Optic nerve atrophy

Clinical Classification [1,2]: By etiology: hereditary: autosomal dominant, autosomal recessive, mitochondrial; non-hereditary.

By ophthalmoscopic picture primary (simple); secondary; glaucomatous.

By degree of damage (preservation of function): initial; partial; incomplete; complete.

By topical level of damage: descending; ascending.

By degree of progression: stationary; progressive.

By localization of the process: unilateral; bilateral.

Indications for consultation with narrow specialists: consultation with therapist – to assess the general condition of the body; consultation with cardiologist – high level of arterial pressure is one of the main risk factors for development of occlusions of retinal and optic nerve vessels; consultation with neurologist - to exclude demyelinating disease of the CNS and clarify the topical zone of damage to the visual pathways; consultation with neurosurgeon - when the patient develops signs of intracranial hypertension or symptoms characteristic of a space-occupying lesion of the brain; consultation with rheumatologist - in the presence of symptoms characteristic of systemic vasculitis; consultation with vascular surgeon to decide on the necessity of surgical treatment in the presence of signs of occlusive process in the system of the internal carotid and ophthalmic artery (appearance of scotoma fugax in the patient); consultation with endocrinologist – in the presence of diabetes mellitus/other pathology of the endocrine system; consultation with hematologist (if blood diseases are suspected); consultation with infectious disease specialist (if vasculitis of viral etiology is suspected). consultation with otolaryngologist – if inflammation or neoplasm in the maxillary or frontal sinus is suspected.

6) Preventive measures [1,3,4, 6,7]: regular visits to ophthalmologist (at least once a year) in the presence of a history of eye diseases, central nervous system diseases, infectious, viral, endocrine diseases, as well as eye and head injuries. rational nutrition - consumption of fruits, vegetables, dairy and meat products, liver; limitation of alcohol consumption, spicy dishes, fried food, quit smoking; intake of antioxidants; observation and treatment by a physician (narrow specialty) depending on the etiology of the disease;

determination of cholesterol level, low-density lipoprotein cholesterol, high-density lipoprotein cholesterol, triglycerides once every 3 months (for patients with atherosclerosis and cardiovascular diseases).

7) Monitoring of patient condition [1,5, 6,7]: Treatment of patients with optic nerve atrophy is carried out taking into account the etiology of the disease. Congenital optic nerve atrophy with stationary course is not subject to treatment, examination by ophthalmologist (visometry, perimetry, tonometry, VEP) once a year.

Patients with optic nerve atrophy caused by intracranial processes and compression of the peripheral neuron of the visual pathway (tumors, aneurysms) are indicated for neurosurgical treatment and further observation and treatment by a neuropathologist or neurosurgeon.

Conservative treatment is carried out in accordance with the underlying disease twice a year in day hospital conditions or on an outpatient basis (with visual acuity below 0.01, conservative therapy or electrostimulation is unpromising).

8) Indicators of treatment effectiveness: increase in electrical sensitivity of the optic nerve by 2-5% (according to computer perimetry data), increase in amplitude and/or decrease in latency by 5% (according to VEP data).

CLINICAL PROTOCOL FOR DIAGNOSIS AND TREATMENT

DIAGNOSIS AND TREATMENT AT OUTPATIENT LEVEL: 1) Diagnostic criteria for diagnosis [1-16,24]: Complaints: gradual decrease in visual acuity (of varying severity), changes in visual field (scotomas, concentric constriction, visual field loss), impaired color perception. History: presence of intracranial mass lesions, intracranial hypertension, demyelinating CNS lesions, carotid artery disease,

systemic diseases (including vasculitis), intoxications (including alcohol), previous optic neuritis or ischemic neuropathy, retinal vascular occlusions, use of medications with neurotoxic effects during the past year; head and neck trauma, cardiovascular diseases, hypertensive disease, acute and chronic cerebrovascular disorders, atherosclerosis, meningitis or meningoencephalitis, inflammatory and mass processes of paranasal sinuses, profuse bleeding.

Physical examination: external examination of the eyeball (limited eye movement, nystagmus, exophthalmos, upper eyelid ptosis) (LE – C) [7]. examination of corneal reflex – may be reduced on the affected side (LE – C) [7].

Laboratory investigations [7]: biochemical blood analysis: blood cholesterol, low-density lipoproteins, high-density lipoproteins, triglycerides; coagulogram; (LE – C) ELISA for herpes simplex virus, cytomegalovirus, toxoplasmosis, brucellosis, tuberculosis, rheumatic tests (as indicated, to exclude inflammatory process). (LE – C)

Instrumental investigations (LE - C) [7]: visometry*: visual acuity may range from 0.7 to practical blindness. With papillomacular bundle involvement, visual acuity is significantly reduced; with minor papillomacular bundle involvement and involvement of peripheral optic nerve fibers, visual acuity is slightly reduced; with involvement of only peripheral nerve fibers - remains unchanged. refractometry: presence of refractive anomalies will allow differential diagnosis with amblyopia. Amsler test - line distortion, blurring of the pattern (papillomacular bundle involvement). perimetry: central scotoma (with papillomacular bundle involvement); various forms of visual field constriction (with peripheral optic nerve fiber involvement); with chiasm involvement - bitemporal hemianopsia, with optic tract involvement - homonymous hemianopsia. With intracranial optic nerve involvement, hemianopsia occurs in one eye. Kinetic color perimetry – visual field constriction for green and red, less commonly – for yellow and blue. Computer perimetry determines the quality and quantity of scotomas in the visual field, including within 30 degrees from the fixation point. dark adaptation testing: impaired dark adaptation.

color vision testing: (Rabkin tables) - impaired color perception (elevated color thresholds), more commonly in the green-red part of the spectrum, less commonly - yellow-blue. tonometry: possible elevation of IOP (in glaucomatous optic nerve atrophy).

biomicroscopy: on the affected side - afferent pupillary defect: reduced direct pupillary light reaction with preserved consensual pupillary reaction. Ophthalmoscopy: - early optic nerve atrophy – pallor appears against the pink color of the optic disc, which subsequently becomes more intense. - partial optic nerve atrophy – pallor of the temporal half of the optic nerve, Kestenbaum's sign (reduction in the number of capillaries on the optic disc to 7 or fewer), arteries narrowed, - incomplete optic nerve atrophy – uniform pallor of the optic nerve, moderately expressed Kestenbaum's sign (reduction in the number of capillaries on the optic disc), arteries narrowed, - complete optic nerve atrophy – total pallor of the optic nerve, vessels narrowed (arteries narrowed more than veins). Markedly expressed Kestenbaum's sign (reduction in the number of capillaries on the optic disc – to 2-3 or capillaries may be absent).

In primary optic nerve atrophy, the optic disc margins are sharp, its color is white, grayish-white, bluish or slightly greenish. In red-free light, the contours remain sharp, whereas the optic disc contours normally become indistinct. In red light with optic disc atrophy - blue color. In secondary optic nerve atrophy, the optic disc margins are indistinct, blurred, the optic disc is gray or dirty-gray, the vascular funnel is filled with connective or glial tissue (in the late period, the optic disc margins become sharp).

optical coherence tomography: reduction in area and volume of the neuroretinal rim of the optic disc, decreased thickness of the nerve fiber layer of the optic disc and in the macula.

Heidelberg retinal laser tomography – decreased depth of the optic nerve head, area and volume of the neuroretinal rim, increased excavation area. In partial optic nerve atrophy, the optic nerve head depth range is less than 0.52 mm, rim area - less than 1.28 mm2, excavation area - more than 0.16 mm2. fluorescein angiography of the fundus: hypofluorescence of the optic disc, arterial narrowing, absence or reduction in the number of capillaries on the optic disc;

electrophysiological studies (visual evoked potentials) - decreased VEP amplitude and prolonged latency. With papillomacular and axial optic nerve bundle involvement, electrical sensitivity is normal; with peripheral fiber involvement, the electrical phosphene threshold is sharply elevated. Lability is especially sharply reduced in axial

lesions. During progression of the atrophic process in the optic nerve, retino-cortical and cortical time significantly increases; USDG of head, neck, and eye vessels: reduced blood flow in the ophthalmic, supratrochlear artery and intracranial portion of the internal carotid artery; MRI of brain vessels: demyelination foci, intracranial pathology (tumors, abscesses, brain cysts, hematomas); orbital MRI: compression of the orbital portion of the optic nerve; orbital radiography by Riese – disruption of optic nerve integrity.

2) Diagnostic algorithm: Appendix No. 1

3) Differential diagnosis and justification for additional examination methods: Table 1 – Differential diagnosis of optic nerve atrophy Diagnosis | Justification | Examinations for differential diagnosis | Criteria for exclusion of diagnosis Amblyopia Significant decrease in vision in the absence of pathology of the anterior segment of the eye and retina.

Physical examinations (external examination of the eyeball) | In a small child - presence of strabismus, nystagmus, inability to clearly fix gaze on a bright object. In older children - decreased visual acuity and absence of improvement with correction, impaired orientation in unfamiliar places, strabismus, habit of closing one eye when looking at an object or reading, head tilt or turn when looking at an object of interest.

Refractometry | Anisometropic amblyopia develops with uncorrected high-degree anisometropia in the eye with more pronounced refractive disorders (myopia more than 8.0 D, hypermetropia more than 5.0 D, astigmatism more than 2.5 D in any meridian), refractive amblyopia - with prolonged absence of optical correction of hypermetropia, myopia or astigmatism with difference in refraction of both eyes: hypermetropia more than 0.5 D, myopia more than 2.0 D, astigmatic 1.5 D.

DIAGNOSIS AND TREATMENT AT INPATIENT LEVEL:

1) Diagnostic criteria at inpatient level: Complaints: gradual decrease in visual acuity (of varying severity), changes in visual field (scotomas, concentric narrowing, visual field loss), impaired color perception. History: presence of intracranial mass lesions, intracranial hypertension, demyelinating CNS lesions, carotid artery lesions, systemic diseases (including vasculitis), intoxications (including alcohol), previous optic neuritis or ischemic neuropathy, retinal vascular occlusions, taking medications with neurotoxic effects during the past year; head and neck trauma, cardiovascular diseases, hypertensive disease, acute and chronic cerebrovascular disorders, atherosclerosis, meningitis or meningoencephalitis, inflammatory and mass processes of paranasal sinuses, profuse bleeding.

Physical examination:

• external examination of the eyeball [7];
• examination of corneal reflex (LE - C) [7];

Laboratory investigations (LE - C) [7]:

• biochemical blood analysis: blood cholesterol, low-density lipoproteins, high-density lipoproteins, triglycerides (as indicated);
• coagulogram (as indicated);
• ELISA for herpes simplex virus, cytomegalovirus, toxoplasmosis, brucellosis, tuberculosis, rheumatic tests (as indicated).

Instrumental investigations (LE - C) [7]:

• visometry (LE - C) [7];
• refractometry (LE - C) [7];
• Amsler test (LE - C) [7];
• perimetry (LE - C) [7];
• dark adaptation testing (LE - C) [7];
• color vision testing (LE - C) [7];
• tonometry (LE - C) [7];
• biomicroscopy (LE - C) [7];
• ophthalmoscopy (LE - C) [7];
• Heidelberg retinal laser tomography (LE - C) [7];
• fluorescein angiography of the fundus (LE - C) [7];
• electrophysiological studies (LE - C) [7];
• USDG of vessels of head, neck, eye (LE - C) [7];
• MRI of cerebral vessels (LE - C) [7];
• MRI of orbit (LE - C) [7];
• Orbital X-ray according to Rhese (LE - C) [7].

List of references:

1 Gustov A.V., Sigriansky K.I., Stolyarova Zh.P. Practical neuro-ophthalmology, Vol.: Nizhny Novgorod.- 2003.- 264 p. 2 Tron E.Zh. Diseases of the visual pathway / 2nd edition revised and expanded.- L.: Medicine, 1968.-551p. 3 Katsnelson L.A., Forofonova T.I., Bunin A.Ya. Vascular diseases of the eye. - M.: Medicine, 1990. - P. 120-149. 4 Therapeutic ophthalmology /Ed. by M.L. Krasnov, N.B. Shulpina.M.: Medicine, 1985.- 559p. 5 Kanski Jack J. Clinical ophthalmology, a systematic approach/ Transl. from Eng.-M.: Logosfera, 2006. 6 Nikiforov A.S., Guseva M.R. Neuro-ophthalmology: GEOTAR-Media.- 2008.644p. 7 Atkov O.Yu., Leonova E.S. Patient management plans "Ophthalmology" Evidence-based medicine // GEOTAR – Media: M., 2011. - P.83-99. 8 Zhaboedov G.N., Skrinnik R.L. Optic nerve lesions.Kiev:"Zdorov'ya",2006.-472p.

Hayreh S. Central retinal vein occlusion. Differential diagnosis and management // Trans. Amer. Acad. Ophthal. - 1977. - Vol. 83. - P. 379-386. 10 Hayreh S., Rojas P., Podnajsky P. et al. Ocular neovascularisation with retinal vascular occlusion. Incidence of ocular neovascularisation with retinal vascular occlusion. // Ophthalmology. - 1983. - Vol. 90. - P. 488-505. 11 Hayreh S., Podnajsky P. et al. 1.Ocular neovascu-larisation with retinal vascular occlusion. 2. Occurence in central and branch retinal artery occlusion / / Arch. Ophthal. - 1982. - Vol. 100. - P. 1585-1596. 12 Katsnelson L.A., Lysenko V.S., Balishanskaya T.I. Clinical atlas of fundus pathology. –M., 4th ed. - 2013. - 120 p. 13 Alyabyeva Zh.Yu., Egorov A.E. Laser scanning ophthalmoscopes: prospects for their application in ophthalmology // Vestnik oftalmologii. – 2000. – №4. – P.36-38. 14 Optical coherence tomography edited by A.G. Shchuko, V.V. Malyshev 2010.-128p. 15 Marchenkova T.E., Ioileva E.E. Method of differential diagnosis of amblyopia and partial optic nerve atrophy in children/ RF Patent, № 2261649. 16 Shamshinova A.M., Volkov V.V. Functional research methods in ophthalmology.– M.: Medicine, 1998.– P. 89. 17 Egorov E.A., Astakhov Yu.S., Stavitskaya T.V. Ophthalmopharmacology. Guide for physicians.- M.: "GEOTAR-Med", 2004.-464p. 18 Egorov E.A. Rational pharmacotherapy in ophthalmology.- M.: Litterra.2004. 19 Kanyukov V.N., Kim S.M., Meshcheryakova G.F. Treatment of partial optic nerve atrophy using peptide preparations// OSU.-2010.-312.-8890. 20 Nepomnyashchikh V.A. Cellular bioregulators in complex therapy of eye diseases: monograph-guide.-M: RegBioMed, 2010.-89p. 21 Tarasova L.N., Kiseleva T.N., Fokin A.A. Ocular ischemic syndrome. – M.: "Medicine", 2003.-176p. 22 Neuroophthalmology: Color atlas and synopsis of clinical ophthalmology/ P.Savino, H.Danesch-Meyer.- New York. Etc.: "McGraw-Hill", 2003.-263р. 23 Avetisov S.E. Ophthalmology. National guideline. M.: GEOTAR-Media, 2008.-1017p. 24 Zhaboedov G.D., Skripnik R.L., Baran T.V. Ophthalmology K.: VSI "Medicine", 2011. - 448 p.

Approved by the Joint Commission on Quality of Medical Services of the Ministry of Health and Social Development of the Republic of Kazakhstan on June 9, 2016

Protocol No. 4

OPTIC NERVE ATROPHY

Contents:

Correlation of ICD-10 and ICD-9 codes 1 Protocol development date 1 Protocol users 2 Patient category 2 Evidence level scale 2 Definition 3 Classification 3

Diagnosis and treatment at outpatient level

Diagnosis and treatment at emergency care stage

13

Diagnosis and treatment at inpatient level

14

Medical rehabilitation

17 Palliative care 17 Abbreviations used in the protocol 17 List of protocol developers 18 Conflict of interest 18 List of reviewers 18 List of references 18

Correlation of ICD-10 and ICD-9 codes:

ICD-10

Code Name H47.2 Optic nerve atrophy.

ICD-9

Protocol development date: 2016.

Protocol users: general practitioners, ophthalmologists, neurosurgeons, neurologists, rheumatologists.

Patient category: adults and children.

Evidence level scale:

Evidence level

I

II

III

IV V

Type of evidence

Evidence obtained from meta-analysis of a large number of well-designed randomized studies. Randomized studies with low levels of false-positive and false-negative errors. Evidence based on results of at least one well-designed randomized study. Randomized studies with high levels of false-positive and false-negative errors. Evidence based on results of well-designed non-randomized studies. Controlled studies with one patient group, studies with historical control group, etc. Evidence obtained from non-randomized studies. Indirect comparative, descriptive correlational studies and clinical case studies. Evidence based on clinical cases and examples.

A High-quality meta-analysis, systematic review of RCTs or large RCT with very low probability (++) of systematic error, the results of which can be applied to the relevant population.

B High-quality (++) systematic review of cohort or case-control studies or high-quality (++) cohort or case-control study with very low risk of systematic error or RCT with low (+) risk of systematic error, the results of which can be applied to the relevant population.

C Cohort or case-control study or controlled study without randomization with low risk of systematic error (+), the results of which can be applied to the relevant population or RCT with very low or low risk of systematic error (++ or +), the results of which cannot be directly applied to the relevant population.

D Description of case series or uncontrolled study or expert opinion.

HRT OCT

Leber's hereditary atrophy Sharp decrease in vision in both eyes in the absence of pathology of the anterior segment of the eye and retina.

Complaints and history

According to HRT data: optic nerve head depth range more than 0.64 mm, optic nerve rim area more than 1.48 mm2, optic nerve excavation area less than 0.12 mm2.

The disease develops in male members of one family aged 13 to 28 years. Girls become ill very rarely and only if the mother is the proband and the father suffers from this disease. Heredity is linked to the X chromosome. Sharp decrease in vision in both eyes within several days. General condition is good, sometimes patients complain of headache.

Ophthalmoscopy Perimetry

Initially, hyperemia and slight blurring of ONH borders appear. Gradually the ONH acquires a waxy character, becomes pale, especially in the temporal half.

In the visual field - central absolute scotoma for white color, peripheral boundaries are normal.

Hysterical amblyopia (amaurosis)

Sudden deterioration of vision or complete blindness in the absence of pathology of the anterior segment of the eye and retina.

Complaints and history

Physical examination (external examination of the eyeball) Visometry

Hysterical amblyopia in adults - sudden deterioration of vision, persisting from several hours to several months, develops against the background of strong emotional shocks. More often observed in women aged 16-25 years. Complete absence of pupillary light reflex is possible.

Decreased visual acuity of varying degrees up to blindness. With repeated examinations, data may differ completely from previous ones.

Ophthalmoscopy ONH is pale pink, contours are clear, Kestenbaum's sign is absent.

Concentric narrowing of visual field, characteristic violation of normal type of boundaries - the widest visual field for red color; less often—hemianopsia (homonymous or heteronymous).

VEP

VEP data - normal.

Optic nerve hypoplasia

Bilateral decrease or complete loss of vision in the absence of pathology of the anterior segment of the eye and retina.

Visometry

Physical examination (external examination of the eyeball)

Optic nerve hypoplasia is accompanied by bilateral decrease in vision (in 80% of cases from moderate degree to complete blindness).

Afferent pupillary reflex is absent. Unilateral ONH change often combines with strabismus and can be noticed by relative afferent pupillary defect, as well as unilateral weak or absent fixation (instead of fixation nystagmus).

The optic nerve head is reduced in size, pale, surrounded by a weakly expressed pigment ring. The outer ring (the size of a normal disc) consists of the lamina cribrosa, pigmented sclera, and choroid. Variants: a small yellow-white disc with a double ring or complete absence of the nerve and aplasia of vessels. In bilateral processes, the disc is often difficult to detect; in this case, it is identified by the course of the vessels.

Perimetry With preservation of central vision, it is possible to detect defects in the visual fields.

Consultation with a neurologist, endocrinologist, laboratory investigations

Optic nerve hypoplasia rarely combines with septo-optic dysplasia (Morsier syndrome: absence of the septum pellucidum and pituitary gland, which is accompanied by disorders of thyroid function and other hormonal disturbances: possible growth retardation, hypoglycemic episodes, combination with mental retardation and malformations of brain structures).

Coloboma of the optic nerve head Optic nerve pathology Ophthalmoscopy

On ophthalmoscopy - the optic nerve head is increased in size (elongation of the vertical dimension), deep excavation or local excavation and increased crescent-shaped pigmentation with partial involvement of the inferior-nasal part of the optic nerve head. When the choroid is also involved, a demarcation line appears, represented by bare sclera. Pigment clumps may mask the boundary between normal tissue and the coloboma. Glial tissue may be present on the surface of the optic nerve head.

MRI

MRI - the sheaths of the optic canal are weakly expressed or absent.

Morning glory syndrome Optic nerve pathology Physical examinations (external examination of the eyeball) Almost all patients with unilateral pathology have strabismus and high myopia of the affected eye.

Visometry Visual acuity is more often reduced but may also be very high.

Refractometry Often in unilateral processes - high myopia of the affected eye.

On ophthalmoscopy - the optic nerve head is enlarged and appears to be located in a funnel-shaped depression. Sometimes the optic nerve head is elevated; the position of the optic nerve head may also vary from staphylomatous depression to its prominence; around the nerve are areas of transparent grayish retinal dysplasia and pigment clumps. The demarcation line between the optic nerve head tissues and normal retina is indistinguishable. Multiple abnormally branching vessels are identified. Most patients have zones of local retinal detachment and radial retinal folds within the excavation.

Perimetry Visual field defects are possible: central scotomas and enlargement of the blind spot.

Consultations with an otolaryngologist

Morning glory syndrome occurs as an independent manifestation or may be combined with hypertelorism, cleft lip, palate, and other anomalies.

4) Treatment tactics: Non-pharmacological treatment: General regimen 3, diet No. 15.

Pharmacological treatment (depending on the severity of the disease) [7,17-20]

Conservative (neuroprotective) treatment is aimed at enhancing blood circulation and improving trophism of the optic nerve, stimulating the vital activity of preserved and/or apoptotic nerve fibers.

Pharmacological treatment includes neuroprotective drugs with direct (directly protect retinal ganglion cells and axons) and indirect (reduce the action of factors causing nerve cell death) action.

List of essential medicines: Retinoprotectors:

ascorbic acid 5% 2 ml intramuscularly once daily for 10 days, to reduce vascular wall permeability and stabilize endothelial cell membranes (LE - C) [7,17,18]. Antioxidants:

tocopherol 100 IU 3 times daily - 10 days, to improve tissue oxygen supply, collateral circulation, strengthen vascular wall (LE - C) [7,17,18]. Drugs improving metabolic processes (direct neuroprotectors):

retinalamin for intramuscular administration 1.0 ml and/or parabulbar injection 5 mg 0.5 ml parabulbar once daily for 10 days (LE - C) [7,17,18, 19,20]; List of additional medicines:

vinpocetine - adults 5-10 mg 3 times daily for 2 months. Has vasodilating, antihypoxic and antiplatelet action (LE - C) [7,17,18].

cyanocobalamin 1 ml intramuscularly once daily for 5/10 days (LE - C) [7,17,18].

Algorithm of actions in emergency situations: none.

Other types of treatment: electrical stimulation - aimed at restoring the function of nerve elements that were functional but did not conduct visual information;

formation of a focus of persistent excitability, which leads to restoration of activity of nerve cells and their connections that previously functioned weakly; improvement of metabolic processes and blood circulation, which promotes restoration of the myelin sheath around the axial cylinders of optic nerve fibers and, accordingly, leads to acceleration of action potential conduction and revival of visual information analysis (LE - C) [6,7,23].

DIAGNOSIS AND TREATMENT AT THE EMERGENCY CARE STAGE: 1) Diagnostic measures: none. 2) Pharmacological treatment: none.

2) Diagnostic algorithm: Appendix No. 1

3) List of main diagnostic measures [7]:

• Visometry (without/with full correction) (LE – C) [7];
• Autorefractometry (LE – C) [7];
• Measurement of intraocular pressure by Maklakov method (LE – C) [7];
• Biomicroscopy (LE – C) [7];
• Ophthalmoscopy (LE – C) [7];
• Perimetry (LE - C) [7]; (for colors) (LE – C) [7].

4) List of additional diagnostic measures:

• Heidelberg retinal laser tomography (LE - C) [7];
• Optical coherence tomography of the optic nerve (LE - C) [7];
• Ultrasound of the eyeball * (LE – C) [7];
• USDG* (LE - C) [7];
• Fluorescein angiography of the fundus (LE - C) [7];
• Computer perimetry (LE - C) [7];
• Electroretinography (LE - C) [7];
• Registration of visual evoked potentials (LE - C) [7];
• Cycloscopy (LE - C) [7];
• MRI of brain vessels (LE - C) [7];
• MRI of the orbit (LE - C) [7];
• X-ray of the orbit by Riese method (LE - C) [7].

5) Treatment tactics:

Treatment tactics for optic nerve atrophy are aimed at reducing edema and inflammatory infiltration, enhancing blood circulation and improving nerve trophism, stimulating the vital activity of preserved nerve fibers and those in a state of parabiosis.

Non-drug treatment:

General regimen 3, diet No. 15.

Drug treatment [7, 17-20]

List of main medications:

Retinoprotectors:

• Ascorbic acid 5% 2 ml intramuscularly once daily for 10 days, to reduce vascular wall permeability and stabilize endotheliocyte membranes (LE – C) [7,17,18].

Antioxidants:

• Tocopherol 100 IU 3 times daily – 10 days, to improve tissue oxygen supply, collateral circulation, strengthen vascular wall (LE – C) [7,17,18].

Drugs improving metabolic processes (direct neuroprotectors):

• Retinalamin for intramuscular administration 1.0 ml and/or parabulbar injection 5 mg 0.5 ml parabulbar once daily for 10 days (LE - C) [7,17,18, 19,20];

List of additional medications:

• Vinpocetine – for adults 5-10 mg 3 times daily for 2 months. Has vasodilating, antihypoxic and antiplatelet action (LE – C) [7,17,18].
• Cyanocobalamin 1 ml intramuscularly once daily for 5/10 days (LE – C) [7,17,18].

Surgical intervention: none.

Other types of treatment:

• Electrostimulation.

6) Indications for consultation with narrow specialists:

• Consultation with therapist – to assess general condition of the body; (LE - C) [7];
• Consultation with cardiologist – high blood pressure level is one of the main risk factors for development of retinal and optic nerve vascular occlusions; (as indicated) (LE - C) [7];
• Consultation with neurologist - to exclude demyelinating disease of the CNS and clarify the topical zone of visual pathway damage; (as indicated) (LE - C) [7];
• Consultation with neurosurgeon - when patient develops signs of intracranial hypertension or symptoms characteristic of brain mass lesion; (as indicated) (LE - C) [7];
• Consultation with rheumatologist - in presence of symptoms characteristic of systemic vasculitis;
• Consultation with vascular surgeon to decide on the need for surgical treatment in presence of signs of occlusive process in the internal carotid and ophthalmic artery system (appearance of scotoma fugax in patient); (as indicated) (LE - C) [7];
• Consultation with endocrinologist – in presence of diabetes mellitus/other endocrine system pathology; (as indicated) (LE - C) [7];
• Consultation with hematologist (when blood diseases are suspected); (as indicated) (LE - C) [7];
• Consultation with infectious disease specialist (when vasculitis of viral etiology is suspected) (as indicated) (LE - C) [7];
• Consultation with otolaryngologist – when inflammation or neoplasm in maxillary or frontal sinus is suspected (as indicated) (LE - C) [7].

7) Indications for transfer to intensive care and resuscitation department: none.

8) Indicators of treatment effectiveness:

• Increase in electrical sensitivity of the optic nerve by 2-5% (according to computer perimetry data);
• Increase in amplitude and/or decrease in latency by 5% (according to VEP data).

Medical rehabilitation: none.

Palliative care: none.

Abbreviations used in the protocol:

AZN – optic nerve atrophy VGD – intraocular pressure DZN – optic nerve disc ZVP – visual evoked potentials ## ZN – optic nerve IBS – ischemic heart disease IFA – immunofluorescent analysis ## KP – computer perimetry NRT – Heidelberg retinal laser tomography OST – optical coherence tomography UZDG – ultrasound Doppler UZI – ultrasound examination FAG – fluorescein angiography CNS – central nervous system EFI - electrophysiological studies

List of protocol developers:

1) Aldasheva Neilya Akhmetovna – Doctor of Medical Sciences, JSC "Kazakh Scientific Research Institute of Eye Diseases", Deputy Chairman of the Board for Science and Strategic Development. 2) Stepanova Irina Stanislavovna – Doctor of Medical Sciences, JSC "Kazakh Scientific Research Institute of Eye Diseases", senior lecturer of the postgraduate education department.

3) Ogai Galina Osifovna – JSC "Kazakh Research Institute of Eye Diseases", highest category physician, Head of Department 1.

4) Mukazhanova Ainagul Serikovna – JSC "Kazakh Research Institute of Eye Diseases", ophthalmologist of the paid services department.

5) Utelbayeva Zauresh Tursunovna – Doctor of Medical Sciences, RSE on REM "Kazakh National Medical University named after S.D. Asfendiyarov", Professor of the Department of Eye Diseases.

6) Panchenko (Puchko) Snezhanna Konstantinovna – Candidate of Medical Sciences, Astana branch of JSC "Kazakh Research Institute of Eye Diseases", ophthalmologist.

7) Tleubayev Nurlan Turebekovich – Candidate of Medical Sciences, SCP on REM "Almaty Multidisciplinary Clinical Hospital" Healthcare Department of Almaty city, Head of Ophthalmology Department.

8) Khudaibergenova Makhira Seyduалиevna – JSC "National Scientific Medical Center of Oncology and Transplantology", clinical pharmacologist.

Conflict of interest: none.

Reviewers: Shusterov Yuri Arkadyevich – Doctor of Medical Sciences, Professor, RSE on REM "Karaganda State Medical University", Head of the Department of Ophthalmology and Resuscitation.

Protocol revision conditions: protocol revision 3 years after its publication and from the date of its entry into force or in the presence of new methods with evidence level.

Appendix 1. Diagnostic algorithm

Indications for Hospitalization

13

INDICATIONS FOR HOSPITALIZATION WITH INDICATION OF TYPE OF HOSPITALIZATION: 10.1 Indications for planned hospitalization:

Inpatient facility with round-the-clock stay (regional eye hospitals, ophthalmology departments of multidisciplinary city or regional hospitals) for comprehensive treatment (electrostimulation + course of conservative therapy):

children and adults with newly diagnosed optic nerve atrophy caused by previous inflammatory (neuritis) or vascular (ischemic neuropathy) disease, with visual acuity not lower than 0.01;

children and adults with bilateral optic nerve atrophy with visual acuity not lower than 0.01 in the functionally better eye (in the absence of otorhinolaryngological, neurological or vascular pathology requiring treatment in a specialized hospital).

Day hospital:

patients with optic nerve atrophy with positive effect from previously conducted therapy (increase in visual acuity, and/or expansion of peripheral visual field boundaries, and/or improvement in VEP parameters);

patients with optic nerve atrophy with visual acuity not lower than 0.01 in the affected eye.

Indications for emergency hospitalization: none.