it is about vascular anomalies and hemangiomas

1. VASCULAR ANOMALIES

2. Introduction
 Most common congenital abnormality
 Most confusing and misunderstood conditions
 Inconsistent terminology used for its classification

3. • Functional disability
• Cosmetic problems
• Life threatening hemorrhage

4. History
 Virchow and Wegener in 1880
 Vascular tumors into angiomas and lymphangiomas
 Simplex, Cavernosum and Racemosum

5. Classification
 Mulliken and Glowacki
 Biological classification
 Clinical behavior and endothelial cell characteristics
 Hemangiomas
 Vascular malformations

6. ISSVA Classification 2014
Hemangioma Vascular Malformations
• Infantile hemangioma
• Congenital hemangioma – RICH
and NICH
• Tufted angioma
• Kaposiform
hemangioendothelioma
• Spindle cell
hemangioendothelioma
• Other hemangioendotheliomas
• Dermatologic acquired vascular
tumors
 Slow Flow vascular malformation
- Capillary malformation
- Venous malformation
- Lymphatic malformation
 Fast Flow vascular malformation
- Arterial malformation
- Arteriovenous malformation
- Arteriovenous fistula
 Complex combined vascular malformation
- CVM, CLM, LVM, CLVM
- AVM-LM, CM-AVM

7. Comparison of Previous Terminology and ISSVA Terminology
 Capillary or Cavernous hemangioma
of any organ
 Infantile hemangioendothelioma of
liver
 Hepatic hemangioma, Cavernous
hemangioma
 Lymphangioma, Cystic hygroma
 Port wine stain, Capillary
hemangioma
Previous ISSVA
• Infantile hemangioma
• Hepatic or Infantile
hemangioma
• Venous malformation
• Lymphatic malformation
• Capillary malformation

8. Hemangioma

9. Hemangioma
 True benign endothelial cell tumor
 Overgrowth of normal vascular tissue
 Most common tumor of the head and neck in children
 7% of all benign soft tissue tumors
 Common in females - 3 : 1 ratio

10. Development
 Usually not present at the time of birth
 Appears weeks or months after birth
 Erythematous, macular patch

11. Hemangioma Stages
 Proliferating stage – first year
 Involuting stage – few years
 Involuted stage – most resolved by 10 years of age

12. Proliferative Stage
 Grows by endothelial proliferation
 Increased number of mast cells within the endothelial wall
 Grows faster than the growth of the child

13. Involutory Stage
 12 months of age - signs of involution
 5 to 9 years

14. Types of Hemangioma
 Infantile hemangioma – most common
 Congenital hemangioma – rare, present at birth, blue/gray
- Rapidly involuting congenital hemangioma (RICH)
- Non involuting congenital hemangioma (NICH)
 Intramuscular hemangioma
 Kaposiform hemangioendothelioma (KHE)

15. Sites
 Hemangiomas may involve superficial tissue, deep tissue or both
 Deep hemangiomas involve muscle or visceral organs - liver, lung,
spleen, and gastrointestinal tract
 Intra-osseous hemangiomas are extremely rare and may deform
the underlying skeleton

16. Clinical Presentation
 Superficial hemangioma - raised, reddish to purple tumor with
a distinct margin
 Deep subcutaneous hemangiomas - deep bluish hue with
normal overlying skin
 Both the lesions are firm on palpation
 Do not pulsate or exhibit any thrills or bruits

17. Investigations
 CT
 MRI - to document the extent of the deep hemangioma
 Arteriography - rarely indicated

18. Management
 Observation and parental support
 Intervention is necessary if functional compromise
such as visual, airway or masticatory compromise,
bleeding, ulceration or infection occurs

19. Management
 Corticosteroids
 Interferon alfa-2a
 Surgery - small lesions and as a secondary procedure
after initial therapy and involution
 Sclerosing agents
 Cryotherapy
 Argon laser ablation

20. Vascular Malformations

21. Vascular Malformations
 Malformed vessels secondary to aberrant development
during the various stages of embryogenesis
 Between the 4th and 10th week of embryogenesis

22.  Incidence - 15% of live births
 Present at birth
 Do not involute
 Grow proportionately with the patient growth

23.  Trauma, infection and hormonal fluctuation (pregnancy/puberty)
 No endothelial proliferation - normal mast cells
 Alteration in the flow dynamics within and around the lesion
 Recruitment of collateral vessels
 Dilatation of involved vessels

24.  Pelvis, extremities and intracranial circulation
 88% are asymptomatic
 Wide range of presentation with an unpredictable course
 Male : Female = 1 : 1

25. Pathophysiology
 Normal endothelium
 Defect in vascular smooth muscle
 Progressive dilation of vascular channels

26. Mulliken Classification
 High flow lesions
 Low flow lesions

27. High Flow Vascular Malformations
 Arterial malformation (AM)
 Arteriovenous malformation (AVM)
 Arteriovenous fistula (AVF)

28. Arterial Malformation
 Aneurysm
 Coarctation
 Ectasia

29. Arteriovenous malformation
 Present at birth
 Skin shows reddish vascular hue
 Warm and Pulsatile
 Thrill and bruit present
 Absence of developed capillary bed
 Pain, ulceration, bleeding, compression / displacement of organs
 High output cardiac failure

30. Stages of AVM
 Stage I - pinkish-bluish stain and warmth
 Stage II - pulsations, thrill and bruit
 Stage III - dystrophic skin changes, ulceration, bleeding, pain
 Stage IV - high-output cardiac failure

31. Arteriovenous Fistula
 Simple arteriovenous connections
 Secondary to penetrating injuries after birth

32. Low Flow Lesions
 Capillary malformation
 Venous malformation
 Lymphatic malformation
 Combined malformation

33. Capillary Malformations
 Port wine stain
 Pink in infancy and darken to deep purple in childhood
 Do not change colour when compressed
 Do not disappear

34. Venous Malformations
 Present at birth
 Bluish, soft and easily compressible
 No thrills nor bruits
 Thin walled, dilated veins with inadequate smooth muscle layer
 Maneuvers that increase venous pressure (Valsalva maneuver or
supine positioning) can enlarge a venous malformation
 Complications – Bleeding, Thrombosis, Pulmonary embolism

36. Phleboliths that may be noted on radiographic examination are found
only in low flow lesions

37. Lymphatic Malformations
 Colourless
 Soft
 Micro cystic – irregular surface, clear or dark
haemmorhagic bullae and vesicles – Salmon eggs
 Macro cystic – large cystic spaces containing lymphatic
fluid – transilluminate - Cystic hygroma
 Mixed

38.  Sturge-Weber syndrome - Port wine staining of the
ophthalmic and maxillary dermatome of the Trigeminal
nerve with convulsions, mental retardation and glaucoma
 Klippel – Trenaunay syndrome – capillary malformation
with lateral megaveins and bone & soft tissue hypertrophy
 Blue Rubber Bleb Nevus syndrome (Bean syndrome) –
multiple cutaneous and GIT venous malformation

39.  Maffucci syndrome – Venous malformation with enchondromatosis
 Parkes Weber syndrome – overgrowth of the affected limb with
small AV fistulas
 Proteus syndrome – Vascular malformation with connective tissue
nevus with disproportionate overgrowth of limbs, ovarian
cystadenoma, localized absence of fat
 Kasabach Merritt syndrome – fast growing vascular tumor
hemangioendothelioma with thrombocytopenia

40. Investigations
 X-Ray - Soap bubble or honeycomb appearance
 MRI - differentiate low-flow from high flow lesions. Fatty deposits,
venous lakes, phleboliths are all indicative of low flow lesions
 CT scan - extension into the surrounding soft tissue
 Doppler imaging - distinguish high flow lesion from low flow lesion
 Angiography delineates the size of the lesion and can also assess the
rate of flow through the lesion

41. Treatment Indications
 Hemorrhage
 Risk of high-output cardiac failure
 Chronic venous hypertension
 Airway impingement
 Lesion threatening vital functions

42.  Disabling pain
 Functional disability
 Cosmetic deformity
 Recurrent infection
 Persistent lymph leakage

44. Capillary Malformation
 Pulsed dye laser therapy
 Argon laser
 Surgical excision

45. Lymphatic Malformation
 Steroids
 Sclerotherapy
 Bleomycin sclerotherapy
 O.K 432 sclerotherapy
 Radiation therapy
 Surgery – Serial excision / Debulking

46. Venous Malformation
 Sclerotherapy for smaller malformations
 Sodium morrhuate, boiling water, alcohol and ethibloc are
effective in fibrosing smaller lesions
 Treatment of choice is transcutaneous sclerotherapy
 80% ethanol is the most commonly used agent

47. Arterial malformation & AV malformation
 Selective arterial embolization
 This procedure is usually done 24 – 72 hours before surgery in
order to prevent the risk of haemorrhage during surgery
 The nidus needs to be embolized
 Goal of surgery should be total excision of the malformation

48. Lesion present at birth
Hemangioma Vascular Malformation
No Yes
Proliferating
Yes No
Life Threatening
Yes No
Steroids
Interferon
Surgery
Observation
Cosmetic Resection
Involuting
Yes
Investigations
High Flow Lesion Low Flow Lesion
Preop Embolisation
Surgical Ablation
Large vessel lakes
Sclerotherapy
No
Yes

49. Take Home Message
Hemangioma
 True tumor
 Endothelial proliferation
 Female 3 : 1
 Absent at birth
 Rapid growth during infancy
 Self limited
 Diagnosis by history and
appearance
Vascular malformation
• No tumor
• Dysplastic vessels
• 1:1 male : female
• Present at birth
• Growth proportional to child
• Do not disappear
• Diagnosis by MRI, Doppler,
Angiography

50. QUIZ

51. 1
 Who first classified vascular anomalies ?
 How was it classified ?

52. 2
 Who classified vascular anomalies based on the
biological characteristics ?
 How was it classified ?

53. 3
 Types of Hemangioma ?
 Kasabach – Merritt syndrome ?

54. 4
 Classify Vascular malformations ?

55. 5
 Difference between Klippel Trenaunay syndrome and
Parkes Weber syndrome ?

56. 6
 Phleboliths are seen in which vascular anomaly ?
 What are Salmon eggs ?

57. 7
 In venous malformation, which layer of the vessel wall
is abnormal ?
 Port wine stain also known as ?

58. 8
 In AVM, which has to be embolized ?
 After embolization, surgery should be done within
what period ?

59. 9
ISSVA ?

60. 10
 3 differences between hemangioma and vascular
malformation ?

61. THANK YOU