LEUKOCORIA causes ,risk factors and treatment.pptx

Leukokoria is a medical term used to describe a white or yellowish reflection in the pupil of the eye. This condition is often associated with abnormalities in the retina or lens of the eye, such as retinoblastoma (a type of eye cancer that usually occurs in young children), cataracts, or other conditions that affect the eye.

Leukokoria can be detected by a healthcare provider during a routine eye exam or by a parent or caretaker noticing the unusual reflection in the eye of a child. Prompt medical evaluation is crucial in order to determine the underlying cause of leukokoria and to initiate appropriate treatment. Early detection and treatment of conditions like retinoblastoma can greatly improve outcomes and prevent further complications.

1. LEUKOCORIA Yordanos Tsehai ( assistance professor of ophthalmology)

2. Leukocoria is an abnormal white reflection of the eye It is a medical signs for a number of medical conditions It can be detected by a clinician using retinoscope or direct ophthalmoscope

3. Differential diagnosis of leukocoria Congenital cataract Retinoblastoma PHPV (PFV) Toxocariasis Coats disease ROP Coloboma of optic disk and retina Retinal detachment Norries disease

4. Anatomic location for leukocoria to occure Pupillary space …….cataract Vitreous ……when clumps of cell present, mass from the retina, retino lental membrane Vitreous hemorrhage retinoblastoma Retina …..posterior retinal fold, defect ( coloboma ), feathery white patch Retinal coloboma

5. Clinical work up for children with leukocoria The differential diagnosis can be narrowed through History Age ate presentation at birth…PFV, congenital cataract 1yr-3 yr ….retinoblastoma (RB), congenital cataract preschool age g…. toxocariasis , Coats disease premature children… ROP Ocular examination …fundus examination Investigation….B-scan, CT-scan, MRI

6. retinoblastoma It is a common congenital malignant tumour arising from the neurosensory retina in one or both eyes. Incidence Retinoblastoma is the most common intraocular malignancy afflicting children  It occurs equally in males and females. 60% of cases are unilateral, and 40% are bilateral. Affect young children less than 4 years of age

7. Genetics and heredity Retinoblastoma (RB) gene has been identified on the long-arm of chromosome 13 (13q 14) and is a ‘cancer suppressor’ or antioncogenic ’ gene. Deletion or inactivation of this protective gene by two mutations results in occurrence of retinoblastoma. Retinoblastoma may arise as hereditary and non herditary forms.

8. 1. Hereditary or familial cases. Accounts for 40% of all cases. All bilateral cases and about 15% of the unilateral cases are hereditary. Most hereditary cases are multifocal. Some hereditary cases have trilateral retinoblastoma ( i.E. , Have associated intracranial pinealoblastoma ). Inheritance is autosomal dominant

9. 2. Non-hereditary or sporadic cases. Accounts for 60% of all cases. All non-hereditary cases are unilateral and unifocal and accounts for 85% of the all unilateral cases of retinoblastoma. Patient is not predisposed to get second nonocular cancer .

10. Clinical presentation 1. Leukocoria or yellowish-white pupillary reflex is the commonest feature noticed in early stage 2. . Squint, usually convergent, may develop in some cases 3. Rarly can present as cellulitis, endophthalmitis , uveitis 4. . Fungating orbital mass in advanced and neglected cases

11. Pattern of growth Entophytic RB Exophytic RB It grows inwards from the retina -it grows outward and separates into the vitreous cavity separates the retina from the choroid

12. Pattern of growth

13. Diagnosis 1.Ophthalmoscopy examination under general anaesthesia : it should be performed in all clinically suspected cases. It should include fundus examination of both eyes after full mydriasis with atropine 2.Ocular U/S: Demonstrate echogenic mass on vitreous on B mode Reflective intrinsic echoes of fine calcification on A mod Accuracy 80%

14. 3. Computed tomography: May demonstrate a solid intraocular tumor with characterstic of intratumor calcification 4. Magnetic resonance imaging Optic nerve involvement The presence of associated intracranial tumor-trilateral RB Prefered for children youger than 1 year ..Ct scan increase cancer risk 5. Histo pathology…for definitive diagnosis

15. Fnac from tumor should be avoided….increase risk of vitreous seeding If there is clear evidence of tumor outside the eye, the full metastatic evaluation should be done…bone marrow aspiration Lumbar puncture

16. treatment 1. Tumour destructive therapy. When tumour is diagnosed early stage I Chemoreduction followed by local therapy (cryotherapy, thermochemotherapy or brachytherapy) is recommended for large tumours Radiotherapy (external beam radiotherapy with chemotherapy) is recommended for medium size tumour Cryotherapy is indicated for a small tumour located anterior to equator. Laser photocoagulation is used for a small tumour located posterior to equator

17. 2 . Enucleation with chemotherapy-if the mass is confined to the eye ball only 3. Exentration with chemotherapy- in advanced cases with extra ocular extension Advanced rb with fungating mass

18. Prognosis .1.If untreated the prognosis is almost always bad and the patient invariably dies. Rarely spontaneous regression with resultant cure and shrinkage of the eyeball may occur due to necrosis followed by calcification; 2. Prognosis is fair (survival rate 70-85%) if the eyeball is enucleated before the occurrence of extraocular extension. 3. Poor prognostic factors are: Optic nerve involvement, undifferentiated tumour cells and massive choroidal invasion

19. Persistent fetal vasculature The fetal vasculature, are presumed to nourish the fetus’s developing lens prior to the production of aqueous humor and formation of the anterior chamber.  In normal development, fetal vasculature regresses without complications However, in 95% of premature infants and 3% of full term infants, fetal vasculature persists PFV Is a failure of the regression of a component of fetal vessels within the eye.

20.   Second most common cause of infantile leukocoria PFV is characterized by persistence of fetal vascuature associated with microophthalmia , cataract, and glaucoma

21. Types of pfv Anterior PFV Occurs when the remnant vascular stalk is seen attached to the back of the lens but not extended back to optic nerve This form is typically associated with cataract , glaucoma and retrolenticular membrane

22. Posterior PFV The remnant vascular stalk is seen arising off the optic nerve but not reacjing the lens, and usually not causing cataract. It may be associated with abnormal development of retina, optic nerve, macula

23. A combination of anterior and posterior PFV The band extend from retina to posterior capsule Is the most common type

24. treatment Anterior form is most often treated with observation, lensectomy , anterior vitrectomy, and glaucoma management Posterior form is usually associated with poor visual prognosis regardless of the treatment due to associated retinal and optic nerve abnormality Combined type is also typically associated with poor prognosis due to posterior segment abnormality

25. Ocular toxocariasis Ocular toxocariasis is a rare infection caused by roundworms, toxocara canis and toxocara cati .  Affect mostly young adults and children get infected by soil contaminated food or orofecal

26. Clinical presentation Ocular toxocariasis typically present as posterior uveitis in 3 different sub types Central posterior granuloma toxocaria Peripheral granuloma with peripheral Chronic endophthalmitis granuloma Laboratoy test … ELISA

27. Treatment- --Minimizing inflammation using anti inflammatory agents … Eliminating the offending agent using antiparasitic ( albendazole preferred) …. Adressing vitreous and retinal complication using surgery Prognosis is generaly good for those patients without sequele

28. coat’s disease Coats disease is a telangiectatic neovascular disease of the retina of unknown etiology that frequently affects unilateral eyes of young males. The disease is primarily due to aneurysms and telangiectatic vessels. That are abnormally twisted and leaky This prevent normal flow of blood, and allows flood leak out of the vessles that result with retinal detachmentnt

29. Coats disease

30. Treatment … Observation in minimal telangectic vessels with little or no exudate --- Laser treatment in telangectesia with exudation but no sub retina fluid …. Cryo therapy for patient with sub retinal fluid ---- surgery for retina detachment ---- enucleation for painful blind eye

31. Retinopathy of prematurity (ROP) ROP Occurs in premature infants who are born before the retinal vessels complete their normal growth. Normal retinovascular development in humans is believed to occur initially through vasculogenesis   At about 14-16 weeks gestation. By 22 week of gestational age posterior pole will be vascularized and following this angiogenesis occure via buding through peripheral retina   Migrating endothelial cells are attracted by a gradient of vascular endothelial growth factor (VEGF) toward the ora serrata .

32. The following infants should be screened for ROP: Low birthweight (1500 grams or less) Gestational age (30 weeks or less) 1500 grams < birthweight < 2000g grams or gestational age > 30 weeks who are believed by their pediatrician or neonatologist to be at risk for ROP ( e.G. History of hypotension requiring inotropic support, received supplemental oxygen for more than a few days or without oxygen saturation monitoring)

33. Stage of rop Stage 1 demarcation line:  this line is thin and flat (in the retina plane) and separates the avascular retina anteriorly from the vascularized retina posteriorly. STAGE 2 elevated ridge: demarcation line has 3D appearance

34. STAGE 3   Extraretinal fibrovascular proliferation:  neovascularization extends from the ridge into the vitreous stage 3 STAGE 4 : Partial retinal detachment stage 4 STAGE 5 : Total retinal detachment stage 5

35. Plus disease Additional signs of increased venous dilatation and arteriolar tortuosity of the posterior retinal vessels which can increase in severity to include Poor pupillary dilatation  Vitreous haze Iris vascular engorgement

36. treatment Retinal ablation Cryotherapy Laser Surgical Scleral buckling Vitrectomy

37. Congenital coloboma is developmental defects Either retinal coloboma or optic nerve coloboma can cause leukocoria

38. Good days