II. Background
- Infantile Hemangioma (referred to on this page)
- Common, benign vascular lesion that appears in the first months of life
- Contrast with Congenital Hemangioma (which is prominent at birth) described in differential diagnosis
III. Epidemiology
-
Prevalence: 1.1 - 2.6% up to 4 to 5% of all live births (up to 10% of infants at one year)
- Contrast with the relatively rare Congenital Hemangioma (compared with Infantile Hemangioma)
- Gender: Female more common than male
- Race: White, non-hispanic
IV. Risk Factors
- Premature Infants (or Low Birth Weight Infants)
- Multiple Gestation
- Maternal factors
- Advanced maternal age
- Maternal Preeclampsia
- Gestational Diabetes
- Placental abnormalities
V. Signs
- Lesions are NOT present at birth (or barely noticeable pale patch of skin)
- Contrast with Congenital Hemangioma (which is prominent at birth)
- Characteristics
- Erythematous (strawberry red to pale) or purple lobulated lesions
- Superficial or Deep (or combination)
- Localized, segmental or multiple
- Timing (see course below)
- Absent at birth (see above)
- Rapidly develop at age 1 to 3 months
- Proliferation typically ceases by 5 months
- Involution begins by 12 months
- Lesions disappear by 4 years in 80% of children
- Distribution
- Midline
- Beard region (airway)
VI. Imaging
- Abdominal Doppler Ultrasound indications
- Five of more Hemangiomas and suspected LUMBAR Syndrome
- Consider Imaging for Spinal Dysraphism
- See Cutaneous Signs of Dysraphism
- Hemangiomas within the perineum, gluteal cleft or lumbosacral region
- Head and Neck MRI Indications
- Suspected PHACE Syndrome
VII. Differential Diagnosis
- Cutaneous Signs of Spinal Dysraphism (e.g. sacral Hemangioma)
- Systemic disorders
- Thrombocytopenia
- Cardiac failure
- Congenital Hemangioma
- Rare compared with Infantile Hemangioma
- Benign vascular tumors present at birth
- Contrast with delayed appearance over the first month of life in Infantile Hemangioma
- Associated mutations GNAQ, GNA11
- Subtypes
- Rapidly Involuting (RICH, typically involutes by 6 to 14 months)
- Partially Involuting (PICH, decreases in size until 12 to 30 months)
- Noninvoluting (NICH)
- Complications
- Ulceration (esp. in large RICH lesions during involution)
- Mild, transient Thrombocytopenia
- Congestive Heart Failure (rare)
VIII. Associated Conditions
- Kasabach-Merritt Syndrome
- PHASEs Syndrome
- Posterior fossa malformations (Dandy Walker)
- Hemangioma (large, segmental, facial)
- Face segment distribution types (central face, V1, V2, V3, periauricular)
- Typically >5 cm diameter
- Arterial abnormalities
- Cardiac anomaly (e.g. coarctation of aorta)
- Eye or endocrine disorders
- Sternal cleft or supraumbilical raphe
- LUMBAR Syndrome
- Lower body Hemangiomas (or Cutaneous Signs of Spinal Dysraphism)
- Urogenital abnormalities
- Myelopathy
- Bony abnormalities
- Anorectal malformations
- Renal anomalies
- Tethered Cord with lumbosacral lesions
IX. Course
- Develops in first four weeks after birth
- Not typically visible at birth (outside of a faint, subtle, flat lesion)
- Proliferates until 9 to 12 months of age
- Rapid growth in first 3 months of age
- Typical Hemangioma reaches 80% of largest size by 5 months of age
- Proliferation after 12 months of age is uncommon
- Spontaneous, Gradual Involution (often incomplete)
- Findings
- Color shifts from bright red to dark red (or violaceous red to gray)
- Ulceration may occur following early white discoloration
- Involution Timing (10% per year)
- Age 5 years: 50% resolution
- Age 7 years: 70% resolution
- Age 9 years: 90% resolution
- Final appearance
- Residual atrophy, Hypopigmentation, Telangiectases, or scarring may persist (up to 50% of cases)
- Findings
X. Management: First-Line
- Best treatment efficacy with treatment started in first 10 weeks of life
- Indications for treatment relates to complication risk
- Disfigurement
- Skin Ulceration (16% of cases)
- May result in minor bleeding, infection or pain
- Functional Impairment (e.g. visual obstruction, overlying joint, sucking and feeding difficulty)
-
Propranolol
- FDA approved for age 5 weeks and older
- Well tolerated and very effective at facilitating Hemangioma involution when used in the first year of life
- Start in first months of life to prevent proliferation and continue until up to 12 to 18 months of age
- Observe for adverse effects (e.g. Hypotension, Sinus Bradycardia, Hypoglycemia)
- Adverse effects are higher with propranol than Atenolol, but Propranolol is more effective
- Considering observing infants in hospital during Propranolol initiation in higher risk infants
- Infants age <5 weeks corrected Gestational age
- Difficult social support systems
- Cardiopulmonary comorbid conditions
- Dosing
- Start 1 mg/kg/day divided orally twice daily
- May increase to 2 mg/kg/day after the first week
- Maximum dose 3 mg/kg/day
- Prevent Hypoglycemia by giving after feeding and witholding dose for decreased feeding or Vomiting
- References
- Topical Timolol
- Dose 0.5% gel ophthalmic solution 1-2 drops twice daily
XI. Management: Refractory (Interventions by Pediatric Dermatology)
- Compression garment or Coban Tape
- Hemangiomas on arms or legs
- Biosynthetic dressing every 24 hours
-
Prednisone
- Indicated where Hemangioma compresses eye, airway or other vital conditions
- Dose: 3 mg/kg daily for 6-12 weeks
- Continue until lesion stops growing or size decreases
- Interferon alfa (if refractory to Steroids)
- Sirolimus
- Topical Timolol Maleate
- Pulsed-dye laser therapy
XII. Management: Indications for referral or further evaluation
- See IHReS link under resources below
- Multiple Hemangiomas (>=5 small localized Hemangiomas)
- Obtain Ultrasound of the Abdomen to evaluate for gastrointestinal and Liver Lesions
- Visceral involvement (esp. liver) is termed diffuse Neonatal Hemangiomatosis
- Isolated Hemangiomas without visceral involvement are considered benign Neonatal Hemangiomatosis
- Deep Hemangiomas
- Large Hemangiomas
- Risk of high-output Heart Failure
- Sacral Hemangiomas
- Airway Hemangioma (beard distribution, subglottic Hemangioma)
- May present with Stridor, Hoarseness or recurrent croup-like episodes
- Associated with vascular engorgement and edema with airway obstructive symptoms
- Severe cases may require Endotracheal Intubation
- Airway Hemangiomas present in first 4 to 6 weeks of life (and enlarge through the first year of life)
- May appear on Lateral neck XRay with asymmetric smooth subglottic swelling
- Typically responds to Propranolol, and resolves in most cases within 5 to 7 days
- Refer to otolaryngology
- Rahbar (2004) Laryngoscope 114(11): 1880-91 [PubMed]
- May present with Stridor, Hoarseness or recurrent croup-like episodes
- Eyelid Hemangioma
- May block Vision
- PHASEs Syndrome (see above)
- Careful physical exam
- MRI/MRA of the head and neck
- Echocardiogram
- Opthalmology exam
- LUMBAR Syndrome
- Obtain MRI Lumbar Spine
XIII. Resources
- Infantile Hemangioma Referral Score Screening Tool (IHReS)
XIV. References
- Anderson (2024) Mayo Clinic Pediatric Days, lecture attended 1/16/2024
- McLaughlin (2008) Am Fam Physician 77: 56-60 [PubMed]
- Snyder (2024) Am Fam Physician 109(3): 212-6 [PubMed]
Images: Related links to external sites (from Bing)
Related Studies
| Definition (CHV) | A benign skin lesion consisting of dense, usually elevated masses of dilated blood vessels |
| Definition (CHV) | A benign skin lesion consisting of dense, usually elevated masses of dilated blood vessels |
| Definition (CHV) | A benign skin lesion consisting of dense, usually elevated masses of dilated blood vessels |
| Definition (CHV) | benign tumors that are made up of small blood vessels. |
| Definition (CHV) | benign tumors that are made up of small blood vessels. |
| Definition (CHV) | a benign tumor of the blood vessels that appears on skin |
| Definition (CHV) | a benign tumor of the blood vessels that appears on skin |
| Definition (CHV) | a benign tumor of the blood vessels that appears on skin |
| Definition (CHV) | a benign tumor of the blood vessels that appears on skin |
| Definition (NCI) | A benign vascular neoplasm characterized by the formation of capillary-sized or cavernous vascular channels. |
| Definition (NCI_CDISC) | A benign vascular neoplasm characterized by the formation of capillary-sized or cavernous vascular channels. |
| Definition (CSP) | extremely common benign tumor, occurring most commonly in infancy and childhood, made up of newly formed blood vessels, and resulting from malformation of angioblastic tissue of fetal life; can occur anywhere in the body but is most frequently noticed in the skin and subcutaneous tissues. |
| Definition (MSH) | A vascular anomaly due to proliferation of BLOOD VESSELS that forms a tumor-like mass. The common types involve CAPILLARIES and VEINS. It can occur anywhere in the body but is most frequently noticed in the SKIN and SUBCUTANEOUS TISSUE. (from Stedman, 27th ed, 2000) |
| Concepts | Neoplastic Process (T191) |
| MSH | D006391 |
| ICD9 | 228.00, 228.0 |
| ICD10 | D18.0 , M9120/0, D18.00 |
| SnomedCT | 2099007, 154625006, 189194008, 189199003, 93474003, 189863005, 269646001, 367337005, 189192007, 189193002, 254822005, 400210000, 253053003 |
| English | Hemangioma, Hemangioma, any site, Angioma - benign, Haemangioma unspecified site, Haemangioma, any site, [M]Haemangioma NOS, [M]Hemangioma NOS, hemangioma (physical finding), hemangioma (diagnosis), hemangioma, Hemangioma, no ICD-O subtype, Hemangioma unspecified site, Angioma NOS, Hemangioma [Disease/Finding], Haemangioma, unspecified site, Hemangioma, unspecified site, haemangiomas, angiomas, hemangiomas, benign hemangioma, angioma, benign hemangiomas, Hemangiomas, Hemangioma of unspecified site (disorder), [M]Hemangioma NOS (morphologic abnormality), Hemangioma NOS (disorder), Angioma - benign (disorder), Haemangioma (disorder), Haemangioma, site unspecified, Hemangioma, no ICD-O subtype (morphologic abnormality), Hemangioma, no International Classification of Diseases for Oncology subtype, Hemangioma, no International Classification of Diseases for Oncology subtype (morphologic abnormality), Hemangioma, site unspecified, ANGIOMA, HEMANGIOMA, BENIGN, BENIGN ANGIOMA, BENIGN HEMANGIOMA, Angioma, Benign haemangioma, Benign hemangioma, Haemangioma, Haemangioma - morphology, Hemangioma - morphology, Hemangioma (disorder), Benign hemangioma (morphologic abnormality), Angioma, NOS, Hemangioma, NOS, Haemangioma, NOS, Hemangioma (morphologic abnormality), Hemangioma of unspecified site, Haemangioma NOS, Haemangioma of unspecified site, Hemangioma NOS, Angioma (benign), Benign Angioma, Benign Hemangioma, haemangioma |
| French | ANGIOME, Angiome, Hémangiome SAI, Hémangiome, tout site, Hémangiome de site non précisé, Angiome (bénin), Hémangiome, site non précisé, Hémangiome à site non précisé, Hémangiome |
| Portuguese | ANGIOMA, Hemangioma de qualquer localização, Angioma, Hemangioma de local NE, Hemangioma NE, Angioma benigno, Hemangioma de localização NE, Hemangioma |
| Spanish | ANGIOMA, Hemangioma, cualquier localización, Hemangioma de localización no especificada, Angioma, Hemangioma NEOM, Angioma (benigno), Hemangioma ensitio no especificado, hemangioma, no clasificado como subtipo en CIE-O (anomalía morfológica), hemangioma, sin subtipo en CIE O, hemangioma, no clasificado como subtipo en CIE-O, hemangioma, sin subtipo en CIE O (anomalía morfológica), angioma benigno, hemangioma, SAI (trastorno), hemangioma de localización no especificada, hemangioma de localización no especificada (trastorno), [M]hemangioma, SAI (anomalía morfológica), [M]hemangioma, SAI, angioma benigno (trastorno), hemangioma, SAI, angioma, hemangioma (anomalía morfológica), hemangioma (trastorno), hemangioma benigno (anomalía morfológica), hemangioma benigno, hemangioma de localización no especificada (concepto no activo), hemangioma, morfología, hemangioma, sin localización específica, hemangioma, Hemangioma |
| Dutch | hemangioom NAO, hemangioom, elke plaats, hemangioom van niet-gespecificeerde plaats, hemangioom op elke plaats, angioom (benigne), hemangioom op een niet-gespecificeerde plaats, angioom, Hemangioom, elke lokalisatie, hemangioom, Angioom, Hemangioom |
| German | Haemangiom an unspezifischer Stelle, Haemangiom NNB, Angiom, Haemangiom, beliebige Stelle, Angiom (gutartig), ANGIOM, Haemangiom, jede Lokalisation, Haemangiom, Hämangiom |
| Italian | Emangioma NAS, Angioma, Emangioma, ogni sede, Emangioma di sede non specificata, Angioma (benigno), Emangioma |
| Japanese | 部位不明の血管腫, 血管腫（良性）, 血管腫、部位を問わない, 血管腫ＮＯＳ, ｹｯｶﾝｼｭNOS, ｹｯｶﾝｼｭ, ﾌﾞｲﾌﾒｲﾉｹｯｶﾝｼｭ, ｹｯｶﾝｼｭﾘｮｳｾｲ, ｹｯｶﾝｼｭﾌﾞｲｦﾄﾜﾅｲ, 血管腫-筋肉内, 血管腫-絨毛, 血管腫-組織細胞様, 組織細胞様血管腫, 絨毛血管腫, 血管腫, 筋肉内血管腫 |
| Swedish | Hemangiom |
| Czech | hemangiom, Hemangiom, jakékoli místo, Angiom (benigní), Hemangiom NOS, Hemangiom blíže neurčeného místa, Hemangiom, Angiom |
| Finnish | Hemangiooma |
| Russian | GEMANGIOMA, ANGIOMA GISTIOTSITOIDNAIA, KHORIOANGIOMA, ANGIOMA INTRAMUSKULIARNAIA, ANGIOMA, АНГИОМА, АНГИОМА ГИСТИОЦИТОИДНАЯ, АНГИОМА ИНТРАМУСКУЛЯРНАЯ, ГЕМАНГИОМА, ХОРИОАНГИОМА |
| Korean | 모든 부위의 혈관종 |
| Croatian | HEMANGIOM |
| Polish | Naczyniak krwionośny, Naczyniak |
| Hungarian | Haemangioma k.m.n., Jóindulatú érdaganat, bármely helyen, Jóindulatú érdaganat, Angioma (jóindulatú), Jóindulatú érdaganat k.m.n., Nem meghatározott elhelyezkedésű haemangioma, Nem meghatározott elhelyezkedésű érdaganat, Angioma, Haemangioma |
| Norwegian | Hemangiom, Blodkarsvulst, Blodkartumor |
Ontology: Strawberry nevus of skin (C0206733)
| Definition (NCI) | A capillary hemangioma that may regress spontaneously. It occurs in infants and children. |
| Definition (MSH) | A dull red, firm, dome-shaped hemangioma, sharply demarcated from surrounding skin, usually located on the head and neck, which grows rapidly and generally undergoes regression and involution without scarring. It is caused by proliferation of immature capillary vessels in active stroma, and is usually present at birth or occurs within the first two or three months of life. (Dorland, 27th ed) |
| Concepts | Neoplastic Process (T191) |
| MSH | D018324 |
| ICD10 | M9131/0 , Q82.5 |
| SnomedCT | 205560007, 402867006, 83343001, 56975005, 254206003 |
| English | Hemangioma, Capillary, Capillary Hemangiomas, Hemangiomas, Capillary, Capillary Hemangioma, STRAWBERRY MARK, Infantile Hemangioendothelioma, Strawberry Nevus, Juvenile Capillary Hemangioma, strawberry nevus (diagnosis), strawberry nevus (physical finding), capillary hemangioma (physical finding), strawberry nevus, capillary hemangioma, hemangioma capillary, strawberry nevus was observed, Cellular Hemangioma, Hemangioma, Capillary [Disease/Finding], capillary hemangiomas, angiomas strawberry, hemangiomas infantile, hemangiomas strawberry, strawberry birthmark, capillary haemangioma, mark strawberry, strawberry hemangioma, strawberry naevus, hemangioma strawberry, juvenile hemangioma, Mark;raspberry, Mark;strawberry, hemangioma simplex, infantile hemangioma, nevus capillary, birthmarks strawberry, marks strawberry, plexiform hemangioma, strawberry angioma, Infantile Hemangioma, Juvenile Hemangioma, Capillary hemangioma, Hemangioma simplex, Infantile hemangioma, Plexiform hemangioma, Juvenile hemangioma, Strawberry mark, Raspberry mark of skin, Strawberry hemangioma of skin, Strawberry mark of skin, Angiomatous naevus, Angiomatous naevus of skin, Angiomatous nevus, Angiomatous nevus of skin, Capillary haemangioma, Capillary haemangioma of skin, Capillary hemangioma of skin, Capillary naevus, Capillary naevus of skin, Capillary nevus, Capillary nevus of skin, Congenital vascular naevus, Congenital vascular nevus, Haemangioma simplex, Infantile haemangioma, Juvenile haemangioma, Plexiform haemangioma, Strawberry angioma, Strawberry naevus, Strawberry nevus, Strawberry haemangioma of skin, Strawberry birthmark, Capillary hemangioma (disorder), Capillary hemangioma (morphologic abnormality), Congenital vascular hamartoma, Congenital vascular nevus (disorder), Strawberry naevus of skin, Strawberry nevus of skin (disorder), Strawberry nevus of skin, capillary; hemangioma, capillary; nevus, hemangioma; capillary, hemangioma; infantile, hemangioma; juvenile, hemangioma; plexiform, hemangioma; simplex, infantile; hemangioma, juvenile; hemangioma, nevus; angiomatous, nevus; capillary, nevus; strawberry, plexiform; hemangioma, simplex; hemangioma, strawberry; nevus, angiomatous; nevus, Strawberry nevus of skin, NOS, raspberry mark, strawberry mark |
| Spanish | nevo en fresa de la piel, nevo angiomatoso cutáneo (trastorno), nevo frutilla, nevo angiomatoso cutáneo, Hemangioma simple, Nevus capilar, Marca de color fresa, Nevus en fresa, nevo en fresa de la piel (trastorno), nevo frutilla (trastorno), NEVUS EN FRESA, Nevus de color fresa, hemangioma capilar (anomalía morfológica), hemangioma capilar (trastorno), hemangioma capilar, hemangioma en fresa de la piel, hemangioma infantil, hemangioma juvenil, hemangioma plexiforme, hemangioma simple, marca en fresa de la piel, nevo angiomatoso, nevo capilar, nevus vascular congénito (trastorno), nevus vascular congénito, nevo vascular congénito (trastorno), nevo vascular congénito, Hemangioma Capilar |
| Dutch | aardbeinaevus, haemangioma simplex, aardbeivlek, hemangioom simplex, capillaire naevus, aardbei; naevus, angiomateus; naevus, capillair; hemangioom, capillair; naevus, hemangioom; capillair, hemangioom; infantiel, hemangioom; juveniel, hemangioom; plexiform, hemangioom; simplex, infantiel; hemangioom, juveniel; hemangioom, naevus; aardbei, naevus; angiomateus, naevus; capillair, plexiform; hemangioom, simplex; hemangioom, Capillair hemangioom, Hemangioom, capillair |
| French | Naevus capillaire, Angiome plan, Angiome simple, Névus fraise, Naevus fraise, Hémangiome simplex, ANGIOME TUBEREUX, Hémangiome capillaire |
| German | Erdbeermal, Haemangioma simplex, kapillaerer Naevus, Erdbeernaevus, Kapillarnaevus, FEUERMAL, Hämangiom, kapilläres |
| Italian | Nevo capillare, Nevo a fragola, Macchia di fragola, Emangioma simplex, Emangioma capillare |
| Portuguese | Nevo morango, Nevo cor de morango, Hemangioma simples, Nevo capilar, Sinal morango, ANGIOMA VIOLACEO, Hemangioma Capilar |
| Japanese | いちご状母斑, 単純血管腫, 毛細血管性母斑, ﾀﾝｼﾞｭﾝｹｯｶﾝｼｭ, ﾓｳｻｲｹｯｶﾝｾｲﾎﾞﾊﾝ, ｲﾁｺﾞｼﾞｮｳﾎﾞﾊﾝ, ｲﾁｺﾞｼﾞｮｳｹｯｶﾝｼｭ, いちご状血管腫 |
| Swedish | Hemangiom, kapillärt |
| Czech | hemangiom kapilární, Prostý hemangiom, Kapilární hemangion, Kapilární hemangiom, Kapilární névus, Jednoduchý hemangiom |
| Finnish | Kapillaarinen hemangiooma |
| Russian | GEMANGIOMA KAPILLIARNAIA, ГЕМАНГИОМА КАПИЛЛЯРНАЯ |
| Polish | Naczyniak krwionośny włośniczkowaty |
| Hungarian | Eper nevus, Capillaris naevus, Haemangioma simplex, Eper naevus, Dermalis haemangioma, Jóindulatú egyszerű érdaganat |
| Norwegian | Kapillært hemangiom |
Ontology: Congenital hemangioma (C0235753)
| Definition (NCI) | A hemangioma present at birth. |
| Concepts | Neoplastic Process (T191) |
| SnomedCT | 32361000119104, 400109004 |
| English | HAEMANGIOMA CONGENITAL, HEMANGIOMA CONGENITAL, Haemangioma congenital, Hemangioma congenital, congenital hemangiomas, congenital hemangioma, Congenital hemangioma (disorder), Congenital haemangioma, Congenital hemangioma (morphologic abnormality), Congenital hemangioma, Congenital Angioma, Congenital Hemangioma |
| Italian | Emangioma congenito |
| Dutch | congenitaal hemangioom, hemangioom, congenitaal |
| German | Haemangiom, kongenital, HAEMANGIOM ANGEBOREN, Haemangiom kongenital |
| Japanese | 先天性血管腫, ｾﾝﾃﾝｾｲｹｯｶﾝｼｭ |
| Portuguese | HEMANGIOMA CONGENITO, Hemangioma congénito |
| Spanish | HEMANGIOMA CONGENITO, hemangioma congénito (anomalía morfológica), hemangioma congénito, Hemangioma congénito |
| French | HEMANGIUME CONGENITALE, Hémangiome congénital |
| Czech | Vrozený hemangiom |
| Hungarian | Veleszületett haemangioma |
Ontology: Congenital hemangiomatosis (C0332977)
| Concepts | Congenital Abnormality (T019) |
| SnomedCT | 88120000 |
| English | Congenital angiomatosis, Congenital haemangiomatosis, Congenital hemangiomatosis (morphologic abnormality), Congenital hemangiomatosis |
| Spanish | angiomatosis congénita, hemangiomatosis congénita (anomalía morfológica), hemangiomatosis congénita |
Ontology: HEMANGIOMA, CAPILLARY INFANTILE (C1865871)
| Concepts | Disease or Syndrome (T047) |
| MSH | C535860 |
| English | HCI, HEMANGIOMA, HEREDITARY CAPILLARY, HEMANGIOMA, CAPILLARY INFANTILE, Hemangioma, capillary infantile, Hemangioma, hereditary capillary |
