Vascular Anomalies - Hemangiomas and Malformations

Vascular malformations are groups of vessels that have formed improperly and don't have a high endothelial cell turnover rate.  There are two forms of vascular anomalies: Vascular Malformations and Vascular Cancers.  The histological characteristic of tumors is the proliferation of endothelial cells, either with or without luminal structure.  Endothelial cells proliferate significantly in vascular tumors, but not in vascular malformations due to lack of substantial proliferation. 

Vascular Tumors

 Hemangiomas rank among the most prevalent vascular tumors. Children frequently exhibit them. Tufted angiomas, Hemangiopericytomas, Hemangioendotheliomas, and malignant ones can be Agiosarcoma.

Hemangiomas

About 20% of premature babies have this condition. The ratio of female to male is F: M-4:1.  More frequently observed in kids with pale complexion. Rare in people with dark skin. These are benign tumors, which are groups of angioblast-formed primitive blood arteries.
Although they can happen anywhere in the body, the head and neck account for about 60% of cases. 20% of cases are multifocal.  The most frequent place is the skin.  The most frequent extracutaneous location is the liver.  Hemangiomas that are congenital or infantile.

1.Infantile Hemangioma

Are typically absent at birth. Most of them, about two thirds, or sixty to seventy percent, show up soon after delivery.  Usually manifests between two weeks and two months after birth.  At birth, 30% of them might have a herald patch.  Perhaps subcutaneous or cutaneous.
During the Proliferative phase, which lasts from 9 to 12 months, cutaneous infantile hemangiomas appear as red bruises or scratches. Over time, they expand quickly, becoming a brilliant red, tight, elevated, and bosselated lesion above the skin's surface. This condition was once known as strawberry angioma. It is characterized by rapidly proliferating endothelial cells mediated by VEGF (vascular endothelial growth factor) and FGF (fibroblast growth factor). Yet, it isn't in use right now. 
Subcutaneous: For the first two to four months, it is not noticeable.  During the proliferative phase, endothelial cells will proliferate quickly and manifest as deep, purple-colored masses of soft tissue. Lesions that are brilliant red, tense, bosselated, and elevated above the skin's surface are indicative of cutaneous ones. Subcutaneous ones, on the other hand, appear as deep, purple or bluish soft tissue lumps. 

During the first nine to twelve months, the hemangiomas undergo a proliferative period during which they gradually begin to retreat and involute.  Fifty percent will mature five years slower. By the time a child reaches the age of seven or twelve, seventy percent of hemangiomas will have resolved.  Pale patches that they leave behind when they involute likewise regress with time. 

Fibrous tissue will accumulate in the perivascular region during the involution phase.  Tissue metalloproteinase 1 levels are raised in relation to it. The expression of VEGF (vascular endothelial growth factor) and FGF (fibroblast growth factor) is linked to the proliferative phase, while tissue metalloproteinase 1 is enhanced during the involutory phase. 

2. Congenital Hemangiomas

Always observed at birth; less prevalent than infantile hemangiomas.  Congenital hemangiomas are always detected at birth, while around 70% of infantile hemangiomas are not detected at birth and manifest between two weeks and two months of life.  There are three possible clinical course types.
1. RICH Congenital Hemangioma with Rapid Involution
2. PICH Congenital hemangioma with partial involuting
3. NICH Congenital Hemangioma Without Involuting 

Thus, while congenital hemangiomas can be classified as either rapidly involuting, partially involuting, or non-involuting, infantile hemangiomas are invariably involute. 

Diagnosis 

Based on the patient's medical history and physical examination, hemangioma can be diagnosed clinically. If further research is required, Doppler ultrasound is the preferred imaging modality. Not every patient needs an MRI. We can only proceed with an MRI if there is ambiguity regarding the diagnosis. 
T1 images seem isointense on MRIs, however they will appear hyperintense on T2.  Lesions may be solitary, multifocal, or unifocal in patients.  Visceral hemangiomas are more common in patients with three or more lesions. Viscera in the abdomen in particular. Therefore, abdominal ultrasound screening is required for these individuals in order to check for visceral hemangiomas. Large liver lesions may result in heart failure with a high output.  Hemangiomas occasionally form a component of another syndrome

Large segmental hemangiomas in cranial nerve V distribution - should raise suspicion of PHACES association. 

• P- Posterior fossa malformations
• H- Hemangiomas
• A- Arterial anomalies
• C- Cardiac defects
• E- Eye anomalies
• S- Sternal defects

Large infantile hemangiomas of the lumbosacral region or lower extremities- should raise suspicion of LUMBAR association.

• L- Lower body hemangiomas
• U- Urogenital anomalies
• M- Myelopathy
• B- Bony deformities
• A- Anorectal/Arterial anomalies
• R- Renal anomalies

PHACES should be suspected when they are found in the V cranial nerve's distribution, and LUMBAR should be suspected when they are found in the lumbosacral region or in hemangiomas of the lower extremities.

Hemangiomas are linked to another syndrome.  Meritt-Kasabach Syndrome  The big hemangioma is present..  Platelet trapping in big hemangiomas, which results in thrombocytopenia.

Hemic anemia microangiopathic, they are superficial, isolated lesions; consumption coagulopathy.  More often observed in children; may involve internal organs such as the liver, typically in the limbs. The major triad consists of consumption coagulopathy, massive hemangioma, and thrombocytopenia brought on by platelet entrapment in the hemangioma. 

Treatment 

The observation is primarily straightforward due to the involvement of the majority of hemangiomas. Keep an eye on asymptomatic patients and wait for their involution to occur naturally.  High-risk lesions that need to be treated right away.  We must act quickly in cases of complex or ulcerated hemangiomas that are bleeding. 

Early management is necessary because periocular hemangioma has the potential to compress the visual axis and cause blindness. The region where the beard will grow is referred to as a hemangioma in beard distribution. They frequently block airways. Cord compression may result from posterior midline lumbosacral hemangiomas, which suggest underlying spinal dysraphism. 

The initial course of treatment for hemangiomas is the use of oral propranolol, a beta blocker.  The first-line treatment for symptomatic or complicated hemangiomas, including underestimated bleeding hemangiomas, is oral propranolol.  Steroids were utilized in the past, but not currently. Propranolol used orally is now a popular usage.  It hastens the proliferative phase's involution.  Provided for a full year.  Earlier, steroids were used.  For superficial lesions, systemic/intralesional triamcinolone was utilized.  Steroids are no longer recommended, nevertheless.

What function does surgery serve?

Hemangiomas spontaneously undergo involution.  Fifty percent will have no obvious sequelae. However, in 50% of cases, there remains a residual fibrofatty mass with atrophic, hypopigmented, and/or telangiectatic overlaying skin. If the patient is experiencing any discomfort from this mass, a surgical excision should be considered. 

During the proliferative period, no surgery. In the proliferative stage, propranolol will be administered orally. It will facilitate quick evolution. If the remaining lump remains after that, it can be surgically removed.

Vascular Malformations

Vascular malformations are always present at birth and may go unnoticed if they are deeply ingrained in the skin. They are clusters of aberrantly formed vessels with no discernible endothelial cell turnover.  Infantile hemangiomas are more common than congenital ones, which are present from birth. 

Grow proportionately slowly to the patient's or child's growth; • Equally present in boys and females M = F.  Hemangiomas are equally common in males and females, but they are more common in men.

Can be categorized according to the aberrant vessels' anatomical origin.
1. Malformations of the capillaries themselves.
2. Vein-related vascular abnormalities.
3. Disorders of the lymphatic system resulting from lymphatics.
4. Arterial malformations resulting from arteries: Arteriovenous malformation is a famous example.

They can be classified into one of two categories based on vascular dynamics

1. High-flow vascular malformations
2. Vascular abnormalities with poor flow 

Arterial malformations (Arteriovenous malformations) are high-flow vascular abnormalities, whereas capillary venous and lymphatic malformations are low-flow vascular malformations. 

Capillary Malformation

Formerly, capillary abnormalities were referred to as port wine stains. However, don't use this language anymore.

Aetiology 

Localized intradermal vasodilation results from the abnormal maturation of cutaneous sympathetic innervation during embryogenesis. This results in capillary abnormalities or Port wine stains.  Existent from birth 

At birth, the skin has smooth, flat pink spots that darken and become intensely to deeply purple-stained.  It is flat, but as it grows, it gets thicker and has a cobblestone appearance. It is not growing as hemangiomas do. avoid involving yourself impulsively.  Could be linked to underlying soft tissue or bone overgrowth.  Frequently shows up as a dermatomal distribution.  Occurs anyplace in the body: in the maxillary and mandibular dermatomes of the trigeminal nerve in the head and neck

A facial capillary abnormality in the trigeminal nerve's distribution.  An strongly purple-stained flat, smooth lesion representing the second capillary malformation.  A component of certain disorders may be capillary abnormalities. 

Sturge-Weber syndrome is a frequent syndrome linked to capillary malformation or port wine stain.  Vascular anomalies of the underlying leptomeninges;  Capillary malformations in the face's V1/V2 nerve distribution  Vascular abnormalities of the eyes.  Individuals with a high risk of glaucoma, stroke, or convulsions.

Diagnosis

The main methods of diagnosis are physical examination and history taking.  More thorough workups and investigations are required for patients with syndromic relationships. 

Treatment

Pulsed dye laser therapy is the cornerstone of treatment; surgical procedures are reserved for cases when there is soft tissue or bone development.  A woman in her 20s who has capillary abnormalities in her right cheek.  It regresses following therapy. Before and  after pulsed dye laser treatment. 

Salmon Patch

It is a pinkish macule that is typically observed near the nape of the neck due to the location where embryonic dermal circulation is still present.
At birth; by the age of one year, it vanishes.  Does not need medical attention. Simply watch as it will integrate by the time the child is a year old. 

Venous Malformations

It is a pinkish macule that is typically observed near the nape of the neck due to the location where embryonic dermal circulation is still present.
At birth; by the age of one year, it vanishes.  Does not need medical attention. Simply watch as it will integrate by the time the child is a year old. 

 Low-flow deformity.  Dilated vein clusters grouped together in lumps. it is devoid of muscle and valves..  Affect the mucosa, subcutaneous tissue, or skin.  50% are more deeply involved. It implies that internal organs and viscera may also be affected.  Typically present from birth
Grow proportionately to the patient, while puberty or pregnancy may cause faster growth. Typically, single

Location - Cutaneous or Visceral. 

On Examination

Superficial VMs are bluish-hued, soft, compressible masses that enlarge in response to a dependent position or Valsalva maneuver.  Phleboliths, or firm tender nodules with calcifications  Elevated extremities circumference and deeper intramuscular VM-pain  Vascular malformations have large, dilated, lobulated veins that may contain stasis. Both thrombosis and qualifications may be present. 

Management

• MRI with contrast is the preferred modality of care.  To prevent sedation in babies, Doppler USG can be employed.  When asymptomatic;  Observation; Compression of the affected extremities to lessen discomfort and size.  Also inhibits the development of phlebolith and thrombosis. Scleropathy is the first-line treatment for symptomatic patients. 

Role of Surgery-

• Why is a high recurrence rate linked to sclerosing rather than to surgery?
• Very challenging to fully dissect.

Indications of surgery 

Extremity lesions close to peripheral nerves; • Small, well-localized lesions amenable to full resection; Remaining deformity following sclerotherapy

The child's lip had a venous abnormality. Three chemotherapy sessions later, the second photograph was taken. In image 3, there is some residual deformity that has been surgically removed. 

AV (Arteriovenous) Malformation

Vascular abnormality with high flow.  Normally, arteries arteriole capillaries, followed by venules and veins; abnormal vascular connections occur between arteries and veins without the need for an intervening capillary bed. However, in this instance, there is aberrant connectivity between the veins and arteries without a capillary bed in between. 

An AV fistula of a kind is present at birth.  Present from birth; noticeable during early life or childhood.  Earlier observation due to high-flow lesions.  AVMs affecting the skin: Due to erratic blood flow from arteries, the skin appears pink and heated to the touch.  Palpation reveals a throb or bruise because of a quick shift in blood flow from the arterial to the venous circulation. 

Although they are frequently intracranial, they can also be extracranial.
Severe ischemia skin ulceration resulting from localized capillary loss. Lack of capillaries causes blood to flow from arteries into veins; a large AVM increases cardiac output, which in turn causes congestive heart failure.

The Schobinger classification
Phase 1: Idle:  Could be present from birth or not. 

Stage 2: Expansile Stage: This stage will see an increase in vein-to-artery flow along with an expansion of swelling. This is the one linked to pain and excitement.

Destructive at Stage 3:  The existence of ulcers 

Step 4: Resulting in Payment. Cardiac failure is caused by an increase in cardiac output.The preferred method of inquiry is Doppler USG. 

If surgery is planned, MR angiography should be performed to determine the extent of the lesion. 

Treatment

Asymptomatic AVM's

• Observation 
• Symptomatic- We can go for Surgery or embolization. 
• Indications for surgery 
• Small well-localized AVMs (arteriovenous malformation).
• Focal deformities resulting from AVM (arteriovenous malformation).
• Symptomatic AVMs are not amenable to embolization.
• Embolization 24-72 hrs before Surgery to decrease blood loss.
• The risk of recurrence is always there in Surgery and embolization because complete resection is not possible. 
• Sclerotherapy- Used as an adjunct to Surgery. 
• Mainstay for venous malformations. 
• Insufficient alone due to the formation of collaterals

Combined Malformations

• AVMs without symptoms 
• Observation
• Symptomatic: Surgery or embolization are options. 

Surgical indications

Small arteriovenous malformations (AVMs) that are well-localized.  Arteriovenous malformation (AVM)-related focal deformities.  Embolization is not a viable treatment for symptomatic AVMs.  To reduce blood loss, embolization should be done 24 to 72 hours before to surgery. 

Because total resection is not achievable with surgery or embolization, there is always a chance of recurrence.  Sclerotherapy: applied as a support measure for surgery.  

Hope you found this blog helpful for your NEET SS Surgery Skin and Subcutaneous preparation. For more informative and interesting posts like these, keep reading PrepLadder’s blogs.