Ellen Mitchell

Lymphoma of the orbit masquerading as Tolosa-Hunt syndrome

BackgroundTolosa-Hunt syndrome is a rare clinical syndrome characterized by painful ophthalmoplegia and ipsilateral cranial neuropathies. It is caused by an inflammatory process of unknown etiology.Case presentationWe present a case of a 77-year-old white man with history of Waldenstrom’s macroglobulinemia transforming to large B-cell lymphoma who presented to a community physician complaining of 4 months of isolated right retro-orbital pain and later with diplopia, ptosis, 6th nerve and pupil-sparing partial 3rd nerve palsies as well as progressive neurological findings. His clinical course was complicated by debilitating neurological symptoms and multiple hospitalizations leading to a delay in diagnosis caused by incomplete initial workup.ConclusionThis case is a reminder that lymphoproliferative disorders often mimic other neurologic disorders and that Tolosa-Hunt is a rare diagnosis that must be considered a diagnosis of exclusion.

Visual task performance in the blind with the BrainPort V100 Vision Aid.

ABSTRACT Introduction: The BrainPort® V100 Vision Aid is a non-invasive assistive device for the blind based on sensory substitution. The device translates camera images into electrotactile stimuli delivered to the tongue. The BrainPort has recently received the CE mark and FDA approval and it is currently marketed to augment, rather than replace, the traditional assistive technologies such as the white cane or guide dog. Areas covered: In this work, we will review the functional studies performed to date with the BrainPort and we will highlight the critical factors that determine device performance, including the technology behind the BrainPort, the impediments to assessing device performance, and the impact of device training and rehabilitation. Expert commentary: The BrainPort enables blind people to perceive light, identify simple objects, recognize short words, localize simple objects, and detect motion and orientation of objects. To achieve this, proper rehabilitation and training regimes are crucial.

Carcinoma-associated retinopathy in a young teenager with immature teratoma of the ovary

A 14-year-old African American girl presented with diminished vision in both eyes 1 week after undergoing an oophorectomy for a right ovarian mass. Systemic metastatic work-up was negative. Visual acuity was 20/40 in the right eye and 20/50 in the left eye. Slit-lamp biomicroscopy was unremarkable in both eyes. Fundus examination showed diffuse patchy areas of retinal pigment epithelial atrophy in the macula and peripheral retina bilaterally. Color vision had decreased in each eye. Electroretinography revealed nondetectable rod and cone responses. Both pattern and flash visual evoked potential (VEP) testing showed delayed latency in both eyes. She was treated with pulse intravenous methylprednisolone for 3 days along with intravenous immunoglobulins and rituximab, followed by systemic prednisolone and biweekly intravenous immunoglobulins and rituximab for 3 months. Antiretinal autoantibodies against 48-kDa (arrestin) and 64-kDa and 94-kDa proteins were positive, suggestive of carcinoma-associated retinopathy. After 3 months, visual acuity was 20/40 in each eye with improvement in color vision and VEP findings.

Variability in acceptance of organ offers by pediatric transplant centers and its impact on wait‐list mortality

Recent data have suggested that pediatric patients wait‐listed for a liver transplantation frequently have liver offers declined. However, factors associated with liver offer decisions and center‐level variability in practice patterns have not been explored. We evaluated United Network for Organ Sharing data on all match runs from May 1, 2007 to December 31, 2015 in which the liver was offered to ≥1 pediatric patient; the transplant recipient was ranked in the first 40 positions for the organ offer; and the donor was brain‐dead and <50 years of age. We used multilevel mixed effects models to evaluate factors associated with organ offer acceptance, among‐center variability, and the association between center‐level acceptance and wait‐list mortality. There were 4088 unique pediatric patients during the study period, comprising 27,094 match runs. Initial Model for End‐Stage Liver Disease or Pediatric End‐Stage Liver Disease score, history of exception points, recipient region, rank on match run, and geographic share type were all associated with probability of offer acceptance. There was significant among‐center variation (P < 0.001) in adjusted liver offer acceptance rates, accounting for donor, recipient, and match‐related factors (adjusted acceptance rates: median, 8.9%; range, 5.1%‐14.6%). Center‐level acceptance rates were associated with wait‐list mortality, with a >10% increase in the risk of wait‐list mortality for every 1% decrease in a centers adjusted liver offer acceptance rate (odds ratio, 1.10; 95% confidence interval, 1.01‐1.19). In conclusion, there is significant among‐center variability in liver offer acceptance rates for pediatric patients that is not explained by donor and recipient factors. A centers liver acceptance behavior significantly impacts whether a pediatric patient will be transplanted or die on the waiting list. Liver Transplantation 24 803–809 2018 AASLD.

Bilateral cavernous sinus thrombosis in a patient with tacrolimus-associated posttransplant thrombotic microangiopathy

Purpose To report a case of bilateral cavernous sinus thrombosis (CST) in a patient with tacrolimus-associated posttransplant thrombotic microangiopathy. Methods Case report. Results An 8-year-old boy with a medical history of orthotopic heart transplant, posttransplant lymphoproliferative disease, and recurrent infections was hospitalized for nausea, vomiting, and diarrhea. His ocular history included accommodative esotropia, hyperopia with astigmatism, Molluscum contagiosum lid lesions, and idiopathic intracranial hypertension. Shortly after presentation, he developed increased intraocular pressure, an afferent pupillary defect, a layered hyphema, and tense proptosis of the left globe requiring an emergent canthotomy and cantholysis. Over the next month, the patients hospital course included subdural and subarachnoid hemorrhage, temporal lobe stroke, serotonin syndrome, bilateral CST, and systemic microangiopathy. After an extensive workup, a diagnosis of tacrolimus-associated thrombotic microangiopathy was made. At this point, vision was 20/20 in the right eye and light perception in the left eye. Eight months after the canthotomy and cantholysis, the patients vision in the left eye deteriorated to no light perception and remained so after 13 months of follow-up. Conclusions An idiosyncratic drug reaction should be considered in the differential diagnosis of CST, especially in a patient on calcineurin inhibitors after solid organ transplant without sinus disease or orbital cellulitis.

A systematic approach to eye muscle surgery for infantile nystagmus syndrome (INS): Results in 100 patients

PURPOSE To report a systematic approach to, and the visual and electrophysiological effect of, eye muscle surgery in 100 patients with infantile nyustagmus syndrome (INS). METHODS Prospective, interventional case cohort analysis of clinical and eye movement data in 100 patients with INS who had virgin extraocular eye muscles operated on for nystagmus with or without combinations of strabismus and an anomalous head posture. All patients were followed at least 9 months after surgery. Outcome measures, part of an IRB approved study, included binocular visual acuity, head position, strabismic deviation, and eye movement recordings, from which waveform types and an Automated Nystagmus Acuityn Function (ANAF) was calculated. Computerized parametric and non-parametric statistical analysis of data were performed using standard software on both individual and group data. RESULTS There were 9 consistent surgical procedures used with the most common being that for a horizontal head posture alone (22%). Age at surgery averaged 14 years with 11 months followup. Sixty-eight percent had associated eye disease (optic nerve, retinal, amblyopia, cataracts). Group means in binocular acuity, strabismic deviation, head posture, abd ANAF measures from eye improved for all procedures. There were 12 (12%) reoperations without any serious surgical complications. Individual analysis revealed only age and head posture differences in outcome measures between the 9 procedures. CONCLUSIONS Using this approach, surgery on the extraocular muscles in patients with INS results in improvements in multiple aspects of ocular motor and visual function.