2. Content
• Introduction
• Historical background
• Classification
• Hemangiomas and other common congenital
vascular tumor
• Vascular Malformation
• Diagnosis
• Management
3. Introduction
• Vascular Anomalies are congenital lesions of
abnormal vascular development
• “Anomalies” signifies any deviation in normal
cutaneous vasculature, either congenital or
acquired
• “Hemangioma” is commonly used in a
generic sense to describe a variety of vascular
lesions – congenital or acquired
4. Introduction : Vascular Anomalies
• Most Commonly divided in to Hemangioma &
Malformations
• Vascular tumors and Malformations may occur
anywhere on the body.
• Importance:
cause significant
Functional
Aesthetic impairment
Anxiety
6. Anatomico-Pathologic Classification
Virchow(1863)
a) Angioma
Angioma simplex: system composed of capillaries
Angioma cavernosum: a replacement of normal
vasculature with large channels
Angioma racemosum: tissue consisted of markedly
dilated interconnected vessels.
b) Lymphangioma
7.
8. Biologic Classification
Mulliken and Glowacki (1982)
On basis of cell kinetics, there are two major types
of vascular anomalies:
Hemangiomas: lesions demonstrating endothelial
hyperplasia.
Vascular Malformations : lesions with normal
endothelial turnover.
• Low flow
Capilary / Lymphatic / Venous
• High flow
Arterial / Arterio-venous / Capillary-lymphatic
Capillary-venous / Lymphatico-venous
Capillary-lymphatico-venous
9.
10. ISSVA Classification
• 1996 Classification modified by International society for the study
of vascular anomalies
• differentiates vascular tumors from vascular malformations.
• VASCULAR TUMORS VASCULAR MALFORMATIONS
1) Benign tumors 1) Slow/low flow
Infantile Hemangioma Venular, venous, lymphatic
Congenital Hemangioma
2) Intermediate tumors 2) Fast /high flow
Kaposiform hemangioendothelioma AVM
Spindle cell hemangioendothelioma AVF
14. Hemangiomas
• The word "hemangioma" comes from the Greek haema-, "blood";
angeio , "vessel"; -oma , "tumor".
• A hemangioma is a benign and usually self-involuting tumor of the
endothelial cells that line blood vessels, and is characterised by
increased number of normal or abnormal vessels filled with blood.
• Exhibits rapid early growth until 6-8 months of age, followed by
regression by 5-9 years of age.
• Capillary Hemangioma: Cavernous Hemangioma
the most common type.
made up of small capillaries is made up of larger blood vessels
that are normal in size and diameter, that are dilated. They are not as
but high in number. are typically closely packed and the spaces
Bright red in colour (or "caverns")
between them are filled with blood.
15. Infantile Hemangioma
• Present shortly after birth
• Well demarcated, flat & erythematous red patches
• Can be confused with several red birth marks
• Rapid proliferation & vertical growth – pathognomic
feature
• Appear during 1st to 4th week of birth, rapid post-
natal growth till 5 -6 month, after 1 year growth rate of
lesion is equivalent to child growth rate
• Five distinct developmental phase
PRODORMAL PHASE
INITIAL PHASE
PROLIFERATION PHASE
MATURATION PHASE
REGRESSION PHASE
16. • PRODORMAL PHASE
Precursor lesion – telengectasia, anemic reddish-blue, port-wine stain
Some times can not be detected
• INITIAL PHASE
Appear with in few days
Infiltrating to surrounding tissue
Clearly protrude from skin – light reddish , shine brightly
• PROLIFERATIVE PHASE
Exophytic or endophytic subcutaneous growth
CCDS (color coded duplex sonography) – hypercapilarization
• MATURATION PHASE
Reduction of bulk along with wrinkles
Gray regression area in dark red hemangioma
• REGRESSION PHASE
Usually accomplished by 6th birthday of child
Small lesions can regress without destroying surrounding tissue
Large lesions leave – telengectasia, areas of atrophy,
skin wrinkles, hypopigmentation
17. Associated malformative anomalies
• IH can arise in association with other structural anomalies
• Female predilection (9:1)
• Not necessarily vascular
• Acronym – PHACE
Posterior fossa anomalies
Hemangioma of face
Arterial abnormalities
Cardiac defect & coarcation
Eye anomalies
• VASCULAR ANOMALIES –
persistent embryonic extra / intra cranial arteries
Hypoplasia / absence of ipsilateral carotid vessels
Aneurysmal dilation of CA
Coarcation & Rt sided aortic notch
• Cerebroocclusive changes – seizure and stroke in infancy
• Chance of Sternal nonunion or Supraumbilical Raphe - PHACES
19. CONGENITAL HEMANGIOMA
RICH (Rapid Involuting Congenital Hemangioma)
• Red-violaceous color
• Central telengectasia
• Peripheral pale halo (occasional)
• Superficial ulceration
• Central nodularity
• Transient Thrombocytopenia
• Platelet level increase with regression
• Mainly seen in trunk & extrimities
• Rapidly involute during first week/ month
20. Congenital Hemangioma
NICH (Non Involuting Congenital Hemangioma)
• Ovoid/ macular/ slightly raised
• Light gray in colour
• Prominent coarse telengectasia
• Warm on palpation
• Predisposition in mandibular border
• Well circumscribed – 5-6 cm diameter
• Commonly diagnosed in late childhood
• Sometime expand in adolescence & exhibit polypoid
excrescence
21. RICH & NICH
• radiologicaly & histologically overlap with IH
• There are more similarities rather than
dissimilarities between His, RICHs, and NICHs
• They all can coexist with His
22. Evolution scheme of untreated IH, RICH, and NICH. IH appears after birth, grows generally
until the end of the first year of life, then stabilizes and gradually involutes most significantly
during years 1 to 2 and continues to improve over the ensuing years.
Michel Wassef et al. Pediatrics 2015;136:e203-e214
23. Tufted Angioma
• Develops in Infancy or early childhood on neck or
upper trunk
• Dull red macules with a mottled appearance
• Histo: Clusters of angiosomes tufts and lobules
scattered in the demis in a so called ‘Cannonball’
pattern
• There is still a controversy that whether this
lesion is a separate type or a variety of NICH
• US reveal – separate lobular structure with
vessels along the edges
• It is a rare lesion of head and neck region
24. Complication of IH
• Ulceration : <5% shows ulceration common in lip, anogenital region
• Obstruction
Vision : obstruction of visual axis, diplopia, can distort growing
cornea – error in refractive index
Respiratory : Hm in nasal tip can obstruct the vestibular passage,
subglottic hemangioma cause sever airway obstruction
Auditory : can obstruct auditory canal – conductive hearing loss
Bleeding : spontaneous bleeding from small puncture of lesion
can occur – occasionally associated with ulceration
Congestive Heart Failure
• Skeletal deformation
Deviation of nasal pyramid
Minor indentation of calvaria
Orbital enlargement
25. Pyogenic Granuloma
• Solitary, red papule, grows
rapidly, forming a stalk.
• Lesions less than 1 cm in
diameter
• Presentation is inversely
correlated with age
29. Vascular Malformations
• Present at birth.
• No rapid ‘proliferative phase’ and do not
‘involute’. They grow commensurately with the
patient.
• # 31% are in the head and neck region.
• Interruption at a particular stage of
development of a vessel may result in VM.
• The type of VM depends on stage at which
normal morphogenesis is interrupted.
30. Development of Vascular Malformations
• ENDOTHELIAL stage multiple
endothelium lined lakes are formed.
• RETIFORM stage, capillary
communication develops between
these lakes. Some of these
interconnecting channels have
muscular sheaths and others do not.
• MATURATION stage, channels that
have muscular lining differentiate to
become arteries and those without
muscle become veins.
Abnormal development of either arterial
or the venous side of vascular network
during this phase of development may
result in vascular malformation.
31. Progression of Vascular Malformations
• Trauma, infection, and hormonal fluctuation
(pregnancy or puberty) increases growth of VM.
• The mechanism of growth is due to alteration in
the flow dynamics within and around the lesion”.
•
• This results in recruitment of “collateral vessels”
and dilatation of involved vessels.
32. Capillary Malformations (Portwine Stain)
• Naevus flammeus neonatorum “Angel’s kiss” or
Stork bite
• appear as reddish-pink macules over facial &
nuchal region
• dermatomes may be smooth initially but
become more “ pebble – like” as the patient
grows.
• in 1999 Waner and Suen based on their
identification of the anomalies re-categorized
them as Venular Malformations
34. Associated Syndromes with CMc
• Sturge-Weber Syndrome SWS
encephalotrigeminal angiomatosis consists of
congenital Hamartomatous Malformations
that may affect the eye, the skin, and the
central nervous system at different times.
• Cutis Marmorata Telengiectatica Congenita
• Macrocephaly Capillary Malformation
35. Venous Malformations - Low-flow lesions
• “Cavernous Hemangioma” a misnomer
• Bluish, soft and easily compressible, no bruits.
• No “pulsations or a thrill”
• They are sequestered from the main vessel
• Undergoes a spontaneous continuous cycle of
thrombosis and thrombolysis,
• may undergo calcifications to form Phleboliths
that are painful on palpation and could be a
radiologic marker in some types of VeM
36. Classification of Venous malformation
Type Description
• Type I Isolated malformation without peripheral drainage
• Type II Malformation that drains into normal veins
• Type III Malformation that drains into dysplastic veins
• Type IV Venous ectasia
37. Genuine Diffuse Phlebactasia of
Bockenheimer
• Associated with Limb Discrepancy
Extensive venous malformtion of
Limb
• Klippel-Trenaunay –Weber
syndrome Triad of capillary
malformations, bone hypertrophy
and venous malformations.
40. Associated Syndromes with VeM
• Glomuvenous Malformation
• Cutaneomucous Venous Malformation
• Blue Rubber Bleb Nevus Syndrome
41. Lymphatic Malformations
-Low flow lesions
• Obstruction or sequestration of the primitive
lymphatic vessels during embryogenesis
• Within the oral cavity the LMs are more
commonly present on anterior 2/3 of tongue,
followed by palate, gingiva and oral mucosa.
• Predilection for head and neck and the axilla,
where embryonic lymph sacs are located
44. Lymphatic Malformations Macrocystic
LMs
(outdated terms include Lymphangioma
Cavernosum, Cystic Hygroma, Lymphangioma
Cysticum)
• Multiple cysts of >2 cm
• Commonly found in posterior triangle of the neck
and in the cervical area
• Localized painless non-pulsatile swelling
• No bruit or thrill
• A rubbery compressible consistency
• Normal appearing skin unless hemorrhage which
can produce a blue discoloration.
46. de Serres Classification of Lymphatic
Malformation of Head and Neck
Stages Location
• I Unilateral infrahyoid
• II Unilateral suprahyoid
• III Unilateral infra & supra hyoid
• IV Bilateral suprahyoid
• V Bilateral supra & infra hyoid
47. Microcystic LM (Outdated term
Lymphangioma)
In the oral cavity appear as multiple translucent non-
compressible cysts or vesicles of <2 cm. containing
viscous clear fluid, producing a pebbly or warty
surface resembling “frog spawn” or “tapioca
pudding”.
50. Arteriovenous Malformation
• Congenital High-Flow
• Growth triggered by
Puberty /Trauma
• Pathogenesis – TGF-
beta & a genetic two
hit hypothesis
(knudson’s)
51.
52. Arterial / Arteriovenous Malformations (AVM) “High-flow
lesions”
( outdated terms – Cirsoid aneurysm, arteriovenous aneurysm)
• Direct communication between the arterial and venous systems
through a nidus formed by arteriovenous shunts, along with
hypertrophy of the afferent arterial and efferent venous system.
• AVM is present at birth
• May become clinically apparent only during the 4-5th decade of
life and is often misdiagnosed due to delay in clinical presentation.
• The most common site for AVM is the brain, followed by the head,
neck, limbs, trunk, and viscera.
• The majority of the head and neck lesions occur on the cheek,
followed by the ear, nose, forehead and upper lip.
53. AVM: Clinical Presentation
• They are purple-blue raised painful macule, are pulsatile
with thrill and bruit, warm to touch.
• Do not empty fully on compression, and refill quickly on
reliving digital pressure.
• Can have embolism, pain, bleeding, ulceration, and
congestive cardiac failure due to increased cardiac load.
• Often a patient presents with severe bleeding as the first
sign that a high flow-lesion is present. They may also
complain of recurrent gingival bleeding and loose or
depressible teeth
54. Staging of arterio-venous malformations
Schobinger Staging of AVM
• Stage 1 (Quiescence) : A blue-skin blush, warmth and AVM on
Ultrasound
• Stage2 (Expansion) : A mass with a pulsation,bruit and a thrill
• Stage 3 (Destruction) :A mass with ulceration, bleeding and pain
•
• Stage 4 (Decompensation) :lesions producing heart failure
55. Combined Vascular Malformation
• CM + VM Capillary-venous malformation
• CM + LM Capillary-lymphatic malformation
• CM + AVMCapillary-arteriovenous malformation
• LM + VM Lymphatic-venous malformation
• CM + LM + VM Capillary-lymphatic-venous malformation
• CM + LM + AVM Capillary-lymphatic-arteriovenous
malformation
• CM + VM + AVM Capillary-venous-arteriovenous
malformation
• CM + LM + VM + AVM Capillary-lymphatic-venous-
arteriovenous malformation
56. Vascular Malformations Associated With Other
Anomalies
• Klippel-Trenaunay syndrome: CM + VM +/− LM + limb overgrowth
• Parkes-Weber syndrome: CM + AVF + limb overgrowth
• Servelle-Martorell syndrome: limb VM + bone undergrowth
• Sturge-Weber syndrome: facial + leptomeningeal CM + ocular anomalies
+/− bone and/or soft tissue overgrowth
• Limb CM + congenital nonprogressive limb hypertrophy
• Maffucci syndrome: VM +/− spindle cell hemangioma + enchondroma
• Macrocephaly-CM (M-CM)/megalencephaly-CM-polymicrogyria (MCAP)
• Microcephaly-CM (MICCAP)
• CLOVES syndrome: LM + VM + CM +/− AVM + lipomatous overgrowth
• Proteus syndrome: CM, VM and/or LM + asymmetric somatic overgrowth
• Bannayan-Riley-Ruvalcaba syndrome: AVM + VM + macrocephaly,
lipomatous overgrowth
• CLOVES, congenital, lipomatous, overgrowth, vascular malformations,
epidermal nevi and spinal/skeletal anomalies and/or scoliosis.
57. Hereditay Haemorrhagic Telengiectasis (Rendu
Osler Weber disease)
• Congenital hereditary disease
• Numerous telangiectatic or angiomatous areas.
• Triad of telangiectasia, recurrent epistaxis, and a
positive family history.
• KASABACK – MERRITT SYNDROME
Hemolytic anaemia ,
thrombocytopenia •
coagulopathy.
58. Kippel- Trenaunay Syndrome
• Also knownas Capillary- Lymphatico-
Venous Malformation (CLVM)-slow
flow type
• Sporadic
• Association with gene not yet known
• Limb hypertrophy present at birth
which
• Progressively worsens with growth
• Complicatio-recurrent infection,
• Fast flow associated with- Parkes –
Weber syndrome
61. Computed tomography
• To define –bony architecture, identify
phleboliths and other dystrophic calcification,
to describe hepatic lesions
• CECT / Helical CT – More informative in
Arteriovenous malformations- arterial,nidus
and venous structures.
62.
63.
64. MRI
• Excellent for evaluating low flow malformation
and soft tissue
• Protocol-spin echoor fast spin echo T1
weighted imaging- for baseline
• T2 weighted short tau inversion recovery
images- hemosiderin, dystrophic calcification
or phlebolith
65.
66. ARTERIOGRAPHY
• An invasive diagnostic test that uses x-rays to
take pictures of blood vessels.
• A long flexible catheter is inserted through the
femoral artery to deliver dye (Iodine & Barium
compounds) into the arteries making them
visible on the x-ray.
• This test can help diagnose an arteriovenous
malformation, tumor, clots, and arterial
stenosis
69. Nuclear Medicine in diagnostic of
Vascular Malformation
• Whole Body Blood Pool Sinctigraphy (WBBPS)
• Make red blood cell visible through physiological contrast medium
• High &/or altered “hematic” signal indicate the presence of VM
• Advantage
Discrimination can be done between high and low flow lesions
• Limitation
• Poorly detailed image
• Do not allow discrimination between venous and arterial structures
• Lymphosinctigraphy
74. Propanolol
• A serendipity – 2008
• Newborn with proliferative hemangioma with
cardiovascular ds- on propranolol therapy
• Shows rapid regression of lesion
75. PROPRANOLOL
• for IH
• Leaute – Labreze and colleagues, 2008, reported the
regressing effect of propranolol on infantile hemangioma
• It has effect on growing hemangiom by 3 different
molecular mechanism
• Vasoconstriction
• Inhibition of angiogenesis
• Induction of apoptosis
• Doses: 1-3 mg/kg/day for 3 to 12 month depending on
severity
• Currently it was suggested to tapper the doses over 2-3 wk
at the end of treatment to avoid REBOUND TACHYCARDIA
79. STEROID THERAPY
• When a large facial hemangioma began to shrink
coincidently with steroid administration for
thrombocytopenia.
• The response is reported to be in range of 30-90%
(Edgerton 1976).
• Effect of corticosteroid
Induction of apoptosis / Inhibition of angiogenesis
• Recommended dosage Prednisolone : 1-5 mg/kg/day
• Initial higher dosage for life threatening condition
• According to recent studies 2mg/kg/day in two
divided dose for 3 month, followed by 6-9 month
tapering dose
80. Vincristine therapy
• used for corticosteroid resistant
hemangiomas
• It is naturally occurring vinca alkaloid isolated
from the leaves of periwinkle plant ,
Catharanthus roseus
• Interfere with mitotic spindle microtubules by
binding to tubulin – inhibit mitosis
• Dose : 1mg/sq.m or 0.05mg/kg – used by IV
route in tapering dose
81. INTERFERON THERAPY
• Giant hemangiomas that have been unresponsive
to corticosteroid therapy
• Interferon therapy, which inhibits angiogenesis,
• It is administered subcutaneously and daily.
• Ezekowitz et al demonstrated 50% reduction in
lesion size in cases which were refractory to
corticosteroid therapy.
• Dose : 1-3 x 10^6 unit/sq.m body surface/day –
via s/c route
82. Cyclophosphamide
• Alkylating agent – used for VM treatment –
reviewed by Hurvitz et al
• Side effect • Nausea • Vomiting • Reversible
alopecia
83. Sclerotherapy
• Sclerotherapy is of irritant solution into lumen
of a vessel- causes thrombosis and
subsequently fibrosis
• Ideal Sclerosing solution
-Painless to inject
- Free of adverse effects
- Specific to affected vessel
85. Ethanol Sclerotherapy
• Gold Standard – widely used and accepted
percutaneous sclerosant
• Readily available, inexpensive, easy to use.
Long shelf life
• Potent Irritant-transmural destruction of
vessel wall
• Avoided near to- large nerves, skin, mucosa,
genitalia, finger / toes.
• Not exceed 1ml/kg at any one visit.
• Ethylcellulose and Ethibloc
86. Bleomycin sclerotherapy
• Glycopeptide antibiotic- Streptomyces
verticillus
• Most comonly used slerosing agent for the
treatment of vascular anomalies in china
• Sclerotherapy has no great pain or nerve
injury- the cervical-facial region
• USG guide is most effective method
87. Sodium tetradecyl sulfate
• An anionic surfactant
• Widely used infor sclerosis of esophageal
varices and varicose veins
• Now also in Vascular Malformation
• Not effective as ethano
• Cause urticaria, anaphylaxis, hemolysis and
hematuria
88.
89.
90. Sclerotherapy for Lymphatic
Malformation
• Newer agent particular for LMs-OK432
• Lyophilized powder made Streptococcus
pyogens (Grp A, type 3, Su strain) inoculated
with benzylpenicillin.
• Multi-institutional randomised trial for Ok-432
• 95% of complete for macrocystic and 0-80%
for micro cystic or cavernous subtype
91. Side effects and Complications
• Allergic reaction
• Matting- is the appearance of fine telangiectasis
which disappears over a period of time
• Cutaneous necrosis
• Superficial thromboplebitis
• Hematoma formation
• Hyperpigmentation
• Damage to surrounding nerve
• Pulmonary embolism ?
94. Endovascular Management
• In management of truncular Malformation
• Thermal Laser Ablation
• Thermal Ablation- mainly used for venous
incompetence
• Minimal role in other Vascular Malformation
95. LASER THERAPY
• Apfelberg advocated the use of Argon Laser
treatment for hemangiomas in proliferative
phase.
• Argon Laser penetrates the skin, and the blue-
green light is absorbed by red cells with in the
hemangioma and normal vessels in the papillary
dermis.
• Disadvantages – Thermal damage with in the skin
may cause ulceration of the superficial portion of
the hemangioma; the end result is scar.
• persistence of deep hemangioma because
currently available argon lasers do not penetrate
deeper than 1.5mm into the skin.
• Laser is useful in treating capillary dermal
malformations
98. OPERATIVE THERAPY
• If every hemangioma began as a localized nest of
cells, the ideal t/t would logically be early excision
before the tumor extended into the surrounding
dermis ( Modlin,1955; Andrews et al,1957)
• It is usually best to wait until the child is 8-12
years of age before trimming the residual that
exists after regression.
• There is usually sufficient extra skin remnant after
involution for linear closure.
99. Indication of Surgical Excision
• Rapidly growing
• Affecting anatomical site where scar would be
easily hidden
• Non involutive congenital Hm
• Hm of nose producing secondary cartilage
deformity
• Ulcered and bleeding Hm not responding to
corticosteroids and/or laser treatment
123. Conclusion
• Inspite of a developed classification – misnomers are still used
• Don’t intervene – till the nature of lesion is not known.
• Controlled aggressive approach is required for management of
vascular malformation.
• Use of minimal invasive technique always have better
outcomes until complication not occurred.
• Most of the Extratruncular lesions almost always requires
multimodality of treatment. Truncular mostly require surgery
• Vascular Malformation with complication or at life threatening
location required urgent intervention.
I am honoured to be here . I thank the organisers for selecting me an d be a part of this. Bottom line her eis I am aplasti c surgeon and even though I have a very large series of to my credit by virtue of my experience oi worjing at Nilofer hospita, events like these alosgive us an opprtunity to learn and upgrade our knowledge
This is a brief overview of my presentation
In common practice any lesion which is vascular is called as hemangiomas. Even though we have come a long way historically speaking. I think if we see the latest classification, we will get a lot of clarity.
VA which all the disease related to vessels which are abnormal. Which are congenital or acquired
Importace:
In short it is important to understand difference between vascular tumours and malformations
Importace: they can cause functional /aesthetic problems and most importantly is anxiety
Anatomically we have artery, capillary veins and lymph channels and pathologially it can be just simple involvement or complex involvement
1982 mulliken tried to simplify it further in to hemangiomas and malformations
Which is called as biologic classification
With or without endothelial hyperplasia
As you see here it is further claasifiede as low flow and hih flow depending on acticvity with in lesion
1193 jaclson tried to separate lymphatic malformation separately which is not though accepted universally but it ease out in separating things
1996 ISSVA formed a classifictaimn by studying the nature of disease in to tumors and malformations
Which is further modified in to adding of other complex syndromes and asso anomalies. As it was found that you can have benign tumours , locally aggressive lesions and malignant tumours. Similarly malformations can be simple ,combined or syndromic or other unclassified ones
Tumour of blood
Further classified originally as capillry or cavernoue type which are not so red
iF is present at bith they are generally smally macules or patches which rapidkey priliferate ad grow
Grow till 1 year
Evolution scheme of untreated IH, RICH, and NICH. IH appears after birth, grows generally until the end of the first year of life, then stabilizes and gradually involutes most significantly during years 1 to 2 and continues to improve over the ensuing years. CHs are fully grown at birth and rapidly involute (RICH) or persist indefinitely (NICH). Reprinted with permission from Mulliken JB and Enjolras O.27 NICH, noninvoluting congenital hemangioma; RICH, rapidly involuting congenital hemangioma. The Y axis show relative size.