1. Vascular anomalies
Reviewed and present by
Mr. Patinya Yutchawit
Miss Kaewalin Thongsawangjang
Miss Withunda Akaapimand
Miss Rattanaporn Sirirattanakul
Miss Tritraporn Sawantranon
Mr. Yotdanai Namuangchan
Mr. Jirarot Wongwijitsook
11. Physical examination
General appearance : a boy , alert
HEENT : pink conjunctiva , anicteric sclera
Heart : normal S1S1 , no murmur
Lung : normal breath sound , no adventitious sound
Abdomen : soft , no tenderness , normoactive bowel
sound , impalpable liver and spleen , no abnormal
mass
12. Physical examination
Hand : Soft, Compressible, Purplish mass
of left upper hand area and left 3rd
finger, size ~ 7cm, smooth surface, non-
mobile, no tenderness, no bruit & thrill
26. Vascular malformations
Abnormal development of vascular elements during
embryogenesis and fetal maturation
Single-vessel forms (capillary, arterial, lymphatic, or
venous) or combined.
No evidence of cellular proliferation
28. VascularMalformations
Boys and girls are affected equally.
All malformations are present at birth.
The physical appearance of vascular
malformations is dependent on the type of
vessels involved.
29. Malformations grow commensurately with the child and do
not undergo the rapid proliferative growth phase exhibited
by hemangiomas.
The greatest distinction between hemangiomas and
malformations is that the former spontaneously involute and
the latter do not.
Vascular Malformations
31. Pathophysiology
• usually manifest by childhood or early adulthood.
• grow commensurately with the developing child.
sometimes are not obvious at birth
• do not regress.
• "slow-flow" lesions
• can expand in response to
– trauma,
– incomplete surgical resection,[9]
– altered hormonal states (pregnancy, puberty, steroid
use).
– thrombosis or in sepsis.
32. Thin walled, dilated, sponge-like
channels of variable size and mural
thickness
normal-appearing endothelial lining but
abnormal smooth muscle architecture
33. Presentation
• present in various ways : from a vague blue patch
to a soft blue mass.
• easily compressible
• usually swell in the dependent position or when
venous pressure increases (ie, when a child cries).
• typically involve the skin of the face, limbs, or trunk
but also are found in the internal viscera and bones.
• low-flow lesions. : Episodic thromboses commonly
occur
34. • Pain : secondary to thrombosis of the
malformation
35. Multifocal forms can be inherited
Turner syndrome (VMs of the intestine and
feet)
Cerebral cavernous VMs :small subgroup,
hyperkeratotic capillary-venous lesions
(disorder KRIT1)
Familial multiple glomangioma
Familial multiple glomangioma
41. The overlying skin is usually normal and has a bluish hue
Lymphangioma circumscriptum : superficial cutaneous-
subcutaneous lymphatic anomaly > vesicle
44. Management
Resection : main treatment ,operative goal is complete resection
with preserve vital structure
Sclerotherapy (ethanol,OK-432,bleomycin)permanent
disappearance of vss.fibrosis
Laser (Nd:YAG)
Treatment of sequelae : bleeding and recurrent infection,
correction of contour, and improvement in function
56. Errors of embryogenic vascular development
Failure of arteriovenous channels in the primitive retiform
plexus to regress
Arteriovenous Malformation
57. • AVM is a high-flow vascular malformation
comprised of micro- and macro-aneriovenous
fistulas (AVFs).
•Arterial and venous vessels connected directly
to one another, without an intervening capillary
bed
•The epicenter of an AVM is called the nidus and
consists of arterial feeders
Arteriovenous Malformation
58.
59. EPIDEMIOLOGY
• Incidence 1.34 : 100,000
• Intracranial : Extracranial – 20 : 1
Head and neck
extremity, truncal, and visceral sites
• The majority of patients (40- 60%)present at birth
equal gender distribution
60. CLINICAL
The pink-bluish stain of the AVM is usually noted at birth
Tuberty or trauma seem to trigger expansion
Local warmth , Palpable thrill , Audible bruit
Headache , seizure in Intracranial AVM
61. The cutaneous stain > erythematous and develops
local warmth, a palpable thrill, and a bruit
64. Investigation
• Color Doppler evaluation and ultrasonography
are useful first-line tools to determine flow
characteristics.
• MRI
defines the anatomy and extent of the lesion
• Angiography
is useful in further characterizing the lesion and
allows therapeutic embolization
66. • AVMs are usually treated when there are endangering
signs and symptoms) such as ischemic pain, recalcitrant
ulceration) bleeding) and increased cardiac output
• Small localized AVMs may be primarily resected and reconstructed
• Larger diffuse AVMs will require primary arterial embolization or
superselective arterial embolization for temporary occlusion of the
nidus
followed by resection 24 to 48 hours alter embolization
Management
72. Vascular tumors
Endothelial neoplasms
Increased endothelial turnover
• Hemangioma is the most common among
vascular tumors – originates from
vasculogenesis (Angiogenesis)
73. Epidemiology
2 weeks after birth
white infants (unusual
in dark-skinned)
Girls > boys
(2:1-5:1)
Premature infants,
Low birth weight
14% in BW 1000-1500 g
10% in BW 1500-2000 g
76. Characteristics
1. Thickening : Macule to Papule
2. Border : Well demarcation
3. Compressible : Moderate to poor
4. Color : Pink to Red if superficial, Blue if deep, Blue with a
superficial overlay of endothelial tissue
5. Non tender
6. Not warm
7. No vessel sign : no bruit, no thrill
8. Solid organ involvement : liver, muscle, bone
92. Interferon alpha
2nd line drug for endangering hemangiomas ,can not use
corticosteroid,
Antiangiogenic
2 to 3 million units/m2, injected subcutaneously every
day
side effects are more serious : several reversible
toxicities, elevation of hepatic transaminases, transient
neutropenia, and anemia, Spastic diplegia (most serious)
94. Others treatment
Embolization
severe congestive heart failure + do not respond well to
drug
Hepatic hemangiomas
Laser therapy
Pulsed dye laser
Nd:YAG : rapid shrinkage but risk of thermal damage
and ulceration
Beta-blocker (propanolol)
95. Operative Management
INFANCY (PROLIFERATIVE PHASE)
Obstruction ( visual or subglottic )
Deformation (periorbital distortion with secondary
astigmatic amblyopia)
Bleeding or ulceration (well-localized or pedunculated
lesions)
unresponsive to topical or systemic therapy
constant pain
scalp and thoracic skin
96.
97. Operative Management
EARLY CHILDHOOD (INVOLUTING PHASE)
Preschool period
Indicated if it is obvious
post ulceration scarring, expanded and in
elastic skin, and fibrofatty residuum
98.
99. Operative Management
LATE CHILDHOOD (INVOLUTED PHASE)
Best to postpone until the involuted phase
Atrophic and telangiectasia
Hypopigmented or yellow-tan scar
Irrevocably expanded skin or fibrofatty tissue
103. "It does not
matter how
slowly you
go as long as
you do not
stop.“
Confucius
104. We’ll briefly
remind you
about…
Key deference between hemangioma and vascular
malformation
Hemangioma ;Tumor characteristic , Investigation
and proper treatment
Vascular malformation ; Categorization—
Arterial/venous/capillary/lymphatic, each
characteristic ,investigation and treatment
How to approach the patient with vascular
anomalies?
108. Tumor
Characteristic
Thickness : Macule to papule
Demarcation :Well demarcation
Compressibility : moderate to poor
No warmth, no tenderness
No vascular signs : No bruit, no thrill ***
Color :