The Isabel Differential Diagnosis Generator for Orbital Diagnosis.

PURPOSE: The Isabel differential diagnosis generator is one of the most widely known electronic diagnosis decision support tools. The authors prospectively evaluated the utility of Isabel for orbital disease differential diagnosis. METHODS: The terms "proptosis," "lid retraction," "orbit inflammation," "orbit tumour," "orbit tumor, infiltrative" and "orbital tumor, well-circumscribed" were separately input into Isabel and the results were tabulated. Then the clinical details (patient age, gender, signs, symptoms, and imaging findings) of 25 orbital cases from a textbook of orbital surgery were entered into Isabel. The top 10 differential diagnoses generated by Isabel were compared with the correct diagnosis. RESULTS: Isabel identified hyperthyroidism and Graves ophthalmopathy as the leading causes of lid retraction, but many common causes of proptosis and orbital tumors were not correctly elucidated. Of the textbook cases, Isabel correctly identified 4/25 (16%) of orbital cases as one of its top 10 differential diagnoses, and the median rank of the correct diagnosis was 6/10. Thirty-two percent of the output diagnoses were unlikely to cause orbital disease. CONCLUSION: Isabel is currently of limited value in the mainstream orbital differential diagnosis. The incorporation of anatomic localizations and imaging findings may help increase the accuracy of orbital diagnosis.

Gaze-evoked amaurosis from idiopathic orbital inflammation.

Gaze-evoked amaurosis is a transient monocular vision loss elicited by eccentric gaze and has been reported in many orbital conditions but is most classically associated with intraconal tumors, such as cavernous hemangioma and optic nerve sheath meningioma. Here, the authors report a case of gaze-evoked amaurosis due to idiopathic orbital inflammation. The patient was a 35-year-old man who presented with vision loss only when abducting his left eye. He had a history of sclerosing idiopathic orbital inflammation with a left orbital intraconal mass diagnosed 15 months prior to the current presentation. The patient had difficulty with immunosuppressive therapy, which was stopped 5 months prior to presentation. Repeat imaging during the current presentation revealed enlargement of the mass. This case demonstrates that idiopathic orbital inflammation should be considered in the differential diagnosis for gaze-evoked amaurosis, which may be the first symptom of disease progression.

Diplopia Following the Insertion of a Canalicular Bypass Tube: Etiology, Risk Factors, Management, and Outcomes.

PURPOSE: To report the etiology, management, and possible risk factors for diplopia after canalicular bypass surgery. METHODS: A multicenter retrospective, noncomparative case series of patients who developed diplopia following canalicular bypass surgery were assessed. RESULTS: Twenty-four cases of diplopia were identified across 12 institutions. Tubes were inserted as a primary procedure with external dacryocystorhinostomy (DCR) (1; 4%) or without DCR (10; 42%) or as a secondary procedure after external (8; 33%) or endonasal (5; 21%) DCR. Factors predisposing to local damage were noted in 17 (71%): these factors included preexisting autoimmune/inflammatory condition (7 cases), medial canthal tumor resection (5 cases), preoperative radiotherapy (2 cases), 2 drug treatments (topical and systemic), and 1 local surgery. Horizontal diplopia was due to restriction of abduction and first noted at a median of 3.5 months (mean: 17.8 months, range: 1 day to 112 months) and persisted in 23 (96%) cases with a mean restriction of -2, affecting primary gaze in 4 patients and activities of daily living in 13 (42%). Seventeen patients received various treatments: 10 were operated on resulting in cure in 1 and improvement in 9. A stable degree of diplopia persisted in all but one patient. CONCLUSIONS: Restriction of abduction causing horizontal diplopia is a rare complication with canalicular bypass surgery and a notably high proportion occurred after tube placement without DCR; carunculectomy was not ubiquitous. Although in some the diplopia may be improved with intervention, the chance of cure is low. This complication should probably be included during informed consent for canalicular bypass tubes.

Guidelines for vismodegib in the management of periocular basal cell carcinoma.

OBJECTIVE: The management of advanced basal cell carcinoma (BCC) in the periocular region remains a clinical challenge. Vismodegib (ErivedgeTM) has been approved in 2013 by Health Canada for adult patients with "histologically confirmed metastatic BCC or locally advanced BCC inappropriate for surgery or radiation." An expert consensus was sought to create a standardised approach in the use of this novel treatment. METHODS: Fourteen practicing oculoplastic surgeons across Canada were involved in formulating and reviewing guidelines until consensus was reached. A consultancy meeting was followed by further ratification of guidelines over email. Two voting surveys were performed of the group to objectively assess agreement over each statement within the guidelines. Ratification continued until at least two-thirds of the group agreed on every guideline statement. RESULTS: The guidelines summarize 21 statements in a major and minor criteria format. A multidisciplinary team review is suggested for each patient with the involvement of recommended specialists. The internal survey revealed 100% agreement over 9 statements, 91.7% agreement over 8 statements, 83.3% agreement over 4 statements, and 2 statements had 66.7% and 58.7% agreement each. All statements with less than 91.7% agreement were surveyed again, and they were kept, modified, or removed on the basis of a consensus of over 66.7%. CONCLUSIONS: These guidelines serve to act as a framework for physicians considering vismodegib for the medical management of patients with advanced or metastatic periocular BCC. Future applications, including neoadjuvant uses of the drug, may become apparent through further research.

Acute Unilateral Ptosis and Myositis Following the H1N1 Influenza Vaccine.

The authors present a rare case of sudden onset unilateral complete ptosis occurring hours after administration of the H1N1 influenza vaccine. A complete workup for autoimmune and neurological disease was negative. MRI of the orbits demonstrated unilateral orbital myositis involving the left superior rectus/levator complex, superior oblique, and medial rectus muscles. Ptosis resolved spontaneously over 2 months without intervention. An autoimmune mechanism may explain rare cases of orbital myositis following administration of the H1N1 influenza vaccination.A case of acute onset and spontaneous resolution of complete unilateral ptosis and orbital myositis post H1N1 influenza vaccination.

Management of a Large Congenital Hemangioma Obstructing Visual Axis: A Case Report and Review of Literature.

Congenital hemangiomas comprise a subset of vascular tumors with clinicopathologic features that are distinct from the more common infantile hemangioma. The authors present a patient with a large congenital hemangioma involving the forehead and brow which obstructed the visual axis and created significant risk for deprivational amblyopia. Management of the congenital hemangioma involved customized headgear to clear the visual axis and early vascular embolization of feeder vessels with the subsequent successful surgical resection at 23 days of life.A large amblyogenic congenital hemangioma required a multidisciplinary approach involving early vascular embolization of feeder vessels and subsequent surgical resection at 23 days of life.

Management of orbital rhabdomyosarcoma in a child with Li-Fraumeni syndrome.

This case highlights the management of orbital rhabdomyosarcoma in a child with Li Fraumeni syndrome (LFS). Treatment with chemotherapy and eventual orbital exenteration enabled margin-free control of the tumor. Radiation therapy was avoided to reduce the risk of inducing additional malignancy. Reactive orbital hyperostosis was observed postoperatively and was confirmed with surgical biopsy of the orbital roof. In this case, systemic surveillance imaging, which is necessary in patients with LFS, revealed an adrenal cortical carcinoma.

Perceptions and use of computer-assisted surgery (CAS) in the orbit.

Computer-assisted surgery (CAS) plays a prominent role in certain surgical disciplines. We investigated the current perceptions and use of this technology for orbital surgery. An online survey was emailed to members of the American Society of Ophthalmic Plastic and Reconstructive Surgery, Canadian Society of Oculoplastic Surgery, and British Oculoplastic Surgery Society. Respondents were asked to describe their practice type and seniority, their frequency of orbital surgery, experience, use, and accessibility of CAS, and their opinion on the technology. There were a total of 151 responses across the societies. 105 respondents (69.5%) had been in attending/consultant practice for over 10 years, with over half (54.7%) working in academic/teaching hospitals. The majority (66.7%) had superficial or no experience with CAS. In total, 84.8% of respondents rarely or never use CAS for orbital surgery (n = 128). Posterior orbital surgery (64.2%) and orbital decompression (49.0%) were the two most useful reasons to implement CAS. Longer operating time (58.3%) and cost (54.8%) were the two most selected weaknesses for CAS, whereas improved accuracy in attaining surgical end point(s) (80.8%) and patient safety (63.6%) were the principal advantages. Type of practice was significantly associated with CAS availability/accessibility (p < 0.05). Proportion of orbital surgery performed in practice was significantly associated with both CAS experience and use (p < 0.05). Our study confirms an expected variation in the perception and use of CAS for orbital surgery. Demonstrated patient benefit and integration of refined and cost-effective CAS systems into operating room environments may influence its future role.

A Case of Pseudomonas Orbital Cellulitis Following Glaucoma Device Implantation.

PURPOSE: Orbital cellulitis is a rare complication of aqueous tube shunt surgery. Nine cases have been described in the literature, though the microbiologic etiology is rarely reported. Management with intravenous antibiotics and/or explantation has been described. METHODS: This is a case report and literature review. CASE: A 64-year-old woman developed pain, periorbital swelling, limited extraocular motility, proptosis, and conjunctival injection 3 days following implantation of an Ahmed Glaucoma Valve. Computed tomography of the orbits with contrast showed soft tissue fat stranding consistent with orbital inflammation. Initial medical management with topical and intravenous ceftriaxone and vancomycin was unsuccessful. Surgical removal of the implant was performed and intraoperative cultures demonstrated florid Pseudomonas aeruginosa growth. Antibiotic coverage was changed to Piperacillin-Tazobactam for 3 days, with eventual resolution of her orbital symptoms. CONCLUSIONS: We report the first case of orbital cellulitis after implantation of a glaucoma device associated with P. aeruginosa. Failure of intravenous and topical antibiotics led to explantation of the valve and targeted intravenous antibiotic therapy with subsequent clinical improvement.

Complete ophthalmoplegia in Ipilmumab and Nivolumab combination treatment for metastatic melanoma.

Ipilimumab and Nivolumab are novel monoclonal antibodies that have recently been used successfully for treatment of metastatic melanoma. Ipilimumab is a human monoclonal antibody against Cytotoxic T Lymphocyte Antigen 4 (CTLA4) receptor, which suppresses T-cell proliferation and stimulates an inflammatory response against cancer cells. Nivolumab is an IgG4 monoclonal antibody against the cytotoxic T lymphocyte associated programmed death 1 receptor (PD-1). Ipilimumab and Nivolumab combination treatment has been shown to induce remission and prolong survival in patients with metastatic melanoma. The side effect profile of these medications has not been well studied. One entity of the side effects reported in the literature is immune-related adverse events (irAEs). There have been few case reports where these events were serious and irreversible. In this case report, we describe a fatal and severe diffuse panmyositis that involved the cardiac, respiratory, and extraocular muscles in a patient with metastatic melanoma secondary to combination treatment with Ipilimumab/Nivolumab.

Endovascular Management of a Traumatic Infraorbital Pseudoaneurysm Causing Orbital Compartment Syndrome.

An 89-year-old woman presented after blunt injury to the left orbit from a fall. Examination findings were suggestive of left-sided orbital compartment syndrome, unresponsive to emergent lateral canthotomy and cantholysis. CT revealed a left-sided orbital floor blowout fracture involving the infraorbital canal, with a large maxillary and infraorbital hematoma. Angiography revealed a pseudoaneurysm supplied by the infraorbital artery. Interventional neuroradiology successfully achieved hemorrhage control by endovascular obliteration of the parent artery close to the pseudoaneurysm. To our knowledge, this is the first reported case of successfully managing active intraorbital hemorrhage causing orbital compartment syndrome by endovascular vessel sacrifice of an infraorbital artery pseudoaneurysm.

Orbital radiation therapy for Graves' ophthalmopathy: measuring clinical efficacy and impact.

PURPOSE: Graves' ophthalmopathy (GO) is an autoimmune condition primarily managed with prolonged courses of glucocorticoids, which can be associated with significant side effects. Orbital radiation therapy (RT) is an alternative treatment that has shown variable efficacy in improving orbital and visual symptoms. In this study, the therapeutic benefit of RT was evaluated in terms of patient's ability to taper their corticosteroid requirements, which may better reflect the proposed mechanism of RT and provide a clinically relevant response endpoint. METHODS AND MATERIALS: This is a retrospective review of consecutive patients treated with orbital RT for GO between 2000 and 2010 at a single tertiary hospital with a dedicated ocular radiation therapy clinic. The primary measure of treatment response was defined as the ability to taper glucocorticoids following RT without any further exacerbation of orbitopathy symptoms. Additional endpoints including ocular symptoms (diplopia, proptosis, visual acuity, extraocular movement) and need for surgical intervention were reported. RESULTS: Of 86 eligible patients, with a mean follow-up of 9.3 months, 81 (94%) patients responded to RT. Of patients taking corticosteroids at baseline, 91% were able to taper off corticosteroids completely and the remaining patients had decreased their doses by 83%. Diplopia, visual acuity, and extraocular movements improved in 29%, 81%, and 58% of patients, respectively. The median reduction in proptosis was 2.5 mm and 2 mm in the left and right eyes, respectively (range, -18 mm to 23 mm). CONCLUSIONS: Orbital RT is a generally well-tolerated treatment that helps minimize the dose and duration of corticosteroid therapy for patients with GO while improving ocular symptoms, including proptosis and diplopia. Prospective research should consider using corticosteroid requirement as a measure of response to orbital RT for GO.

Cadaveric anatomical comparison of the lateral nasal wall after external and endonasal dacryocystorhinostomy.

PURPOSE: Much literature has accumulated espousing the relative merits of endonasal and external dacryocystorhinostomy (DCR). However, there is comparatively little information on the relative anatomic differences between these 2 approaches. The purpose of this study is to investigate the anatomic relationships of the lateral nasal wall for endonasal and external DCR. METHODS: Ten cadaver half heads were used in this study. Half were subject to endonasal and half to external DCR procedures. The lateral nasal wall was then dissected and measurements were taken of ostium and anastomosis size and position relative to other landmarks on the lateral nasal wall. Relationships were compared between the 2 procedures. RESULTS: The dimensions and area of the ostium and the anastomosis were similar between the 2 procedures. The lower portion of the ostium was located more inferiorly in endonasal DCR. Additionally, the ostium was more likely to be found lateral to the axilla of the middle turbinate in endonasal DCR, when compared with anterior for external. External DCR was also more likely to involve opening the anterior ethmoid air cells than endonasal approach. CONCLUSION: Endonasal and external DCR osteomies appear to be of similar size, with the endonasal opening being located slightly lower and more posterior on the lateral nasal wall.

Indications for orbital imaging by the oculoplastic surgeon.

OBJECTIVE: To determine the indications for ordering orbital imaging and the indications for ordering CT versus MRI by oculoplastic surgeons and to assess the correlation between surgeon's clinical indications for imaging and the radiologist's diagnosis. DESIGN: Retrospective review of imaging requisitions and radiology reports. PARTICIPANTS: Patients of 4 oculoplastic surgeons who required CT or MRI scans. METHODS: Imaging requisitions and radiology reports of patients from 4 oculoplastic surgeons were reviewed to determine the indication for ordering a CT or MRI scan between March 2006 and March 2009. The indications were then compared with the radiologist's diagnosis. RESULTS: A total of 735 patients were included: 449 (61.1%) female and 286 (38.9%) male, with an average age of 50.1 years and an age range of 7 months to 93 years. Of these patients, a total of 632 CT and 223 MRI scans were ordered, 135 of which were follow-up scans. CONCLUSIONS: The most common indication for CT scan was thyroid disease, followed by orbital tumors and then inflammatory disease, while the most common indication for MRI scan was orbital tumors, followed by inflammatory disease and then thyroid disease. CT scans were more commonly ordered than MRI, largely for trauma and to rule out orbital foreign body.

Bifocal orbital and nasopharyngeal amyloidomas presenting as Graves disease.

A 72-year-old man presented with a slowly progressive left hyperglobus, left infraduction deficit, bilateral lower eyelid retraction, and dysphagia. He had a notable chin-down head position, diplopia in primary position, and 3 mm of left proptosis. He had been diagnosed with Graves disease 3 years before presentation. CT scans showed enlargement of the left inferior and medial rectus muscles with associated stranding of the retrobulbar fat and a low-density heterogeneous mass in the left aspect of the neck protruding in the nasopharynx. Biopsies of the orbit and nasopharynx revealed focal areas of amyloid. This represents the first report of bifocal amyloidomas of the orbit and nasopharynx.

American Joint Committee on Cancer classification predicts outcome of patients with lacrimal gland adenoid cystic carcinoma.

PURPOSE: To investigate whether American Joint Committee on Cancer (AJCC) classification at initial diagnosis of lacrimal gland adenoid cystic carcinoma predicts outcome of treatment on local recurrence. DESIGN: Retrospective chart review. PARTICIPANTS: Consecutive patients with adenoid cystic carcinoma of the lacrimal gland treated at 8 institutions between January 1986 and December 2007. METHODS: Clinical records, including pathology reports and imaging studies, were reviewed. MAIN OUTCOME MEASURES: AJCC classification, histologic subtype, local recurrence rate, and survival. RESULTS: AJCC classification at initial diagnosis was assessable for 53 patients and was as follows: T1N0M0, 7 patients; T2N0M0, 8 patients; T3aN0M0, 14 patients; T3aNxM0, 1 patient; T3aN0M1, 1 patient; T3bN0M0, 13 patients; T3bN0M1, 1 patient; T4aN0M0, 2 patients; T4bN0M0, 4 patients; T4bN0M1, 1 patient; and T4bNXM0, 1 patient. Thirty-eight (72%) of the 53 patients had >T3 tumors at presentation. Of the 38 patients with >T3 tumors, 20 were treated with orbital exenteration and postoperative adjuvant radiotherapy (RT), 6 were treated with orbital exenteration without RT, and 12 were treated with globe-preserving surgery (10 with RT and 2 without RT). Of the 15 patients with T3 tumors, the risk of local recurrence (in the orbit or skull base) was higher in patients treated with conservative surgery as opposed to orbital exenteration and RT. Only 4 (20%) of the 20 patients treated with orbital exenteration and RT had local recurrence, compared with 3 (50%) of the 6 patients treated with orbital exenteration without RT and 8 (67%) of the 12 patients treated with globe-preserving surgery. Overall, 17 (45%) of the 38 patients with >T3 tumors and only 1 (7%) of the 15 patients with T3 disease at initial diagnosis correlates with worse outcomes than does AJCC

Digital photography as a novel technique of measuring ocular surface dimensions.

PURPOSE: To introduce a novel technique for measuring ocular surface dimensions using digital photography and computerized image analysis in the context of ptosis repair surgery. METHODS: Digital photographs and patient questionnaires on dry eye symptoms were obtained from 31 patients before and after ptosis repair. Patients were examined preoperatively and at 1 and 6 weeks postoperatively. Adobe Photoshop 7.0 (Adobe Systems Incorporated, 345 Parkl Avenue, San Jose, CA 95110-2704, USA) was used to digitally measure palpebral fissure height, fissure width, and ocular surface area (OSA). Similar digital measurements were obtained on 30 control subjects as well. Digital calculations of OSA for control, preoperative, and postoperative groups were compared with other published techniques. RESULTS: Graphical comparison between our method of measuring OSA and the mathematical estimations proposed by previous studies suggests that our method is more precise in measuring OSA, and that it is also better able to identify individual variations of OSA from general population trends. CONCLUSION: Digital ocular photography combined with computerized image analysis is a fast, easy to use, and reliable method of measuring ocular surface dimensions. In addition to ptosis surgery, this method can be used in other ocular surface studies.