Haemangioma and vascular anomelies

HEMANGIOMAS & VASCULAR

MALFORMATION

DR. ANINDYA2ND YR PGT OMFS

content

Introduction Historical background Classification Hemangiomas and other common congenital vascular tumor Vascular Malformation Diagnosis Management

Introduction Vascular anomalies are congenital lesions of abnormal vascular development

“Hemangioma” is commonly used in a generic sense to describe a variety of vascular lesions – congenital or acquired

“Anomalies” signifies any deviation in normal cutaneous vasculature, either congenital or acquired

Depending on clinical and cellular studies – Mulliken and Glowacki (1982) – vascular anomalies of infancy in 2 group – Hemangioma & Malformation

Both vascular tumors and malformations may occur anywhere on the body.

These lesions predominantly occur within the head and neck and effect 1 in 22 children (Dorolet et al 1999, Greene et al 2008)

Depending upon the size and location, significant functional and esthetic impairment can result from the growth of such hemangiomas or vascular malformations.

Historical Background First report by Guido Guidi – personal physician of King Francis I in 16 th century

In 1628 William Harvey – “ Excercitatio anatomica de motu cordis et sanguinis in animalibus” in Frankfort Germany – about blood circulation

In 1757 William Hunter – signs of AV malformation – bruit, that disappear on compression

1872- George Bushe – Congenital temporal AV fistula

1843 – Norris – surgical treatment by Double Ligature of artery

1923 – introduce ARTERIOGRAPHY – Sicard and Foresier

1976 – Young & Mulliken – a small meeting for such problems

1992 – Yakes – meet in Denver – formed – International Society for the Study of Vascular Anomalies (ISSVA)

Classification Anatomicopathologic Classification

The earliest attempt at classification of vascular lesions was made by Virchow(1863) a) angioma b) lymphangioma

1.Angioma simplex: system composed of capillaries.2.Angioma cavernosum: a replacement of normal vasculature with large chanels.3.Angioma racemosum: tissue consisted of markedly dilated interconnected

vessels.

Biologic ClassificationMulliken 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

In 1996 this classification was modified by ISSVA( International society for the study of vascular anomalies)

Presently ISSVA differentiates vascular tumors from vascular malformations.

VASCULAR TUMORS VASCULAR MALFORMATIONS1) Benign tumors 1) Slow/low flow

Infantile Hemangioma Venular Congenital Hemangioma venous lymphatic 2) Intermediate tumors 2) Fast /high flow

Kaposiform hemangioendothelioma AVMSpindle cell hemangioendothelioma AVF

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: (occur in superficial layer of skin) This is the most common type of hemangioma. It is made up of small capillaries that are normal in size and diameter, but high in number. Because of their proximity to the surface of the skin, capillary hemangiomas are typically brighter red in color

Cavernous Hemangioma In contrast with a capillary hemangioma, a cavernous hemangioma is made up of larger blood vessels that are dilated. The blood vessels are not as closely packed as in a capillary hemangioma, and the spaces (or "caverns") between them are filled with blood.

PATHOGENESIS of Hemangioma Various theories:1. Virchow speculated that mechanism was a progressive

irritation of tissue, likely to occur about the margins of fetal clefts that are well supplied by blood vessels.

2. Malan (1974) proposed that “dormant angioblasts” become activated to form hemangiomas- a delayed expression of genetically programmed growth and involution of embryonic capillary network.

3. Kaplan (1983) stated that hemangioma is a failure of normal morphogenesis from the embryonic stage of undifferentiated capillary network.

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

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

Histologically Proliferative phase

Rapidly growing endothelial cells Sinusoidal channel organized into lobular compartments, separated by fibrous septa that contain large

caliber feeding and draining vessels Mast cells produce heparin which stimulates migration of cultured capillary endothelium

and potentiates the proliferation of endothelium by endothelial cell growth factor Angiogenic marker (FGF, VEGF) – high Enzymes (type IV collagenase, metalloprotiase) - high

Regression phase Angiogenic marker – reduce to normal level enzymes

Involution phase Proliferation and involution occur concurrently after the first year. i.e., even when

involution is well under way there are scattered proliferative foci. (Mulliken and Glowacki, 1982).

Reduced endothelial proliferation Increased apoptosis Preponderance of stromal cell There are islands of fat and dense collagen deposited in the perivascular areas

(Dethlfsen, Mulliken and Glowacki, 1986).

Clinical Features- proliferative phase

Most manifest during 1st-4 weeks of life. Initial sign is either an erythematous macular

patch, a blanched spot, or localized talengiectasia surrounded by a pale halo.

May grow as a single localized tumor or may simultaneously proliferate in multiple sites anywhere in body.

The hallmark of hemangioma is rapid neonatal growth.

Clinical Features- Involution phase

After a period of rapid growth, hemangioma stabilize with time, growing at same rate as the child.

First sign of regression is fading of the shiny crimson surface to a dull purple hue.

In time surface assumes a mottled grayish mantle that spreads centrifugally toward periphery of the lesion.

Lesion is less tense to palpation. When child cries lesion does not swell. By age of 5 years last traces of color are fading.

SUPERFICIAL Raised Crimson in color Firm & rubbery

DEEP Appear in lower dermis Slightly raised Bluish hue in overlying skin

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

CONGENITAL HEMANGIOMARICH (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

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

RICH & NICH radiologicaly & histologically overlap with IH There are more similarities rather than dissimilarities between

His, RICHs, and NICHs They all can coexist with IHs

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

Kaposiform Hemangioendothelioma Uncommon aggressive vascular tumor associated with Kasabach-

Merrit Phenomenon Sever coagulopathy due to platelet trapping It involve multiple tissue plane with edema Haemosederin deposition is found ill defined perpuric mass is evedent Extremely low platelet count and fibrinogen level

Complication of IH Ulceration : <5% IH shows ulceration – common in lip, anogenital region Obstruction

Vision : obstruction of visual axis, diplopia, growing Hm 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 : Hm in parotid region 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

Vascular Malformations Vascular malformations are present at birth and unlike

hemangiomas, do not go through a a “rapid proliferative phase” and they do not “involute”.

They grow commensurately with the patient.

Approximately 31% of these malformations are found in the head and neck region.

Vascular malformations are thought to “result when there is interruption at a particular stage of development of a vessel”.

The type of vascular malformation that results depends on the stage at which normal morphogenesis is interrupted.

Development of Vascular Malformations Blood vessels form as the result of series of steps.

First stage: ENDOTHELIAL stage, multiple endothelium lined lakes are formed.

Second stage: RETIFORM stage, capillary communication develops between these lakes.

Some of these interconnecting channels have muscular sheaths and others do not.

Final stage: 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.

Trauma, infection, and hormonal fluctuation (pregnancy or puberty) may stimulate increased growth of the vascular malformation.

The mechanism of growth is not increased endothelial proliferation - which is within a normal range in these lesions, as is the number of mast cells – “but alteration in the flow dynamics within and around the lesion”.

This results in recruitment of “collateral vessels” and dilatation of involved vessels.

Capillary Malformations ( portwine stain)

appear as reddish-pink macules over facial dermatomes may be smooth initially but become more “ pebble – like” as the patient grows.

In older classifications these malformations are denominated as Capillary Malformations,while in 1999 Waner and Suen based on their identification of the anomalies of these lesions in the post-capillary venules (rather than in the capillaries) re-categorized them as Venular Malformations

Lymphatic Malformations - Low- flow lesions Obstruction or sequestration of the primitive

lymphatic vessels during embryogenesis produce ectopic lymphatic systems, and the resulting failure of drainage from these areas lead to increase in intravascular pressure and LMs.

Within the oral cavity the LMs are more commonly found on the 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.

Lymphatic Malformations

In the oral cavity appear as multiple translucent non-compressible cysts or vesicles of <2 cm.

containing viscous clear fluid, producing apebbly or warty surface resembling “frogspawn” or “tapioca pudding”.

MICROCYSTIC LM ( Outdated term Lymphangioma)

Lymphatic Malformations

Macrocystic LMs (outdated terms include lymphangioma cavernosum,cystic hygroma, lymphangioma cysticum)

• usually presents as multiple cysts of >2 cm

and are commonly found in the supra-clavicular fossa of the• posterior triangle of the neck, and in the cervical area just below the angle of the mandible.

• They clinically appear as localized painless non-pulsatile swelling with• no bruit or thrill, having a rubbery• compressible consistency, and covered by• normal appearing skin unless hemorrhage or• communication with venous malformations• produce a blue discolouration.

deSerres classification of lymphatic malformation

stage LocationI Unilateral infrahyoidII Unilateral suprahyoidIII Unilateral infra & supra hyoidIV Bilateral suprahyoidV Bilateral supra & infra hyoid

Venous Malformations - Low-flow lesions

Venous malformations are bluish, soft and easily compressible, and auscultation reveals no bruits.

The clinical absence of “pulsations or a thrill” generally indicates a low flow Venous vascular malformation

Most of the VeM that are sequestered from the main vessel undergoes a spontaneous continuous cycle of thrombosis and thrombolysis, and these thrombus may undergo calcifications to form phleboliths that become painful on palpation and could be a radiologic marker for these type of malformations

Venous Malformations – Phleboliths

.Phleboliths that may be noted on radiographic examination are found only in low flow lesions.

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

Arterial / Arteriovenous Malformations (AVM)

“High-flow lesions”( outdated terms - cirsoid aneurysm,arteriovenous aneurysm)

They represent a group of congenital malformations that create a 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, but 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.

Arterial / Arteriovenous Malformations They appear as 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. They are associated with 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.

Staging of arterio-venous malformationsSchobinger Staging of AVM

Stage 1 (Quiescence) : A blue-skin blush Stage2 (Expansion) : A mass associated with a bruit and a

thrill Stage 3 (Destruction) :A mass associated with ulceration,

bleeding and pain Stage 4 (Decompensation) :lesions producing heart failure

Clinical Differences Hemangioma A hemangioma may or may not

be present at birth.

They involute spontaneously with age.

Rapid postnatal growth and very slow involution.

Females are more commonly affected; 3:1 (Mulliken and Glowacki, 1982).

They are true benign neoplasm of endothelial cells.

Vascular malformation Vascular malformations are always

present at birth. They donot involute usually.

A vascular malformation grows proportionately with the child.

Vascular malformations have no gender predilec tion.

Are localized defects of vascular morphogenesis that results in formation of abnormal, tortuous and enlarged vascular channels

Cellular Differences. Hemangioma The rapidly growing

hemangioma is composed of rapidly dividing endothelial cells.

In addition, mast cells, known to play a role in neoangiogenesis, increase during the proliferating phase

The mast cells fall to normal levels as involution is concluded.

Vascular malformation No evidence of cellular

hyperplasia but rather progressive ectasia of structurally abnormal vessels.

The malformed channels are lined by flat, quiescent endothelium, lying on a thin basal lamina.

No increase in mast cells.

Radiographic differences Hemangioma

Angiographic study shows hemangioma as a well circumscribed mass with intense, prolonged tissue staining that is usually organized in a lobular pattern.

Feeding arteries may form an equatorial network at the periphery of the tumor.

Vascular malformation Vascular malformations are

diffuse lesions consisting entirely of vessels without intervening parenchymal staining.

The angiographic pattern depends on the predominant chanel type, i.e., capillary, venous, arterial or a combination.

Skeletal Differences Hemangioma

Proliferating hemangioma rarely causes bony/ cartilagenous distortion or hypertrophy.

Maxillary or mandibular overgrowth may occur secondary to increased blood flow during proliferation.

May produce mass effect, e.g depression of the outer calvaria, shift of nasal skeleton, or secondary enlargement of orbit.

Vascular malformation

Low flow vascular malformations are frequently associated with diffuse skeletal hypertrophy, distortion or elongation.

High flow arteriovenous malformations often cause destructive intraosseous changes.

Associated syndromes:

Rendu-osler-weber syndrome Sturge-weber syndrome Kasabach-merritt syndrome Maffucci syndrome Klippel-Trenaunay –Weber syndrome

HEREDITARY HEMORRHAGIC TELANGIECTASIA (Rendu Osler Weber disease)

Congenital hereditary disease Numerous telangiectatic or angiomatous

areas. Triad of telangiectasia, recurrent epistaxis, and a positive family history.

• Hemolytic anaemia ,• thrombocytopenia • coagulopathy.

KASABACK – MERRITT SYNDROME

(Sturge-Weber syndrome) encephalotrigeminal angiomatosis

It consists of congenital  Hamartomatous Malformations that may affect the eye, the skin, and the central nervous  system at different times.

Klippel-Trenaunay –Weber syndromeTriad of cappilary malformations, bone hypertrophy and venous malformations.

DIAGNOSIS History Clinical examination MRI Doppler Ultrasound CT Arteriography

Diagnostic approach by Waner & Suen

PRESENT AT BIRTH

RAPID PROLIFERATION

INVOLUTION PRESENT IN ADULT

+ ++ +

- ---

VM

VM VM VMH

HHH

IMAGING TECHNIQUE

NON-INVASIVEINVASIVE

FUNCTIONAL

MORPHOFUNCTIONAL IMAGING

Pressure measurement

Volume measurement

Continuous Wave Doplar Plathysmographic Device Evaluate venous

disease

Duplex scanning

• Segmental examination of vasculature• Blood flow alteration measured

Plain film

Ultra Sound CT MRI Only for secondary

effect on soft tissue

Demonstration of phleboliths

Duplex CW doplar Color doplar Doplar spectral

analysis

Phleboliths demonstration

Evaluate bone overgrowth

T1 : with gadolinum

T2 : evaluate extent of abnormality

Invasive

Phlebography Angiography Lymphography

Arteriography Venography Direct intralesional Contrast

Mainly for lower extrimities

Demonstrate deep vein, muscular vein, collecting vein Evaluate high flow VA

Demonstrate direct and clear view of afferent/feeding vessels

No diagnostic value for low-flow lesions

MRA TOF (time of flight) PC (Phase Contrast) Contrast enhanced imaging

MRV MRL

Contrast is less toxic than normal contrast of MRI

Magnetic resonance images (MRI) may differentiate low-flow from high flow lesions. The presence of fatty deposits, venous lakes, phleboliths in the MRI are all indicative of low- flow lesions.

CT scans document a lesion’s extension into the surrounding soft tissue.

Doppler imaging can also distinguish high flow lesion from low flow lesions .

If the lesion involves bone, then a “soap bubble” or a “honeycomb appearance” is the usual radiographic finding.

Contrast enhanced MRI and computed angiography are the

commonly used modality for evaluating vascular lesions

Coronal MRI showing the extent of the high flow lesion in temporalis muscle

Axial MRI shows arterio-venous malformation as lobulated, high-signal-intensity mass (arrows)

Axial CT mandible shows arterio-venous malformation showing thinning of cortical plates

DOPPLER ULTRASOUNDDOPPLER EFFECT The variation of the apparent frequency of sound waves, with

change in distance between the source and the receiver.

DOPPLER ULTRASOUND is an adaptation of ultrasound technology. Depends on the frequency shift of ultrasound reflected from moving red cells being proportional to the velocity of flow.

The ‘chaotic’ or turbulent flow results from the area being filled with numerous individual velocity vectors randomly occurring in all directions.

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.

The catheter is advanced from the femoral artery to one of four arteries in the neck that lead to lesion and tissues.

While viewing an x-ray monitor, called a fluoroscope, the doctor steers the catheter through the blood vessels.

80-90 ml of Contrast is injected into the bloodstream to make the blood vessels visible on the monitor. The result is a kind of roadmap of the arteries.

The X-ray images taken may either be still images, displayed on a image intensifier or film, or motion images.

The images are usually taken using a technique called digital subtraction angiography (DSA).

This technique "subtracts" the bones and other organs so only the vessels filled with contrast agent can be seen.

Dyes used are – Iohexol (Omnipaque 350) Iopromide (Ultravist 370) Iodixanol (Visipaque 320) Diatrizoate (Hypaque 50) Metrizoate (Isopaque 370) Ioxaglate(Hexabrix)

Digital subtraction carotid angiogram showing an arteriovenous malformation of the tongue involving the lingual artery.

Arteriovenous malformation of the tongue

Hemangiomas could be distinguished from vascular malformations by the presence of a well circumscribed mass demonstrating intense tissue staining, usually organized in a lobular pattern

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

OLD FASHION TREATMENT FOR IH

1. LIGATION AND EXCISION In 1714, Turner favored surgical resection, ligation and caustics for

vascular birthmarks

During 19th century, surgeons devised ingenious methods of interrupting the vascular supply to a hemangioma using figure-of-eight, spiral or inter-locking subcutaneous sutures of catgut,wire or silk.

Untill the natural involution of hemangiomas was fully appreciated in 20th century, surgical excision continued to be a primary mode of therapy ( Davis and Wilgis,1934; Matthews,1954; Modlin 1955).

2. ARTIFICIAL ULCERATION The old observation that a hemangioma that ulcerates goes on to heal,

leaving skin of pale color, suggested that artificially induced ulceration would work as well.

A variety of astringents and caustics have been applied to superficial hemangiomas – potash and lime, fuming nitric acid, liquid arsenical, croton oil (Gross, 1859; Kingston,1862; Blair,1884)

Efforts to freeze hemangiomas became popular early in 19th century (Pusey,1907; Bunch 1911)

Carbon dioxide slush or solid CO2 crayon techniques were once commonly employed ( Semon, 1934; MacCollum, 1935)

3. ELECTROLYSIS AND THERMOCAUTERY A hot wire of silver or platinium were placed on the hemangioma, or

needles were inserted subcutaneously prior to activation of a number of batteries to adjust the voltage (Knott,1875; Coombs,1881).

Endothermy Coagulation - Modern thermocautery units, with a needlepoint attachment, were used to puncture deep hemangiomas or to cause surface coagulation (MacCollum, 1935). This modality is the antecedent of today’s sophisticated laser technology.

4. SCLEROSANT THERAPY Injection of “stimulating solutions” for treatment of hemangiomas had its

shadowy beginnings in 19th century : Ergot (Hammond,1876), Tannic acid, Carbonic acid (Bradley, 1876), Iron perchloride and 95% alcohol ( Holgate, 1889)

In 20th century, sclerosant therapy continued with 5% sodium morrhuate( Watson & McCarthy,1940) Quinine hydrochloride, hypertonic saline(Andrews & Kelly, 1932), Ethamolin (Mathews,1954) and Sodium Tetradecyl sulfate ( Walsh and Tompkins,1956)

5. RADIATION Radiation for hemangiomas was remarkably successful in 1930 to 1950 era.

Several modalities were used : Thorium-X varnish, Interstitial gamma irradiation and external beam radiation.

Difficult to correct the late skin changes – atrophy, contracture, pigmentation.

The advent of the steroid therapy now limits the need for radiation therapy.

6. COMPRESSION Compression therapy can be traced to the early 19th century.

Pressure also was advocated by Forster (1860); for an infant with a scalp hemangioma, he used a lead plate, Plaster of Paris and Elastic bands applied for 6-8 weeks.

There are contemporary reports of success from use of compressive elastic garments for hemangiomas of extremities (Moore 1964).

Difficult to document the efficacy of any proposed remedy.

CURRENT MANAGEMENT

Description of spontaneous involution of hemangioma can be found scattered throughout 19th century medical literature.

Lister (1938) published his prospective study, in which he observed hemangioma in 77 children and concluded: “No exception has been found to rule out that naevi which grow rapidly during the early months of life subsequently retrogress and disappear of their own accord, on the average about 5th year of life”.

Photographs and measurements should be taken during the initial visit to document the subsequent changes.

Monitor the growth and reassure the parents.

By 6-8 months of age, when growth begins to plateau and early signs of regression are seen and compared with earlier measurements and pictures.

2. STEROID THERAPY Serendipitous discovery, when a large facial hemangioma began to shrink

coincidently with steroid administration for thrombocytopenia.

Subsequently investigators confirmed that Prednislone may hasten the onset and involution of hemangioma (Fost & Esterly,1968; Brown,Neerhout & Fonkalsrud,1972).

The response is reported to be in range of 30-90% (Edgerton 1976). Effect of corticosteroid

Induction of apoptosis Inhibition of angiogenesis

Recommended dosage Prednisone/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

3. 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

4. INTERFERON THERAPY Giant hemangiomas that have been unresponsive to corticosteroid therapy

have been successfully treated using interferon therapy. Interferon therapy, which inhibits angiogenesis, could be considered for

life-threatening hemangiomas due to its high success rate, although it is expensive, burdensome, and possibly toxic. 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

5. Cyclophosphamide• Alkylating agent – used for VM treatment – reviewed by Hurvitz et

al• Side effect

• Nausea• Vomiting• Reversible alopecia

6. 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

7. LASER THERAPY

Apfelberg (1981) & Hobby(1983) 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. The absorbed light energy is transformed into heat, causing thrombosis or destruction of the vascular channels and perivascular tissue.

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 donot penetrate deeper than 1.5mm into the skin.

Laser is useful in treating capillary dermal malformations

8. 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 untill 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.

Indication of surgical excision Rapidly growing with expectance of relevant fibrofatty residuum 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

Technique Lenticular shaped incision and liner closure technique

Traditionally used for removing skin masses Lesion >3cm diameter at base Incision line oriented with the axis of relaxed tension line To minimize bleeding – hemostatic squeezing at tumor base employed

Round-block technique Described by Mulliken Indicated for smaller lesion <30 mm Purse-string closure technique for removing localized Hm are due to

minimization of the subsequent scar – 50% lesser than traditional incision

Sclerotherapy

venous malformations have been treated with irradiation, electrocoagulation (Figi,1948), Freezing techniques (Goldwyn & Rosoff, 1969; Jarzab, 1975), Intravascular Magnetic needles (Martin & Papp,1981) and Sclerosants : Boiling water (Wyeth,1903), Alcohol, Sodium Morrhuate, Quinine, Silver Nitrate and Iron or Zinc Chloride(Boman,1940)

A more appealing stratagem is direct injection of a Sclerosing solution into the epicentre of the venous anomaly during occlusion of arterial inflow and venous outflow.

A liquid vegetable protein, Ethibloc has been used extensively; particularly effective in obliterating AV and pure venous malformations.

Other agents are – Sodium tetradecyl sufate, ethanol, hypertonic saline,

Surgical resection Total excision is the definitive treatment for a venous malformation. Resection is indicated to reduce bulk and improve contour and function.

VENOUS MALFOMATIONS

LYMPHATIC MALFORMATIONS Several agents have been utilized for lymphatic malformations including

ethanol, bleomycin, OK-432, and doxycycline.

Several authors say LMs donot respond well to sclerosing agents, pressure therapy, almost all are either tolerated well by the patient or treated surgically.

Infection should be treated aggressively with antibiotics and the mainstay of therapy, when indicated is Surgical Excision. Cold knife dissection is frequently the modality of choice A well-localized cystic lymphatic anomaly can be dissected from surrounding tissue.

A lymphatic anomaly is not neoplastic. But it invades adjacent tissue,such as muscle of lip or tongue. Surgery risks deformity or functional loss, nerve injury. It is not uncommon to excise a LM in 2/3 stages.

Radio frequency ablation: An attractive method for treating LM It destroys tissue at low temperature (40* - 70*C) Minimal damage to adjacent tissue

ARTERIOVENOUS MALFORMATIONSEMBOLIZATION Also known as Embolotherapy or Endovascular therapy. This procedure involves the injection of glue or other non-reactive liquid adhesive material into the AVM in order to block it

off. For this purpose, a small catheter is passed through a groin vessel

all the way up into the blood vessels supplying the AVM.

Access is gained through a retrograde femoral approach. Digital subtraction is used and the catheter is guided by fluoroscopy.

Embolization materials used are ethanol, Gelform, Steel coils and wisps of cotton, polyvinyl alcohol, and isobutyl cyanoacrylate. In some cases, occlusion of the proximal vasculature or tortuosity of feeding vessel results in failure of conventional embolization technique.

Percutaneous embolization has been described using a 20-guaze Seldinger needle inserted directly into the lesion through the skin and thinned bone.

Not all AVMs can be treated with embolization. AVMs are carefully studied at the time of a preliminary angiogram by highly skilled interventional radiologists to determine if catheters can be passed up into the AVM without any complications before they are considered for embolization.

COMPLICATIONS:Arterial spasm, vessel rupture, necrosis, inadvertant embolization

of internal carotid artery, production of pulmonary emboli due to escape of material through the lesion.

The goals of surgery are to completely remove the lesion while maintaining control of hemorrhage, and to reconstruct the defect to functional and aesthetic level.

An extraoral incision is preferable when the lesion extends proximally into the angle or ramus; a transoral approach does not allow good visibility and rapid control of haemorrhage.

Smaller lesions can be unroofed and packed as removal is carried out.

RESECTION with immediate replantation( EXTRACORPOREAL APPROACH) Resection of the mandible containing the lesion, and extracorporeal curretage and extarction of

teeth An osteotomy can be made distal to the lesion, and the involved segment can then be rotated

laterally to allow direct visualization of lingual surface of mandible. The resected mandible can be modified to form an autologous tray for the bone graft.

The hollowed mandible is packed with cancellous bone. The reimplanted mandible and bone grafts are stabilized with plates.

IMF is placed to assist in maintaining proper jaw position and for immobilization during healing

SURGERY

OPG Showing moth eaten radiolocency (AVM) in left mandibular parasymphysis region

Resected mandibular segment containing focal lesion

Extracorporeal removal of teeth and curretage of lesion Reimplantation of mandibular

segmenr and stabilization with mini plates.

History and examination

Vascular malformation

Hemangioma

MRI. Doppler US,Arteriogram

High flow Low flow

Embolize

Ablative surgery

Observe

Excision , Laser, Sclerosing agents

Observe

Proliferation phase

Life threatening or visual disturbance

Steroids

Control

Observe

Fail to control

Interferon

Observe

Parent education

Observe

Involution complete

No residual lesion

Residual lesion

Excision , Laser, Sclerosing agents

Observe Observe

YES NO

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