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Hemangiomas and Vascular Malformations Yağmur AYDIN, M.D. University of Istanbul, Cerrahpasa Medical Faculty Department of Plastic, Reconst. and Aesthetic.

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Presentation on theme: "Hemangiomas and Vascular Malformations Yağmur AYDIN, M.D. University of Istanbul, Cerrahpasa Medical Faculty Department of Plastic, Reconst. and Aesthetic."— Presentation transcript:

1 Hemangiomas and Vascular Malformations Yağmur AYDIN, M.D. University of Istanbul, Cerrahpasa Medical Faculty Department of Plastic, Reconst. and Aesthetic Surgery

2 Vascular Anomalies In U.S, babies with vascular anomailes are born in a year 1of 10 children has vascular anomaly A common mistake has been done with the naming – Strawbery, cavernous, capillary hemangioma Mulliken and Glowacki in 1982 – classification according to the biological characteristics (endothelial properties)

3 Vascular Anomalies Tumors – Hemangioma – pyogenic granuloma – Kaposiphorm hemangio-endothelioma Malformations – Capillary – Lymphatic – Venous – Arteriovenous – combined

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5 Hemangioma The most common tumor of infancy and childhood (4- 10%) 3-5 times more seen in girls More seen in premature infants (<1200 grams% 23) Not frequent in darker-skinned babies Usually occurs in first 2 weeks after birth Initially, a pale-colored, telangiectatic or macular red stain or purple-colored stain Single lesion in 80%, 20% more than one lesion In patients with more than one lesion accompanies other system hemangiomas ( liver etc.)

6 Clinical Appearance Lesions located in the superficial dermis – Hard, shiny dark-red coloured bulky lesion Lesion located in deep dermis, subcutaneous fat or muscle – Showing a slight bulking, hot, bluish coloured lesion

7 The incidence of hemangiomas Craniofacial area (60%) The body (25%) Extremities (15%)

8 3 phases of hemangiomas Proliferation period (0-1 years) – Rapidly dividing endothelial cells İnvolution period (1-5 years) – Reduced endothelial proliferation, increased apoptosis, fibrofatty replacement, reduced tumor volume, skin softening Involution completion term (> 5 years) – Thin veins and capillaries that drain current fibrofatty islets

9 Proliferation period: – Rapid growth (up to months) Involution period (1-7 years) – Growth slows down,compatible with the child's growth rate – Fading of the skin starting from the center of the lesion,softening – The color disappears untill 5-7 years – Normal skin takes place in nearly 50% of children

10 Differential Diagnosis Lymphatic malformation –deep located hemangioma (neck, axilla) Capillary malformation - macular hemangioma Vascular tumors of infancy - Fibrosarcoma etc. – Radiological studies – Biopsy

11 Clinical evaluation Clear and open dialogue with the family Confirm the diagnosis Documentation with photos The need for medical or surgical treatment Other diagnostic studies to investigate the extension of hemangiomas and other anomalies Provide support groups and publications for family

12 Complications Ulceration, bleeding Infection Visual impairment Airway obstruction Obstruction in the ear canal Congestive heart failure – diffuse neonatal hemangiomathosis – large visceral hemangiomas

13 Problems Hemangiomas located in head&neck (PHACES syndrome.) Hemangiomas covering the visual area Perioral location Respiratory distress (subglottic hemangioma U lcer

14 hemangioma covering the visual field in 2 months old infant Rapid reduction after oral and intra lesional corticosteroid injection Residue mass at age 1,atrophy, telangiectasia

15 Treatment Follow up and observation Training and convincing the family Systemic corticosteroid Second-generation drugs (vincristine, interferon alpha) Laser therapy - pulsed-dye laser – ulcerated hemanjyom – Remaining telangiectasia after involution

16 Hemangiomas requiring treatment Covering the visual area, the air way, the ear canal Leading to congestive heart failure Showing ulceration and bleeding

17 Treatment Dangerous and life-threatening complications occur in 10% of patient The first option in medical treatment application of corticosteroids (90% response) Corticosteroid – Topical / injection into the lesion Triamcinolone 3-5 mg / kg 3-5 times application (6-8 weeks apart) – systemic application Oral prednisone or prednisolone 2-3 mg / kg /day Once every 2-4 weeks (10-12 months)

18 Other medical treatment agents(vicristine, interpheron alpha) Cases non –responsive to corticosteroid therapy If long-term use of corticosteroids is contraindicated If complications develop after the use of corticosteroids In cases that the family does not want to use (rare)

19 Systemic steroid treatment

20 Ulcer local diffuse

21 Ulcer Treatment Dressings Corticosteroid Laser( flashlamp dye laser) Total excision (if primary closure is possible)

22 Hemangioma Ulceration

23 residual athrophic tissue and wrinkled skin after involution of hemangioma

24 Clinical appearance after involution of a large perioral and periorbital hemangioma

25 Vascular Malformations As a result of an error during embryological and fetal development Classification – Clinical – Radiological – Histological

26 Vascular Malformations Capillary Lymphatic Venous Combined – Arteriovenous – Lymphatico-venous – Lympathico-capillary-venous

27 Vascular Malformations Slow-Flow – Capillary – Lymphatic – venous Fast-flow – Arteriovenous

28 Capillary Malformations "Port wine stain“ present at birth Pink or red-colored intradermal discoloration Small, or large enough to cover the extremity or the face Seen in 3 infants per 1000 live births

29 Real-capillary malformations – Progressive – Grow thicker over time, darken and nodule develops inside "Salmon patch", "Nevus simplex", "vascular stain ” “birth stain” – Often seen in the middle part of the face, neck – Generally fade and reduce at the age of 1

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31 Other underlying disease or syndrome Capillary malformation on midline lumbar or cervical region,(spinal dysraphism, tethered spinal cord) Sturge-Weber syndrome (capillary malformation of the distribution area of the trigeminal nerve,lepthomeningeal vascular abnormalities,seizures) Klippel-Trenaunay syndrome(slow- flow capillary-lympho- venous malformation, elongation on axial plane and overgrowth of a limb

32 Sturge-Weber syndrome capillary malformation of the trigeminal nerve distribution area lepthomeningeal vascular anomaly Seisures

33 Klippel-Trenaunay syndrome capillary malformation soft tissue and bone overgrowth varices

34 Treatment Flashlamp-pumped pulsed dye laser – 577, 585, or 595 nm wavelength – Targets oxyhemoglobin – Provokes intravascular thrombosis % of patients present discoloration Treatment gives the best results in early childhood Other lasers (non-responsive patients) – Alexandrite (755 nm) – Neodymium: yttrium-aluminum-garnet (1064nm) – Intense pulsed light (IPL)

35 Port wine stain The result obtained after two applications of pulsed dye laser

36 Lympathic Malformations Seen as local sponge-like lesions or difuse lesions covering an anatomical organ or area Radiological and histological – Microcystic – Macrocyctic – Mixed Usually occurs at birth or first 2 ages most common seen in cervicofacial area Axilla, chest, mediastinum, retroperitoneal area,perineum, gluteal area Overlying skin is intact, looks bluish Small,thin vesicles are pathognomonic for dermal involvement

37 Treatment Bleeding Recurrent infection (cellulitis) Body contouring Correction of funtional deficits – Sclerotherapy (macrocyctic lesions) – Intralesional injection of bleomycin – OK-432 – Argon, neodynium: YAG, or carbon dioxide laser

38 Surgical treatment indications Lesions blocking the respiratory way Lesions that create feeding problems Lesions making distortion

39 Venous Malformation Soft, fading with pressure, bluish coloured masses under the skin Swelling with physical activity and when slouched down Palpable thrombi Morning pain (stasis and microthrombi) Frequent localization of head and neck More expansive than it looks (muscle,bone, oral mucosa, salivary gland)

40 Diagnosis Magnetic resonance imaging (MRI) – To diagnose – To evaluate the extent of malformation Bleeding-coagulation profile should be assessed – coagulopathy

41 Treatment Percutaneous sclerotherapy – absolute ethanol – hypertonic saline – sodium sulfate tetradesil Elastic compression stockings (extremity) Aspirin (daily) – painful thrombus – For prophylaxis of phlebitis Surgical Treatment – head and neck lesions causing cosmetic problem – severe pain and bleeding – Lesions with well-defined borders

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43 Venous malformation spreading into the vulva and inner thigh muscles Partial excision and injection of sclerosing agents

44 Venous malformation: 2 months after1 time the Nd: YAG laser application complete regression

45 Arterio-venous malformations Connection exists between the arterial system and venous system They usually present at birth Often incorrectly diagnosed (KM or hemangioma) There is a rapid flow between the two systems Fast flow is evident in childhood

46 Arterio-venous malformations Over time the stain on the skin – Erythema – The local rise of temperature –T hrill – M urmur The mass may expand Rapid growth may be seen during puberty or trauma

47 Arterio-venous malformations Arteriovenous shunts – Ischemia and related symptoms and signs – painless ulceration Persistent pain, occasional bleeding Widespread AVM may increase cardiac output and cause congestive heart

48 Diagnosis Ultrasonography Coloured Doppler MRI Angiography – Feeder and draining vessels – Varying degrees of arterial dilatation – curved vessels – A-V shunts – Enlarged draining veins

49 Treatment Embolization Sclerotherapy Surgical resection and reconstruction – Preoperative angiography Determines feeder and draining vessels Embolization (adhesive or with special springs)

50 Arterio-venous malformations Intracranial (most common) Extracranial – Head and neck – Extremity – Body – İnternal organs

51 AVM in the ear 1.Preoperative embolization 2.Ear amputation and partial thickness skin grafting 3. ear prosthesis

52 Differential Diagnosis Hemangioma Occurs shortly after birth Rapid growth, stagnation and reduction phases More seen in girls Endothelial hyperplasia vascular malformation Development commensurate with the growth More noticable at puberty Does not reduce No gender difference Lack of normal endothelium


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