ocular tumors among pediatric patient .

This document contains an overview of different types of ocular neoplastic disorders or ocular tumors among pediatric patients. you can have a quick basic concept about ocular tumors among children and a basic management strategy. You will have perfect idea about almost 8 ocular tumors among pediatric patients .

1. NEOPLASTIC DISORDER PRESENTED BY :RIYA BIST PU,HEI PEDIATRIC OPTOMETRY AND EYE BANKING

2. An abnormal mass of tissue forms when cells grow and divide more than they should or do not die when they should. Neoplasms may be benign (not cancer) or malignant (cancer). The overall incidence of childhood ocular malignancy is more during the first five year of life. INTRODUCTION A neoplasm is an abnormal growth of cells, also known as a tumor.

3. Different types of pediatric ocular neoplastic lesions are: Retinoblastoma Rhabdomyosarcoma Neuroblastoma Choroidal melanoma Iris stromal cysts Capillary hemangioma Medulloepithelioma Leukemia & Lymphangioma

4. Primary malignant neoplasm of the retina that arises from the immature retinal cells. unilateral 70 %&bilateral30% non- herediatary (50-60%)or hereditary (40-50%. Incidence: - 1 in 17000 live births RETINOBLASTOMA

5. Unilateral leukokoria Bilateral leukocoria Secondary glaucoma and buphthalmos Iris nodules and pseudohypopyon Orbital inflammation Orbital invasion SIGNS AND SYMPTOMS

6. Quiescent stage 2 ) Glaucomatous stage 3) Stage of extraocular extension 4) Stage of distant metastasis. A pathology specimen of a retinoblastoma tumor from an enucleated eye of a 3-years -old female STAGES OF RETINOBLASTOMA

7. Red reflex testing General examination – Tonometry Measurement of corneal diameter, axial length of the eye Examination with hand-held slit-lamp Ophthalmoscopy • Ocular U/S (B-Scan) - size of tumor, detects calcification CT scan MRI • Systemic assessment - High-risk cases bone scans, bone marrow aspiration, lumber puncture for CSF study INVESTIGATION

8. Treatment options: Enucleation & Exenteration External beam radio therapy( EBRTx ) Local therapies: Plaque Radiotherapy Laser photocoagulation Cryotherapy Thermotherapy Chemotherapy

9. Rhabdomyosarcoma is a rare type of cancer that starts as a growth of cells in soft tissue Soft tissues support and connect organs and other parts of the body. Although rhabdomyosarcoma can start anywhere in the body, it's more likely to start in the: Head and neck area. Urinary system, such as the bladder. Reproductive system, such as the vagina, uterus and testes. Arms and legs. R h a b d o m y o s a r c o m a

10. OCULAR RHABDOMYOSARCOMA Rhabdomyosarcomas develop in the orbital soft tissues. Patients generally present with proptosis (80-100%), globe displacement (80%)   blepharoptosis (30-50%) conjunctival and eyelid swelling (60%) palpable mass (25%) ptosis (25%) and pain (10%) .

11. TREATMENT Surgery Orbital exenteration Radiotherapy chemotherapy

12. Neuroblastoma Neuroblastoma is a cancer that develops from immature nerve cells found in several areas of the body Neuroblastoma most commonly arises in and around the adrenal glands. However, neuroblastoma can also develop in other areas of the abdomen and in the chest, neck and near the spine, where groups of nerve cells exist. Neuroblastoma most commonly affects children age 5 or younger, though it may rarely occur in older children. Some forms of neuroblastoma go away on their own, while others may require multiple treatments.  

13. Metastatic neuroblastoma in the orbit typically produces proptosis and periorbital ecchymosis including drooping of eyelids and unequal pupil size . Dark circles around the eyes.

14. CHOROIDAL MELONOMA Choroidal melanoma is the most common primary malignant intraocular tumor and the second most common type of primary malignant melanoma in the body. Dome-shaped choroidal melanoma The exact cause are not fully understood. Certain risk factors such as : Exposure to UV radiations Fair skin color Light colored eyes ,may increase the chances of developing this condition .

15. Blurred visual acuity Painless and progressive visual field loss Floaters, (developing when areas of necrosis within the tumor or adjacent structures produce vitreous hemorrhage or hyphema ) Severe ocular pain, (occasionally occurring secondary to impingement of choroidal melanomas on posterior ciliary nerves or to high intraocular pressure from acute angle-closure glaucoma) Sign and symptoms

16. INVESTIGATION FUNDUS PHOTO OCT B SCAN

17. I r i s c y s t s An iris cyst is an epithelial-lined cyst that involves a layer of the iris. Primary Cysts Iris pigment epithelium cysts Iris stromal cysts Secondary Cysts Epithelial downgrowth Post-surgical Post-traumatic Cysts secondary to intraocular tumors Iris melanoma Medulloepithelioma Medication-induced Miotics ( phospholine iodide, pilocarpine ) Prostaglandins including latanoprost

18. A rare condition that can occur in children. These cysts are fluid-filled sacs that develop in the stroma , which is the middle layer of the iris, the colored part of the eye. Iris stromal cysts can affect individuals of any age, they are relatively more common in children . The exact cause is still unknown but can be congenital or caused due to trauma . Discomfort. Blurred vision, sensitivity to light, eye redness, or eye irritation. cysts are asymptomatic (many cases) SIGNS AND SYMPTOMS IRIS STROMAL CYST

19. In most cases, iris stromal cysts in children do not require treatment if they are not causing any symptoms or affecting vision. However, if the cysts are large, causing discomfort, or impacting vision, the ophthalmologist may recommend treatment options like surgical removal or laser. TREATMENT

20. Meduloepithelioma age of onset between 2-5 years inner surface of the ciliary body rare in the retina and optic nerve Medulloepithelioma is an tumor within non-pigmented ciliary epithelium . Besides the ciliary epithelium, medulloepithelioma has been noted in the CNS and even orbit Ciliary body medulloepithelioma is the most common tumor arising from the non-pigmented ciliary epithelium. LOCATION

21. Due to the slow-growing nature of medulloepithelioma , patients frequently do not experience symptoms until tumor size is clinically observable . Enucleation is the standard therapy for advanced ciliary body medulloepithelioma .

22. Capillary hemangioma Capillary hemangiomas may not be present at birth but usually appear within the first 6 months of life. Most go away nearly completely by 5 or 6 years of age. Capillary hemangiomas are more common in premature infants and in girls. Capillary hemangiomas can be found anywhere on the body. For the eye, they usually grow on eyelids, conjunctiva, and the eye socket or orbit A capillary hemangioma (“strawberry” birthmark) is a benign (non-cancerous) tumor made up of abnormal tiny blood vessels (capillaries).

23. SIGNS AND SYMPTOMS   Astigmatism amblyopia ptosis strabismus optic nerve atrophy Laser treatments ( Carbon dioxide, argon, neodymium-YAG, and flash-lamp pumped-dye laser) Radiation therapy  Surgical excision   TREATMENT

24. Leukemia   Leukemia  is a type of cancer that affects the blood and bone marrow. It results from abnormal growth of white blood cells  Leukemia occurs most often in adults older than 55, but it is also the most common cancer in children younger than 15. Ocular involvement in leukemia may occur due to direct leukemic infiltration or because of secondary ophthalmic involvement as a result of abnormalities related to blood like anemia, thrombocytopenia, and leukocytosis or because of chemotherapy or immunosuppression .

25. AFFECT ON RESULTS

26. Lymphangioma Lymphangiomas are uncommon, benign malformations of the lymphatic system that can occur anywhere on the skin and mucous membranes. When lymphangioma develops in the eye, it is referred to as orbital lymphangioma or lymphangioma of the orbit Orbital lymphangioma is characterized by the abnormal growth of lymphatic vessels in the tissues surrounding the eye. The tumor is usually present at birth or develops in early childhood.

27. Common signs and symptoms may include; eyelid swelling, proptosis (bulging of the eye), ptosis (drooping of the eyelid), tearing, blurry vision and in rare cases, visual impairment. Some common treatment approaches include surgical excision, cryotherapy , laser therapy to shrink the cystic spaces

28. https://en.wikipedia.org/wiki/Medulloepithelioma https://emedicine.medscape.com/article/1218805-overview?form=fpf https://aapos.org/glossary/capillary-hemangioma https://eyewiki.aao.org/Capillary_Hemangioma https://eyewiki.aao.org/Orbital_Lymphangioma REFERENCES https://www.mayoclinic.org/diseases-conditions/retinoblastoma/symptoms-causes/syc-20351008

29. THANK YOU