This area of surgery has been clouded by a variety of confusing and often nonsensical classification systems. Differentiation is important as the potential for overgrowth exists, and this may cause serious deformity in these unfortunate patients. Terms such as cavernous or capillary haemangoma, port-wine stains as well as any references to a fruit, such as a cherry or a strawberry should obviously be discouraged!
Overview and Nomenclature
The current standard classification was described by Mulliken & Glowacki in PRS, 1982. It is called the BIOLOGICAL CLASSIFICATION, and takes into account the physical findings, natural history and cellular features. It divides anomalies of the lympho-vascular system into two broad groups: tumours and malformations.
- VASCULAR TUMOURS (Endothelial neoplasms)
- Haemangioendothelioma (KHE – Kasabach-Merritt Phenomenon)
- Tufted Angioma
- Pyogenic Granuloma (Acquired inflammatory vascular “tumour”)
- VASCULAR MALFORMATIONS (Abnormalities of embryogenesis / maturation)
1. Single Vessel Forms (Or based on dominant type)
- Capillary Malformations (formerly called Port-wine stains)
- Sturge-Weber Syndrome
- Cutis Marmorata Telangiectasia Congenita (CMTC)
- Lymphatic Malformations
- Microcystic (formerly called Lymphangioma)
- Macrocystic (formerly called Cystic Hygroma)
- Venous Malformations
2. Arteriovenous Malformations (AVM)
3. Complex Forms
- Low flow Capillary-Lymphatico-Venous Malformation (CLVM) also known as “Klippel-Trénaunay Syndrome”
- High flow Capillary Arteriovenous Malformation (CAVM) also known as “Parkes-Weber Syndrome”
- VASCULAR TUMOURS
Vascular tumours are true neoplasms characterized by increased cellular growth rates. The most important tumour is the haemangioma.
Definition :This is a true vascular tumour with a very unique natural history: rapid growth, slow regression and no recurrence. This is reflected in the 3 stages of a haemangioma: the proliferating phase, the involuting phase and the involuted phase.
Incidence: These are the most common tumours of infancy, affecting up to 1:10 babies and are much more common in caucasians, females and in premature infants. 80% are solitary and 20% are multiple.
Features: Haemangiomas manifest (usually) within 2 weeks of birth. A herald spot may be present, which is usually a pink or red patch.
In the first year of life, the tumour undergoes rapid growth but rate does not predict ultimate size. They could be raised, firm and a deep crimson to blue in colour.
Between 1 and 5 years the growth slows down and then begins to regress. Signs of regression include a colour change from red to purple, central paleness and greyish discolouration. The rate of regression has no correlation to depth, size, site, gender or colour. Involution is complete by 5 years in 50% and 70% by 7 years.
Normal appearance is restored in 50% of cases. Residual skin laxity, yellow patches and alopecia (baldness) may occur.
Diagnosis: Clinical diagnosis is often sufficient. Ultrasound and MRI are useful for visceral and intracranial disease exclusion, and Tc-99 red cell scans can also be used. Biopsy is a last resort as profuse bleeding may occur.
Treatment: Most haemangiomas are small, harmless tumours which can be expected to regress without further treatment. Parental reassurance is required and serial photography is useful for follow up. There are several instances where more severe cases require intervention before regression.
Steroid Injections repeated 4 to 8 week intervals for 3-6 sessions is my preferred treatment option. Oral steroids are used for severe cases.
Interferon-Alpha is used as a second line therapy in cases of steroid resistance as the complications are more significant.
Chemotherapy: Vincristine and cyclophosphamide have been used for life-threatening haemangiomas. I have occasionally used low dose bleomycin (1-2 Units per session) for intra-lesional injection in resistant cases – caution should be exercised and skin necrosis may be encountered.
Radiation: Low dose radiotherapy was given in the past, but is no longer recommended.
Embolization: This is mainly used for visceral (hepatic) haemangiomas that are responsible for severe CCF. Improvement is transient (a bridge while awaiting an effect of another modality).
Laser: The usefulness of flash-lamp dye lasers is limited to cosmetic improvement of the superficial portion of a cutaneous lesion.
Surgical Excision: This should follow the principles of maximal tissue preservation and concealed scar placement. Revision of the scars is done at a later stage. Refinement of the involuted phase lesions requires cosmetic surgery.