Any deviation from normal vasculature in human body during developmental process.

Classification: ISSVA (International Society of Study of Vascular Anomalies)
Classification

1. Vascular Tumors :
    a. Hemangioma
      i. Infantile hemangioma
      ii. Congenital hemangioma:
         (a) RICH (rapidly involuting congenital hemangioma)
         (b) NICH (non-involuting congenital hemangioma)
         Phase : 3 a. Proliferating phase. b. Involuting phase. c.
                       Involuted phase
         Looking for any resting phase ?

    b. Hemangioendotheliomas
    c. Kaposiform hemangioendothelioma
    d. Angiosarcoma

Associated anomalies may be seen with hemangiomas:

PHACES includes

• P- Dandy-Waker and other cystic malformations in the Posterior
• cranial fossa.
• H- large often plaque like facial Hemangioma
• C- Cardiac defects
• E- Eye anomalies
• S – Sternal cleft

LUMBAR anomalies (affect lower half of the body) 63

Lipomeningocele

• Lower body infantile hemangioma
• Tethered cord
• Urogenital anomalies
• Myelopathy
• Arterial anomalies
• Bony deformities

With perineal and Lower limb hemangioma

• Urogenital anomaly
• Anorectal Anomaly

Management of Hemangioma:

• Observation (Expected Treatment)
• Local treatment for ulceration and bleeding
• Pharmacological therapy:
• Corticosteroids: Prednisolone 5 mg/kg in a single morning dose for 6 weeks – if reduced, continue                                                  for further 6 weeks than tapered slowly over several months and discontinued by the 12 months of age. Can add, or
•  Propanolol: 2 mg/kg/day in three divided doses till hemangioma subsides.

For non responsive cases-
Chemotherapy:

• Vincristine Vincristine
• Cyclophosphamide (rarely used)

Surgical Management:
(a) Early resection prevents complications and disfigurement.
(b) Aim of resection of an involuted hemangioma- Reconstruction of (i) damaged skin, (ii) abnormal contour, (iii) distorted or deformed anatomical structure.
(c) Indication of operation
1. Distorted or deformed anatomical structure
2. Rapidly growing large lesions obstructing vital structure/function
3. Reconstruction of disfigured skin after resolution.
4. Freshly appearing lesion in a vital area
5. Frequently bleeding lesion.

LASER therapy- (usually after involution): pulse dye laser

Vascular malformations :

Slow-flow vascular malformations:

• Capillary malformations
• Port-wine stain
• Sturge-Weber syndrome
• Hyperkeratotic vascular stains
• Cutis marmorata talengiectatica congenita
• Macrocephaly-cutis marmorata
• Capillary malformation-arteriovenous malformations
• Telangiactasias
• Hereditary hemorrhagic telangiactasia (HHT), etc.

Management of capillary malformations:

• Laser: Pulse dye LASER
• Surgery

Lymphatic malformations

• Macrocystic (old term: “Cystic Hygroma”)
• Microcystic (old term: “Lymphangioma”)
• Combined

Management of Lymphatic malformations:

• Ethanolamine Oleate
• Sodium Tetradecyl
• Sulfate
• Bleomycin
• OK-4321
• Absolute Ethanol
• Doxycycline
• Acetic Acid

Venous malformations

• Sporadic
• Cerebral-cavernous
• Glomuvenous (GVM)
• Cutaneous-mucosal (CMVM)
• Blue rubber bleb nevus syndrome

Associated Malformations

• Maffucci Syndrome
• Klippel-Trenaunay syndrome
• Bean Syndrome (Blue rubber bleb nevus syndrome)
• Turner Syndrome

Management of Venous malformations:

• 5% Ethanolamine Oleate
• 1% and 3% sodium tetradecyle sulfate
• Absolute Ethanol (100% Ethanol)
• Polidocanol

Other options of management:

• Combined sclerotherapy and resection – for excision of residual fibrosis, phleboliths, and deformed skin.
• Laser therapy for tiny cutaneous VMs (controversial)

Fast-flow vascular malformations:
    Arterial

• Aneurysm
• Coarctation
• Ectasia
• Stenosis

    Arteriovenous Fistula (AVF)
    Arteriovenous Malformations (AVM)

The natural history of AVMs is documented by clinical staging system of Schobinger:

• Stage –I (Quiescence) : Pink-blush stain, warmth, and AV shunting by continuous Doppler or 20-MHz color Doppler.
• Stage – II ( Expansion): Same as stage I, plus enlargement, pulsations, thrill, bruit, and tortuous/tense veins.
• Stage – III (Destruction): Same as above plus dystrophic changes, ulceration, bleeding, persistent pain, and expansion/destruction.
• Stage – IV (Decompensation): Same as stage – III, plus cardiac failure.