Vascular malformations- By Dr. Ishita Srivastava

1. VASCULAR MALFORMATIONS
Presented by-
Ishita Srivastava

2. Mesodermal cells
Hemangioblasts
Blood islands
Hematopoietic cells Angioblasts
Primary capillary
plexus
Remodeling
Large caliber
vessels
Small
capillaries
Regression,sprouting
splitting,fusion of
pre-existing vessels
Mature
vasculature
VASCULOGENESIS
•3rd wk of embryonic life

3. • Development of lymphatic system
– 6th-7th wk of embryonic life
– 2 mechanisms
Primary lymph sacs
Veins
LymphangioblastsNew lymphatic
capillaries
Mesenchymal cells
Anastomosis of
sprouting lymphatic
vessels with lymphatics
from lymphangioblasts

4. Classification:-
Mulliken & Glowascki(1982)- Based on clinical &
microscopic features
• Hemangiomas - lesions demonstrating endothelial hyperplasia
• Vascular malformations - lesions with normal endothelial
turnover

5. • ISSVA (International Society for the Study of Vascular
Anomalies classification:- categorize vascular anomalies into
two basic types:
(1) vasoproliferative or vascular neoplasms such as hemangioma
(2) developmental vascular abnormalities called congenital
vascular malformations (CVMs)

7. Vascular Malformations
• Vascular malformations are collections of abnormal vessels
with a normal flat endothelium with a normal turnover rate.
They exhibit a thin basement membrane with a normal mast
cell count. These collections can be arterial, venous, and
capillary, or any combination above
• Defect in vascular smooth muscle
• Progressive dilation of vascular channels

8. Etiology & Pathogenesis
Morphogenesis of Blood vessels
Stages :
1. Endothelial stage
2. Retiform stage
3. Maturation stage
Abnormal development of either the arterial or venous
side of vascular network during this phase of
development result in vascular malformation.

9. Classification:-
On the basis of flow velocity:-

10. • On the basis of type of vessel:-

11. ISSVA Classification(2014):-

12. Clinical features:-
VENOUS MALFORMATIONS
• Most common
• Occur in vessels that are morphologically
and histologically similar to veins and hence
have low blood flow and are compressible
• Categorized as either superficial or deep,
and as localized, multicentric, or diffuse
• Appearance of most superficial ones is
purple color
• Subcutaneously located or mucosal ones
appear more bluish or greenish
• Deeper intramuscular ones may appear as
ill-defined swelling with normal overlying
skin

13. • Soft to the touch and empty on applying pressure as well as
on limb elevation. This is compressibility or the sign of
emptying
• Sluggish flow and stasis lead to phlebothrombosis, which
presents clinically as recurrent pain and tenderness and later
may have palpable phleboliths

14. • Expand with Valsalva maneuver or
dependency
• “bag of worms” texture caused by
multiple palpable varicosities
• most likely to affect bony size & shape
Most common intraosseous location-
mandible>maxilla
• Stasis & turbulence associated with
venous malformation-localized
intravascular coagulation &
sometimes DIC
• Fibrinopeptide – may be elevated
• Fibrinogen & platelets – decreased
• Only when coagulopathy is corrected are
surgical procedures feasible
– Heparin treatment is instituted

15. CAPILLARY MALFORMATIONS
• Also called venular malformations and are recognized as
birthmarks or port-wine stains and are common in the
trigeminal dermatome distribution
• In early stages, they are flat and pink
• May evolve into a raised, thickened red to purple plaque as
the child matures and eventually may become studded with
vascular papules, imparting a cobblestone-like appearance
• Pyogenic granulomas commonly develop in an area of
capillary stain-mouth

16. • Facial lesions in trigeminal nerve distribution can often be a
part of the Sturge–Weber Syndrome (SWS) known
(improperly) as encephalotrigeminal angiomatosis
• SWS is characterized by facial CM and intracranial vascular
malformation of the arachnoid and pia mater meninges and
presents with intractable seizures, mental retardation, or
glaucoma

17. LYMPHATIC MALFORMATIONS
• Based on the size of the lymphatic lumen, LMs (previously
termed lymphangiomas) can be divided into microcystic
lesions (previously termed lymphangioma circumscriptum)
and macrocystic lesions (previously termed cystic hygromas)
and a combined form
• Occur most commonly in the head and neck, followed by
axilla, chest wall, and extremities
• Present as soft, easily compressible
masses with thin overlying skin that
may swell in dependent positions or
when venous pressures increase
(crying or Valsalva)

18. • Bleeding within the cyst or a mixed veno-LM may result in
blue discoloration of the overlying skin
• Microcystic LMs are soft and noncompressible masses with an
overlying area of small vesicles involving the skin or mucosa,
which can weep and occasionally cause pain or minor
bleeding
• Macrocystic LMs (cystic hygroma)-
Almost a 50% association with chromosomal
disorders such as Turner syndrome, trisomy 21,
trisomy 18.
Often located below the level of the mylohyoid muscle

19. • I/O lymphatic malformations
-very irregular surface
• Most likely to affect bony size & shape
• Common sites-tongue,floor of
mouth,mandible,submandibular & neck soft tissues

20. ARTERIOVENOUS MALFORMATIONS
– Any vascular lesion that contains an arterial component is
considered a high-flow malformation
– Abnormal direct communication between…
• 2 routes of circulation created-normal&fistulous
• Path of least resistance-blood flows through fistulous pathway
• High flow malformation with multiple arteriovenous shunting
within a nidus which consists of a capillary network
• Most common sites are intracranial followed by extracranial head
and neck, extremity, trunk, and visceral
• Clinical presentation ranges from an asymptomatic mass to cardiac
failure
• Superficially located AVMs present as firm mass with warmth, bruit,
and thrill

21. • Increased flow & pressure result in increase in size of lesion
• Do not classically empty readily on compression or limb
elevation
• Bleeding occurs more frequently
• Other presenting symptoms include ulcer, ischemic steal, or
skin changes of venous hypertension

22. Schobinger clinical staging system for arteriovenous
malformations
Stage Description Clinical findings
1 Quiescence Cutaneous blush or warmth
2 Expansion Bruit or thrill, increasing size, pulsations
3 Local destruction Bleeding, infection, skin necrosis, or
ulceration
4 Decompensation High-output cardiac failure
4 stages of AVM as proposed by Schobinger

23. Signs & symptoms-depend on site of involvement & size of lesion
Usually pts.present with Swelling-poorly
defined,soft,compressible,pulsating,warm,thrill/bruit
Asymptomatic/toothache/earache/birthmark
Pulsating tinnitus
• Physical examination often reveals a bluish mass with a palpable
thrill and bruit secondary to the increase in blood flow
• Local or regional overgrowth of tissues
• Life threatening bleeding is a possibility
• Difficult to recognize on small biopsies or without knowledge of
angiographic findings

24. COMBINED VASCULAR MALFORMATIONS
• Complex syndromes
• Often associated with overgrowth of musculoskeletal tissue
• Classified according to high or low blood flow:-
1. Low flow:-
a. Klippel–Trenaunay Syndrome (KTS)
Combined capillary, lymphatic and VM in
one or more limbs in association with skeletal
or soft tissue overgrowth.
Classical triad includes atypical varicose veins,
nevus, and limb overgrowth
Main complication of KTS is thrombophlebitis

25. b. Proteus Syndrome: characterized by
asymmetric vascular, skeletal, and soft-
tissue lesions of varying size
Asymmetric body growth and macrodactyly
are the classic features with cutaneous or
CM spots and small microcystic lymphatic
or low flow venous malformations
c. Maffucci Syndrome: associated with
venous, capillary, and occasionally LMs,
with exostosis and enchondromas
These endochondromas can lead to
deformities or pathological fractures with a
long-term possibility of malignant
transformation into chondrosarcoma.

26. 2. High flow
Parkes Weber Syndrome: characterized by diffuse reddish
pink macule with evenly spreading geometric or blotchy
borders
high-flow with arteriovenous fistulas
main complication in the is cardiac failure and cutaneous
ischemia

27. • High-flow vascular malformations
– Asymptomatic
– 1st clinical indication-near fatal hemorrhage
– Clinical signs
• Spontaneous bleeding from gingival sulcus of teeth in the area
• Hyperthermia over the lesion
• Gingival discoloration
• Facial swelling/asymmetry
• Subjective feeling of pulsation
• Presence of bruit
DIAGNOSIS

28. • Large venous malformation
– Bluish,soft,compressible lesion
– Absence of bruit/pulsation
– Dramatic change in size with Valsalva’s maneuver or any
maneuver that increases venous pressure
• If low suspicion of intraosseous high-flow vascular malformation
– Aspiration of osseous defects prior to biopsy is a standard
recommendation
• If high suspicion of intraosseous high-flow vascular malformation-
arteriography without aspiration

29. Investigations
Hematologic evaluation
• Coagulation disorders occur at a high frequency in patients with CVMs
• Associated with potentially severe thromboembolic events and
hemorrhagic complications
• LIC is of important clinical concern due to the potential for more serious
thromboembolic events, including superficial thrombosis, deep venous
thrombosis, or pulmonary embolism as well as thrombohemorrhagic
disseminated intravascular coagulation (DIC) with life-threatening
hemorrhage, which can occur during or following surgical resection or
sclerotherapy

30. • The platelet count in LIC is minimally diminished (100,000–
150,000/ml range).Conversion of LIC to DIC can be detected
early by an increased prothrombin time as well as reduction in
platelet counts
• Extensive CVM with large surface area, muscle involvement,
and/or palpable phleboliths are strong predicting criteria for
coagulation disorders associated with CVM
• Assessment of the coagulation profile and D-dimer levels is
indicated in patients with extensive CVMs.
• Anticoagulation with Low-molecular-weight heparin (LMWH)
can be used to treat the pain caused by LIC and to prevent
decompensation of severe LIC to DIC

31. Ultrasonography
• Most widely used
• To evaluate vascularity and determine types of vessels present
Plain X-rays
• Soap-bubble or honeycomb radiolucencies
• Movement/resorption of teeth
• Phleboliths are classic of VM
and noted on plain X-ray
Doppler imaging
• Distinguishes high-flow from low-flow lesions

32. Magnetic resonance imaging
• Determine the extent of larger lesions for planning medical,
interventional, and/or surgical therapy
• Indicates presence & extent
• May allow determination of nature of flow

33. Computed tomography scan
• Examination of bony VM
• AVMs appear as a tortuous collection of vessels with one or
more enlarged feeding arteries
• In an asymptomatic AVM, there should be no associated soft-
tissue enhancement. The presence of soft-tissue
enhancement should raise the possibility of a tumor, such as a
sarcoma

34. Digital subtraction angiography
• To assess flow rate, visualize anatomy of the nidus in greater
detail
• Necessary for definitive diagnosis of intraosseous high-flow
vascular malformations

39. Treatment

41. Drug therapy
• Anticoagulants- to prevent recurrent thrombophlebitis
• Antiepileptic drugs- for intracranial VM

42. • Sclerosing agents
– Substances that cause a marked tissue irritation or
thrombosis with subsequent local inflammation &
tissue necrosis.
– Sclerosing agents used-Sodium morrhuate,boiling
water,nitrogen mustard,sodium tetradecyl sulfate
Fibrosis & tissue contraction

43. – Early attempts to treat high-flow lesions using
sclerosing agents were ineffective as the agent
moved from the site of action due to blood
flow & resulted in other side effects
– Used for treating
• Symptomatic hemangiomas
• Venous malformations
• Embolization of high-flow vascular
malformation
• Bony vascular malformations via trans-
osseous method

44. – Advantages of sclerotherapy over surgery
• No external scaring
• Less morbidity
• Few complications-mild fever,pain,trismus
• Small contracted lesions after sclerotherapy offer more
convenient & safer excision
– Sclerotherapy viewed as a first-line treatment because of its
effectiveness & low level of morbidity

45. • Embolization
– Embolization is defined as the "therapeutic
introduction of various substances into the circulation
to occlude vessels, either to arrest or prevent
hemorrhage, to devitalize a structure, tumor, or organ
by occluding its blood supply, or to reduce blood flow
to an arteriovenous malformation" (Stedman, 2000).
– Goal-to decrease flow within the actual malformation
while avoiding disruption of flow through proximal
feeders

46. – Undesirable effects of proximal embolization
• Encourages development of collateral supply
• Limits further access to central lesion for additional
embolization-collaterals that replace embolizes vessel are
usually more tortuous,making catheter placement difficult
– Thus, selective cannulation of only feeding vessels with deposition
of material within the microvasculature of the lesion is desirable

47. • Materials used
– Commonly used materials-Gelfoam,
isobutyl cyanoacrylate, polyvinyl
alcohol particles, steel coils
– Other less often used materials-balloons,
microfibrillar collagen (Avitene),
ethiodized oil (Ethiodol), autologous
materials, ethylene vinyl alcohol,
alginates, phosphoryl choline, sodium
morrhuate, hot contrast material, 50%
dextrose

48. – Advantages
• Deep areas can be treated
• Testing of the area can be done before and after
permanent treatment is done
– Disadvantages
• Often requires multiple treatments
• Less likely to totally obliterate the AVM when used
alone
– Embolization + surgery – most predictable
treatment modality…ensures resolution of
lesion with least chance of recurrence
– Embolization followed with surgical removal of
lesion within 24-48hrs

49. – POTENTIAL COMPLICATIONS
• Arterial spasm
• Vessel rupture
• Contrast material- systemic reaction or adverse
renal effects
• Tissue necrosis
• Inadvertent embolization of ICA through reflux of
material from ECA
– Infarction of blood supply to CNS or retina
• Pulmonary embolism

50. • Surgery
– Goals
• Complete removal of lesion
• Reconstruct defect to a functional & aesthetic level
– Transoral approach-lesions anterior to angle of
mandible with minimal or no lingual cortical
perforation
– Extraoral incision-lesion extends proximally into the
angle or ramus

51. – Resection
• Any doubt about extent of lesion
• Large no.of lingual feeders
• Compromised access
– If resection is used,reconstruction will be
necessary
• Immediate temporary reconstruction or Definitive
reconstruction

52. – Advantages
• The AVM is obliterated at the time of surgery
• Surgery has the longest “track record”
• Both small and large AVM’s can be treated
– Disadvantages
• Operation requires GA and recovery period
• Produces morphological alterations in the growing
face
– Postop follow-up
• Arteriogram repeated approx.1yr postop.

53. • Radiation
– Advantages
• Can be done without craniotomy if AVM is in the
brain
• Small deep lesions can safely be treated this way
– Disadvantages
• Lesions greater than 2.5 cm not treated well

54. • Vascular Lasers
– Anderson & Parrish
Deep cutaneous
vascular lesions
• 585-nm pulsed dye
laser
• 1064-nm Nd:YAG laser

55. Follow-up
• Close postoperative observation with expected management
of local recurrence is required.
• After completion of repeat treatment sessions, a 6 monthly
or annual Doppler or MRI is recommended to assess the
effectiveness of treatment and detect persistence or late
recurrence.
• If treated appropriately, most patients will experience at least
symptomatic improvement after endovascular therapy and
possible cure after surgery.

56. THANKYOU