Retinal Diseas 3s6n2h

RETINAL DISEASES Marliyanti N. Akib

CONGENITAL AND DEVELOPMENTAL DISORDERS • CLASSIFICATION 1.

Anomalies of the optic disc. These include crescents, situs inversus,

2.

Anomalies of the nerve fibres e.g., medullated

congenital pigmentation, coloboma, drusen and hypoplasia of the optic disc.

(opaque) nerve fibres.

3. Anomalies of vascular elements, such as persistent hyaloid artery and congenital tortuosity of retinal vessels.

4. Anomalies of the retina proper. These include albinism, congenital night blindness, congenital day blindness, Oguchi’s disease, congenital retinal cyst, congenital retinal detachment and coloboma of the fundus.

5. Congenital anomalies of the macula

aplasia, hypoplasia and coloboma.

Medulated nerve fiber layer

RETINITIS I. Non-specific retinitis.

INFLAMMATORY DISORDERS OF THE RETINA

It is caused by pyogenic organisms and may be either acute or subacute. 1. Acute purulent retinitis

2. Subacute retinitis of Roth II. Specific retinitis bacterial (tuberculosis, leprosy, syphilis and actinomycosis), viral

(cytomegalic inclusion disease, rubella, herpes zoster), mycotic, rickettsial or parasitic in origin.

Fundus photograph showing typical cytomegalovirus (CMV) retinitis in a patient with AIDS. Note white necrotic retina associated with retinal haemorrhages

Toxoplamosis congenital

RETINAL VASCULITIS

inflammation of the retinal vessels may be primary, exp (Eales’ disease) or secondary to uveitis.

Eales’ disease idiopathic inflammation of the peripheral retinal veins. It is characterised by recurrent vitreous haemorrhage; so also referred to as primary vitreous haemorrhage

Note venous congestion, perivascular exudates and sheets of haemorrhages pr-esent near the affected veins.

VASCULAR DISORDERS OF RETINA •

Retinal artery occlusions

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Retinal vein occlusions

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Diabetic retinopathy

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Hypertensive retinopathy

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Sickle cell retinopathy

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Retinopathy of prematurity

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Retinal telengiectasias

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Ocular ischaemic syndrome

•

Others : retinopahty leucemia, trombocytophenia anemia

Fundus normal

Papil normal

RETINOPATHY

DIABETIC RETINOPATHY Frequent cause of blindness in USA, aged 20 to 64 years

Indonesia blindness due to D.R. increase

PATHOGENESIS The exact cause is still unclear It is believed : Hiperglycemia over an extended period results in a number biochemical and physiologic changes --endothelial damage. Retinal vascular changes : loss of pericyte basement membrane thickening---compromises capillary lumen--- decompensation of endothelial barrier function

Hematologic and biochemical abnormalities Increased platelet adhesiveness Increaser erythrocyte aggregation Abnormal serum lipids

Defective fibrinolysis Abnormal levels of growth hormone : ex

vascular endothelial growth factor (VEGF) Abnormalities in serum and whole blood viscosity

CLASSIFICATION Non proliferative diabetic retinopathy (NPDR) = back ground diabetic retinopathy : Mild Moderate Severe Very severe

Proliferative Diabetic Retinopathy : Early High risk Advanced

Macular edema

NPDR : Retinal microvasculer changes

limited to the retina Micro aneurysme Dot & blot hemorrhage Retinal edema Hard exudates Dilatation & bleading of the vein Intraretinal micro vascular abnormalites (IRMA) Nerve fiber layer infarct (cotton wool spot) Areas of capillary non perfussion

RD : mikroaneurisma + hard exudate

RD 1: cotton wool + Flame shaped

Affect visual function through Capillary disease ischemic Vascular permeability edema Diabetic macular edema The most common : Cause of VA

Proliferative diabetic retinopathy (PDR) Extra retinal fibrovascular proliferation extends beyond the ILM

NPDR

Ischemic retina

Release of vaso poliferative factors

Neovascularization of the retina optic nerve head, anterior segment

PDR Neovascularisation at disc

PDR Neovascularisation elsewhere

COMPLICATION Reduced of visual acuity Vitreous hemorrhage Traction retinal detachment Neurovascular glaucoma

TREATMENT Regulation of blood glucose level Laser photocoagulation

Vitrectomy

HYPERTENSIVE RETINOPATHY  Effect of systemic arterial hypertension to

chronic retinal vascularization Hypertensive retinopathy Hypertensive choroidopathy Hypertensive opticneuropathy

HYPERTENSIVE RETINOPATHY (HR) Hypertensive vascular changes & arterio sclero vascular disease express in HR. Classification of HR, the modified scheir. Grade 0 : No change (a/v 2 : 3) Grade 1 : barely table arterial narrowing a : v = 1 : 2

CLASSIFICATION OF HR Grade 2 : obvious arterial narrowing with focal irregularities. Copper wire arteries Silver wire arteries Banking sign Salus sign

Gunn Sign

Gunn phenomen

Gunn Phenomen

Classification. cont Grade 3 : grade 2 + retinal hemorrhages and/or exudate

Grade 4 : grade 3 + disc swelling

HYPERTENSIVE CHOROIDOPATHY Typically occur in young patients : with acute hypertension ex : Preeclampsia, eclampsia Pheochromacytoma Acute renal failure

Retinopathy Hypertensive pada Hipertensi Renal

Retinopathy Hypertensive pada Hipertensi Renal + edem papil

Hypertensive Optic Neuropathy Flame-shaped hemorrhages around the disc Blurring of the disc margin

Congestion of the retinal vein Secondary macular exudates

Retinopati angiospastik : cotton wool + flame shaped + dot hemorrhage

OKLUSI VENA RETINA SENTRALIS

Hambatan aliran darah vena dari retina Etiologi :

- arteriosklerosis & hipertensi - trombus  peny jantung, DM

- perubahan viskositas darah Gambaran fundus khas berupa dilatasi dan berkelok-keloknya p. drh vena, edema papil N.optik, perdarahan intraretina dan edema retina.

Etiology

1. Pressure on the vein by a sclerotic retinal artery 2.

Hyperviscosity of blood as in polycythemia, hyperlipidemia and macroglobulinemia.

3.

Periphlebitis retinae which can be central or

peripheral. 4.

Raised introcular pressure.

5.

Local causes are orbital cellulitis, facial erysipelas and cavernous sinus thrombosis.

Klasifikasi 1. noniskemik (venous stasis retinopathy), btk ringan kadang dikenal sbg partial, perfused atau retinopati venus statis. 2. Iskemik (haemorrhagic retinopathy), khas  plng sdkt area yg terkena 10 DD Mekanisme Diduga terjadi trombosis di posterior pada lamina kribrosa menyebabkan turbulensi, kerusakan endotel dan pembentukan thrombus

NONISKEMIK - Pembuluh darah dilatasi ringan dan berkelok-kelok pd semua cabang V.Retina sentralis - perdarahan retina bentuk dot dan flame shape di semua kuadran.

- edema makula dgn penurunan visus dgn atau tanpa edema papil. - jarang terjadi neovaskularisasi di segmen anterior

ISKEMIK perdarahan lbh ekstensif di 4 kuadran dan edema retina. ada dilatasi vena dan cotton wool spot. prognosis biasanya jelek. neovask. iris > 60%, tjd 3 – 5 bln sesudah onset.

Gambaran klinik: Subyektif : - visus  tiba-tiba atau bertahap Obyektif : Flame shape hemorrhage Vena retina kongesti & berkelok- kelok

Perdarahan pd membran hyaloid & vitreus Neovaskularisasi di sekitar n.optik & iris Defek lapang pandang sesuai cabang oklusi

CRVO NON-ISCHAEMIC

CRAO - ISCHAEMIC

BRVO

Diagnosis

Anamnesis : KU, riwayat penyakit sistemik Gejala klinik : - funduskopi - FFA

Lab  mendukung peny sistemik Evaluasi : - TIO - Gonioskopi - FFA

Penanganan : Kausal, tdk ada R/ efektif utk noniskemik OVRS. Fotokoagulasi utk neovaskularisasi & edema makula. Kortikosteroid & terapi utk me(-) adhesi platelet.

Komplikasi Trombotik glaukoma (glaukoma sekunder) Retinal detachment Prognosis Komplikasi  jelek

OKLUSI ARTERI RETINA SENTRALIS

Marliyanti N. Akib

Bagian Ilmu Kesehatan Mata

Fakultas Kedokteran Unhas

Hambatan aliran darah ke retina

A. retina sentralis cab I a. oftalmika msk ke dlm n. optik 8 – 15 mm di belakang bola mata 2/3 lapisan bgn dalam retina

Daerah yang paling sering mengalami obstruksi  lamina cribrosa

Etiologi

Atherosklerosis  trombosis  penyebab terbanyak Hipertensi Emboli Penyakit jantung Carotid artery disease Penyakit sistemik (DM) Hiperviskositas darah Trauma (fraktur tulang panjang  emboli lemak)

Gejala : Tajam penglihatan  secara tiba-tiba Tidak nyeri

Amaurosis fugax

CENTRAL RETINAL ARTERY OCCLUSION (CRAO) Oftalmoskopi Retina pucat  iskemi Cherry red spot  refleks merah dr p. drh koroid di bwh fovea

tiny thread like  pembuluh darah kolaps, mengecil seperti benang & halus boxcar phenomenon  arteri sangat menciut, mengempes tanpa darah, kadang terputusputus

BRANCH RETINAL ARTERY OCCLUSION (BRAO)

Retina di bagian distal dari okluasi menjadi edem dengan penyempitan arteriol

Lama kelamaan daerah yang terkena akan menjadi atrofi dan terjadi defek lapangan pandang sektoral

CRAO: milky white

BRAO supero temporal

Kerusakan irreversibel terjadi dlm 90 – 100 mnt Setelah beberapa minggu : edem menghilang atrofi papil dgn batas tegas

neovaskularisasi iris tjd pd 18% 1-12 mgg setelah serangan rata2 : 4 - 5 mgg

Diagnosis berdasarkan : - anamnesis - gambaran klinik Penanganan:

Blm memuaskan  masih dipertanyakan Tujuan  mengembalikan aliran darah secepat mungkin

Menurunkan TIO : Massage bola mata  emboli lepas Parasentesis BMD Acetazolamide

Vasodilator pd daerah retrobulbar

Prognosis

Tergantung : kausa derajat obstruksi lamanya oklusi menetap

Komplikasi :

Glaukoma neovaskuler

Retinopathy of Prematurity

Pathogenesis In the normal foetus, vascular development of the retina occurs in two phases. Fase 1 : True vasculogenesis : 8-21 weeks of foetal development Fase 2 : Angiogenesis : 22 to 40 weeks of development : VEGF dependant Physiologic angiogenesis Pathologic angiogenesis (ROP pathogenesis)

Risk Factors Crucial risk factors : Birth weight Gestational age Number of days oxygen istered

Other risk factors : Multiple births Blood transfusions Respiratory Distress Syndrome (RDS) Sepsis Intra Ventricular Hemorrhage (IVH) Intra Uterine Growth Retardation (IUGR) Vit E deficiency Anemia Seizures.

Screening criteria in Indonesia BW < 1500 gr or GA < 34 minggu Pemeriksaan pada bayi dengan berat lahir lebih atau usia gestasi lebih dapat diminta oleh neonatologis atau dokter anak tergantung keparahan faktor risiko seperti tingginya saturasi O2 selama lebih dari 1 minggu, transfusi berulang Bayi dengan usia gestasi 37 mgg atau lebih tidak perlu pemeriksaan ROP

When to screen ?? Jika usia gestasi > 30 mgg, diperiksa 2-4 mgg setelah kelahiran Jika usia gestasi kurang atau sama dengan 30 mgg, diperiksa 4 mgg setelah kelahiran Setidaknya satu kali pemeriksaan sebelum pulang dari rumah sakit

Stage 1 : Demarcation line

Stage 2 : Ridge

Stage 2 : Ridge

Stage 3 : Extraretinal fibrovascular proliferation

Stage 3 : Extraretinal fibrovascular proliferation

Stage 3 : Extraretinal fibrovascular proliferation

Stage 4 : Partial retinal detachment

Stage 4 : Partial retinal detachment

Stage 5 : Total retinal detachment

How to treat ? Principle is ablation of the ischemic peripheral retina stops release of angiogenic factors Cryotherapy Laser photocoagulation

Surgical treatment Scleral buckling Lens sparing vitrectomy for stage 4 Lensectomy + vitrectomy Open sky vitrectomy for stage 5

Other types of retinopathy Leukemia retinopathy

Thrombocytopenia retinopathy Anemia retinopathy

Trombositopenia

Hemofilia

Leukemia akut

RETINAL DETACHMENT

EMBRIOLOGY OF THE EYE

SUBRETINAL SPACE

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RETINAL DETACHMENT •

Separation of the sensory part of the retina from

the retinal pigment epithelium (RPE). •

There is an accumulation of fluid in the space

between the neural retina and the RPE known as Subretinal fluid.

Pathogenesis There is an embryological explanation for retinal detachment in that the separating layers open up a potential space that existed during the early development of the eye. The inner lining of the eye develops as two layers. The outer of the two layers remains as a single layer of pigmented cells, known as the pigment epithelium. The inner of the two layers becomes many cells thick and develops into the sensory retina.

CLASSIFICATION Rhegmatogenous Retinal Detachment

Tractional Retinal Detachment

Exudative Retinal Detachment

Rhegmatogenous Retinal Detachment This is the most common form of retinal detachment, caused by the recruitment of fluid from the vitreous cavity to the subretinal space via a full-thickness discontinuity (a retinal “break”) in the sensory retina. Etiology 





Retinal degeneration of peripheral retina (lattice degeneration in high myopia >>>. Vitreous change ( posterior vitreous detachment/PVD…. Vitreous traction). Trauma

Vitreus traction

prediposes to retinal breaks

Tractional Retinal Detachment This form of retinal detachment develops as a result of tractional forces within the vitreous gel pulling on the retina, causing the retina to be tented up from the RPE. No retinal breaks. Etiology The retinal detachment can be pulled away by the contraction of fibrous bands in the vitreous, advanced proliferative diabetic retinopathy is the common cause of tractional retinal detachment.

Exudative Retinal Detachment The fluid gains access to the subretinal space through an abnormal choroidal circulation can be found from a choroidal malignant melanoma or, rarely, secondary to inflammation of the RPE or deeper layers of the eye (e.g., scleritis).

Signs and Symptoms Retinal Detachment

Flashes (“Photopsiae”)

Floaters Shadow

Ophthalmoscopy : -

Grey retinal bullous seen in the part of retinal detachment. Retinal vasculatures were the bullous retina. In rhegmatogenous RD, retinal break can be identified.

Tractional ret. Detachment in PDR

Large retinal tears

MANAGEMENT Rhegmatogenous retinal detachment Prophylaxis Retinal tears without significant subretinal fluid can be sealed by means of light coagulation. A powerful light beam from a laser is directed at the surrounds of the tear. Laser spots

Retinal tears

Retinal Surgery Modern retinal reattachment surgery is carried out using either the cryobuckling or vitrectomy technique. Addition treatment are unrarely performed with scleral buckling/vitrectomy are internal drainage, endolaser photocoagulation, or gas/silicon intravitreal injection.

Cryobuckle This involves the sewing of small inert pieces of material, usually silicone rubber, onto the outside of the sclera in such a way as to make a suitable indent at the site of the tear. This is combined with cryopexy to the break. It is often necessary to drain off the subretinal fluid and inject air or gas into the vitreous. In more difficult cases, the eye can be encircled with a silicone strap to provide allround to a retina with extensive degenerative changes.

Scleral buckling

Vitrectomy The detached retina is reattached from within the vitreous cavity. Cannula infusion is inserted to the globe for maintaining the intraocular pressure. A light probe is used to illuminate the operative field

Vitrectomy cutter is used to remove the vitreous, hence relieving the abnormal vitreous adhesions that produced the retinal tear in the first instance

Infusion cannula

light port

vitrectomy cutter port

Injecting air or gas into the vitreous.

Cutting fibrous membrane

Laser endophotocoagulation

Tractional Retinal Detachment Fibrous tissues pulling retinal layer are cut away till retinal re-attach. Sometime combined with silicon injection or endolaser photocoagulation.

Serous Retinal Detachment Depend on the cause of the retinal detachment.

Prognosis The retina can now be successfully reattached by one operation in about 85% of cases. Those in which the macular region was affected by the retinal detachment do not achieve a full restoration of their central Vision. The main cause of failure of surgery is proliferative vitreoretinopathy.This is characterised by excessive “scarring” following initial retinal reattachment surgery When retinal surgery has failed, further surgery might be required and for a few patients a series of operations is necessary.

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