Dilated Superior Ophthalmic Vein: Clinical and Radiographic Features of 113 Cases.

PURPOSE: Dilated superior ophthalmic vein (SOV) is an uncommon radiographic finding. The authors review the presentation, etiology, radiography, and visual implications of 113 patients with dilated SOV. METHODS: An observational case series and multicenter retrospective chart review were conducted. There were 113 patients with a dilated SOV. Outcome measures included patient demographics, clinical features, radiographic findings, diagnosis, and treatment, and treatment outcomes were assessed. RESULTS: Cases included 75 women (66%) and 38 men (34%) with a mean age of 49 ± 24 years (range, 0.4-90 years). Diagnoses fell under 6 categories: vascular malformation (n = 92, 81%), venous thrombosis (n = 11, 10%), inflammatory (n = 6, 5%), traumatic hemorrhage (n = 2, 2%), lymphoproliferative (n = 1, 1%), and infectious (n = 1, 1%). Imaging modalities utilized included MRI (n = 98, 87%), digital subtraction angiography (n = 77, 68%), CT (n = 29, 26%), and ultrasonography (n = 4, 4%). Disease status at last follow up included no evidence of disease (n = 57, 50%), alive with persistent disease (n = 53, 47%), and expired from disease (n = 3, 3%). Treatment and management was tailored to the underlying disease process with a mean follow up of 18 months (range, 1 day to 180 months). Visual impairment observed at presentation and last follow up across all cases was 26% and 22%, respectively. CONCLUSION: Dilated SOV is a rare radiographic finding resulting from a wide spectrum of etiologies with clinical implications ranging from benign to sight- and life-threatening. Dilated SOV is most often found with dural-cavernous fistula or carotid-cavernous fistula, orbital or facial arteriovenous malformation, and venous thrombosis. Recognition of this finding and management of the underlying condition is critical.

Orbital floor fracture with entrapment: Imaging and clinical correlations in 45 cases.

Orbital floor fractures (OFF) with entrapment require prompt clinical and radiographic recognition for timely surgical correction. Correct CT radiographic interpretation of entrapped fractures can be subtle and thus missed. We reviewed the clinical, radiographic and intraoperative findings of 45 cases of entrapped OFF to correlate pre- and intraoperative findings with radiography. Retrospective review and statistical analysis of 45 patients with OFF using the chi squared and Kruskal-Wallis tests. Main outcome measures included patient demographics, clinical features, radiologic interpretation, intraoperative findings, and treatment outcomes. Twenty-one cases (47%) had radiologic evaluations of orbital CT scans that included commentary on possible entrapment. Intraoperatively, 16 (76%) of these patients had the inferior rectus muscle incarcerated in the fracture, while 5 (24%) patients had incarceration of the orbital fat. Possibility of entrapment was not commented on in the radiology reports of the remaining 24 (53%) cases. Intraoperatively, 13 (54%) of these patients had the inferior rectus muscle incarcerated in the fracture, while 11 (46%) patients had incarceration of the orbital fat. It is vital to assess the possibility of entrapment, especially in young patients, in the setting of OFF as a delay in diagnosis may lead to persistent diplopia, disfigurement, or bradycardia. Most radiology reports did not mention the possibility of entrapment in this cohort. A key concept is that entrapment occurs when any orbital tissue (muscle or fat) is trapped in the fracture site.

Topical Imiquimod in the Treatment of Conjunctival Actinic Keratosis.

Conjunctival actinic keratosis is rare and difficult to treat, as recurrences are common. Imiquimod, an immune response modulator, is currently Food and Drug Administration-approved for cutaneous actinic keratosis and superficial basal cell carcinomas. Emerging reports have shown it to be effective in treating some periocular and conjunctival lesions. The authors present a case of a 68-year-old white man with recurrent actinic keratosis involving the pretarsal conjunctiva, which was successfully treated with 5% topical imiquimod following previous failure with cryotherapy and interferon α-2b. The patient had ocular irritation that resolved on cessation of treatment. To the authors' knowledge, this is the first report of conjunctival actinic keratosis being treated with and successfully eradicated by topical imiquimod.

Orbital volume augmentation using expandable hydrogel implants in acquired anophthalmia and phthisis bulbi.

The purpose of this study is to describe our experience using expandable spherical hydrogel implants and injectable hydrogel pellets for orbital volume augmentation in cases of post-enucleation socket syndrome after acquired anophthalmia or phthisis bulbi. We retrospectively reviewed the clinical records of all adult patients who received an expandable hydrogel implant for orbital volume loss following enucleation or phthisis bulbi at the Emory Eye Center between 2004 and January 2007 and the Yale Eye Center between 2009 and 2011. The study included 9 women and 5 men with a mean age of 51.2 years old (range 35-76 years old). Follow-up spanned 6 to 71 months (median of 18.5 months). Four patients received spherical hydrogel implants and 10 patients received hydrogel pellet injections. On average, nine pellets (range 5-16) were placed in each patient over an average of 1.7 injections (range 1-3). Most commonly, five pellets were injected per session, as was the case for 13 of the 17 treatment sessions. Post-operative complications included 2 cases of pellet migration, one subcutaneously and one anteriorly due to insufficiently posterior implant placement, and 1 hospital admission for pain after injection of 10 pellets in one visit. All patients experienced an overall subjective improvement in cosmesis. Self-expandable hydrogel implants appear to offer several advantages over other existing options for orbital volume augmentation, as they are easy to place, generally well-tolerated, volume-titratable, and to the extent that our follow-up shows, may be a safe and durable means of treating orbital volume loss in patients with acquired anophthalmia and phthisis bulbi.

Periocular Melanoma In Situ Treated With Imiquimod.

PURPOSE: To evaluate the efficacy of topical 5% imiquimod cream in the treatment of periocular melanoma in situ (lentigo maligna). DESIGN: Retrospective case series. SUBJECTS: There were 12 patients in this series, and the mean patient age was 77 years. The anatomical locations were the lower eyelid (n=5), upper and lower eyelid (n=4), lower eyelid including the eyelid margin (n=1), brow (n=1), and the medial canthus (n=1). Topical 5% imiquimod cream was used as a primary treatment (n=6) or as an adjunctive therapy following local excision (n=2), cryotherapy (n=2), or excisional biopsy with cryotherapy (n=2). METHODS: Twelve patients with periocular melanoma in situ were treated with topical 5% imiquimod cream daily for a mean treatment period of 3.9 months. The clinical features of the patients and the responses to treatment were evaluated in a retrospective case series. MAIN OUTCOME MEASURES: Histologic clearance of atypical melanocytes. RESULTS: Eleven patients achieved complete histologic clearance of atypical melanocytes on post-treatment biopsy. One patient could not tolerate local irritation from imiquimod and stopped in the first month of therapy with residual disease. The median follow-up time was 1.5 years. Side effects included redness (n=12), discomfort (n=6), swelling (n=4), ectropion (n=1), and conjunctival chemosis (n=1). The patients experienced no systemic side effects from the treatment. CONCLUSIONS: Topical 5% imiquimod cream is an effective option as primary or adjunct therapy in the treatment of periocular melanoma in situ.

Clinical Parameters and Outcomes in Periorbital Necrotizing Fasciitis.

PURPOSE: To describe the clinical features associated with periorbital necrotizing fasciitis and to correlate these features with clinical outcomes. METHODS: The case logs of 3 surgeons were used to identify cases of necrotizing fasciitis. Chart reviews were performed to characterize clinical metrics, and statistical analyses were performed. RESULTS: Seventeen patients (9 males, 8 females; mean age = 48.1 years, standard deviation = 22.6 years) were identified with periorbital necrotizing fasciitis. Of these patients, 52.9% did not have immunodeficiencies, and 52.9% did not have antecedent trauma or infected facial lesions. One patient died from necrotizing fasciitis. A history of immunosuppression correlated with the requirement for exenteration, but did not correlate visual acuity of worse than 20/40 upon discharge from the hospital. Most of the patients (68.75%) were discharged with visual acuity of better than 20/40 in the affected eye. CONCLUSIONS: This study represents the largest case series of patients with periorbital necrotizing fasciitis. Most of the patients in this series did not have immunodeficiencies, and the majority were discharged with favorable visual acuities. Nonetheless, a history of immunosuppression correlated with the need for exenteration, but was not statistically linked with worse visual outcomes.

Electromagnetic image-guided orbital decompression: technique, principles, and preliminary experience with 6 consecutive cases.

PURPOSE: To assess the feasibility of routine use of electromagnetic image guidance systems in orbital decompression. METHODS: Six consecutive patients underwent stereotactic-guided three wall orbital decompression using the novel Fusion ENT Navigation System (Medtronic), a portable and expandable electromagnetic guidance system with multi-instrument tracking capabilities. The system consists of the Medtronic LandmarX System software-enabled computer station, signal generator, field-generating magnet, head-mounted marker coil, and surgical tracking instruments. In preparation for use of the LandmarX/Fusion protocol, all patients underwent preoperative non-contrast CT scan from the superior aspect of the frontal sinuses to the inferior aspect of the maxillary sinuses that includes the nasal tip. RESULTS: The Fusion ENT Navigation System (Medtronic™) was used in 6 patients undergoing maximal 3-wall orbital decompression for Graves' orbitopthy after a minimum of six months of disease inactivity. Preoperative Hertel exophthalmometry measured more than 27 mm in all patients. The navigation system proved to be no more difficult technically than the traditional orbital decompression approach. CONCLUSION: Electromagnetic image guidance is a stereotactic surgical navigation system that provides additional intraoperative flexibility in orbital surgery. Electromagnetic image-guidance offers the ability to perform more aggressive orbital decompressions with reduced risk.

The relationship between sex and symmetry in thyroid eye disease.

PURPOSE: To examine the relationships between sex and symmetry in the context of disease activity, severity, and thyroid status in thyroid eye disease. METHODS: Retrospective chart review of 31 men and 31 women with untreated thyroid eye disease. Subjective complaints, smoking status, thyroid status, and objective findings pertinent to the clinical activity score (CAS) and "NO SPECS" classification were recorded. Overall disease asymmetry was defined as having simultaneous asymmetry of both more than one symptom and more than one external finding. Asymmetry was compared across sex and thyroid status. CAS and NO SPECS severity were compared across sex, symmetry, and thyroid status. RESULTS: Asymmetric appearance was reported by 58% of men and 19% of women. Asymmetric proptosis (>2 mm difference) was seen in 45% of men and 23% of women (P=0.036). Overall asymmetry was seen in 55% of men and 19% of women (P=0.017). Thyroid status and sex had a combined effect on symmetry, as 15 of 16 hyperthyroid females (94%) demonstrated symmetric disease. Average NO SPECS severity was 3.5 (standard deviation [SD] 1.4) in men and 3.3 (SD 1.1) in women (P=0.51), and was 3.8 (SD 1.4) in asymmetric patients versus 3.2 (SD 1.3) in symmetric patients (P=0.08). The CAS was higher in asymmetric than symmetric patients (1.84 versus 0.97; P=0.012). CONCLUSION: Men demonstrated more asymmetric disease (proptosis and overall asymmetry) than women, while hyperthyroid females demonstrated more symmetry than euthyroid and hypothyroid males and females. NO SPECS severity score was unaffected by sex, thyroid status, or symmetry. Asymmetric patients demonstrated higher clinical activity scores.

Extraction of Fronto-orbital Shower Hook through Transcranial Orbitotomy.

Transorbital foreign bodies threaten both the integrity of the globe and the brain. We present an unusual case of a penetrating right frontal lobe-orbital metallic shower hook. Extensive intracranial involvement necessitated transcranial orbitotomy allowing for the removal of the object without loss of the globe.

Cutaneous melanoma metastatic to the orbit: review of 15 cases.

PURPOSE: To describe the clinical features, management, and outcomes of 15 patients with cutaneous melanoma metastatic to the orbit. The authors review emerging treatments for metastatic melanoma and their ocular implications. METHODS: Retrospective chart review of 15 patients with orbital metastasis from cutaneous melanoma. RESULTS: At presentation of the orbital metastasis, systemic metastatic cutaneous melanoma was present in 13 (87%) patients. The mean interval from diagnosis of cutaneous melanoma to orbital metastasis was 40 months (median, 37 months; range, 0-117 months). The most common presenting signs were dysmotility (63%), proptosis (56%), and blepharoptosis (19%). Four patients (25%) presented with pain. Metastasis involved extraocular muscle in 6 orbits (35%), intraconal space in 4 (24%), extraconal space in 7 (41%), and lacrimal sac in 1 (6%). The tumor was unifocal in all cases, unilateral in 13 patients (87%), and bilateral in 2 (13%). The mean tumor basal dimension was 20 × 20 mm and mean thickness was 16 mm. Treatments included complete surgical excision in 1 patient (6%), external beam radiotherapy (EBRT) in 7 (47%), systemic chemotherapy in 8 (53%), and immunotherapy in 5 (33%). Orbital tumor control was achieved in 2 orbits (18%) following focal therapy alone (excision or EBRT), 4 (36%) following systemic therapy alone (chemotherapy or immunotherapy), and 3 (27%) following combination focal plus systemic therapy. Three patients required exenteration. Survival rates at 1 year/2 years were 100%/0% following focal therapy, 50%/25% following systemic therapy, and 100%/66% following combination therapy. CONCLUSIONS: Cutaneous melanoma metastatic to the orbit tends to involve muscle (35%) or intraconal soft tissue (24%) as a painless (75%), circumscribed (87%) mass. Treatment with systemic chemotherapy and/or immunotherapy resulted in orbital tumor control in 80% of cases. Overall survival was 25.1 months.

Neonatal orbital abscess after sinus surgery: case report and brief review.

Orbital abscess formation in neonates is rare, with only 12 cases reported in the literature. We present a case of orbital cellulitis with orbital abscesses following repair of congenital choanal atresia in a 2-week-old neonate. This is the first reported case of an orbital abscess following a sinus procedure in a neonate.

Demodex folliculitis presenting as periocular vesiculopustular rash.

PURPOSE: To report an unusual case of Demodex folliculitis presenting as periocular vesiculopustular rash. DESIGN: Case report. RESULTS: A 68 year-old woman presented with a unilateral periocular rash that was initially treated by her primary ophthalmologist with topical steroids and antivirals. Slit-lamp examination revealed severe bilateral blepharitis, right greater than left, with waxy sleeves around the eyelashes. The diagnosis of Demodex infestation was considered. Treatment with daily lid scrub with polyhexamethylene biguanide (PHMB), 1,2-hexanediol and 1,2-octanediol (OCuSOFT PLUS) and erythromycin ointment twice a day resulted in complete resolution of the symptoms after 4 weeks. CONCLUSIONS: Ophthalmologists should be aware of Demodex and consider it in the differential diagnosis of periocular skin lesions.

Spontaneous subperiosteal orbital hemorrhage.

Subperiosteal orbital hemorrhage typically results from trauma. Spontaneous subperiosteal orbital hemorrhage (SSOH) is rare and has been reported with sudden elevation of cranial venous pressure, bleeding diathesis, and sinusitis. This article presents a series of 9 patients (11 orbits) with SSOH and review the associated systemic conditions. 10 out of 11 orbits (91%) underwent surgical intervention due to advanced orbital signs or poor vision.

Periocular necrotizing fasciitis causing blindness.

IMPORTANCE: Periocular necrotizing fasciitis is a rare but potentially devastating disease, accompanied by high rates of morbidity and mortality. OBSERVATIONS: We report 5 cases of periocular necrotizing fasciitis resulting in severe vision loss, 3 of which required exenteration to contain the disease and only 1 of which recovered vision. Three cases were caused by group A streptococcus; 1, by methicillin-resistant Staphylococcus aureus; and 1, by Streptococcus anginosus constellatus. CONCLUSIONS AND RELEVANCE: Providers should maintain a high clinical suspicion for necrotizing fasciitis and distinguish it from more common forms of cellulitis. As seen in these 5 cases, periocular necrotizing fasciitis may cause severe visual loss more often than previously recognized. To our knowledge, this is also the first report of Streptococcus anginosus constellatus causing necrotizing fasciitis.

Extraction of fronto-orbital metallic radio antenna through Stallard orbitotomy.

A 39-year-old man presented after falling on a radio antenna while in his yard. Computed tomography revealed that the antenna entered the right superolateral orbit, penetrating through the right orbital roof and extending into the right frontal lobe. A Stallard orbitotomy was performed to remove the antenna resulting in blepharoptosis.

Periocular abscesses following brow epilation.

PURPOSE: The aim of this article was to report the clinical presentation, radiography, culture results, treatment modalities, and outcomes of periocular abscesses associated with brow epilation. METHODS: This was a retrospective case series including 26 patients referred for periocular abscess following brow epilation. RESULTS: Twenty-six female patients with a median age of 20.5 (range, 12-73) years were referred for oculoplastic evaluation of periocular abscesses related to recent brow epilation. All patients were treated with incision and drainage along with systemic antibiotics. Culture results revealed 16 cases of methicillin-resistant Staphylococcus aureus, 3 of methicillin-sensitive Staphylococcus aureus, and 7 cultures that showed no growth. All patients had resolution of their abscesses at 1-month follow-up visits without progression to orbital cellulitis. CONCLUSIONS: Periocular abscess formation after brow epilation has been previously described in only a single case report in the literature. The authors believe this entity is underreported given their current report describing 26 such cases. Given the high prevalence of cosmetic brow epilation in females, the authors believe a careful history regarding brow epilation in any patient presenting with a periocular abscess or preseptal cellulitis is essential to explore the possible cause of their infection. The majority of patients in the current study's cohort had methicillin-resistant Staphylococcus aureus-related abscesses, and treatment with antibiotics with methicillin-resistant Staphylococcus aureus coverage may be a prudent first line choice in such patients.

Bilateral corneal perforations and autoproptosis as self-induced manifestations of ocular Munchausen's syndrome.

PURPOSE: To report a patient with bilateral corneal perforations and autoproptosis in a case of ocular Munchausen's syndrome. DESIGN: Case report. PARTICIPANT: A 26-year-old white male referred to the oculoplastics service with one month history of decreased vision bilaterally and painful right eye. Multiple eyelid scars and right corneal opacity were noted. The patient was previously seen at another institution for rapid loss of vision in both eyes. INTERVENTIONS: An orbit decompression among many procedures failed to controlled extreme pain and proptosis. MAIN OUTCOME MEASURES: Resolution of proptosis, stabilization of vision, pain resolution. RESULTS: Three weeks after enucleation of the right eye was offered, patient presented with spontaneous left ruptured globe. After multiple episodes of self-mutilation and infections, both eyes were exenterated. CONCLUSIONS: Munchausen syndrome can be seen with ophthalmic manifestations and should be considered in the differential diagnosis when ocular abnormalities cannot be explained after a thorough evaluation. Recognition of this psychiatric disease is not only important for correct medical diagnosis and treatment, but also essential in protecting the patients from unnecessary invasive and aggressive medical procedures.

Acellular human dermal matrix as a skin substitute for reconstruction of large periocular cutaneous defects.

PURPOSE: To describe our experience using acellular human dermal matrix (AlloDerm) as a full-thickness skin graft substitute for large periocular cutaneous defects. METHODS: Retrospective review of the clinical records of all patients who received an acellular dermal allograft between 1997 and 2006 to reconstruct a large periocular cutaneous defect. Clinical outcomes, including graft viability, patient acceptance, and postoperative appearance, were assessed, and complications were noted. RESULTS: AlloDerm was used in 6 patients who were not ideal candidates for autologous skin grafts or local flap reconstruction. Four patients had severe comorbid conditions limiting the extent of surgery that could be performed safely. Four patients received AlloDerm following excision of extensive skin cancer. Two patients had extensive facial and periorbital trauma necessitating rapid defect closure at the time of primary repair. Follow-up ranged from 6 to 33 months.AlloDerm was easy to handle and shape, and it effectively covered the defects. Grafts were successfully epithelialized in all patients. One patient required a full-thickness skin graft 3 months following the original surgery for a cicatricial ectropion. No other postoperative complications were noted. Postoperative photographs demonstrated acceptable cosmetic appearance after epithelialization was complete. CONCLUSIONS: In patients for whom large full-thickness periorbital skin grafts or flaps are either inappropriate or extremely difficult to perform, the use of AlloDerm to cover a large cutaneous defect may be a viable option. Clinicians should consider covering large periorbital skin defects with AlloDerm in situations where autologous skin grafting or flap construction may be problematic or where extensive surgery may be hazardous to the patient.

Occult temporal arteritis in a 54-year-old man.

A 54-year-old white man with a remote history of pars planitis reported transient monocular visual loss (TMVL) in the left eye on standing. The following week he experienced multiple similar episodes. He denied associated systemic symptoms. Initial examination showed old peripheral retinal vascular sheathing and delayed retinal arterial filling time. Complete blood count, erythrocyte sedimentation rate, and MRI studies of the head and neck were normal. One week later, there were multiple cotton wool spots in the posterior pole, a relative afferent pupillary defect, and subtle visual field loss in the left eye. Evaluation for infectious, inflammatory, or embolic etiologies was nonrevealing. Biopsy of the prominent but nontender temporal arteries showed granulomatous inflammation, fragmentation, and duplication of the internal elastic lamina consistent with the temporal arteritis (TA). Radiography and MRI of the chest revealed dilation of the ascending aorta. The patient began treatment with high-dose oral steroids with resolution of his TMVL and retinal cotton wool spots and decrease in the size of the temporal arteries. Our case demonstrates the importance of considering TA in the setting of TMVL, visual loss, cotton wool spots, or dilated nontender temporal arteries in an otherwise asymptomatic patient even with normal inflammatory markers. Long-term follow-up is essential in unusual cases such as this one, given the high risk of ocular and systemic morbidity with TA.