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

Vascular Anomalies In U.S, 40000 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)

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

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.)

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   

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

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

Proliferation period: Involution period (1-7 years) Rapid growth (up to 10-12. 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 

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

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

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

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

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

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

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

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)

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)

Systemic steroid treatment

Ulcer diffuse local

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

Hemangioma Ulceration

residual athrophic tissue and wrinkled skin after involution of hemangioma

Clinical appearance after involution of a large perioral and periorbital hemangioma

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

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

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

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

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

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

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

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

Treatment Flashlamp-pumped pulsed dye laser 577, 585, or 595 nm wavelength Targets oxyhemoglobin Provokes intravascular thrombosis 50- 90% 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)

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

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

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

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

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)

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

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

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

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

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

Arterio-venous malformations Over time the stain on the skin  Erythema  The local  rise of temperature Thrill   Murmur The mass may expand Rapid growth may be seen during puberty  or trauma

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

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

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

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

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

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