacryoliths were removed from the sac lumen, and biopsy of the sac wall showed spicules of lamellar bone within a fibrous stroma. Diagnosed as fibrous dysplasia of the lacrimal sac, this rare entity represents the second such case in the literature.

Purpose : To describe clinical features requiring urgent surgical intervention for superior orbital blow-in fractures with and without globe compression. Methods : Retrospective, consecutive case series between 6/1/2018 and 12/1/2019. Results : Six patients (6 eyes) with superior orbital blow-in fractures were identified, 50% with bone fragments compressing the globe. The mean age of presentation was 31.8 +/- 10.5 years. The majority of patients were male (83%), involved the left eye (83%), and were assault victims with a swinging object (50%). All patients had periorbital edema and a brow laceration. No patients had clinical evidence of a ruptured globe. The visual acuity of the affected eye ranged from 20/20 to 20/30. Post-operative visual acuity worsened in one case (16%) due to vitreous hemorrhage. Surgical approaches included lateral orbitotomy through the brow laceration (66%), through an eyelid crease incision (17%) and a craniotomy (17%). The most common fracture types on computed tomography included communicated fractures of the frontal sinus to the superior orbital rim (83%), involvement of the zygomatic bone (33%) and an inferiorly displaced superior orbital rim (17%). Additional orbital injuries included left rectus hematoma (16%), bone fragment abutting the lacrimal gland (50%) and intraconal hematoma (33%). An intracranial injury was seen in 33% of patients, including a parieto-occipital hematoma and frontal white matter contusion. Fifty percent of patients had bone fragments directly compressing the globe with deformation of the globe contour, which was visible on fundoscopic examination and bedside ocular ultrasound. Distinguishing features of orbital blow-in fractures with globe compression compared to those without included supra-and abduction motility deficits, proptosis, globe dystopia and abnormal posterior segment exam, which were seen in all cases. Posterior segment findings included choroidal folds (100%), choroidal rupture (33%), commotio (33%) and vitreous hemorrhage (33%), however there were no retinal tears or detachments. Conclusions : In cases of periocular trauma, the presence of exophthalmos, globe dystopia, ocular motility deficits, and choroidal folds may suggest an orbital blow in fracture with globe compression. These findings should prompt expedited imaging and surgical intervention when appropriate

A 37-year-old woman underwent excision of a congenital melanocytic nevus of the right lower eyelid margin for cosmetic reasons. She suffered from a severe dry eye syndrome which was partially alleviated with various topical eye drops. Histopathology of the excised tissue displayed flecks of calcium that were present within the immediate subepithelial conjunctival fibrous tissue but not the nevocytes or the cutaneous surface. Unlike calcinosis cutis, wherein calcium is deposited within the cutaneous dermis, calcification of the conjunctival substantia propria is rare and may be related to the dry eye state.

A flesh-colored, supraciliary lesion of the left upper eyelid in a 47-year-old man was excised for cosmetic reasons. Histopathology and immunohistochemistry demonstrated CD34-positive benign spindle cells, factor XIIIa-positive dendritic cells, and CD163-positive histiocytes, all dispersed within a diffuse collagenous background. Prominent loose perivascular cuffs of fibroblastic cells and collagen surrounded small blood vessels. Interpreted as an angiofibroma, the histopathology bore resemblance to that of a single previously-reported forearm lesion termed a "dermal fibroma with a distinctive perivascular cell arrangement." The lesion represents the first eyelid example of an unusual variant of angiofibroma.

A yellow cystic lesion of the caruncle in a 23-year-old woman proved to be a solitary steatocystoma, a rare occurrence in that location. While the histopathologic diagnosis was evident from clusters of sebaceous cells within the cyst wall, a panel of immunohistochemical stains further distinguished the lesion from a keratinous cyst. The most useful stains for differentiating the two conditions were carcinoembryonic antigen, epithelial membrane antigen, cytokeratins 17 and 19, and calretinin. Only three previous cases of caruncular steatocystoma simplex have been reported, none of which included immunohistochemical studies. The current findings support the origin of the cyst from the small duct that connects the unilobular sebaceous gland associated with vellus hairs to the follicular canal.

A 74-year-old man presenting with proptosis and orbital inflammation was found on magnetic resonance imaging to have a unilateral intraconal mass. Biopsy revealed a high-grade malignant tumor that was interpreted as squamous cell carcinoma. Positron emission tomography-computed tomography imaging subsequently identified a primary lesion in the esophagus. Esophageal squamous cell carcinoma is a rare cause of orbital metastasis, with only 4 previously reported cases. The authors discuss an approach to orbital malignancies of obscure origin.

Conjunctival amyloid is usually of the immunoglobulin light chain (AL) variety. The authors report an exceptional case of bilateral limbal amyloid deposits that were identified by mass spectrometry as keratin-related specifically regarding to basal keratinocyte keratins 5 and 14. Cytokeratin-related amyloid has been described by immunohistochemical analysis of formalin-fixed cutaneous and mucous membrane lesions but not ocular tissues. The conjunctival lesion in the right eye contained intraepithelial dyskeratotic cells that extended to the surface, causing a lacy leukoplakia on clinical examination. The authors excluded the diagnosis of hereditary benign intraepithelial dyskeratosis in this patient. Collection of patient data and all protected patient health information was compliant with the Health Insurance Portability and Accountability Act.