ANOMALI VASKULAR

BACKGROUND
• Vascular tumor: endothelial neoplasms characterized by increased endothelial turnover
• Vascular malformation: abnormal development of vascular elements during
embryogenesis
• Designated according to the predominant type
• Arterial component  fast flow, remainder  slow flow
• International Society for Vascular Anomalies in 1996
 PATHOGENESIS

Vascular tumor Vascular malformation

• Hiperplasia sel endotel • Defek pertumbuhan dan remodelling
• pertumbuhan yang cepat postnatal dan pembuluh darah
regresi perlahan saat masa kanak- • Tidak mengalami regresi
kanak
 VASCULAR ANOMALIES CLASSIFICATION

Neligan. Vascular anomalies.2013

VASCULAR TUMORS
 VASCULAR ANOMALIES CLASSIFICATION

Neligan. Vascular anomalies.2013

INFANTILE HEMANGIOMA
 PATHOGENESIS & PHASES OF INFANTILE
HEMANGIOMA

Proliferating Phase Involuted Phase

Involuting Phase
0-1 year > 4 year
1-4 years
Clusters of plump endothelial Replaced by loose adipocytes +
Mature blood vessels formed. ↑ connective tissue
cells, with small vascular
ECM, multilaminated basement
channels, minimal connective 5 year- 50 %
membranes
tissue 7 year – 70 %
 CLINICAL FEATURES
• Occurs in 4-5% of Caucasian infants
• More frequent in premature children and females (4:1)
• Typically single (80%), involve head and neck (60%), trunk (25%), extremity (15%)
• Median age of appearance: 2 weeks; 30-50% noted at birth as telangiectatic stain, pale
spot, ecchymotic area
• Red when involve the superficial dermis; lesion beneath skin may not be seen until 3-4
months (overlying skin may appear bluish)
• After involution: ½ of children will have residual telangiectasias, scarring, fibrofatty
residuum, redundant skin, destroyed anatomic structures
 CLINICAL FEATURES

• Head & neck hemangiomas : majority small, harmless lesions. Minority : significant
deformity/complications (eyelid, ear, nose, lip).
• Of scalp/eyebrow  alopecia
• Periorbital  block visual axis/distort cornea
• Subglottic  obstruct airway

• Multiple hemangiomas : 20% of infants, more risk of IH of internal organs

• Hepatic hemangiomas : Focal, multifocal, or diffuse
• Most non-problematic and discovered incidentally, but large tumors can cause heart failure,
hepatomegaly, anemia or hypothyroidism
 HEMANGIOMAS & STRUCTURAL ANOMALIES

• w/ malformations in the head/neck or lumbosacral regions

• PHACE : 8% of children have a stroke in infancy
• P – posterior fossa brain malformation LUMBAR
• L – Lower body infantile hemangioma
• H - hemangioma • U – Urogenital anomalies
• M – Myelopathy
• A – Arterial cerebrovascular anomalies • B – Bony deformity
• A – Anorectal malformations
• C – coarctation of the aorta and cardiac defects • R – Renal anomalies
• E – Eye/endocrine abnormalities
• S – Sternal cletfting/ supraumbilical raphe
 DIAGNOSIS

• History & Physical exam

• IH : soft-tissue mass with fast-flow, decreased arterial resistance, increased venous
drainage
• Fast-flow : using hand held doppler
• GLUT 1 + differentiates from other tumors/ malformations
 COMPLICATION

• Ulceration, pain, infection, hemorrhage (at rapid periode)

• Hemangioma of eyelid  astigmatism, strabismus, ambliopia
• Hemangioma of pinna  external ear deformity & hearing loss
• Airway Hemangioma/ Subglotic hemangioma
 NONOPERATIVE MANAGEMENT

• Observation: most  small, localized, do not involve anatomically important areas

• Observed closely monthly during proliferative phase if potential to destroy
• Wound care (ulceration) – moist & topical antibiotic/ petroleum gauze barrier, topical
lidocaine
• Topical corticosteroid for superficial hemangioma ineffective
 THERAPY
• Intralesional corticosteroid
• Small, well-localiezed (<3 cm in diameter) that obstruct visual axis/nasal airway/risk damage
structures
• Triamsinolon (not exceed 3 mg/kg) lasts 2-3 weeks, infants may require 2-3 injections during
proliferative phase

• Systemic pharmacotherapy
• Problematic IH > 3-4 cm : require >3 mg/kg triamcinolone for injection
• Oral prednisolone 3 mg/kg/day for one month, tapered 0.5 cc every 2-4 weeks until
discontinue (10-12 months)
• Propanolol therapy dose 2 mg/kg/day, inpatient recommended for premature <3 mo
 THERAPY
• Interferon alfa: kombinasi dari IFN alfa 2a dan 2b. Baru digunakan apabila terapi
kortikosteroid gagal. Kortikosteroid dan IFN tidak boleh digabung. Efek samping
terburuk yaitu spastic diplegia, yang menghilang dengan berhentinya pemberian IFN
• Vincristine
• Pulsed dye laser (PDL): efektif menangani lesi yang superfisal dan datar, karena daya
penetrasi tidak terlalu kuat
 TREATMENT RESPONSE

• Usually evident within 1 week of therapy

• Signs of involution:
• Decreased growth rate
• Fading color
• Softening of lesion
 OPERATIVE MANAGEMENT

• Proliferative phase
• Not recommended during early growth, highly vascular
• Indications: (1) failure /contraindication to pharmacotherapy; (2) well-localized tumor in
anatomically favorable; (3) if future resection necessary, scar will be the same

• Involuted Phase
• 50% leave behind fibrofatty tissue/ damaged skin
• Optimal during 3-4 years ; lesion wont improve much, before self esteem/ long-term memory
formed
CONGENITAL HEMANGIOMAS
 CONGENITAL HEMANGIOMA

• Tampilan klinis dan radiologis sama seperti IH, tapi tanpa GLUT-1
• Sudah muncul sepenuhnya saat lahir
• RICH (Rapid Involuting Congenital Hemangioma)
• Involutes rapidly after birth (50% regress completely by mo 7); remaining by 14 mo
• Head or neck (42%), limbs (52%), trunk (6%)
• Doesn’t leave behind a significant adipose component

• NICH (Noninvoluting Congenital Hemangiom)

• Doesn’t regress, remains unchange persistent fast flow
• Head & neck (43%), limbs (38%), trunk (19%)
 MANAGEMENT OF CONGENITAL
HEMANGIOMA
• RICH  do not require resection
• After regression: leave atrophic scar & subcutaneous tissue

• NICH is observed until diagnosis is clear  resection recommended if improve

appearance
VASCULAR MALFORMATIONS
CAPILLARY MALFORMATION
 CAPILLARY MALFORMATION

Pathogenesis
• “portwine” stain
• Geographic pattern often regional/dermatomal
• Mutation in GNAQ
 CAPILLARY MALFORMATION

Clinical
• Appear anywhere on body; localized/extensive
• Cutaneous discoloration usually evident at birth, pink color may darken, skin thicken, sometimes
with raised fibrovascular cobblestoning
• Pyogenic granuloma may develop in CM = ulceration & bleeding
• Can soft-tissue & skeletal overgrowth below stain = hypertrophy of lip, cheek, forehead
• Grow proportionately and enlarge during puberty
 CAPILLARY MALFORMATION

Management
• Pulse-dye laser therapy by lightening the color
• Outcome better for smaller lesions & treated younger age
• CM often re-darken over time
• Malocclusion correction if orthodontics are affected
LYMPHATIC MALFORMATION
 LYMPHATIC MALFORMATION

Pathogenesis
• Sacs or lymphatic channels become ‘pinched off’ from main lymphatic system = aberrant collections of
lymphatic fluid-filled spaces
• Somatic mutation in PIK3CA

Clinical
• Commonly @ head/neck, or axilla, chest, perineum
• Soft & compressible
• Overlying skin may be normal, bluish hue, studded with pink-red vesicles
• Common complications: bleeding & infection
Microcystic lymphatic malformations (<1 cm)

Treatment: Observation, treatment symptomatic

Prognosis: Curative treatment rare and recurrence common

Macrocystic lymphatic malformations (>1 cm)

Treatment: Sclerotherapy (bleomycin, ethanol, STS and doxycycline).

Prognosis: varies with lesion characteristics and sclerosing agent used.

Typically good response, but rarely achieve complete resolution

Combined

With septations of variable thickness

 RESECTION

• May cause significant morbidity (blood loss,

iatrogenic injury, deformity)
• For:
1. Symptomatic microcystic LM causing bleeding,
infection, distortion of vital structures, significant
deformity
2. Symptomatic mcrocystic/combined LM that can’t be
managed by sclerotherapy
3. Small, well-localized
VENOUS MALFORMATION
 PATHOGENESIS

• Composed of thin-walled, dilated, sponge-like channels of variable size & mural

thickness
• Normal endothelial lining (smooth muscle abnormal  cells in clumps, not
concentric)
• Intralesional clotting; simple fibrin deposition, to calcified ‘phlebolith’
• Mutation in TIE2
VENOUS MALFORMATION

Clinical
• > 5 cm, single, @head & neck
• Soft, compressible, blue subcutaneous mass, phleboliths can be palpated, may enlarge with physical
activity
• Complications: pain, swelling, psychosocial issues
• Grow proportionately and enlarge during puberty
• In contrast to LM: VM enhances with contrast, often show phlebolith (tiny calcifications (masses of
calcium) located within a vein), more likely involve muscle
 MANAGEMENT

- Custom fitted compression garments to reduce stagnation, phlebolith & pain

- Daily aspirin to prevent thrombosis
- LMWH for patients at risk of DIC

Sclerotherapy
- Symptomatic (pain, deformity, obstruction, gastrointestinal bleeding)
- Safer than resection
- Sodium tetradecyl sulfate & ethanol

Resection
- Considered for : (1) small, well-localized that can be completely removed (2) persistent mass after
sclerotherapy
VENOUS MALFORMATION TREATMENT
ARTERIOVENOUS MALFORMATION
 PATHOGENESIS

• Absent capillary bed = shunting directly from

arterial to venous through fistula/nidus
• Enlarge from collateralization, dilation of vessels,
thickening of adjacent arteries & veins, aneurysms
• Nidus : epicenter of an AVM, consists of arterial
feeders, micro- and macroarteriovenous fistulas, and
ectatic veins
 ARTERIOVENOUS MALFORMATION (AVM)
Clinical :
- Head & neck
- Early often mistaken with CM / IH
- Risk of pain, ulceration, bleeding
- Warm pink-bluish lesions with a pulsatile thrill, can progress to dystrophic skin changes,
bleeding, ulceration and necrosis.
- Associated cardiac enlargement & heart failure in severe cases
Imaging: USG & MRI: confirm diagnosis, determine extent of lesion, plan treatment. Angiography if
diagnosis is still unclear. CT if AVM involve bone
 MANAGEMENT

• Not cure, but control

• Alleviating symptoms, preserving vital functions
• Embolization, resection, or combination
• Intervention
1. Size & location of AVM
2. Age of patient
3. Schobinger stage
 EMBOLIZATION

• Delivery of inert substance through catheter into AVM nidus

• Scarring reduce shunting, shrink lesion, diminish symptoms
• Preoperative to resection/monotherapy
• Goal to nidus and proximal venous outflow, not arterial feeding vessels
USG Doppler, MRI, CT angiography
Treatment : Primary arterial embolization / superselective arterial embolization
+ resection 24-28 hours
 TERIMAKASIH

• Sumber :
• Grab and Smith 7th ed. 2014
• Neligan. Vascular anomalies. 2013