This document discusses recent trends in the management of vascular malformations. It begins by classifying vascular lesions and anomalies, discussing the evolution of classification systems from Virchow in 1863 to the International Society for the Study of Vascular Anomalies in 2014. The key differences between hemangiomas and vascular malformations are outlined. Various types of vascular malformations are then described in more detail, including capillary, venous, lymphatic, arterial and combined malformations. The document concludes by discussing principles of management, including conservative measures, drug therapy, sclerotherapy, embolotherapy and surgery.

1. Recent Trends in Management of
Vascular Malformations
Moderator-Prof A K Khanna Speaker- Awaneesh Katiyar

2. • A thought -BIRTH MARK GIVEN BY GOD
• VASCULAR MALFORMATION – INBORN ERROR
OF VASCULAR MORPHOGENESIS

3. Classification of Vascular Anomalies
• In 1863 Virchow – attempt to classify Vascular
lesions
1. Angioma
a) Angioma Simplex
b) Angioma Cavernosum
c) Angioma Racemosum
2. Lymphangioma

4. • In 1982 , Mulliken and Glowacki
• 49 specimen , 2 categories-
Hemangioma/malformation(23)
• Hemangioma – proliferative(14) and
involutary(12)
• Clinical history ,Endothelial hyperplasia
incorporation with tritiated thymidine and
basement membrane.

5. Classification of vascular lesions in
infants and children(Mulliken,1982)
Vascular lesions
Hemangiomas Malformation
Proliferative phase
Involuting Phase
Capillary
Venous
Arterial
Lymphatic
Fistulae

6. Hamburg Classification,1988
Type Truncular Extratruncular
Predominantly Arterial
Defects
Aplasia or Obstructive
Dilation
Infiltrating
Limited
Predominantly venous
Defects
Aplasia or Obstructive
Dilation
Infiltrating
Limited
Predominantly Lymphatic
Defects
Aplasia or Obstructive
Dilation
Infiltrating
Limited
Predominantly
Arteriovenous shunting
Defects
Deep
Superficial
Infiltrating
Limited
Combined / mixed vascular
defects
Arterial and
venous
Hemolymphatic
Infiltrating
hemolymphatic
Limited
hemolymphaticBased on seventh Meeting of the International Workshop on Vascular Malformations. Hamburg, Germany, 1988.

7. Classification of Vascular Anomalies
By Jackson, 1993
From Jackson IT, Carreno R, Potparic Z, et al. Hemangiomas, vascular malformations, and lymphovenous malformations: classification
and methods of treatment. Plast Reconstr Surg 1993;91:1217.
Vascular Anomalies
Hemangiomas Vascular Malformation Lymphatic
malformation
Low flow High flow

8. Classification of Vascular Anomalies
ISSVA ,Italy, 1996
Vascular Tumors Vascular Malformation
Simple Combined
Hemangioma Capillary(C) Arteriovenous
Fistula(AVF)
Others Lymphatic (L) AVM
Venous(V) CVM
LVM
CAVM
CLAVM
Based on Scientific Committee of the Eleventh Meeting of the International Society for the Study of Vascular Anomalies. Rome, Italy, 1996.

9. Classification of Vascular Anomalies
ISSVA , Melbourne,2014
Vascular tumors Vascular Malformation
Benign Simple
Locally Aggressive or Borderline Combined
Malignant of major named vessels
associated with other anomalies
Provisionally unclassified vascular
anamolies

11. Vascular Malformation
Simple Combined of major named
vessels
Aka “channel”or
“truncular”Vascular
Anomalies
associated with other
anomalies
Provisionally
unclassified
Capillary
Malformation
CVM,CLM Affect
lymphatic
veins
Arteries
Klippel-trenauanay Verucous
Hemangioma
Parke -Weber Angiokeratoma
Lymphatic
Malformation
LVM,CLVM Servelle- Martorell Kaposiform
lymphangiomatosis
Sturge-Weber others
Venous
Malformation
CAVM
Anomalies of
origin
course
number
length
diameter
communication
persistence (of
embryonal vessel)
maffucci
Macrocephaly-CM
Arteriovenous
malformation
CLAVM Microcephaly-CM
CLOVES
Arteriovenous
fistula
Others Proteus
Bannayan-Riley-Ruvalcaba
Limb CM+congenital non
pregressive limb hypertrophy

12. Hemangioma Vs Vascular Malformaton
Hemangiomas Vascular Malformations
May or May not be present at
birth
Always present at birth but
may not be evident
Grow rapidly and slowly
involute
Progress in proportion with
body
Involute spontaneously Usually don’t involute
Females are more affected Equally affected
True benign neoplasm Defect of morphogenesis
Associated with endothelial
hyperplasia with rapidly
dividing endothelial cells
Ectasia of abnormal vessels
Mast cells increase in
proliferative phase
No evidence
hemangioma
VMs
Gerald M. Legiehn, Ca,Manraj K.S. Heran, Classification, Diagnosis, and Interventional Radiologic
Management of Vascular Malformations Orthop Clin N Am 37 (2006) 435–474
hemangioma
VMs

13. Hemangioma Vs Vascular Malformaton
Hemangiomas Vascular Malformations
Rare causes bony
/cartilagenous distortion or
hypertrophy
Low flow malformation
usually causes skeletal /
bone hypertrophy
Due to mass effect may
cause depression over bone
High flow causes destructive
bony changes
Angioigraphy – well
circumscribed mass with
intense prolong tissue
staining
Diffuse lesion without
intervening parenchymal
staining
Feeding arteries may form
equitorial network in
periphery
Depends on predominantly
channel type

14. Vascular tumors- Benign
Hemangiomas
infantile congenital
Presentation
Site
Involution
Sex
Skin color
GLUT-1

15. Pyogenic Granuloma
(Lobular capillary Hemangioma)
• Solitary, red papule, grows rapidly, forming a
stalk.
• Lesions are less than 1 cm in diameter.
• presentation is inversely
correlated with age

16. Uncommon Vascular tumors
• Kaposiform hemangioendothelioma
• Associated with kasabach- merritt
syndrome

17. Angiosarcoma
• Rare
• Aggressive malignant vascular neoplasm
• Female preponderance
• Average age of
onset 3.7yrs
• poor prognosis
Angiosarcoma in a 2-year-old female patient with an
abdominal mass. Axial contrast-enhanced CT image shows
a heterogeneously enhancing right hepatic mass.Note focal
pooling of contrast (arrow).

18. Vascular Malformation
Slow/low flow
Fast/high flow
CM VM LM AVM AVFAM

19. Capillary Malformation
• Naevus flammeus neonatorum (“angel’s kiss”
or “stork bite” or “port-wine stains”
• In new born
• At face and nuchal region
• Red macular lesions
• Persist for throughout life.
• CMs may occurs in association with
syndromes , best known SWS.

20. Associated syndromes with CMs
• Sturge-Weber Syndrome
• Cutis Marmorata Telangiectatica Congenita
• Macrocephaly–Capillary Malformation

21. Venous Malformation
• “cavernous Hemangioma” A misnomer.
• Always present at birth –may not be obvious.
• various presentions.
• Include from small swelling to
large body deforming swelling
lethal outcomes.

22. • “Genuine Diffuse Phlebectasia of
Bockenheimer”
– Extensive Venous Malformation of limb
– Associated with limb discrepency
• Kasabach- merritt phenomenon
• Hemolytic anemia
• Thrombocytopenia
• hypofibrogenemia

23. Syndromes associated with VMs
• Glomuvenous Malformation.
• Cutaneomucosal Venous Malformation.
• Blue Rubber Bleb Nevus Syndrome.

24. Lymphatic Malformation
Microcystic LM or
Lymphangiomas
(<2cm)
Macrocystic LM or
Cystic Hygroma
(>2cm)
Visceral LM or
Lymphangiomatosis
Common cystic LM

25. Congenital/Primary Lymphedema
• “Milroy Disease”.
• Anomalous Lymphatic
Channels
• Maily manifest at birth
• Compression, suction
lipectomy
• lymphaticovenous
anastomosis

26. Arterial Malformation
• Aneurysms,
• Fistulae,
• Ectasias,
• Stenoses

27. Arteriovenous Malformation
• Congenital high-flow vascular malformations.
• Growth Triggered by
– Puberty
– Trauma
• Pathogenesis – TGF-ß
signaling and a genetic two hit
hypothesis(Knudson’s).

28. • Extracranial Vs Intracranial
Arteriovenous malformation in a 16-year-old male
patient with a left chest wall mass.

29. Schobinger Staging System for Arteriovenous Malformation
Stage I Quiescence Cutaneous blush, skin warmth, arteriovenous shunt on
Doppler ultrasound
Stage II Expansion Darkening blush, lesion shows pulsation, thrill and bruit
Stage III Destruction Steal, distal ischemia, pain, dystrophic skin changes,
ulceration, necrosis, soft tissue and bony changes
Stage IV Decompensation High-output cardiac failure
Based on Eighth Meeting of the International Workshop on Vascular Malformations. Amsterdam, Netherlands,1990.

30. Combined Malformations and
Overgrowth Syndromes
• Klippel-Trenaunay Syndrome
• Maffucci Syndrome
• Parkes Weber Syndrome
• Capillary Malformation-Arteriovenous
Malformation
• Bannayan-Riley-Ruvalcaba Syndrome (PTEN
Hamartoma Syndrome).
• CLOVE(S) (Congenital, Lipomatous, Overgrowth,
Vascular Malformations, and Epidermal Nevi)
Syndrome

31. Klippel-Trenaunay Syndrome
• Also known as Capillary-Lymphatico-Venous
Malformation(CLVM)- slow flow type.
• Sporadic
• Association with gene not yet known.
• Limb hypertrophy present at birth which
progressively worsens with growth.
• Complication-recurrent infection,
thrombophlebitis, 1% systemic complication.
• Fast flow associated with – Parkes-Weber
syndrome.

32. Investigation
• Ultrasonography with CD- noninvasive , inexpensive
and readily available- initial & gold standard.
Mixed venous
waveforms
No doppler
flow
Arterial waveforms

33. Computed tomography
• To define – bony architecture, identify
phleboliths and other dystrophic calcification,
to describe hepatic lesions
• CECT/ Helical CT -More informative in
Arterionenous malformations – arterial , nidus
and venous structures.

34. CECT – venous malformation CECT- AVM in mid arterial phase
(A) CECT –AVM around Rt humerus (B) CT angio of same lesion

35. Magnetic Resonance Imaging
• Replaced CT- no ionizing radiation.
• Better for serial follow up- after
sclero/embolotherapy.
• Excellent to evaluate low flow malformation and
soft tissue.
• Protocol-spin echo or fast spin echo T1 weighted
imaging – for baseline
• T2 weighted short tau inversion recovery images-
hemosiderin, dystrophic calcification or
phlebolith,

36. Gerald M. Legiehn, Manraj K.S. Heran, Classification, Diagnosis, and Interventional Radiologic Management of Vascular
Malformations Orthop Clin N Am 37 (2006) 435–474

38. Management of Vascular Malformation
Multi-disciplinary Team

39. Multimodality approach
Vascular
Anomalies
conservative
Drug
therapy
sclerotherapy
embolization
endovascular
surgery
Pychological
support

40. • Surgical Excision –limited knowledge of the
natural and biology – poor prognosis
• New Endovascular intervention- Embolo/
Sclero therapy/balloon/stenting/ablation

41. Principles of Management
• None of the single modality of treatment can
treat any VM alone.
• Classify before to intervene- truncular/
Extratruncular
• Extratruncular- poor outcome in diffuse
variety.
• Truncular- Fair out if they intervene early

42. • Wiser – “Not to intervene”-if nature not
known.
• Refer to experienced hand / center-
where better facilities are available
• Life threatening lesions treated – As early as
possible

43. General Measures for Management
• Explain the Proper Diagnosis- patient , parent,
guardians and to referring doctor.
• Treat associated complications
– Hemorrhage
– Ulcer
– Anemia
– Recurrent thrombophlebitis
– thrombocytopenia

44. • Graduated compression stocking and
Garments
• Support and Education.
• Refer to and consult other specialist
• Family members screening and genetic
counseling-for associated syndromes

45. Indications for therapy
• Lee(2005) devised “decision to treat”-At least
one absolute and two relative indications.
• Absolute indications-
1. Hemorrhage
2. Progressive high output failure
3. Complication secondary to Venous hypertension
4. Lesion located in life threatening area.
5. Lesion located in life treatening vital functions

46. • Relative indications-
1. Progressive disabling pain or discomfort.
2. Functional disability or impairment affecting
daily life and quality of life.
3. Severe cosmetic deformity.
4. Vascular-bone Syndrome- causing growth
discrepency.
5. Location at high risk for complication.
6. Recurrent infection or sepsis.

47. Treatment options
Management of Vascular
Malformation
Non–surgical Surgical
•Conservative
•Drug therapy
• Sclerotherapy
• Embolotherapy
• Endovascular
management
• Resective
• Recontructive
• Endovascular
recontructive surgries

48. Conservative Management
• Proper skin care .
• Local wound care-prevent hemorrhage.
• Compression therapy.
• Life style modification and appropriate
physical therapy- orthopedic footwear
• Psychological support.

49. Drug thrapy
• Anticoagulation- to prevent recurrent
thrombophlebitis
• Rheological/venotonic agents –Trental /
Daflon
• Antiepileptic drugs – for intracranial VM

50. • A serentipity – in 2008 .
• Newborn with proliferative hemangioma with
cardiovascular ds.- on propranolol therapy
• Shows rapid regression of lesion
After 2 months of therapy- 2 mg/kg divided tid

51. • Corticosteroids
• Vincristine and interferon alfa-2a&2b
• cyclophosphamide

52. Sclerotherapy
• Sclerotherapy is of irritant solution into lumen
of a vessel –causes thrombosis and
subsequently fibrosis.
• Ideal sclerosing solution
– Painless to inject
– Free of adverse effects
– Specific to affected vessel

53. Sclerosing solutions
Detergent Agent Osmotic Agent Chemical Irritants
Sod . Tetradecyl
sulphate
Palidocanol
Sod Murrhuate
Ethanolamine
oleate
Ethanol
Hypertonic Saline
Hypertonic Saline
Dextrose
Chromated
Glycerin
Polyiodinated
Iodine

54. Ethanol sclerotherapy
• Gold standard – widely used and accepted
percutaneous sclerogent
• Readly available , inexpensive, easy to use ,
long shelf life.
• Potent irritent- transmural destruction of
vessel wall.
• Avoided near to – large nerves, skin, mucosa,
genitalia, finger/toes.

55. • In Europe – only approved for slow flow
malformations
• Not exceed 1ml/kg at any one visit.
• Ethylcellulose and Ethibloc

56. Bleomycin sclerotherapy
• Glycopeptide antibiotic- Streptomyces
verticillus.
• Most commonly used sclerosing agent for the
treatment of vascular anomalies in China.
• Sclerotherapy has no great pain or nerve
injury-the cervical-facial region.
• USG guide is most effective method

57. • 75 patients(2010)- 42 patients (56%) one time of
treatment, 21 (28%) two times, nine (12%) three times,
and three (4%) patients received four times.
• 84% (63 patients) were cured, 13.33% (10 patients)
were basically cured, 2.67% (two patients) were
improved, and 0% were ineffective.

58. Sodium tetradecyl sulphate
• An anionic surfactant.
• Widely used in for sclerosis of esophageal
varices and varicose veins.
• Now also in – vascular malformation.
• Not effective- as ethanol.
• Cause urticaria, anaphylaxis, hemolysis, and
hematuria

59. Sclerotherapy for Venous Malformation
• Puig and Associates-classifies for sclerotherapy
I II
III IV

60. • Type I and to a lesser extent type II lesions
respond best
• Higher complication rates -attributed to type III
and type IV anatomy

61. MRI monitoring for sclerothrepy outcomes(A) presclerothrapy (B) 6 month after
one session of Ethanol sclerotherapy

62. Sclerotherapy for Lymphatic
Malformation
• Newer agent particular for LMs- OK432.
• Lyophilized powder made of Streptococcus
pyogenes (group A, type 3, Su strain)
inoculated with benzylpenicillin.
• Multi-institutional randamised trial for OK-432
• 95% of complete for macrocystic and 0-80 %
for micro cystic or cavernous subtype

63. • MRI monitoring for sclerothrepy outcomes(A)
presclerothrapy (B) 8 month after two session of OK-
432 sclerothrapy

64. Percutaneous image guided
sclerotherpy
• Real time Ultrasound Guided foam
sclerotherapy
• Fluoroscopic & Ultrasound guided
Sclerotherapy(FUGS)
(2004)

65. Side effect and complications of
sclerotherapy
• Allergic reaction.
• Matting - is the appearance of groups of new fine telangiectasias
surrounding or replacing a previously treated area in blush-like
manner. Resolution occurs spontaneously within a three to twelve
month period with 70-80% spontaneous resolution within the first
6months.
• Cutaneous necrosis
• Superficial thrombophlebitis .
• Haematoma formation.
• Post sclerotherapy hyper pigmentation
• Damage to the nerve- an extra vascular injection may cause pain or
Paraesthesia and numbness in the site of distribution of the
involved nerve.
• Pulmonary embolism - Probably occurs from extension of a
superficial thrombus into the deep venous system.

66. Embolotherapy
• Embolic Agents-
• Liquid-dehydrated ethanol,
• Semiliquid - N-butyl cyanoacrylate glue and
ethylene vinyl alcohol copolymer [Onyx])
• Solid (e.g.,coils, particles) embolic agents.
• Dominant draining veins -coils, glue, and
ethanol.
• AVFs use -coils and plugs.

67. Ineffective AVM embolization techniques
The use of glues in AVM
embolization

68. Retrograde venous route to AVM nidus
Temporary arterial inflow or venous outflow
occlusion during embolosclerotherapy of AVMs.

69. Permanent arterial inflow occlusion. The judicious
use of coils (arrows) and cyanoacrylate (arrowhead)
can decrease total arterial inflow to an AVM
allowing greater concentrations and ‘‘dwell time’’ of
sclerosant to be delivered to the nidus.
Permanent venous outflow occlusion. Coil
embolization of the venous portion of the
AVM nidus can reduce total flow within the
lesion allowing greater effectiveness of
transarterial sclerosant. This technique also
may serve as the most definitive AVM closure
technique during multitechnique therapy.

70. Endovascular Management
• In management of truncular Malformation
• Thermal or Laser Ablation.
• Thermal Ablation – mainly used for Venous
incompetence
• Minimal role in other Vascular Malformation

71. Laser Treatment
• Commonly used for Venous malformation.
• Includes – capillary malformations, localised
venous anomalies, diffuse venous ectasias.
• Also useful in vascular tumour and truncular
venous anomalies.

72. Laser Ablation –truncular VM
• Least local and no systemic side effects
• For MV/KTS
• Endovenous Laser Ablation – wavelenth of
1310-1470 nm.
• Isolated laser ablation - often inadequate
• Required – combined with embolization of
venous outflow or sclerotherapy(pre and post)

73. Laser ablation-Extratrunclar VM
• Soft Tissue Phlebectasias- perforator vein laser
coagulation.
• Glomuvenous Malformation -Nd:YAG laser .
• Capillary-Lymphatic Malformation- Flash Lamp
Pumped Dye Laser (FDL)/KTP
• Capillary Malformation-
double pulse pulsed Nd:YAG
/pulsed dye laser ,
transcutaneous ice cube-
cooled Nd:YAG laser CM- pre and post FDL

74. Endoscopic laser
• Treatment of airway, intestinal and genito-
urinary tract lesions.
• Nd:YAG laser
Blue Rubber Bleb
Nevus Syndrome
HHT – Nd:YAG Laser

75. Surgical Management for Vascular
Anomalies
• “Combined” surgical approach.
• To correct hemodynamic derangements.
• Various surgical procedures .

76. Reconstructive surgeries
• Reconstructive (open) management -truncular
lesions with direct involvements of all the
named vessels.
• Excision of the web/membrane.
• Aneurysm

77. Resective Surgical Therapy
• Extratruncular- combined with the
endovascular therapy (embolo/ sclerotherapy.
• A “limited”
resection.

78. Baby with CLOVE syndrome – under went serial
surgical resection of lesion

79. Endovascular recontructive surgeries
• For treating stenosing truncular VM lesions.
• Webs, septum, and stenosis -iliac vein, IVC,
jugular vein, and azygous vein.
• fail to respond
• Excisional surgical therapy with/without
bypass reconstruction is the treatment of
choice.

80. Conclusion
• Inspite of well developed
classification – misnomer
still are used.
• Don’t intervene – till the nature of lesion is not
known.
• Controlled aggressive approach is required for
management of vascular malformation.
• Classify the lesion by Hamburg classification
before intervention.
Strawberry CavernousXX

81. • Use of minimal invasive technique always have
better outcomes – untill complication not
occurred.
• Most of the Extratruncular lesion almost always
required multimodality of treatment.
• Truncular lesion – mostly required surgery
• May needs to combined with pre or post sclero/
embolotherapy.
• Vascular Malformation with complication or at
life threatening location required urgent
intervention.