1
Q Vascular Lesions of the Orbit Two basic lesion types
?? 2
A Vascular Lesions of the Orbit Two basic lesion types
Hemangiomas Malformations 3
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
?? 4
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary hemangioma hemangioma 5
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary ? hemangioma hemangioma ? ? ? 6
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine stain 7
Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Note: The 2018-19 edition of the BCSC Peds book indicates that the terms strawberry hemangioma and capillary hemangioma are outdated, and such Port wine lesions should be referred to simply as ‘hemangiomas.’ However, it also stain acknowledges that these terms persist in the ophthalmic literature. Because our community continues to use them, they will be included in this slide-set. 8
Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Note: The 2018-19 edition of the BCSC Peds book indicates that the terms strawberry hemangioma and capillary hemangioma are outdated, and such Port wine lesions should be referred to simply as ‘hemangiomas.’ However, it also stain acknowledges that these terms persist in the ophthalmic literature. Because our community continues to use them, they will be included in this slide-set. Note also that the so-called cavernous hemangioma is considered (and therefore included with) the ‘malformations.’ 9
Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma OrbitalLymphangioma lymphatic malformation AVM Finally: Note that, per both the Peds and Orbit books, the term Port wine lymphangioma has been replaced with orbital lymphatic malformation. stain 10
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 11
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the mostmost v common least Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 12
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 13
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a genderWhat predilection? proportion of newborns have an orbital hemangioma? Yes, it is more1-3% common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 14
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a genderWhat predilection? proportion of newborns have an orbital hemangioma? Yes, it is more1-3% common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 15
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence? second- AVM It is the most common ^ ? Port wine stain Is there a gender predilection? What vascular tumor is the next most common? Yes, it is more common in girls Orbital lymphangioma At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 16
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence? second- AVM It is the most common ^ ? Port wine stain Is there a gender predilection? What vascular tumor is the next most common? Yes, it is more common in girls Orbital lymphangioma At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 17
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 18
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection?
Yes, it is more common in girlsM v F
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 19
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 20
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbitalFemale cap illarygender hemangiomas is the major ripresent?sk factor. What is another one? About 1/3 are presentPrematurity at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 21
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbitalFemale cap illarygender hemangiomas is the major ripresent?sk factor. What is another one? About 1/3 are presentPrematurity at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 22
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 23
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3% are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 24
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 25
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
Of Whatthose ispresent the origin at birth, of these how lesions?many are clinically significant from jump street? AlmostIt’s not none known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 26
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
Of Whatthose ispresent the origin at birth, of these how lesions?many are clinically significant from jump street? AlmostIt’s not none known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 27
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin Howof these does lesions? capillary hemangioma present? It’s not known for certain,As a bluish but someor strawberry-like experts believe theylesion origi of natethe periorbital as nests of skin placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 28
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin Howof these does lesions? capillary hemangioma present?
It’s not known for certain,As a bluish butcolor someor strawberry-like expertscolor (-like) believe theylesion origi of natethe periorbital as nests of skin placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 29
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin Howof these does lesions? capillary hemangioma present? It’s not known for certain,As a bluish but someor strawberry-like experts believe theylesion origi of natethe periorbital as nests of skin placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 30
Vascular Lesions of the Orbit 31
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin Howof these does lesions? capillary hemangioma present? It’s not known for certain,As a bluish but someor strawberry-like experts believe theylesion origi of natethe periorbital as nests of skin placental cells that ‘metastasized’ to the fetus What determines whether it appears bluish vs strawberry-like? What is the ‘life cycle’ of capillary hemangiomas? It appears bluish if it’s…deep --Initially they grow, reaching maximum size by around age 12 months It appears strawberry-like if it’s…superficial --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 32
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin Howof these does lesions? capillary hemangioma present? It’s not known for certain,As a bluish but someor strawberry-like experts believe theylesion origi of natethe periorbital as nests of skin placental cells that ‘metastasized’ to the fetus What determines whether it appears bluish vs strawberry-like? What is the ‘life cycle’ of capillary hemangiomas? It appears bluish if it’s…[one depth] --Initially they grow, reaching maximum size by around age 12 months It appears strawberry-like if it’s…[the other depth] --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 33
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin Howof these does lesions? capillary hemangioma present? It’s not known for certain,As a bluish but someor strawberry-like experts believe theylesion origi of natethe periorbital as nests of skin placental cells that ‘metastasized’ to the fetus What determines whether it appears bluish vs strawberry-like? What is the ‘life cycle’ of capillary hemangiomas? It appears bluish if it’s…deep --Initially they grow, reaching maximum size by around age 12 months It appears strawberry-like if it’s…superficial --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 34
Vascular Lesions of the Orbit
Capillary hemangioma Stretching the skin of the upper lid clearly shows two distinct portions of the lesion -- an elevated, red area where there is superficial hemangioma and a smooth, bluish-purple area representing a deeper component 35
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 36
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 37
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 38
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 39
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 40
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 41
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 42
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 43
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a gender predilection? Yes, it is more common in girls
At what age do orbital capillary hemangiomas present? About 1/3 are present at birth; almost all have ‘declared’ by age 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 44
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a genderWhat predilection? is the feared visual sequelae of orbital capillary hemangiomas? Yes, it is more commonAmblyopia in girls
At what age do orbitalBy what cap twoillary mechanisms hemangiomas do hemangiomas present? induce amblyopia? About 1/3 are presentDeprivation at birth; (ie, almostoccluding all havethe visual ‘declared’ axis), by or ageastigmatism 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 45
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a genderWhat predilection? is the feared visual sequelae of orbital capillary hemangiomas? Yes, it is more commonAmblyopia in girls
At what age do orbitalBy what cap twoillary mechanisms hemangiomas do hemangiomas present? induce amblyopia? About 1/3 are presentDeprivation at birth; (ie, almostoccluding all havethe visual ‘declared’ axis), by or ageastigmatism 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 46
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a genderWhat predilection? is the feared visual sequelae of orbital capillary hemangiomas? Yes, it is more commonAmblyopia in girls
At what age do orbitalBy what cap twoillary mechanisms hemangiomas do hemangiomas present? induce amblyopia? About 1/3 are presentDeprivation at birth; (ie, almostoccluding all havethe visual ‘declared’ axis), by or ageastigmatism 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 47
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a genderWhat predilection? is the feared visual sequelae of orbital capillary hemangiomas? Yes, it is more commonAmblyopia in girls
At what age do orbitalBy what cap twoillary mechanisms hemangiomas do hemangiomas present? induce amblyopia? About 1/3 are presentDeprivation at birth; (ie, almostoccluding all havethe visual ‘declared’ axis), by or ageastigmatism 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 48
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a genderWhat predilection? are the options for managing orbital capillary hemangiomas? Yes, it is more-- Observationcommon in girlsis reasonable for small, nonamblyogenic lesions --For lesions requiring treatment, systemic propranolol is probably first-line At what age do--Superficial orbital cap lesionsillary hemangiomas can be treated present? with topical timolol , or laser About 1/3 are--Treatment-resistant present at birth; almost lesions all havemay require‘declared’ surgical by age excision 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 49
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a genderWhat predilection? are the options for managing orbital capillary hemangiomas? Yes, it is more-- Observationcommon in girlsis reasonable for small, nonamblyogenic lesions --For lesions requiring treatment, systemic propranolol is probably first-line At what age do--Superficial orbital cap lesionsillary hemangiomas can be treated present? with topical timolol , or laser About 1/3 are--Treatment-resistant present at birth; almost lesions all havemay require‘declared’ surgical by age excision 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 50
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a genderWhat predilection? are the options for managing orbital capillary hemangiomas? Yes, it is more-- Observationcommon in girlsis reasonable for small, nonamblyogenic lesions --For lesions requiring treatment, systemic propranolol is probably first-line At what age do--Superficial orbital cap lesionsillary hemangiomas can be treated present? with topical timolol , or laser About 1/3 are--Treatment-resistant present at birth; almost lesions all havemay require‘declared’ surgical by age excision 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 51
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a genderWhat predilection? are the options for managing orbital capillary hemangiomas? Yes, it is more-- Observationcommon in girlsis reasonable for small, nonamblyogenic lesions --For lesions requiring treatment, systemic propranolol is probably first-line At what age do--Superficial orbital cap lesionsillary hemangiomas can be treated present? with topical timolol , or laser About 1/3 are--Treatment-resistant present at birth; almost lesions all havemay require‘declared’ surgical by age excision 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 52
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a genderWhat predilection? are the options for managing orbital capillary hemangiomas? Yes, it is more-- Observationcommon in girlsis reasonable for small, nonamblyogenic lesions --For lesions requiring treatment, systemic propranolol is probably first-line At what age do--Superficial orbital cap lesionsillary hemangiomas can be treated present? with topical timolol , or laser About 1/3 are--Treatment-resistant present at birth; almost lesions all havemay require‘declared’ surgical by age excision 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 53
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a genderWhat predilection? are the options for managing orbital capillary hemangiomas? Yes, it is more-- Observationcommon in girlsis reasonable for small, nonamblyogenic lesions --For lesions requiring treatment, systemic propranolol is probably first-line At what age do--Superficial orbital cap lesionsillary hemangiomas can be treated present? with topical timolol , or laser About 1/3 are--Treatment-resistant present at birth; almost lesions all havemay require‘declared’ surgical by age excision 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 54
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a genderWhat predilection? are the options for managing orbital capillary hemangiomas? Yes, it is more-- Observationcommon in girlsis reasonable for small, nonamblyogenic lesions --For lesions requiring treatment, systemic propranolol is probably first-line At what age do--Superficial orbital cap lesionsillary hemangiomas can be treated present? with topical timolol , or laser About 1/3 are--Treatment-resistant present at birth; almost lesions all havemay require‘declared’ surgical by age excision 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 55
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a genderWhat predilection? are the options for managing orbital capillary hemangiomas? Yes, it is more-- Observationcommon in girlsis reasonable for small, nonamblyogenic lesions --For lesions requiring treatment, systemic propranolol is probably first-line At what age do--Superficial orbital cap lesionsillary hemangiomas can be treated present? with topical timolol , or laser About 1/3 are--Treatment-resistant present at birth; almost lesions all havemay require‘declared’ surgical by age excision 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 56
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a genderWhat predilection? are the options for managing orbital capillary hemangiomas? Yes, it is more-- Observationcommon in girlsis reasonable for small, nonamblyogenic lesions --For lesions requiring treatment, systemic propranolol is probably first-line At what age do--Superficial orbital cap lesionsillary hemangiomas can be treated present? with topical timolol , or laser About 1/3 are--Treatment-resistant present at birth; almost lesions all havemay require‘declared’ surgical by age excision 6 months
What is the origin of these lesions? It’s not known for certain, but some experts believe they originate as nests of placental cells that ‘metastasized’ to the fetus
What is the ‘life cycle’ of capillary hemangiomas? --Initially they grow, reaching maximum size by around age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 57
Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma Among the orbital vascular lesions, where does the capillary hemangioma rank in termsAVM of prevalence? It is the most common Port wine stain Is there a genderWhat predilection? are the options for managing orbital capillary hemangiomas? Yes, it is more-- Observationcommon in girlsis reasonable for small, nonamblyogenic lesions --For lesions requiring treatment, systemic propranolol is probably first-line At what age do--Superficial orbital cap lesionsillary hemangiomas can be treated present? with topical timolol , or laser About 1/3 are--Treatment-resistant present at birth; almost lesions all havemay require‘declared’ surgical by age excision 6 months
What is the origin of these lesions? It’s not knownDisclaimer for certain,: These but treatment some experts guidelines believe were they taken origi fromnate the as latestnests (2018-1of placental9) version cells that of ‘metastasized’the BCSC to the Peds fetusbook. It should be noted that the Orbit book indicates that steroids (systemic or local) are the preferred first-line treatment for capillary hemangiomas. What is Inthe contrast, ‘life cycle’ the of Peds capillarybook hemangiomas? doesn’t even mention them. Which book is right? I’m going --Initiallywith they the grow, Peds reachingversion. maximum As always, size caveat by around emptor. age 12 months --Then they involute beginning at about age 2 years --75% will resolve by age 4-5 years 58
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma syndromes Lymphangioma AVM ? ? Port wine stain
With respect to capillary hemangiomas, the word syndrome should evoke two conditions. What are they? 59
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma syndromes Lymphangioma AVM Kasabach- PHACE Port wine Merritt stain
With respect to capillary hemangiomas, the word syndrome should evoke two conditions. What are they? Kasabach-Merritt, and PHACE 60
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma syndromes Lymphangioma AVM Kasabach- PHACE Port wine Merritt stain
What is the Kasabach-Merritt syndrome? A thrombocytopenic coagulopathy secondary to sequestration of platelets in an associated large capillary hemangioma located in the gut
Is it serious? Quite; it carries a high mortality rate 61
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma syndromes Lymphangioma AVM Kasabach- PHACE Port wine Merritt stain
What is the Kasabach-Merritt syndrome? A thrombocytopenichematologic cell type, sort of coagulopathy secondary to sequestrationaction word of plateletshematologic cell type in an associated large capillary hemangioma located in the GI tractbody system
Is it serious? Quite; it carries a high mortality rate 62
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma syndromes Lymphangioma AVM Kasabach- PHACE Port wine Merritt stain
What is the Kasabach-Merritt syndrome? A thrombocytopenic coagulopathy secondary to sequestration of platelets in an associated large capillary hemangioma located in the GI tract
Is it serious? Quite; it carries a high mortality rate 63
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma syndromes Lymphangioma AVM Kasabach- PHACE Port wine Merritt stain
What is the Kasabach-Merritt syndrome? A thrombocytopenic coagulopathy secondary to sequestration of platelets in an associated large capillary hemangioma located in the GI tract
Is it serious? Quite; it carries a high mortality rate 64
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma syndromes Lymphangioma AVM Kasabach- PHACE Port wine Merritt stain
What is the Kasabach-Merritt syndrome? A thrombocytopenic coagulopathy secondary to sequestration of platelets in an associated large capillary hemangioma located in the GI tract
Is it serious? Quite; it carries a high mortality rate 65
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma syndromes Lymphangioma AVM Kasabach- PHACE Port wine Merritt stain
I have a hunch PHACE is an acronym, with the ‘H’ standing for ‘hemangiomas.’ Assuming I’m right, what does the rest stand for? P Hemangiomas A C E 66
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma syndromes Lymphangioma AVM Kasabach- PHACE Port wine Merritt stain
I have a hunch PHACE is an acronym, with the ‘H’ standing for ‘hemangiomas.’ Assuming I’m right, what does the rest stand for? Posterior fossa malformations Hemangiomas Arterial lesions Cardiac anomalies Eye anomalies 67
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma syndromes Lymphangioma AVM Kasabach- PHACE Port wine Merritt stain
I have a hunch PHACEWhereis anis theacronym, hemangioma with the located? ‘H’ standing for ‘hemangiomas.’ Assuming I’m right, Thewhat face, does +/- the scalp rest involvementstand for? Posterior fossa malformations Hemangiomas Is it large, or small? Arterial lesions Large Cardiac anomalies What is the classic term for its shape? Eye anomalies ‘Plaquelike’ 68
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma syndromes Lymphangioma AVM Kasabach- PHACE Port wine Merritt stain
I have a hunch PHACEWhereis anis theacronym, hemangioma with the located? ‘H’ standing for ‘hemangiomas.’ Assuming I’m right, Thewhat face, does +/- the scalp rest involvementstand for? Posterior fossa malformations Hemangiomas Is it large, or small? Arterial lesions Large Cardiac anomalies What is the classic term for its shape? Eye anomalies ‘Plaquelike’ 69
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma syndromes Lymphangioma AVM Kasabach- PHACE Port wine Merritt stain
I have a hunch PHACEWhereis anis theacronym, hemangioma with the located? ‘H’ standing for ‘hemangiomas.’ Assuming I’m right, Thewhat face, does +/- the scalp rest involvementstand for? Posterior fossa malformations Hemangiomas Is it large, or small? Arterial lesions Large Cardiac anomalies What is the classic term for its shape? Eye anomalies ‘Plaquelike’ 70
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma syndromes Lymphangioma AVM Kasabach- PHACE Port wine Merritt stain
I have a hunch PHACEWhereis anis theacronym, hemangioma with the located? ‘H’ standing for ‘hemangiomas.’ Assuming I’m right, Thewhat face, does +/- the scalp rest involvementstand for? Posterior fossa malformations Hemangiomas Is it large, or small? Arterial lesions Large Cardiac anomalies What is the classic term for its shape? Eye anomalies ‘Plaquelike’ 71
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma syndromes Lymphangioma AVM Kasabach- PHACE Port wine Merritt stain
I have a hunch PHACEWhereis anis theacronym, hemangioma with the located? ‘H’ standing for ‘hemangiomas.’ Assuming I’m right, Thewhat face, does +/- the scalp rest involvementstand for? Posterior fossa malformations Hemangiomas Is it large, or small? Arterial lesions Large Cardiac anomalies What is the classic term for its shape? Eye anomalies ‘Plaquelike’ 72
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma syndromes Lymphangioma AVM Kasabach- PHACE Port wine Merritt stain
I have a hunch PHACEWhereis anis theacronym, hemangioma with the located? ‘H’ standing for ‘hemangiomas.’ Assuming I’m right, Thewhat face, does +/- the scalp rest involvementstand for? Posterior fossa malformations Hemangiomas Is it large, or small? Arterial lesions Large Cardiac anomalies What is the classic term for its shape? Eye anomalies ‘Plaquelike’ 73
Vascular Lesions of the Orbit
PHACE syndrome 74
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma syndromes Lymphangioma AVM Kasabach- PHACE Port wine Merritt stain
I have a hunch PHACE is an acronym, with the ‘H’ standing for ‘hemangiomas.’ Assuming I’m right, what does the rest stand for? Posterior fossa malformations Hemangiomas Arterial lesions Cardiac anomalies What sorts of eye anomalies’ may be involved? Eye anomalies Pretty much anything--the usual suspects (cataracts; glaucoma) along with stigmata of interrupted development (colobomas; optic nerve hypoplasia; microphthalmia) and one rather obvious co-morbidity (hemangioma, of the choroid) 75
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma syndromes Lymphangioma AVM Kasabach- PHACE Port wine Merritt stain
I have a hunch PHACE is an acronym, with the ‘H’ standing for ‘hemangiomas.’ Assuming I’m right, what does the rest stand for? Posterior fossa malformations Hemangiomas Arterial lesions Cardiac anomalies What sorts of eye anomalies’ may be involved? Eye anomalies Pretty much anything--the usual suspects (cataracts; glaucoma) along with stigmata of interrupted development (colobomas; optic nerve hypoplasia; microphthalmia) and one rather obvious co-morbidity (hemangioma, of the choroid) 76
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…slowly progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? It is the most common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 77
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma
The cavernous hemangioma presents as…progressive proptosisone word in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? It is the most common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 78
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? It is the most common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 79
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? It is the most common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 80
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? It is the most common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 81
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow Rapid during…
Is there a gender predilection? Yes,What it commonis more commonmedical status in women is associated with more rapid proptosis? IsPregnancy it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? It is the most common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 82
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow Rapid during…pregnancy
Is there a gender predilection? Yes,What it commonis more commonmedical status in women is associated with more rapid proptosis? IsPregnancy it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? It is the most common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 83
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? It is the most common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 84
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in womenM v F
Is it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? It is the most common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 85
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? It is the most common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 86
Vascular Lesions of the Orbit
62-year-old female with painless proptosis noticeable over the last year. Right hyperglobus leading to inferior scleral show is evident. 87
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? It is the most common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 88
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? It is the most common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 89
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? It is the most common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 90
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? most v It is the mostleast common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 91
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? It is the most common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 92
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? It is the most common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 93
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? It is the most common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 94
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign How might a cavernous hemangioma be clinically significant? --Where does the cavernous hemangioma rank among benign orbital tumors in adults? --It is the most common benign orbital tumor of adults -- Does it require surgical excision? Yes, if it is clinically significant 95
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign How might a cavernous hemangioma be clinically significant? --ByWhere affecting does visionthe cavernous hemangioma rank among benign orbital tumors in adults? --ByIt is theincreasing most IOP common benign orbital tumor of adults --By causing strabismus Does it require surgical excision? Yes, if it is clinically significant 96
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign How might a cavernous hemangiomaHow might abe cavernous clinically significant?hemangioma affect vision? --ByWhere affecting does the vision cavernous--By hemangioma causing retinal rank striae, among or hyperopia benign orbital tumors in adults? --ByIt is theincreasing most IOP common-- benignBy compressing orbital tumor the opticof adults nerve --By causing strabismus Does it require surgical excision? Yes, if it is clinically significant 97
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign How might a cavernous hemangiomaHow might abe cavernous clinically significant?hemangioma affect vision? --ByWhere affecting does the vision cavernous--By hemangioma causing retinal rank striae, among or hyperopia benign orbital tumors in adults? --ByIt is theincreasing most IOP common--By benign compressing orbital tumor the opticof adults nerve --By causing strabismus Does it require surgical excision? Yes, if it is clinically significant 98
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women How could a cavernous hemangioma cause Is it benign, or malignant? striae and/or hyperopia? Benign By compressing the back of the globe How might a cavernous hemangiomaHow might abe cavernous clinically significant?hemangioma affect vision? --ByWhere affecting does the vision cavernous--By hemangioma causing retinal rank striae, among or benignhyperopia orbital tumors in adults? --ByIt is theincreasing most IOP common--By benign compressing orbital tumor the opticof adults nerve --By causing strabismus Does it require surgical excision? Yes, if it is clinically significant 99
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma The cavernous hemangioma presents as…progressive proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women How could a cavernous hemangioma cause Is it benign, or malignant? striae and/or hyperopia? Benign By compressing the back of the globe How might a cavernous hemangiomaHow might abe cavernous clinically significant?hemangioma affect vision? --ByWhere affecting does the vision cavernous--By hemangioma causing retinal rank striae, among or benignhyperopia orbital tumors in adults? --ByIt is theincreasing most IOP common--By benign compressing orbital tumor the opticof adults nerve --By causing strabismus Does it require surgical excision? Yes, if it is clinically significant 100
Vascular Lesions of the Orbit
Color fundus photograph of the right eye demonstrating choroidal folds in a patient with an intraconal cavernous hemangioma compressing the posterior pole. 101
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinct sorts of events can cause the rapid lesion enlargement that leads to rapid-onset proptosis. What are these events? --Lymphoid hyperplasia --Intralesional hemorrhage 102
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinct sorts of events can cause the rapid lesion enlargement that leads to rapid-onset proptosis. What are these events? --Lymphoid hyperplasia --Intralesional hemorrhage 103
Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Note that lymphangioma is Lymphangioma associated with rapid proptosis… AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinct sorts of events can cause the rapid lesion enlargement that leads to rapid-onset proptosis. What are these events? --Lymphoid hyperplasia --Intralesional hemorrhage 104
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma …whereas the cavernous hemangioma Lymphangioma The cavernous hemangiomais associated presents with as…progressive slow proptosis. proptosis in adults AVM Port wine Is the proptosis slow, or rapid? stain Slow
Is there a gender predilection? Yes, it is more common in women
Is it benign, or malignant? Benign
Where does the cavernous hemangioma rank among benign orbital tumors in adults? It is the most common benign orbital tumor of adults
Does it require surgical excision? Yes, if it is clinically significant 105
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentationDuring what of age an rangeorbital does lymphangioma? this typically occur? Rapid-onset proptosis Per the Peds book, the second decade stain Per the Orbit book, the first decade What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinct sorts of events can cause the rapid lesion enlargement that leads to rapid-onset proptosis. What are these events? --Lymphoid hyperplasia --Intralesional hemorrhage 106
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentationDuring what of age an rangeorbital does lymphangioma? this typically occur? Rapid-onset proptosis Per the Peds book, the second decade stain Per the Orbit book, the first decade What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinct sorts of events can cause the rapid lesion enlargement that leads to rapid-onset proptosis. What are these events? --Lymphoid hyperplasia --Intralesional hemorrhage 107
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinct sorts of events can cause the rapid lesion enlargement that leads to rapid-onset proptosis. What are these events? --Lymphoid hyperplasia --Intralesional hemorrhage 108
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinct sorts of events can cause the rapid lesion enlargement that leads to rapid-onset proptosis. What are these events? --Lymphoid hyperplasia --Intralesional hemorrhage 109
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinctAre sorts lymphangiomas of events can encapsulated? cause the rapid lesion enlargement that leads toNo, rapid-onset and this is proptosis.an important What point are to these remember events? --Lymphoid hyperplasia --IntralesionalWhy hemorrhage is it important? You’ll see… 110
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinctAre sorts lymphangiomas of events can encapsulated? cause the rapid lesion enlargement that leads toNo, rapid-onset and this is proptosis.an important What point are to these remember events? --Lymphoid hyperplasia --IntralesionalWhy hemorrhage is it important? You’ll see… 111
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinctAre sorts lymphangiomas of events can encapsulated? cause the rapid lesion enlargement that leads toNo, rapid-onset and this is proptosis.an important What point are to these remember events? --Lymphoid hyperplasia --IntralesionalWhy hemorrhage is it important? You’ll see… 112
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinctAre sorts lymphangiomas of events can encapsulated? cause the rapid lesion enlargement that leads toNo, rapid-onset and this is proptosis.an important What point are to these remember events? --Lymphoid hyperplasia --IntralesionalWhy hemorrhage is it important? Un momento, por favor 113
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinct sorts of events can cause the rapid lesion enlargement that leads to rapid-onset proptosis. What are these events? --Lymphoid hyperplasia --Intralesional hemorrhage 114
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinct sorts of events can cause the rapid lesion enlargement that leads to rapid-onset proptosis. What are these events? --Lymphoid hyperplasia --Intralesional hemorrhage 115
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinct sorts of events can cause theWhat rapid about lesion endothelial-cell enlargement proliferation? that leads to rapid-onset proptosis. WhatCouldn’t are these that events? cause lesion enlargement (and --Lymphoid hyperplasia thus proptosis)? --Intralesional hemorrhage No, for the simple reason that the endothelial --Endothelial proliferation? cells of a lymphangioma do not proliferate 116
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinct sorts of events can cause theWhat rapid about lesion endothelial-cell enlargement proliferation? that leads to rapid-onset proptosis. WhatCouldn’t are these that events? cause lesion enlargement (and --Lymphoid hyperplasia thus proptosis)? --Intralesional hemorrhage No, for the simple reason that the endothelial --Endothelial proliferation? No! cells of a lymphangioma do not proliferate 117
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinct sorts of events can cause theWhat rapid about lesion endothelial-cell enlargement proliferation? that leads to rapid-onset proptosis. WhatCouldn’t are these that events? cause lesion enlargement (and --Lymphoid hyperplasia thus proptosis)? --Intralesional hemorrhage No, for the simple reason that the endothelial --Endothelial proliferation? No! cells of a lymphangioma do not proliferate
‘The endothelial cells do not proliferate’--what does this fact indicate about the clinical/histologic status of lymphangioma? It indicates that lymphangiomas are not neoplasms 118
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinct sorts of events can cause theWhat rapid about lesion endothelial-cell enlargement proliferation? that leads to rapid-onset proptosis. WhatCouldn’t are these that events? cause lesion enlargement (and --Lymphoid hyperplasia thus proptosis)? --Intralesional hemorrhage No, for the simple reason that the endothelial --Endothelial proliferation? No! cells of a lymphangioma do not proliferate
‘The endothelial cells do not proliferate’--what does this fact indicate about the clinical/histologic status of lymphangioma? It indicates that lymphangiomas are not neoplasms 119
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces What is the classic health event for precipitating lymphoid hyperplasia in these patients? Two distinct sorts of events can cause the rapid lesion enlargement Upper respiratory tract infection that leads to rapid-onset proptosis. What are these events? --Lymphoid hyperplasia --Intralesional hemorrhage 120
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces What is the classic health event for precipitating lymphoid hyperplasia in these patients? Two distinct sorts of events can cause the rapid lesion enlargement Upper respiratory tract infection that leads to rapid-onset proptosis. What are these events? --Lymphoid hyperplasia --Intralesional hemorrhage 121
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinct sorts of events can cause the rapid lesion enlargement that leads to rapid-onset proptosis. What are these events? --Lymphoid hyperplasia --Intralesional hemorrhage What is the name for a lymphangioma in which blood becomes loculated? A chocolate cyst 122
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinct sorts of events can cause the rapid lesion enlargement that leads to rapid-onset proptosis. What are these events? --Lymphoid hyperplasia --Intralesional hemorrhage What is the name for a lymphangioma in which blood becomes loculated? A chocolateyum cyst 123
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine What is the classic clinical presentation of an orbital lymphangioma? Rapid-onset proptosis stain
What does histologic examination of a lymphangioma reveal? Lymphatic-type endothelial cells lining lymph-filled spaces
Two distinct sorts of events can cause the rapid lesion enlargement that leads to rapid-onset proptosis. What are these events? --Lymphoid hyperplasia --Intralesional hemorrhage What is the name for a lymphangioma in which blood becomes loculated? A chocolate cyst 124
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine stain
How are lymphangiomas managed? If threatening vision or producing unacceptable cosmesis--resection. Otherwise, conservatively. 125
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine stain
How are lymphangiomas managed? If threatening vision or producing unacceptable cosmesis--resection. Otherwise, conservatively. 126
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine stain
How are lymphangiomas managed? If threatening vision or producing unacceptable cosmesis--resection. Otherwise, conservatively.
Is complete excision of the lesion the surgical goal? No, that would be an impossible task
Why is excision impossible? Because lymphangiomas are not encapsulated…they tend to be highly infiltrative, and this renders them essentially un-exciseable 127
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine stain
How are lymphangiomas managed? If threatening vision or producing unacceptable cosmesis--resection. Otherwise, conservatively.
Is complete excision of the lesion the surgical goal? No, that would be an impossible task
Why is excision impossible? Because lymphangiomas are not encapsulated…they tend to be highly infiltrative, and this renders them essentially un-exciseable 128
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine stain
How are lymphangiomas managed? If threatening vision or producing unacceptable cosmesis--resection. Otherwise, conservatively.
Is complete excision of the lesion the surgical goal? No, that would be an impossible task
Why is excision impossible? Because lymphangiomas are not encapsulated…they tend to be highly infiltrative, and this renders them essentially un-exciseable 129
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine stain
How are lymphangiomas managed? If threatening vision or producing unacceptable cosmesis--resection. Otherwise, conservatively.
Is complete excision of the lesion the surgical goal? No, that would be an impossible task
Why is excision impossible? Because lymphangiomas are not encapsulated…they tend to be highly infiltrative, and this renders them essentially un-exciseable (at last we’ll see why this fact is important) 130
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine stain
How are lymphangiomas managed? If threatening vision or producing unacceptable cosmesis--resection. Otherwise, conservatively.
Is complete excision of the lesion the surgical goal? No, that would be an impossible task
Why is excision impossible? Because lymphangiomas are not encapsulated…they tend to be highly infiltrative, and this renders them essentially un-exciseable 131
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine stain
How are lymphangiomas managed? If threatening vision or producing unacceptable cosmesis--resection. Otherwise, conservatively.
Is there a less-drastic surgical intervention that can be tried first? Yes, a sclerosing agent can be injected into the lesion
What potentially devastating complication can arise from this injection? Sclerosis of the optic nerve leading to an iatrogenic optic neuropathy 132
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine stain
How are lymphangiomas managed? If threatening vision or producing unacceptable cosmesis--resection. Otherwise, conservatively.
Is there a less-drastic surgical intervention that can be tried first? Yes, a sclerosing agent can be injected into the lesion
What potentially devastating complication can arise from this injection? Sclerosis of the optic nerve leading to an iatrogenic optic neuropathy 133
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine stain
How are lymphangiomas managed? If threatening vision or producing unacceptable cosmesis--resection. Otherwise, conservatively.
Is there a less-drastic surgical intervention that can be tried first? Yes, a sclerosing agent can be injected into the lesion
What potentially devastating complication can arise from this injection? Sclerosis of the optic nerve leading to an iatrogenic optic neuropathy 134
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine stain
How are lymphangiomas managed? If threatening vision or producing unacceptable cosmesis--resection. Otherwise, conservatively.
Is there a less-drastic surgical intervention that can be tried first? Yes, a sclerosing agent can be injected into the lesion
What potentially devastating complication can arise from this injection? Sclerosis of the optic nerve leading to an iatrogenic optic neuropathy 135
Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine Because isolated AVMs of the orbit are both extremely rare stain and only lightly covered in the BCSC books, they do not warrant further attention here 136
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber? A phakomatosis
By what noneponymous name is Sturge-Weber known? Encephalotrigeminal angiomatosis 137
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber? A phakomatosis
By what noneponymous name is Sturge-Weber known? Encephalotrigeminal angiomatosis 138
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber? A phakomatosis
By what noneponymous name is Sturge-Weber known? Encephalotrigeminal angiomatosis 139
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber? A phakomatosis
By what noneponymous name is Sturge-Weber known? Encephalotrigeminal angiomatosis 140
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber? A phakomatosis
By what noneponymous name is Sturge-Weber known? Briefly, what is a phakomatosis?Encephalotrigeminal angiomatosis A congenital condition involving hamartomatous lesions of multiple organ systems, usually including the CNS , eyes and skin
By what more-descriptive name does the BCSC Peds book refer to them? As neuro-oculocutaneous syndromes 141
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber? A phakomatosis
By what noneponymous name is Sturge-Weber known? Briefly, what is a phakomatosis?Encephalotrigeminal angiomatosis A congenital condition involving hamartomatous lesions of multiple organ systems, usually including the CNSabb. , eyes and skin
By what more-descriptive name does the BCSC Peds book refer to them? As neuro-oculocutaneous syndromes 142
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber? A phakomatosis
By what noneponymous name is Sturge-Weber known? Briefly, what is a phakomatosis?Encephalotrigeminal angiomatosis A congenital condition involving hamartomatous lesions of multiple organ systems, usually including the CNS , eyes and skin
By what more-descriptive name does the BCSC Peds book refer to them? As neuro-oculocutaneous syndromes 143
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber? A phakomatosis
By what noneponymous name is Sturge-Weber known? Briefly, what is a phakomatosis?Encephalotrigeminal angiomatosis A congenital condition involving hamartomatous lesions of multiple organ systems, usually including the CNS , eyes and skin
By what more-descriptive name does the BCSC Peds book refer to them? As neuro-oculocutaneous syndromes 144
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber? A phakomatosis
By what noneponymous name is Sturge-Weber known? Briefly, what is a phakomatosis?Encephalotrigeminal angiomatosis A congenital condition involving hamartomatous lesions of multiple organ systems, usually including the CNS , eyes and skin
By what more-descriptive name does the BCSC Peds book refer to them? As neuro… 145
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber? A phakomatosis
By what noneponymous name is Sturge-Weber known? Briefly, what is a phakomatosis?Encephalotrigeminal angiomatosis A congenital condition involving hamartomatous lesions of multiple organ systems, usually including the CNS , eyes and skin
By what more-descriptive name does the BCSC Peds book refer to them? As neuro-oculo… 146
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber? A phakomatosis
By what noneponymous name is Sturge-Weber known? Briefly, what is a phakomatosis?Encephalotrigeminal angiomatosis A congenital condition involving hamartomatous lesions of multiple organ systems, usually including the CNS , eyes and skin
By what more-descriptive name does the BCSC Peds book refer to them? As neuro-oculocutaneous syndromes 147
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber? A phakomatosis
By what noneponymous name is Sturge-Weber known? Encephalotrigeminal angiomatosis 148
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber? A phakomatosis
By what noneponymous name is Sturge-Weber known? Encephalotrigeminal angiomatosis 149
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma In one word, what sort of lesion is the PWS?Lymphangioma An angioma AVM By what ‘official’ name is it known? Port wine With whatNevus condition flammeus is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome When does it present? In oneAt word, birth what sort of condition is Sturge-Weber? A phakomatosis What is the typical pattern of distribution? By whatIt comports noneponymous to the distribution name is Sturge-Weber of one or more known? divisions of CN5 Encephalotrigeminal angiomatosis All infants with SWS have a port-wine stain. Do all infants with a port-wine stain have SWS? No 150
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma In one word, what sort of lesion is the PWS?Lymphangioma An angioma AVM By what ‘official’ name is it known? Port wine With whatNevus condition flammeus is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome When does it present? In oneAt word, birth what sort of condition is Sturge-Weber? A phakomatosis What is the typical pattern of distribution? By whatIt comports noneponymous to the distribution name is Sturge-Weber of one or more known? divisions of CN5 Encephalotrigeminal angiomatosis All infants with SWS have a port-wine stain. Do all infants with a port-wine stain have SWS? No 151
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma In one word, what sort of lesion is the PWS?Lymphangioma An angioma AVM By what ‘official’ name is it known? Port wine With whatNevus condition flammeus is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome When does it present? In oneAt word, birth what sort of condition is Sturge-Weber? A phakomatosis What is the typical pattern of distribution? By whatIt comports noneponymous to the distribution name is Sturge-Weber of one or more known? divisions of CN5 Encephalotrigeminal angiomatosis All infants with SWS have a port-wine stain. Do all infants with a port-wine stain have SWS? No 152
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma In one word, what sort of lesion is the PWS?Lymphangioma An angioma AVM By what ‘official’ name is it known? Port wine With whatNevus condition flammeus is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome When does it present? In oneAt word, birth what sort of condition is Sturge-Weber? A phakomatosis What is the typical pattern of distribution? By whatIt comports noneponymous to the distribution name is Sturge-Weber of one or more known? divisions of CN5 Encephalotrigeminal angiomatosis All infants with SWS have a port-wine stain. Do all infants with a port-wine stain have SWS? No 153
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma In one word, what sort of lesion is the PWS?Lymphangioma An angioma AVM By what ‘official’ name is it known? Port wine With whatNevus condition flammeus is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome When does it present? In oneAt word, birth what sort of condition is Sturge-Weber? A phakomatosis What is the typical pattern of distribution? By whatIt comports noneponymous to the distribution name is Sturge-Weber of one or more known? divisions of CN5 Encephalotrigeminal angiomatosis All infants with SWS have a port-wine stain. Do all infants with a port-wine stain have SWS? No 154
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma In one word, what sort of lesion is the PWS?Lymphangioma An angioma AVM By what ‘official’ name is it known? Port wine With whatNevus condition flammeus is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome When does it present? In oneAt word, birth what sort of condition is Sturge-Weber? A phakomatosis What is the typical pattern of distribution? By whatIt comports noneponymous to the distribution name is Sturge-Weber of one or more known? divisions of CN5 Encephalotrigeminal angiomatosis All infants with SWS have a port-wine stain. Do all infants with a port-wine stain have SWS? No 155
Vascular Lesions of the Orbit
Sturge-Weber: Port-wine stain 156
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma In one word, what sort of lesion is the PWS?Lymphangioma An angioma AVM By what ‘official’ name is it known? Port wine With whatNevus condition flammeus is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome When does it present? In oneAt word, birth what sort of condition is Sturge-Weber? A phakomatosis What is the typical pattern of distribution? By whatIt comports noneponymous to the distribution name is Sturge-Weber of one or more known? divisions of CN5 Encephalotrigeminal angiomatosis All infants with SWS have a port-wine stain. Do all infants with a port-wine stain have SWS? No 157
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma In one word, what sort of lesion is the PWS?Lymphangioma An angioma AVM By what ‘official’ name is it known? Port wine With whatNevus condition flammeus is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome When does it present? In oneAt word, birth what sort of condition is Sturge-Weber? A phakomatosis What is the typical pattern of distribution? By whatIt comports noneponymous to the distribution name is Sturge-Weber of one or more known? divisions of CN5 Encephalotrigeminal angiomatosis All infants with SWS have a port-wine stain. Do all infants with a port-wine stain have SWS? No 158
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma In one word, what sort of lesion is the PWS?Lymphangioma An angioma AVM By what ‘official’ name is it known? Port wine With whatNevus condition flammeus is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome When does it present? In oneAt word, birth what sort of condition is Sturge-Weber? A phakomatosis What is the typical pattern of distribution? By whatIt comports noneponymous to the distribution name is Sturge-Weber of one or more known? divisions of CN5 Encephalotrigeminal angiomatosis All infants with SWS have a PWS. Do all infants with a PWS have SWS? No 159
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma In one word, what sort of lesion is the PWS?Lymphangioma An angioma AVM By what ‘official’ name is it known? Port wine With whatNevus condition flammeus is the port wine stain (PWS) closely associated? stain Sturge-Weber syndrome When does it present? In oneAt word, birth what sort of condition is Sturge-Weber? A phakomatosis What is the typical pattern of distribution? By whatIt comports noneponymous to the distribution name is Sturge-Weber of one or more known? divisions of CN5 Encephalotrigeminal angiomatosis All infants with SWS have a PWS. Do all infants with a PWS have SWS? No 160
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine other Next question… With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber^ syndrome
In one word, what sort of condition is Sturge-Weber? A phakomatosis
By what noneponymous name is Sturge-Weber known? Encephalotrigeminal angiomatosis 161
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine other Next question… With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber^ syndrome Klippel-Trénaunay syndrome (KTS)
In one word, what sort of condition is Sturge-Weber? A phakomatosis
By what noneponymous name is Sturge-Weber known? Encephalotrigeminal angiomatosis 162
Vascular Lesions of the Orbit
Klippel-Trénaunay syndrome 163
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine other With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber^ syndrome Klippel-Trénaunay syndrome (KTS)
InIs one KTS word, a phakomatosis? what sort of condition is Sturge-Weber? AYes phakomatosis
ByIs whatthe PWS noneponymous in KTS limited name to the is Sturge-Weberface, as in Sturge-Weber? known? EncephalotrigeminalNo, it is also found on angiomatosis the trunk as well as a limb (usually a leg )
The involved limb has another important finding--what is it? It is hypertrophied 164
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine other With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber^ syndrome Klippel-Trénaunay syndrome (KTS)
InIs one KTS word, a phakomatosis? what sort of condition is Sturge-Weber? AYes phakomatosis
ByIs whatthe PWS noneponymous in KTS limited name to the is Sturge-Weberface, as in Sturge-Weber? known? EncephalotrigeminalNo, it is also found on angiomatosis the trunk as well as a limb (usually a leg )
The involved limb has another important finding--what is it? It is hypertrophied 165
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine other With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber^ syndrome Klippel-Trénaunay syndrome (KTS)
InIs one KTS word, a phakomatosis? what sort of condition is Sturge-Weber? AYes phakomatosis
ByIs whatthe PWS noneponymous in KTS limited name to the is Sturge-Weberface, as in Sturge-Weber? known? EncephalotrigeminalNo, it is also found on angiomatosis the trunk as well as a limb (usually a leg )
The involved limb has another important finding--what is it? It is hypertrophied 166
Q/A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine other With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber^ syndrome Klippel-Trénaunay syndrome (KTS)
InIs one KTS word, a phakomatosis? what sort of condition is Sturge-Weber? AYes phakomatosis
ByIs whatthe PWS noneponymous in KTS limited name to the is Sturge-Weberface, as in Sturge-Weber? known? EncephalotrigeminalNo, it is also found on angiomatosis the trunk as well as a limb (usually a leg )
The involved limb has another important finding--what is it? It is hypertrophied 167
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine other With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber^ syndrome Klippel-Trénaunay syndrome (KTS)
InIs one KTS word, a phakomatosis? what sort of condition is Sturge-Weber? AYes phakomatosis
ByIs whatthe PWS noneponymous in KTS limited name to the is Sturge-Weberface, as in Sturge-Weber? known? EncephalotrigeminalNo, it is also found on angiomatosis the trunk as well as a limb (usually a leg )
The involved limb has another important finding--what is it? It is hypertrophied 168
Q Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine other With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber^ syndrome Klippel-Trénaunay syndrome (KTS)
InIs one KTS word, a phakomatosis? what sort of condition is Sturge-Weber? AYes phakomatosis
ByIs whatthe PWS noneponymous in KTS limited name to the is Sturge-Weberface, as in Sturge-Weber? known? EncephalotrigeminalNo, it is also found on angiomatosis the trunk as well as a limb (usually a leg )
The involved limb has another important finding--what is it? It is hypertrophied 169
A Vascular Lesions of the Orbit
Hemangiomas Malformations
Strawberry Capillary Cavernous hemangioma hemangioma hemangioma Lymphangioma AVM Port wine other With what condition is the port wine stain (PWS) closely associated? stain Sturge-Weber^ syndrome Klippel-Trénaunay syndrome (KTS)
InIs one KTS word, a phakomatosis? what sort of condition is Sturge-Weber? AYes phakomatosis
ByIs whatthe PWS noneponymous in KTS limited name to the is Sturge-Weberface, as in Sturge-Weber? known? EncephalotrigeminalNo, it is also found on angiomatosis the trunk as well as a limb (usually a leg )
The involved limb has another important finding--what is it? It is hypertrophied 170
Vascular Lesions of the Orbit
Klippel-Trénaunay syndrome