Soft tissue hemangiomas are benign vascular tumours composed of proliferating blood vessels - the most common benign soft tissue tumour of infancy/childhood. Phleboliths (round calcifications with lucent centres) are pathognomonic and visible on plain radiographs in 50% of cases. MRI shows characteristic hyperintense T2 signal with flow voids and enhancement. Management is typically observation for asymptomatic lesions (infantile type: 50% involute by age 5); surgical excision for symptomatic, recurrently bleeding, or functionally impairing lesions. Key trap: incomplete excision results in 40% recurrence rate.

Histologic Subtypes and Characteristics

Soft tissue hemangiomas represent a spectrum of benign vascular proliferations with distinct histologic patterns that correlate with clinical behavior and imaging characteristics.

Capillary Hemangioma:

• Composed of small, capillary-sized vessels
• Prominent endothelial cells with minimal atypia
• Dense cellularity with little intervening stroma
• Most common type in subcutaneous tissue
• Lobular architecture with feeding vessels
• Usually presents in infants and young children
• High proliferative index in infantile variant
• Associated with GLUT-1 positivity in infantile type

Cavernous Hemangioma:

• Large, dilated, blood-filled vascular spaces
• Thin-walled vessels lined by flattened endothelium
• More common in deep soft tissue and muscle
• Significant thrombosis and phlebolith formation
• Slow blood flow with stasis phenomena
• Accounts for most intramuscular hemangiomas
• May cause local mass effect and compression
• Lower proliferative rate than capillary type

Venous Hemangioma:

• Thick-walled veins with smooth muscle
• Most common type within skeletal muscle (60% of intramuscular)
• Often infiltrates between muscle fibers
• Associated with pain due to venous distension
• Phleboliths present in up to 50% of cases
• Calcifications visible on radiographs
• May cause compartment syndrome-like symptoms
• Difficult to completely excise due to infiltrative growth

Arteriovenous Hemangioma:

• Direct arteriovenous communications without capillary bed
• High-flow lesion with prominent arterial feeders
• Pulsatile mass with audible bruit or palpable thrill
• Risk of bleeding with biopsy or surgery
• May cause distal ischemia via steal phenomenon
• Often requires preoperative embolization
• Higher recurrence rate after incomplete excision
• Can enlarge with hormonal changes or trauma

Molecular and Cellular Mechanisms

Endothelial Proliferation Pathways:

• VEGF (vascular endothelial growth factor) upregulation drives angiogenesis
• FGF-2 (fibroblast growth factor) promotes endothelial cell proliferation
• Angiopoietin-TIE2 system regulates vessel maturation
• GLUT-1 expression distinguishes infantile from congenital hemangiomas
• Hypoxia-inducible factor (HIF) activation in proliferating lesions
• Loss of apoptotic signals allows persistent endothelial growth
• Mast cell infiltration contributes to vascular remodeling
• Pericyte recruitment and smooth muscle differentiation in maturing lesions

Natural History Phases (Infantile Type):

Proliferative Phase (0-12 months):

• Rapid endothelial proliferation with high GLUT-1 expression
• Increased mitotic activity and cellular density
• VEGF and FGF-2 levels peak during this period
• Tumor typically doubles in size during first 5 months
• Minimal apoptosis with net accumulation of vessels
• Bright red appearance due to high blood flow
• May cause functional impairment if near critical structures

Plateau Phase (12-18 months):

• Balance between proliferation and apoptosis
• Stable size with decreased growth velocity
• Beginning of endothelial maturation processes
• Color change from bright red to purple
• Decreased GLUT-1 and proliferation markers
• Structural reorganization of vascular channels
• Opportunity for observation before involution

Involutionary Phase (1-7 years):

• Apoptosis exceeds proliferation with net vessel loss
• Replacement of endothelial cells with fibrofatty tissue
• Progressive decrease in lesion size (50% by age 5)
• Color fades to gray or skin tone
• Decreased vascularity on imaging studies
• May leave residual fibrofatty tissue or skin changes
• Complete involution occurs in 50% by age 5, 70% by age 7

Distribution Patterns

Intramuscular Hemangiomas (75% of deep soft tissue types):

• Lower extremity: 50% (quadriceps, gastrocnemius most common)
• Upper extremity: 25% (deltoid, biceps, forearm flexors)
• Trunk: 15% (paraspinal, abdominal wall muscles)
• Head and neck: 10% (masticatory muscles, tongue)
• Infiltrative growth pattern along fascial planes
• May involve multiple muscle groups in complex cases
• Difficult surgical planes due to muscle infiltration
• High recurrence with incomplete excision

Subcutaneous and Cutaneous (20%):

• More common in head and neck region in children
• Superficial location allows easier clinical diagnosis
• Better defined margins than intramuscular types
• More amenable to complete surgical excision
• May respond to laser therapy if purely cutaneous
• Associated with overlying skin discoloration
• Lower recurrence rate after excision
• Better cosmetic outcomes with modern techniques

Synovial and Periarticular (5%):

• Knee joint most frequently affected (40% of joint hemangiomas)
• Causes recurrent hemarthrosis and joint swelling
• Synovial hypertrophy with hemosiderin deposition
• Progressive joint damage if untreated
• May mimic pigmented villonodular synovitis
• Requires synovectomy for definitive treatment
• Arthroscopic evaluation and treatment possible
• Risk of arthrofibrosis post-treatment

Histologic Classification (Enzinger & Weiss)

Capillary Hemangioma:

Microscopic Features:

• Lobules of closely packed capillaries with narrow lumens
• Prominent plump endothelial cells without significant atypia
• Pericytes present surrounding endothelial tubes
• Minimal intervening stroma between vascular channels
• May have focal mitotic activity in proliferating lesions
• Immunohistochemistry: CD31+, CD34+, factor VIII+
• GLUT-1 positive in infantile variant only
• Ki-67 proliferation index elevated in proliferative phase

Clinical Correlates:

• Most common in subcutaneous tissue and dermis
• Bright red color due to high blood flow
• Soft, compressible mass that blanches with pressure
• May demonstrate rapid growth in infants
• Better defined margins than other subtypes
• More amenable to sclerotherapy or laser treatment
• Lower recurrence rate after complete excision
• 50% of infantile types involute by age 5 years

Cavernous Hemangioma:

Microscopic Features:

• Large, thin-walled vascular spaces filled with blood
• Flattened endothelial lining with minimal cellular atypia
• Fibrous septa separating vascular spaces
• Thrombosis and organization within vascular channels
• Dystrophic calcification forming phleboliths
• Variable smooth muscle in vessel walls
• Hemosiderin deposition from prior hemorrhage
• Immunohistochemistry similar to capillary type

Clinical Correlates:

• Deep soft tissue location, especially muscle
• Bluish discoloration of overlying skin if superficial
• Compressible mass that refills slowly after compression
• Phleboliths visible on radiographs (50% of cases)
• May cause pain from thrombosis or venous distension
• Infiltrative borders make complete excision challenging
• Recurrence rate 20-40% after attempted excision
• Does not involute spontaneously in adults

Arteriovenous Hemangioma:

Microscopic Features:

• Thick-walled arteries with muscular media
• Direct arteriovenous communications (shunts)
• Absence of normal capillary bed intermediaries
• Variable caliber vessels with high flow
• Reactive intimal thickening in feeding arteries
• May have aneurysmal dilatation of draining veins
• Cellular proliferation in growing lesions
• Hemosiderin and thrombus less common than venous type

Clinical Correlates:

• Pulsatile mass with palpable thrill or audible bruit
• Warm skin temperature over lesion due to arterial flow
• May enlarge with increased cardiac output (pregnancy, exercise)
• Risk of high-output cardiac failure in massive lesions
• Bleeding risk with biopsy or incomplete surgery
• Requires preoperative angiography and possible embolization
• Often invades multiple tissue planes
• Highest recurrence rate of all hemangioma types

Venous Hemangioma:

Microscopic Features:

• Thick-walled veins with prominent smooth muscle
• Dilated vascular spaces with slow blood flow
• Frequent thrombosis with organized thrombi
• Phlebolith formation (dystrophic calcification)
• Infiltration between muscle fibers in intramuscular type
• Variable amounts of fibrous stroma
• Hemosiderin deposition throughout lesion
• Immunoreactive for smooth muscle actin in vessel walls

Clinical Correlates:

• Most common type in skeletal muscle (60% of intramuscular)
• Deep location with ill-defined margins
• Pain with activity (venous distension) or positional changes
• Phleboliths on radiographs (pathognomonic finding)
• MRI shows hyperintense T2 signal with flow voids
• Infiltrative growth makes complete excision difficult
• Recurrence in 30-40% after incomplete excision
• May require preoperative embolization if extensive

Epithelioid Hemangioma:

Microscopic Features:

• Plump epithelioid endothelial cells lining vessels
• Well-formed vascular channels with variable lumens
• Inflammatory infiltrate with eosinophils and lymphocytes
• May have solid areas mimicking angiosarcoma
• Low mitotic activity distinguishes from malignancy
• Immunoreactive for endothelial markers (CD31, ERG)
• Negative for cytokeratin (excludes epithelial tumors)
• May have lymphoid aggregates or germinal centers

Clinical Correlates:

• Previously termed "histiocytoid hemangioma"
• Often occurs in head and neck or distal extremities
• May present as multiple lesions
• Associated with trauma or arteriovenous malformation
• Can recur locally after excision
• Very rarely progresses to angiosarcoma
• Important to distinguish from epithelioid angiosarcoma
• Complete excision curative in most cases

Clinical Classification (Mulliken-Glowacki)

Infantile Hemangioma:

• Not present or minimally present at birth
• Rapid proliferation during first year of life
• GLUT-1 positive (distinguishes from congenital type)
• Female predominance (3:1 ratio)
• 50% involute by age 5, 70% by age 7
• May require propranolol for complicated cases
• Indications for treatment: functional impairment, ulceration, disfigurement
• Observation appropriate for uncomplicated lesions

Congenital Hemangioma:

Rapidly Involuting (RICH):

• Fully formed at birth, then rapidly regresses
• Complete involution by 12-14 months of age
• GLUT-1 negative (key distinguishing feature)
• Equal sex distribution
• Usually solitary lesion
• May leave residual skin changes
• Observation appropriate for most cases
• Surgical excision if symptomatic

Non-Involuting (NICH):

• Fully formed at birth, remains stable
• Does not proliferate or involute over time
• GLUT-1 negative like RICH variant
• Persists into adulthood without treatment
• May require surgical excision for symptoms or cosmesis
• Complete excision usually curative
• Lower recurrence than intramuscular types
• Consider excision in childhood for accessible lesions

Presenting Signs and Symptoms

Asymptomatic Mass (40% of cases):

• Incidental finding during examination for other reasons
• Palpable soft tissue fullness or swelling
• No associated pain, tenderness, or functional limitation
• May have been present for months to years
• Slow growth pattern without recent change
• No overlying skin changes in deep lesions
• Patient or parent notices visible lump
• More common in older children and adults

Pain Syndromes (35% of cases):

Intramuscular Venous Hemangioma Pain:

• Dull, aching pain with activity or muscle contraction
• Venous distension and engorgement during exercise
• Positional pain (worse with dependent positioning)
• Relief with rest and elevation
• Night pain if significant venous stasis
• Tenderness to palpation over lesion
• Exacerbated by Valsalva maneuver
• May mimic chronic compartment syndrome

Acute Thrombosis Pain:

• Sudden onset severe pain in previously asymptomatic lesion
• Firm, tender mass with local inflammation
• Overlying skin warmth and erythema possible
• Usually self-limited episode lasting days to weeks
• May have palpable phleboliths after thrombosis resolves
• Risk of recurrent thrombotic episodes
• Distinguish from deep vein thrombosis or infection
• May require imaging to exclude other pathology

Functional Impairment (15% of cases):

• Restricted range of motion in adjacent joint
• Muscle weakness from infiltration or compression
• Neurologic symptoms if compressing adjacent nerve
• Vascular compromise if compressing major vessels
• Compartment syndrome in rapidly enlarging lesions
• Gait abnormality with lower extremity lesions
• Hand dysfunction with upper extremity involvement
• Cosmetic concerns in exposed areas

Bleeding and Hemarthrosis (5% of cases):

• Recurrent hemarthrosis in intra-articular lesions
• Spontaneous bleeding episodes with trauma
• Ecchymosis after minor injury
• Hemarthrosis causes joint swelling and pain
• Progressive joint damage from recurrent bleeding
• Hemosiderin deposition in synovium
• May mimic hemophilia or pigmented villonodular synovitis
• Requires synovectomy for definitive treatment

Consumption Coagulopathy (less than 1%):

• Kasabach-Merritt phenomenon (NOT with true hemangiomas)
• Occurs with kaposiform hemangioendothelioma or tufted angioma
• Thrombocytopenia, hypofibrinogenemia, elevated D-dimer
• Petechiae, purpura, or spontaneous bleeding
• Life-threatening complication requiring urgent treatment
• Differentiate true hemangioma from kaposiform type
• Treatment with corticosteroids, vincristine, or sirolimus
• Surgical excision contraindicated during acute phase

Physical Examination Findings

Inspection:

• Visible swelling or fullness in affected region
• Bluish or purple skin discoloration if superficial
• Dilated superficial veins over lesion (venous drainage)
• Skin temperature normal or slightly warm
• No skin ulceration in uncomplicated cases
• Asymmetry compared to contralateral limb
• Muscle atrophy if chronic compression of muscle
• Scars from previous biopsy or surgery attempts

Palpation:

• Soft, compressible mass that refills after compression
• Non-tender in most cases (except thrombosed lesions)
• Ill-defined margins with infiltrative lesions
• Reducible with elevation and compression (venous type)
• May feel phleboliths as hard nodules within soft mass
• Increased size with dependent positioning
• Valsalva maneuver may increase lesion size
• No associated lymphadenopathy

Special Tests:

Empty-Refill Test:

• Elevate extremity and compress mass to empty venous spaces
• Lower extremity and release compression
• Slow refilling over 15-30 seconds suggests low-flow venous hemangioma
• Rapid refilling suggests high-flow arteriovenous type
• Helps distinguish hemangioma from solid tumor
• Correlates with venographic findings
• Useful for clinical differentiation of subtypes
• Positive test supports diagnosis of vascular lesion

Auscultation:

• Audible bruit suggests arteriovenous hemangioma or malformation
• Absence of bruit in venous or capillary types
• Palpable thrill with high-flow arteriovenous lesions
• Increased heart rate or cardiac output with massive lesions
• Bruit accentuated with exercise or increased flow
• Helps identify high-risk lesions requiring embolization
• Important to assess before surgical intervention
• May indicate need for preoperative angiography

Age-Specific Presentations

Infants (0-12 months):

• Rapidly enlarging red or purple mass
• May not be present at birth (grows after birth)
• Alarming growth rate concerns parents
• Risk of ulceration with rapid proliferation
• Functional impairment if near vital structures
• Airway compromise with head and neck lesions
• Visual obstruction with periorbital location
• Consider propranolol for complicated cases

Children (1-10 years):

• Slower growth or stable size after infancy
• Pain with activity in intramuscular lesions
• Functional limitations during sports or play
• Cosmetic concerns in exposed areas
• May have phleboliths visible on radiographs
• School-age children more aware of differences
• Psychological impact of visible deformity
• Optimal time for surgical excision if indicated

Adolescents and Adults (greater than 10 years):

• Long-standing stable mass with recent growth
• Pain more prominent than in children
• Hormonal influences may cause enlargement
• Pregnancy-associated growth in females
• Occupational impact from functional limitations
• Diagnostic challenge if first presentation in adulthood
• Higher suspicion for malignancy in adult presentations
• MRI and possible biopsy to exclude sarcoma

Radiographic Imaging

Plain Radiographs:

Pathognomonic Findings:

• Phleboliths: Round or oval calcifications with radiolucent centers
• Present in 50% of soft tissue hemangiomas
• More common in cavernous and venous subtypes
• Size ranges from 2 mm to greater than 10 mm
• Multiple phleboliths create "grapes in a bag" appearance
• Calcifications follow venous distribution pattern
• May be only radiographic finding in chronic lesions
• Absence does not exclude diagnosis

Associated Findings:

• Soft tissue mass without calcification in 50% of cases
• Periosteal reaction if adjacent to bone (rare)
• Bone remodeling or scalloping from mass effect
• Increased bone density from hyperemia (children)
• Joint space widening with intra-articular lesions
• Osteopenia from disuse if chronic pain present
• Comparison views help assess asymmetry
• Low sensitivity for diagnosis but high specificity if phleboliths present

Ultrasound:

Advantages:

• Non-invasive, no radiation exposure
• Useful in infants and young children
• Real-time assessment of compressibility
• Doppler evaluation of blood flow characteristics
• Differentiates solid from cystic lesions
• Can guide biopsy if needed
• Monitors response to treatment over time
• Readily available and cost-effective

Sonographic Features:

• Heterogeneous echogenicity with mixed solid-cystic areas
• Hyperechoic phleboliths with posterior acoustic shadowing
• Doppler shows low-flow venous signals (venous type)
• High-flow arterial waveforms (arteriovenous type)
• Compressibility confirms vascular nature
• Septations within lesion common
• May demonstrate thrombosed areas as hypoechoic regions
• Limited for deep lesions or extent determination

Magnetic Resonance Imaging

Click to expand

MRI Protocol for Soft Tissue Hemangioma:

• T1-weighted sequences: Isointense to muscle, hyperintense if fat present
• T2-weighted sequences: Markedly hyperintense (diagnostic hallmark)
• Fat-suppressed sequences: Remain hyperintense (confirms fluid, not fat)
• T1 post-contrast: Serpentine or diffuse enhancement pattern
• Dynamic contrast-enhanced: Early enhancement with gradual filling
• Gradient echo sequences: Blooming artifact from phleboliths (flow voids)
• Include entire anatomic compartment to assess extent
• Coronal and sagittal planes for surgical planning

T2-Weighted Signal Characteristics:

Hyperintense Signal:

• Bright signal equal to or greater than fat on T2 sequences
• Reflects slow-flowing blood and vascular spaces
• Serpentine or lobulated hyperintense areas
• Multiple flow voids (signal dropout) from vessels
• Distinguishes hemangioma from solid tumors
• Heterogeneous signal from thrombosis or hemorrhage
• Septations appear as low signal bands
• Surrounding muscle edema if rapid growth or thrombosis

Flow Voids:

• Serpiginous areas of signal dropout on all sequences
• Represent rapidly flowing blood in feeding/draining vessels
• More prominent in arteriovenous subtypes
• Helpful to identify vascular nature of lesion
• Distinguish from cystic lesions (no flow voids)
• May see layering fluid-fluid levels from blood products
• Phleboliths appear as signal voids with GRE blooming
• Number of flow voids correlates with vascularity

Enhancement Patterns:

Venous Hemangioma:

• Gradual progressive enhancement over 5-10 minutes
• Peripheral to central filling pattern
• Enhancement persists on delayed images
• Heterogeneous due to thrombosis and fibrosis
• Septations enhance more than vascular spaces
• Feeding and draining veins enhance prominently
• No rapid arterial phase enhancement
• Correlates with slow-flow venous lesion

Arteriovenous Hemangioma:

• Rapid arterial phase enhancement
• Prominent feeding arteries and draining veins
• Early venous return on dynamic imaging
• Diffuse homogeneous enhancement
• Enlarged feeding vessels with flow voids
• May see arteriovenous shunting on time-resolved MRA
• High-flow characteristics require preoperative planning
• Embolization often needed before surgery

Extent and Margin Assessment:

• Infiltration into multiple muscle groups common
• Follows fascial planes and neurovascular bundles
• Poorly defined margins in 80% of intramuscular cases
• May encase nerves or vessels without invasion
• Involvement of adjacent joints in 10% of cases
• Subcutaneous extension from deep lesions
• Atrophy of infiltrated muscles on chronic imaging
• Compare to contralateral side for subtle findings

Advanced Imaging Studies

Angiography (Conventional or CT/MR Angiography):

Indications:

• Suspected high-flow arteriovenous hemangioma
• Preoperative planning for large or complex lesions
• Embolization treatment planning
• Differentiate from arteriovenous malformation
• Assess feeding vessels before surgery
• Map venous drainage patterns
• Guide sclerotherapy interventions
• Failed prior surgery with recurrence

Angiographic Findings:

• Low-flow lesions: Slow opacification, venous pooling, delayed washout
• High-flow lesions: Early arterial filling, arteriovenous shunting, enlarged feeders
• Phleboliths visible as filling defects
• "Puddle sign" of contrast pooling in cavernous spaces
• Draining veins without capillary blush (AV type)
• Feeding arteries may be hypertrophied
• Distinguish from vascular malformations (dysplastic vessels)
• Embolization coils or particles visible if prior treatment

Computed Tomography:

Limited Role:

• Inferior soft tissue contrast compared to MRI
• Radiation exposure limits use in children
• Useful if MRI contraindicated (pacemaker, metal)
• Excellent for detecting phleboliths
• CT angiography for vascular mapping in select cases
• Three-dimensional reconstruction for surgical planning
• Assess bony involvement adjacent to lesion
• Generally not first-line imaging modality

CT Findings:

• Heterogeneous soft tissue mass
• Calcified phleboliths (easier to detect than on MRI)
• Enhancement with intravenous contrast
• Infiltration of muscle compartments
• Enlarged feeding or draining vessels in high-flow lesions
• Bony remodeling or erosion in chronic cases
• Fluid-fluid levels from hemorrhage or thrombosis
• Similar findings to MRI but less specific

Histopathology and Biopsy

Biopsy Indications:

• Atypical presentation in adults (exclude sarcoma)
• Rapid growth or change in character
• Absence of characteristic imaging features
• Diagnostic uncertainty after MRI
• Solid-appearing lesion without vascular features
• Patient age greater than 40 at first presentation
• Consider core needle biopsy over open biopsy
• Avoid biopsy of high-flow lesions without embolization

Histologic Features:

Capillary Hemangioma:

• Lobules of capillary-sized vessels with narrow lumens
• Plump endothelial cells without significant atypia
• Mitotic figures may be present in proliferating lesions
• Pericytes surrounding endothelial channels
• Minimal intervening stroma
• Immunohistochemistry: CD31+, CD34+, ERG+, GLUT-1+ (infantile)
• Low-grade cytologic atypia acceptable
• Distinguish from angiosarcoma (high-grade atypia, infiltration, necrosis)

Cavernous/Venous Hemangioma:

• Large dilated vascular spaces lined by flat endothelium
• Thrombosis within vascular channels (common)
• Organized thrombi with recanalization
• Dystrophic calcification (phleboliths) in vessel walls
• Smooth muscle in walls of larger vessels
• Hemosiderin deposition from prior hemorrhage
• Fibrous septations between vascular spaces
• Infiltration between muscle fibers in intramuscular type

Immunohistochemistry Panel:

• CD31 (endothelial marker): Positive in all subtypes
• CD34 (endothelial and stromal): Positive
• ERG (endothelial transcription factor): Positive
• Factor VIII (von Willebrand factor): Variable positive
• GLUT-1 (glucose transporter): Positive only in infantile hemangioma
• Ki-67 (proliferation): Elevated in proliferating infantile type
• Smooth muscle actin: Positive in pericytes and vessel walls
• Cytokeratin: Negative (excludes epithelial tumors)

Differential Diagnosis on Histology:

• Angiosarcoma: High-grade cytologic atypia, infiltration, necrosis, mitoses
• Kaposi sarcoma: Spindle cell proliferation, HHV-8 positive, fascicular pattern
• Intramuscular myxoma: Hypocellular myxoid stroma, no vessels
• Vascular malformation: Dysplastic vessels, no proliferation
• Hemangiopericytoma/Solitary fibrous tumor: Staghorn vessels, STAT6+
• Epithelioid hemangioendothelioma: Epithelioid cells, myxoid stroma, keratin+
• Kaposiform hemangioendothelioma: Infiltrative, glomeruloid vessels
• Synovial sarcoma: Biphasic pattern, SYT-SSX fusion, cytokeratin+

Conservative Management

Observation Protocols:

Indications for Observation:

• Asymptomatic small lesions (less than 3 cm)
• Infantile hemangiomas in proliferative phase (first 12 months)
• No functional impairment of adjacent structures
• No rapid growth or concerning features
• Diagnosis confirmed on imaging
• Patient preference for non-surgical approach
• Elderly patients with medical comorbidities
• Lesions in areas where excision would cause significant morbidity

Monitoring Strategy:

• Clinical examination every 3-6 months initially
• Baseline MRI to document size and characteristics
• Repeat MRI at 12 months or if symptoms develop
• Photographic documentation for superficial lesions
• Symptom diary for pain or functional limitations
• Parent/patient education on warning signs
• Expected natural history discussion (involution in infantile type)
• Consider intervention if enlargement or new symptoms

Medical Management:

Propranolol for Infantile Hemangiomas:

• Indications: Rapidly proliferating infantile hemangiomas causing functional impairment, ulceration, or disfigurement
• Mechanism: Beta-blocker causing vasoconstriction, decreased VEGF, increased apoptosis
• Dosing: Start 1 mg/kg/day divided, increase to 2-3 mg/kg/day over 2 weeks
• Duration: Continue through proliferative phase (usually 6-12 months)
• Monitoring: Baseline ECG, blood pressure, heart rate checks
• Side effects: Bradycardia, hypotension, hypoglycemia, bronchospasm
• Efficacy: 60-80% response rate with size reduction
• Timing: Most effective when started before 5 months of age
• Contraindications: Asthma, heart block, hypotension

Corticosteroids (Second-Line):

• Prednisolone 2-3 mg/kg/day for proliferating infantile hemangiomas
• Less effective than propranolol (30-40% response rate)
• Side effects: Growth suppression, Cushingoid features, infection risk
• Reserved for cases with contraindications to propranolol
• Duration: 4-8 weeks with gradual taper
• Combination with propranolol for refractory cases
• Monitor growth, blood pressure, glucose
• Intralesional steroids for small localized lesions

Compression Therapy:

• Elastic compression garments for superficial lesions
• Reduces venous pooling and discomfort
• May decrease size through chronic compression
• Useful adjunct to other treatments
• Compliance challenging in pediatric patients
• Limited efficacy for deep intramuscular lesions
• Custom-fitted garments provide better results
• Continue for 6-12 months for maximal effect

Minimally Invasive Interventions

Sclerotherapy:

Technique:

• Ultrasound or fluoroscopic guidance
• Percutaneous injection of sclerosant into vascular spaces
• Agents: Ethanol, sodium tetradecyl sulfate, bleomycin, polidocanol, doxycycline
• Multiple sessions usually required (3-5 treatments)
• Interval of 4-6 weeks between treatments
• May combine with compression after treatment
• Performed under sedation or general anesthesia
• Avoid injection into feeding arteries

Indications:

• Low-flow venous or cavernous hemangiomas
• Accessible lesions amenable to percutaneous approach
• Patients preferring non-surgical treatment
• Poor surgical candidates due to comorbidities
• Recurrent lesions after incomplete excision
• Multiple small hemangiomas (diffuse hemangiomatosis)
• Lesions in anatomically challenging locations
• Adjunct to surgery for large lesions

Outcomes and Complications:

• Success rate: 60-80% for appropriately selected lesions
• Complete resolution uncommon (less than 30%)
• Pain and swelling post-procedure (common, self-limited)
• Skin necrosis from extravasation of sclerosant (2-5%)
• Nerve injury from sclerosant diffusion (rare)
• Compartment syndrome if large volume injected
• Deep vein thrombosis from venous sclerosis
• May require subsequent surgery for residual lesion

Embolization:

Indications:

• High-flow arteriovenous hemangiomas
• Preoperative reduction of blood flow before surgery
• Large lesions with significant bleeding risk
• Failed sclerotherapy or surgical excision
• Lesions not amenable to complete surgical excision
• Symptomatic relief without definitive surgery
• Recurrent bleeding episodes
• Part of multimodal treatment strategy

Technique:

• Selective catheterization of feeding arteries
• Injection of embolic materials (coils, particles, glue, Onyx)
• Proximal and distal embolization to prevent collateral flow
• Staged embolization for extensive lesions
• Perform 24-72 hours before planned surgery (if preoperative)
• General anesthesia or conscious sedation
• Avoid non-target embolization to normal tissues
• Post-procedure angiography confirms occlusion

Complications:

• Skin necrosis from non-target embolization (3-5%)
• Nerve injury from ischemia (rare)
• Post-embolization syndrome: Pain, fever, nausea (common, self-limited)
• Recanalization and recurrence (10-20%)
• Infection of necrotic tissue
• Incomplete embolization requiring repeat procedures
• Distal ischemia if collateral circulation inadequate
• Migration of embolic material

Laser Therapy:

Indications:

• Superficial cutaneous hemangiomas
• Infantile hemangiomas with ulceration or bleeding
• Residual telangiectasias after involution
• Cosmetic improvement of color and texture
• Not effective for deep intramuscular lesions
• Adjunct to other treatments for mixed lesions
• Early treatment may reduce proliferation
• Multiple sessions required for optimal results

Modalities:

• Pulsed dye laser (595 nm): Targets hemoglobin, superficial lesions
• Nd:YAG laser (1064 nm): Greater penetration, thicker lesions
• KTP laser (532 nm): Alternative to pulsed dye laser
• CO2 laser: For ulcerated or friable lesions (resurfacing)
• Settings adjusted based on lesion characteristics
• Cooling devices protect epidermis
• General anesthesia for extensive lesions in children
• Interval of 6-8 weeks between treatments

Surgical Management

Surgical Indications:

Absolute Indications:

• Symptomatic compression of neurovascular structures
• Recurrent bleeding or thrombosis causing pain
• Functional impairment limiting activities of daily living
• Diagnostic uncertainty requiring excisional biopsy
• Failed conservative management with progression
• Recurrent hemarthrosis causing joint damage
• Compartment syndrome from rapidly enlarging lesion
• Pathologic fracture through bone involvement (rare)

Relative Indications:

• Cosmetic concerns in visible locations
• Persistent pain despite conservative treatment
• Patient preference for definitive treatment
• Lesion in location amenable to complete excision
• Age appropriate for elective surgery (greater than 1 year)
• Stable lesion unlikely to involute spontaneously
• Psychological impact on patient or family
• Interference with normal development or activities

Preoperative Planning:

Imaging Review:

• MRI assessment of extent in all three planes
• Identify relationship to neurovascular structures
• Determine involvement of muscle groups and compartments
• Plan surgical approach based on anatomic location
• Angiography if high-flow features on MRI
• Mark skin incision and expected extent of resection
• Review images with patient/family to set expectations
• Consider interventional radiology consultation for embolization

Blood Management:

• Type and cross-match blood products (2-4 units for large lesions)
• Cell saver for large resections (if not contaminated)
• Tranexamic acid administration (10-15 mg/kg IV bolus, then infusion)
• Tourniquet use for extremity lesions (tourniquet time limits apply)
• Consider preoperative embolization for high-flow or greater than 5 cm lesions
• Timing of surgery 24-72 hours after embolization optimal
• Hemostatic agents available (thrombin, gelatin, oxidized cellulose)
• Discuss bleeding risk with patient and anesthesia team

Consent Discussion:

• Risk of incomplete excision and recurrence (20-40%)
• Bleeding risk and possible transfusion requirement
• Nerve or vessel injury from dissection (5-10% for large lesions)
• Muscle weakness from sacrifice of involved muscle
• Scar and cosmetic outcome
• Possibility of staged procedures for massive lesions
• Alternative treatments and risks of observation
• Realistic expectations about functional outcome

Surgical Technique:

Approach and Exposure:

• Longitudinal incision along line of limb for extremity lesions
• Extensile approach allowing proximal and distal extension
• Identify and protect neurovascular structures early
• Develop plane between lesion and uninvolved muscle
• Recognize infiltrative nature - often appears worse than imaging
• Use loupe magnification for small vessel ligation
• Tourniquet inflated after exsanguination (extremity)
• Adequate assistant and retraction essential

Resection Principles:

• Complete excision of all involved tissue (most important factor)
• Sacrifice involved muscle if necessary for negative margins
• Follow fascial planes to limit dissection
• Sequential ligation of feeding vessels reduces bleeding
• Preserve major nerves and vessels (may require careful dissection from lesion)
• Remove all phleboliths and thrombosed areas (often at periphery)
• Frozen section if margins unclear (distinguish from normal muscle)
• Accept some muscle dysfunction to achieve complete excision

Hemostasis Techniques:

• Meticulous electrocautery of small vessels
• Suture ligation of larger feeding and draining vessels
• Topical hemostatic agents in resection bed
• Oxidized cellulose or gelatin sponges for oozing
• Avoid excessive cautery (causes muscle necrosis and complications)
• Tourniquet deflation before closure to check hemostasis
• Closed suction drainage (Jackson-Pratt or Blake) for dead space
• Compression dressing postoperatively

Reconstruction and Closure:

• Muscle approximation if possible without tension
• Drain placement in deep dead space (avoid hematoma)
• Layered closure with absorbable sutures
• Skin closure with subcuticular or interrupted sutures
• No role for primary muscle flap reconstruction usually
• Consider skin graft if skin loss from extensive resection
• Splint or immobilize if near joint (allow soft tissue healing)
• Compression dressing for venous hemangiomas

Postoperative Management:

Immediate Postoperative (0-48 hours):

• Monitor drain output (expect 50-200 mL/day initially)
• Pain control with multimodal analgesia
• Check distal neurovascular status frequently
• Elevate extremity to reduce swelling
• Remove drain when output less than 30 mL/day (usually 3-7 days)
• Monitor for signs of compartment syndrome if extensive resection
• Deep vein thrombosis prophylaxis for lower extremity
• Early range of motion of digits to prevent stiffness

Rehabilitation:

• Physical therapy starting postoperative day 1-2
• Gentle active range of motion initially
• Progress to strengthening at 4-6 weeks
• Scar massage starting at 2-3 weeks
• Compression garment if significant edema
• Return to full activities at 3-4 months
• Monitor for delayed complications (seroma, hematoma)
• Long-term surveillance for recurrence (clinical exam every 6 months for 2 years)

Special Surgical Scenarios:

Intra-articular Hemangiomas:

• Arthroscopic versus open synovectomy
• Remove all involved synovium to prevent recurrence
• Hemarthrosis causes hemosiderin deposition and further synovitis
• May require total synovectomy for diffuse involvement
• Postoperative arthrofibrosis risk (aggressive rehabilitation)
• Consider radiosynovectomy for elderly or high-risk patients
• Recurrence common if incomplete synovectomy (30-50%)
• Joint replacement may be necessary for advanced arthritis

Recurrent Hemangiomas:

• MRI to define extent before reoperation
• Scar tissue makes dissection more challenging
• Higher risk of nerve and vessel injury
• Consider alternatives (sclerotherapy, embolization) before reoperation
• May require more extensive resection at reoperation
• Discuss realistic expectations (may not achieve complete excision)
• Staged procedures for massive recurrent lesions
• Some patients managed with symptom control rather than cure

Outcomes and Prognosis

Surgical Outcomes:

• Complete excision curative in 60-80% of cases
• Recurrence rate 20-40% with incomplete excision
• Functional outcomes generally good if nerve preserved
• Some muscle weakness expected if muscle sacrificed
• Cosmetic outcomes acceptable with modern techniques
• Patient satisfaction high if expectations managed
• Most patients return to full activities by 4-6 months
• Recurrent bleeding rare after complete excision

Natural History Without Treatment:

• Infantile hemangiomas: 50% involute by age 5, 70% by age 7
• Congenital hemangiomas (RICH): Complete involution by 12-14 months
• Congenital hemangiomas (NICH): Persist without involution
• Intramuscular hemangiomas in adults: Persistent without involution
• Slow growth over years common in adult lesions
• Acute thrombosis episodes may occur intermittently
• Malignant transformation essentially never occurs (benign lesion)
• Quality of life impact depends on symptoms and location

Treatment-Related Complications

Surgical Complications:

Intraoperative Bleeding:

• Incidence: 5-15% require transfusion
• Risk factors: Large lesions (greater than 5 cm), high-flow type, no preoperative embolization
• Presentation: Diffuse oozing from resection bed, tourniquet conceals extent
• Management: Topical hemostatics, suture ligation, tranexamic acid, blood transfusion
• Prevention: Preoperative embolization, meticulous technique, adequate blood available
• Outcome: Rarely causes surgery to be aborted
• Tourniquet use in extremity limits intraoperative blood loss
• Cell saver reduces allogeneic transfusion requirements

Incomplete Excision and Recurrence:

• Incidence: 20-40% with subtotal excision, less than 10% with complete excision
• Cause: Infiltrative margins difficult to identify intraoperatively
• Presentation: Recurrent mass at surgical site months to years later
• Diagnosis: MRI showing recurrent enhancing tissue
• Prevention: Wide margins, sacrifice involved muscle, frozen section guidance
• Management: Observation vs reoperation vs sclerotherapy/embolization
• Some patients managed symptomatically rather than repeat surgery
• Discuss possibility of incomplete excision in preoperative consent

Nerve Injury:

• Incidence: 5-10% for large lesions near neurovascular bundles
• Mechanisms: Stretch injury, iatrogenic transection, ischemia from embolization
• Presentation: Numbness, weakness, neuropathic pain in nerve distribution
• Common nerves at risk: Sciatic (thigh/buttock lesions), radial (arm lesions), median/ulnar (forearm)
• Management: Observation for neurapraxia (6-12 months), exploration and repair if transection
• Prevention: Loupe magnification, meticulous dissection, protect nerves with vessel loops
• Most injuries are neurapraxias that recover over 3-6 months
• Document preoperative neurologic exam for medicolegal protection

Vascular Injury:

• Incidence: Less than 5% clinically significant injuries
• Presentation: Distal ischemia, hematoma, pseudoaneurysm, arteriovenous fistula
• High-risk lesions: Those encasing major vessels, high-flow arteriovenous type
• Management: Vascular surgery consultation, repair or reconstruction as needed
• Prevention: Angiography, careful dissection, vascular instruments available
• May require vein graft reconstruction if vessel sacrificed
• Document distal pulses before and after surgery
• Early recognition and intervention prevents limb loss

Infection:

• Incidence: 2-5% of surgical cases
• Risk factors: Large dead space, hematoma, diabetes, immunosuppression
• Presentation: Erythema, drainage, fever, elevated inflammatory markers
• Organisms: Staphylococcus aureus most common, consider MRSA
• Management: Antibiotics, drainage if abscess or hematoma present
• Prevention: Perioperative antibiotics, sterile technique, drain placement
• Superficial infections managed with oral antibiotics
• Deep infections may require operative debridement

Sclerotherapy Complications:

Skin Necrosis:

• Incidence: 2-5% of sclerotherapy procedures
• Cause: Extravasation of sclerosant, high concentration near skin
• Presentation: Pain, skin discoloration, then ulceration days after procedure
• Management: Wound care, debridement if extensive, skin graft if needed
• Prevention: Careful injection technique, dilute sclerosant concentrations
• Worse with ethanol (most caustic sclerosant)
• Healing by secondary intention acceptable for small areas
• Large areas may require reconstruction

Nerve Injury:

• Incidence: Less than 2% with careful technique
• Mechanism: Direct sclerosant neurotoxicity from diffusion to adjacent nerve
• Presentation: Numbness, dysesthesias, weakness in nerve distribution
• Usually transient with recovery over weeks to months
• Management: Observation, neuropathic pain medications if needed
• Prevention: Avoid injection near major nerves, use image guidance
• Document neurologic exam before procedure
• Permanent injury rare

Deep Vein Thrombosis:

• Risk with sclerotherapy of large venous hemangiomas
• Sclerosant causes thrombosis that may propagate to deep veins
• Symptoms: Calf pain, swelling, positive Homan's sign
• Diagnosis: Duplex ultrasound of deep veins
• Management: Anticoagulation if DVT confirmed
• Prevention: Limit volume of sclerosant, compression after procedure
• Consider prophylactic anticoagulation for high-risk patients
• Monitor for pulmonary embolism if extensive thrombosis

Embolization Complications:

Non-target Embolization:

• Incidence: 3-5% clinically significant events
• Presentation: Skin necrosis, nerve palsy, distal ischemia
• Cause: Reflux of embolic material, collateral flow to normal tissues
• Management: Supportive care, wound care for skin loss, nerve recovery usually complete
• Prevention: Careful catheter placement, avoid overfilling, use appropriate embolic agents
• Most serious complication of embolization
• May require subsequent reconstruction or debridement

Post-embolization Syndrome:

• Incidence: 50-80% after embolization (expected response)
• Symptoms: Pain, fever, nausea, malaise for 3-7 days
• Cause: Tissue ischemia and inflammatory response
• Not infection (cultures negative)
• Management: NSAIDs, antiemetics, hydration, reassurance
• Self-limited with complete resolution
• Distinguish from infection (prolonged course, positive cultures)
• Warn patients preoperatively about expected symptoms

Recanalization and Recurrence:

• Incidence: 10-20% develop collateral flow after embolization
• Presentation: Recurrent symptoms months to years later
• More common with proximal embolization only (allows collateral development)
• Diagnosis: Repeat angiography showing new feeding vessels
• Management: Repeat embolization or surgical excision
• Prevention: Distal and proximal embolization, multiple feeding vessels
• Some lesions require repeated embolizations for symptom control
• Not curative in most cases (palliative treatment)

Disease-Related Complications

Kasabach-Merritt Phenomenon (NOT with true hemangiomas):

• Occurs with kaposiform hemangioendothelioma or tufted angioma, NOT hemangioma
• Consumption coagulopathy with thrombocytopenia, hypofibrinogenemia
• Presentation: Petechiae, purpura, bleeding, rapidly enlarging firm mass
• Life-threatening if untreated (mortality historically 20-30%)
• Diagnosis: Platelets less than 25,000, low fibrinogen, elevated D-dimer, MRI showing infiltrative lesion
• Management: Corticosteroids, vincristine, sirolimus (mTOR inhibitor), supportive care
• Surgery contraindicated during acute phase (high bleeding risk)
• Important to distinguish true hemangioma (no Kasabach-Merritt) from kaposiform type
• Biopsy shows infiltrative glomeruloid capillary proliferation in kaposiform type

Compartment Syndrome:

• Rare complication of rapidly enlarging hemangioma
• Mechanisms: Hemorrhage into lesion, acute thrombosis with swelling, trauma to lesion
• Presentation: Severe pain, tense compartment, pain with passive stretch, sensory changes
• Diagnosis: Clinical, compartment pressure greater than 30 mmHg confirms
• Management: Urgent fasciotomy, do NOT delay for imaging
• Prevention: Early treatment of symptomatic lesions
• Postoperative compartment syndrome risk after extensive resection
• High index of suspicion in perioperative period

Pathologic Fracture (Intraosseous Hemangiomas):

• Rare with soft tissue hemangiomas (occurs with bone hemangiomas)
• Vertebral hemangiomas most prone to fracture
• Presentation: Sudden pain, deformity, neurologic deficit if spine
• Imaging: Lytic lesion with trabeculated "corduroy" or "polka dot" appearance
• Management: Stabilization and fixation, embolization, vertebroplasty for spine
• Soft tissue hemangiomas rarely involve bone to degree causing fracture
• Adjacent bone may have remodeling or scalloping from mass effect
• Periosteal reaction suggests aggressive process (reconsider diagnosis)

Joint Destruction (Intra-articular Hemangiomas):

• Recurrent hemarthrosis causes synovitis and cartilage damage
• Hemosiderin deposition accelerates degenerative changes
• Progression to secondary osteoarthritis if untreated
• Symptoms: Pain, stiffness, limited range of motion, effusions
• Imaging: Joint space narrowing, subchondral sclerosis, osteophytes
• Management: Synovectomy early prevents progression, arthroplasty if end-stage
• Similar pathophysiology to hemophilic arthropathy
• Early treatment prevents irreversible joint damage

Australian Epidemiology and Management

Incidence and Referral Patterns:

• Infantile hemangiomas affect approximately 4-5% of Australian infants
• Intramuscular hemangiomas in adults are uncommon, typically referred to musculoskeletal tumour units
• State-based sarcoma services at major metropolitan centres manage complex vascular tumours
• Multi-disciplinary team (MDT) approach recommended for large or symptomatic lesions

RACS Orthopaedic Training Relevance:

• Hemangiomas appear in FRACS Orthopaedic examination as differential for soft tissue masses
• Key examination topics: phlebolith identification on radiographs, MRI interpretation, surgical principles
• Viva scenarios commonly test differentiation from sarcoma and understanding of incomplete excision risk
• Kasabach-Merritt trap: know this occurs with kaposiform hemangioendothelioma, NOT hemangiomas

Interventional Radiology Services:

• Sclerotherapy and embolization available at tertiary centres
• Preoperative embolization coordinated with orthopaedic surgery for large or high-flow lesions
• IR referral pathway well-established in Australian vascular anomalies clinics

Pharmaceutical Benefits Scheme (PBS) Considerations:

• Propranolol (oral solution) listed for complicated infantile hemangiomas in infants
• Requires paediatric specialist (dermatologist, paediatrician, or plastic surgeon) initiation
• Hemaniol oral solution 3.75 mg/mL PBS listed for functional impairment, ulceration, or disfigurement risk
• Monitoring requirements: baseline ECG, regular BP and HR checks during initiation
• Corticosteroids not PBS listed specifically for hemangioma but available on general PBS items

State-Based Vascular Anomalies Clinics:

• Royal Children's Hospital Melbourne - Vascular Anomalies Clinic
• Sydney Children's Hospital Network - Vascular Birthmarks Service
• Queensland Children's Hospital - Dermatology and Vascular Lesions Service
• Princess Margaret Hospital Perth - Paediatric dermatology service for vascular lesions
• Adult soft tissue tumour referrals through state sarcoma services