Faculty Bibliography

PURPOSE/OBJECTIVE:To evaluate the association of risk of obstructive sleep apnea (OSA) and thyroid eye disease-compressive optic neuropathy (TED-CON). METHODS:The authors performed a retrospective observational study. All TED patients evaluated by the principal investigator over the past 5 years were screened with the snoring tired observed pressure (STOP)-Bang questionnaire to assess their risk for OSA. Patients were grouped into 2 age-matched cohorts based on the presence (TED-CON) or absence of CON. The groups were then compared using chi-square analysis for binary variables and Student t test for continuous variables. RESULTS:Three hundred twenty-six patients were identified to have TED. Of those, 109 were enrolled in the study. The prevalence of high risk of OSA was significantly higher in the TED-CON (59.2%) when compared with the noncompressive TED group (32.8%; p = 0.006). CONCLUSIONS:Obstructive sleep apnea and TED have each been independently associated with elevated serologic and tissue inflammatory mediators. The systemic inflammation associated with OSA has been implicated in the pathogenesis of disease states aggravated by untreated OSA. Effective treatment of OSA decreases the levels of circulating inflammatory mediators. Currently, smoking is the only known modifiable risk factor in TED. There is evidence to implicate the pathologic elevation of inflammatory cytokines in the mechanism of smoking on TED. This preliminary investigation reveals a significantly greater prevalence of OSA risk factors among patients with TED-CON, suggesting that this may be another modifiable risk factor associated with TED. Based on screening with the STOP-Bang questionnaire, there is a higher percentage of patients at high risk of OSA with TED with CON than with TED without CON.

Adult xanthogranulomatous disease of the orbit is a rare and incompletely understood entity. It can be limited to the orbit or be associated with systemic disease. While routine systemic surveillance is the standard of care for patients with orbital xanthogranulomatous disease, there is no universally accepted protocol for managing periorbital and orbital involvement. The authors report a case of adult orbital xanthogranuloma, without systemic disease, who, after 10 years of observation, demonstrated sustained radiographic regression of the lesions. To the authors' knowledge, this represents one of the first reports of spontaneous regression of untreated orbital xanthogranuloma, and supports observation of lesions that do not compromise ocular function. We present our case in the context of a major review of various treatment strategies described in the literature, including surgical resection, radiotherapy, plasmapheresis, corticosteroids, multiple immunomodulatory medications.

Buphthalmos can create treatment challenges for socket rehabilitation following enucleation due to compression atrophy and mechanical stretching of adnexal tissues. The authors report a case demonstrating a surgical technique for simultaneous volume augmentation of the anophthalmic socket, expansion of the conjunctival fornix, and repair of lower eyelid retraction in a patient with history of buphthalmos and a previously placed 22 mm orbital implant at the time of enucleation. The authors utilized a single-stage, bipedicle dermis fat graft. Postoperatively, the patient demonstrated improvement in lower eyelid position, lower eyelid volume, improved prosthesis fit, improved three-dimensional projection of the ocular prosthesis, and an improvement in the superior sulcus deformity. Bipedicle dermis fat grafts are an option for surgical rehabilitation of the anophthalmic socket when orbital volume deficiency, conjunctival fornix contracture, and eyelid retraction are present and an adequately sized orbital implant has previously been placed.

Orbital decompression surgery and medical therapy for thyroid eye disease (TED) have evolved over the past 150 years and afforded the opportunity to restore pre-disease appearance and visual function. This manuscript explores the past 150 years of surgical innovation for the treatment of TED. The "Age of Surgical Heroism" spans the time from 1888 to 1979 during which the pioneers of orbital decompression developed lateral orbitotomy, transcranial decompression, paranasal sinus decompression, and transantral decompression despite an incomplete understanding of the pathophysiology of both TED and a limited ability to non-invasively assess their patients. The "Age of Surgical Refinement" dawned with the development of computed tomography and represents the years 1979-2000. During this time, the "swinging eyelid" approach for two- and three-wall decompressions was introduced, a combined orbital-extradural four wall decompression procedure was developed, fat decompression was explored, and endoscopic decompression techniques were advanced. At the beginning of the 21st century, our understanding of the orbital pathophysiology of TED evolved significantly. Clinicians recognized the age-related phenotype of TED based largely on the relative contribution of extraocular muscle enlargement vs. orbital fat expansion. The "Modern Age" of Customized Orbital Decompression features both "medical decompression" during the active phase of TED and, in the stable phase, customized surgical plans incorporating individual patients' anatomy, orbital pathology, and surgical goals that collectively maximize therapeutic benefit while minimizing therapeutic morbidity.

Superficial subcutaneous lipomas are benign, slow-growing neoplasms comprised mature adipose cells that may be surrounded by a fibrous tissue capsule. While found commonly on the body, simple lipomas are rarely described in the eyelids. Reports of two eyelid lipomas in the literature describe the anatomy to be posterior to the orbital septum. The presenting authors report the case of a simple lipoma of the eyelid found fully in the preseptal plane. Lipomas can occur anterior to the orbital septum and should be considered in the differential when presented with a painless, slow-growing eyelid mass in adults.

A 15-month-old male was referred for biopsy of presumed rhabdomyosarcoma in the setting of rapidly progressing left-sided proptosis. Examination revealed left periorbital edema and left hypoglobus. MRI revealed a soft-tissue density mass within the left lateral retrobulbar space. Several days later, he developed acute periorbital ecchymosis and increasing edema. With high suspicion for a vascular lesion, a CT scan was performed with dynamic arterial and venous imaging. Central filling was noted in the lateral retrobulbar component with increased enhancement on delayed venous imaging in the middle cranial fossa component favoring the diagnosis of a low-flow orbital venous malformation. In the setting of spontaneous orbital hemorrhage and risk of future vision loss, the decision was made to proceed with a combined neurosurgical approach treating the intracranial component and debulking the orbital component. This case highlights the importance of thorough radiographic evaluation prior to proceeding with a surgical procedure in the appropriate clinical context.

Dermoid cysts are histologically defined as surface epithelium encapsulating an inner lumen. They are well described in the literature as discrete, single masses, either circumscribed or dumbbell-shaped, with or without a longstanding fistula. Chronic granulomatous inflammation is often a feature of dermoid cysts, contributing to local soft tissue and bony destruction. Isolated multicystic dermoids are not well described. We present a case of a multilobular dermoid characterized both radiographically and histopathologically. These findings may be attributed to repeated rupture and reformation of the dermoid cyst. When possible, our experience favours early excision of orbital dermoid cysts to minimize morbidity.

PURPOSE/OBJECTIVE:Dermatofibromas are common cutaneous lesions, but rarely occur in the eyelid skin. The reason for the low incidence in the palpebral skin has not been elucidated. In this study, we analyze the histopathologic features of an illustrative case of dermatofibroma and review previously published cases to determine whether eyelid dermatofibroma develops differently from the prototypical dermatofibroma. METHODS:Histopathologic analysis of a new illustrative case of eyelid dermatofibroma and retrospective review of published cases. RESULTS:The distinguishing features of the illustrative lesion included a rounder gross appearance, nonacanthotic epithelium, basophilic staining, cellular character, and a paucity of "collagen trapping." These features deviated from the typical features associated with classic dermatofibroma. Review of the 11 previously published cases of eyelid dermatofibroma revealed that they were more similar in appearance to the illustrative lesion than to classic dermatofibroma. DISCUSSION/CONCLUSIONS:The rarity and histological deviations of the eyelid dermatofibroma suggest that the dermal substrate from which the lesion develops differs from that of the classic dermatofibroma. This difference may be explained microanatomically based on the fact that the dermis of the eyelid is predominantly papillary, whereas the dermis of extrapalpebral skin where dermatofibromas are more common is predominantly reticular. CONCLUSIONS:Although related, eyelid dermatofibromas appear to be histologically distinct from classic dermatofibromas, owing to the unique dermal composition of the site of origin.

Orbital infection can be caused by numerous pathogens, and accurate diagnosis informs appropriate therapy. The authors report a case of a 78-year-old man with well-controlled diabetes mellitus and recurrent sino-orbital infection following multiple surgical procedures with negative microbiologic results. This case presented a diagnostic and treatment challenge and was aided by the use of internal transcribed spacer sequencing for pathogen identification. The fungal pathogen, Tilletiopsis minor, has not previously been described as a human pathogen in the sinus and orbit. This report describes a novel orbital pathogen and highlights the importance of diagnostic diligence and utilizing internal transcribed spacer sequencing in the workup of atypical orbital infection.

A 48-year-old man presented with a longstanding left upper eyelid mass that increased in size when bending forward. The mass was associated with enophthalmos and slowly increased in size with Valsalva maneuver. Magnetic resonance imaging and arteriography demonstrated a vascular malformation without an abnormal arterial component. Because of his increasing pain, the vascular malformation was excised in a multidisciplinary approach involving intraoperative fluoroscopic mapping, cyanoacrylate embolization, and surgical excision. Histopathology was consistent with a benign vascular malformation. Clinically, this was a low-flow, distensible venous malformation-or orbital varix.