Ann P. Murchison

Validity of the American College of Rheumatology Criteria for the Diagnosis of Giant Cell Arteritis

PURPOSE To assess the clinical utility of the American College of Rheumatology criteria for the diagnosis of giant cell arteritis (GCA) in patients with positive and negative temporal artery biopsies. DESIGN Retrospective case series of all patients undergoing temporal artery biopsy. METHODS Retrospective chart review of all patients seen in the Neuro-ophthalmology Service of the Wills Eye Institute undergoing biopsy. One hundred twelve patients were identified between October 2001 and May 2006. Charts were reviewed for American College of Rheumatology criteria, biopsy results, and progression of visual loss after diagnosis. RESULTS Nine of 35 patients (25.7%) with positive biopsies would not have been diagnosed with GCA using American College of Rheumatology criteria alone. An additional 16 patients (45.7%) met only 2 criteria and required the positive biopsy to establish the American College of Rheumatology diagnosis of GCA. Eleven of 39 patients (28.2%) with negative biopsies met the criteria and would have been diagnosed with GCA. Diagnostic agreement between the American College of Rheumatology criteria without biopsy results and biopsy results alone was 51.4%; with the addition of biopsy results to the criteria, this increased to 73.0%. CONCLUSIONS The current American College of Rheumatology criteria should not be used to diagnose GCA and all patients suspected of having GCA should undergo a temporal artery biopsy.

Endoscopic approach to the orbital apex and periorbital skull base

To review cases of endoscopic orbital apex and periorbital skull base surgery and stratify the pathology and lesion location. Variations in surgical technique and the outcomes are reviewed. We report the results of all cases of endoscopic orbital apex surgery over a 40‐month period. Eighteen cases with a variety of pathology, location in the orbital apex, and surgical technique are reviewed.

The ultrasonic bone aspirator in transnasal endoscopic dacryocystorhinostomy.

Purpose: To evaluate the outcomes of endoscopic dacryocystorhinostomy (eDCR) with and without the use of ultrasonic bone aspirator (UBA; Sonopet). Methods: A retrospective, institutional review board approved chart review of all eDCRs over 49 months. Data included demographics, indication/etiologic factors of nasolacrimal duct obstruction, comorbidities, intraoperative findings, epiphora symptoms pre- and postoperatively, and complications. Patients were grouped in eDCR with or without UBA. Results: One hundred and twenty-three primary eDCRs in 99 patients were included, 59 with UBA and 64 without UBA. Most patients were Caucasians (80.8%) and women (72.0%), with a mean age of 55.9 years (range, 9–89). There were no significant differences in the demographics of the 2 subgroups. Complete resolution of symptoms was obtained in 81.3% of procedures without UBA and in 79.7% with UBA. Most patients (72.7%) were deemed idiopathic preoperatively. Lacrimal sac biopsy demonstrated significant pathologic factors in 9 (7.3%) cases, with 7 (5.7%) of these resulting in a new diagnosis for the patient. There were no cases of cerebrospinal fluid leakage, visual loss, diplopia, infection, or uncontrolled epistaxis in either group. Conclusions: Early results of eDCR with UBA appear to show reasonable efficacy. The overall success and failure rates of eDCR with and without UBA are similar. Neither group had any complications in this study, although any conclusion on the overall safety of the procedure is limited by the power of this study.

Non-surgical treatments of primary, non-melanoma eyelid malignancies: a review

The diagnosis and management of periocular cutaneous malignancies are essential components of an ophthalmologists practice. Skin cancers comprise nearly one‐third of newly diagnosed malignancies. Furthermore, the incidence of skin cancer appears to be increasing. Multiple treatment modalities exist for periocular cutaneous malignancy. Surgical extirpation, often with the combined expertise of a Mohs micrographic surgeon, is typically the first line therapy and is often curative in the periocular region, depending on a variety of factors, including tumour histology, specific location, depth of invasion and surgical technique. However, there are instances where a less invasive, non‐surgical treatment option is warranted, including patients who are poor surgical risks or those with diffuse disease. The purpose of this article is to review the literature and describe the non‐surgical treatment options, indications and efficacies for non‐melanoma primary eyelid malignancies.

Lupus Erythematosus Profundus Masquerading as Idiopathic Orbital Inflammatory Syndrome

Idiopathic orbital inflammatory syndrome (IOIS) is a nonspecific inflammation of orbital tissue. As it is a diagnosis of exclusion, systemic testing and, at times biopsy, is utilized to rule out other inflammatory etiologies. Since some inflammatory etiologies that masquerade as typical IOIS can be vision or life threatening, it is important to consider these diagnoses. Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune process that can affect the eye and visual system in 20% of individuals. In this idiopathic process, the deposition of pathogenic autoantibodies and immune complexes damage tissues and cells. Some common ocular manifestations of SLE include keratoconjunctivitis sicca, periocular skin lesions, orbital inflammation, retinal hemorrhages and vasculitis, retinal vaso-occlusive disease, iritis, scleritis, optic neuritis and optic neuropathy. One rare clinical entity in the SLE spectrum is panniculitis, also known as lupus erythematosus profundus (LEP), which is a nodular inflammation of adipose tissue. Panniculitis involving orbital structures as the primary presenting symptom of SLE is quite unusual and has only rarely been previously reported in the literature and has not been reported presenting as IOIS. This uncommon presentation can make the diagnosis more difficult. We describe a patient who had presented with ptosis evolving to orbital inflammation, which was consistent with IOIS by laboratory and histologic examinations. The patient later developed extensive panniculitis and a final diagnosis of LEP was made.

Brow ptosis after temporal artery biopsy: incidence and associations.

OBJECTIVE Temporal artery biopsy (TAB), performed for the diagnosis of giant cell arteritis, has a low reported rate of complications. One complication is damage to the facial nerve branches, which can result in brow ptosis and/or orbicularis oculi weakness. However, the incidence of facial nerve damage after TAB is unknown. DESIGN Prospective, institutional review board-approved study of all TABs performed by 2 surgeons over a 17-month period. PARTICIPANTS Seventy patients undergoing 77 TABs. METHODS Demographic data, including age, gender, and race/ethnicity, were collected for all patients. Frontalis and orbicularis oculi muscle function were evaluated pre- and postoperatively in all patients. The use of blood thinners, location of the incision, length of incision and biopsy, biopsy results, and procedure difficulty were recorded. Incidence of postoperative facial nerve damage, other complications, and rates of facial nerve recovery were evaluated. Analysis of variables was performed for any potential correlation with facial nerve damage. MAIN OUTCOME MEASURES Incidence of facial nerve damage. RESULTS Analysis included 75 biopsies performed in 68 patients. The majority of the patients were white (75.0%) and female (67.6%). The mean age was 72.6 years (range, 51-96). Postoperative facial nerve damage was found in 12 patients (16.0%) and 58.3% of these fully resolved at an average of 4.43 months (range, 1-6). Two patients (2.7%) had postoperative infections. There was no correlation with facial nerve damage and use of blood thinners, biopsy result, surgeon, procedure difficulty, incision length, or specimen length. The distance from the incision to both the orbital rim and the brow was significant: Incisions farther from the orbital rim and brow were less likely to have postoperative facial nerve damage. CONCLUSIONS There is a 16.0% incidence of postoperative facial nerve damage with TABs, which recovers fully in over half of patients. Incisions closer to the orbital rim and brow were more likely to have postoperative facial nerve dysfunction. Incisions >35 mm from both the orbital rim and brow or above the brow were less likely to have postoperative brow ptosis. FINANCIAL DISCLOSURE(S) The authors have no proprietary or commercial interest in any of the materials discussed in this article.

Pyoderma gangrenosum of the eyelids: recurrence in a skin graft.

A 41-year-old woman presented with pyoderma gangrenosum involving the right eyelids. Over the course of 3 years, she developed progressive scarring of the eyelids with lagophthalmos and corneal exposure. Multiple reconstructive procedures were performed with varying degrees of success. Despite aggressive medical control of her disease, it recurred in grafted skin on her upper and lower eyelids as confirmed by tissue biopsy. Periocular pyoderma gangrenosum is a rare disease where the management may be complicated by disease recurrence in reconstructed tissues, including skin grafts as this case demonstrates.

IMPACT OF PHYSICIAN COMMUNICATION ON DIABETIC EYE EXAMINATION ADHERENCE: Results From a Retrospective Cohort Analysis.

Purpose: To evaluate the effect of written communication between an ophthalmologist and a primary care physician (PCP) on patient adherence to diabetic eye examination recommendations. Methods: In a retrospective cohort study of a multiethnic population at an urban ophthalmology center, records of all patients with diabetes and clinic visits between 2007 and 2010 were reviewed. Data collected included patient demographics, insurance status, hemoglobin A1C, severity of diabetic retinopathy, follow-up examinations, and written communication between a patients ophthalmologist and PCP. Statistical analyses were performed to examine the relationship between physician communication and adherence to diabetic eye examination based on the American Academy of Ophthalmology–published recommendations. Results: A total of 1,968 people with diabetes were included. Written communication from an ophthalmologist to a PCP was associated with increased adherence to follow-up eye examination recommendations (Odds Ratio: 1.49; 95% Confidence Interval: 1.16–1.92; P = 0.0018). Communication from a PCP to an ophthalmologist was also associated with increased adherence (Odds Ratio: 1.94; 95% Confidence Interval: 1.37–2.77; P = 0.0002). Multivariable analysis controlling for other factors associated with examination adherence confirmed that communication both to and from an ophthalmologist was independently and significantly associated with increased follow-up adherence. Conclusion: Patients with communication between ophthalmologists and PCPs are more likely to adhere to diabetic eye examinations.

Quality and diagnostic utility of mydriatic smartphone photography: The smartphone ophthalmoscopy reliability trial

BACKGROUND AND OBJECTIVE Establish quality and diagnostic utility of mydriatic smartphone ophthalmoscopy (SO) fundus images compared to fundus camera (FC) images. PATIENTS AND METHODS In this prospective, cross-sectional study, 94 consecutive patients in an urban eye emergency department underwent SO and FC fundus imaging via one of three study arms: medical student 1 (MS1), medical student 2 (MS2), and ophthalmology resident (OR). Images of 188 eyes were graded for overall quality by two masked reviewers, and observed critical fundus findings were compared to dilated fundus examination documentation. RESULTS SO images were higher quality in the OR arm than in the MS1 and MS2 arms (P < .017). There were no differences in FC image quality between photographers (all P > .328). In the OR arm, SO images detected 74.3% of critical fundus findings, whereas FC images detected 77.1%. CONCLUSION SO produces fundus images approaching the quality and diagnostic utility of traditional FC photographs.

Treatment of mucous membrane pemphigoid with mycophenolate mofetil.

Purpose: To evaluate the clinical outcomes of mycophenolate mofetil (MMF) treatment of mucous membrane pemphigoid (MMP). Methods: This is a retrospective analysis of consecutive patients with clinical MMP seen in the Ocular Surface Disease Clinic at the Wills Eye Institute, between January 1, 2004, and December 31, 2010, treated with MMF. The main outcomes measured were control of inflammation and discontinuation of MMF. Results: A total of 23 MMP patients taking MMF were identified. The median age of the MMF-treated patients was 77.0 years. Eleven of the 23 patients (47.8%) had biopsy-proven MMP. All patients were at least Foster grading system stage 2, with most stage 3 or 4. Eight patients (34.8%) failed previous treatments with dapsone, methotrexate, prednisone, azathioprine, cyclophosphamide, or 6-mercaptopurine. The average duration of MMF treatment was 23.32 ± 33.17 months (range 1–124.83 months, median 7.4 months). Of the 23 patients with MMP, control of inflammation was achieved with MMF within 3 months for 56.5% [95% confidence interval (CI) 54.5–59.6], within 6 months for 69.6% (95% CI 65.2–76.6), and within 12 months for 82.6% (95% CI 75.3–92.4) of the patients. Nineteen patients (82.4%) achieved control of inflammation, with 16 of the 19 (84.2%) achieving control of inflammation with MMF as monotherapy. Fifteen patients were treated with MMF as initial therapy. Twenty-one percent of patients (5 of 23) were taken off MMF for failure of inflammatory control (4) or an allergic reaction (1). Conclusions: Treatment of MMP with MMF in this uncontrolled case series resulted in control of inflammation in the majority of patients with minimal side effects. Our data support consideration of MMF as an initial treatment option for active ocular MMP.