The majority of the vascular anomalies are seen in the head and neck region. Even though the incidence of this anomaly could be construed as a rare disease entity, with only 5% of overall affliction, the lack of knowledgeable management has disfigured many. A comprehensive understanding of this benign yet complex life-changing entity is essential. A historical perspective, pathophysiology-logical evolution, and the current knowledge of management modalities are essential for rendering clinical care in this subspecialty care. We propose a very succint Head and Neck Surgical classification for vascular anamolies, which has been a good guide for surgical approaches.
Key points
- •
No single susceptible population group has been identified. So classifications and historical understanding of vascular lesions is critical, to know the self-limiting versus aggressive lesions, typical clinical appearance etc.
- •
When surgical or interventional therapy is contemplated, need-based imaging studies play a major role.
- •
Majority are benign entities and not “cancers”. Conservative management versus resection for lesion eradication should not be a piecemeal methodology.
- •
There is no one treatment modality which would fit every vascular anomaly.
- •
Biopsy of the lesions may not be necessary; malformations are more common. Biopsy only if you understand it has malignant behavior.
- •
Vascular anomalies have a ” dynamic lifecycle ”. Biological syndrome-based grouping was logistical convenience, but with mutational foundations, the syndromes are reclassified.
- •
Between vascular tumors and malformations, tumors are more common, but you may encounter ‘malformation’ more often as they persist and need varied care. Patients usually present with multiple earlier investigations and interventions.
- •
Vascular anomalies—‘group’ of different entities of varied etio-pathological which may not share same treatments approaches.
- •
Airway/vision must be a consideration in managing head and neck anomalies, especially with syndromes and segmental presentations.
- •
Great potential for molecular and genetic research in this field-so multispecialty clinics save tissue samples for research laboratories for discovering targeted therapies.
Introduction
This article may provide a comprehensive understanding of vascular-related entities and updates on classifications.
Majority of the vascular anomalies are seen in the head and neck region. Even though the incidence of this anomaly could be construed as a rare disease entity, with only 5% of overall affliction, the lack of knowledgeable management has disfigured many. A comprehensive understanding of this benign yet complex life-changing entity is essential. A historical perspective, pathophysiology-logical evolution, and the current knowledge of management modalities are essential for rendering clinical care in this subspecialty care.
History
The first scientifically documented “surgery under an ether anesthetic agent” by Morton in 1846 done in Boston, Massachusetts, USA, was for excision of a “low-flow” vascular malformation. Rudolph Virchow, in 1863, first categorized vascular anomalies by microscopic architectural patterns. From earlier use words “port wine stain,” “angiomas,” etc. Virchow attempted an acceptable cellular classification as angioma simplex, angioma cavernosum, and angioma rac-emosum. The further biological classification was proposed in 1982 by Mulliken and Glowacki. The international collaborative group—International Society for the Study of Vascular Anomalies (ISSVA)—is a way forward to advance constant review of the current and updated science in this field.
Classifications for vascular lesions are complex. Vascular tumors have increased mitotic activity and will appear soon after birth, with approximately 30% already apparent at the time of birth. , Vascular tumors are divided by the ISSVA as benign, locally aggressive or borderline, and malignant. Benign tumors include hemangiomas. Locally aggressive or borderline tumors include Kaposi Sarcoma and hemangioendothelioma. Malignant tumors include angiosarcomas and epithelioid hemangioendothelioma ( Table 1 ).
| Year | Author | Basis of classification |
| 1863 | Virchow RLK | Microscopic channel architecture |
| 1877 | Wegener | Histomorphic subclassification of Virchow’s classification |
| 1973 | Degni and coworkers | Site of origin of the detect |
| 1974 | Malan | Embryologic site of origin of the defect |
| 1982 | Mulliken JB and Glowacki J | Endothelial characters |
| 1983 | Burrows and colleagues | Angiographic flow patterns |
| 1988 | International Society for the Study of Vascular Anomalies (ISSVA), Hamburg | Anatomopathologic classification of vascular defects (Hamburg classification) |
| 1989 | Belov | Etiologic and pathophusiologic classification system |
| 1992 | ISSVA, Colorado | Cellular features, vascular flow, characteristics and clonincal behavior |
| 1993 | Jackson and associates | Flow rate |
| 1996 | ISSVA, Rome | Modified ISSVA classification |
| 2011 | S C Nair | Anatomical presentation |
| 2014 | ISSVA, Melbourne | Modified ISSVA classification |
Incidence
The overall incidence of vascular lesions is around 5%. Among tumors, infantile hemangiomas have the same percentage of incidence. Among vascular lesions, venous malformations are commonly like lymphatic malformations with an incidence of 1:5000 to 1:10000.
More than 50% of them are present in the head and neck anatomic area.
Terminology and classifications
Terminology of vascular lesions evolved from descriptive to the more scientific basis for nomenclature, the former possibly arising from maternal explanations of the lesions. It posted 1982 after Mulliken and Glowacki and colleagues attributed biologic differences within the lesion that they were described as separate entities. A neoplastic hemangioma was found to be distinctly different from the developmental vascular malformation.
Historical Terminology on Appearances
Descriptive terms and historical nosology of vascular anomalies offer an array of overlapping descriptive and histopathologic terms.
Port wine can be used for “capillary hemangiomas” historically. It can be as small and superficial.
Histopathologic terms used historically
- •
Capillary
- •
Cavernous
- •
Arterial
- •
Mixed
Strawberry lesion or a “capillary-cavernous hemangioma” was commonly used for lesions like present on the tongue. Such terminology added to the confusion as shown in a tongue vascular lesion, which is a low-flow vascular lesion, with sequela of trauma.
Mulliken and Glowacki’s biological classification (1982)
The ISSVA classification has been modified and based on the biologic classification by Mulliken and Glowacki and further with the mutational knowledge ( Box 1 and Fig. 1 ).
-
International society for the study of vascular Anomalies classification system (ISSVA, last revision 2022, please review at ISSVA.org)
-
Vascular tumors
-
Benign
-
Locally aggressive or borderline
-
Malignant
-
-
Vascular malformations
-
Simple
-
Capillary malformations
-
Lymphatic malformations
-
Venous malformations
-
Arteriovenous malformations
-
Arteriovenous fistula
-
-
-
Combined (2 or more VMs in one lesion)
-
CVM, CLM, LVM, CLVM, CAVM, CLAVM, others
-
Hemangiomas
Proliferating phase
- •
Involuting phase.
Malformations
- •
Capillary
- •
Venous
- •
Arterial
- •
Lymphatic
- •
Fistulas
- •
Hemangioma
Further advances in the understanding of the vascular dynamics allowed changes in the classification, and this was first proposed by Ian Jackson and colleagues, allowing major breakthroughs in treatment planning and management of vascular lesions.
Central nervous system and non-central nervous system list of vascular lesions
Central nervous system vascular malformations are rare abnormalities of blood vessels in your brain or spinal cord and their membranes. They distinctly differ in their appearance and management ( Boxes 2–5 and Table 2 ).
-
Haemangiomas (proliferative lesions or tumors)
-
Vascular malformations (non-proliferative)
-
High flow
- •
Arteriovenous malformations
- •
-
Low flow:
- •
Capillary malformations
- •
Venous malformations
- •
Lymphatic malformations
- •
Mixed malformations
- •
-
-
Arteriovenous shunts
-
Classical AV Malformations
-
Pial AV fistulas
-
Dural AV shunts
-
Galenic shunts
-
-
Cavernous malformations
-
Capillary telangiectasias
-
Venous malformations
-
Developmental venous
-
Anomalies
-
-
Mixed malformations
-
IAN JACKSON IN 1992 further expanded on the earlier classification. Based on this, Hemangiomas were described on their location and VM on the dynamixs of blood flow.
-
Hemangiomas
-
Superficial (capillary hemangioma)
-
Deep (cavernous hemangioma)
-
Compound (capillary-cavernous hemangioma)
-
-
Vascular malformations
-
Simple lesions
-
Low flow lesions
-
Capillary malformations (capillary hemangioma, port wine satin)
-
Venous malformation (cavernous hemangioma)
-
Lymphatic malformation (lymphangioma, cystic hygroma)
-
-
High-flow lesions
-
Arterial malformation
-
-
Combine lesions
-
Arteriovenous malformations
-
Lymph venous malformations
-
Other combinations
-
-
| Stage I | Quiescence | Cutaneous blush, skin warmth, arteriovenous shunt on doppler |
| Ultrasound | ||
| Stage II | Expansion | Darkening blush, lesion shows pulsation, thrill and |
| bruit | ||
| Stage III | Destruction | Steal, distal ischemia, pain, dystrophic skin changes, ulceration, necrosis, soft tissue and bony changes |
| Stage IV | Decompensation | High-output cardiac failure |
Pathogenesis
Blood vessels form consolidated aberrations in limited anatomic sites of the human body. They result in sporadic anomalies or hereditary occurrences. Hemangiomas are vascular neoplasms that have different phases of development. They go through the proliferative phase, quiescent phase, and finally involutory phase. Histologically they are characterized by the proliferation of plump endothelial cells with mast cell infiltration. , Infantile hemangiomas show increased expression of glucose transporter-1. The cellular activity during involution shows the reduction in angiogenesis with apoptosis of endothelial cells. Congenital hemangiomas in contrast are fully mature at birth and do not show a proliferative phase. Pathogenesis is a complex interaction of genetic and environmental factors. The role of human papillomavirus-8 infection, chronic villus sampling, and hormonal, especially estrogen disturbances with possible hypoxia causing endothelial cell proliferation is another theory. The role and efficacy of beta 2 blockers in the management of proliferating hemangiomas are documented. Vascular malformations are the result of errors in angiogenesis. The growth and differentiation of vascular lesion and endothelium are driven by 2 major pathways: RAS/RAF and PIK3CA (Refer to Fig. 2 ).
