A case of venous overload choroidopathy in the setting of superior vena cava syndrome.

Purpose: To report a case of overload venous choroidopathy in a patient with superior vena cava syndrome. Observations: A patient presented with episcleral vessel dilation, bilateral subretinal fluid accumulations in the maculae and unilateral serous choroidal detachment. He had a medical history of kidney transplantation and was on chronic corticosteroids. Separately he had also experienced recurrent bilateral innominate vein occlusion and superior vena cava stenosis, consistent with a diagnosis of superior vena cava syndrome. His presentation was further complicated by a retinal vein occlusion in the left eye which was treated with anti-vascular endothelial growth factor intravitreal injections. The bilateral subretinal fluid accumulations responded well to photodynamic therapy. Conclusions and Importance: We report a constellation of findings including venous overload choroidopathy and retinal vein occlusion as ocular manifestations of superior vena cava syndrome. The pathophysiologic changes leading to these findings could aid in the understanding of these related conditions.

PURTSCHER-LIKE RETINOPATHY ASSOCIATED WITH ATYPICAL HEMOLYTIC UREMIC SYNDROME: CASE REPORT AND REVIEW OF OUTCOMES.

PURPOSE: The purpose of this study was to report the case of a patient presenting with newly diagnosed atypical hemolytic uremic syndrome (aHUS) and Purtscher-like retinopathy. METHODS: This is an observational case report and review of literature. A 38-year-old woman presented with 3 months of rashes, fevers, arthralgias, and abdominal pain. Initial workup was suggestive of hypereosinophilic syndrome or adult-onset Still's disease. The patient developed acute renal failure and progressively blurry vision bilaterally over the course of 5 days. Funduscopic examination was notable for numerous Purtscher flecken and cotton-wool spots, with rare intraretinal hemorrhages at the posterior pole. The constellation of renal failure, hemolytic anemia, and thrombocytopenia prompted a workup for thrombotic microangiopathy that was remarkable for a mutation in the gene coding for complement protein C9. RESULTS: The patient was diagnosed with aHUS and treated with intravenous pulse dose steroids for 3 days and an extended course of eculizumab. The patient's renal failure resolved, and her visual acuity improved, although she had residual visual field constriction and developed bilateral optic atrophy. Outcomes of other cases of Purtscher-like retinopathy related to aHUS are reviewed. CONCLUSION: Purtscher-like retinopathy is a rare but severe ophthalmic complication of aHUS. Eculizumab is an effective treatment for the systemic illness caused by aHUS, and anatomical resolution of Purtscher-like retinopathy may follow, although visual prognosis remains guarded. Recovery of visual acuity may lag behind resolution of macular edema in these patients.

Perifoveal Chorioretinal Atrophy after Subretinal Voretigene Neparvovec-rzyl for RPE65-Mediated Leber Congenital Amaurosis.

PURPOSE: To report an anatomic change following subretinal injection of voretigene neparvovec-rzyl (VN) for RPE65-mediated Leber congenital amaurosis. DESIGN: Multicenter, retrospective chart review. PARTICIPANTS: Patients who underwent subretinal VN injection at each of 4 participating institutions. METHODS: Patients were identified as having perifoveal chorioretinal atrophy if (1) the areas of atrophy were not directly related to the touch-down site of the subretinal cannula; and (2) the area of atrophy progressively enlarged over time. Demographic data, visual acuity, refractive error, fundus photographs, OCT, visual fields, and full-field stimulus threshold (FST) were analyzed. MAIN OUTCOME MEASURES: Outcome measures included change in visual acuity, FST, visual fields, and location of atrophy relative to subretinal bleb position. RESULTS: A total of 18 eyes of 10 patients who underwent subretinal injection of VN were identified as having developed perifoveal chorioretinal atrophy. Eight of 10 patients (80%) developed bilateral atrophy. The mean age was 11.6 years (range, 5-20 years), and 6 patients (60%) were male. Baseline mean logarithm of the minimum angle of resolution visual acuity and FST were 0.82 (standard deviation [SD], 0.51) and -1.3 log cd.s/m2 (SD, 0.44), respectively. The mean spherical equivalent was -5.7 diopters (D) (range, -11.50 to +1.75 D). Atrophy was identifiable at an average of 4.7 months (SD, 4.3) after surgery and progressively enlarged in all cases up to a mean follow-up period of 11.3 months (range, 4-18 months). Atrophy developed within and outside the area of the subretinal bleb in 10 eyes (55.5%), exclusively within the area of the bleb in 7 eyes (38.9%), and exclusively outside the bleb in 1 eye (5.5%). There was no significant change in visual acuity (P = 0.45). There was a consistent improvement in FST with a mean improvement of -3.21 log cd.s/m2 (P < 0.0001). Additionally, all 13 eyes with reliable Goldmann visual fields demonstrated improvement, but 3 eyes (23.1%) demonstrated paracentral scotomas related to the atrophy. CONCLUSIONS: A subset of patients undergoing subretinal VN injection developed progressive perifoveal chorioretinal atrophy after surgery. Further study is necessary to determine what ocular, surgical delivery, and vector-related factors predispose to this complication.

Incidence of Proliferative Diabetic Retinopathy and Other Neovascular Sequelae at 5 Years Following Diagnosis of Type 2 Diabetes.

OBJECTIVE: To determine the incidence and risk factors for developing proliferative diabetic retinopathy (PDR), tractional retinal detachment (TRD), and neovascular glaucoma (NVG) at 5 years after the initial diagnosis of type 2 diabetes. RESEARCH DESIGN AND METHODS: Insured patients aged ≥18 years with newly diagnosed type 2 diabetes and 5 years of continuous enrollment were identified from a nationwide commercial claims database containing data from 2007 to 2015. The incidences of PDR, TRD, and NVG were computed at 5 years following the index diagnosis of type 2 diabetes. Associations between these outcomes and demographic, socioeconomic, and medical factors were tested with multivariable logistic regression. RESULTS: At 5 years following the initial diagnosis of type 2 diabetes, 1.74% (1,249 of 71,817) of patients had developed PDR, 0.25% of patients had developed TRD, and 0.14% of patients had developed NVG. Insulin use (odds ratio [OR] 3.59, 95% CI 3.16-4.08), maximum HbA1c >9% or >75 mmol/mol (OR 2.10, 95% CI 1.54-2.69), renal disease (OR 2.68, 95% CI 2.09-3.42), peripheral circulatory disorders (OR 1.88, 95% CI 1.25-2.83), neurological disease (OR 1.62, 95% CI 1.24-2.11), and older age (age 65-74 years) at diagnosis (OR 1.62, 95% CI 1.28-2.03) were identified as risk factors for development of PDR at 5 years. Young age (age 18-23 years) at diagnosis (OR 0.46, 95% CI 0.29-0.74), Medicare insurance (OR 0.60, 95% CI 0.70-0.76), morbid obesity (OR 0.72, 95% CI 0.59-0.87), and smoking (OR 0.84, 95% CI 0.70-1.00) were identified as protective factors. CONCLUSIONS: A subset of patients with type 2 diabetes develop PDR and other neovascular sequelae within the first 5 years following the diagnosis with type 2 diabetes. These patients may benefit from increased efforts for screening and early intervention.

Protection of Retinal Function by Nucleoside Reverse Transcriptase Inhibitors Following Retinal Ischemia/Reperfusion Injury.

Purpose: Retinal ischemia/reperfusion (I/R) injury is a common cause of visual impairment and blindness for which there remain limited treatment options. Nucleoside reverse transcriptase inhibitors (NRTIs), such as zidovudine (AZT), have been shown to block the NLRP3 inflammasome and prevent retinal degeneration in a mouse model of age-related macular degeneration. The NLRP3 inflammasome has also been shown to be triggered in I/R injury. Therefore, we studied the neuroprotective effects of AZT using a pressure-induced retinal ischemia mouse model. Methods: C57BL/6J mice were randomly assigned to 1 of 2 treatment groups: vehicle-treated retinal I/R injury (n = 6) or AZT-treated retinal I/R injury (n = 6). Vehicle (1% dimethyl sulfoxide [DMSO] in phosphate-buffered saline [PBS]) or AZT 50 mg/kg in 1% DMSO in PBS were injected intraperitoneally twice daily for 5 days. On day 2 of treatment, retinal ischemia was induced by transient elevation of intraocular pressure for 45 min. Scotopic electroretinography (ERG) was used to quantify retinal function before and 1 week after retinal ischemic insult. Retinal morphology was examined 1 week after ischemic insult. Terminal deoxynucleotidyl transferase dUTP nick-end labeling (TUNEL) assays and caspase 1 immunostaining was performed 24 h after retinal I/R injury. Results: Following I/R injury, ERG a- and b-wave amplitudes were significantly reduced in the vehicle-treated mice. AZT treatment significantly attenuated I/R-induced loss of retinal function as compared with vehicle-treated mice. Additionally, AZT-treated mice experienced significantly less inner retinal thinning as compared with vehicle-treated mice. TUNEL-positive cells were prevalent in the vehicle-treated I/R injury mouse retinas compared with the AZT-treated I/R injury mouse retinas. More caspase-1 immunoreactivity was detected in ganglion cell layer and inner nuclear layer (INL) in vehicle-treated I/R injury group than in AZT-treated I/R injury group. Conclusion: AZT treatment resulted in relative preservation of retinal structure and function following ischemic insult as compared with controls. This suggests AZT may have therapeutic value in the management of retinal ischemic diseases.

Rates of Eye Care and Diabetic Eye Disease among Insured Patients with Newly Diagnosed Type 2 Diabetes.

PURPOSE: To determine rates of eye examinations and diabetic eye disease in the first 5 years after diagnosis of type 2 diabetes (DM2) among continuously insured adults. DESIGN: Retrospective, longitudinal cohort study. PARTICIPANTS: Insured patients aged 40 years or older with newly diagnosed DM2 (n = 42 684), and control patients without diabetes matched on age, sex, and race were identified from a nationwide commercial claims database containing data from 2007 to 2015. METHODS: All patients were tracked for 6 years: 1 year before and 5 years after the index diabetes diagnosis. Receipt of eye care for individual patients was identified using International Classification of Diseases 9th edition (ICD-9) procedure codes or Current Procedural Terminology (CPT) codes indicating an eye examination, as well as encounters indicating the patient was seen by an ophthalmologist. A diagnosis of diabetic eye disease was determined by using ICD-9 codes. MAIN OUTCOME MEASURES: Outcome measures included annual receipt of eye care and development of diabetic eye disease, namely, diabetic retinopathy (DR). Associations between these outcomes and demographic factors were tested with multivariable logistic regression. RESULTS: Diabetic patients received more eye examinations than controls in each year, but no more than 40.4% of diabetic patients received an examination in any given year. Patients with Medicare Advantage received fewer eye examinations at 5 years (odds ratio [OR], 0.79; P < 0.01) than those with private insurance but were less likely to develop DR (OR, 0.71; P < 0.01). Hispanic patients had higher rates of DR (OR, 1.60; P < 0.01) and received fewer eye examinations (OR, 0.75; P < 0.01) at 5 years compared with White patients. Men received fewer eye examinations (OR, 0.84; P < 0.01) and were more likely to develop DR at 5 years (OR, 1.17; P < 0.01) than women. Patients with higher education were more likely to receive an eye examination and less likely to develop DR. CONCLUSIONS: The majority of diabetic patients do not receive adequate eye care within the 5 years after initial diabetes diagnosis despite having insurance. Efforts should be made to improve adherence to screening guidelines, especially for vulnerable populations.

Assessing the ocular surface microbiome in severe ocular surface diseases.

PURPOSE: There is growing evidence for a critical role of the microbiome in ocular health and disease. We performed a prospective, observational study to characterize the ocular surface microbiome (OSM) in four chronic ocular surface diseases (OSDs) and healthy controls. METHODS: Sterile swabs were used to collect samples from each eye of 39 patients (78 eyes). Sterile technique and multiple controls were used to assess contamination during DNA extraction, amplification and sequencing. Concurrent use of topical antibiotics, steroids, and bandage contact lenses (BCLs) was documented. RESULTS: Despite the low biomass of the ocular surface, 47/78 (60%) eyes sampled had positive sequencing reads. We observed that half of patients (8/17, 47%) had distinct microbiomes in each eye. Healthy controls had a Lactobacillus/Streptococcus mixture or significant Corynebacterium. Staphylococcus predominated in 4/7 (57%) patients with Stevens-Johnson Syndrome (SJS) in at least one eye, compared to 0/10 healthy controls. Interestingly, 8/11 (73%) eyes with SJS were using BCLs, including 4/5 (80%) eyes dominated by Staphylococcus. Lax eyelid syndrome (LES) and Dry Eye Disease (DED) patients had similar OSMs, with Corynebacterium being the most prevalent bacteria. Alpha diversity was higher in controls and ocular graft-vs-host (oGVHD) patients compared to the other OSDs. CONCLUSIONS: Only 50% of the 39 patients had similar microbiomes in each eye. A majority of healthy eyes had a Lactobacillus/Streptococcus mix or Corynebacterium microbiome. Staphylococcus predominated in SJS, Lactobacillus in oGVHD, and Corynebacterium in DED and LES. There may be an association between different OSDs and the microbiome.

Thirty-day hospital revisits after prostate brachytherapy: who is at risk?

BACKGROUND: Transperineal prostate brachytherapy is a common outpatient procedure for the treatment of prostate cancer. Whereas long-term morbidity and toxicities are widely published, rates of short-term complications leading to hospital revisits have not been well described. MATERIALS AND METHODS: Patients who underwent brachytherapy for prostate cancer in an ambulatory setting were identified in the Healthcare Cost and Utilization Project State Ambulatory Surgery Database for California between 2007 and 2011. Emergency department (ED) visits and inpatient admissions within 30 days of treatment were determined from the California Healthcare Cost and Utilization Project State Emergency Department Database and State Inpatient Database, respectively. RESULTS: Between 2007 and 2011, 9,042 patients underwent brachytherapy for prostate cancer. Within 30 days postoperatively, 543 (6.0%) patients experienced 674 hospital encounters. ED visits comprised most encounters (68.7%) at a median of 7 days (interquartile range 2-16) after surgery. Inpatient hospitalizations occurred on 155 of 674 visits (23.0%) at a median of 12 days (interquartile range 5-20). Common presenting diagnoses included urinary retention, malfunctioning catheter, hematuria, and urinary tract infection. Logistic regression demonstrated advanced age {65-75 years: odds ratio [OR], 1.3 [95% confidence interval (CI) 1.06-1.60, P = 0.01]; >75 years: OR 1.5 [95% CI 1.18-1.97, P = 0.001]}, inpatient admission within 90 days before surgery [OR 2.68 (95% CI 1.8-4.0, P < 0.001)], and ED visit within 180 days before surgery [OR 1.63 (95% CI 1.4-1.89, P < 0.001)] as factors that increased the risk of hospital-based evaluation after outpatient brachytherapy. Charlson comorbidity score did not influence risk. CONCLUSIONS: ED visits and inpatient admissions are not uncommon after prostate brachytherapy. Risk of revisit is higher in elderly patients and those who have had recent inpatient or ED encounters.

IGG4-RELATED OPHTHALMIC DISEASE PRESENTING AS CHOROIDAL AND ORBITAL LESIONS.

PURPOSE: To report a case of IgG4-related ophthalmic disease (IgG4-ROD) which presented as choroidal and orbital lesions. METHODS: Case report. RESULTS: A 64-year-old man presented with left eye photopsias and a history of IgG4-related perirenal fibrosis. Fundoscopic examination showed multiple bilateral yellow choroidal lesions, and optical coherence tomography showed multiple choroidal lesions. Magnetic resonance imaging of the orbits showed an enhancing lesion present circumferential to the optic nerve, but greater medially, abutting the posterior surface of the left globe. Workup for infectious, autoimmune, and malignant etiologies was negative, and the patient has responded well to treatment with rituximab. CONCLUSION: IgG4-related disease is a systemic fibroinflammatory disease, which often presents in another location, as in our patient. In cases of uncertain choroidal and orbital lesions, a thorough workup for other etiologies is indicated, and lymphoma must be ruled out. Steroids are the mainstay of treatment for IgG4-ROD, however, small case series and our patient responded well to rituximab. To our knowledge, this is the first reported case of choroidal and orbital lesions secondary to IgG4-ROD.

The LACE Score as a Tool to Identify Radical Cystectomy Patients at Increased Risk of 90-Day Readmission and Mortality.

INTRODUCTION: Radical cystectomy for bladder cancer is associated with high rates of readmission. We investigated the LACE score, a validated prediction tool for readmission and mortality, in the radical cystectomy population. MATERIALS & METHODS: Patients who underwent radical cystectomy for bladder cancer were identified by ICD-9 codes from the Healthcare Cost and Utilization Project State Inpatient Database for California years 2007-2010. The LACE score was calculated as previously described, with components of L: length of stay, A: acuity of admission, C: comorbidity, and E: number of emergency department visits within 6 months preceding surgery. RESULTS: Of 3,470 radical cystectomy patients, 638 (18.4%) experienced 90-day readmission, and 160 (4.6%) 90-day mortality. At a previously validated "high-risk" LACE score ≥ 10, patients experienced an increased risk of 90-day readmission (22.8 vs. 17.7%, p = 0.002) and mortality (9.1 vs. 3.5%, p < 0.001). On adjusted multivariable analysis, "high risk" patients by LACE score had increased 90-day odds of readmission (adjusted OR = 1.24, 95% CI: 0.99-1.54, p = 0.050) and mortality (adjusted OR = 2.09, 95% CI: 1.47-2.99, p < 0.001). CONCLUSION: The LACE score reasonably identifies patients at risk for 90-day mortality following radical cystectomy, but only poorly predicts readmission. Providers may use the LACE score to target high-risk patients for closer follow-up or intervention.

Ophthalmic Complications Following Acoustic Neuroma Resection.

BACKGROUND: Past studies have shown high rates of ocular complications with the need for ophthalmic surgery following acoustic neuroma resection (ANR). OBJECTIVE: To determine the rates of ophthalmic complications, referrals, and surgery following ANR, and the factors associated with poor outcomes. METHODS: A retrospective study of ophthalmic outcomes in patients who underwent ANR was conducted, following institutional review board approval. Surgical approach, tumor size, tumor characteristics, completeness of resection, postoperative House-Brackmann grades, ocular complications, referrals to ophthalmology, and ophthalmic treatments were recorded. RESULTS: Between 2007 and 2012, 174 patients underwent ANR. There were 3 surgical groups: retrosigmoid (n = 97), translabyrinthine (n = 59), and combined retrosigmoid and translabyrinthine (n = 18). Median tumor size was 2.2 cm. Postoperatively, 30% of patients had facial nerve dysfunction (House-Brackmann ≥3), which recovered to 19% by 1 mo and 8.6% by 1 yr following ANR. Fifty-six (32.9%) patients experienced ocular complications postoperatively, with lagophthalmos, dry eye, and blurry vision as the most common complications. Thirty-six (67.9%) of the patients who required ophthalmic treatment were managed nonsurgically, with just 13 (7.6%) patients requiring referral to an ophthalmologist. In total, only 9 (5.3%) patients received an ophthalmic procedure. Patients with tumors >2 cm, those undergoing combined retrosigmoid and translabyrinthine resection, and those with severe facial nerve dysfunction which did not improve in the first month following surgery were more likely to have poor ophthalmic outcomes. CONCLUSION: We present lower rates of ophthalmic complications following ANR than previously reported. Improved surgical technique, better postoperative eye care, and facial nerve monitoring most likely accounted for the improved ocular outcomes.

A Case of Renal Metastasis of Uterine Leiomyosarcoma.

A 49-year-old woman with a distant history of uterine leiomyosarcoma underwent robotic-assisted laparoscopic partial nephrectomy for a 3.5 cm right renal mass, which was presumed to be a primary renal cell carcinoma. Surgical margins were negative, and the histologic analysis confirmed leiomyosarcoma. Uterine leiomyosarcoma is traditionally a locally aggressive disease with only rare reports of renal involvement. We report a case of a metastatic leiomyosarcoma to the kidney four years following initial treatment for uterine leiomyosarcoma.

Oligometastatic Growing Teratoma Syndrome: A Case for an Aggressive Surgical Approach.

Growing teratoma syndrome is an infrequent presentation of testicular cancer. We present a case of growing teratoma syndrome in a patient who initially presented with clinical stage I nonseminomatous testicular germ cell tumor, who subsequently developed large volume oligometastases to the retroperitoneum, thorax, and thigh. Despite two regimens of chemotherapy, his disease progressed. Complete surgical extirpation of all gross tumors confirmed mature teratoma. An aggressive surgical approach, including postchemotherapy resection of all known metastatic sites, can provide long-term disease-free survival.