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

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: Involution period (1-7 years)
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
Ulcer

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
diffuse
local

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

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
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)

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

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
Thrill 
 Murmur
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
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