?

What is AVM
 Vascular anomalies are among the most

common congenital abnormalities

observed in infants and children.

 Vascular anomalies represent a spectrum

of disorders from a simple “birthmark” to

life- threatening entities

 vascular malformations are one of the
most challenging subgroup of diseases
treated by vascular surgeons and
interventionalists.
 Currently, there exists a lot of
misunderstandings and controversies in
terminology, diagnosis, and management
of patients with these problems.
Infantile hemangioma
 Distinguishing features of hemangiomas
:and AVM
HEMANGIOMA AVM
neoplasm congenital anomaly
30% present at birth, 70% 90% present at birth
present in first 3 months
Female: male is 5:1 equally
Endothelial proliferation No cellular proliferation
Growth in tissue culture No growth
Increased mast cells No mast cells
Spontaneous involution in 95% No spontaneous involution,
by age 7 grow with individual
No treatment required in vast May or may not require
majority treatment
 ?What are AVMs

 AVMs are defects of the

circulatory system that
generally arise during
embryonic or fetal
development or soon
after birth.

 They consist of masses

of abnormal blood
vessels.
AVM & AVF
Capillary Malformation
Lymphatic Malformation
 Treatment indications
 Absolute
hemorrhage
Risk of high output failure
Chronic venous hypertension
Life threatening region
 Relative indication
 Pain of progressive nature
 Cosmetically sever deformity
 Vascular bone syndrome
 High risk of complications
 Recurrent infection
 Persistent lymph leakage
 MANAGEMENT
 Management of Arteriovenous malformation (AVM) remains a

major challenge to vascular surgeons.

 Management of AVM by a surgical and endovascular approach

can deliver excellent results with acceptable morbidity with no

recurrence in the early follow up period..

 AORTIC
ANEURYSM
 Definition
 localized dilation of an
artery that is 2 x its
normal diameter
 true aneurysm:

involving all vessel wall

layers

 false aneurysm:
disruption of aortic wall
with containment of
blood by some layers of
the aorta or a fibrous
capsule made of
surrounding tissue
 Etiology
 DEGENERATIVE (matrix metalloproteinases)
Degenerative aneurysms account for more
than 90% of all infrarenal AAAs.
 atherosclerosis association
 infection
 cystic medial necrosis
 trauma
 vascultitis
 connective tissue disease (Marfan
syndrome, Ehlers-Danlos)
 Clinical Features

 Vast majority ASYMPTOMATIC

 RUPTURE
back pain

hypotension/syncope

pulsatile abdominal mass

~100% mortality if untreated

Pulsatile abdominal mass
Atheroemboli from small abdominal
aortic aneurysms produce livedo
reticularis of the feet (ie, blue toe
.syndrome)
 Investigations
 abdominal US
(100% sensitive)

 CT

 Aortogram (false
negative normal
lumen size due to
thrombus
formation)
 Treatment
 Risk of rupture depends on size
<5 cm <5% / yr
5-6 cm 10% / yr
6-7 cm 15-20% / yr
>7 cm >20% / yr

 Risk of dying from aneurysm surgery = ~5%

 Treatment
 Operate when
 AAA reaches 5.5 cm in an otherwise healthy

individual
 >5 mm expansion in 6 months

 symptomatic AAA

 Rupture

 contraindications: life expectancy < 1 year, terminal

disease (cancer), significant co-morbidities (recent MI,
unstable angina), severe dementia, advanced age
 Treatment: Surgical
 Surgical options:

 open

surgery with
graft
replacement

 Endovascula

r aneurysm
repair
Venous Disorders

Ayman saad
Assist. Prof. & Consultant
Vascular Surgery
 Venous Disorders

Disorders of the venous system can be divided into

.thrombotic disease and venous insufficiency
Thrombotic disease of the veins can and does frequently
.lead to venous insufficiency
Venous insufficiency can be mildly symptomatic varicose
veins to severe chronic venous insufficiency (CVI) with
ulceration
.
 Chronic venous insufficiency
(CVI )
:Definition
CVI is disease of the lower limb veins in which venous
return is impaired usually over a number of years ,
. by reflux , obstruction or calf muscle pump failure
This leads to sustained venous hypertension and
ultimately clinical complications including odema , VV
.,eczema , lipodermatosclerosis and ulceration
 
Signs & Symptoms
 Poor cosmesis.
 Erythema and skin color changes.

 Lipodermatosclerosis.
 Itching and dull aching, burning,
pressure pain.  Varicose vein
 Lower limb edema.  Venous stasis ulcers.
: Causes of venous hypertension

A- Superficial venous reflux

LSV reflux-
SSV reflux-
B- Deep venous reflux and occlusion
1ry ( idiopathic )-
2ry go DVT or injury-
C- Perforating vein reflux
D- Abnormal calf pump
Neurological-
Musculoskeletal-
E- Combination of the above
: Etiology
To understand CVI , one must consider the
changes that occur on both the larger
veins ( macrocirculation ) and the capillary
bed ( microcirculation )
 
: Macrocirculation
During exercise in the normal individual effective
contraction of calf muscles combined with vein
patency and valvular competence aids venous
return and reduces venous pressure in the lower
. leg from about 90 mmHg to 30 mmHg
Failure of any of these mechanisms may result in
post-ambulatory venous hypertension which
. lead to CVI
 
The ambulatory venous pressure
is defined as end-exercise venous
( pressure at the ankle less 30mmHg
) normal is 15 -30 mmHg

At pressure less 30 mmHg, ulceration

is rare, while at pressure 90 mmHg,
there is nearly 100% incidence of
.ulceration
 
: Microcirculation
:The pathology in CVI is still not fully understood and the best explained by

: a- White cell trapping hypothesis

Increase venous pressure across the capillary bed , so white cell plug the
capillaries , adherence of white cells to the endothelium is then
. stimulated
The trapped white cells release proteolytic enzymes and O2 free radicals ,
causing endothelial and tissue damage . As a consequence , there is a
release of vascular endothelial growth factor
This increase microvascular permeability , which may explain the presence
of Fibrin cuff, and also produces excessive amount of nitric oxide . This
. in turn damages the tissue
  
:b- Fibrin cuff hypothesis

A rise in venous pressure causes capillary elongation

and widening of the pores between the endothelial
cells , this results in passage of larger molecules out
. of the intravascular compartment into the tissue
Fibrinogen is deposited and polymerises to form
fibrin . This may act as a barrier to O2 , resulting in
local tissue ischemia and cell death producing
. ulceration
 
c- A possible explanation lies with matrix
:metalloproteinases

These enzyme help remodel the extracellular

matrix by protein degeneration, and
enhanced MMPs activity has been
. demonstrated in lipodermatosclerosis
An imbalance in matrix turnover then exsits,
  leading to degradation and ulceration
 
 : Clinical features

swelling : due to odema fluid , which is initially -1

. pitting , then fibrosis and induration occur
skin changes : varicose eczema common as -2
. dry , scaly and itchy skin
Skin become friable with pigmentation due to
deposition of haemosiderin in the tissue ,
produces a brown discoloration which together
.with fibrosis lead to lipodermatosclerosis
ulceration : it occur predominantly on the-3
lower leg on the medial aspect . these are
usually surrounded by eczema or
pigmentation and exude fluid that can cause
.maceration of the surrounding skin
.varicose veins -4

pain : patient complain of a general ache and -5

heaviness in the leg of long periods of
standing . this is worse towards the end of
. the day but improve with elevation
 CEAP classification of severity &
etiology of lower venous disease :
 CEAP Classification (1996):
 C = Class (0-6)
 E = Etiology (congenital, 1ry, 2ry)
 A = Anatomy (Superficial, Deep,
perforators)
 P = Pathology (Reflux, Obstruction,

both)
(C) Class
 Class 1

 Class 2

 Class 3

 Class 4

 Class 5

 Class 6
(E) Etiology
 Congenital

 Primary

 Secondary
 Congenital

 Valvular aplasia or dysplasia.

 Primary
 Structural abnormality in the wall

or valve.
 Valve is redundant & have

tendency to evert.

 Secondary
 Post-thrombotic.
(A) Anatomy (P) Pathology

 Superficial  Reflux

 Deep  Obstruction

 Perforators  Both
 INVESTIGATIONS
Hand – held Doppler probe 

:Duplex ultrasound
gold standard for assessing
venous competence, allows
measurement of flow through
valves and identification of
sources of venous reflux &
provides functional as well as
.anatomical information
The red color demonstrates forward flow in the
common femoral vein, and the blue color
represents reversed flow in the incompetent long
.saphenous vein
Magnetic Resonance Venography
Contrast Venography
 Venography:
 Ascending
 Descending

 Used to detect:
 Obstruction
 Incompetence: - Deep
- Superficial
- perforators
 Management
Non-operative
 Leg elevation.

 Exercises.

 Compression stockings.

 Unna boots.

 Pharmacological.

.
Operative Management
 Indications:
 Failure if conservative treatment in class 4 or
above.
 Cannot tolerate conservative treatment.
 Massive reflux.

 Contraindication:
 Painless swelling without skin changes.
Deep Veins Valve incompetence
Valvoplasty

 Most frequent procedure used

in primary deep reflux.

 >70% achieved good clinical

outcome after 5 year follow up.
(French article)

Or Valve transfer
Deep Vein Obstruction
Bypass graft

 Synthetic

 Saphenous
 varicose veins

 
:Definition
superficial vein showing elongation , dilatation
.and , tortuosity (sacculation)
 
 :Pathophysiology
:VVs are always due to superficial venous reflex which is due to
primary valve failure: primary degenerative changes in -1
.the valve annulus and leaflets
secondary valve failure: developmental weakness in the -2
vein wall leads to 2ry widening of the valve commissars and
.incompetence
 
It's likely that both mechanisms are involved in different ●
. Pt's
 
  
 Also can classify VVs as
: Primary
Unknown cause ; often familial & probably a
.weakness of vein walls
: Secondary
: Obstruction to venous outflow
Pregnancy
Ovarian cyst
Abdominal LAP
Retroperitoneal fibrosis
Pelvic cancer
Valve destruction : DVT
High flow & pressure : AVM
 Risk Factors : 1ry VV
A number of these factors are believed to mediate ther
effects through increase LLs hydrostatic pressure
directly or through an increase in intra-abdominal
pressure with resultant compromise in blood return
.to the iliac veins

Heredity: the genetics of vvs is not understood

Age
Gender
Occupation (( prolonged standing with strong evidence))
Obesity : A BMI of greater than 30 kg/m2 carries of five
.fold increase in the prevalence of vvs mainly in women
Pregnancy: Although the enlarging uterus is believed to
obstruct venous return & contribute to the formation of
vvs , most women report an increase in vvs during their
1st trimester , when the gravid uterus is not yet large
enough to cause mechanical obstruction .The role of
estrogen on smooth muscle relaxation has been well
.described
Women with multiple pregnancies also appear to have a
.higher prevalence of vvs
Hormones ((oral contraceptive or hormone replacement
therapy))
 : Types of varicose vein
: primary trunk VVs )1
These are derived from the long saphenus vein
. and \ or their major tributaries
:secondary trunk VVs)2
Dilated superficial trunk veins may be acting as
collateral venous return in the presence of
. deep venous disease
: reticular varices )3
These vein lies just beneath or in the deep
layers of the skin and even when normal ,
may form visible reticular pattern of dark blue
. veins
 : hyphenweb veins )4
These dilated intradermal venules ( also called spider
veins , telengectasia , phlebectasia and venous
. flares )

: vascular malformations )5
The commonest venous malformation of the leg is
Kippel-trenaunay synelrom (KTS ) . KTS is
characterized by port-wine stain ,VVs , and bony
and soft tissue hypertrophy . in KTS surgery is
. best avoided
 
 :Treatment

Compression therapy-1
:Surgery-4

Surgical treatment provides symptomatic relief and

significant improvement in quality of life in patient
.with uncomplicated varicose vein

High ligation of long saphenous vein at SFJ with

stripping of long saphenous and stab avulsion
 
Sapheno-pop ligation
 
Perforator ligation by open surgery or superficial
endoscopic perforator surgery (SEPS)
  
: Vein Stripping

The surgery involves making one .

or more incisions upon the
desired area (usually the groin
or leg) followed by insertion of
.a special wire into the vein
The wire is tied to and advanced.
through the vein to a desired
.depth
The vein is then pulled out from.
.the body
The incisions are stitched up, and.
pressure dressings are applied
.to the incision
 Endovenous laser ablation (EVLA)

Endovenous laser treatment uses a laser probe to induce

.thermal damage
Radio frequency ablation (RFA)

RF pulses are used to heat the vessel wall

.resulting in closure and fibrosis
 Catheter Directed Foam
Sclerotherapy
- Sclerotherapy: injection of 1% to 3% solution of
sodium tetradecyl sulfate, or 5% morrhuate
sodium.
- Used alone for isolated varicosity or to
supplement surgical stripping
THE MANAGEMENT
OF LYMPHATIC
DISORDERS
 Lymphedema
When the transport capacity of the
lymphatic system is reduced by
obstruction or abnormal development of
the lymph vessels or lymph nodes,
protein-rich interstitial fluid accumulates
.and lymphedema develops
 Anatomy
The lymph draining from both lower extremities,
the gastrointestinal tract, and the left upper body
(upper extremity, chest wall, upper back, shoulder,
and breast) enter the venous circulation through
the thoracic duct, which opens into the venous
angle between the left subclavian vein and left
.internal jugular vein
The lymph draining from the right upper body
drains into the right venous angle via the right
.lymphatic duct
 ”Review of the “Flow
Interstitial fluid has The capillaries join
The lymph capillaries
diffused from blood to form vessels that
absorb this interstitial
capillaries carry the lymph fluid
fluid
back to the heart.

Thoracic Duct

The lymph fluid then On the trip back, the

enters either the thoracic lymph fluid passes
duct or right lymphatic duct through lymph nodes,
where it is cleaned and
filtered

Mader, 1994
The fluid is then enters the
subclavian veins and is returned
to the circulatory system.
 Primary lymphedema
Primary lymphedema is due to a congenital and/or inherited
condition associated with pathologic development of the
lymphatic vessels, which may include the following
:abnormalities

i. Reduced numbers of lymphatic collectors

ii. Lymphatic hypoplasia (decreased diameter of the lymphatic vessels)
iii. Lymphatic aplasia (absence of lymphatic system components)
iv. Lymph node fibrosis
 Primary lymphedema
Classification by morphology

Aplasia
no collecting vessels can be identified
Hypoplasia
Diminished number of lymph vessels
Numerical hyperplasia
Increased number of vessels
Hyperplasia
Increased number of vessels
Valvular incompetence
Tortuousity and dilatation (megalymphatics,
lymphangiectasia)
 Primary lymphedema
Classification by age of onset
Congenital lymphedema

 May involve a single extremity, multiple limbs, the

genitalia or the face
 Occurs before the age of 2 years
 Can be associated with hereditary syndromes .
 Primary lymphedema
Classification by age of onset
Lymphedema praecox

 Most common form 94%

 More common in women 10:1
 Onset during childhood or early
teens
 Involves the foot and calf, usually
unilateral.
 lymphedema praecox is
better known as Meige's
disease
 Primary lymphedema
Classification by age of onset

Lymphedema tarda

 < 10% of primary lymphedema

 Onset after the age of 35 yr
 Invovlment non-uniform
 Secondary lymphedema
 Identifiable cause
 Malignancy, infection, autoimmune disorders
 In developed countries, malignancy or its
treatment is the leading cause, mainly axillary
dissection for breast cancer .
 Worldwide, the most common cause of
secondary lymphedema is filariasis, due to
infection by the nematode Wuscheria bancrofti .
 Diagnosis
History & physical examination

 Heaviness and fatigue of the affected limb

 Limb size increases over the day and reduces at night time
 Family history
 Past history of malignancy, surgery or irradiation
 Travel history
 Hyperkeratosis
 Weeping fluid from lymph filled vescicles
 Stemmer sign (in which the examiner is unable to tent
the skin of the interdigital webs, based on the
characteristic loss of cutaneous elasticity in
lymphedema )
The distribution of
swelling in lymphedema
is characteristic. It starts
distally in the extremity
in most patients and
involves the
perimalleolar area, with
disappearance of the
contours of the ankle in
advanced cases (tree
trunk or elephantine
configuration).
Lymphedema of the
forefoot showing the
typical “buffalo
hump

Squaring of the
toes, small verrucae
of the skin, and
onychomycosis in a
patient with primary
lymphedema
praecox
 Diagnosis
Clinical staging;
It permits evaluation of effectiveness of treatment and
comparison of different modalities.

 Latent phase: excess fluid accumulates and fibrosis occurs

around lymphatics. No apparent edema
 Grade I: pitting edema that resolves with elevation of the limb.
No clinical evidence of fibrosis
 Grade II: non- pitting edema that does not resolve with
elevation. Moderate to severe fibrosis is evident
 Grade III: irreversible edema, repeated inflammation, fibrosis
and sclerosis of the skin and subcutaneous tissue. Lymphostatic
elephantiasis.
 Diagnosis
Diagnostic modalities ;

 Duplex ultrasonography
 Isotopic lymphoscintigraphy
 Indirect/ direct lymphography
 Lymphatic capillaroscopy
 MRI and CT scanning
 Complications
Infection
 Protein rich fluid provides a good medium for bacterial
growth.
 Lymphatic dysfunction impairs local immune responses
 Soft tissue infection further exacerbates the edema.

Malnutrition and immunodificiency

 Lymphangiectasia may be complicated by protein losing

enteropathy, chylous ascites or chylothorax resulting in severe

protein, long chain triglyceride and calcium loss
 Loss of lymphocytes, immunoglobulins and cytokines
 Complications
Malignancy

 Lymphangiosarcoma resulting from long standing

lymphedema
 Presents as multicentric lesions with bluish nodules,
sclerotic plaques or bullous changes
 Other malignancies include Kaposi’s sarcoma, lymphoma
and melanoma
 May result in limb loss or death .
 Management
Nonoperative treatment
Strategies to reduce edema
 Limb elevation
 Compression garments
 Sequential pneumatic compression
 Lymphatic massage
 Exercise
 Weight reduction
 Management
Surgical treatment
 Patient selection
 Proper workup

 A thorough understanding of the

spectrum of options
Types of Surgical treatment
 Surgical excision

 Liposuction

 Lymphatic diversion

 Lymphatic, venous, artificial grafts