Flashcards in Lecture 4 - Orbital Tumors Deck (24)
Benign, Abnormal proportion of vascular elements present at a cutaneous site - the orbit for our purposes, two types, Which one is associated with high blood flow, and which one is associated with low blood flow?
Orbital Vascular Hamartoma
Capillary and Cavernous Hemangiomas
Capillary Hemangioma- High Blood flow
Cavernous Hemangioma- Low Blood flow
High flow hamartomatous proliferation of primitive vasoformative tissues - Known as orbital hemangioblastic hemangioma or Infantile capillary hemangioma. Most common orbital vascular tumor of childhood - 1 to 2% of infants. Affects Females 3:2, can be heritable, prematurity and maternal chorionic villus sampling are predisposing factors
Capillary Hemangioma - most common orbital vascular tumor of childhood
A concerned mother brings in her 6 month old newborn because she is concerned about a strawberry-like nevus on his upper eyelid. It is unilateral, diffuse subcutaneous, dimpled. Mom says it has been getting larger but seemed to stop growing recently. It started flat, became bulky with mass, and appears to be associated with a small degree of proptosis. Diagnose, Test to confirm, and Treat.
1. Capillary Hemangioma
2. Enhanced MRI or B scan ultrasonography showing smooth lobular mass, often confused with orbital cellulitis
3. Intralesional injections of long and short acting corticosteroids
40 mg triamcinolone acetate and 6 mg betamethasone
40 mg methylprednisolone and 4 mg dexamethasone, repeated at 6 week intervals as needed
What is the most common ocular complication of capillary hemangiomas of the adnexa?
Vision loss resulting from amblyopia (44% to 64% of affected infants) or rarely, optic atrophy,
Explain how a hemangioma on the eyelid could cause Anisometropia
Mass in the eyelid compresses the cornea to cause axial myopia - remember myopic surprise from cornea compression for IOLs- astigmatism could also be induced- look for a cylinder axis pointing toward the mass
Strabismus is present in 34% of patients with periorbital infantile capillary hemangioma. Why does this make sense?
As the mass on the eye lid compresses the cornea to create axial myopia in one eye, the unaffected eye becomes preferred because of the more clear image, and so the affected eye will eventual develop a strabismus as keeping it in focus will only make images more blurry
Most frequent visceral hemangioma manifestation
Network of vascular channels with irregular lumina formed by plump proliferating endothelial cells - histological appearance of what condition
Parents will often want their child's problems addressed immediately, but sometimes management does not need to be aggressive. How do you educate a parent of a child with a Capillary Hemangioma in terms of treatment and natural disease course?
Capillary Hemangiomas will completely resolve with treatment 60% of the time in 4 y/o and up to 76% of the time in 7 y/o, with varying percentages up to 12 years old. The treatment with long and short acting steroids is sufficient, and will usually show considerable improvement in as little as 2-4 weeks. Surgical removal is not necessary.
Which part of the treatment for Capillary Hemangiomas is directed deep into the lesion to prevent deposits from being visible under the skin?
Long acting corticosteroid (triamcinolone or methylprednisolone)
Which part of the treatment for Capillary
hemangiomas is given subcutaneously around the periphery of the hemangioma to cause arteriolar constriction and narrowing of pre-capillary sphincters?
Short Acting Corticosteroids - Betamethasone or Dexamethasone
Bening, hamartous, low- blood flow hamartoma, vascular malformation that has relatively large blood-filled spaces. no tissue of the organ in which they are situated, MOST COMMON PRIMARY ORBITAL TUMOR of ADULTS
32 year old female with primary orbital tumor and painless proptosis. Gave birth to her second kid 8 months ago, and has developed a bright red lesion on her lower lid area. What does she have, what is your reasoning?
typically in those aged 40 to 50 but also can be caused by hormonal changes after a pregnancy
Also has 70% preference for females
If a patient tells you that they are anemic due to iron deficiency, and you notice the presence of hemangiomas on their arms or trunk, what would you assume?
Multiple Cavernous hemangiomas creating visceral lesions the small intestine are causing GI bleeding, resulting in Anemia. Occurs very rarely.
50 year old female with painless proptosis and some eyelid swelling is in your office. She comes back with her CT scan you ordered and you see a well demarcated, oval mass within the muscle cone, at the lateral part of the middle third of the orbit.
How do you treat it?
She needs surgical excision, but the good news is, because it is a low-flow cavernous hemangioma, it can be punctured which helps with the excision, and it will not ever come back once the surgery is over with. There would be some risk if it is located at the orbital apex.
Benign tumor lesions of the ON glial tissue inside the orbit and adjacent to proximal optic nerve or intracranial optic nerve. Can produce ONH swelling and atrophy leading to progressive visual loss.
ONH Glioma - Juvenile
Meg Griffin is in your clinic because of her proptosis. When doing VA's you find 20/25-2, OD and no improvement when doing pinhole. Color vision test reveals a Red/Green deficiency OD. When doing the swinging flash test, you find that the pupil dilates instead of constricts when the light is shining in it the second time. examination of the ONH shows normal pale ONH with the presence of optociliary shunt vessels. CT shows tumor on optic nerve. Diagnose, Test, Treat,
1. ONH Glioma, look for bumps all over the body- sign of Neurofibromatosis type 1
2. CT-MRI or MRI
3. Surgical resection, then chemotherapy/radiation after if older than 5.
VA did not improve with pinhole because the issue is not with refraction, Red/green defect indicated ONH issue, presence of RAPD indicates signals not getting to brain through ON, optociliary shunts (collaterals) form when blood needs rerouted around an obstruction
Benign tumors from the intraorbital ON sheath surrounding the ONH, arising from meningothelial cells of meninges - arachnoid cap cells of arachnoid villi, affects women under 40 or children, unilateral, associated with neurofibromatosis type 2, especially if bilateral
ON Sheath Meningiomas
49 y/o Pamela Anderson is in your clinic saying she cant see very well with her left eye. VA shows 20/50 and no improvement with pinhole. pupils show RAPD when doing marcus gunn, CV shows red/green defect, and diplopia during EOM's. Loss of monocular vision when eccentric gaze in certain direction. BIO shows swelling and pallor, optociliary shunt vessels. VF shows central scotoma. CT scan shows bright white enlarged optic nerve within the cone. MRI confirms suspicion. Diagnose and Treat.
2. test for mutation
3. follow up
4. what if follow up shows progression
5. ultimate fix, but risk of...
1. ON Sheath Meningioma
2. Get genetic testing for mutations in the NF2 gene
3. observe monthly because worse VA than 20/40
4. If the VA gets worse, recommend radiation
5. Surgery can be done, but very risky and may result in blindness
Solid MALIGNANT neoplasm that originates from leukocyte- T and B lymphocytes and dendritic histiocytes, commonly occurs in lymph nodes and primary lymphoid tissue like marrow, spleen thymus, or in extranodal sites like the orbit.
type of lymphoma characterized by a systematic spread of disease, systemic symptoms when advanced, multi-nucleated lymphocytes: Reed Sternberg Cells, associated with EBV. That is very rare in the orbit because it does not contain lymph nodes, but there are lymphocytes native to the stroma of the conjunctiva and the lacrimal gland,
Painless lymph node enlargement in one or more nodes, usually affects those in 50's and 60's, no fever, weight loss, night sweats like HL's, Splenomegaly in 20%, normal CBC, B-cell type tend to develop BACTERIAL infection, T- cell type may develop VIRAL infections
Systemic Non-Hodgkins Lymphomas
Third most common cause of proptosis in an adult besides inflammation from Graves or orbital pseudotumor, and cavernous hemangioma. 50% are reactive or atypical hyperplasia and 50% are malignant lyphomas
Lymphoid lesions - orbital lymphomas