Vascular anomalies -د.سمير جبعيتي

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هذه محاضرة ال vascular anomalies للدكتور سمير جبعيتي

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Vascular anomalies

Vascular anomalies: Are congenital anomalies caused by abnormal growth of blood vessels, leading to masses originating and consisting of blood vessels with variable shapes.

Classification:
1-According to morphology:
(Salmon patch, strawberry hemangioma, port wine stain, cherry)

2-According to the diameter of the blood vessel:
Thin small channel: capillaries wide cavernous
Mixed: cavernous and capillaries

3-According to the histological features of the endothelial cells:
Will be divided into two groups:

Group 1 Hemangioma:
This definition is restricted to vascular anomalies caused by endothelial
Hyperplasia
Consisting of young endothelial cells that are: (Plump, active with high mitotic activity, high no. of mitotic figures indicating division of endothelial cells.
Have receptors for cortisone to mediate cellular proliferation).
In between the endothelial cells there are mast cells.
Considered as embryonic cells with short doubling time
Have 2 phases: proliferative and involution.
In the proliferative mast cells will increase in no. playing a role in neoangiogenesis, so the lesion will expand and rapid growth will occur till the age of one year, then the lesion will stabilize, then starts to involute which is characterized by a decrease in size ,fading of color.
So all in all, it will start as small lesions at the age of 3-4 months; they will grow to reach their maximum size at the age of one year, then involutes and disappear to become a fibro fatty skin.
Will grow and behave as a neoplasm forming blood vessel, so were called hemangioma, and is considered as the most common tumor of infancy.
Female to male is 3:1.
80% are solitary, 20% are multiple.
Most of them (60%) are in the head and neck, less in trunk and less in the extremities.
Resolution is complete in 50% of cases by the age of 5 years.

So all in all, hemangioma is one of the 2 divisions of vascular anomalies, forming blood vessels, multiply, proliferate, involutes and disappear.
So no need to treat, it resolves spontaneously unless complicated you should treat.

Complications:
1-the most important is that hemangioma can grow in the eyelid obstructing the eye leading to Amblyopia: lazy eye. This is considered as no. 1
2-bleeding.
3-very large one entrapping platelets leading to thrombocytopenia; Kassbach-Merit syndrome.
4-skeletal distortion.
5-congestive heart failure due to multiple hemangiomas.
6-ulceration and infection.

Treatment:
If complicated, no.1 treatment is to use steroids. They will induce involution.
And only if complicated.
Other methods are to use laser or operation.

Group 2: Vascular malformation
They are structural abnormalities resulting from errors in the morphogenesis of embryonic vessels between weeks 4-10of gestation.
Almost always sporadic.
They appear at birth, but not always congenital, can appear years after birth the endothelial cells are normal, mature cells with normal turnover rate throughout their natural history.
Grow parallel to the child's growth, being part of him.
Have no receptors for cortisone, no mast cells.
Can be one of tow types: either high flow or low flow (capillary, venous, lymph or combined).
Never goes spontaneously.
It may need treatment if complicated.

Complications: 1-erosion of bones leading to fractures.
2-stealing blood from a limb leading to atrophy of distal parts.
3-entrapment of platelets.
4-bleeding.
Treatment:
1-Surgery 2-Laser
3-Embolization: by injecting material embolizinng the feeding artery.

So all in all, grouping up information:
Vascular malformation
Usually seen at birth but may appear late.


Grows as same rate as the child

Female: male is 1:1

Low flow VM lead to diffuse hyperplasia
.
High flow can lead to destructive changes. Hemangioma
Not seen at birth, usually seen as small maculae after birth.

Rapid post natal growth then involution.

Female: male is 3:1

Large ones lead to platelet entrapping and thrombocytopenia.

Rarely to cause any complications.

Color (pigmentation) will not distinguish between hemangioma and vascular malformation.

Why is it important to differentiate between vascular malformation and hemangioma?
If it is hemangioma you reassure the family, it will involute and resolve
Spontaneously.

Port Wine stain:
They are capillary vascular malformation, not hemangioma.
Usually not evident at birth.
Mentioned by the dr. as being part Sturge-Weber syndrome.


In the OSCE , the questions will be as the following:

CASE 1:
The patient is presented to you having a skin lesion.
1st of all, the color (pigmentation) of this spot is it due to melanin (brown) or due to RBCs presented in blood vessel (red, pink,).
If it was brown or its degrees it will be called nevus (شامة، خال), and on average each individual has around 30 nevi.
If it was due blood vessel containing RBCs color will be ranging from red, pink, and violet.
So by looking at the spot you should think of 2 possibilities: either it is a nevus or a vascular anomaly, depending on color.

CASE 2:
The patient is9months, presented to you having a neck spot:
1st think is it a nevus or vascular anomaly? Depending on the color it was violet.
2nd is it vascular malformation or hemangioma? Depending on HISTORY.
What to ask, so as to have proper history from the mother is the following:
1-Was it presented at birth?
2-When did it start?
3- Is it increasing in size?
4-Is it changing in color?

The answers were, no not at birth it started the age of 4 months was increasing in size becoming darker.
So diagnosis by history is hemagioma.
Prognosis:it will go spontaneously after the age of 1 year.

CASE 3:
the patient is 1.5 year of age ,the mom is saying he had a spot starting as small , then enlarging becoming darker finally becoming white .
So what's your Dx ?
It is hemangioma , becoming white indicating mottling.

So all in all; in exams the question will be as photos, the questions you should ask:
1-what is it vascular anomaly or nevus?
2-is it hemangioma or vascular malformation?
3-what is the prognosis?
4-what are the indications for treatment? What is the first line of treatment?

Port-wine stain: capillary vascular malformation not ( hemangioma) .
Usually evident at birth.
Facial lesions are restricted to one of the three trigeminal sensory area (ophthalmic, maxillary, mandibullar ) but some can occur bilaterally ,cross the midline or overlap more than one division.

They are flat and sharply demarcated, grow proportionally eith the child.
Their surface is studded with nodules.
Can be part of Sturge-Weber syndrome:
(1-vascular malformations of the face, chorroid plexus and meninges.
2-the stain mainly involving the ophthalmic division.
3-local or generalized seizures.
4-intracerebral glaucoma.
5-ipsilateral glaucoma.)

Treatment of port-wine stain:
Scarification, LASER, surgical excision, tattoos and cosmetics.




Other information not mentioned by dr.Samir is in doseat Plastic Surgery pages (109-111).
The dr. emphasized many times, this is the info. You should know and you will be asked about.