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Vascular Lesions of the Orbit
1
? ?
QTwo
basic lesion types
Vascular Lesions of the Orbit
2
Hemangiomas Malformations
ATwo
basic lesion types
Vascular Lesions of the Orbit
3
Hemangiomas Malformations
? ?
Q
Vascular Lesions of the Orbit
4
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
A
Vascular Lesions of the Orbit
5
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
??
?
?
Q
Vascular Lesions of the Orbit
6
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Vascular Lesions of the Orbit
7
Malformations
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Note: The 2018-19 edition of the BCSC Peds book indicates that the terms strawberry hemangioma and capillary hemangioma are outdated, and such lesions should be referred to simply as ‘hemangiomas.’ However, it also acknowledges that these terms persist in the ophthalmic literature. Because our community continues to use them, they will be included in this slide-set.
Hemangiomas
Strawberryhemangioma
Capillaryhemangioma
Vascular Lesions of the Orbit
8
Malformations
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Note: The 2018-19 edition of the BCSC Peds book indicates that the terms strawberry hemangioma and capillary hemangioma are outdated, and such lesions should be referred to simply as ‘hemangiomas.’ However, it also 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.’
Hemangiomas
Strawberryhemangioma
Capillaryhemangioma
Vascular Lesions of the Orbit
9
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
Lymphangioma
Cavernoushemangioma
Port winestain
Finally: Note that, per both the Peds and Orbit books, the term lymphangioma has been replaced with orbital lymphatic malformation.
Orbital lymphatic malformation AVM
Vascular Lesions of the Orbit
10
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Vascular Lesions of the Orbit
11
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
most v least
Vascular Lesions of the Orbit
12
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Vascular Lesions of the Orbit
13
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What proportion of newborns have an orbital hemangioma?1-3%
Vascular Lesions of the Orbit
14
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What proportion of newborns have an orbital hemangioma?1-3%
Vascular Lesions of the Orbit
15
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What vascular tumor is the next most common?Orbital lymphangioma
second-^ ?
Vascular Lesions of the Orbit
16
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
AVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What vascular tumor is the next most common?Orbital lymphangioma
second-^ ?
Lymphangioma
Vascular Lesions of the Orbit
17
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Vascular Lesions of the Orbit
18
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
M v F
Vascular Lesions of the Orbit
19
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Vascular Lesions of the Orbit
20
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Female gender is the major risk factor. What is another one?Prematurity
Vascular Lesions of the Orbit
21
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Female gender is the major risk factor. What is another one?Prematurity
Vascular Lesions of the Orbit
22
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Vascular Lesions of the Orbit
23
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
%
Vascular Lesions of the Orbit
24
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Vascular Lesions of the Orbit
25
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Of those present at birth, how many are clinically significant from jump street?Almost none
Vascular Lesions of the Orbit
26
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Of those present at birth, how many are clinically significant from jump street?Almost none
Vascular Lesions of the Orbit
27
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
How does capillary hemangioma present?As a bluish or strawberry-like lesion of the periorbital skin
Vascular Lesions of the Orbit
28
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
How does capillary hemangioma present?As a bluish or strawberry-like lesion of the periorbital skincolor color (-like)
Vascular Lesions of the Orbit
29
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
How does capillary hemangioma present?As a bluish or strawberry-like lesion of the periorbital skin
30
Vascular Lesions of the Orbit
Vascular Lesions of the Orbit
31
Hemangiomas Malformations
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
How does capillary hemangioma present?As a bluish or strawberry-like lesion of the periorbital skin
What determines whether it appears bluish vs strawberry-like?It appears bluish if it’s…deepIt appears strawberry-like if it’s…superficial
Strawberryhemangioma
Vascular Lesions of the Orbit
32
Hemangiomas Malformations
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
How does capillary hemangioma present?As a bluish or strawberry-like lesion of the periorbital skin
What determines whether it appears bluish vs strawberry-like?It appears bluish if it’s…[one depth]It appears strawberry-like if it’s…[the other depth]
Strawberryhemangioma
Vascular Lesions of the Orbit
33
Hemangiomas Malformations
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
How does capillary hemangioma present?As a bluish or strawberry-like lesion of the periorbital skin
What determines whether it appears bluish vs strawberry-like?It appears bluish if it’s…deepIt appears strawberry-like if it’s…superficial
Strawberryhemangioma
34
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
Vascular Lesions of the Orbit
Vascular Lesions of the Orbit
35
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Vascular Lesions of the Orbit
36
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Vascular Lesions of the Orbit
37
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Vascular Lesions of the Orbit
38
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Vascular Lesions of the Orbit
39
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Vascular Lesions of the Orbit
40
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Vascular Lesions of the Orbit
41
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Vascular Lesions of the Orbit
42
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Vascular Lesions of the Orbit
43
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
Vascular Lesions of the Orbit
44
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What is the feared visual sequelae of orbital capillary hemangiomas?Amblyopia
By what two mechanisms do hemangiomas induce amblyopia?Deprivation (ie, occluding the visual axis), or astigmatism
Vascular Lesions of the Orbit
45
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What is the feared visual sequelae of orbital capillary hemangiomas?Amblyopia
By what two mechanisms do hemangiomas induce amblyopia?Deprivation (ie, occluding the visual axis), or astigmatism
Vascular Lesions of the Orbit
46
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What is the feared visual sequelae of orbital capillary hemangiomas?Amblyopia
By what two mechanisms do hemangiomas induce amblyopia?Deprivation (ie, occluding the visual axis), or astigmatism
Vascular Lesions of the Orbit
47
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What is the feared visual sequelae of orbital capillary hemangiomas?Amblyopia
By what two mechanisms do hemangiomas induce amblyopia?Deprivation (ie, occluding the visual axis), or astigmatism
Vascular Lesions of the Orbit
48
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What are the options for managing orbital capillary hemangiomas?--Observation is reasonable for small, nonamblyogenic lesions--For lesions requiring treatment, systemic propranolol is probably first-line--Superficial lesions can be treated with topical timolol , or laser--Treatment-resistant lesions may require surgical excision
Vascular Lesions of the Orbit
49
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What are the options for managing orbital capillary hemangiomas?--Observation is reasonable for small, nonamblyogenic lesions--For lesions requiring treatment, systemic propranolol is probably first-line--Superficial lesions can be treated with topical timolol , or laser--Treatment-resistant lesions may require surgical excision
Vascular Lesions of the Orbit
50
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What are the options for managing orbital capillary hemangiomas?--Observation is reasonable for small, nonamblyogenic lesions--For lesions requiring treatment, systemic propranolol is probably first-line--Superficial lesions can be treated with topical timolol , or laser--Treatment-resistant lesions may require surgical excision
Vascular Lesions of the Orbit
51
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What are the options for managing orbital capillary hemangiomas?--Observation is reasonable for small, nonamblyogenic lesions--For lesions requiring treatment, systemic propranolol is probably first-line--Superficial lesions can be treated with topical timolol , or laser--Treatment-resistant lesions may require surgical excision
Vascular Lesions of the Orbit
52
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What are the options for managing orbital capillary hemangiomas?--Observation is reasonable for small, nonamblyogenic lesions--For lesions requiring treatment, systemic propranolol is probably first-line--Superficial lesions can be treated with topical timolol , or laser--Treatment-resistant lesions may require surgical excision
Vascular Lesions of the Orbit
53
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What are the options for managing orbital capillary hemangiomas?--Observation is reasonable for small, nonamblyogenic lesions--For lesions requiring treatment, systemic propranolol is probably first-line--Superficial lesions can be treated with topical timolol , or laser--Treatment-resistant lesions may require surgical excision
Vascular Lesions of the Orbit
54
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What are the options for managing orbital capillary hemangiomas?--Observation is reasonable for small, nonamblyogenic lesions--For lesions requiring treatment, systemic propranolol is probably first-line--Superficial lesions can be treated with topical timolol , or laser--Treatment-resistant lesions may require surgical excision
Vascular Lesions of the Orbit
55
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What are the options for managing orbital capillary hemangiomas?--Observation is reasonable for small, nonamblyogenic lesions--For lesions requiring treatment, systemic propranolol is probably first-line--Superficial lesions can be treated with topical timolol , or laser--Treatment-resistant lesions may require surgical excision
Vascular Lesions of the Orbit
56
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What are the options for managing orbital capillary hemangiomas?--Observation is reasonable for small, nonamblyogenic lesions--For lesions requiring treatment, systemic propranolol is probably first-line--Superficial lesions can be treated with topical timolol , or laser--Treatment-resistant lesions may require surgical excision
Vascular Lesions of the Orbit
57
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Among the orbital vascular lesions, where does the capillary hemangioma rank in terms of prevalence?It is the most common
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
What are the options for managing orbital capillary hemangiomas?--Observation is reasonable for small, nonamblyogenic lesions--For lesions requiring treatment, systemic propranolol is probably first-line--Superficial lesions can be treated with topical timolol , or laser--Treatment-resistant lesions may require surgical excision
Disclaimer: These treatment guidelines were taken from the latest (2018-19) version of the BCSC Peds book. It should be noted that the Orbit book indicates that steroids(systemic or local) are the preferred first-line treatment for capillary hemangiomas. In contrast, the Peds book doesn’t even mention them. Which book is right? I’m going with the Peds version. As always, caveat emptor.
Vascular Lesions of the Orbit
58
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangiomasyndromes Lymphangioma
AVM
Cavernoushemangioma
Port winestain
Q
? ?
With respect to capillary hemangiomas, the word syndrome should evoke two conditions. What are they?
Vascular Lesions of the Orbit
59
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangiomasyndromes Lymphangioma
AVM
Cavernoushemangioma
Port winestain
A
Kasabach-Merritt
PHACE
With respect to capillary hemangiomas, the word syndrome should evoke two conditions. What are they?Kasabach-Merritt, and PHACE
Vascular Lesions of the Orbit
60
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangiomasyndromes Lymphangioma
AVM
Cavernoushemangioma
Port winestain
Q
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
Kasabach-Merritt
PHACE
Vascular Lesions of the Orbit
61
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangiomasyndromes Lymphangioma
AVM
Cavernoushemangioma
Port winestain
Q/A
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
Kasabach-Merritt
PHACE
hematologic cell type, sort of action word hematologic cell type
body system
Vascular Lesions of the Orbit
62
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangiomasyndromes Lymphangioma
AVM
Cavernoushemangioma
Port winestain
A
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
Kasabach-Merritt
PHACE
Vascular Lesions of the Orbit
63
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangiomasyndromes Lymphangioma
AVM
Cavernoushemangioma
Port winestain
Q
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
Kasabach-Merritt
PHACE
Vascular Lesions of the Orbit
64
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangiomasyndromes Lymphangioma
AVM
Cavernoushemangioma
Port winestain
A
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
Kasabach-Merritt
PHACE
Vascular Lesions of the Orbit
65
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangiomasyndromes Lymphangioma
AVM
Cavernoushemangioma
Port winestain
Q
I have a hunch PHACE is an acronym, with the ‘H’ standing for ‘hemangiomas.’ Assuming I’m right, what does the rest stand for?PHemangiomasACE
Kasabach-Merritt
PHACE
Vascular Lesions of the Orbit
66
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangiomasyndromes Lymphangioma
AVM
Cavernoushemangioma
Port winestain
A
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 malformationsHemangiomasArterial lesionsCardiac anomaliesEye anomalies
Kasabach-Merritt
PHACE
Vascular Lesions of the Orbit
67
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangiomasyndromes Lymphangioma
AVM
Cavernoushemangioma
Port winestain
Q
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 malformationsHemangiomasArterial lesionsCardiac anomaliesEye anomalies
Kasabach-Merritt
PHACE
Where is the hemangioma located?The face, +/- scalp involvement
Is it large, or small?Large
What is the classic term for its shape?‘Plaquelike’
Vascular Lesions of the Orbit
68
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangiomasyndromes Lymphangioma
AVM
Cavernoushemangioma
Port winestain
A
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 malformationsHemangiomasArterial lesionsCardiac anomaliesEye anomalies
Kasabach-Merritt
PHACE
Where is the hemangioma located?The face, +/- scalp involvement
Is it large, or small?Large
What is the classic term for its shape?‘Plaquelike’
Vascular Lesions of the Orbit
69
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangiomasyndromes Lymphangioma
AVM
Cavernoushemangioma
Port winestain
Q
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 malformationsHemangiomasArterial lesionsCardiac anomaliesEye anomalies
Kasabach-Merritt
PHACE
Where is the hemangioma located?The face, +/- scalp involvement
Is it large, or small?Large
What is the classic term for its shape?‘Plaquelike’
Vascular Lesions of the Orbit
70
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangiomasyndromes Lymphangioma
AVM
Cavernoushemangioma
Port winestain
A
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 malformationsHemangiomasArterial lesionsCardiac anomaliesEye anomalies
Kasabach-Merritt
PHACE
Where is the hemangioma located?The face, +/- scalp involvement
Is it large, or small?Large
What is the classic term for its shape?‘Plaquelike’
Vascular Lesions of the Orbit
71
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangiomasyndromes Lymphangioma
AVM
Cavernoushemangioma
Port winestain
Q
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 malformationsHemangiomasArterial lesionsCardiac anomaliesEye anomalies
Kasabach-Merritt
PHACE
Where is the hemangioma located?The face, +/- scalp involvement
Is it large, or small?Large
What is the classic term for its shape?‘Plaquelike’
Vascular Lesions of the Orbit
72
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangiomasyndromes Lymphangioma
AVM
Cavernoushemangioma
Port winestain
A
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 malformationsHemangiomasArterial lesionsCardiac anomaliesEye anomalies
Kasabach-Merritt
PHACE
Where is the hemangioma located?The face, +/- scalp involvement
Is it large, or small?Large
What is the classic term for its shape?‘Plaquelike’
73
Vascular Lesions of the Orbit
PHACE syndrome
Vascular Lesions of the Orbit
74
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangiomasyndromes Lymphangioma
AVM
Cavernoushemangioma
Port winestain
Q
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 malformationsHemangiomasArterial lesionsCardiac anomaliesEye anomalies
Kasabach-Merritt
PHACE
What sorts of eye anomalies’ may be involved?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)
Vascular Lesions of the Orbit
75
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangiomasyndromes Lymphangioma
AVM
Cavernoushemangioma
Port winestain
A
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 malformationsHemangiomasArterial lesionsCardiac anomaliesEye anomalies
Kasabach-Merritt
PHACE
What sorts of eye anomalies’ may be involved?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)
Vascular Lesions of the Orbit
76
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
The cavernous hemangioma presents as…slowly progressive proptosis in adults
Is the proptosis slow, or rapid?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
Vascular Lesions of the Orbit
77
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
one word
Vascular Lesions of the Orbit
78
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
Vascular Lesions of the Orbit
79
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
Vascular Lesions of the Orbit
80
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
Vascular Lesions of the Orbit
81
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
What common medical status is associated with more rapid proptosis?Pregnancy
Rapid during…
Vascular Lesions of the Orbit
82
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
What common medical status is associated with more rapid proptosis?Pregnancy
Rapid during…pregnancy
Vascular Lesions of the Orbit
83
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
Vascular Lesions of the Orbit
84
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
M v F
Vascular Lesions of the Orbit
85
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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.
Vascular Lesions of the Orbit
87
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
Vascular Lesions of the Orbit
88
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
Vascular Lesions of the Orbit
89
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
Vascular Lesions of the Orbit
90
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
most v least
Vascular Lesions of the Orbit
91
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
Vascular Lesions of the Orbit
92
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
Vascular Lesions of the Orbit
93
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
Vascular Lesions of the Orbit
94
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
How might a cavernous hemangioma be clinically significant?------
Vascular Lesions of the Orbit
95
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
How might a cavernous hemangioma be clinically significant?--By affecting vision--By increasing IOP--By causing strabismus
Vascular Lesions of the Orbit
96
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
How might a cavernous hemangioma be clinically significant?--By affecting vision--By increasing IOP--By causing strabismus
How might a cavernous hemangioma affect vision?--By causing retinal striae, or hyperopia--By compressing the optic nerve
Vascular Lesions of the Orbit
97
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
How might a cavernous hemangioma be clinically significant?--By affecting vision--By increasing IOP--By causing strabismus
How might a cavernous hemangioma affect vision?--By causing retinal striae, or hyperopia--By compressing the optic nerve
Vascular Lesions of the Orbit
98
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
How might a cavernous hemangioma be clinically significant?--By affecting vision--By increasing IOP--By causing strabismus
How might a cavernous hemangioma affect vision?--By causing retinal striae, or hyperopia--By compressing the optic nerve
How could a cavernous hemangioma cause striae and/or hyperopia?By compressing the back of the globe
Vascular Lesions of the Orbit
99
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
How might a cavernous hemangioma be clinically significant?--By affecting vision--By increasing IOP--By causing strabismus
How might a cavernous hemangioma affect vision?--By causing retinal striae, or hyperopia--By compressing the optic nerve
How could a cavernous hemangioma cause striae and/or hyperopia?By compressing the back of the globe
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.
Vascular Lesions of the Orbit
101
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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
Vascular Lesions of the Orbit
102
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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
Vascular Lesions of the Orbit
103
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestainWhat is the classic clinical presentation of an orbital lymphangioma?
Rapid-onset proptosis
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
Note that lymphangioma is associated with rapid proptosis…
Vascular Lesions of the Orbit
104
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
The cavernous hemangioma presents as…progressive proptosis in adults
Is the proptosis slow, or rapid?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
…whereas the cavernous hemangioma is associated with slow proptosis.
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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
Vascular Lesions of the Orbit
105
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
During what age range does this typically occur?Per the Peds book, the second decadePer the Orbit book, the first decade
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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
Vascular Lesions of the Orbit
106
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
During what age range does this typically occur?Per the Peds book, the second decadePer the Orbit book, the first decade
Vascular Lesions of the Orbit
107
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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
Vascular Lesions of the Orbit
108
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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
Vascular Lesions of the Orbit
109
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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
Are lymphangiomas encapsulated?No, and this is an important point to remember
Why is it important?You’ll see…
Vascular Lesions of the Orbit
110
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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
Are lymphangiomas encapsulated?No, and this is an important point to remember
Why is it important?You’ll see…
Vascular Lesions of the Orbit
111
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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
Are lymphangiomas encapsulated?No, and this is an important point to remember
Why is it important?You’ll see…
Vascular Lesions of the Orbit
112
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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
Are lymphangiomas encapsulated?No, and this is an important point to remember
Why is it important?Un momento, por favor
Vascular Lesions of the Orbit
113
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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
Vascular Lesions of the Orbit
114
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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
Vascular Lesions of the Orbit
115
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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--Endothelial proliferation?
What about endothelial-cell proliferation? Couldn’t that cause lesion enlargement (and thus proptosis)?No, for the simple reason that the endothelial cells of a lymphangioma do not proliferate
Vascular Lesions of the Orbit
116
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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--Endothelial proliferation? No!
What about endothelial-cell proliferation? Couldn’t that cause lesion enlargement (and thus proptosis)?No, for the simple reason that the endothelial cells of a lymphangioma do not proliferate
Vascular Lesions of the Orbit
117
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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--Endothelial proliferation? No!
What about endothelial-cell proliferation? Couldn’t that cause lesion enlargement (and thus proptosis)?No, for the simple reason that the endothelial 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
Vascular Lesions of the Orbit
118
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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--Endothelial proliferation? No!
What about endothelial-cell proliferation? Couldn’t that cause lesion enlargement (and thus proptosis)?No, for the simple reason that the endothelial 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
Vascular Lesions of the Orbit
119
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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 classic health event for precipitating lymphoid hyperplasia in these patients?Upper respiratory tract infection
Vascular Lesions of the Orbit
120
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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 classic health event for precipitating lymphoid hyperplasia in these patients?Upper respiratory tract infection
Vascular Lesions of the Orbit
121
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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
Vascular Lesions of the Orbit
122
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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 cystyum
Vascular Lesions of the Orbit
123
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
What is the classic clinical presentation of an orbital lymphangioma?Rapid-onset proptosis
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
Vascular Lesions of the Orbit
124
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
How are lymphangiomas managed?If threatening vision or producing unacceptable cosmesis--resection.Otherwise, conservatively.
Vascular Lesions of the Orbit
125
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
How are lymphangiomas managed?If threatening vision or producing unacceptable cosmesis--resection.Otherwise, conservatively.
Vascular Lesions of the Orbit
126
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
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
Vascular Lesions of the Orbit
127
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
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
Vascular Lesions of the Orbit
128
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
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
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
Vascular Lesions of the Orbit
129
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
How are lymphangiomas managed?If threatening vision or producing unacceptable cosmesis--resection.Otherwise, conservatively.
(at last we’ll see why this fact is important)
Vascular Lesions of the Orbit
130
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
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
Vascular Lesions of the Orbit
131
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
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
Vascular Lesions of the Orbit
132
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
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
Vascular Lesions of the Orbit
133
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
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
Vascular Lesions of the Orbit
134
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
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
Vascular Lesions of the Orbit
135
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestainBecause isolated AVMs of the orbit are both extremely rare
and only lightly covered in the BCSC books, they do not warrant further attention here
Vascular Lesions of the Orbit
136
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
With what condition is the port wine stain (PWS) closely associated?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
Vascular Lesions of the Orbit
137
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
With what condition is the port wine stain (PWS) closely associated?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
Vascular Lesions of the Orbit
138
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
With what condition is the port wine stain (PWS) closely associated?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
Vascular Lesions of the Orbit
139
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
With what condition is the port wine stain (PWS) closely associated?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
Vascular Lesions of the Orbit
140
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
With what condition is the port wine stain (PWS) closely associated?Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber?A phakomatosis
By what noneponymous name is Sturge-Weber known?Encephalotrigeminal angiomatosisBriefly, what is a phakomatosis?
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
Vascular Lesions of the Orbit
141
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
With what condition is the port wine stain (PWS) closely associated?Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber?A phakomatosis
By what noneponymous name is Sturge-Weber known?Encephalotrigeminal angiomatosisBriefly, what is a phakomatosis?
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
abb.
Vascular Lesions of the Orbit
142
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
With what condition is the port wine stain (PWS) closely associated?Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber?A phakomatosis
By what noneponymous name is Sturge-Weber known?Encephalotrigeminal angiomatosisBriefly, what is a phakomatosis?
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
Vascular Lesions of the Orbit
143
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
With what condition is the port wine stain (PWS) closely associated?Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber?A phakomatosis
By what noneponymous name is Sturge-Weber known?Encephalotrigeminal angiomatosisBriefly, what is a phakomatosis?
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
Vascular Lesions of the Orbit
144
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
With what condition is the port wine stain (PWS) closely associated?Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber?A phakomatosis
By what noneponymous name is Sturge-Weber known?Encephalotrigeminal angiomatosisBriefly, what is a phakomatosis?
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…
Vascular Lesions of the Orbit
145
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
With what condition is the port wine stain (PWS) closely associated?Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber?A phakomatosis
By what noneponymous name is Sturge-Weber known?Encephalotrigeminal angiomatosisBriefly, what is a phakomatosis?
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…
Vascular Lesions of the Orbit
146
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
With what condition is the port wine stain (PWS) closely associated?Sturge-Weber syndrome
In one word, what sort of condition is Sturge-Weber?A phakomatosis
By what noneponymous name is Sturge-Weber known?Encephalotrigeminal angiomatosisBriefly, what is a phakomatosis?
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
Vascular Lesions of the Orbit
147
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
With what condition is the port wine stain (PWS) closely associated?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
Vascular Lesions of the Orbit
148
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
With what condition is the port wine stain (PWS) closely associated?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
Vascular Lesions of the Orbit
149
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
With what condition is the port wine stain (PWS) closely associated?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
In one word, what sort of lesion is the PWS?An angioma
By what ‘official’ name is it known?Nevus flammeus
When does it present?At birth
What is the typical pattern of distribution?It comports to the distribution of one or more divisions of CN5
All infants with SWS have a port-wine stain. Do all infants with a port-wine stain have SWS?No
Vascular Lesions of the Orbit
150
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
With what condition is the port wine stain (PWS) closely associated?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
In one word, what sort of lesion is the PWS?An angioma
By what ‘official’ name is it known?Nevus flammeus
When does it present?At birth
What is the typical pattern of distribution?It comports to the distribution of one or more divisions of CN5
All infants with SWS have a port-wine stain. Do all infants with a port-wine stain have SWS?No
Vascular Lesions of the Orbit
151
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
With what condition is the port wine stain (PWS) closely associated?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
In one word, what sort of lesion is the PWS?An angioma
By what ‘official’ name is it known?Nevus flammeus
When does it present?At birth
What is the typical pattern of distribution?It comports to the distribution of one or more divisions of CN5
All infants with SWS have a port-wine stain. Do all infants with a port-wine stain have SWS?No
Vascular Lesions of the Orbit
152
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
With what condition is the port wine stain (PWS) closely associated?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
In one word, what sort of lesion is the PWS?An angioma
By what ‘official’ name is it known?Nevus flammeus
When does it present?At birth
What is the typical pattern of distribution?It comports to the distribution of one or more divisions of CN5
All infants with SWS have a port-wine stain. Do all infants with a port-wine stain have SWS?No
Vascular Lesions of the Orbit
153
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
With what condition is the port wine stain (PWS) closely associated?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
In one word, what sort of lesion is the PWS?An angioma
By what ‘official’ name is it known?Nevus flammeus
When does it present?At birth
What is the typical pattern of distribution?It comports to the distribution of one or more divisions of CN5
All infants with SWS have a port-wine stain. Do all infants with a port-wine stain have SWS?No
Vascular Lesions of the Orbit
154
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
With what condition is the port wine stain (PWS) closely associated?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
In one word, what sort of lesion is the PWS?An angioma
By what ‘official’ name is it known?Nevus flammeus
When does it present?At birth
What is the typical pattern of distribution?It comports to the distribution of one or more divisions of CN5
All infants with SWS have a port-wine stain. Do all infants with a port-wine stain have SWS?No
155
Sturge-Weber: Port-wine stain
Vascular Lesions of the Orbit
Vascular Lesions of the Orbit
156
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
With what condition is the port wine stain (PWS) closely associated?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
In one word, what sort of lesion is the PWS?An angioma
By what ‘official’ name is it known?Nevus flammeus
When does it present?At birth
What is the typical pattern of distribution?It comports to the distribution of one or more divisions of CN5
All infants with SWS have a port-wine stain. Do all infants with a port-wine stain have SWS?No
Vascular Lesions of the Orbit
157
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
With what condition is the port wine stain (PWS) closely associated?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
In one word, what sort of lesion is the PWS?An angioma
By what ‘official’ name is it known?Nevus flammeus
When does it present?At birth
What is the typical pattern of distribution?It comports to the distribution of one or more divisions of CN5
All infants with SWS have a port-wine stain. Do all infants with a port-wine stain have SWS?No
Vascular Lesions of the Orbit
158
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
With what condition is the port wine stain (PWS) closely associated?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
In one word, what sort of lesion is the PWS?An angioma
By what ‘official’ name is it known?Nevus flammeus
When does it present?At birth
What is the typical pattern of distribution?It comports to the distribution of one or more divisions of CN5
All infants with SWS have a PWS. Do all infants with a PWS have SWS?No
Vascular Lesions of the Orbit
159
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
With what condition is the port wine stain (PWS) closely associated?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
In one word, what sort of lesion is the PWS?An angioma
By what ‘official’ name is it known?Nevus flammeus
When does it present?At birth
What is the typical pattern of distribution?It comports to the distribution of one or more divisions of CN5
All infants with SWS have a PWS. Do all infants with a PWS have SWS?No
Vascular Lesions of the Orbit
160
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
With what condition is the port wine stain (PWS) closely associated?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
other^
Next question…
Vascular Lesions of the Orbit
161
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
With what condition is the port wine stain (PWS) closely associated?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
other^ Klippel-Trénaunay syndrome (KTS)
Next question…
162
Klippel-Trénaunay syndrome
Vascular Lesions of the Orbit
Vascular Lesions of the Orbit
163
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
With what condition is the port wine stain (PWS) closely associated?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
other^ Klippel-Trénaunay syndrome (KTS)
Is KTS a phakomatosis?Yes
Is the PWS in KTS limited to the face, as in Sturge-Weber?No, it is also found on the trunk as well as a limb (usually a leg )
The involved limb has another important finding--what is it?It is hypertrophied
Vascular Lesions of the Orbit
164
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
With what condition is the port wine stain (PWS) closely associated?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
other^ Klippel-Trénaunay syndrome (KTS)
Is KTS a phakomatosis?Yes
Is the PWS in KTS limited to the face, as in Sturge-Weber?No, it is also found on the trunk as well as a limb (usually a leg )
The involved limb has another important finding--what is it?It is hypertrophied
Vascular Lesions of the Orbit
165
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
With what condition is the port wine stain (PWS) closely associated?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
other^ Klippel-Trénaunay syndrome (KTS)
Is KTS a phakomatosis?Yes
Is the PWS in KTS limited to the face, as in Sturge-Weber?No, it is also found on the trunk as well as a limb (usually a leg )
The involved limb has another important finding--what is it?It is hypertrophied
Vascular Lesions of the Orbit
166
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q/A
With what condition is the port wine stain (PWS) closely associated?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
other^ Klippel-Trénaunay syndrome (KTS)
Is KTS a phakomatosis?Yes
Is the PWS in KTS limited to the face, as in Sturge-Weber?No, it is also found on the trunk as well as a limb (usually a leg )
The involved limb has another important finding--what is it?It is hypertrophied
Vascular Lesions of the Orbit
167
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
With what condition is the port wine stain (PWS) closely associated?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
other^ Klippel-Trénaunay syndrome (KTS)
Is KTS a phakomatosis?Yes
Is the PWS in KTS limited to the face, as in Sturge-Weber?No, it is also found on the trunk as well as a limb (usually a leg )
The involved limb has another important finding--what is it?It is hypertrophied
Vascular Lesions of the Orbit
168
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
Q
With what condition is the port wine stain (PWS) closely associated?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
other^ Klippel-Trénaunay syndrome (KTS)
Is KTS a phakomatosis?Yes
Is the PWS in KTS limited to the face, as in Sturge-Weber?No, it is also found on the trunk as well as a limb (usually a leg )
The involved limb has another important finding--what is it?It is hypertrophied
Vascular Lesions of the Orbit
169
Hemangiomas Malformations
Strawberryhemangioma
Capillaryhemangioma
LymphangiomaAVM
Cavernoushemangioma
Port winestain
A
With what condition is the port wine stain (PWS) closely associated?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
other^ Klippel-Trénaunay syndrome (KTS)
Is KTS a phakomatosis?Yes
Is the PWS in KTS limited to the face, as in Sturge-Weber?No, it is also found on the trunk as well as a limb (usually a leg )
The involved limb has another important finding--what is it?It is hypertrophied
170
Klippel-Trénaunay syndrome
Vascular Lesions of the Orbit
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