Intraocular

1. Intraocular Tumours

2. RETINOBLASTOMA OR GLOMA OF RETINA: Malignant proliferation of neurologia such as occur in the brain and optic nerve are rare in the retina. The usual maliganant growth of the retina is due to proliferation of neural cells (inner or outer nuclear layers) and is better termed retinoblastoma

3. Etiology

4. 1)Age incidence-it is probably always congenital and occurs in childern under 5 years of age. 2)Usually it starts in one eye. When bilateral the second eye is affected independently and not by metastasis or continuity via the optic chiasma. 3)Heredity: Most of the cases are sporadic but a familial from is seen in 4,5% cases.

5. Pathology

6. The tumour consists of small round cell with large basophilic nuclei .The cells may be arranged in rosettes, pseudorosettes and fleurettes. a)Rosettes: The cells tend to be arranged around acentral space. Rosettes are of two types:1)Flexner-Wintersteiner which are typical ofretinoblastoma and contain mucopolysaccharide in the lumen

7. 2)Homer-Wright which may be found in other tumours also and contain fibrillar material in the lumen b) Pseudorosettes: Here, the cells tend to be arranged around blood vessel. c)Fleurettes :The arrangement of retinoblastoma cells here resemble the pattern of a flower

8. The cells show a tendency to necrosis and the mass is permeated by calcium deposits. The tumour may show multiple foci which are independent and do notdisseminate from a primary focus . • The cells may be poorly differentiated or well differentiated (neuro-epithelioma).

9. The mass may grow 1)Outward separtating retina from choroid(glioma exophytum ). 2)Inward-toward vitreous (glioma (endophytum 3)Sideward(glioma planum).

10. Symptoms

11. Mother bring the child with complaints of: 1) Amaurotic(blind)cats eye reflex(yellow reflex)or leucocoria(white reflex).in the pupillary area. 2)Visual disability :the child bumps into things on the side of the affected eye indicating visual loss. 3)Convergent squint due to poor vision from involvement of macular area . 4) Red painfuleye.

12. Signs

13. These can be divided into 4 stages .

14. 1)Quiescent stage: Externally the eye is quiet .With ophthalmoscope small yellow or whitish plaques are seen in one or more location in the retina .The plaques increase in size and sometimes fuse into a single large yellow or whitish mass, causing a yellow or white reflex in the pupillary area (amaurotic cats eye or leucocoria ).The surface of the growth shows new vessels, spots of haemorrhage and even calcification. The pupil usually becomes dilated.

15. 2) Stage of glaucoma: The causes of secondary glaucoma are : a) Involvement of vortex veins . b) Involvement of the angle of AC by extension of growth. c) Iris lens diaphragm pushed forward by growth giving rise to peripheral anterior synechiae .

16. Owing to the extensibility of the sclera (upto the age of 3 years) under the increase of pressure, the entire cornea and sclera stretch so the globe enlarges and presents the picture of secondary buphthalmos . In addition acute inflammatory reaction (uveitis) results from extensive necrosis within the tumour.

17. a)Circumcorneal congestion and later conjunctival congestion and chemosis b)cornea large and hazy . c)AC may be cloudy and may show pseudohypopyon (deposit of malignant cells unlike inflammatory cells as in true hypopyon) d)Vitreous :is frequently filled with tumour producing an appearance resembling vitreous abscess.

18. Pain is sever, but is relieved when the tumour bursts through the sclera usually at the limbus . 3)Stage of extraocular extension: Extraocular extenstion occurs through the site of perforation of: a) Anterior and posterior ciliary arteries. b)Vortex veins. c) A long the optic nerve. d) Tumour bursts through the sclera usually at the limbus .

19. The eye ball show proptosis and restriction of movements . The extraocular portion of the tumour grows rapidly and produces a fungating mass protruding between the lids .

20. 4) Stage of metastasis a)Direct extension by continuity along the optic nerve and brain. b)Preauricular and cervical lymphnodes . c)Cranial and other bones like humerus ,sternum. d)Liver is rarely affected.

21. Differential diagnosis

22. Retinoblastoma must be distinguished from a number of condition which have been grouped together under the term pseudoglioma:

23. Inflammation

24. 1)Cyclitic membrane 2)Suppyrative endophthalmitis 3)T.B. of the choroid –confluent or conglomerate type 4)Toxocara infestation (visceral larva migrans)

25. Congenital malformations

26. Congenital Malformation

27. 1)Persistent hyper-plastic primary vitreous . 2)Congenital detached retina.

28. Retrolental fibroplasia

29. Retrolental fibroplasia

30. Intraocular haemorrhage which has become organised

31. Intraocular haemorrhage(1)

32. Intraocular haemorrhage(2)

34. Retinoblastoma(1)

35. Retinoblastoma(2)

36. Pseudoglioma

37. Pseudorosettes

39. In all the cases 1% atropine should be instilled and both eyes examined thoroughly with the opthalmoscope under general anaesthsia. Tension should be checked with the tonometer. In some cases it is impossible to be certain of the diagnosis . Considering that the life of the patient is stake and that the eye in any case is useless as an organ of sight ,these cases should be treated as retinoblastoma.

40. Treatment

41. 1)Quiescent and glaucomatous stage:Enucleation at the earliest opportunity is the safest and still the most effective method of treatment of retinoblastoma .In this operation the importance of excising as long a portion of the optic nerve as possible cannot be over stressed .This is achieved by pulling on 2 traction sutures passed through the insertion of the 2 horizontal rectus muscles and then passing the enucleation scissors along the nasal wall of the orbit towards the optic foramen.

42. 2)Stage of extraocular extension :Exenteration of the orbit has to be done i.e. removal of all the orbital contents including the periosteum

43. 3) Stage of distant metastasis a)Radiotherapy with deep X-ray to the orbit b)Chemotherapy i)T.E.M(Triethylene melanine)0.08mg/kg body weight I.M. ii)Cyclophosphamide- given as oral tablet or I.V.

44. Cryotherapy or photocoagulation is indicated in cases where one eye has been enucleated and small tumour plaques are seen in the seen in the seeing eye. Cryotherapy is employed in peripheral lesions with hazy media . photocoagulation is used in posterior lesion with clear media .

45. Prognosis

46. is poor .Death usually occurs due to intracranial extension . 2)MALIGANT MELANOMA OF CHOROID:

47. Malignant Melanoma(1)

48. Malignant Melanoma(2)

49. Etiology

50. 1)It may be mesodermal or neurectodermal in origin . 2)Age incidence :It usually occurs in adults between 40-60 years . 3)It is always primary ,single and unilateral .