Infantile haemangioma and vascular malformations

01OverviewDefinition, clinical context and the essential points that orientate the chapter.

The useful first split is tumour behaviour versus malformed vessels. Infantile haemangioma displays a postnatal growth curve: a precursor mark may be present at birth, then endothelial proliferation creates a red superficial plaque, a blue subcutaneous mass or a mixed lesion during the first months. Growth slows and involution follows, but residual telangiectasia, fibrofatty tissue, scar or distortion can remain. A congenital haemangioma is already fully formed at birth and may involute rapidly or persist, so it should not be forced into the infantile timeline.

Vascular malformations reflect capillary, venous, lymphatic, arteriovenous or combined channels. A capillary malformation is a flat persistent patch that darkens or thickens with time. Venous lesions are soft blue and enlarge with dependency or Valsalva; lymphatic lesions create soft swelling or surface vesicles with infection and bleeding; arteriovenous malformations are warm, pulsatile and may have a thrill or bruit. Colour can look red, violet, brown or blue-grey depending on depth and pigmentation, so compressibility, warmth, flow and growth history matter as much as hue.

Early recognition prevents irreversible harm. A small eyelid tumour can cause astigmatism and amblyopia, a lip lesion can ulcerate and impair feeding, and an airway lesion can narrow before obvious external bulk. Families need a named referral route, feeding and ulcer safety advice and a serial photographic plan. Observation is active management for low-risk lesions, not abandonment; progression, function, pain and parental priorities are reviewed during the proliferative period.

Key points

Infantile haemangioma is usually absent or faint at birth, becomes evident in the first weeks, proliferates rapidly during early infancy and then involutes over years.
Vascular malformations are structural channel abnormalities present at birth, although sometimes subtle; they grow with the child, expand with hormonal or traumatic change and do not follow spontaneous tumour involution.
Superficial infantile haemangioma is bright red and raised, deep disease is skin-coloured or blue and compressible, and mixed lesions show both levels across every skin tone.
Referral urgency depends on function and future distortion, not diameter alone: eyelid, nose, lip, ear, airway beard distribution, perineum, scalp and segmental patterns are high risk.
Five or more cutaneous infantile haemangiomas should prompt specialist consideration of liver ultrasound because diffuse hepatic disease can cause high-output heart failure or hypothyroidism.
A large segmental facial or scalp haemangioma raises PHACE concern; extensive lumbosacral or perineal disease raises spinal, genitourinary and anorectal anomaly concern.
First-line active treatment for a problematic proliferating infantile haemangioma is oral propranolol started early through a paediatric specialist protocol, usually before the fastest growth window closes.
Give propranolol during or immediately after a feed and withhold it during poor intake, vomiting or significant illness to reduce hypoglycaemia risk.
Capillary malformations use laser, venous and lymphatic malformations may use sclerotherapy or surgery, and arteriovenous malformations need flow imaging and staged embolisation or surgery rather than propranolol.
Kasabach–Merritt consumptive coagulopathy is associated with kaposiform haemangioendothelioma or tufted angioma, not an ordinary infantile haemangioma.

02AetiologyUnderlying causes, associations and risk factors, with why each one matters.

Infantile endothelial tumour

A clonally expanding endothelial population emerges after birth, with placental-like markers and growth signals that later switch towards programmed involution.

Developmental channel error

Capillary, venous, lymphatic and arteriovenous malformations arise from abnormal vascular morphogenesis and persist as structurally disorganised channels throughout life.

Somatic signalling variants

Post-zygotic variants in vascular growth pathways create mosaic malformations, explaining segmental distribution, tissue overgrowth and variable involvement within one person.

Syndromic field involvement

Large segmental patterns can mark a wider developmental field involving brain, arteries, heart, eyes, spine, genitourinary or anorectal structures.

03PathophysiologyThe causal sequence from the underlying abnormality to symptoms and harm.

1
Haemangioma proliferates then involutes
Early endothelial multiplication and high blood flow enlarge the tumour, followed by apoptosis and replacement with fibrofatty tissue over subsequent years.
2
Capillary channels remain superficial
Persistent ectatic dermal capillaries create a flat patch that grows with the child and can thicken or develop nodules later.
3
Venous channels pool blood
Slow-flow compressible spaces distend with dependency and can thrombose locally, producing pain, swelling, phleboliths and consumption of coagulation factors in extensive disease.
4
Lymphatic spaces collect fluid
Malformed lymphatic cisterns and vesicles enlarge with infection, bleeding or trauma and can cross tissue planes without respecting ordinary anatomical boundaries.
5
Arteriovenous shunts steal flow
Direct high-flow connections bypass capillaries, causing warmth, pulsation, venous hypertension, tissue ulceration and sometimes progressive cardiac volume overload.

04Clinical features and red flagsSymptoms, examination findings, patterns of presentation and time-critical warnings.

Postnatal proliferating tumour

A faint precursor becomes a rapidly enlarging red, blue or mixed mass during the first weeks and months, supporting infantile haemangioma.

Superficial strawberry plaque

A bright red or red-brown lobulated raised surface remains warm but is not pulsatile and represents dermal infantile-haemangioma tissue.

Deep compressible component

A skin-coloured, violet or blue soft swelling beneath normal surface appears later and can obscure true depth and anatomical extent.

Congenital persistent patch

A flat capillary malformation is evident from birth, grows proportionally and may deepen in colour or thicken rather than involuting.

Low-flow malformation

Venous or lymphatic lesions compress, swell with dependency, infection or Valsalva and may contain phleboliths or translucent surface vesicles.

High-flow malformationRed flag

Warmth, pulsation, bruit, thrill, rapid expansion, pain or bleeding indicates arteriovenous shunting and makes unplanned biopsy hazardous.

High-risk haemangioma siteRed flag

Periocular, airway-distribution, nasal-tip, lip, ear, ulcerated, large segmental or perineal lesions need early specialist review during proliferation.

Red flags requiring action

Stridor, eye occlusion, ulceration, severe pain, poor feeding, failure to thrive, recurrent bleeding, palpable thrill, bruit, limb overgrowth, neurological signs, five or more cutaneous haemangiomas or a large segmental facial or lumbosacral lesion requires expedited specialist assessment.

05InvestigationsWhat to request, why it matters and how to interpret it.

Investigation order

Read from the initial assessment onwards. Tests may run in parallel in urgent care; first-line, preferred, confirmatory, definitive and gold-standard labels appear only when the chapter explicitly states them.

01
Birth and growth chronologyFirst step
Why
Distinguish infantile tumour proliferation from a malformation or congenital tumour.
Interpretation and limitations
Record visibility at birth, onset week, speed, proportional growth, triggers and any plateau or involution; dated parental photographs often provide the most discriminating evidence.
02
Structured examination and photography
Why
Map morphology, flow, function and future disfigurement risk without unnecessary procedures.
Interpretation and limitations
Measure in three dimensions and assess compressibility, warmth, pulse, bruit, eye, airway, feeding, hearing, ulcer and limb asymmetry; obtain consent for serial images.
03
Ultrasound with Doppler
Why
Confirm flow and depth when diagnosis is uncertain or a deep component is suspected.
Interpretation and limitations
A proliferating haemangioma is a vascular solid mass, while low-flow channels and high-flow shunts have different Doppler behaviour; operator expertise and age affect interpretation.
04
MRI and MR angiography
Why
Define deep extent, tissue planes and arterial or venous architecture before treatment.
Interpretation and limitations
Use for segmental, syndromic, airway-adjacent, limb or complex malformations and high-flow lesions; imaging should answer a multidisciplinary planning question and may require anaesthetic risk review.
05
Site-specific functional review
Why
Detect threatened vision, airway, hearing, feeding or neurological development before irreversible loss.
Interpretation and limitations
Ophthalmology assesses refraction and visual axis, ENT evaluates stridor and airway, and neurology, cardiology or surgery joins according to lesion distribution and symptoms.
06
Syndrome-directed imaging
Why
Find associated arterial, cardiac, brain, eye, spinal, renal or anorectal anomalies in a segmental pattern.
Interpretation and limitations
Large facial or scalp disease prompts PHACE assessment and extensive lumbosacral or perineal disease prompts regional structural evaluation; screening is anatomy-led rather than universal.
07
Liver and thyroid assessment
Why
Detect occult hepatic haemangiomas and physiological consequences when cutaneous burden is high.
Interpretation and limitations
Specialists consider liver ultrasound with five or more skin lesions and assess cardiac output and thyroid function if hepatic disease is extensive or the infant is symptomatic.
08
Blood count and coagulation for atypical tumour
Why
Identify consumptive coagulopathy when a painful infiltrative vascular tumour is enlarging.
Interpretation and limitations
Profound thrombocytopenia and hypofibrinogenaemia suggests kaposiform haemangioendothelioma or tufted angioma, not ordinary infantile haemangioma, and needs emergency specialist therapy.

06Differential diagnosisRealistic alternatives and the features that help distinguish them.

Congenital haemangioma

A vascular tumour fully formed at birth may rapidly involute, partly involute or remain unchanged and therefore lacks the classic postnatal proliferative phase.

Kaposiform vascular tumour

A firm infiltrative painful plaque with bruising, oedema and thrombocytopenia suggests kaposiform haemangioendothelioma or tufted angioma with Kasabach–Merritt phenomenon.

Dermoid or other mass

A fixed nonvascular subcutaneous lump lacks compressibility and flow and requires anatomical imaging when close to orbit, skull or midline structures.

Port-wine capillary malformation

A flat patch present at birth persists and grows proportionally, with facial distribution sometimes signalling ocular or neurological association.

Bruise or infection

Trauma and cellulitis change over days with tenderness, systemic context and no organised tumour growth curve, although ulcerated lesions can become secondarily infected.

07ManagementImmediate care, first-line treatment, alternatives and escalation.

01First classification sequenceUse the timeline before the colourFirst stepAn infant or child presents with a vascular-appearing birthmark or swelling.

1Establish presence and completion at birth, postnatal onset, speed and trigger-related expansion using dated photographs and pregnancy and birth history.
2Examine surface, depth, compressibility, warmth, pulse and bruit and assess vision, airway, feeding, hearing, ulceration, bleeding, pain and limb growth.
3Diagnose clinically when classic, obtain expert Doppler for uncertainty and refer high-risk sites or flow features promptly rather than waiting to observe the natural history.

02First-line low-risk careObserve actively through the growth windowFirst lineA small uncomplicated infantile haemangioma is away from functional and high-distortion sites.

1Explain proliferation and involution honestly, including possible residual skin, and give photographs and measurements a scheduled review interval during early growth.
2Use bland emollient and protect from friction; provide an ulcer contact plan and avoid folk remedies, ligation or trauma to the lesion.
3EscalationEscalate immediately if growth accelerates, surface breaks, pain develops or eye, airway, feeding, hearing or anatomy becomes threatened.

03First-line propranolol routeTreat function-threatening proliferation earlyFirst lineAn infantile haemangioma threatens function, ulcerates, risks major disfigurement or forms part of a high-risk pattern.

1Confirm phenotype and comorbidity, assess heart and lungs, feeding and glucose risk and perform ECG or specialist tests only when history or examination indicates them.
2EscalationBegin licensed paediatric oral propranolol through an experienced team with weight-based weekly titration and heart-rate and blood-pressure observation at initiation and escalation.
3Give every dose with a feed, suspend during poor intake or vomiting and review growth, ulcer healing, function, sleep, breathing and cold extremities throughout the planned course.

04Malformation routeMatch intervention to channel and flowThe lesion was present at birth, grows proportionally or has low- or high-flow malformation features.

1Use specialist Doppler and MRI or angiography to classify capillary, venous, lymphatic, arteriovenous or combined anatomy and assess functional, coagulation and overgrowth consequences.
2Offer pulsed-dye laser for selected capillary malformations, image-guided sclerotherapy or surgery for symptomatic low-flow lesions and compression where anatomically safe.
3Manage arteriovenous malformation through a vascular-anomalies team with staged embolisation and surgery where indicated; avoid proximal ligation or incomplete opportunistic excision that can accelerate collateral growth.

05Ulcer and bleeding escalationControl pain and preserve functionEscalationA haemangioma or malformation becomes ulcerated, infected or bleeds.

1Apply firm continuous non-adherent pressure for bleeding and seek emergency help if it does not stop or the child becomes unwell; do not repeatedly lift the dressing to inspect.
2Use gentle cleansing, non-stick moisture-balanced dressings and age-appropriate analgesia and culture only clinical infection rather than every colonised surface.
3Expedite specialist tumour treatment or malformation intervention because healing depends on controlling growth, flow, friction and site pressure as well as dressing choice.

Key medicines and prescribing safety1 treatment · regimens, roles and cautions

Licensed first-line systemic treatment for proliferating infantile haemangioma requiring therapy, ideally started between 5 weeks and 5 months corrected age.

Propranolol oral solution for infantile haemangioma

For eligible infants, start 0.5 mg/kg per dose twice daily in week 1, increase to 1 mg/kg per dose twice daily in week 2, then 1.5 mg/kg per dose twice daily from week 3, at least 9 hours apart, usually for 6 months under specialist care.

Give during or immediately after feeds and omit doses if the child is not feeding, is vomiting or significantly unwell. Monitor heart rate and blood pressure after initiation and increases; avoid relevant bradycardia, hypotension, heart block and bronchospasm and teach hypoglycaemia warning signs.

08ComplicationsImportant consequences, why they occur and why they matter clinically.

Visual or airway loss

Periocular growth can cause deprivation amblyopia and astigmatism, while subglottic disease causes progressive stridor and potentially fatal airway obstruction.

Ulceration and pain

Rapid growth, friction and poor local perfusion break the surface, causing severe pain, feeding difficulty, infection, bleeding and permanent scar.

High-output cardiac failure

Extensive hepatic haemangioma or arteriovenous shunting returns excessive flow to the heart, producing tachycardia, hepatomegaly, poor feeding and failure to thrive.

Functional overgrowth and thrombosis

Venous, lymphatic and combined malformations cause limb discrepancy, recurrent inflammation, local thrombosis, bleeding, pain and reduced mobility.

Permanent appearance and distress

Residual fibrofatty tissue, telangiectasia, scar, facial distortion and visible malformation can affect social development and require reconstructive and psychological support.

09Monitoring and follow-upTreatment response, safety checks and longer-term review.

During observation, use the same view, scale and lighting to record growth and review more frequently during the first months when proliferation is fastest.
On propranolol, recalculate dose with weight through the specialist schedule and track feeding, illness, wheeze, sleep, cold extremities, heart rate and blood pressure.
Tell carers in writing to give propranolol with feeds, leave at least nine hours between doses and withhold it during fasting, vomiting or poor intake.
For periocular disease, follow refraction, visual axis and amblyopia even when surface size improves; appearance alone cannot prove normal visual development.
For low-flow malformations, monitor pain, swelling, thrombosis, bleeding, infection, limb discrepancy and function across growth and puberty.
For AV malformation, monitor warmth, thrill, expansion, ulceration, haemorrhage and cardiac symptoms and maintain long-term specialist surveillance after intervention.
Address residual scar, fibrofatty tissue, pigment and psychosocial concerns after involution and time reconstructive decisions to anatomy, growth and family goals.

10Special situationsVariants, exceptions and circumstances that change the usual approach.

The clock is diagnostic

Postnatal appearance and proliferation is more discriminating for infantile haemangioma than red or blue colour, which is shared by several malformations.

Small can be high risk

A modest eyelid or subglottic lesion can cause permanent visual or airway harm, while a much larger trunk lesion may be safely observed.

Five skin lesions matter

Multiple cutaneous haemangiomas act as a marker for possible hepatic burden, where shunting and hormone inactivation create cardiac and thyroid consequences.

Warmth changes the procedure

A thrill or bruit indicates high flow and makes blind biopsy, sclerotherapy or proximal vessel ligation dangerous without angiographic planning.

Propranolol safety follows feeding

Beta blockade can hide adrenergic warning signs and impair recovery from fasting hypoglycaemia, which is why doses stop when intake is unreliable.

Kasabach–Merritt is not common haemangioma

Platelet consumption belongs to kaposiform haemangioendothelioma and tufted angioma and should trigger urgent reclassification rather than routine propranolol alone.

11Common pitfallsFrequent interpretation and management errors.

01
Calling every vascular birthmark a haemangioma and waiting for a malformation to involute.
02
Delaying referral of eyelid, airway, lip, nasal-tip or ulcerated disease until after the rapid proliferation window.
03
Judging flow from colour alone instead of palpating warmth, pulse and thrill and using Doppler.
04
Giving propranolol during fasting, vomiting or poor feeding and exposing an infant to hypoglycaemia.
05
Biopsying or ligating a pulsatile lesion before high-flow imaging and vascular-anomalies review.
06
Misattributing profound thrombocytopenia to ordinary infantile haemangioma rather than an aggressive vascular tumour.
07
Treating surface closure as proof that vision, airway, hearing or deep anatomy is no longer threatened.