Infantile Haemagioma

Infantile Haemagioma

Infantile haemangiomas are benign proliferative tumours. This article details their aetiology, clinical features, investigations and management.

Summary Card


Define
A benign vascular tumour of proliferative endothelium.

Aetiology
Premature Caucasian females with low birth weight and somatic mutations.

Clinical Features
Superficial red, deep blue, focal or segmental lesions appear after birth or as a 'herald patch' and grow faster than the patient.

Phases of Growth
An infantile hemangioma exhibits 3 phases of growth: proliferating, involuting and involuted.

Complications
Ulceration, infection, and obstruction to the eye, ear and airway. It is associated with PHACES.

Investigations
MRI and ultrasound are common imaging modalities to assess infantile hemangioma.

Management
Active surveillance, symptomatic management, medications, laser and surgery can all be considered.


Definition of Infantile Haemangioma

An infantile haemangioma is a benign vascular tumour of proliferative endothelium. It exhibits proliferating, involuting, and involuted phases. They are classified based on two facts:

  • Depth: superficial, deep, mixed
  • Pattern: Focal, multifocal, segmental

Definition of Infantile Haemangioma

💡
Tip: Focal is defined as one cutaneous region. Segmental tumours have “geographic patterning”. 


Aetiology of Infantile Haemangioma

Infantile haemangiomas generally occur in premature Caucasian females with low birth weight. Current genetic theories are based on intrinsic and extrinsic theories.

  • Intrinsic: somatic mutation in one progenitor cell
  • Extrinsic: haemangioma endothelial cells respond to VEGF or Interferon B.

Infantile Haemangiomas

😎
Fun Fact: Infantile haemangiomas are GLUT1+. This is differs them from congenital haemangiomas. 


Clinical Features of Infantile Haemangioma


Key Point

Infantile Haemangiomas can be superifical red, deep blue, focal or segmental. They generally appear after birth or as a 'herald patch' and grow faster than the patient.


Parents may notice a ‘herald patch’ at birth. But the majority of tumours appear within 1 month of birth. They do not grow in proportion to the patient.

The clinical appearance of an infantile haemangioma is dependent on its depth and pattern.

Depth

In reality, infantile haemangiomas over have a mixed component.

  • Superficial: bright red, well-demarcated, elevated, non-compressible
  • Deep: blue hue, poorly defined, warm

Pattern

  • Focal: one cutaneous region
  • Segmental: geographic patterning, such as the “bearded appearance”.

These appearances of infantile haemangiomas are illustrated below.

💡
Tip: Unlike infantile haemangiomas, congenital haemangiomas are completely formed at birth, rare, grow in proportion, and are GLUT1 negative. 


Phases of Infantile Haemangioma


Key Point

An infantile hemangioma exhibits 3 phases of growth: proliferating, involuting and involuted.

Proliferating Phase

  • Increased endothelial activity and angiogenesis due to VEGF and bFGF.
  • Usually lasts for <1 year.
  • Superficial tumours are red and raised, deep tumours become bluer.

Involuting Phase

  • Reduced endothelial activity and angiogenesis due to TIMP1
  • Spontaneous involution occurs in the majority of patients (~90%)
  • Colours become paler

Involuted Phase

  • The skin attempts to return to normal due to sparse capillaries and veins
  • 1/3 by age 3, 50% by 5 years and nearly all by  age 10.
  • There may be residual telangiectasia, atrophy and a “fibrofatty residuum”.

These phases of infantile hemangioma can be visualised in the illustration below.

Phases of Infantile Haemangioma


Complications of Infantile Haemangioma


Key Point

The primary issues relating to infantile haemangiomas are ulceration, infection, and aesthetic concerns—other significant complications related to eye, ear and airway obstruction.


The most common complication of infantile haemangiomas is ulceration. Other complications to be aware of are related to obstruction and PHACES.

Obstruction

  • Eye: amblyopia (lazy eye), anisometropia (unequal refraction), strabismus (cross-eyed)
  • Ear: deformation, conductive hearing loss due to blockage of external acoustic meatus.
  • Airway: can occur in “beard” facial haemangioma or subglottic lesions. Consider an endoscopy!

Beard distribution in infantile haemangiomas 

PHACES Association

Infantile haemangiomas can be associated with other anomalies. These include:

  • Posterior Cranial Fossa: Dandy-Walker Complex, hypoplasia, microcephaly
  • Haemangiomas: segmental, cervicofacial region
  • Arterial
  • Cardiac: coarctation, aneurysm, patent ductus arteriosus
  • Eye: “morning glory” discs, retinal vascular issues, Horner Syndrome
  • Sternal defects or supraumbilical raphe

Dandy-Walker Complex

😎
Fun Fact: Dandy-Walker is a congenital brain malformation in which the cerebellar vermis does not fully form. This results in an increased CSF in the 4th ventricle and posterior fossa.


Investigations


Key Point

MRI and ultrasound are common imaging modalities to assess infantile hemangioma.


Radiology:

  • MRI: differentiate slow-flow and fast-flow malformations, brain in PHACES.
  • X-Ray: skeletal growth disturbances and phleboliths in venous malformations.
  • Colour Doppler: assess superficial lesions and blood flow velocity.
  • Liver ultrasound ± Biopsy: hepatic haemangiomas if >5 skin lesions
  • Angiography: therapeutic embolization after initial workup.

Doppler of Vascular Anomaly

Bedside Investigations

  • Airway evaluation with Endoscopy.
  • Cardiac: ECHO, ECG.
  • Eyes: Ophthalmic Exam (PHACES)
  • Bloods: Full Blood Count + other baseline bloods.  


Management of Infantile Haemangioma


Key Point

Through an MDT approach, infantile haemangiomas can be treated with medications (eg. B-blockers, steroids), Laser, surgical debulking, or active surveillance.


The management of infantile haemangiomas is centred on a multi-disciplinary team involving surgeons, dermatologists, interventional radiologists and patient education. All patients should undergo active surveillance to focus on education, and support measures for pain, ulceration or infections

Treatment options include:

  • Medical: Beta-blockers, steroids, chemotherapeutic agents
  • Intervention: steroids, laser, surgical debulking, post-involution correct

Propranolol

This beta-blocker has been used as an ‘off-label’ treatment option for infantile haemangiomas since the 2008 NEJM publication. Important points about this treatment option include:

  • Dose: 1-2mg/kg for 12 months, consult local guidelines.
  • Pre-investigations: Vital Signs, ECG, glucose, ECHO
  • Contraindications: Sick sinus syndrome, Heart block (2nd or 3rd degree)
  • Side effects: hypotension, hypoglycaemia, bronchospasm, hypothermia.

💡
Tip: Topical B-blockers are an option if the patient is not suitable for oral propranolol.

Steroids

Oral Prednisolone is another option to be used instead or in conjunction with B-blockers. Important points about this treatment option include:

  • Dose: 12 months; consult local guidelines.
  • Side effects: GI upset, behavioural changes, immunosuppression, adrenal suppression.
  • Tapering: there can be “rebound growth” during the steroid tapering.

💡
Tip: Intralesional steroid injections can be considered in localised superficial haemangiomas.

Chemotherapeutic Agents

  • Vincristine: as per the oncology team
  • Interferon alpha-2a: consider if resistant to propranolol and steroids

Laser

Lasers are generally reserved for flat, superficial lesions with residual telangiectasia that have undergone involution.

  • Argon: 488nm
  • Pulse dye: 585nm

Surgery

There is a potential role for surgery in the acute or involuted phase.

  • Acute: a large haemangioma may require
  • Involuted: scar correction, removal of fibrofatty residuum


In this Article
Great! Next, complete checkout for full access to thePlasticsFella.
Welcome back! You've successfully signed in.
You've successfully subscribed to thePlasticsFella.
Success! Your account is fully activated, you now have access to all content.
Success! Your billing info has been updated.
Your billing was not updated.