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HAEMANGIOMA OF INFANCY
Habakuk larry omondi
DEFINITION
• It is a localized proliferative process of
angioblastic mesenchyme.
EPIDEMIOLOGY
• It is the most common tumour of infancy.
• Incidence:
– 1-2% in newborns.
– 10% by the age of 1 year.
• Male to female ratio is 3:1.
ETIOLOGY AND PATHOGENESIS
• Clonal expansions of endothelial cells that
may result from somatic mutations of genes
regulating endothelial cell proliferation.
CLINICAL PRESENTATION
• Proliferative phase occurs during the first year.
• Involution phase occurs during 2-6 years and is
complete by 10 years.
• Skin lesions:
– Soft, bright red to deep purple
– Compressible
– Nodule or plaque, 1-8 cm wide
– Ulceration may occur
– White to gray area appears on the surface with onset
of regression.
• Distribution :
– Solitary and localized or may extend over an entire
region.
– Head and neck:50%
– Trunk: 25%
– Legs
– Oral mucosa
SPECIAL PRESENTATIONS
1) DEEP HAEMANGIOMA
• In lower dermis and subcutaneous fat.
• Localized, firm, rubbery mass.
• Bluish or normal skin colour.
• Telangiectasia occurs in overlying skin.
• Can be combined with superficial
haemangioma.
• Does not involute as well as superficial type.
2) MULTIPLE HAEMANGIOMA OF INFANCY
• Small (<2cm) and multiple.
• Cherry red
• Papular
• Involves :
– skin alone(benign cutaneous haemangiomatosis)
– or skin and internal organs (diffuse neonatal
haemangiomatosis).
3) CONGENITAL HEMANGIOMA
• Develop in utero.
• 2 types:
– Rapidly involuting congenital haemangiomas
(RICH).
– Non-involuting congenital haemangiomas (NICH).
• Presentation:
– Violaceous tumours.
– Overlying telangiectasia.
– Large veins in periphery.
• Or as:
– Red-violaceous plaques invading deeper tissues
• NICH are fast flow haemangiomas requiring
surgery.
DIAGNOSIS
• Diascopy : lesion does not blanch completely.
• Arteriography to demonstrate fast flow
• Doppler ultrasound
• MRI
• Clinical findings
• Lab: dermopathology
– Proliferation of endothelial cells in dermis and/or
subcutaneous tissue.
– GLUT-1 immunoreactivity is present
TREATMENT
• Observe and wait for spontaneous involution.
• Medical care
– Intralesional and systemic high dose
glucocorticoids
– Interferon alfa
– Beta blocker (propranolol)
• Surgical care
– Continuous phase or pulsed dye laser
– cryosurgery
COMPLICATIONS
• Ulceration
• Airway obstruction
• Visual obstruction
• Psychological problems
PROGNOSIS
• In 80%, there is no skin changes after
involution.
• Atrophy, depigmentation, telangiectasia and
scarring occurs in 20%.

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Haemangioma of infancy

  • 2. DEFINITION • It is a localized proliferative process of angioblastic mesenchyme.
  • 3. EPIDEMIOLOGY • It is the most common tumour of infancy. • Incidence: – 1-2% in newborns. – 10% by the age of 1 year. • Male to female ratio is 3:1.
  • 4. ETIOLOGY AND PATHOGENESIS • Clonal expansions of endothelial cells that may result from somatic mutations of genes regulating endothelial cell proliferation.
  • 5. CLINICAL PRESENTATION • Proliferative phase occurs during the first year. • Involution phase occurs during 2-6 years and is complete by 10 years. • Skin lesions: – Soft, bright red to deep purple – Compressible – Nodule or plaque, 1-8 cm wide – Ulceration may occur – White to gray area appears on the surface with onset of regression.
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  • 7. • Distribution : – Solitary and localized or may extend over an entire region. – Head and neck:50% – Trunk: 25% – Legs – Oral mucosa
  • 8. SPECIAL PRESENTATIONS 1) DEEP HAEMANGIOMA • In lower dermis and subcutaneous fat. • Localized, firm, rubbery mass. • Bluish or normal skin colour. • Telangiectasia occurs in overlying skin. • Can be combined with superficial haemangioma. • Does not involute as well as superficial type.
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  • 10. 2) MULTIPLE HAEMANGIOMA OF INFANCY • Small (<2cm) and multiple. • Cherry red • Papular • Involves : – skin alone(benign cutaneous haemangiomatosis) – or skin and internal organs (diffuse neonatal haemangiomatosis).
  • 11. 3) CONGENITAL HEMANGIOMA • Develop in utero. • 2 types: – Rapidly involuting congenital haemangiomas (RICH). – Non-involuting congenital haemangiomas (NICH).
  • 12. • Presentation: – Violaceous tumours. – Overlying telangiectasia. – Large veins in periphery. • Or as: – Red-violaceous plaques invading deeper tissues • NICH are fast flow haemangiomas requiring surgery.
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  • 16. DIAGNOSIS • Diascopy : lesion does not blanch completely. • Arteriography to demonstrate fast flow • Doppler ultrasound • MRI • Clinical findings • Lab: dermopathology – Proliferation of endothelial cells in dermis and/or subcutaneous tissue. – GLUT-1 immunoreactivity is present
  • 17. TREATMENT • Observe and wait for spontaneous involution. • Medical care – Intralesional and systemic high dose glucocorticoids – Interferon alfa – Beta blocker (propranolol) • Surgical care – Continuous phase or pulsed dye laser – cryosurgery
  • 18. COMPLICATIONS • Ulceration • Airway obstruction • Visual obstruction • Psychological problems
  • 19. PROGNOSIS • In 80%, there is no skin changes after involution. • Atrophy, depigmentation, telangiectasia and scarring occurs in 20%.