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CONGENITAL VASCULAR DISEASES, PRESENTATIONS pptx
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- 2. • Vascular anomaliescan be broken down into two major categories: Tumors and malformations • There are several different vascular tumors, but infantile hemangiomas are the most common.
- 3. Others vascular tumorsinclude; • Tuft angiomas • Pyogenic granulomas • Angiosarcomas • Kaposiform hemangioendotheliomas. Management depends on their medical history, diagnosis, and most importantly their location.
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- 6. Introduction • Tumors andtumor-like conditions of the vascular system are divided into three categories according to their degree of malignancy: • Childhood vascular tumors may be classified into four groups. • Benign tumors • Intermediate tumors that may spread locally • Intermediate tumors that may spread to other parts of the body • Malignant tumors • vascular tumor arise from endothelial cells and supporting cells • Classification of these lesions is still the source of much controversy and is based on clinical appearance, pathology, embryology, and endothelial growth character
- 7. Differences between benignand malignant vascular tumors • Benign Vascular channels with blood cells or lymph Monolayer of normal appearing ECF. • Malignant. Cytologic atypia Proliferative Vessels not well-organize
- 8. Benign vascular tumors •Infantile hemangioma. • Congenital hemangioma. • Benign vascular tumors of the liver. • Spindle cell hemangioma. • Epithelioid hemangioma. • Pyogenic granuloma (lobular capillary hemangioma). • Angiofibroma. • Juvenile nasopharyngeal angiofibroma.
- 9. MALIGNANT VASCULAR TUMORS •Epithelioid hemangioendothelioma • Angiosarcoma: Idiopathic (Head and neck), Associated with chronic lymphedema (Stewart-Treves), Post radiation, Epithelioid
- 10. Tests and treatmentoverview of vascular tumors • Tests • U/s scan • CT scan • MRI • X-ray • Biopsy • Treatment overview • Beta-blocker therapy • Surgery (Excision, Laser surgery, Post radiation hepatectomy) • Photocoagulation • Cryotherapy • Embolization • Chemotherapy • Sclerotherapy • Radiation therapy • Targeted therapy • Immunotherapy • Other drug therapy • Observation
- 11. Benign vascular tumors 1.INFANTILE/JUVENILE/STRAWBERRY/CAPILLARY HAEMANGIOMA • It contains immature Vaso formative tissues. There will be eventually intravascular thrombosis, fibrosis and mast cell infiltration. Most common type of benign vascular tumor in children. • IH form when immature cells that are meant to form blood vessels form a tumor instead. • AKA "strawberry mark." • These tumors are not usually seen at birth • Onset is few weeks after birth with biphasic growth showing initial rapid growth with gradual involution over 5-7 years.
- 12. The hemangiomas slowlyfade away over the next several years, but a red mark or loose or wrinkled skin may remain. They are usually on the head and neck but can be anywhere on or in the body. It is commonly seen in skin and subcutaneous tissue but can occur anywhere in the body like in liver, brain, lungs or other organs Hemangiomas may appear as a single lesion, one or more lesions spread over a larger area of the body, or multiple lesions in more than one part of the body. Lesions that are spread over a larger area of the body or multiple lesions are more likely to cause problems.
- 14. • Infantile hemangioma withminimal or arrested growth (IH-MAG) is a certain type of infantile hemangioma that is seen at birth and does not tend to get bigger. • The lesion appears as light and dark areas of redness in the skin.
- 15. Cont.….. • Involution causescolor fading, softness, shrinkage leaving crepe paper like area. • Often large hemangiomas may be associated with visceral anomalies. • Head and neck hemangioma is associated with ocular and intracranial anomalies; sacral with spinal dysraphism. • Multiple cutaneous hemangiomas may be associated with hemangioma of liver causing hepatomegaly, cardiac failure (CCF}, anemia.
- 16. Variants of Capillaryhemangioma Lobular capillary hemangioma (pyogenic granuloma). It’s a common proliferative lesion, related to trauma. Site: superficial dermis like lips, gums(EPULIS), hands, fingers, Tufted angioma Acquired in childhood (1-5 yrs), congenital forms exist May persist unchanged or regress completely in few yrs Resembles cellular JCH but discontinuous capillary lobules.
- 17. Cherry angioma •Suspected involuted LCH • Bright red, soft, raised, dome-shaped lesion of varying in size often present in large numbers. Microscopy: • Numerous capillaries with narrow lumina • Prominent endothelial cells, arranged in lobular fashion in subpapillary region • Intercapillary stroma edema and collagen
- 18. Pyogenic Granuloma • Pyogenicgranuloma also called lobular capillary hemangioma. • It is most common in older children but may occur at any age. • The lesions are sometimes caused by injury or from the use of certain medicines, including birth control pills and retinoids. • Some lesions may be associated with capillary malformations. Usually there is only one lesion, but sometimes multiple lesions occur in the same area or the lesions may spread to other areas of the body.
- 19. Signs • Pyogenic granulomasare raised, bright red lesions that may be small or large and smooth or bumpy. • They grow quickly over weeks to months and may bleed a lot. • These lesions are usually on the surface of the skin, but may form in the tissues below the skin and look like other vascular lesions.
- 20. Risk factors • Girls. •White patients. • Premature babies. • Twins, triplets, or other multiple births. • Babies of mothers who are older at the time of the pregnancy, have pre-eclampsia • Positive family history • PHACE syndrome
- 21. Diagnosis • Physical examand history are usually all that are needed to diagnose infantile hemangiomas. • USS; • If the hemangioma is deeper inside the body with no change to the skin, are spread across a large area of the body. • If five or more on the skin pt should have an USS of the liver for liver hemangioma. • If the hemangiomas are part of a syndrome, more tests may be done, such as an echocardiogram, MRI, magnetic resonance angiogram, and eye exam. • Biopsy for suspicious leisoins
- 22. Treatment • Most hemangiomasfade and shrink without treatment. Treatment may include the following: • Steroid therapy, before beta-blocker therapy is begun or when beta-blockers cannot be used. Rapidly growing hemangioma may need systemic/oral and intralesional Antiangiogenic interferon 2a may be useful. • Topical beta-blocker therapy (propranolol or timolol). • Excision or curettage. • Photocoagulation. • Laser surgery, including pulsed dye laser surgery, may be used for ulcerative hemangiomas • Some pyogenic granulomas often come back after RX
- 23. • Sclerotherapy/cryotherapy/C02 snowtherapy cause unpleasant scarring. • Life-threatening platelet trapping may be controlled by cyclophosphamide chemotherapy.
- 24. Congenital hemangiomas • Congenitalhemangioma is a benign vascular tumor that begins forming before birth and is fully formed when the baby is born, it's non progressive. • They're usually on the skin but can be in another organ. • A congenital hemangioma may occur as a rash of purple spots and the skin around the spot may be lighter
- 25. Treatment Treatment of rapidlyinvoluting congenital hemangioma and partial involuting congenital hemangioma may include the following: • Observation only. Treatment of non-involuting congenital hemangioma may include the following: • Surgery to remove the tumor, depending on where it is and whether it is causing symptoms.
- 26. Malignant vascular tumors EpithelioidHemangioendothelioma • Common in adults aged 30 to 50 years. • Can occur in children. • They usually occur in the liver, lung, or in bone. • They may be fast growing or slow growing.
- 27. Signs • On theskin, the tumors can be raised and rounded or flat, red- brown patches that feel warm. • In the lung ; • Chest pain. • Spitting up blood. • Anemia (weakness, feeling tired, or looking pale). • Trouble breathing (from scarred lung tissue).
- 28. Treatment • Treatment ofslow-growing epithelioid hemangioendotheliomas includes the following: • Observation. • Treatment of fast-growing epithelioid hemangioendotheliomas may include the following: • Surgery. • Immunotherapy (interferon) and targeted therapy (thalidomide, sorafenib, pazopanib, sirolimus) for tumors that are likely to spread. • Chemotherapy. • Radiation therapy. • Total hepatectomy and liver transplant when the tumor is in the liver.
- 29. Angiosarcoma • Angiosarcomas arefast-growing tumors that form in blood vessels or lymph vessels in any part of the body, usually in soft tissue. • Most angiosarcomas are in or near the skin. Those in deeper soft tissue can form in the liver, spleen, and lung. • These tumors are very rare in children. Children sometimes have more than one tumor in the skin and/or liver.
- 30. Treatment • Surgery tocompletely remove the tumor. • A combination of surgery, chemotherapy, and radiation therapy for angiosarcomas that have spread. • Targeted therapy (bevacizumab) and chemotherapy for angiosarcomas that began as infantile hemangiomas. • A clinical trial of immunotherapy (nivolumab and ipilimumab).
- 31. Risk factors • Beingexposed to radiation. • Chronic (long-term) lymphedema, a condition in which extra lymph fluid builds up in tissues and causes swelling. • Having a benign vascular tumor. A benign tumor, such as a hemangioma, may become an angiosarcoma but this is rare.
- 32. Treatment • A combinationof surgery, chemotherapy, and radiation therapy for angiosarcomas that have spread. • Targeted therapy (bevacizumab) and chemotherapy for angiosarcomas that began as infantile hemangiomas. • A clinical trial of immunotherapy (nivolumab and ipilimumab).
- 33. Reference • Postgraduate VascularSurgery 10th edition • Srb manual of surgery 5th edition • Teach me anatomy • Uptodate