VASCULAR MALFORMATION

By :
Dwiana ardianti (G99142004)
Andreas Agung K (G99142007)
Novian Anindito (G99142008)
Mutiani Rizki (G99152073)
Okky Dhevi S (G99161070)
Yolanda Ravenia S

(G99161105)

Preceptor:

dr. Amru Sungkar, Sp.B, Sp.BP-RE(K)

KEPANITERAAN KLINIK SMF ILMU BEDAH

FAKULTAS KEDOKTERAN UNS/ RSUD DR MOEWARDI
SURAKARTA
2016

Background

An understanding of the term hemangioma and vascular malformation is not

well defined till now

Previously referred to as vascular birthmarks, vascular anomalies are now

classied based on a system developed in 1982 by Mulliken and Glowacki that
considers histology, biological behavior, and clinical presentation of these
entities

That classification is the most widely used and accepted for now

(Adegboyega P and Qui S, 2005 )

VASCULAR MALFORMATION

Classified by the dominant vascular type and its flow there are :

1.

Slow flow that include capillary, venous and lymphatic malformation

2.

Fast flow that include arterious malformation, arteriovenous fistula and

arteriovenous malformation

(Riechter and Friedman, 2012)

Capillary malformation

Capillary malformation commonly referred to as port-wine stain

Capillary malformations are sporadic lesions consisting of dilated capillarylike channels

It can present on anypart of the body, but are mostly found in the
cervicofacial region
(Eerola et al, 2003)

Etiology

The etiology of this dissease is unknown but it is suspected that this occurs
because the abnormal formation of capillary in the early life of the embryo
after the blood vessels are formed

Diagnosis

(Eerola et al, 2003)

Capillary malformation present at birth as at, red or purple, cutaneous patches

with irregular borders. They are painless and do not spontaneously bleed

Capillary malformation tend to progress with time as the vessel ectasia extends to
involve deeper vessels to the level of the subcutaneous tissues. This causes the
lesion to become darker in color, as well as more raised and nodular

(Riechter and Friedman, 2012; Tark et al, 2011)

Therapy

Laser therapy: The laser slowly causes the redness of the lesion to fade.
Early treatment of these lesions appears to slow the progression of the
disease

Surgical excision : an option in lesions not amenable to laser therapy dan if

the lesion has invaded into soft tissue or the bone

(Eerola et al, 2003; Chiu A et al, 2011)

Venous malformation

Venous malformations are the abnormal formation and enlargement of the

superficial veins and profunda veins.

The growth of venous malformations is slow and steady, but if there are
trigger such as surgery, trauma, infection, or hormonal changes associated
with puberty, pregnancy or menopause can cause rapid growth

The lesions of venous malformations can be found on the skin, mucous

membrane or organ system (brain, intestine, liver, and spleen)
(Pavlov, 2009; Claudio et al, 2006)

Etiology

The etiology of this dissease is unknown but it is suspected that this occurs
because of smooth muscle decreased on the blood vessel wall
(Claudio et al, 2006)

Clinical manifestation

Occurs since birth and progressive

Thrombosis often occurs due to the activation of protein C, protein S or other

antithrombin abnormalities

Bluish discoloration on the overlying skin

On histology, we can find the endothelial layer is thin and stiff.

(Pavlov, 2009; Claudio et al, 2006)

Diagnosis

MRI is the imaging modality of choice when diagnosing Venous Malformation

and oers superior delineation of disease for treatment planning

(Boon et al, 2004)

Therapy

Watchfull waiting : when the lessions is small that only affect the aesthetics

Bandage : is used to control swelling and pain in lesions on the extremities

Sclerotherapy: is used to shrink the abnormal blood vessels by injection at the

lesion area

Surgical excision : used if the lesion is localized

Low molecular weight heparin (LMWH) : is used for patients who have local
intravascular coagulopathy. It is used before and after surgical procedures.

(Boon et al, 2004)

Lymphatic malformation

Lymphatic malformations (LMs) are composed of dilated lymphatic vessels

with inappropriate communication, lined by endothelial cells and lled with
lymphatic uid

(Khunger N, 2010)

Etiology

The etiology of LM is unclear. Although most are congenital,there have been

reports of LM occurring after trauma or infection

Receptors involved in the formation of lymphatic vascular channels, such as

VEGFR3 and Prox-1, may play a role in the development of this disease
(Khunger N, 2010 ; Riechter and Friedman, 2012)

Clinical manifestation

Lesions are most commonly found in the neck and axilla but can also be found
in other body areas

There are two types of lymphatic malformations which are macrocytic and
microcytic malformations

In macrocytic malformation, lesions were found large, soft dan bluish

In microcytic malformation, lesions were found small, raised lesions

containing lymph fluid

Lesions can be suddenly enlarged and temporary on certain conditions, such

as trauma or infection.
(Khunger N, 2010)

Diagnosis

These malformations usually can be diagnosed by physical examination, but

we can suggest MRI examination to conrm diagnosis, identify cystic
architecture, and determine extent of disease
(Khunger N, 2010)

Therapy

Surgical excision : is performed on localized lession

Sclerotherapy : is done by directly inject irritant agents such as alcohol or

picibanil on makrositik lesions

Chemotherapy : is usually performed on lesions that can not be excised.

Examples of commonly used chemotherapeutic drug is rapamycin.
(Khunger N, 2010)

Arteriovenous malformation

Arteriovenous malformations (AVMs) are congenital highow vascular

malformations composed of anomalous capillary beds shunting blood from the
arterial system to the venous system

They are often misdiagnosed at birth as other vascular lesions because of the
delay in presentation of characteristic signs of the malformation. Puberty and
trauma trigger the growth of the lesion and manifestation of its troublesome
symptom

(Adegboyega P and Qui S, 2005 )

Etiology

Extrinsic factor : sytemic blood pressure, the hearts ability to pump the
blood into systemic circulation, the quality of blood vessel and blood viscosity

Intrinsic factor : autoregulation of cerebral arteri and regional biochemical

factor
(Adegboyega P and Qui S, 2005 )

Patophysiology

Pathophysiology of AVM formation is generally due to malfunctions

differentiation of primitive blood vessels in the embryonic age of 3 weeks

In AVM, occurs angiogenesis disorder of the vascular tissue. This will cause
blood accumulation on the blood vessel between the arteries and veins which
called nidus
(Vikkula M, 2007)

Clinical manifestation

Bleeding

Convulsion

Mass effect

Ischemia, steal phenomenenon

Cephalgia

Bruit; usually find in dural AVM

Increase of intracranial pressure

Hidrocephalus

Vertigo

Neck stiffness

(Vikkula M, 2007; Chiu A, 2011)

Patology anatomy
Nerve bundle

Movat pentichrome stained (D) sections showing the

presence of intralesional nerves in arteriovenous
malformation

(Adegboyega P and Qui S, 2005 )

Diagnosis

Diagnosis of AVM is based upon clinical examination and imaging (patology

anatomy, MRI or CTA)

A growing hypervascular lesion may have been present as a slight blush at

birth

The distinguishing characteristics of an AVM will be palpable warmth, pulse,

or thrill due to its high vascular ow

The overlying skin may have a well-demarcated blush with elevated

temperature relative to adjacent skin

Imaging is essential in identifying the extent of AVM. MRI may be useful, but
MRA and CTA can give a superior outline of these lesions
(Adegboyega P and Qui S, 2005 )

Therapy

Pharmacological treatment

Pharmacological treatment is done to overcome the symptoms such as headaches

or seizures. Phenytoin can be given to control seizures

Non-pharmacological

1. Resection: should be done at the AVM rupture and been anticipated results
were slightly better than the unruptured AVM.
2. Radiosurgery : is done by using a device called a gamma-knife, effective at
AVM measuring <2 cm
3. Conservative therapy : is an alternative therapy if the risk of the other
therapy is too much. Conservative therapy is to treat the symptoms that occurs
on the patient
(Chiu A, 2011)

Arteriovenous fistula

The occurrence of an AVF between arteries and veins is due to the persistence
of the relationship between the artery and vein formation

AVF can give symptoms soon after birth or no symptoms at all

Factors that can accelerate the onset of clinical symptoms is puberty,

pregnancy and trauma.
(Brinjikji W et al, 2015)

Diagnosis

Usually patients present with dilation of the veins, changes in color and
temperature of the parts involved

Sometimes there are palpitations, tachycardia or other cardiac abnormalities

In the area of fistula, there are murmur and thrill, and when we compress
this area, the pulse will decrease and systolic and diastolic pressures increase

A definitive diagnosis is obtained by arteriography. We can see arteries and

veins are filled simultaneously by the contrast material
(Brinjikji W et al, 2015; Brouillard et al, 2002)

Therapy

Total ressection

Embolization

Fistula ressection

Arteria ligation

(Brinjikji W et al, 2015; Brouillard et al, 2002)

CONCLUSION

Vascular malformations caused by abnormalities of the development of the

vascular system

An understanding of the terms of hemangiomas and vascular malformations

should be well understood

Diagnosis by history, physical examination, and supported examination are

needed to determine the therapy

DAFTAR PUSTAKA

Richter GT, Friedman AB. Hemangiomas and Vascular Malformations: Current Theory and Management.
International Journal of Pediatrics, Vol. 2012. 2012

Vikkula M. Angiogenomics: towards a genetic nasology and understanding of vascular anomalies.

Eroupean Journal of Human Genetics, 15,821-822. 2007.

Chiu A, et. al. Clonal X-chromosome inactivation suggests that splenic cord capillary hemangioma is a
true neoplasm and not a subtype of splenic hamartoma. Modern Pathology, 24, 108116. 2011.

Tark KC, Lew DH, Lee DW. The fate of long- standing port-wine stain and its surgical management.
Plastic and Reconstructive Surgery, vol. 127, no. 2, pp. 784791, 2011.

Pavlov KA. Expression of Growth Factors in Endotheliocytes in Vascular Malformations. Bulletin of

Experimental Biology and Medicine, Volume 47, Number 3. 2009.

Claudio P, Enrica R, Michele D. Immunodetection of the signal tranducer and activator of transcription-3
in canine haemangioma and haemangiosarcoma. Research in Veterinary Science, 80:186-188, 2006.

L. M. Boon, J. B. Mulliken, O. Enjolras, and M. Vikkula, Glomuvenous malformation (glomangioma) and

venous malformation: distinct clinicopathologic and genetic entities, Archives of Dermatology, vol.
140, no. 8, pp. 971976, 2004.

Brouillard P, Boon LM, Mulliken JB, et al. Mutations in a novel factor, glomulin, are
responsible for glomuvenous malformations ("glomangiomas"). Am J Hum Genet. 2002.

Brinjikji W, Nasr DM, Morris JM, Rabinstein AA, Lanzino G. Clinical Outcomes of Patients
with Delayed Diagnosis of Spinal Dural Arteriovenous Fistulas. AJNR Am J Neuroradiol.
2015

Adegboyega P and Qui S. Hemangioma versus vascular malformation: presence of nerve

bundle is a diagnostic clue for vascular malformation. Arch Pathol Lab Med, 129: 772-775.
2005

Khunger N. Lymphatic malformation: Current status. Journal Cutaneous and aesthetic

surgery. 3(3): 137138. 2010

Eerola, Laurence M, Boon, John B. Mulliken, Patricia E. Burrows, Anne Dompmartin, Shoji
Watanabe, Romain Vanwijck, and Miikka Vikkula. Capillary MalformationArteriovenous
Malformation, a New Clinical and Genetic Disorder Caused by RASA1 Mutations. American
journal of human genetics, 73:12401249. 2003

TERIMAKASIH