Vascular anomaly topic for medical student

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

3. Content
Case conference : Example
Interactive topics
- Vascular malformation
- Hemangioma
Wrap-up

4. Case conference
Mr. Yotdanai Namuangchan
Mr. Patinya Yutchawit

5. Patient identification
ผู้ป่วยเด็กชาย อายุ 2 ปี
ภูมิลาเนา อ.บ้านฝาง จ.ขอนแก่น

6. Chief complaint
มีก้อนที่มือซ้าย ตั้งแต่แรกเกิด

7. Present illness
แรกเกิด พบก้อนที่มือซ้าย บริเวณนิ้วกลาง ก้อนนิ่ม
รูปร่างเป็นปล้อง ขนาดประมาณ 1cm ไม่เจ็บ ไม่มี
แผล ผิวเรียบ พอขยับนิ้วได้ก้อนโตขึ้นเรื่อยๆ
ปัจจุบัน (อายุ 2 ปี) ก้อนโตขึ้นตามตัว ขนาดประมาณ
7cm สีม่วงแดง ขยับนิ้วลาบาก ลักษณะก้อนเป็น
ปล้องๆตามนิ้วกลางและโคนนิ้วมือซ้าย

8. Past history
ไม่มีโรคประจาตัว
ไม่มีประวัติแพ้ยา แพ้อาหาร
G1P1, Normal labor, Term

9. Social and personal history
ไม่มีประวัติใช้ยาใดๆเป็นประจา
ฉีดวัคซีนครบตามเกณฑ์

10. Family history
ไม่มีโรคในครอบครัว

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

17. Differential Diagnosis

18. Investigation

23. Treatment

24. Mini interactive
lecture
- Vascular malformation
- Hemangioma

25. Vascular
malformation
Miss Withunda Akaapimand
Miss Rattanaporn Sirirattanakul
Miss Tritraporn Sawantranon
Mr. Jirarot Wongwijitsook

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

27. Vascular malformations
Classification
Capillary
Venous
Lymphatic
Artery
Combined
Slow flow
Fast flow

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

30. Venous
malformation

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

36. Management
Indication : appearance, functional,pain
Conservative therapy: elastic compression
+baby aspirin (prophylaxis painful
thromboses)
Sclerotherapy : main treatment
Laser (Nd:YAG)
Resection

37. Lymphatic
malformation

38. Lymphatic Malformations
Embryogenic disturbance of lymphatic system
At birth -2 yrs
No involution
Microcystic malformation (lymphagiomas)
Macrocystic (cystic hygroma)
Combined

40. Rich lymphatic area :
H&N
Axilla
Mediastinum
groin retroperitoneum

41. The overlying skin is usually normal and has a bluish hue
Lymphangioma circumscriptum : superficial cutaneous-
subcutaneous lymphatic anomaly > vesicle

42. Facial soft tissue distortion
Bony hypertrophy
Macroglossia
Mandible overrowth
Proptosis

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

45. Capillary
malformation

46. Capillary Malformations(CMs)
Port-wine stain
Equal gender distribution
Birth prevalence is 0.3%

47. Clinical presentation
At birth
Well-circumscribed red macular lesions
Commonly occur on the face in the trigerminal
distribution

48. • 45% restricted to 1/3
trigeminal dermatomes
• 55% overlap
dermatomes, cross the
midline, bilaterally

50. CM in a limb often
is associated with
axial and
transverse
hypertrophy.

51. Sturge-Weber syndrome
Facial capillary malformaion
Ipsilateral occular and
leptomeningeal vascular anomalies
Neurologic : seizures , hemiparesis ,
delayed motor and cognitive
development
Ophthalmologic : glaucoma

53. MANAGEMENT
flashlamp pulsed-dye laser
: the results are better if initiated in infancy and childhood.
Laser treatment

54. Arteriovenous
malformation

55. Arteriovenous Malformation

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

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

63. Schobinger's staging system
Clinacal staging system for documenting the presentation and evolution of an AVM

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

65. AVM Angiography

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

70. Hemangioma
Mr. Patinya Yutchawit
Miss Kaewalin Thongsawangjang

71. VASCULAR TUMORS

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

75. Characteristics
1. Thickening :
2. Border :
3. Compressible :
4. Color : Superficial 43 (Superficial dermis)
Deep 16 (Deep dermis and subcutaneous tissue)
Mixed 41
5. Tenderness :
6. Warmth :
7. Vessel signs :
8. Extracutaneous manifestation (Internal visceral involvement:

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

77. Superficial Deep

78. Craniofacial (60%)
Trunk (25%)
Ext. (15%)
Single(80%)
Multiple(20%)
** visceral organ
involvement
Disseminated hemangiomatosis

79. Proliferating phase
(O-1yr)
Involuting phase
(1-5yr)
Involuted phase
(>5yr)
telltale signs crepe-like laxity
(anetoderma)

80.  Proliferative phase (1st Wk-9 month ;
80 % accomplished by the end of 4th
month)
 Involute 10 % /yr
 At 5 yr : 50 % completed involute
(stigmata remains)

81. Congenital hemangiomas : develop during
prenatal life  fully developed at birth
RICH
(Rapid involuting CH)
NICH
(Non Involuting CH)

82. Complications
Ulceration ,Bleeding,Infection
Dressing
Topical ATB
Visual – Ambryopia
Airway obstruction - subglottic hemangioma
Congestive heart failure

84. RADIOLOGIC FEATURES
Ultrasonography
Hallmarks : localized, dense parenchyma and fast flow

85. MRl

86. Nuclear Scanning
Tc 99m- tagged red blood cells
Deep multiple hemangiomas in the GI or CNS

87. Management
Observe
When to intervene?

88. Management
Indication for therapeutic intervention
Compromised airway
Deviation of visual axis
Oral / digestive tract obstruction
Bilateral auditory obstruction

89. Management
Decision for management
Lesion size and location
Complication
Age
Phase of growth

90. Management
Pharmacotherapy
Chemotherapy
Laser therapy
Surgical excision

91. Pharmacologic treatment
Corticosteroid
 1st line treatment of problematic hemangiomas
 Intralesion : small, well-localized cutaneous
hemangioma (face ,nasal ,lip,periorbital region)
 Retinal artery embolization ,thrombosis
 Systemic : problematic, endangering, or life-threatening
hemangiomas

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)

93. Chemotherapy
vincristine and cyclophosphamide
effective for life-threatening vascular tumors, including
hemangioma.
no role for cutaneous hemangiomas
intrahepatic hemangiomas is debatable

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

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

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

101. Reference
Grabb and Smith's Plastic Surgery Grabb's Plastic Surgery
BMJ Best practice
Medscape

102. Wrap-up
Vascular anomalies
Present by Mr.PatinyaYutchawit

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?

105. Key difference

106. Key difference
Hemangioma Vascular malformation
Definition True vascular tumor Vascular disorganization
Age At 1st month At birth (Congenital)
Growth pattern

107. Hemangioma
Tumor characteristic , Investigation
and proper treatment

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 :

109. Investigation
 MRI ****** Gold standard
 Doppler U/S
 Arteriogram (Not use -- ?)
 ********** Clinical Dx *************

110. Proper
Treatment
 Observe and parent education
 Steroid injection
 Surgery

111. Vascular
malformation
Categorization—Arterial/venous/capillary/lymphatic,
each characteristic ,investigation and treatment

112. Categorization

113. Arterial/AVM Venous Capillary Lymphatic
Characteristic
Color Vary Blue Pink Colorless
Demarcation Well (mass) Moderate Well Poor
Pain / X X X
Compressibility X / X +/-
Keywords Thrill/Bruit
Bleeding
Gravity
dependent
Port-wine
Salmon egg
(face)
Cystic hygroma
Peau d'orange
Infection if
combined with
vein
Investigation Arteriogram Doppler U/S MRI U/S
Treatment Embolization Sclerosing
agents
Argon laser Sclerosing agents
(Bleomycin)

115. Special thanks!
Dr.Poj
Our beloved resident 