Multiple Cranial Neuropathies as the Initial Presentation of Primary Ductal Adenocarcinoma of the Lacrimal Gland.

Primary ductal adenocarcinoma of the lacrimal gland is a rare, aggressive malignancy that clinically and histologically resembles salivary duct carcinoma. Similar to other malignant epithelial lacrimal gland tumors, ductal adenocarcinoma typically presents with unilateral proptosis, pain, upper eyelid swelling, palpable mass, diplopia, ptosis, and blurred or decreased vision. Rarely, primary malignant epithelial lacrimal gland tumors may first present with multiple cranial neuropathies due to occult spread to the cavernous sinus, as in this case. With such a vast differential diagnosis, a practical yet systematic approach to multiple cranial neuropathies, as guided by clinical history, exam, and neuroimaging, allows for a more targeted diagnostic evaluation, especially when multiple diagnostic tests and interventions return unrevealing. A repeat biopsy or complete excision of the lacrimal gland may be necessary to yield the correct diagnosis.

Outcomes and lessons learned from two decades' experience with glaucoma drainage device implantation for refractory Sturge Weber-associated childhood glaucoma.

PURPOSE: To evaluate glaucoma drainage device (GDD) implantation for refractory pediatric glaucoma associated with Sturge-Weber syndrome (SWS). METHODS: The medical records of consecutive children with SWS-associated glaucoma at a single center who were treated by a single surgeon using GDDs over 20 years were reviewed retrospectively. The main outcome measure was GDD treatment success, defined as absence of any of the following indications of treatment failure: (1) intraocular pressure (IOP) of >21 mm Hg on two consecutive visits despite maximal medical therapy, (2) additional IOP-lowering surgery, and (3) sight-threatening complications. RESULTS: A total of 22 eyes of 22 children were included. The median age at glaucoma diagnosis was 0.73 years (range, 0.06-13.2), and the median age at GDD surgery was 4.8 years (range, 0.6-13.3). Most eyes (14 [68%]) had prior glaucoma surgery. Mean follow-up was 2.8 ± 1.5 years. Success (95% confidence interval) by Kaplan-Meier analysis for GDD surgery at 1, 3, and 5 years was 91% (68-98), 75% (50-89), and 52% (24-73), respectively. Failure occurred in 8 eyes (36%). Complications were common, occurring in 50% of eyes, with 23% of eyes having more than one complication. Severe vision-threatening complications (n = 3) included one case each of possible infection requiring GDD removal, persistent hypotony, and cilioretinal artery occlusion. CONCLUSIONS: GDDs are an effective treatment for SWS-associated glaucoma but have a high rate of complications. We report several severe complications that prompted surgical modifications for increased safety when implanting GDDs in eyes with SWS-associated glaucoma.

QUANTITATIVE TOPOGRAPHIC CURVATURE MAPS OF THE POSTERIOR EYE UTILIZING OPTICAL COHERENCE TOMOGRAPHY.

PURPOSE: Deformations of the retina such as staphylomas in myopia or scleral flattening in high intracranial pressure can be challenging to quantify with en face imaging. We describe an optical coherence tomography-based method for the generation of quantitative posterior eye topography maps in normal and pathologic eyes. METHODS: Using "whole eye" optical coherence tomography, we corrected for subjects' optical distortions to generate spatially accurate posterior eye optical coherence tomography volumes and created local curvature (KM, mm-1) topography maps for each consented subject. We imaged nine subjects, three normal, two with myopic degeneration, and four with papilledema including one that was imaged longitudinally. RESULTS: Normal subjects mean temporal KM was 0.0923 mm-1, nasal KM was 0.0927 mm-1, and KM local variability was 0.0162 mm-1. In myopic degeneration, subjects KM local variability was higher at 0.0836 mm-1. In papilledema subjects nasal KM was flatter compared with temporal KM (0.0709 vs. 0.0885 mm-1). Mean intrasession KM repeatability for all subjects was 0.0036 mm-1. CONCLUSION: We have developed an optical coherence tomography based method for quantitative posterior eye topography that offers the ability to analyze local curvature with micron scale resolution and offers the potential to help clinicians and researchers characterize subtle, local retinal deformations earlier in patients and follow their development over time.

Granulomatosis with polyangiitis-associated ischemic optic neuropathy in a previously healthy 50-year-old female.

PURPOSE: To describe a case of anterior ischemic optic neuropathy as a presenting sign of granulomatosis with polyangiitis. OBSERVATIONS: A previously healthy 50-year-old female developed right eye, then left eye, redness and pruritis and was diagnosed with allergic versus viral conjunctivitis. Five days later, she noted an acute decline in vision in the right eye, corresponding with a decrease on Snellen testing from 20/30 to 20/100 with correction. She was noted to have a right relative afferent pupillary defect, 2+ pallid disc edema, and OCT (Spectralis, Heidelberg Engineering, Carlsbad, CA) findings of significant retinal nerve fiber thickening. Review of systems revealed a three-month history fatigue, right-sided headaches, jaw claudication, bronchitis, cough without hemoptysis, and epistaxis, as well as interval development of a petechial rash across her body, migratory polyarthralgias, fevers, and tachycardia. ESR and CRP were markedly elevated, and the patient was admitted to the hospital for a systemic vasculitis workup. She was started on IV methylprednisolone. Her vision improved dramatically with steroids, measuring 20/50 with correction in the right eye after 24 hours and returning to baseline after five days. An extensive workup including imaging, bloodwork, and biopsies led to a diagnosis of granulomatosis with polyangiitis, with PR3-positive ANCA. CONCLUSIONS: Ocular findings, including anterior ischemic optic neuropathy, may be the presenting signs for patients with granulomatosis with polyangiitis. Prompt recognition and treatment with high-dose steroids and immunomodulatory therapy is important for visual recovery. IMPORTANCE: Prompt recognition of potential vasculitis-related vision loss can lead to timely initiation of vision-saving treatment.

A lot of nerve.

A 71-year-old woman with a history of acute lymphoblastic leukemia presented with unilateral optic disc edema. Laboratory evaluations for infectious, inflammatory, and neoplastic etiologies were negative. Magnetic resonance imaging of the orbits with and without contrast demonstrated enhancement and thickening of the right optic nerve with mild stranding of the intraconal fat. She underwent three large-volume lumbar punctures, all of which were negative for malignancy. An optic nerve biopsy was ultimately performed, demonstrating malignant cells on immunohistochemical staining. This case illustrates a rare case of biopsy-proven leukemic infiltration of the optic nerve head as the presenting sign of leukemic relapse despite a negative systemic workup. Leukemic infiltration should be considered in the differential diagnosis for cases of acquired disc edema, especially in patients with a past medical history of leukemia.

Corneal Endothelial Cell Migration and Proliferation Enhanced by Rho Kinase (ROCK) Inhibitors in In Vitro and In Vivo Models.

Purpose: To explore the role of Rho-associated kinases (ROCK) in corneal physiology and regeneration, and the effects of suppressing its activity in stimulating corneal endothelial cell proliferation and migration in vitro and in vivo. Methods: Immunohistochemistry was performed to detect RhoA and ROCK-1 and ROCK-2 in human corneal tissue. Adult porcine corneal endothelial cells (CECs) were isolated, grown to confluence, and further characterized. Under the treatment of ROCK inhibitors, changes in the cellular distribution profile of ZO-1 and F-actin were examined by immunofluorescence staining. Corneal endothelial cells migration was evaluated by scratch assay and analyzed with Axiovision software. Cell proliferation was quantified using Click-iT EdU HCS Assay. In vivo, the corneal endothelia of rabbits were surgically injured and H-1152 was topically applied for 10 days. Progress of wound healing was evaluated daily by monitoring corneal edema, inflammation, and thickness using slit-lamp examination, photography, and pachymetry. Rabbits were euthanized and enucleated for further evaluation. Results: H-1152 exhibited significant stimulatory effect on CEC migration and proliferation in vitro compared with both untreated and Y-27632-treated cells. Furthermore, topical administration of H-1152 led to marked reduction in corneal edema and formation of multinucleate CECs in vivo suggestive of proliferation associated with healing. Conclusions: H-1152 exhibited a better stimulatory effect on CEC migration and proliferation in vitro than Y-27632. Our findings suggest that topical administration of H-1152 promotes healing of injured corneal endothelium in vivo. These results demonstrate the efficacy of ROCK inhibitors as a potential topical therapy for patients with corneal endothelial disease.

Streptococcal pharyngitis leading to corneal ulceration.

PURPOSE: To report a patient with a history of exposure keratopathy who presented with bilateral bacterial keratitis associated with streptococcal pharyngitis and use of bilevel positive airway pressure (BiPAP). DESIGN: Case report. METHODS: Pulsed-field gel electrophoresis of patient isolates from ocular and pharyngeal cultures. RESULTS: Ocular and oropharyngeal cultures from a 24-month-old child with a history of cerebral palsy on BiPAP at night revealed the same strain of Streptococcus pyogenes. CONCLUSIONS: Use of mechanical ventilation, such as BiPAP, may precipitate inoculation of eye with respiratory or oropharyngeal pathogens. To the authors' knowledge this is the first report demonstrating the association between concurrent streptococcal pharygitis, keratitis, and use of BiPAP.