VASCULAR MALFORMATION

AND LYMPHOEDEMA
HISTORY
 Birth mark implies a causal relationship
between the mother's experience and
child's blemish
imagination
cravings
 Malformations were the result of faulty
embryological development and not
maternal impression.
CLASSIFICATION
Multiple classification schemes
 Obstacle to our understanding and management of lesions
 Often a single term for various kind of lesions or several
terms may be given to the same anomaly

Four major categories

1. DESCRIPTIVE
 Strawberry hemangioma, cherry angioma
 Cirsoid aneurysm, erectile tumor
CLASSIFICATION (contd.)
2. Anatomicopathological
 VIRCHOW
 angioma simplex-capillary tumor
 angioma cavernosum –larger channels
 angioma racemosum _large, dilated , and
interconnecting vessels
 Wegner (lymphatic condition)
 lymphangioma simplex
 lymphangioma cavernosum
 lymphangima cystoides
CLASSIFICATION (contd.)
3. Embryological-theory based on errors in
embryogenesis
 Capillary network formation
 capillary hemangiomas
 Large vessel formation
 cavernous hemangiomas
 Later stage of fetal development
 arteriovenous fistula
CLASSIFICATION (contd.)
4. Biological
 Distinction between the true tumors of vascular origin and
vascular malformation made in early 20th century
 In 1982 Mulliken and Glowacki published classification scheme
which correlates the cellular features of vascular anomalies with
clinical characteristics and natural history, thus giving rise to
two main categories:

A) Hemangioma
 Antenatal or postnatal endothelial proliferation, rapid growth
during infancy, slow regress during childhood and never
appears in adulthood.
B) Vascular malformation
 Developmental error
dysplastic vessels lined by quiescent endothelium ,usually
obvious at birth never regress and often expand .
HEMAGIOMA
EPIDEMOLOGY
 Most common tumors of infancy
 Exact prevalence difficult to assess
 Most harmless benign lesion
 Many deep in the tissue
 Generally 1 in10 children
 Incidence increase (30%) in premature infant
 Head and neck common site
 Female to male ration 3:1
 80%of the case seen in the 1st moth of life
 14 to 20% of the affected infant has more than
one lesion.
HEMAGIOMA (Contd.)
PATHOGENESIS
 Etiology of hemangioma is not well understood
 The key stone to any theory of the origin of
hemangioma is the point during fetal
development at which a hemangioma begins.
 Different believe
 sequestrated embryonic mesoderm
 analogous to retrolenal fibrovascular proliferation
 True tumor arising independently from normal
tissue
HEMAGIOMA (Contd.)
PATHOGENESIS (Contd.)
 Growth potential depend on its location over normally
situated subcutaneous artery
 Proliferating hemangioma are composed of rapidly dividing
endothelial activity gradually diminishes and the cells
flatten and mature
 Mast cells abundant in proliferating hemangioma (40%). It
influence migration of capillary endothelial cells by
producing heparin.
 Proliferative hemangioma has multilaminated basement
membrane.
 Rate of turn over endothelium angiogenesis dependent.
HEMAGIOMA (Contd.)
NATURAL HISTORY
 Proliferative phase
 Extend from birth to 6-20 month
 Herald spot (localized telangiectasia) - expanding
irregular pebbly crimson grows up to it reach a plateau.
 Involution phase
 Begin at 6-20month and can continue for the next five to
10 years age.
 1st fading from bright red to pink, lesion becomes softer
and flatter
 2nd gradual decrease in size and thickness
 3rd pebbly skin texture recedes ,the mass soften,
overlying skin shrink and finally it become normal or
excessive baggy wrinkles.
HEMAGIOMA (Contd.)
SIGN & SYMPTOM
 Typically appear neonatal period up to 2-3 month
 Affect head &neck in most patient
 Red (flat or raised) (patches or plaque) with or
with out cluster of superficial veins
 Pale spot (anemic nevus)
 Telangiectic or ecchymotic patch
 Firm and rubbery consistency
 Darken during involutional phase
 Scar if ulcerated
HEMAGIOMA (Contd.)
INVESTIGATION
 ULTRA SOUND
 C/T SCAN
 ARTRIOGRAPHY
 MRI
HEMAGIOMA (Contd.)
COMPLICATION
 Bleeding
 Ulceration
 Infection
 Obstruction
 Kasabach Merritt syndrom
 C.H.F
 Skeletal distortion
 Cosmetic deformity
HEMAGIOMA (Contd.)
Associated malformation
 Kasabach merritt phenomenon
 Cutaneous-visceral hemangiomatosis
 Cervicofescial hemangioma
HEMAGIOMA (Contd.)
Differential diagnosis
 Vascular malformation
 Pyogenic granulomas
 Tufted angioma
HEMAGIOMA (Contd.)
Management
 Observation
 Majority will regress (No treatment required)
 Some require treatment
 Mode of treatment
 Medical: corticosteriod, sclerosing agent, irradiation,
compression, chemotherapy, antiplatelate
 Steroids
 Oral predinsolone 2mg/kg---tappering---
 Steroid injection(dexametason) 0.1ml ,1cm
apart(large dose---skin necrosis)
 Invasive: surgical excision, laser therapy, cryotherapy,
embolic therapy
VASCULAR MALFORMATIONS
 structural abnormalities resulting from
faulty embryonic morphogenesis
 present at birth
 grow proportionately with the child
 do not regress
 are not neoplastic
 low-flow or high-flow
VASCULAR MALFORMATIONS
(Contd.)
PATHOGENESIS
 Errors in morphogenesis during retiform
stage of embryonic life result in vascular
malformation but central events and
causes remain purely speculative (e.g.
genetic)
VASCULAR MALFORMATIONS
(Contd.)
LOW-FLOW VM
1. Capillary malformation
2. Venous malformation
3. Lymphatic malformation
4. Lymphatic-venous malformation
VASCULAR MALFORMATIONS
(Contd.)
1. Capillary Malformation
 macular red vascular stain, present at
birth, persist throughout life.
 Can be localized or extensive (face)
 Most are harmless cutaneous birthmarks
 May be found in combination with other
malformation (Sturge Weber syndrome,
klippel-Trenauny
 Treatment is flashlamp pulsed dye laser
VASCULAR MALFORMATIONS
(Contd.)
2. Venous malformations
 spongy, masslike lesions composed of abnormal
veins, ie, veins with a relative lack of smooth
muscle cells in their walls
 It present at birth (not always evident )
 Usually solitary but multiple cutaneous or
visceral lesion can occur
 Present as faint blue patch or soft blue vascular
mass ,pain & stiffness at the area of lesion,
episodic thrombosis occurs
 Mass effect seen craniofacial VM
 Treatment- elastic support stocking, analgesics,
low dose ASA, surgical extirpation, sclerosant
Caption: Picture 9. Vascular anomalies . Venous malformation (VM).
Photograph of a young child shows a facial venous malformation that appears
as a bluish skin discoloration without a mass.
Caption: Picture 9. Vascular anomalies. Venous malformation (VM). Photograph of a young child
shows a facial venous malformation that appears as a bluish skin discoloration without a mass. all
VM in the lower lip, which is characterized by bluish mucosal discoloration and mild soft-tissue
swelling.
Normal vein and venous malformation.
Figure 2
VASCULAR MALFORMATIONS
(Contd.)
3. LYMPHATIC MALFORMATION
 Composed of dysplastic vesicles or pouches filled with
lymphatic fluid.
 Classified as microcystic, macrocystic or combined form
 most LM are evident at birth or detected before 2 yrs
 Common site of occurrence is neck, followed by the axilla
&pectoral region. Within the head the tongue cheek and
floor of the mouth.
 Pure lymphatic malformation can involute spontaneously
at least partially due to change in lyphaticovenous
connections or repeated bouts of inflammation with
secondary scarring.
VASCULAR MALFORMATIONS
(Contd.)
3. LYMPHATIC MALFORMATION (Contd.)
SIGN & SYMPTOM
 Tiny vesicle
 Mandibular body over growth
 Vesicles ,swelling, bleeding
 Air way involvement
 Proptosis
 Diffuse or localized swelling with soft tissue and
skeletal over growth
 Bladder out let obstruction constipation
recurrent infection
Large lymphatic malformation in the left shoulder region. The lesion is easily
compressible because it consists of large cysts and numerous microcysts. A
surgical scar is due to a previous attempt to remove the lesion
surgically.Figure 14
VASCULAR MALFORMATIONS
(Contd.)
3. LYMPHATIC MALFORMATION (contd.)
TREATMENT
 Antibiotics
 Staged partial or planned excisions
 sclerotherapy
VASCULAR MALFORMATIONS
(Contd.)
4. Lymphatic venous malformations (LVMs)
 consist of mixed clinical and imaging
findings of lymphatic malformations and
venous malformations
VASCULAR MALFORMATIONS
(Contd.)

High Flow
1. ARTERIOVENOUS MALFORMATION
2. ARTERIOVENOUS Fistula
VASCULAR MALFORMATIONS
(Contd.)
1. ARTERIOVENOUS MALFORMATION
 Have a nidus, a central confluence of tortuous and
dysplastic vessels where the arterial blood is shunted to
veins
 First noted several year after birth or after certain
triggering change

 . Arteriovenous malformations generally enlarge in size and

number (eg, a single nidus may become multiple) and may
cause significant health problems as a result of progressive
arteriovenous shunting resulting in high-output cardiac
failure, ischemia or bleeding
 Redness, increased warmth, pulsatil mass,thrill
 Pain and skin change
 Bleeding from dental manipulation
 Cynosis, clubbing, polycythemia
Arteriovenous malformation nidus is an abnormal communication network between the
arteries and veins (dark vessels represent arteries and lighter vessels represent veins).
Caption: Picture 4. Vascular anomalies. Arteriovenous
malformation (AVM). Photograph of the right hand of a patient
shows soft-tissue swelling but no skin discoloration (see Image
5).
Caption: Picture 5. Vascular anomalies. Arteriovenous malformation (AVM). A young boy
(patient in Image 4) presented with soft-tissue swelling and pain in the right hand. Arteriogram of
the right arm (ulnar artery injection) shows an AVM with multiple feeders. Multiple, large, early
draining veins and aneurysmal lesions are depicted
VASCULAR MALFORMATIONS
(Contd.)
2. Arteriovenous fistulas (AVFs)
 Simple arteriovenous connections
 Most AVFs are secondary to penetrating
injuries after birth, although some are
believed to be congenital.
 Clinical manifestation: high out put
cardiac failure, ischemia, bleeding
VASCULAR MALFORMATIONS
(Contd.)
Investigation
 Arteriography
 Magnetic resonance angiography
 CT angiography
 Ultrasonography
VASCULAR MALFORMATIONS
(Contd.)
TREATMENT
 EXCISION
 EMBOLIZATION
LYMPHEDEMA
 abnormal collection of protein-rich
interstitial fluid within the skin and
subcutaneous tissue due to defective
function of the lymphatic system.
LYMPHEDEMA (contd.)
SURGICAL ANATOMY
 The lymphatic of extremities consists of
EPIFASCIAL & SUBFASCIAL
 Epifascial- Dermis drained by valveless lymphatics
toward valved lymphatic at subdermal level then it
drains into larger valved trunks above the deep fascia
 Subfascial-lies below the deep fascia ,ascend as 2to4
channels alongside the major vein of the extremity
 Communications exist between the epifascial
&subfascial lymphatics ,it is fewer and the valves
direct the flow from deep to superficial
LYMPHEDEMA (contd.)
PHYSIOLOGY
 Lymphatic system function –recycling of high
molecular weight proteins with their osmotically
associated water from interstitial space to the
blood circulation &passage of lymphocytes and
antigen-presenting cell to lymph node
 Lymphatic load from prelymphatics space through
a flap valve mechanism enters the lymphatics to
become lymph
 From lymphatics to collecting lymphatics to
regional lymph nodes and venous circulation
 Two-4 lit of fluid and 50-80%of total plasma
proteins recycle
LYMPHEDEMA (contd.)
PATHOPHYSIOLOGY
 Lymphedema confined to the tissue superficial to
deep fascia
 Extravasation of protein-rich fluid occurs when
lymphatic load exceed lymphatic transport
capacity due to lymph vascular system
insufficiency
 Infection & interstitial fibrosis
 The obstructed lymphatics system progressively
dilate and edema accumulates finally
subcutaneous fibrosis and brawny edema form
LYMPHEDEMA (contd)
CLASSIFICATION:
1. Primary lymphedema : occurring
without apparent cause
Congenital (Milroy’s disease)
Praecox
Tarda (Meige’s disease)
2. Secondary : filariasis ,malignancy ,
surgical excision , radiotherapy , trauma,
infection , chronic inflammation
LYMPHEDEMA (contd.)
INVESTIGATION
Radioisotope lymphography
Contrast lymphangiography
CT & MRI
Ultrasound scanning
LYMPHEDEMA (contd.)
MANAGEMENT :
1. Medical treatment :
 Physical treatment - aims improving lymphatic drainage by
mechanical means (eg exercise ,compression hosiery,
massage, multilayer bandaging, elevation of limbs and
pneumatic compression)
 Prevention of acute infection
 Diuretics
2. Surgical treatment : indications
marked disability or sever deformity from limb swelling
proximal lymphatic obstruction
lymphocutaneous fistulae and megalymphatics
 Reduction operation and lymph drainage procedure