Publications by authors named "Christine C Nelson"

38 Publications

Vismodegib for Preservation of Visual Function in Patients with Advanced Periocular Basal Cell Carcinoma: The VISORB Trial.

Oncologist 2021 May 14. Epub 2021 May 14.

Rogel Comprehensive Cancer Center, University of Michigan, Ann Arbor, Michigan, USA.

Background: Basal cell carcinoma (BCC) is a common skin cancer often curable by excision; however, for patients with BCC around the eye, excision places visual organs and function at risk. In this article, we test the hypothesis that use of the hedgehog inhibitor vismodegib will improve vision-related outcomes in patients with orbital and extensive periocular BCC (opBCC).

Materials And Methods: In this open-label, nonrandomized phase IV trial, we enrolled patients with globe- and lacrimal drainage system-threatening opBCC. To assess visual function in the context of invasive periorbital and lacrimal disease, we used a novel Visual Assessment Weighted Score (VAWS) in addition to standard ophthalmic exams. Primary endpoint was VAWS with a score of 21/50 (or greater) considered successful, signifying globe preservation. Tumor response was evaluated using RECIST v1.1. Surgical specimens were examined histologically by dermatopathologists.

Results: In 34 patients with opBCC, mean VAWS was 44/50 at baseline, 46/50 at 3 months, and 47/50 at 12 months or postsurgery. In total, 100% of patients maintained successful VAWS outcome at study endpoint. Compared with baseline, 3% (95% confidence interval [CI], 0.1-15.3) experienced major score decline (5+ points), 14.7% (95% CI, 5 to 31.1) experienced a minor decline (2-4 points), and 79.4% experienced a stable or improved score (95% CI, 62.1-91.3). A total of 56% (19) of patients demonstrated complete tumor regression by physical examination, and 47% (16) had complete regression by MRI/CT. A total of 79.4% (27) of patients underwent surgery, of which 67% (18) had no histologic evidence of disease, 22% (6) had residual disease with clear margins, and 11% (3) had residual disease extending to margins.

Conclusion: Vismodegib treatment, primary or neoadjuvant, preserves globe and visual function in patients with opBCC. Clinical trail identification number.NCT02436408.

Implications For Practice: Use of the antihedgehog inhibitor vismodegib resulted in preservation of end-organ function, specifically with regard to preservation of the eye and lacrimal apparatus when treating extensive periocular basal cell carcinoma. Vismodegib as a neoadjuvant also maximized clinical benefit while minimizing toxic side effects. This is the first prospective clinical trial to demonstrate efficacy of neoadjuvant antihedgehog therapy for locally advanced periocular basal cell carcinoma, and the first such trial to demonstrate end-organ preservation.
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http://dx.doi.org/10.1002/onco.13820DOI Listing
May 2021

Change in Postoperative Opioid Prescribing Patterns for Oculoplastic and Orbital Procedures Associated With State Opioid Legislation.

JAMA Ophthalmol 2021 02;139(2):157-162

Department of Ophthalmology and Visual Sciences, University of Michigan Medical School, Ann Arbor.

Importance: Understanding whether statewide legislation, such as the Michigan Opioid Laws, is associated with reduction in postoperative opioid prescriptions is informative in guiding future legislation.

Objective: To identify changes in opioid prescribing patterns for oculoplastic and orbital procedures associated with the enactment of the Michigan Opioid Laws in 2017 and 2018.

Design, Setting, And Participants: This cross-sectional study included 3781 patients who underwent any of 10 common oculoplastic and orbital procedures between June 1, 2016, and November 30, 2019, at a tertiary care institution.

Exposures: From 2017 to 2018, Michigan enacted a series of laws to address the state's worsening opioid epidemic. Two major components of this legislation enacted on June 1, 2018, required prescribers to review a report of patients' opioid use history and obtain signed consent after educating patients on the use and disposal of opioids prior to prescribing.

Main Outcomes And Measures: Demographic information, type of surgery, type and amount of opioid prescriptions, and morphine milligram equivalent (MME) were analyzed. MME was calculated as the product of dose, quantity, and opioid-specific conversion factor for each prescription. Linear interpolation spline regression was used to evaluate the association of prescription MME with time.

Results: Of 3781 patients, 1614 (42.7%) were male. The mean (SD) age at the time of surgery was 63.3 (16.6) years. Of 2026 patients undergoing surgery before June 1, 2018, 1782 (88.0%) were prescribed postoperative opioids; of 1755 patients undergoing surgery after June 1, 2018, 878 (50.0%) were prescribed postoperative opioids (P < .001). There was no difference in age, sex, race/ethnicity, surgery type, or opioids prescribed between these 2 cohorts. Linear interpolation spline regression showed a decrease of 26.025 MMEs (equivalent to a 36.2% reduction of mean MME) between June 1, 2017, and September 30, 2018 (β, -1.735; 95% CI, -0.088 to -0.024; P < .001), stabilizing at a persistently reduced rate of MME prescribed through the end of the study period (October 1, 2018, to November 30, 2019; β, -0.005; 95% CI, -0.039 to 0.016; P = .42). Changes in MME in the 12 months before or 12 months after the period of legislation enactment were not identified.

Conclusions And Relevance: In this cross-sectional study, reduction in opioid prescriptions for oculoplastic and orbital procedures was observed during the enactment period of the Michigan Opioid Laws and appeared to be sustained through the end of the study period. Similar statewide or national legislations aimed at increasing prescriber awareness and patient education on opioid use may help curtail the prescription opioid epidemic.
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http://dx.doi.org/10.1001/jamaophthalmol.2020.5446DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7729578PMC
February 2021

Glaucoma Awareness and Knowledge Among Ethiopians in a Tertiary Eye Care Center.

Ophthalmol Ther 2021 Mar 29;10(1):39-50. Epub 2020 Oct 29.

St. Paul's Hospital Millennium Medical College, Addis Ababa, Ethiopia.

Introduction: Glaucoma outcomes are closely associated with patients' awareness of the disease. However, little is known about glaucoma awareness and knowledge in Addis Ababa, a densely populated and ethnolinguistically diverse capital city in Ethiopia, the second largest country in Africa.

Methods: We performed a cross-sectional survey in the ophthalmology waiting room at St. Paul's Hospital, a tertiary care center in Addis Ababa. Respondents included patients, patient family members, and non-clinical staff. Participants were asked if they had read or heard about glaucoma to gauge basic awareness of the disease; those with awareness were asked to take a quiz to measure their objective glaucoma knowledge. We performed multivariable regression to identify factors associated with glaucoma awareness, quiz performance, and self-rated ability to use eye drops.

Results: Of 298 respondents, 145 (48.7%) were female, and the average age was 44.9 ± 17.2 years. A majority (167; 56.0%) had primary school or less than primary school education. Only 131 (44.0%) had basic glaucoma awareness. Of these, 95 (72.5%) knew that glaucoma causes permanent vision loss, 103 (78.6%) knew glaucoma is often asymptomatic in early stages, 62 (47.3%) identified elevated intraocular pressure as a glaucoma risk factor, and 124 (94.7%) knew glaucoma was treatable. A majority of this subset (126; 96.2%) said they would be willing to use medicated eye drops, and 130 (99.2%) indicated a willingness to undergo surgery if recommended. Education level was independently associated with glaucoma awareness (p < 0.001) and glaucoma quiz performance (p = 0.03).

Conclusion: In a population sample from an ophthalmology waiting room in Addis Ababa, glaucoma awareness was poor and most strongly associated with education level. Educational interventions at public health and provider-patient levels are warranted. Our results suggest high receptiveness to both medical and surgical treatment.
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http://dx.doi.org/10.1007/s40123-020-00314-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7886940PMC
March 2021

Teprotumumab: a novel therapeutic monoclonal antibody for thyroid-associated ophthalmopathy.

Expert Opin Investig Drugs 2020 Jul 10;29(7):645-649. Epub 2020 Jun 10.

Department of Ophthalmology and Visual Sciences, W. K Kellogg Eye Center, University of Michigan Medical School , Ann Arbor, MI USA.

Introduction: Thyroid-associated ophthalmopathy (TAO) is a disfiguring, potentially blinding, and sub-optimally managed autoimmune condition. Current therapy of active TAO consists most frequently of glucocorticoid steroids, orbital radiation, or B-cell depletion; all of which are associated with substantial side effects. Teprotumumab (Tepezza) is a human monoclonal antibody against the insulin-like growth factor type I receptor (IGF-IR), recently evaluated in two clinical trials for active moderate-to-severe TAO that was recently approved by the United States Food and Drug Administration (FDA) for use in TAO.

Areas Covered: This article reviews phase II and III placebo-controlled, double-masked, prospective, multicenter studies assessing the efficacy and safety of teprotumumab for the treatment of active, moderate-to-severe TAO.

Expert Opinion: Teprotumumab has demonstrated substantial and rapid improvement in Clinical Activity Score and proptosis reduction in TAO compared to placebo. Subjective diplopia and quality of life were also improved in both clinical trials. Teprotumumab exhibited a favorable safety profile, with transient hyperglycemia, muscle cramps, and auditory side effects being associated with the drug; these were usually transient. The trial findings indicate that teprotumumab is a promising, potential first-line therapy for treating TAO.
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http://dx.doi.org/10.1080/13543784.2020.1772752DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7529924PMC
July 2020

Supernumerary Lacrimal Caruncle: A Case Series.

Ophthalmic Plast Reconstr Surg 2020 May/Jun;36(3):e70-e73

Department of Ophthalmology and Visual Sciences, W. K Kellogg Eye Center, Ann Arbor, Michigan, U.S.A.

A 12-year-old boy and 63-year-old woman were incidentally found to have a solitary, well-defined, raised, ovoid lesion involving the inferomedial palpebral conjunctiva. Both lesions were separate from the lacrimal caruncle with normal conjunctiva surrounding the lesions. Excisional biopsies were consistent with caruncular tissue. In the English literature, supernumerary lacrimal caruncle has only been previously described in adults despite the congenital nature of the lesion.
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http://dx.doi.org/10.1097/IOP.0000000000001584DOI Listing
March 2021

The Use of Umbilical Amnion for Conjunctival Socket, Fornix, and Eyelid Margin Reconstruction.

Ophthalmic Plast Reconstr Surg 2020 Jul/Aug;36(4):365-371

Department of Ophthalmology and Visual Sciences, W. K Kellogg Eye Center, Ann Arbor, Michigan, U.S.A.

Purpose: To describe outcomes using umbilical amnion for conjunctival fornix, socket, and eyelid margin reconstruction.

Methods: A medical record review was performed to identify patients who had received umbilical amnion over a 2-year period in their department. Patient demographics, disease etiology, and data regarding surgical outcomes were collected. The primary outcome was the success rate of the surgical intervention.

Results: Twenty-one patients received umbilical amnion for anophthalmic socket contracture (n = 16), orbital implant exposure (n = 3), ocular surface burn (n = 1), and cicatricial entropion repair (n = 1). The primary outcome was met in 76% of patients overall. In anophthalmic socket contracture, the primary outcome was met in 86% and 0% of patients with acquired and congenital anophthalmia, respectively. The primary outcome was met in all cases of orbital implant exposure and cicatricial entropion. The primary outcome was not met in a Roper-Hall grade IV ocular surface burn.

Conclusions: Umbilical amnion is an ideal substrate graft for reconstruction of the conjunctival fornix, socket, and eyelid margin. Umbilical amnion appears to be efficacious for the management of socket contracture in acquired anophthalmia, orbital implant exposure, and cicatricial entropion. Further experience is needed to determine the efficacy of umbilical amnion in ocular surface burns.
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http://dx.doi.org/10.1097/IOP.0000000000001555DOI Listing
March 2021

Characteristics of Industry Payments to Ophthalmologists in the Open Payments Database.

JAMA Ophthalmol 2019 Jul 3. Epub 2019 Jul 3.

Department of Ophthalmology and Visual Sciences, W. K. Kellogg Eye Center, Ann Arbor, Michigan.

Importance: An increased awareness of the interactions between the medical industry and health care professionals may lead to lower health care costs and more effective health care practices.

Objective: To assess the characteristics of industry payments made to ophthalmologists between 2013 and 2017.

Design, Setting, And Participants: This analysis included data reported in the June 29, 2018, update of the Centers for Medicare & Medicaid Services Open Payments Database (OPD). The OPD contains public records of industry payments made to physicians and teaching hospitals from August 1, 2013, to December 31, 2017, as reported by the medical industry. All general or research payments distributed to US ophthalmologists and contained in the OPD were included in this study. Data are summarized by practitioner, manufacturer, payment category, and geographic location.

Main Outcomes And Measures: Main outcomes were the distribution, quantity, and value of payments made to ophthalmologists practicing in the United States or US territories. The financial characteristics of payment category, manufacturer, product, and location were also assessed.

Results: This analysis revealed that the OPD showed industry reporting a total of 20 943 ophthalmologists receiving 736 517 payments worth $543 679 603.53 (1.67% of all industry-reported funds in the OPD). The median payment value was $22.44. Most payments were for food and beverages (581 588 [78.96%]), whereas most funds were allocated toward research ($310 142 151.88 [57.05%]) and consulting fees ($73 565 327.71 [13.51%]). The median payout to each ophthalmologist was $637.75 (interquartile range, $167.33-$2065.54). California was the highest-grossing state, receiving $101 135 980.34 (18.60%) of all payments. Fifteen companies were responsible for 87.68% of all funds distributed ($476 719 470.11) and were mostly involved in the production of pharmaceutical agents (anti-vascular endothelial growth factor agents, glaucoma eyedrops, and ocular lubricants) and surgical devices (cataract and glaucoma).

Conclusions And Relevance: Although there is no way to know the veracity of these reports, the findings suggest the financial ophthalmologist-industry relationship is substantial. These relationships may be adding to health care costs and affecting the quality of care, although those associations were not evaluated in this study.
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http://dx.doi.org/10.1001/jamaophthalmol.2019.2456DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6613310PMC
July 2019

Periocular Reconstruction.

Facial Plast Surg Clin North Am 2019 Feb;27(1):105-118

Kellogg Eye Center, Michigan Medicine, 1000 Wall Street, Ann Arbor, MI 48105, USA.

Options for periorbital reconstruction include primary wound closure, local flaps, regional/distant flaps, or full-thickness skin grafts. Optimal aesthetic and functional outcomes are achieved by assessing regional contours, skin type, and facial aesthetic units. Like tissue should replace like tissue; for example, skin with skin, tarsus with tarsus (or equivalent material, eg, hard palate, ear cartilage, or autologous substitute), and conjunctiva with mucous membrane or like substitute (buccal mucous membrane, amniotic membrane). Patient characteristics including wound care needs, transportation needs, smoking status, and history of radiation can influence the reconstruction plan. Techniques most commonly used in our practice are reviewed.
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http://dx.doi.org/10.1016/j.fsc.2018.08.011DOI Listing
February 2019

Novel Use of Cryopreserved Ultra-thick Human Amniotic Membrane for Management of Anophthalmic Socket Contracture.

Ophthalmic Plast Reconstr Surg 2019 Mar/Apr;35(2):193-196

Department of Ophthalmology and Visual Sciences, W. K Kellogg Eye Center, Ann Arbor, Michigan U.S.A.

Purpose: To describe the use of cryopreserved ultra-thick human amniotic membrane for management of anophthalmic socket contracture.

Methods: A chart review was performed to identify patients undergoing fornix reconstruction with cryopreserved ultra-thick human amniotic membrane for management of anophthalmic socket contracture. Patient demographics, disease etiology, and data regarding postoperative prosthesis fit and complications were collected.

Results: The technique is described in 3 female patients with anophthalmic socket contracture who underwent fornix reconstruction using cryopreserved ultra-thick human amniotic membrane. All patients had excellent prosthesis fit at final follow up (range, 10-14 months). There were no clinically significant complications and no reoperations were performed. One pyogenic granuloma developed and was excised without affecting ocular prosthesis fit.

Conclusions: Cryopreserved ultra-thick human amniotic membrane is easy to use, well tolerated, and produces good outcomes for management of anophthalmic socket contracture.
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http://dx.doi.org/10.1097/IOP.0000000000001264DOI Listing
December 2019

Lymphedema-Distichiasis Syndrome in a Male Patient Followed for 16 Years.

Ophthalmic Plast Reconstr Surg 2018 Mar/Apr;34(2):e63-e65

Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan Medical School, Ann Arbor, Michigan, U.S.A.

Distichiasis is a challenging condition that may require multiple surgical interventions. Besides ophthalmologic concerns in children, distichiasis may be part of the lymphedema-distichiasis syndrome, which presents with lymphedema of variable time of onset. Other significant systemic disorders such as coarctation of the aorta and varicose veins have been reported in association with this syndrome and must be reviewed for proper patient care. The authors report the case of a 22-year-old male patient who had been treated for distichiasis and followed for 16 years. At his initial evaluation, at 6 years of age, he presented solely with ocular complaints due to distichiasis. Only after 13 years of repeated ophthalmic treatments and continuous follow up, lymphedema was observed. Lymphedema-distichiasis syndrome diagnosis must be considered in children with distichiasis, even in the absence of lymphedema.
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http://dx.doi.org/10.1097/IOP.0000000000001037DOI Listing
July 2018

Neonatal Graves' Eye Disease.

Ophthalmology 2017 05;124(5):673

Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan Health Systems, Ann Arbor, Michigan.

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http://dx.doi.org/10.1016/j.ophtha.2016.11.009DOI Listing
May 2017

Tangent screen perimetry in the evaluation of visual field defects associated with ptosis and dermatochalasis.

PLoS One 2017 29;12(3):e0174607. Epub 2017 Mar 29.

Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota, United States of America.

Purpose: To determine if tangent visual fields gathered during assessment of superior visual field deficits caused by blepharoptosis and dermatochalasis offer good correlation to clinical exam in a time and cost efficient manner.

Methods: Prospective, observational case series. Subjects included all patients referred to a single surgeon (CCN) who underwent surgical correction of blepharoptosis and/or dermatochalasis. Preoperatively and postoperatively, upper margin-to-reflex distances were assessed. Tangent visual fields were performed in a timed fashion and analyzed for degrees of intact vision in the vertical meridian and degrees squared of area under the curve. Data were compared by Student t-tests and Pearson correlation coefficients.

Results: Mean preoperative superior visual fields with the eyelid in the natural position measured 8° in the vertical meridian. Measurements in the vertical meridian and area under the curve showed excellent correlation (r = 0.87). Patients with ptosis showed strong correlation between margin-to-reflex distance and superior visual fields. Patients completed field testing faster than reported times for automated or Goldmann testing. Finally, tangent screens were the least expensive type of equipment to purchase.

Conclusions: Tangent visual fields are a rapid and inexpensive way to test for functional loss of superior visual field in patients with upper eyelid malposition. Our data revealed potential differences between tangent screen results and published results for automated or Goldmann visual field testing which warrants further studies.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0174607PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5371337PMC
September 2017

Evaluation of Patients with Facial Palsy and Ophthalmic Sequelae: A 23-Year Retrospective Review.

Ophthalmic Epidemiol 2017 10 20;24(5):341-345. Epub 2017 Mar 20.

c Department of Ophthalmology and Visual Sciences , University of Michigan Medical School , Ann Arbor , MI , USA.

Purpose: Facial palsy (FP) can result in serious ophthalmic sequelae including loss of vision. We describe the clinical characteristics of FP patients presenting for ophthalmologic evaluation and the timing for initiating ophthalmologic care.

Methods: We performed a retrospective review of 96 consecutive FP patients presenting between 1992 and 2015 at a tertiary eye center. The main outcome measures were time interval from the diagnosis of FP and onset of ocular symptoms to the initial ophthalmologic evaluation (IOE), and the severity of exposure keratopathy and eyelid malposition on IOE.

Results: The median time interval from the diagnosis of FP to the IOE was 1.6 years (interquartile range; IQR = 0.5-9.2), and from the onset of ocular symptoms to IOE was 0.8 years (IQR = 0.3-2.3). The most common ocular symptoms were dryness (47.9%; n = 46), irritation (39.6%; n = 38), and tearing (30.2%; n = 29). A total of 26.0% (n = 25) of patients were bothered by the appearance of their eyes. Only 13.5% (n = 13) noted change in vision. On IOE, 81.6% (n = 75) of patients had punctate epithelial erosions (PEE), of which 52.3% (n = 35) had moderate to severe PEE, 3.3% (n = 3) had corneal ulcers and 2.2% (n = 2) had corneal abrasions. The average margin-reflex distance 2 was 6.4 mm (SD = 2.4) with average lagophthalmos of 3.5 mm (SD = 3.1).

Conclusions: Among FP patients presenting for ophthalmologic evaluation, exposure keratopathy (as evidenced by PEE and corneal ulcers/abrasions) is highly prevalent and moderately severe on IOE, despite only 13.5% of patients noting decreased vision. These findings underscore the importance of timely and thorough ophthalmologic evaluation of FP patients.
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http://dx.doi.org/10.1080/09286586.2017.1294186DOI Listing
October 2017

Prevalence of Body Dysmorphic Disorder and Surgeon Diagnostic Accuracy in Facial Plastic and Oculoplastic Surgery Clinics.

JAMA Facial Plast Surg 2017 Jul;19(4):269-274

Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.

Importance: Body dysmorphic disorder (BDD) is a relative contraindication for facial plastic surgery, but formal screening is not common in practice. The prevalence of BDD in patients seeking facial plastic surgery is not well documented.

Objective: To establish the prevalence of BDD across facial plastic and oculoplastic surgery practice settings, and estimate the ability of surgeons to screen for BDD.

Design, Setting, And Participants: This multicenter prospective study recruited a cohort of 597 patients who presented to academic and private facial plastic and oculoplastic surgery practices from March 2015 to February 2016.

Methods: All patients were screened for BDD using the Body Dysmorphic Disorder Questionnaire (BDDQ). After each clinical encounter, surgeons independently evaluated the likelihood that a participating patient had BDD. Validated instruments were used to assess satisfaction with facial appearance including the FACE-Q, Blepharoplasty Outcomes Evaluation (BOE), Facelift Outcomes Evaluation (FOE), Rhinoplasty Outcomes Evaluation (ROE), and Skin Rejuvenation Outcomes Evaluation (SROE).

Results: Across participating practices (9 surgeons, 3 sites), a total of 597 patients were screened for BDD: 342 patients from site 1 (mean [SD] age, 44.2 [16.5] years); 158 patients, site 2 (mean [SD] age, 46.0 [16.2] years), site 3, 97 patients (mean [SD] age, 56.3 [15.5] years). Overall, 58 patients [9.7%] screened positive for BDD by the BDDQ instrument, while only 16 of 402 patients [4.0%] were clinically suspected of BDD by surgeons. A higher percentage of patients presenting for cosmetic surgery (37 of 283 patients [13.1%]) compared with those presenting for reconstructive surgery (21 of 314 patients [6.7%]) screened positive on the BDDQ (odds ratio, 2.10; 95% CI, 1.20-3.68; P = .01). Surgeons were only able to correctly identify 2 of 43 patients (4.7%) who screened positive for BDD on the BDDQ, and the positive likelihood ratio was only 1.19 (95% CI, 0.28-5.07). Patients screening positive for BDD by the BDDQ had lower satisfaction with their appearance as measured by the FACE-Q, ROE, BOE, SROE, and FOE.

Conclusions And Relevance: Body dysmorphic disorder is a relatively common condition across facial plastic and oculoplastic surgery practice settings. Patients who screen positive on the BDDQ have lower satisfaction with their facial appearance at baseline. Surgeons have a poor ability to screen for patients with BDD when compared with validated screening instruments such as the BDDQ. Routine implementation of validated BDD screening instruments may improve patient care.

Level Of Evidence: NA.
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http://dx.doi.org/10.1001/jamafacial.2016.1535DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5543317PMC
July 2017

Assessment of the Abbreviated National Eye Institute Visual Function Questionnaire (NEI VFQ 9) in blepharoptosis and dermatochalasis.

Arq Bras Oftalmol 2016 Jul-Aug;79(4):226-8

Department of Ophthalmology, Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan, USA.

Purpose: To evaluate the Abbreviated National Eye Institute Visual Function Questionnaire (NEI VFQ 9), which is shorter than those previously published, as a tool for assessing vision-related quality of life in patients with ptosis and dermatochalasis.

Methods: This is a cohort study of 46 patients who underwent blepharoptosis and/or upper eyelid blepharoplasty surgery by a single surgeon (CN) in 2013 in a public, academic, ambulatory care referral center. Patients included 29 who underwent blepharoplasty, 11 who underwent ptosis surgery, and 6 who underwent combined surgery. The NEI-VFQ 9 was administered pre- and postoperatively, and the composite scores were compared using Student's t-test. Survey duration was timed in a subset of patients. The hypothesis was that the NEI VFQ 9 could detect a statistically significant improvement in composite score after surgical intervention.

Results: The mean pre- and postoperative NEI VFQ 9 composite scores were 74.9 and 86.8, respectively, in the blepharoplasty-only group (P<0.0001), 72.07 and 86.41, respectively, in the ptosis-only group (P=0.004), and 75.8 and 87.2, respectively, in the combined group (P=0.022). There was no correlation between the gain in composite score and the change in upper eyelid margin to reflex distance. Twenty-five patients were timed filling out the survey, and the mean was 2.3 min.

Conclusions: The NEI VFQ 9 consistently demonstrates a significant increase in visual function for blepharoptosis and dermatochalasis patients. Thus, it may be a useful tool for assessing vision-related quality of life in patients with ptosis and dermatochalasis.
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http://dx.doi.org/10.5935/0004-2749.20160065DOI Listing
April 2017

Recurrence and Survival in Patients With Merkel Cell Carcinoma Undergoing Surgery Without Adjuvant Radiation Therapy to the Primary Site.

JAMA Dermatol 2016 09;152(9):1001-7

Department of Dermatology, University of Michigan Health System, Ann Arbor.

Importance: The use of adjuvant radiation therapy (RT) to the primary site in Merkel cell carcinoma (MCC) is not uncommon. However, the need for adjuvant RT to the primary site in patients at low risk for local recurrence is questionable.

Objectives: To examine the occurrence of true local, satellite, in-transit, regional, and distant recurrences in patients undergoing surgery alone without adjuvant RT to the primary site. To establish overall survival (OS), MCC-specific survival (MCCSS), and disease-free survival (DFS) relationships in a cohort of patients with MCC.

Design, Setting, And Participants: Our University of Michigan Multidisciplinary MCC Program database was used to obtain characteristics and outcome measures for 104 patients (105 primary MCCs) with tumors less than 2 cm in diameter. The majority of patients were treated between July 2006 and November 2012.

Main Outcomes And Measures: Outcome measures included the occurrence of true local, satellite, in-transit, regional, and distant recurrences. End points included OS, MCCSS, and DFS.

Results: Overall, information for 55 men and 49 women with 105 primary MCCs was obtained; 19 patients developed recurrent disease, and the mean time to first recurrence was 10.7 months. True local recurrence occurred in 1 patient with concurrent in-transit recurrence. Satellite recurrence occurred in 1 patient with concurrent regional recurrence. Four additional patients developed in-transit metastases. Thirteen patients had a regional recurrence component, 4 patients had distant metastases, and 6 patients developed subsequent regional and/or distant recurrences. Stratified by initial pathologic stage, the OS and MCCSS at 48 months were estimated to be 85.0% (95% CI, 71.8%-92.3%) and 94.4% (95% CI, 83.4%-98.2%) for patients with stage 1A/B disease and 63.2% (95% CI, 36.6%-81.1%) and 78.1% (95% CI, 50.0%-91.5%) for patients with stage 3A disease. The OS and MCCSS at 24 months for patients with stage 3B disease were both 50.0% (95% CI, 5.8%-84.5%).

Conclusions And Relevance: In selected MCC patients with primary tumors less than 2 cm in diameter treated with surgery alone without adjuvant RT to the primary site, we found a low occurrence of true local recurrences and satellite recurrences. This relatively low rate of local recurrence questions the need for adjuvant RT to the primary tumor site in patients with small low-risk lesions.
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http://dx.doi.org/10.1001/jamadermatol.2016.1428DOI Listing
September 2016

Efficacy of Vismodegib (Erivedge) for Basal Cell Carcinoma Involving the Orbit and Periocular Area.

Ophthalmic Plast Reconstr Surg 2015 Nov-Dec;31(6):463-6

Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan, U.S.A.

Purpose: Evaluate the effectiveness of vismodegib in the management of basal cell carcinoma with orbital extension and/or extensive periocular involvement.

Methods: Retrospective chart review of 6 consecutive patients with biopsy-proven orbital basal cell carcinoma and 2 additional patients with extensive periocular basal cell carcinoma who were treated with oral vismodegib (150 mg/day) was performed.

Results: Basal cell carcinoma extended in the orbit in 6 of 8 patients (involving orbital bones in 1 patient), and 2 of 8 patients had extensive periocular involvement (1 with basal cell nevus syndrome). Vismodegib therapy was the only treatment in 6 patients, off-label neoadjuvant in 1 patient, and adjuvant treatment in 1 patient. Orbital tumors in all 4 patients who received vismodegib as sole treatment showed partial response with a mean 83% shrinkage in tumor size after a median of 7 months of therapy. In the 2 patients receiving vismodegib as neoadjuvant or adjuvant therapies, there was complete response after a median of 7 months of therapy and no evidence of clinical recurrence after discontinuing therapy for a median of 15 months. The 2 patients with extensive periocular involvement experienced complete clinical response after a median 14 months of treatment. During treatment, the most common side effects were muscle spasm (75%) followed by alopecia (50%), dysgeusia (25%), dysosmia, and episodes of diarrhea and constipation (13%).

Conclusions: Basal cell carcinoma with orbital extension and extensive periocular involvement responds to vismodegib therapy. The long-term prognosis remains unknown, and additional prospective studies are indicated.
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http://dx.doi.org/10.1097/IOP.0000000000000388DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4564370PMC
June 2016

Compressive Optic Neuropathy and Repeat Orbital Decompression: A Case Series.

Ophthalmic Plast Reconstr Surg 2015 Sep-Oct;31(5):385-90

Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan, U.S.A.

Purpose: To characterize the presenting characteristics, preoperative clinical activity score (CAS), surgical approach, and visual outcomes in patients with thyroid eye disease undergoing repeat orbital decompression for recurrent or recalcitrant compressive optic neuropathy (CON).

Methods: The medical records of patients with recurrent or recalcitrant CON undergoing repeat orbital decompressions were retrospectively reviewed. The primary outcome measures included pre- and postoperative Humphrey visual field mean deviation, visual acuity (VA) measured in logarithm of the minimal angle of resolution, color vision measured by Ishihara plates, and presence of relative afferent pupillary defect. Details of the surgical procedure and each patient's CAS at presentation were also recorded.

Results: Six patients, 9 orbits, with a mean preoperative CAS of 3.8 were included in this review. The mean time between initial decompression and presentation to our center for recurrent or persistent CON symptoms was 8.6 years (range, 1 to 15 years). At presentation, the average Humphrey visual field mean deviation was -16.5 (standard deviation: 8.8), improving to -3.8 (2.4) postoperatively with a mean of 9.3 months follow up (mean improvement of 75%). Preoperative VA was 0.34 (0.23) LogMAR, improving to 0.05 (0.10) LogMAR with a mean follow up of 10.4 months. Pre- to postoperative comparisons of clinical measures all showed statistically significant improvement (p < 0.05). Eight eyes presented with decreased VA (any VA < 20/20), 4 with decreased color vision (any color vision < 11), and 1 with a relative afferent pupillary defect, and all these patients demonstrated improvement following repeat orbital decompression.

Conclusions: In patients with thyroid eye disease, symptoms of recurrent CON occurred up to 15 years following initial orbital decompression underscoring the smoldering, progressive nature of the disease. Repeat decompression that focused on the orbital apex resulted in visual improvement in all 6 patients. Despite clinical evidence of CON, the mean CAS of these patients at presentation was only 3.8, highlighting the importance of close monitoring of patients with thyroid eye disease following decompression regardless of the external manifestations of disease activity.
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http://dx.doi.org/10.1097/IOP.0000000000000356DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5036444PMC
May 2016

Increased CD40+ Fibrocytes in Patients With Idiopathic Orbital Inflammation.

Ophthalmic Plast Reconstr Surg 2015 May-Jun;31(3):202-6

Department of Ophthalmology and Visual Sciences, University of Michigan; and Department of Surgery, Ann Arbor Veteran's Administration Hospital, Ann Arbor, Michigan, U.S.A.

Objective: To investigate the phenotypic and functional characteristics of peripheral and tissue-infiltrating stem cells called fibrocytes in patients with idiopathic orbital inflammation (IOI).

Methods: Seven patients with IOI were studied. In the 3 patients requiring orbital biopsy, fibrocytes were identified in orbital tissue from patients with IOI compared with healthy controls using immunohistochemistry. Fibrocytes from the peripheral blood of all 7 patients and controls were quantified and phenotyped by flow cytometry and immunofluorescence for expression of CD34, alpha smooth muscle actin, CD40, and collagen 1. Quantitation of CD40-mediated interleukin-6 (IL-6) production was measured using enzyme-linked immunosorbent assay.

Results: Orbital biopsy specimens from patients with IOI demonstrate tissue infiltration by fibrocytes (n = 3). Fibrocytes are present in the peripheral blood of IOI patients (n = 7) but are scarce in healthy donors (n = 19). Fibrocytes from IOI patients express substantial levels of CD40, and ligation of CD40 increases IL-6 expression.

Conclusions: Fibrocytes are present in the peripheral blood and orbital tissues of patients with IOI and constitutively express CD40 and express IL-6 in response to ligation. This site-specific predilection of CD34(+) fibrocytes to sites of orbital inflammation and fibrosis may suggest a role in IOI. Moreover, CD40-mediated activation cytokine production may contribute to the proinflammatory and profibrotic features of IOI and may provide a mechanism for future targeted therapy.
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http://dx.doi.org/10.1097/IOP.0000000000000243DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4318793PMC
February 2016

External dacryocystorhinostomy surgery in patients with Wegener granulomatosis.

Ophthalmic Plast Reconstr Surg 2012 Nov-Dec;28(6):389-92

Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan, USA.

Purpose: To determine surgical outcomes after external dacryocystorhinostomy (DCR) surgery in patients with Wegener granulomatosis (WG).

Methods: The authors retrospectively reviewed the charts of consecutive patients with WG who underwent primary or secondary external DCR surgery between January 2001 and January 2010. Clinical data reviewed included patient demographics, systemic disease involvement and immunosuppression therapy, intraoperative biopsy findings, and postoperative outcomes and complications. Success was defined as resolution of epiphora.

Results: Sixteen primary external DCRs were performed on 9 patients with WG, and 2 secondary external DCRs were performed on 2 patients. At the time of surgery, all patients with WG were on systemic immunosuppressive agents including methotrexate, rapamycin, sirolimus, tacrolimus, azathioprine, cyclophosphamide, rituximab, and prednisone, and no patients received increased corticosteroids after surgery. Intraoperative biopsy in patients with WG revealed chronic inflammation (4 patients) and fibrosis (1 patient). Silicone stents were removed at an average of 5.8 months (range, 3-12 months). All surgeries were successful in resolving epiphora with an average follow up of 3.5 years (range, 10 months-6 years) and no complications.

Conclusions: Primary and secondary external DCR surgery successfully treats nasolacrimal duct obstruction in patients with WG on systemic immunosuppression.
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http://dx.doi.org/10.1097/IOP.0b013e3182601041DOI Listing
March 2013

External dacryocystorhinostomy outcomes in sarcoidosis patients.

Ophthalmic Plast Reconstr Surg 2012 Jan-Feb;28(1):47-9

Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan, USA.

Purpose: To determine surgical outcomes after external dacryocystorhinostomy (DCR) surgery in patients with sarcoidosis.

Methods: We retrospectively reviewed the charts of all patients with sarcoidosis who underwent external DCR surgery between January 2001 and January 2010. Clinical data reviewed included patient demographics, immunosuppressive therapies, biopsy results, use of intraoperative triamcinolone, and postoperative outcomes and complications. Success was defined as resolution of epiphora.

Results: External DCR was performed on 13 sides of 9 patients with sarcoidosis. Four patients were systemically immunosuppressed with methotrexate or plaquenil, and 4 patients used inhaled corticosteroids only. Intraoperative biopsy in 10 cases (9 patients) revealed non-necrotizing granulomatous inflammation (8 cases) and chronic inflammation (2 cases). Silicone stents were removed at a mean of 2.9 months. Initial DCR surgery was successful in 10 of 13 (87%) surgeries with an average follow up of 31 months (range, 14 to 48 months). None of the 5 surgeries (4 patients) with intralesional triamcinolone injections failed, compared with 3 of 8 (38%) surgeries without intralesional triamcinolone. Of the 3 failures, 2 early failures (3 months) were successfully treated with balloon catheter dilation. In the one patient with a late failure (47 months), subsequent balloon catheter dilation failed. All 3 patients who experienced failures used inhaled corticosteroids only. In contrast, 4 of the 6 patients with successful surgery were systemically immunosuppressed. Complications such as punctal erosion, wound necrosis, or cerebrospinal fluid leak did not occur.

Conclusions: External DCR surgery successfully treats nasolacrimal duct obstruction associated with sarcoidosis. Intralesional triamcinolone may improve the success rate without added complications. Long-term success may be less in patients not receiving systemic immunosuppressive therapy.
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http://dx.doi.org/10.1097/IOP.0b013e3182364ad2DOI Listing
March 2012

The effect of orbital decompression surgery on lid retraction in thyroid eye disease.

Ophthalmic Plast Reconstr Surg 2011 Nov-Dec;27(6):436-8

Department of Ophthalmology and Visual Sciences, University of Michigan, Ann Arbor, Michigan, USA.

Purpose: To determine the effect of orbital decompression surgery on lid retraction as a function of proptosis reduction in the setting of thyroid eye disease.

Methods: Retrospective interventional case series of all consecutive medial and lateral orbital decompressions performed by the authors for thyroid eye disease from 1999 to 2008. Primary outcome measures included postoperative proptosis and lid retraction.

Results: One hundred sixty-five eyes of 89 patients were included. The average amount of proptosis reduction at final follow up (average 30 months) was 4.6 mm. The average improvement in upper lid retraction was 0.9 mm, and the average improvement in lower lid retraction was 0.8 mm. There was a statistically significant correlation (0.12, p = 0.005) between the amount of proptosis reduction and lower lid elevation. No such correlation (-0.010, p = 0.90) was seen for upper lid position. In cases where inferior rectus recession was performed subsequent to decompression surgery (n = 20), the effect of proptosis reduction on lower lid position was negated (correlation -0.01, p = 0.980).

Conclusion: A statistically significant correlation exists between the amount of proptosis reduction from orbital decompression surgery and improvement in lower lid retraction. No such correlation exists between proptosis reduction and upper lid retraction. Inferior rectus recession negates the positive effect of orbital decompression on lower lid position.
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http://dx.doi.org/10.1097/IOP.0b013e3182232465DOI Listing
January 2012

Intralesional interferon for extensive squamous papilloma of the eyelid margin.

Ophthalmic Plast Reconstr Surg 2012 Mar-Apr;28(2):e47-8

Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan, USA.

Eyelid margin papillomas are challenging because complete excision may be cosmetically unacceptable. Interferon has been previously reported as an effective treatment for conjunctival papillomas. We report successful treatment of a large upper eyelid margin papilloma with intralesional interferon.
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http://dx.doi.org/10.1097/IOP.0b013e31821f1df3DOI Listing
June 2012

Thyroid-associated orbitopathy: Current insights into the pathophysiology, immunology and management.

Saudi J Ophthalmol 2011 Jan 11;25(1):15-20. Epub 2010 Nov 11.

Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan, Ann Arbor, MI 48105, USA.

Half the patients suffering from Graves' disease develop thyroid-associated orbitopathy (TAO). The severity of TAO varies considerably with a mild form characterized by dry eyes and discomfort to the severe form with sight-threatening exposure keratopathy and optic neuropathy. The pathogenesis and immunologic mechanisms underlying Graves' disease and TAO is unknown, however, advances toward this understanding have indicated a prominent role of orbital fibroblasts, T cells and B cells. These advances have led to novel strategies for clinical treatment using immunomodulatory modalities. Initial results included use of infliximab and etanercept (anti-TNF agents), but currently there is an increasing interest in anti-B cell (Rituximab) therapy. Rituximab has shown promising results in progressive, sight-threatening TAO. It has also shown encouraging results in halting or slowing the disease process in patients unresponsive to corticosteroids. The primary advantage of these immunomodulatory agents is based upon targeting the molecular mediators of the disease and avoiding the potential side effects of non-specific therapies.
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http://dx.doi.org/10.1016/j.sjopt.2010.11.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3729841PMC
January 2011

Marcus Gunn jaw-winking synkinesis: clinical features and management.

Ophthalmology 2010 Jul 25;117(7):1447-52. Epub 2010 Feb 25.

Eye Plastic, Orbital and Facial Cosmetic Surgery Service, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan 48105, USA.

Objective: To evaluate the clinical features including eyelid excursion and management of Marcus Gunn jaw-winking synkinesis (MGJWS).

Design: Observational case series.

Participants: Forty-eight consecutive patients with MGJWS.

Methods: Clinical features and management of 48 patients with MGJWS were reviewed retrospectively. Upper eyelid excursion was measured and graded. Complications of surgical intervention were evaluated.

Main Outcome Measures: Resolution of MGJWS and symmetry of upper eyelids in primary position.

Results: Excursion of the ptotic eyelid with jaw movement in MGJWS was graded as mild (<2 mm) in 16% of patients, moderate (2-4 mm) in 76% of patients, and severe (> or = 5 mm) in 8% of patients. Thirty patients with moderate or severe MGJWS underwent disabling of the involved levator muscle and bilateral or unilateral frontalis suspension and had more than 6 months of follow-up. After a mean follow-up of 62 months, MGJWS resolved in 29 (97%) patients and improved from 6 mm to 2 mm in 1 (3%) patient. Relative upper eyelid height was within 1 mm in 87% of patients in primary position and within 1 mm in 80% of patients in downgaze. Twenty-six patients had bilateral frontalis suspension with disabling of unilateral levator muscle on the involved side. Relative upper eyelid height was within 1 mm in 88% of patients in the primary position and within 1 mm in 88% of patients in downgaze. Four non-amblyopic patients had unilateral frontalis suspension with levator muscle disabling. Relative upper eyelid height was symmetrical in 75% of the patients in primary position and in 25% of patients in downgaze. Complications included eyelash ptosis in 10% of the patients, loss of eyelid crease in 10%, and entropion in 3%.

Conclusions: Most of the patients with MGJWS exhibited moderate eyelid excursion. Disabling of the involved levator muscle and bilateral frontalis suspension and, in selected cases, disabling of the involved levator muscle and unilateral frontalis suspension were effective in the treatment of MGJWS. Eyelash ptosis and loss of eyelid crease were the most common complications, each occurring in 10% of the patients.

Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
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http://dx.doi.org/10.1016/j.ophtha.2009.11.014DOI Listing
July 2010

Outcomes in silicone rod frontalis suspension surgery for high-risk noncongenital blepharoptosis.

Ophthalmic Plast Reconstr Surg 2009 Sep-Oct;25(5):361-5

Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan Medical School, Ann Arbor, Michigan 48105, USA.

Purpose: To evaluate the outcomes of silicone rod frontalis suspension for complicated cases of ptosis with poor levator function.

Methods: A retrospective interventional case series of consecutive patients undergoing silicone rod frontalis suspension by 2 surgeons over 20 years generated records of 51 eyelids (33 patients) with ptosis secondary to cranial nerve III palsy, myasthenia gravis, chronic progressive external ophthalmoplegia, or oculopharyngeal dystrophy. Outcome measures included postoperative change in eyelid height, lagophthalmos, and corneal fluorescein staining; need for reoperation; patient and physician satisfaction; grading of postoperative photographs; and interobserver agreement.

Results: Forty-five percent of patients had preoperative corneal staining. Surgery resulted in a significant increase in eyelid height (+2.8 mm; p < 0.0001), lagophthalmos (+0.4 mm, p < 0.0001), and corneal staining grade (+0.3 units; p = 0.02). Most patients achieved a subjectively acceptable result. Patient and physician agreement with outcome assessment was good (kappa = 0.65, p < 0.0001). Twenty eyelids (39%) required revision of the silicone sling, most often for adjustment of eyelid height. Survival analysis differed by diagnosis (p = 0.0154) and was most favorable for patients with myasthenia gravis. Interobserver agreement on postoperative photographs was marginal.

Conclusions: Silicone rod frontalis suspension surgery increases the eyelid height in complicated blepharoptosis patients with minimal eyelid excursion. Patients and physicians concur closely regarding satisfaction; masked assessment of photographic outcome parameters by observers yields marginal agreement. Because most patients undergoing this procedure have a narrow window for appropriate postoperative eyelid height, a relatively high proportion of patients required revision. Silicone rod frontalis suspension surgery is chosen in these challenging cases for the ease of adjustment.
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http://dx.doi.org/10.1097/IOP.0b013e3181b3b183DOI Listing
February 2010

Nondiagnostic conjunctival map biopsies for sebaceous carcinoma.

Arch Ophthalmol 2009 Aug;127(8):961-3

University of Michigan, Department of Ophthalmology and Visual Sciences, Ann Arbor, MI, USA.

Objective: To compare the prevalence of nondiagnostic conjunctival map biopsies in patients with extensive pagetoid sebaceous carcinoma (defined as involvement of 3 or 4 quadrants of the ocular surface) and in patients without extensive pagetoid tumor (defined as involvement of 1 or 2 quadrants of the ocular surface).

Methods: Retrospective medical record and pathologic specimen review of 20 patients treated for sebaceous carcinoma at a tertiary care center. Biopsies with artifactual loss of or damage to the epithelium were categorized as nondiagnostic.

Results: One hundred forty-four map biopsies were reviewed, an average of 7.2 (standard deviation [SD], 4.4) biopsies per patient. Sixteen patients had extensive pagetoid tumor, 4 did not. Fifteen percent of biopsies were nondiagnostic. The frequency of nondiagnostic biopsies in patients with and without extensive pagetoid tumor was 37% and 10%, respectively. The odds ratio of nondiagnostic biopsy in the setting of extensive pagetoid tumor was 5.9 (95% confidence interval, 2.3-15.6; P = .004). Six of the sixteen patients (38%) without extensive pagetoid tumor had at least 1 nondiagnostic biopsy, with an average of 1.8 (SD, 1.6) nondiagnostic biopsies per patient (22% of biopsies). Two of the 4 patients (50%) with extensive pagetoid tumor had at least 1 nondiagnostic biopsy, with an average of 5.5 (SD, 3.5) nondiagnostic biopsies per patient (57% of biopsies).

Conclusions: Nondiagnostic, de-epithelialized conjunctival map biopsies are more common in patients with extensive pagetoid tumor than in those with limited or no pagetoid tumor. Artifactual epithelial loss may result from disruption of epithelial adhesion to the basement membrane by infiltrating tumor cells.
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http://dx.doi.org/10.1001/archophthalmol.2009.177DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3208231PMC
August 2009

The transglabellar/subcranial approach for surgical excision of periocular second tumors in retinoblastoma.

Orbit 2008 ;27(4):285-91

Department of Ophthalmology, W. K. Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan 48105, USA.

Purpose: To evaluate the use of transglabellar/subcranial approach for surgical resection of periocular second non-ocular tumors in retinoblastoma patients.

Methods: Seven retinoblastoma patients with periocular second tumor involving anterior skull base underwent surgical resection by transglabellar/subcranial approach in a single center. The medical records of these patients were retrospectively evaluated.

Results: The most common presenting symptom in retinoblastoma patients with periocular second tumor was difficulty in maintaining the prosthesis in three patients (43%), followed by epistaxis in one (14%), palpable orbital mass in one (14%), persistent periocular swelling in one (14%), and visual loss in one (14%) patient. Periocular second tumors were leiomyosarcoma in three (43%) patients, osteosarcoma in three (43%), and sphenoid wing meningioma in one (14%) patient. Surgical resection by the transglabellar/subcranial approach was the only treatment in one (14%) patient with sphenoid wing meningioma and was combined with chemotherapy in three (43%) patients, and with both external beam radiotherapy and chemotherapy in three (43%) patients. Surgical margins were negative in three (43%) patients and microscopically positive in four (57%) patients. Complications were minor, including cerebral spinal fluid (CSF) leak in one (14%) patient and CSF leak and subgaleal hematoma in one (14%) patient. After 31 months mean follow-up, three (43%) patients were alive and four patients (57%) were dead.

Conclusions: Retinoblastoma patients with periocular second tumors have a poor prognosis. The transglabellar/subcranial approach can be used for surgical resection of periocular second tumor involving skull base with low morbidity.
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http://dx.doi.org/10.1080/01676830802222951DOI Listing
November 2008

Management of periocular cutaneous melanoma with a staged excision technique and permanent sections the square procedure.

Ophthalmology 2008 Dec 9;115(12):2295-2300.e3. Epub 2008 Aug 9.

Department of Ophthalmology and Visual Sciences, University of Michigan Medical School, Ann Arbor, Michigan 48105, USA.

Objective: To evaluate the outcome of the square procedure, a multidisciplinary, staged excision technique using standard formalin-fixed, vertically oriented sections for periocular cutaneous melanoma.

Design: Observational, retrospective, case series.

Participants: Forty patients with periocular cutaneous melanoma treated with the square procedure.

Methods: Demographic features, tumor data, and recurrence rate were reviewed retrospectively for 40 patients with periocular cutaneous melanoma treated with the square procedure.

Main Outcome Measure: Local recurrence rate.

Results: Of 40 patients, 26 (65%) had lentigo maligna melanoma in situ (MIS), 12 (30%) had lentigo maligna melanoma, and 2 (5%) had superficial spreading melanoma. Tumor-free margins were reached within a mean margin of 13 mm for patients with MIS and of 16 mm for patients with invasive melanoma. There was no statistical difference for the margin width in patients with MIS and invasive melanoma. The lesion size and margin width were significantly correlated. Recurrence was observed in 1 (2.5%) patient at 8 months after the square procedure, and a Kaplan-Meier survival curve estimated a local recurrence rate of 2.5% at 8 years.

Conclusions: The square procedure is an effective procedure for management of periocular lentigo maligna melanoma in situ and lentigo maligna melanoma with a low local recurrence rate of 2.5% at 8 years.

Financial Disclosure(s): The authors have no proprietary or commercial interest in any materials discussed in this article.
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http://dx.doi.org/10.1016/j.ophtha.2008.06.011DOI Listing
December 2008

Radioiodine therapy and Graves' ophthalmopathy.

J Nucl Med 2008 Jun 15;49(6):923-30. Epub 2008 May 15.

Nuclear Medicine Division, Department of Radiology, University of Michigan Health System, Ann Arbor, Michigan 48109-0028, USA.

Unlabelled: Appearances of and increases in Graves' ophthalmopathy (GO) have been reported after treatment of patients with hyperthyroidism with radioiodine. We sought to determine the rates of appearance or increase in manifestations of GO in American patients treated with radioiodine for hyperthyroidism.

Methods: The study population, which consisted of 76 patients (range, 10.6-72 y), included 61 women and individuals of diverse ethnicity. The patients were followed for 1 y after radioiodine treatment. The clinical activity score (CAS) included 10 items of ophthalmic change that were evaluated at 2 and 6 mo and at 1 y; appearance of a new item scored 1 point. We evaluated interactions of 6 covariates-prolonged hyperthyroidism, prolonged hypothyroidism, smoking, treatment with an antithyroid drug (ATD), and serum levels of thyroid-stimulating immunoglobulin (TSI) and of high free T3 (FT3)--with the numbers of patients with 2 or more CAS points and with exophthalmometer readings increased by at least 2 mm. In addition, patients completed a scored quality-of-life (QOL) questionnaire at baseline and at 1 y to assess eye symptoms.

Results: The mean CAS points for all patients at 2 mo was 0.63 and was not significantly different at 1 y. In 9 of 10 CAS items, there were few patients affected at 1 y and for the most part there were fewer patients affected than at baseline. However, exophthalmometer readings increased in 39% of patients by a mean of 2.6 mm. Individual patients frequently exhibited increases and decreases in item manifestations. Exophthalmometer readings decreased by 2 mm or less in 13%. Of the covariates, only hyperthyroidism prolonged by at least 2.5 mo was significantly associated with 2 or more CAS points at 1 y; no covariate was significantly associated with the development of increased exophthalmometer readings. Eye symptoms recorded in the QOL were insignificantly improved over the year; symptoms did not correlate with CAS points or with exophthalmometer readings.

Conclusion: After radioiodine treatment, no substantial change was seen in manifestations of CAS items except for a modest increase in exophthalmometer readings in 39% of patients. Manifestations of CAS items frequently appeared and disappeared. Prolonged hyperthyroidism is best avoided. Ocular symptoms were insignificantly fewer at 1 y after radioiodine therapy. The observed changes do not warrant prophylactic treatment of patients with steroids.
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http://dx.doi.org/10.2967/jnumed.107.049437DOI Listing
June 2008