Contemporary Outcomes After Partial Resection of Infected Aortic Grafts.

INTRODUCTION: Aortic graft infection remains a considerable clinical challenge, and it is unclear which variables are associated with adverse outcomes among patients undergoing partial resection. METHODS: A retrospective, multi-institutional study of patients who underwent partial resection of infected aortic grafts from 2002 to 2014 was performed using a standard database. Baseline demographics, comorbidities, operative, and postoperative variables were recorded. The primary outcome was mortality. Descriptive statistics, Kaplan-Meier (KM) survival analysis, and Cox regression analysis were performed. RESULTS: One hundred fourteen patients at 22 medical centers in 6 countries underwent partial resection of an infected aortic graft. Seventy percent were men with median age 70 years. Ninety-seven percent had a history of open aortic bypass graft: 88 (77%) patients had infected aortobifemoral bypass, 18 (16%) had infected aortobiiliac bypass, and 1 (0.8%) had an infected thoracic graft. Infection was diagnosed at a median 4.3 years post-implant. All patients underwent partial resection followed by either extra-anatomic (47%) or in situ (53%) vascular reconstruction. Median follow-up period was 17 months (IQR 1, 50 months). Thirty-day mortality was 17.5%. The KM-estimated median survival from time of partial resection was 3.6 years. There was no significant survival difference between those undergoing in situ reconstruction or extra-anatomic bypass (P = 0.6). During follow up, 72% of repairs remained patent and 11% of patients underwent major amputation. On univariate Cox regression analysis, Candida infection was associated with increased risk of mortality (HR 2.4; P = 0.01) as well as aortoenteric fistula (HR 1.9, P = 0.03). Resection of a single graft limb only to resection of abdominal (graft main body) infection was associated with decreased risk of mortality (HR 0.57, P = 0.04), as well as those with American Society of Anesthesiologists classification less than 3 (HR 0.35, P = 0.04). Multivariate analysis did not reveal any factors significantly associated with mortality. Persistent early infection was noted in 26% of patients within 30 days postoperatively, and 39% of patients were found to have any post-repair infection during the follow-up period. Two patients (1.8%) were found to have a late reinfection without early persistent postoperative infection. Patients with any post-repair infection were older (67 vs. 60 years, P = 0.01) and less likely to have patent repairs during follow up (59% vs. 32%, P = 0.01). Patients with aortoenteric fistula had a higher rate of any post-repair infection (63% vs. 29%, P < 0.01) CONCLUSION: This large multi-center study suggests that patients who have undergone partial resection of infected aortic grafts may be at high risk of death or post-repair infection, especially older patients with abdominal infection not isolated to a single graft limb, or with Candida infection or aortoenteric fistula. Late reinfection correlated strongly with early persistent postoperative infection, raising concern for occult retained infected graft material.

Subretinal Injection of Voretigene Neparvovec-rzyl in a Patient With RPE65-Associated Leber's Congenital Amaurosis.

Leber's congenital amaurosis (LCA) is a rare inherited retinal degeneration (IRD) that causes severe vision loss, nyctalopia, and nystagmus within the first few years of life. RPE65 gene mutations cause approximately 6% of LCA cases and have become the target for therapy since voretigene neparvovec-rzyl became the first U.S. Food and Drug Administration-approved gene therapy product for IRDs in 2017. The surgery involves pars plana vitrectomy with subretinal injection of a viral vector that carries a functional copy of the RPE65 gene. Intraoperative optical coherence tomography is a useful adjunctive tool to confirm the injection has reached the subretinal space.

Vitreoretinal lymphoma followed by systemic diffuse large B cell lymphoma.

Vitreoretinal lymphoma as the presenting diagnosis in association with a systemic lymphoma without central nervous system involvement is exceedingly rare, and the classification of this condition is not well-established. Here, we describe a patient with intermittent blurry vision in the left eye for 2 years in the setting of a recent incidental diagnosis of diffuse large B cell lymphoma from an axillary lymph node biopsy. The diagnosis of panuveitis with an extensive exudative retinal detachment was made. The patient was treated with pars plana vitrectomy as well as systemic chemotherapy, intrathecal methotrexate, intravitreal methotrexate, and intravitreal rituximab with good post-operative outcomes.

Circumferential retinal hemorrhages after ophthalmic examination with scleral depression in an infant with anti-VEGF treated retinopathy of prematurity.

We report a case of retinal hemorrhages in a baby with retinopathy of prematurity (ROP) following examination with indirect ophthalmoscopy and scleral depression. There have been rare reports of examination-induced retinal hemorrhages during ROP screening, although those hemorrhages were diffusely scattered in the posterior pole. In this report the hemorrhages were found on the surface of the neovascular ridge. Changes in intraocular pressure caused by scleral depression may result in rupture of the fragile and immature retinal vessels, which have poor autoregulation in these premature babies. Ophthalmologists performing ROP screening examinations should be aware of the possibility of causing retinal hemorrhages with scleral depression, although the hemorrhages will resolve spontaneously.

An Eight-Year-Old Girl Presents With Two Weeks of Vision Loss and Nightly Headaches.

Intraocular medulloepithelioma is a rare congenital tumor that arises from the nonpigmented epithelium of the ciliary body. It is the second most common primary intraocular neoplasm during the first decade of life. It may present with an iris mass or cyst (56%), glaucoma (48%), cataract (26%), leukocoria (18%), decrease in vision (41%), or pain (30%). Here, the authors present a case of a medulloepithelioma investigated with multimodal imaging, including the first characterization with intraoperative optic coherence tomography. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:314-317.].

A Novel Pathogenic Variant in NDP Gene With Incomplete Penetrance Manifests as X-Linked Familial Exudative Vitreoretinopathy.

Familial exudative vitreoretinopathy (FEVR) is a rare hereditary ocular disorder characterized by incomplete or abnormal development of peripheral retinal vasculature. The genes responsible for this disorder are associated with the wingless-related integration site (Wnt) signaling pathway, a critical pathway for the development of normal retinal vasculature. A pathogenic variant in any one of these genes may disrupt retinal vasculogenesis. Furthermore, the type and number of pathogenic variants may influence the severity of disease and clinical course. Here, the authors identify a novel pathogenic variant in the NDP gene, not previously described in the literature. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:120-124.].

Microcornea, posterior megalolenticonus, persistent fetal vasculature, chorioretinal coloboma (MPPC) syndrome: Case series post vitrectomy.

PURPOSE: MPPC syndrome has been described as a syndrome that presents with chorioretinal coloboma, posterior megalolenticonus, persistent fetal vasculature, and chorioretinal coloboma. The purpose of our study is to report three patients who present with a variation of MPPC syndrome who each underwent pars plana vitrectomy, pars plana lensectomy, and amblyopic management. Clinical characteristics, ancillary test findings, and post-surgical functional results are compared to what is reported in the literature. METHODS: Retrospective review of medical records of patients who presented with microcornea, persistent fetal vasculature, chorioretinal coloboma, and microphthalmia who underwent surgical correction at Bascom Palmer Eye Institute. RESULTS: 3 patients (6 eyes) were included, two males and one female. All patients were born full term, vaginally, and had no family history of genetic abnormalities affecting the eye. All patients had color fundus images, fluorescein angiography (FA) and echography. Four eyes underwent surgery. Following surgical intervention, patients demonstrated improved visual acuity, and improved functional status. CONCLUSION AND IMPORTANCE: Patients with microcornea, PFV, chorioretinal coloboma, and microphthalmos, can benefit from surgical intervention when functional decline or media opacities are noted. Though difficult to assess accurate visual acuity and visual improvement pre-operatively and post-operatively, it is evident that our patients demonstrated improvement in functionality and vision following surgical intervention.

Comparison of Athletes and Nonathletes Undergoing Thoracic Outlet Decompression for Neurogenic Thoracic Outlet Syndrome.

BACKGROUND: Neurogenic thoracic outlet syndrome (NTOS) is the most common form of thoracic outlet syndrome (TOS) and may occur from injury, occupational stress, or athletic endeavors. Although most patients with NTOS will improve after first-rib resection and scalenectomy (FRRS), the prognostic risk factors for success remain unclear. Athletes are a very motivated and disciplined demographic and therefore should be a group more likely to respond to FRRS for NTOS than nonathletes. We hypothesized that athletes would do better after FRRS than nonathletes despite the added physical stress that sporting activity imposes. METHODS: We reviewed our office records for all patients treated for TOS from July 2009 to May 2014 and extracted demographic, historical, procedural, and follow-up data. We contacted these patients to complete a survey to assess patient-centered outcomes of FRRS and compared athlete versus nonathlete survey responses. RESULTS: Five hundred sixty-four patients had FRRS for NTOS, and 184 (33%) responded to the survey. Of the 184 who responded, 97 were athletes (53%) and 87 were nonathletes (47%). Survey results suggested that 87% were improved in pain medication use (athletes 93% vs. nonathletes 80%, P = 0.013), 77% would undergo FRRS on the contralateral side if needed (athletes 75% vs. nonathletes 79%, P = 0.49), 73% had resolution of TOS symptoms (athletes 80% vs. nonathletes 65%, P = 0.02), and 86% could perform activities of daily living without limitation (athletes 95% vs. nonathletes 77%, P = 0.0004). Although 24% of respondents required another non-TOS procedure (athletes 27% vs. nonathletes 22%, P = 0.6), 89% felt that they had made the right decision (athletes 93% vs. nonathletes 80%, P = 0.09). Multivariable analysis of age, race, gender, previous surgery, preoperative physical therapy, preoperative narcotic use, and athletic status confirmed that athletic status was a significant predictor for improvement in pain medication use, complete TOS resolution, and the ability to perform activities of daily living. CONCLUSIONS: Most patients undergoing FRRS for NTOS are improved and satisfied with the result and indicate they made the correct choice to have FRRS. Although being an athlete was an independent variable for better outcomes in activity and pain medication use, their satisfaction after FRRS was similar to that in nonathletes. Further investigation is needed to determine if these findings are due to physical and/or psychosocial factors.

Retinal vasproliferative tumor in a case of X-linked retinoschisis detachment.

PURPOSE: To describe the first published case of X-linked retinoschisis (XLRS) detachment with retinal vasoproliferative tumor (RVPT) and provide a literature review of the subject. OBSERVATIONS: The authors describe a case of a 17 year old male with X-linked retinoschisis who presented with a retinal detachment and a retinal vasoproliferative tumor. The patient was treated with pars plana vitrectomy, endolaser, subtenon's kenalog and anti-VEGF (vascular endothelial growth factor) intravitreal injections. He regained 20/60 vision with a flat macula and had significant resolution of the associated vasoproliferative leakage seen on fluorescein angiography. CONCLUSIONS AND IMPORTANCE: This case adds XLRS to the conditions associated with RVPT and gives support for treatment with laser photocoagulation and anti-VEGF therapy with bevacizumab to control the exudative process.

Clinical features, antimicrobial susceptibilities, and treatment outcomes of patients with culture positive endophthalmitis after penetrating keratoplasty.

PURPOSE: To report the clinical features organisms and treatment outcomes in patients with endophthalmitis after penetrating keratoplasty (PK). METHODS: Retrospective noncomparative case series. RESULTS: Eleven eyes of 11 patients with culture positive endophthalmitis after PK were included. The time to diagnosis of endophthalmitis from last PK was less than 1 week in 3/11 (27%), between 1 and 4 weeks in 3/11 (27%), and greater than one month in 5/11 (46%) (range 2-924 days). The distribution of isolates included gram positive (GP) 9/11 (82%), gram negative (GN) 1/11 (9%), and fungal 1/11 (9%) species, respectively. Of GP bacteria tested, 9/9 (100%) were sensitive to Vancomycin. Of fungal isolates tested, none (0/1) were sensitive to Amphoteracin, Fluconazole, and/or Voriconazole. Among patients with rim culture data available, 1/7 (14%) donor rims were culture positive for Candida glabrata and 6/7 (86%) were culture negative. Patients were treated with primary tap and inject in 10/11 (91%) and primary vitrectomy in 1/11 (9%). VA of ≥5/200 was present in 2/11 (18%) at time of endophthalmitis diagnosis, and was recorded in 6/11 (55%) at last follow-up. CONCLUSIONS AND IMPORTANCE: Patients with endophthalmitis after PK presented at variable time points after surgery. Gram positive organisms were the most common isolate. VA outcomes after treatment were generally poor.

Management of Retinopathy of Prematurity--Use of Anti-VEGF Therapy.

Retinopathy of prematurity (ROP) is a leading cause of childhood blindness worldwide. Although laser photocoagulation re-mains the gold standard for treating threshold and prethreshold disease (type 1 ROP), the off-label use of anti‒vascular endothelial growth factor (anti-VEGF) therapy to treat ROP is increasing. Benefits include acute regression of ROP, growth of retinal vasculature beyond the demarcation line, lesser degree of myopia and peripheral visual field loss, and avoidance of sedation and intubation required for laser. However, controversies regarding anti-VEGF in this vulnerable population persist including choice of anti-VEGF agent, dosing, systemic absorption, safety, and late recurrence. This review updates recent evidence regarding the use of anti-VEGF therapy in the management of ROP.

Clinical Features, Antibiotic Susceptibilities, and Treatment Outcomes of Endophthalmitis Caused by Staphylococcus epidermidis.

PURPOSE: To report the clinical features, antibiotic susceptibilities, and visual acuity outcomes with endophthalmitis caused by methicillin-sensitive Staphylococcus epidermidis and methicillin-resistant S. epidermidis. DESIGN: Retrospective case series. PARTICIPANTS: All patients seeking treatment at a tertiary referral center between 2006 and 2016 with endophthalmitis caused by S. epidermidis. METHODS: All records were reviewed for patients with a clinical diagnosis of endophthalmitis and positive vitreous culture results for S. epidermidis. Data were compared with the prior series at the same institution. MAIN OUTCOME MEASURES: Clinical settings, antibiotic susceptibilities, and visual acuity. RESULTS: Among 96 eyes of 96 patients, the most common postprocedural clinical settings were cataract surgery (47/96 [49%]), intravitreal injection (21/96 [22%]), trauma (8/96 [8%]), glaucoma surgery (7/96 [7%]), and penetrating keratoplasty (5/96 [5%]). The initial treatment included intravitreal vancomycin and ceftazidime in 89 of 96 eyes (93%) and intravitreal vancomycin and amikacin in 7 of 96 eyes (7%). A vitreous tap and injection with antibiotics was performed as the initial treatment in 83 of 96 eyes (86%) and pars plana vitrectomy was performed in 13 of 96 eyes (14%). All isolates were sensitive to vancomycin in both decades. In the most recent series, visual acuity at last follow-up was 5/200 or better in 68 of 96 eyes (71%) compared with 71 of 86 eyes (83%) in the prior study. In the current study, susceptibility to methicillin and moxifloxacin was present in 45 of 96 eyes (47%) and 29 of 85 eyes (34%), respectively, compared with 34 of 86 eyes (40%) and 27 of 39 eyes (69%) in the prior study. Final visual acuity was not significantly different between those eyes that were methicillin or fluoroquinolone sensitive and those that were resistant. CONCLUSIONS: In the current and prior series, all S. epidermidis isolates were sensitive to vancomycin. Visual acuity outcomes were not dependent on methicillin or fluoroquinolone sensitivity.

Antiphosphospholipid syndrome presenting with amaurosis fugax and cotton wool spots.

PURPOSE: To describe the importance of considering vaso-occlusive disease on the differential diagnosis of a patient presenting with amaurosis fugax (AF) and unilateral cotton wool spots (CWS). OBSERVATIONS: A 69-year-old female with history of obesity, hyperlipidemia and recent orthopedic surgery, presented with 3 days of worsening monocular AF and CWS in the right eye. She was diagnosed with antiphosphospholipid syndrome based on positive serologic testing for antiphosphatidylserine IgM, anticardiolipin IgM. The patient was treated with lipid lowering medication, long-term aspirin, and has followed a weightloss and physical therapy program under medical supervision. The CWS resolved and AF symptoms have not recurred. CONCLUSIONS AND IMPORTANCE: Antiphospholipid syndrome can be considered in the differential diagnosis of patients presenting with AF, assymetric CWS, and/or rapid progression of symptoms.

Midterm and long-term follow-up in competitive athletes undergoing thoracic outlet decompression for neurogenic thoracic outlet syndrome.

BACKGROUND: Neurogenic thoracic outlet syndrome (NTOS) results from compression of the brachial plexus by the clavicle, first rib, and scalene muscles and may develop secondary to repetitive motion of the upper extremity. Athletes routinely perform repetitive motions, and sports requiring significant arm and shoulder use may put the participant at increased risk for NTOS. Competitive athletes who develop NTOS may require first rib resection and scalenectomy (FRRS) for symptomatic relief. However, the effectiveness of FRRS has not previously been studied in this vulnerable population. METHODS: This is a cross-sectional study of competitive athletes with NTOS who received FRRS by the senior author between 2009 and 2014. Eligible patients were contacted by phone and invited to complete a nine-item survey assessing the long-term effects of FRRS on pain medication use, postoperative physical therapy duration, patient satisfaction, symptom relief, activities of daily living, athletic performance, time to return of athletic performance, and need for other operations. Multivariate analyses of the following risk factors were performed: age, pectoralis minor release, preoperative narcotic use, athletic shutdown, and involvement in a throwing sport. RESULTS: There were 232 competitive athletes who met the inclusion criteria, and 67 of these (age, 14-48 years; 35 male; 99% white) responded to the survey. The average time between surgery and survey completion was 3.9 years (range, 2.2-7.0 years). The most frequent sports conducted by this group were baseball and softball (n = 44 [66%]), volleyball (n = 7 [10%]), and cheerleading and gymnastics (n = 5 [7%]), ranging from high-school to professional levels. The survey results revealed that 96% were improved in pain medication use, 75% would undergo FRRS on the contralateral side if needed, 82% had resolution of symptoms, and 94% were able to perform activities of daily living without limitation; 70% returned to the same or better level of athletic activity after FRRS, and this occurred within 1 year in 50%. Multivariate regression analysis identified younger age as a predictor of the length of physical therapy and preoperative narcotics use as a predictor of symptom resolution. CONCLUSIONS: At our center, >40% of patients requiring FRRS for NTOS are competitive athletes. The results of this study show that the majority of them are able to return to their precompetitive state after FRRS, and few experience limitations in their daily living activities. Half can return to competition at or exceeding their premorbid ability level within 6 months of surgery. The majority are pleased with their decision to undergo FRRS. Further investigation is needed to identify predictive factors for successful return to competitive athletics.

Adenoid cystic carcinoma of the lacrimal gland in a 14-year-old male.

Adenoid cystic carcinoma (ACC) of the lacrimal gland is an aggressive, malignant epithelial neoplasm. This tumor is rarely seen in adults and even less commonly seen in children and adolescents; thus, there have been no large studies to date describing the optimal treatment of this malignancy in the pediatric population. Here, we report a case of lacrimal gland ACC in a 14-year-old male treated with neoadjuvant intra-arterial chemotherapy followed by globe-sparing tumor resection and chemoradiation. At 2-year follow-up, he remains disease free without evidence of tumor recurrence.

Avascular Retinal Findings in a Child With Achondroplasia.

The authors present clinical and angiographic findings in a 12-year-old girl with achondroplasia who presented with bilateral retinal peripheral nonperfusion and unilateral rhegmatogenous retinal detachment, which has not been previously described in achondroplasia. This report contributes incremental knowledge regarding aberrant retinal vascular phenomena observed in pediatric disease states and implicates the possible role of mutations in the FGFR3 gene in peripheral vascular abnormalities. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:272-274.].

Assessing the Effectiveness of Arnica montana and Rhododendron tomentosum (Ledum palustre) in the Reduction of Ecchymosis and Edema After Oculofacial Surgery: Preliminary Results.

PURPOSE: The purpose of this study was to provide preliminary data on the potential effectiveness of the combination of topical Arnica montana and Rhododendron tomentosum (Ledum palustre) in reducing postoperative ecchymosis and edema in a select population of healthy volunteers after oculofacial surgery. METHODS: This retrospective review examined the postoperative course of healthy volunteers using topical Arnica and Ledum after undergoing common oculofacial procedures, including blepharoplasty, browpexy, and rhinoplasty, in the hands of 4 surgeons at tertiary referral centers from July 1, 2012 to December 31, 2012 using medical records review. Each patient included had used topical hydrogel pads (OcuMend, Cearna Inc., Chicago, IL) containing Arnica 50 M (10) 50% and Ledum 50 M (10). The pads were applied bilaterally after surgery through postoperative day 6. At each postoperative visit, the patients were evaluated by their respective surgeons and assigned a subjective physician-patient rating score comparing each patient's observed healing compared with expected healing if not using Arnica/Ledum. Photographs of patients undergoing equivalent procedures, but not using Arnica/Ledum were used as controls for comparison. Physician-patient rating scores were categorical: markedly accelerated healing defined as approximately 7 days ahead of expected, accelerated healing, defined as <7 days ahead of expected, and no appreciable difference from expected. The proportion of patients with each physician-patient rating score was calculated for postoperative days 1 to 2, 3 to 5, 6 to 8, and overall. Difference of proportions was calculated with 95% confidence intervals using Newcombe unpaired difference comparison of proportions. Photographs documenting the clinical progression of selected patients are provided. RESULTS: A total of 27 patients (16 females, 11 males) were included in the study. Age range was 18 to 70 years. The majority of patients were white (52.9%), and underwent blepharoplasty (78.9%). The median duration of follow-up was 7 days, range 1 to 14 days. The proportions of patients with markedly accelerated healing were 38.5% (5 of 13), 85.7% (6 of 7), 60.0% (12 of 20), and 51.9% (14 of 27) at POD 1 to 2, 3 to 5, 6 to 8, and overall, respectively. The proportions of patients with accelerated healing at the same time points were 15.4% (2 of 13), 14.3% (1 of 7), 30.0% (6 of 20), and 37.0% (10 of 27), respectively. The proportions of patients with no appreciable difference at the same time points were 46.2% (6 of 13), 0% (0 of 7), 10.0% (2 of 20), and 11.1% (3 of 27) of patients, respectively. The proportion of patients using Arnica/Ledum with markedly accelerated healing was significantly more than the proportion of those demonstrating no appreciable difference from expected at POD 3 to 5 (85.7% vs. 0%, p = 0.05), POD 6 to 8 (60.0% vs. 10.0%), and overall (51.9% vs. 11.1%, p = 0.05). No adverse effects were reported. CONCLUSIONS: The preliminary results from this study demonstrate that the combination of topical Arnica montana and Rhododendron tomentosum (Ledum palustre) may be effective in reducing postoperative ecchymosis and edema after oculofacial surgery.

Mastication induced retrobulbar hemorrhage.

Retrobulbar hemorrhage is a feared potentially sight threatening complication after orbital decompression surgery. We present a patient, 36 hours after surgery, while forcefully biting, suddenly developed a retrobulbar hemorrhage arising from the temporalis muscle causing an orbital compartment syndrome. Rapid intervention with canthotomy and cantholysis was associated with recovery of vision from absent light perception to 20/20. A mastication induced retrobulbar hemorrhage has not been previously described.

Stem cell transplantation methods.

Just a few short years ago, we still used to think that we were born with a finite number of irreplaceable neurons. However, in recent years, there has been increasingly persuasive evidence that suggests that neural stem cell (NSC) maintenance and differentiation continue to take ace throughout the mammal's lifetime. Studies suggest that neural stem cells not only persist to mammalian adulthood, but also play a continuous role in brain tissue repair throughout the organism's lifespan. These preliminary results further imply that NSC transplantation strategies might have therapeutic promise in treating neurodegenerative diseases often characterized by isolated or global neuronal and glialloss. The destruction of neural circuitry in neuropathologies such as stroke, Parkinson's disease, MS, SCI prevents signals from being sent throughout the body effectively and is devastating and necessitates a cure. NSC transplantation is among one of the foremost researched fields because it offers promising therapeutic value for regenerative therapy central nervous system (CNS) diseases. Both chemotropic and exogenous cell graft mechanisms ofCNS repair are under review for their therapeutic value and it is hoped that one day, these findings will be applied to human neurodegenerative disorders. The potential applications for NSC transplantations to treat both isolated and global neurodysfunction in humans are innumerable; these prospects include inherited pediatric neurodegenerative and metabolic disorders such as lysosomal storage diseases including leukodystrophies, Sandhoff disease, hypoxic-ischemic encephalopathy and adult CNS disorders characterized by neuronal or glial cell loss such as Parkinson's disease, multiple sclerosis, stroke and spinal cord injury.