HAEMANGIOMA

Issah J. kiswagala

(M.B.B.S).

DEFINITION

• Haemangioma is a usually benign vascular tumor derived from blood vessel cell types
• By definition, It is a collection of small blood vessels that form a lump
• A hemangioma can occur anywhere on the body, but most commonly appears on. 
• It is commonly seen in skin (the face, scalp, chest or back) and subcutaneous tissue but can occur anywhere in the body like in liver, brain, lungs or other organs.

PHYSIOLOGICAL ANATOMY

EPIDEMIOLOGY

• Hemangioma are common amongst the vascular tumors, with an overall incidence of about 4 - 5%.
• Infantile hemangioma occurs more commonly in females than in males girls (3 : 1).
• Hemangioma occurs more frequently in white infants than in infants of other ethnic groups.
• Prematurity and low birth weight also correlate with an increased risk of hemangioma. Low birth weight seems to be more significant than prematurity.

• Incidence of hepatic hemangiomas is approximately 2%
• Women, especially with a history of multiparity, are affected more often than men. The female-to-male ratio is 4-6:1 (hepatic haemangioma).
• Hepatic hemangiomas can occur at all ages, but most are diagnosed in individuals aged 30-50 years. Female patients often present at a younger age and with larger tumors.

RISK FACTORS/ AETIOLOGY

• Low birth weight was shown to be the most significant associated risk factor for the development of infantile hemangioma.
• Maternal demographic factors associated with an increased risk for infantile hemangioma include:
• Advanced maternal age
• Multiple gestation
• Pre-eclampsia
• Maternal history of infertility
• Infertility treatment
• Chorionic villus sampling.

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CLASSIFICATION

Hemangiomas are benign (noncancerous) vascular tumors, and many different types occur. 

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1. Infantile hemangioma most common benign (noncancerous) tumor found in children
2. Cavernous liver haemangioma or hepatic hemangioma (the most common liver tumour)
3. Congenital hemangioma (usually visible on the skin at birth, unlike infantile hemangioma, which appear later)

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NOTE

Hemangioma can be classified either according to the origin or behavior of the lesion

CAPILLARY HAEMANGIOMA

1. Salmon patch (stork bite): It presents at birth.
• It commonly occurs in nape of the neck (50%), face, scalp and limbs.
• It usually involves wide area of skin.
• With age, it goes for spontaneous regression and disappears completely (usually in one year). Hence masterly inactivity is the treatment.

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2. Strawberry haemangioma: It is the commonest haemangioma.
• It may start at birth or child is normal at birth; between one to three weeks it appears as red mark which rapidly increases in size in 3 months to form strawberry/raspberry haemangioma.

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• It is a true capillary haemangioma.
• It is 20 times more common than port wine stain.
• It is common in white girls. Male to female ratio 3 : 1.
• It is common in head and neck region.
• It is clinically compressible, warm with bluish surface.
• Bleeding can occur after minor trauma and also ulceration.
• It involves skin, subcutaneous tissues and often muscles also.
• After one year of age, it slowly begins to disappear, and completely in 7-8 years (70% in 7 years).

3. Port wine stain (Naevus flammeus):
• It presents at birth and persists throughout life without any change.
• Spontaneous regression will not occur.
• It presents as smooth, flat, reddish blue/intensely purple area; common in head, neck and face; often with maxillary and mandibular dermatomes of 5th cranial nerve. Eventually surface becomes nodular and keratotic.
• It needs treatment – laser (pulsed dye/diode); excision and grafting; cosmetic coverage. Expected result by treatment is not possible many times.

• Indications for surgery or intervention–
• Uncontrolled growth
• Functional impairment like vision or hearing.
• Accidental haemorrhage

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Treatment

• Allowed for spontaneous regression.
• Otherwise by laser therapy – pulsed dye laser (diode laser).
• CO2 snow therapy.
• Sclerosant therapy.
• Steroid - oral / intralesional / systemic.
• Therapeutic embolisation

CAVERNOUS HAEMANGIOMA

• It is present at birth and consists of a multiple venous channels.
• Its size increases gradually and may cause problems.
• It often contains feeding vessels which is of surgical importance.

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• Sites: Head, neck, face, limbs, tongue, liver and other internal organs.
• Cavernous haemangioma is often mixed with lymphatic component also (mixed vascular and lymphatic).
• Large or multiple cavernous haemangiomas can cause congestive heart failure (hyperdynamic) due to shunting of large quantity of blood.

CLINICAL FEATURES

• It is smooth, soft, well-localised, warm, fluctuant, compressible, non pulsatile swelling with bluish surface occurring in skin and subcutaneous tissue (often in mucosa like oral cavity) without any transillumination.
• Compressibility and bluish surface is diagnostic. When swelling is pressed it reduces partially/often completely but when pressure is released it slowly attains its original size and shape. Vascular and lymphatic malformations are compressible.
• It is usually nontender unless it gets infected or undergoes thrombosis or causes haemorrhage

DIFFERENTIAL DIAGNOSIS

• Lymphangioma – It is brilliantly transilluminant unless it is infected or fibrosed.
• Lipoma, cold abscess, lymph cyst – clinically it is easier to differentiate.

INVESTIGATIONS

• Ultrasound.
• Doppler.
• Angiogram to find out feeding vessel.
• Platelet count.
• MRI/MR angiogram to see feeding vessels and deeper extension.

TREATMENT

• Sclerosant therapy – It is the initial first line of therapy. It causes aseptic thrombosis and fibrosis of the cavernous haemangioma with less vascularity and smaller size.
• Later excision of the lesion is done.
• Ligation of feeding artery and often at later stage excision is done once haemangioma shrinks.
• If small and located in accessible area, excision is the initial therapy.

COMPLICATIONS

• Haemorrhage.
• DIC.
• Thrombosis.
• Infection, ulceration and septicaemia.
• Erosion into the adjacent bone.
• High output cardiac failure.

CIRSOID ANEURYSM

• It is a rare variant of capillary haemangioma occurring in skin, beneath which abnormal artery communicates with the distended veins.
• Commonly seen in superficial temporal artery and its branches.
• Often the underlying bone gets thinned out due to pressure.
• Occasionally extends into the cranial cavity.
• Ulceration is the eventual problem which will lead to uncontrollable haemorrhage

TREATMENT�

• Ligation of feeding artery and excision of lesion, often
• requires preliminary ligation of external carotid artery.
• Intracranial extension requires formal neurosurgical approach.

PROGNOSIS

Is good for all haemangioma