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Vascular anomaly topic for medical student
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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
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