Acute Zonal Occult Outer Retinopathy in a Patient Suffering from Epilepsy: Five-Year Follow-Up

We report an unprecedented case of a young patient with epilepsy coexisting with acute zonal occult outer retinopathy (AZOOR), a rare white dot syndrome of unknown etiology, associated with damage to the large zones of the outer retina. Recently, it has been established that epileptic episodes contribute to an inflammatory response both in the brain and the retina. A 13-year-old male patient with epilepsy was referred by a neurologist for an ophthalmologic consultation due to a sudden deterioration of visual acuity in the left eye. The examination, with a key role of multimodal imaging including color fundus photography, fluorescein angiography, indocyanine green angiography (ICGA), fundus autofluorescence (FAF), swept-source optical coherence tomography (SS-OCT) with visual field assessment, and electroretinography indicated AZOOR as the underlying entity. Findings at the first admission included enlargement of the blind spot in visual field examination along a typical trizonal pattern, which was revealed by FAF, ICGA, and SS-OCT in the left eye. The right eye exhibited no abnormalities. Seminal follow-up revealed no changes in best corrected visual acuity, and multimodal imaging findings remain unaltered. Thus, no medical intervention is required. Our case and recent laboratory findings suggest a causative link between epilepsy and retinal disorders, although this issue requires further research.

Multimodal Primary Open Angle Glaucoma Early Diagnosing Program Based on Clinical Process

Background: Glaucoma is one of the leading causes of blinding disease. Early detection can improve patients’ quality of vision. Effectively identifying primary open angle glaucoma (POAG) using structural and functional examination is critical. Computer aided diagnosis of glaucoma requires multimodal data to find an accurate model for early glaucoma diagnosis. Methods: This study collected 87 early POAG eyes, 85 suspected POAG eyes, and 129 healthy eyes from the ophthalmology department at Second Affiliated Hospital of Harbin Medical University. Retinal nerve fiber layer thickness (RNFLt), intraocular pressure (IOP) value, visual field examination parameters and age were obtained. A powerful deep learning network segmented the FP and extracted optic nerve head (ONH) features. Machine learning classifiers (MLCs) were adopted to get the final classification results and compared with the diagnosis results of glaucoma specialists and general non-glaucoma ophthalmologists. Result: The program diagnosing early POAG, suspected POAG, and healthy eyes made overall Area Under the Curve of 0.97. Dice of optic disc and optic cup segmentation is 0.9631, 0.8435 respectively. Accuracy of the program (0.9004) is higher than general ophthalmologists (0.8195). Specificity of the program (0.9635) is higher than glaucoma specialists (0.9366).Conclusions: The program delivers superior results in diagnosing early POAG. This study’s hybrid deep learning-machine learning framework can assist with clinical decision for early POAG effectively.

Craniopharyngioma and arteriovenous malformation operated using the same craniotomy

Craniopharyngiomas (CPs) are rare benign epithelial tumours. Brain arteriovenous malformations (AVMs) are also rare lesions occurring in young adults. The appearance of both these lesions in the same patient is rare. A 42-year-old patient presented with headaches for 3 months and a progressive decrease in his visual acuity. Bitemporal hemianopsia was detected in the visual field. Magnetic resonance imaging (MRI) revealed a tumour with cystic and solid components located in the suprasellar region and AVM in the right temporal lobe. AVM (Martin–Spetzler grade III) was visualised using digital subtract angiography (DSA), which was fed from the right middle cerebral artery and drained through the sigmoid sinus via the inferior petrosal sinus. The patient was operated with enlarged right frontotemporal craniotomy. AVM nidus was totally removed at the first operation. Embolisation was not preferred before the AVM surgery. After 3 days, sylvian dissection was performed using the same craniotomy. The tumour was completely removed via the carotid cistern by making sharp dissection from the infundibulum. Post-operatively, the patient showed normal neurological examination and significant improvement in his visual field examination. There was no residual/recurrent tumour or AVM on contrast-enhanced MRI and DSA at post-operative 6 months. Histopathological examination revealed AVM in the first operative material and papillary-type CP in the second. The coexistence of these two rare pathologies has previously been reported in only one patient. This is the first case of surgical resection of CP and AVM using the same craniotomy.

Calcium oxalate crystals retinopathy following domino liver transplant: A case report

Introduction: Hyperoxaluria is a rare cause of hereditary crystalline retinopathy. We report the first case of acquired calcium oxalate crystalsretinopathy following domino liver transplantation (DLT). Clinical case: A 72-year-old patient was referred for bilateral visual impairment 9 months after DLT. Slit lamp examination was unremarkable. Fundus examination revealed calcium oxalate crystals accumulation within both retina. Owing to multi-organ failure, the patient underwent combined liver-kidney retransplantation. During the following two years, calcium oxalate crystals accumulation within the retina gradually decreased and visual acuity improved. Nevertheless, OCT-angiography revealed abnormalities in the inner and outer retinal vascular plexus (i.e. retinal vessels occlusion and dilatation). Visual field examination revealed bilateral constriction associated with decreased optic nerve fibre layer thickness suggesting optic nerve atrophy. Conclusion: This case highlights the need for ophthalmologists to consider the diagnosis of acquired hyperoxaluria in patients with progressive bilateral visual impairment following DLT, especially if the postoperative course is marked by renal failure. Moreover, even after liver-kidney transplantation with a conventional graft, visual function can remain impaired owing to maculopathy and optic atrophy.

Enlarged Foveal Avascular Zone after Whiplash Injury-Acquired Berlin’s Edema

We describe a case of optical coherence tomography angiography (OCTA) changes in the foveal avascular zone (FAZ) in a patient that had suffered Berlin’s edema after a whiplash neck injury. The patient reported central scotoma throughout the 1-year follow-up, confirmed by visual field examination. OCTA showed FAZ enlargement of the left eye as compared to the healthy right eye in the superficial capillary layer and even more in the deep capillary layer. To the best of our knowledge, FAZ enlargement has not been previously described by OCTA after whiplash-related macular injury.

Pituitary hyperplasia causing complete bitemporal hemianopia with resolution following surgical decompression: case report

In this report, the authors demonstrated that idiopathic pituitary hyperplasia (PH) can cause complete bitemporal hemianopia and amenorrhea, even in the setting of mild anatomical compression of the optic chiasm and normal pituitary function. Furthermore, complete resolution of symptoms can be achieved with surgical decompression.PH can occur in the setting of pregnancy or end-organ insufficiency, as well as with medications such as oral contraceptives and antipsychotics, or it can be idiopathic. It is often found incidentally, and surgical intervention is usually unnecessary, as the disorder rarely progresses and can be managed by treating the underlying etiology. Here, the authors present the case of a 24-year-old woman with no significant prior medical history, who presented with bitemporal hemianopia and amenorrhea. Imaging revealed an enlarged pituitary gland that was contacting, but not compressing, the optic chiasm, and pituitary hormone tests were all within normal limits. The patient underwent surgical decompression of the sella turcica and exploration of the gland through an endoscopic endonasal transsphenoidal approach. Pathology results demonstrated PH. A postoperative visual field examination revealed complete resolution of the bitemporal hemianopia, and menstruation resumed 3 days later. The patient remains asymptomatic with no hormonal deficits.

Pigment Dispersion Syndrome and Pigmentary Glaucoma in an Emmetropic Young Male

Introduction: Pigment dispersion Syndrome (PDS) is a disorder with an onset in mid–twenties. There occurs a disruption of the iris pigment epithelium and deposition of pigment granules throughout the anterior segment. The incidence of PDS is 4-8/ 100,000. This condition is more commonly seen In Caucasians and is considered to be rare in Indians. Case: A 33-year-old male presented with the complaint of headache for three months. He had normal vision in both eyes with visual acuity of 6/6. Observation: Krukenberg’s spindle, a classic sign of pigment dispersion syndrome was evident on slit-lamp examination over the posterior corneal surface. Gonioscopy revealed a heavy and uniformly pigmented trabecular meshwork. OCT (Optical Coherence Tomography) demonstrated a characteristic iris configuration in the form of a mid-peripheral posterior bowing of the iris .Retinal nerve fibre layer analysis done on OCT revealed glaucomatous thinning in the right eye and a more advanced defect in the left eye. A visual field examination revealed the field to be outside normal limits in both the eyes pointing towards a diagnosis of pigment dispersion glaucoma. Conclusion: The purpose of presenting this case is to caution the clinicians to carefully examine young emmetropes who present with Krukenberg’s spindle as it could be associated with PDS. Patients with Krukenberg’s spindle and without elevated lOP are often treated as normal. These patients must be cautioned regarding possible future consequences of the disease and counseled regarding the hereditary nature of the syndrome.

Correlation between Corneal Elevation Topography and Perimetric Changes in Patients with Primary Open Angle Glaucoma

Aim: The aim of this study is to assess Scheimpflug topographic elevation maps in patients with POAG and correlate the results with their perimetric changes. Methods: This was an analytical observational cross-sectional study. The study included 130 eyes of 70 subjects which were divided into 78 eyes of 44 patients diagnosed with POAG and 52 eyes of 26 control subjects. Measurement of IOP, visual field examination in patients with POAG using Humphrey Field Analyzer (2003 Carl Zeiss Meditec), Germany were done. Subjects were scanned using TMS-5 topographer (Topographic Modeling System, version 5. Tomey Corp. Nagoya, Japan) to measure central corneal thickness, mean anterior keratometry, maximum anterior and posterior topographic elevation maps in the central 3, 5, and 7 mm. Results: 78 patients with POAG classified according to visual field deterioration using Hodapp-Anderson-Parrish grading scale into mild glaucoma 33 eyes, moderate glaucoma 19 eyes, severe glaucoma 26 eyes, and 52 eyes control were included in the study. The mean age of the patients with POAG was 57.82 ± 7.78 years; 22 eyes (50%) were male and 22 eyes (50%) were female. The average age of control subjects was 56.62 ± 8.48 years; 12 eyes (46.2%) were male and 14 eyes (53.8%) were female, average CCT was 530.3 ± 23.58 µm, average mean anterior keratometry (MAK) was 42.97 ± 1.42 D, average maximum anterior elevation (MAE) in 3,5 and 7mm zone was 5.31 ± 2.28, 12.10 ± 6.94 and 44.04 ± 21.99 µm respectively and average maximum posterior elevation (MPE) in 3,5 and 7mm zone was 8.46 ± 2.10, 19.90 ± 9.39 and 62.72 ± 28.82 µm respectively in patients with POAG, whereas average CCT was 543.0 ± 31.02µm, average MAK was 43.11 ± 1.73 D, average MAE in 3,5 and 7mm zone was 4.52 ± 1.97, 5.90 ± 2.71 and 27.19 ± 8.55 µm respectively. Conclusion: Evaluation of corneal elevation topography by scheimpflug imaging showed forward shifting of the anterior and posterior corneal surfaces in POAG.