Mechanical Deformation of Peripapillary Retina in Response to Acute Intraocular Pressure Elevation

Elevated intraocular pressure (IOP) may cause mechanical injuries to the optic nerve head (ONH) and the peripapillary tissues in glaucoma. Previous studies have reported the mechanical deformation of the ONH and the peripapillary sclera (PPS) at elevated IOP. The deformation of the peripapillary retina (PPR) has not been well-characterized. Here we applied high-frequency ultrasound elastography to map and quantify PPR deformation, and compared PPR, PPS and ONH deformation in the same eye. Whole globe inflation was performed in ten human donor eyes. High-frequency ultrasound scans of the posterior eye were acquired while IOP was raised from 5 to 30 mmHg. A correlation-based ultrasound speckle tracking algorithm was used to compute pressure-induced displacements within the scanned tissue cross-sections. Radial, tangential, and shear strains were calculated for the PPR, PPS, and ONH regions. In PPR, shear was significantly larger in magnitude than radial and tangential strains. Strain maps showed localized high shear and high tangential strains in PPR. In comparison to PPS and ONH, PPR had greater shear and a similar level of tangential strain. Surprisingly, PPR radial compression was minimal and significantly smaller than that in PPS. These results provide new insights into PPR deformation in response of IOP elevation, suggesting that shear rather than compression was likely the primary mode of IOP-induced mechanical insult in PPR. High shear, especially localized high shear, may contribute to the mechanical damage of this tissue in glaucoma.

Peripapillary Neuroendocrine Carcinoma Metastasis: A Novel Approach to Treatment

Peripapillary and circumpapillary retinal intraocular metastases are rare and present a treatment challenge for ophthalmologists because of the high risk of iatrogenic injury to the optic nerve. There are no clear guidelines on the management of these lesions, and many clinicians will initially observe for improvement of the metastases with systemic chemotherapy before considering local therapy with external beam radiation. Radiation to the optic disc carries a significant risk of injuring the optic nerve, leading to worsening of vision. Alternative treatment approaches are needed. We present a patient with large-cell neuroendocrine carcinoma with metastasis to the peripapillary retina who was treated with intravitreal topotecan and with intravitreal aflibercept. Serial fundus photos, ultrasound, and optical coherence tomography demonstrated a reduction in size of the lesion and a decrease in subretinal fluid with intravitreal topotecan and aflibercept. In addition, visual acuity was stabilized during treatment. Intravitreal chemotherapy for intraocular metastases in vision-sensitive areas such as the peripapillary retina may be a viable alternative for patients who seek to preserve their vision and maintain their quality of life.

A rare triad of morning glory disc anomaly, moyamoya vasculopathy, and transsphenoidal cephalocele: pathophysiological considerations and surgical management

Morning glory disc anomaly is a congenital abnormality of the optic disc and peripapillary retina reported as an isolated condition or associated with various anomalies, including basal encephaloceles and moyamoya vasculopathy. However, the co-occurrence of these three entities is extremely rare and the pathogenesis is still poorly understood. Moreover, data on the surgical management and long-term follow-up of the intracranial anomalies are scarce. Here, we describe the case of a 11-year-old boy with morning glory disc anomaly, transsphenoidal cephalocele, and moyamoya vasculopathy, who underwent bilateral indirect revascularization with encephalo-duro-myo-arterio-pericranio-synangiosis at the age of 2 years, and endoscopic repair of the transsphenoidal cephalocele at the age of 6 years. A rare missense variant (c.1081T>C,p.Tyr361His) was found in OFD1, a gene responsible for a X-linked ciliopathy, the oral-facial-digital syndrome type 1 (OFD1; OMIM 311200). This case expands the complex phenotype of OFD1 syndrome and suggests a possible involvement of OFD1 gene and Shh pathway in the pathogenesis of these anomalies.

Evaluation of the efficacy of neural network technology in the analysis of the condition of the optic nerve disc and peripapillary retina in healthy individuals examined for glaucoma

Objective: Evaluation of efficacy of the application of artificial intelligence technology and neural networks in the analysis of the condition of the optic disc and the peripapillary retina in healthy individuals.Methods: Prospective analysis of the condition of visual organs in 54 patients aged from 49 to 71 years (100 eyes) was conducted. The examination included autorefractometry, visometry, tonometry, automated perimetry, spectral domain optical coherence tomography, Heidelberg retina tomography. A pre-trained neural network evaluated only a photograph of the optic nerve disc and the peripapillary retina.Results: Neural network identified twelve images with suspected glaucoma, five of which were selected by medical experts. The comparison of all study groups has demonstrated the presence of statistically significant differences between them according to a range of visiometric indicators.Conclusions: The study results showed high efficiency of artificial intelligence and the prospects of its use for the diagnosis of glaucoma.

Various Types of Neuroleukemia with Optic Nerve Involvement

Neuroleukemia is a severe complication of hemoblastosises characterized by infiltration of blast cells of the meninges, the substance of the brain, cranial and peripheral nerves.The purpose. To analyze the clinical manifestations of leukemia with optic nerve damage in leukemia and malignant lymphomas.Patients and Methods. From January 2016 to January 2020, one center-based non-randomized prospective study was conducted on patients with leukemia and non-Hodgkin’s lymphomas who were treated in the hematology department of MONIKI and made complaints about reduced vision. 26 patients were examined, 4 of them were diagnosed neuroleukosis with leukemic infiltration of the optic nerves: three women aged 41, 44, 46 years and a man aged 50 years.Results. All patients had one common ophthalmological symptom — decreased visual acuity. Complaints of reduced vision in one patient appeared at the onset of the disease, in other patients-during a relapse. The picture of the eye’s fundus in all patients was identical: the optic disk had blurred borders, and elevated above the retina. In the first days, the vascular funnel was viewed, then closed by infiltration. A dense white infiltrate of the peripapillary retina spread over the entire macular area. The retinal veins were sharply dilated of uneven caliber. Along the course of the vessels, there were a large number of different sizes and shapes of hemorrhages. Various types of neuroleukemia with lesion of the optic nerve were described: a combination of focal lesions of the brain and the optic nerve, isolated damage to the optic nerve with blast cytosis in the cerebrospinal fluid (CSF), isolated damage to the intraocular part of the optic nerve without changing the cerebrospinal fluid. In three patients the diagnosis of neuroleukemia exhibited on the basis of changes according to magnetic resonance imaging (MRI) and/or the number of blast cells in punctate liquorologic research. In the fourth patient, in the absence of changes in the liquor and MRI, the diagnosis of leukemic infiltration of the optic nerve was confirmed by data from a cytological examination of a punctate from the peripapillary retina. After the beginning of antitumor treatment, the significant improvement visual acuity and decrease in leukemic infiltrate of the optic disc and retina of the affected eye was noted in all patients.Discussion. Damage of the optic nerve can develop both at the onset of the disease, and relapses of leukemia or aggressive non-Hodgkin’s lymphomas. Leukemic infiltration evolves in all parts of the optic nerve, but more often affects its intraocular portion. The damage to the intraocular part is characterized by the presence of a dense infiltrate both in the area of the papilla and the peripapillary retina.Conclusion. Leukemic infiltration of the optic nerve is manifested by a significant decrease in visual acuity. Careful attention to complaints of reduced vision in patients with leukemia can help to recognize the development of the disease’s debut or its progression. Timely antitumor treatment leads to an improvement in visual acuity and, consequently, an improvement in the quality of life of patients.