PARACENTRAL ACUTE MIDDLE MACULOPATHY AS A PRESENTING SIGN OF CENTRAL RETINAL ARTERY OCCLUSION IN SICKLE CELL DISEASE TREATED WITH TISSUE PLASMINOGEN ACTIVATOR.

PURPOSE: We report the case of a 27-year-old monocular woman with a history of sickle cell disease who received intra-arterial tissue plasminogen activator (tPA) after presenting with acute painless vision loss secondary to incomplete central retinal artery occlusion presenting as paracentral acute middle maculopathy in her left eye. METHODS: Ultrawidefield fundus photography, ultrawidefield fluorescein angiography, en face optical coherence tomography (OCT), and optical coherence tomography angiography (OCTA) were obtained and reviewed, followed by cerebral angiography and infusion of intra-arterial tissue plasminogen activator. RESULTS: A patient with a history of sickle cell disease presented with a new onset of a dense central scotoma, and the visual acuity diminished to 20 of 200 from baseline 20 of 20 in her left eye. Fluorescein angiogram was nondiagnostic. Optical coherence tomography revealed perifoveal hyper-reflective bands in the inner nuclear layer in a pattern characteristic of paracentral acute middle maculopathy. The patient received intra-arterial tissue plasminogen activator through her left ophthalmic artery shortly after presentation, resulting in a gradual restoration of the visual acuity to 20 of 20 in the three months after the procedure. CONCLUSION: This is the first report describing the use of intra-arterial tissue plasminogen activator to treat incomplete central retinal artery occlusion presenting as a paracentral acute middle maculopathy lesion in a patient with sickle cell disease.

Micro-incision, trans-iridal aspiration cutter biopsy for ciliary body tumours.

OBJECTIVE: To perform micro-incision, trans-iridal, aspiration-cutter-assisted biopsy for ciliary body tumours. DESIGN: Retrospective, nonrandomized, observational, interventional case series. METHODS: Five consecutive patients undergoing ciliary body tumour biopsy were clinically diagnosed using slit-lamp photography, gonioscopy, high-frequency ultrasound imaging, and systemic radiographic staging. A 1-2 mm clear cornea incision was placed opposite to the central clock hour of the ciliary body tumour. Viscoelastic was infused into the anterior chamber for stabilization and endothelial protection. Then, a 27-gauge aspiration cutter was used to make an iridotomy at the iris root and then extend through the iris into the tumour. Biopsy was performed using mechanical cutting starting at 300 cuts per minute and aspiration at 600 mm Hg. After withdrawal of the cutter from the eye, the effluent tube was flushed into a 3 cc syringe, inspected for specimen under the operating microscope and sent for pathology. Multiple biopsies were performed on each patient. Viscoelastic was removed and Seidel examination of the corneal wound performed. RESULTS: Five eyes were biopsied. A mean 3.6 passes were used to obtain tumour tissue. Tumour cells and tissue were obtained in all cases. Cytologic, histopathologic, and immuno-histochemical analysis were performed (100%, n = 5/5). Diagnoses included melanoma (60%, n = 3/5), melanocytoma (20%, n = 1/5), and leiomyoma (20%, n = 1/5). Transient postoperative hyphemas cleared within 1 week (80%, n = 4/5). No secondary glaucoma, infection, or cataracts were noted. CONCLUSION: Aspiration-cutter biopsy through the iris root provided a minimally invasive, safe method for obtaining ciliary body tissue for cytology, histopathology, and immunohistochemical analysis.

Augmented Posterior Hyaloid Adhesion Associated With Retinal Detachment After Macular Hole Repair.

BACKGROUND AND OBJECTIVE: There remains a low but intractable risk of rhegmatogenous retinal detachment (RRD) after surgical repair of macular holes (MHs). The purpose of this study is to identify potential causes for RRDs after MH surgery. PATIENTS AND METHODS: The authors retrospectively examined a single surgeon series of stage 3 MH repair surgeries during a 5-year period. Clinical data, including preoperative, intraoperative, and postoperative evaluations, were reviewed to determine potential causes of RRD. RESULTS: Of the 332 eyes that received MH surgery, 12 (3.6%) developed postoperative RRD. Seven RRD cases exhibited no evident precursor pathology; however, augmented posterior hyaloid adhesions (APHAs) were found intraoperatively and postoperatively. CONCLUSIONS: In MH surgery, APHA increases risk for RRD. During PPV, the vitreous typically separates to the peripheral vitreous base. Some eyes have APHAs near the equator that cannot be elevated, consistent with strong adhesion. The authors believe the high postoperative RRD rate is due to continued hyaloid elevation after surgery. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:635-638.].

Pediatric Thyroid Eye Disease: Clinical Characteristics and Orbital Decompression Outcomes.

PURPOSE: To review the clinical presentation, disease severity, and surgical interventions of children with thyroid eye disease (TED) at a single institution over a 7-year period. METHODS: Retrospective cohort study of children 0 to 18 years of age with a diagnosis of TED, seen at the Children's Hospital of Philadelphia from 2009 to 2016. Ophthalmic examination findings, neuroimaging study results, and ophthalmological management were recorded. t-Tests were used to test for statistical significance. Institutional Review Board approval was obtained for the study. RESULTS: Sixty-seven subjects with pediatric TED were studied: 59 female and 8 male. The mean age at diagnosis was 11.6 years (SD 4.2, range 2.3-17.9). All subjects had proptosis (67, 100%). Other clinical findings were eyelid retraction in 46 (69%), chemosis in 7 (10%), punctate epithelial erosions in 8 (12%), and strabismus in 1 (1.5%). No subjects had evidence of optic neuropathy. Five subjects with static-phase TED received orbital decompression surgery for exposure keratopathy and disfigurement. All had significant cosmetic improvement and resolution of exposure keratopathy. DISCUSSION: In this series, children with TED generally exhibit mild ocular signs, which is in agreement with past studies. Our study presented a high incidence of proptosis compared with other studies; however, this may represent a referral bias to our quaternary care center. CONCLUSION: Pediatric TED patients exhibit mild symptoms, such as proptosis and eyelid retraction without strabismus, vision loss, or optic neuropathy. Some children with TED have severe disfigurement and corneal exposure due to the disease and benefit from orbital decompression.