Lecture 4 - Orbital Tumors Flashcards
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.
- Capillary Hemangioma
- Enhanced MRI or B scan ultrasonography showing smooth lobular mass, often confused with orbital cellulitis
- Intralesional injections of long and short acting corticosteroids
40 mg triamcinolone acetate and 6 mg betamethasone
or
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
Laryngeal hemangioma
Network of vascular channels with irregular lumina formed by plump proliferating endothelial cells - histological appearance of what condition
Capillary Hemangioma
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
Cavernous Hemangioma
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?
Cavernous hemangioma
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.
