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vascular malformation

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Hamartoma:(missing the mark in spear throwing)

tumor like

developmental malformation

of various tissues of which the part of the region is made of

haemangioma: vascular tumor like structure present at / around/after birth
malformation-abnormal sized and numbered blood vessel
Mulliken and Glowacki classified VM on their (i)biological behavior and (ii)endothelial structure in 1986
Self involuting tumor that disappear gradually(haemangioma) and persist permanently(Malformation)
In 1996 International Society of vascular anomali encompass all these in a common term of” VASCULAR ANOMALI” and divided them into(A.) “tumor “and “ (B)malformation”

Epidemiology- 10% of total child hood tumor
M:F=1:3
Site-face and neck-80%,next-liver
Vascular anomaly
A.tumor
i. Infantile haemangioma
ii. Haemangio endothelioma
iii. Congenital haemangioma(RICH,NICH)
iv. miscellaneous
B.malformations
a.High flow
i.simple Arterial(AVM,AVF
duplication,
hypoplasia
stenosis/coarctation,
Dilatation-ectasia,aneurysm
ii.AVF
single arterio venous
combination
iii.AVMs
localized mass of small communications(small inflow arteries)
truncal- large inflow arteries and distended outflow veins)
Diffuse– large inflow arteries with rapid filling surrounding venous tree(more on lower limbs)
High shunting
b.low flow
i.capillary-port wine stain
ii.lymphatic
iii.venous
.simple
Absence
Hypoplasia
duplication
valvular anomaly
abnormal enlargement
.spongy
C.combined
Parks Weber syndrome
Kipple Trenauray syndrome

presentation
scratch mark,brise,red or pink area surrounding pallor/erythematous macular patch/ ripen straw berry like growth
fast growing (faster than child’s growth)
noticed by parents during giving bath with in 1st year
involution from end of 1st year and takes upto 5th to 9 th year even upto 12 years.after 7 years 50% involution is evident

    common questions
1.Name age address occupation
2.Whats your sufferings
3.How long you are suffering from this
4.How it started
5.Is it painful at all
6.Is there any thing alike any where else
7.Does it go back/changes in size
8.Did you have any treatment before for this

9.family history

10.any other relevant query

history of trauma

is there any epilepsy/convulsion

sight disturbance

bleeding history

bone pain / deformity

 

 

10.examination

   i.local

 

INSPECTION

Inspection(start with skin condition,end with special tests like caugh,deglutation/potrution of tongue)-7S

 

1.single/multiple
2.site
3.size
4.shape
5.spread(border/edge/extent)
6.skin condition(colour change,discharge,punctum,hair distribution,scar,visible engorged vein,pulsation)
7.special(caugh impulse,deglutation,potrution of tongue)

 

palpation

Palpation(start with back of hand/temparature and end with lymph node/distal pulse/sensation/joint movement)-11T

1.temparature
2.tenderness
3.Top/surface
4.texture/consistency
5.temper/flactuation(if soft in consistency)
6.transillumination
7.thrill9expansibility/pulsatality)
8.twiching(compressible/reduciblity)
9.tap
10.tethering(overlying skin/underlying structure at contraction against resistance)
11.special9distal pulse/sensation/joint movement/draining lymph node

 

percussion

 

Auscultation

 

ii.other systemic examinations

oral cavity

neurological

eye

musculo skletal

there is diffuse   purple/dark red discolouration over right side of face extending from fore head to chin incluidung right peri orbital area nose lip and right side of neck measuring about 20x 15 sqcm

which is worm  non tender  with ill defined edge    irregular surface   soft  in consistency blanches on pressure  non pulsatile   non / partially compressible overlying skin cant be lifted up and free from underlying stractures

no regional lymphadenopathy

oral cavity normal

my provisional diagnosis is vascular malformation due to haemangioma most probably capillary haemangioma (port wine stain variety/naevus flammeus)

Differentials

why its haemangioma

i.staining

ii.since birth

iii. no evolution so far

iv.soft

v.irregular surface

vi.blanches on pressure

why its capillary haemangioma

i.diffuse staining

ii.purple to dark red staining

iii. no evolution so far

iv.soft

v.irregular surface

vi.blanches on pressure

vii.not pulsatile(so not arterial)

viii.not compressible(so not cavernous)

ix.overlying skin cant be lifted up(so less chance of cavernous)

why it is port wine stain

i.since birth

ii.no involution so far

confirmation:by color doppler study

investigations

 

a.For confirmation of diagnosis

b.For detection of distant Extension

CT- brain-as ipsi hemispherical haemangioma present in sturge weber syndrome

CT abdomen as visceral (GIT,GUT,liver haemangioma are not  un common(Osler Rendu Weber syndrome)

bone scan/simple xray-(as osteo hypertrophy present in Klippel Trenauny weber syndrome)
c.For diagnosis of Co morbidities/end organ damage

d.For Fitness

e.For follow up /prognosis

f.For planning

treatment:

  a.conservative

          councelling and assurance

          skill full cosmetic

  b.interventional

         laser:

         cryo therapy

         surgery:excision followed by skin graft

there is swelling over root of nose measuring approximately 3 x 2  sq cm elongated and  hemispherical in shape dark red in colour with few hypertrophic scars and pitted epithelium on it without any visible caugh impulse

which is worm  non tender  with ill defined edge    irregular surface   soft  in consistency non blanching   non pulsatile   but  partially compressible overlying skin cant be lifted up and free from underlying stractures

no regional lymphadenopathy

oral cavity normal

my provisional diagnosis is vascular malformation due to recurrent haemangioma most probably capillary haemangioma (strawberry variety)

Differentials

recurrent menigocele

recurrent dermoid

recurrent lipoma

why its haemangioma

i.since birth

ii. no evolution so far

iii.soft

iv.irregular surface

v.partially compressible

why it is strawberry haemangioma

i.appeared 2 weeks after birth and still persisting

ix.overlying skin cant be lifted up(so less chance of cavernous)

why its recurrent

previous incision  mark is present

what extra caution to be taken to diagnose a swelling at this site?

meningocele and dermoid must be ruled out incase of any midline swelling

confirmation:by color doppler study

investigations

 

a.For confirmation of diagnosis

color doppler study

FNAC

b.For detection of distant Extension/exclusion of differentials

xray skull( to exclude meningocele)

 

c.For diagnosis of Co morbidities/end organ damage

d.For Fitness

e.For follow up /prognosis

f.For planning

treatment:

  a.conservative

          councelling and assurance

          watchful waiting

                  salmon patch-up to 1-2  years

                  port wine stain-persists

                  strawberry-upto 8 years even upto 12 years

          skill ful application of cosmetics(in port wine stain)

  b.interventional

      non surgical

          repeated sclero therapy-hot water,hypertonic solution,hypertonic glucose,corticosteroid

          laser:Nd:YAG,Co2

         cryo therapy-CO2 snow

         surgery:

                 small-excision followed by skin graft

                  large-identification and ligation of feeding vessels followed by excision + skin graft after shrinking  the size by repeated sclerotherapy

c.radio therapy by radium ion

ulcerated vascular malformation involving upper lip and left side of face probably due to capillary haemangioma

ulcerated vascular malformation over upper lip probably due to capillary haemangioma

 

cavernous haemangioma

vascular malformation over right cheek probably due to capillary haemangioma(strawberry variety) in a 2 years old boy

two vascular malformation over right cheek probably due to capillary haemangioma(strawberry variety) in a 13months old boy

 

 

arterial haemangioma

INSPECTION

Inspection(start with skin condition,end with special tests like caugh,deglutation/potrution of tongue)-7S

 

1.single/multiple
2.site
3.size
4.shape
5.spread(border/edge/extent)
6.skin condition(colour change,discharge,punctum,hair distribution,scar,visible engorged vein,pulsation)
7.special(caugh impulse,deglutation,potrution of tongue)

 

palpation

Palpation(start with back of hand/temparature and end with lymph node/distal pulse/sensation/joint movement)-11T

1.temparature
2.tenderness
3.Top/surface
4.texture/consistency
5.temper/flactuation(if soft in consistency)
6.transillumination
7.thrill9expansibility/pulsatality)
8.twiching(compressible/reduciblity)
9.tap
10.tethering(overlying skin/underlying structure at contraction against resistance)
11.special9distal pulse/sensation/joint movement/draining lymph node

 

percussion

 

Auscultation

 

ii.other systemic examinations

limb length

limb girth

pulse-collapsing pulse

BP

Branhams/Nicoladoni’s sign

crisoid aneurysm over left mid arm

there is diffuse   reddish/pinkish  swelling over extensor surface of left fore arm  measuring about 10x 5 sqcm with visible tortuous veins and few nodules

which is worm  non tender  with ill defined edge    irregular surface   soft  in consistency feeling like a bag of earth worm blanches on pressure   pulsatile thrilling  compressible overlying skin cant be lifted up and free from underlying stractures

on auscultation there is audible bruit

limb lengths are equal but  girth is more in left fore arm are ,pulse is 88 beats/min and collapsing and BP is 140/90 mmHg Branham’s test is positive

no regional lymphadenopathy and distal sensation and joint movements are well intact

my provisional diagnosis is vascular malformation due to haemangioma most probably arterial/crisoid aneurysm(congenital AVF)

Differentials

why its haemangioma

i.staining

ii.since birth

iii. no evolution so far

iv.soft

v.irregular surface

vi.blanches on pressure

vii.pulsatile/thrill

viii.compressible

why its arterial haemangioma

i..compressible(so not cavernous)

ii . pulsatile(so  arterial)

iii. no involution so far

iv.soft.feels like bag of worm

v.irregular surface

vi.blanches on pressure

vii..reddish/pinkish

viiidiffuse ,elevated

ix.overlying skin cant be lifted up(so less chance of cavernous)

why it is crisoid

i.elevated from skin/raised from surface

ii.no involution so far

iii.diffuse

iv.visible tortuous veins,thick walled veins

v.pulsatile

vi.bruit present

what the nodules signify?

thrombosis/incomplete resected veins

confirmation:by duplex study

investigations

 

a.For confirmation of diagnosis

duplex study

b.For detection of distant Extension

local part xray-osteoporosis/thinning out/destruction

CXR-cardiomegaly

ECG

Echo

 

c.For diagnosis of Co morbidities/end organ damage

d.For Fitness

e.For follow up /prognosis

f.For planning

treatment:

  a.conservative

          councelling and assurance

  b.interventional

     i.  quadriple ligation with  bypass revascularization

    ii.  selective embolization

     iii. amputation

congenital AVF/arterialization of vein

macrodactili of 2nd toe (left) due to AVM

giant middle finger due to congenital AVM

giant right 3rd toe with soft tissue swelling over sole of the foot

 

 

salmon patch

i. mid line (fore head/occiput/mid chest/neck)

ii.from birth

iii.disappear after 1 year

iv.treatment is councelling assurance watchful waiting for 1 year

FAQ

1.hall mark of hamartoma

improper distribution with prominance of one particular tissue

2.example of hamartoma

blodd vessel:haemangioma

lymphatics:lymphangioma,cystic hygroma

principle nerves: neurofibroma

skin:mole,pigmented naevus,lipoma

neck:TGC,brancheal cyst,dermoid

bowel:congenital polyp

3.charecteristics of hamartoma

i.presence since birth

ii.growth proportional to physiological growth of same tissue upto adulthood and growth ceases when physiological growth is ceased

iii.usually persists but some may regress spontaneously(salmon patch,strawberry haemangioma)

iv.may be multiple(lymphangioma simplex,neurofibroma)

v.very rare malignant transformation

4.association with port wine stain?

i. ipsi hemispherical haemangioma present in sturge weber syndrome

jacksonian epilepsy,mental retardation,glaucoma

ii.GIT,GUT,liver haemangioma are not  un common(Osler Rendu Weber syndrome)

iii. osteo hypertrophy present in Klippel Trenauny weber syndrome)

5.common sites of haemangioma

i.cutaneous

ii.sub cutaneous

iii.internal organ

face,neck,tongue,lips,oral cavity are more common

6.natural history of haemangioma

i.proliferative phase

ii.involution phase

7.natural history of strawberry haemangioma?

start as a red mark(after 1st week to 3 months)

proliferation up to 1st year

disappear by 7-8 years(even 12years)

hemispherical

8.haemorrhage common in ?

sub cutaneous variety

9.types of lymphangioma

i.L.simplex/circumscriptum(superficial variety)-blisters/slightly elevated pach,multiple vesicles

ii.cavernous-from larger lymphatic channels

iii.cystic hygroma-multiloculated cystic lesion

10.what is lymphangioma

localized dilatated lymphatic sac that fail to connect to lymphatic system

11.charecters of lymphangioma

i.from birth

ii.junctional area(limb with trunk,neck with trunk/around axilla,shoulder,buttok,groin,root of neck)

iii.contains yellow/white/watery fluid/brown or black if there is blood

iv.brilliantly translucent

11.features of congenital AVF

i.more diffuse

ii.bone involvement/destruction

iii.local giantism

iv.port wine stain and superficial angiomata

v.selective embolization is needed

12.effect of con.AVF ?

a.structural

arterialization of vein

b.physiological effect

i.increased venous flow>increased venous return>high BP>heart failure

ii.giantism

iii.ulceration

iv.bony destruction

13.Branham’s test

pressure over proximal of fistula causes

decreased swelling

decreased thrill

decreased pulse

decreased pressure

14. indication of amputation

severe pain ishchaemia

ii.CF

iii.giant limb

iv.failed Rx(ligation /embolization)

15.why ligation is not always ideal

as there are many bypass communications other than the actual prime AVF

 

 

 

 

10.

 

points Salmon patch PWS strawberry crisoid cavernous
onset birth birth 1wk – 3 mo birth birth
Start as Patch/stork bite Diffuse telangiectasia

Red mark

Raised from skinCongenital AVF involution1yearnever7-8 years/12yearsneverneversiteMid line

Fore head,occiput,chest,neckAny where,mucous membraneHead neckLimp,scalpAny wheresize Diffuse,flashed with skinHemispherical

1-2 cmRaised,diffuseraisedshape     colorBright redPurple/dark redBright red/darkRed/pink with black nodulesPurple/bluishSkin condition  Pitted epitheliumTortuous veinsPhlebolith/black nodule due to venous thrombosistempwormwormwormwormwormtenderness     surfaceirregularirregularirregularirregularirregularconsistencysoftsoftsoftSoft/bag of wormspongyblanchingyesyes   compression  √√√pulsatile   √ bruit   √

 

 

 

10.points differentiating  haemangioma and  lymphangioma?

points haemangioma lymphangioma
birth From birth(except strawberry) From birth
single May be multiple(H.simplex)
Color*** Color change evident not
temparature warm normal
tenderness Non tender Tender if infected
Consistency*** soft Soft cystic
fluctuation +ve +ve
Transillumination*** -ve Brilliantly positive,except infected and haemorrhage
Pulsality*** +ve if arterial -ve
Compressibility*** +ve -ve
LN -ve +ve if infected
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