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Giuseppe Mannino,1 Barmak Abdolrahimzadeh,2 Silvia Calafiore,1 Gianmario Anselmi,1 Cristina Mannino,1 Alessandro Lambiase3 1Ophthalmology Unit, Saint Andrea Hospital, Department of Neurosciences, Mental Health and Sense Organs, University of Rome “Sapienza”, Rome, Italy; 2Polimed Beltramelli Medical Centre, Rome, Italy; 3Department of Sense Organs, Ophthalmology Unit, University of Rome “Sapienza”, Rome, Italy Abstract: Ultrasound biomicroscopy is a non-invasive imaging technique, which allows high-resolution evaluation of the anatomical features of the anterior segment of the eye regardless of optical media transparency. This technique provides diagnostically significant information in vivo for the cornea, anterior chamber, chamber angle, iris, posterior chamber, zonules, ciliary body, and lens, and is of great value in assessment of the mechanisms of glaucoma onset. The purpose of this paper is to review the use of ultrasound biomicroscopy in the diagnosis and management of rare diseases of the anterior segment such as mesodermal dysgenesis of the neural crest, iridocorneal endothelial syndrome, phakomatoses, and metabolic disorders. Keywords: glaucoma, rare diseases, ultrasound biomicroscopy, neural crest, iridocorneal syndrome, phakomatoses, metabolic disorders

Abstract Background Hyphema is a complication that can occur after glaucoma filtering surgery. Biomicroscopic examination of the anterior segment is commonly used to diagnose it and gonioscopy may provide a useful support to find the source of the haemorrhage. Unfortunately, when the blood hides the structure of the anterior segment the gonioscopic examination fails. In this case we performed ultrabiomiscroscopy with 50–80 MHz probes to overcome the limits of gonioscopy. The use of this technique to study the anterior segment of the eye has previously been reported in literature, but we illustrates its importance for performing a correct diagnosis in a specific case of hyphema. Case presentation We report a case of a sixty-year-old caucasian male with recurrent hyphema in the left eye. The episodes of hyphema were four in two years and the patient came to the hospital for the first time in the last occasion. The past episodes were managed with topical corticosteroids and mydriatic drops. He referred surgical trabeculectomy in both eyes 5 years before the first symptoms and no specific eye trauma before the first episode. The examination of the anterior segment revealed a 2 mm hyphema in the left eye due to blood leakage through the superior iridectomy. Gonioscopy could not identify the source of the haemorrhage. B-scan ultrasound and ultrabiomiscroscopy, with 50–80 MHz probes, were performed. Ultrabiomiscroscopy, mainly with the probe of 80 MHz, provided images of high resolution of the structures of the anterior segment and it allowed the visualization of an abnormal vessel at the inner margin of the trabeculectomy opening, probably responsible of the recurrent hyphema. Conclusion Ultrabiomicroscopy proved to be a useful diagnostic technique for identifying the cause of the recurrent hyphema when other examination techniques are not applicable.

Background: The relation of retinal thickness to neuropsychological indexes of cognitive impairment in patients with Alzheimer disease (AD) remains an area of investigation. The scope of this investigation was to compare volume and thickness changes of neuronal retinal layers in subjects with AD with those of age-matched healthy controls and to estimate the relation between cognitive functioning evaluated by neuropsychological assessment and thickness changes of the retina., Methods: This was a prospective single-site study where we evaluated 25 subjects with probable AD matched for age, sex, and education to 17 healthy control subjects (HC). All participants underwent a full medical evaluation, neuropsychological assessment, and optical coherence tomography (OCT) to evaluate the peripapillary retinal nerve fiber layer (pRNFL) thickness, ganglion cell complex (GCC) thickness, and macular volume., Results: The pRNFL thickness of AD patients showed a significant overall reduction compared with healthy controls (P = <0.0001). Furthermore, pRNFL was reduced in each retinal quadrant, particularly the inferior, nasal, and superior quadrants. GCC thickness and macular volume were reduced in AD patients in comparison with HC (P = 0.004; P = 0.001). Of particular interest was the correlation between OCT findings and neuropsychological assessment; we did not find a significant association of retinal thinning with worse MMSE score, but reduction of macular volume was associated with worse constructional praxis performance. Impairment of semantic-lexical and processing speed was associated with attenuation of macular GCC thickness., Conclusions: OCT can show early thickness changes in AD patients with subtle memory disturbances. These results suggest that correlations between retinal thinning and cognitive performance warrant further investigation.

Purpose: To assess safety and efficacy of deep topical anesthesia with ropivacaine-soaked sponge compared with topical anesthesia with oxybuprocaine in patients undergoing phacoemulsification., Methods: This was a retrospective study where records of patients operated for cataract were evaluated. Patients using a visual analogue scale scored pain during surgery, and the surgeon on a questionnaire recorded ease of operation. Medical records were evaluated for patients who received topical anesthesia with multiple administrations of oxybuprocaine 0.4% or those who received deep topical anesthesia with a polyvinyl acetal sponge impregnated with ropivacaine 0.75% and positioned under the eyelid 30 min before surgery., Results: A total of one hundred patient records, equally divided in patients receiving deep topical anesthesia or topical anesthesia, were included. The visual analogue scale scores among the groups were statistically significant for a lower pain score in patients who received deep topical anesthesia with ropivacaine-soaked sponges (p = 0.0069). The average surgeon score was significantly higher for the deep topical anesthesia group indicating favorable ease of surgery (p = 0.0341). Six patients had major complications during surgery. No additional anesthesia was necessary to manage the complications in four patients in the deep anesthesia group, whereas propofol was used for the induction and maintenance of anesthesia in two patients in the topical anesthesia group., Conclusions: Deep topical anesthesia with ropivacaine-soaked sponges performed as well as topical oxybuprocaine regarding safety and efficacy. It provided a lower patient pain score, favorable surgeon satisfaction, and long-lasting anesthesia.

Carotid cavernous fistulas (CCF) are vascular communications between the carotid artery and the cavernous sinus. Ophthalmologists are called to diagnose and manage the condition in cases that present with ocular features. A 73-year-old female was referred to our glaucoma center clinic. Eight years before, she had started receiving medication for glaucoma and had undergone laser iridotomy, but a satisfactory management of intraocular pressure (IOP) had not been achieved. The patient was complaining of intermittent diplopia, bilateral proptosis, and conjunctival chemosis over the past 6 months. Best-corrected visual acuity in the right (OD) and left eye (OS) was 9/10 and 10/10, respectively. Visual field testing showed slight paracentral field defects mostly in OS. IOP was 20 mm Hg in OD and 34 mm Hg in OS. We referred the patient to neuroradiology, and MRI angiography revealed a CCF with angiographic classification of Cognard grade 2. Closure of the CCF by transarterial embolization was performed in the neuroradiology department. One week following the procedure, the clinical signs of diplopia, proptosis, and conjunctival chemosis had greatly improved, and IOP was reduced to 12 mm Hg OD and 19 mm Hg in OS. Glaucoma treatment was maintained with topical brimatoprost, brinzolamide, and timolol. Owing to the risk of vision loss associated with vascular stasis, retinal ischemia, and high IOP, ophthalmologists must be aware of the clinical features of CCF and should request appropriate imaging studies such as MRI angiography in order to confirm the diagnosis and plan multidisciplinary treatment.

Purpose: To present a case of Weber-Christian disease with symptomatic ocular involvment. Weber-Christian disease is a relapsing febrile nodular nonsuppurative panniculitis. It is characterized by malaise and fever accompanied by subcutaneous inflammatory nodules on the trunk and extremities. It can affect several organs, but ocular signs have been infrequently described in literature., Methods: A 20-year-old woman with Weber-Christian disease presented with severe bilateral ocular inflammation. A complete ophthalmologic examination was performed., Results: Visual acuity was 20/100 in both eyes and slit-lamp examination showed bilateral iridocyclitis. Bilateral cortico-nuclear cataract did not allow funduscopy and she underwent cataract extraction. Retinal vasculitis was detected., Conclusions: Patients with Weber-Christian disease can develop severe ocular inflammation. A complete ophthalmolgic examination should be done in these patients, especially when ocular involvement is the main sign of the disease. A further understanding of the severity of ocular inflammation proved fundamental in the management of the disease.