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

Content

Case conference : Example

Interactive topics- Vascular malformation- Hemangioma

Wrap-up

Case conferenceMr. Yotdanai Namuangchan

Mr. Patinya Yutchawit

Patient identification

ผูป่้วยเดก็ชาย อาย ุ2 ปี ภูมิล าเนา อ.บา้นฝาง จ.ขอนแก่น

Chief complaint

มีกอ้นท่ีมือซา้ย ตั้งแต่แรกเกิด

Present illness

แรกเกิด พบกอ้นท่ีมือซา้ย บริเวณน้ิวกลาง กอ้นน่ิม รูปร่างเป็นปลอ้ง ขนาดประมาณ 1cm ไม่เจบ็ ไม่มีแผล ผวิเรียบ พอขยบัน้ิวได ้กอ้นโตข้ึนเร่ือยๆปัจจุบนั (อาย ุ2 ปี) กอ้นโตข้ึนตามตวั ขนาดประมาณ 7cm สีม่วงแดง ขยบัน้ิวล าบาก ลกัษณะกอ้นเป็นปลอ้งๆตามน้ิวกลางและโคนน้ิวมือซา้ย

Past history

ไม่มีโรคประจ าตวัไม่มีประวติัแพย้า แพอ้าหารG1P1, Normal labor, Term

Social and personal history

ไม่มีประวติัใชย้าใดๆเป็นประจ าฉีดวคัซีนครบตามเกณฑ์

Family historyไม่มีโรคในครอบครัว

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

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

Differential Diagnosis

Investigation

Treatment

Mini interactive lecture- Vascular malformation

- Hemangioma

Vascular malformationMiss Withunda Akaapimand

Miss Rattanaporn Sirirattanakul

Miss Tritraporn SawantranonMr. Jirarot Wongwijitsook

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

Vascular malformations Classification

Capillary

Venous

Lymphatic

Artery

Combined

Slow flow

Fast flow

Vascular Malformations

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.

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

Venous malformation

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.

Thin walled, dilated, sponge-like channels of variable size and mural thickness

normal-appearing endothelial lining but abnormal smooth muscle architecture

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

• Pain : secondary to thrombosis of the malformation

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

Management

Indication : appearance, functional,pain

Conservative therapy: elastic compression +baby aspirin (prophylaxis painful thromboses)

Sclerotherapy : main treatment

Laser (Nd:YAG)

Resection

Lymphatic malformation

Lymphatic Malformations

Embryogenic disturbance of lymphatic system

At birth -2 yrs

No involution

Microcystic malformation (lymphagiomas)

Macrocystic (cystic hygroma)

Combined

Rich lymphatic area : H&NAxillaMediastinumgroin retroperitoneum

The overlying skin is usually normal and has a bluish hue

Lymphangioma circumscriptum : superficial cutaneous-subcutaneous lymphatic anomaly > vesicle

Facial soft tissue distortionBony hypertrophy

Macroglossia

Mandible overrowth

Proptosis

ManagementResection : main treatment ,operative goal is complete resectionwith 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

Capillary malformation

Capillary Malformations(CMs)

Port-wine stain

Equal gender distributionBirth prevalence is 0.3%

Clinical presentationAt birth

Well-circumscribed red macular lesions

Commonly occur on the face in the trigerminaldistribution

• 45% restricted to 1/3 trigeminal dermatomes

• 55% overlap dermatomes, cross the midline, bilaterally

CM in a limb often

is associated with

axial and

transverse

hypertrophy.

Sturge-Weber syndrome

Facial capillary malformaion

Ipsilateral occular and leptomeningeal vascular anomalies

Neurologic : seizures , hemiparesis , delayed motor and cognitive development

Ophthalmologic : glaucoma

MANAGEMENT

flashlamp pulsed-dye laser

: the results are better if initiated in infancy and childhood.

Laser treatment

Arteriovenousmalformation

Arteriovenous Malformation

Errors of embryogenic vascular development

Failure of arteriovenous channels in the primitive retiformplexus to regress

Arteriovenous Malformation

• AVM is a high-flow vascular malformationcomprised of micro- and macro-aneriovenousfistulas (AVFs).

•Arterial and venous vessels connected directlyto one another, without an intervening capillarybed

•The epicenter of an AVM is called the nidus andconsists of arterial feeders

Arteriovenous Malformation

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

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

The cutaneous stain > erythematous and develops local warmth, a palpable thrill, and a bruit

Schobinger's staging systemClinacal staging system for documenting the presentation and evolution of an AVM

Investigation• Color Doppler evaluation and ultrasonography

are useful first-line tools to determine flow characteristics.

• MRIdefines the anatomy and extent of the lesion

• Angiography is useful in further characterizing the lesion andallows therapeutic embolization

AVM Angiography

• AVMs are usually treated when there are endangeringsigns and symptoms) such as ischemic pain, recalcitrantulceration) 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 nidusfollowed by resection 24 to 48 hours alter embolization

Management

HemangiomaMr. Patinya Yutchawit

Miss Kaewalin Thongsawangjang

VASCULAR TUMORS

Vascular tumors

Endothelial neoplasms

Increased endothelial turnover

• Hemangioma is the most common among

vascular tumors – originates from

vasculogenesis (Angiogenesis)

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

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:

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

Superficial Deep

Craniofacial (60%)

Trunk (25%)

Ext. (15%)

Single(80%)

Multiple(20%)

** visceral organ

involvement

Disseminated hemangiomatosis

Proliferating phase

(O-1yr)

Involuting phase

(1-5yr)

Involuted phase

(>5yr)

telltale signs crepe-like laxity

(anetoderma)

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)

Congenital hemangiomas : develop during prenatal life fully developed at birth

RICH

(Rapid involuting CH)

NICH

(Non Involuting CH)

Complications

Ulceration ,Bleeding,Infection

Dressing

Topical ATB

Visual – Ambryopia

Airway obstruction - subglottic hemangioma

Congestive heart failure

RADIOLOGIC FEATURES

Ultrasonography

Hallmarks : localized, dense parenchyma and fast flow

MRl

Nuclear Scanning

Tc 99m- tagged red blood cells

Deep multiple hemangiomas in the GI or CNS

Management

Observe

When to intervene?

Management

Indication for therapeutic intervention

Compromised airway

Deviation of visual axis

Oral / digestive tract obstruction

Bilateral auditory obstruction

Management

Decision for managementLesion size and location

Complication

Age

Phase of growth

Management

Pharmacotherapy

Chemotherapy

Laser therapy

Surgical excision

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

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)

Chemotherapy

vincristine and cyclophosphamide

effective for life-threatening vascular tumors, including hemangioma.

no role for cutaneous hemangiomas

intrahepatic hemangiomas is debatable

Others treatmentEmbolization

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)

Operative Management

INFANCY (PROLIFERATIVE PHASE)

Obstruction ( visual or subglottic )

Deformation (periorbital distortion with secondary astigmatic amblyopia)

Bleeding or ulceration (well-localized or pedunculatedlesions)

unresponsive to topical or systemic therapy

constant pain

scalp and thoracic skin

Operative Management

EARLY CHILDHOOD (INVOLUTING PHASE)

Preschool period

Indicated if it is obvious

post ulceration scarring, expanded and in elastic skin, and fibrofatty residuum

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

Reference

Grabb and Smith's Plastic Surgery Grabb's Plastic Surgery

BMJ Best practice

Medscape

Wrap-upVascular anomalies

Present by Mr.PatinyaYutchawit

"It does not matter how slowly you go as long as you do not stop.“

Confucius

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?

Key difference

Key difference

Hemangioma Vascular malformation

Definition True vascular tumor Vascular disorganization

Age At 1st month At birth (Congenital)

Growth pattern

HemangiomaTumor characteristic , Investigation

and proper treatment

TumorCharacteristic

Thickness : Macule to papule

Demarcation : Well demarcation

Compressibility : moderate to poor

No warmth, no tenderness

No vascular signs : No bruit, no thrill ***

Color :

Investigation

MRI ****** Gold standard

Doppler U/S

Arteriogram (Not use -- ?)

********** Clinical Dx *************

Proper Treatment

Observe and parent education

Steroid injection

Surgery

Vascular malformationCategorization—Arterial/venous/capillary/lymphatic,

each characteristic ,investigation and treatment

Categorization

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/BruitBleeding

Gravity dependent

Port-wineSalmon egg (face)

Cystic hygromaPeau d'orangeInfection ifcombined with vein

Investigation Arteriogram Doppler U/S MRI U/S

Treatment Embolization Sclerosingagents

Argon laser Sclerosing agents (Bleomycin)

Special thanks!Dr.Poj

Our beloved resident