Abstract
Vascular anomalies are amongst the most common congenital abnormalities observed in infants and children. Their occurrence in the head and neck region is a source of functional and aesthetic compromise. This article reviews the surgical management of 115 cases of vascular anomalies involving the head and neck area treated by the authors between 1998 and 2009. It discusses the diagnostic aids, treatment protocol and the results obtained. A new classification based on the anatomical location and depth of the lesion has been proposed. This allows guidelines for surgical ablation of the vascular lesions. The complications encountered are discussed. The use of external carotid artery control as opposed to pre-surgical embolization has proved effective and the technique is described. The location and extent of a vascular malformation should dictate the preoperative investigations, surgical procedure and subsequent outcome.
Vascular anomalies are a group of lesions derived from blood vessels and lymphatics with widely varying histology and clinical behaviour. They constitute the most common congenital abnormalities in infants and children. James Wardrop, a London surgeon, first recognized the differences between true hemangiomas and the less common vascular malformations in 1818 . Despite Dr. Wardrop’s work, descriptive identifiers such as Strawberry hemangioma and salmon patch continued to be used until the 1980s. This terminology did not correlate with the biological behaviour or histology of these lesions. In 1982, Mulliken and Glowacki greatly advanced the field by introducing a biological classification which differentiated vascular lesions into two distinct entities: hemangiomas and vascular malformations . The term hemangioma now describes a lesion that is neoplastic, demonstrating endothelial hyperplasia. Vascular malformations, conversely, do not demonstrate cellular hyperplasia but display progressive ectasia of abnormal vessels lined by flat endothelial on a thin basal lamina. A more practical classification integrating their biological behaviour with dynamics of flow was later advanced ( Table 1 ) .
| A. Hemangiomas |
| Superficial (capillary hemangioma) |
| Deep (cavernous hemangioma) |
| Compound (capillary cavernous hemangioma) |
| B. Vascular malformations |
| Simple lesions |
| Low-flow lesions |
| Capillary malformations (capillary hemangioma, port-wine stain) |
| Venous malformation (cavernous hemangioma) |
| Lymphatic malformation (lymphangioma, cystic hygroma) |
| High-flow lesions |
| Arterial malformation |
| Combined lesions |
| Arteriovenous malformations |
| Lymphovenous malformations |
| Other combinations |
The diagnosis of this group of lesions primarily depends on the history of the lesion and the clinical presentation. Radiographic evaluation may be helpful in determining the exact extent, location and flow dynamics of some lesions.
Patients and methods
One hundred and fifteen patients treated by the authors between 1999 and 2009 were reviewed retrospectively. Relevant data including gender, age, age at presentation of symptoms, anatomical site of lesion, relevant radiographic investigations and period of follow up were tabulated. Exclusion criteria included segmental lesions and those associated with syndromes such as Sturge-Weber.
All patients underwent surgery as the principal modality of treatment. Computed tomography (CT) with contrast, magnetic resonance imaging (MRI) and angiography were used based on the anatomical location and flow dynamics of the lesion. Selective control of the external carotid artery to reduce blood flow into the lesion was used effectively by the author in lieu of routine preoperative embolization.
Technique for external carotid control
The external carotid artery (ECA) of the involved side is exposed through a cervical incision, which often forms part of the access for removal of the malformation ( Fig. 1 ). The sternocleidomastoid muscle is retracted posteriorly at the level of the greater cornu of the hyoid bone, exposing the carotid sheath. The external carotid distal to the carotid bifurcation is identified. The vessel is snared with a vascular sling passed through a red rubber catheter. Gentle strangulation of the vessel can be accomplished by advancing the catheter. This additional compression of the vessel serves to reduce blood flow to the lesion. The lesion is exposed with great care taken not to disturb the vascular network. Feeding arteries and draining vessels are identified and ligated, permitting total excision of the lesion. The wound is closed primarily with vacuum drains in situ . The malformations were categorized into five types depending on their anatomy and depth of location in the head and neck region ( Table 2 ). In type I superficial lesions requiring excision of skin or mucosa, local or regional flaps have been used in defect reconstruction ( Fig. 2 ). Type II submucosal lesions require complete excision after elevation of skin flaps ( Fig. 3 ). Type III lymphovenous malformations or venous malformations involving salivary glands are excised along with the affected gland ( Fig. 4 ). Type IV intraosseous lesions require excision with involved bone and reconstruction when required ( Fig. 5 ). Type V lesions involving deep visceral spaces, such as the parapharyngeal or infra-temporal fossa, require mandibular access osteotomy for complete exposure and total excision ( Fig. 6 ). The above classification helped in determining the surgical approach and reconstruction necessary for the type of vascular lesion.
| Type I – Mucosal/cutaneous ( Fig. 2 ) |
| Type II – Submucosal/subcutaneous ( Fig. 3 ) |
| Type III – Glandular ( Fig. 4 ) |
| Type IV – Intraosseous ( Fig. 5 ) |
| Type V – Deep visceral ( Fig. 6 ) |
Results
Of the 115 patients evaluated, 63 were male and 52 female. The youngest patient was a 2-year-old girl with a lymphatic malformation in the parotid region (type III) and the oldest was a 58-year-old male with a venous malformation involving the entire tongue and submandibular region (type II). Table 3 shows the patients categorized into types with gender distribution. 38 patients with type I, 44 patients with type II, 12 patients with type III, 11 patients with type IV and 10 patients with type V anomalies were treated successfully by surgical ablation of their vascular lesions. Four patients with type I lesions required reconstruction with local or regional flaps and 2 patients with type IV lesions required reconstruction of resected mandible. Only 88 patients could provide an approximate time of appearance of the lesion. In 27 patients the lesion had been noticed at birth or soon after. The remaining 61 patients were clinically aware of it shortly before their first surgical visit. Table 4 highlights the different imaging techniques used according to the type of malformation. At the authors’ centre CT scanning with contrast is the most frequently used imaging modality. Table 5 demonstrates the method of haemorrhage control used for the malformation. Pre-surgical embolization was restricted to two patients and external carotid artery control was required in 52 patients. Complications encountered are listed in Table 6 . One hundred and eleven patients gained an acceptable aesthetic outcome with a single procedure. Table 7 summarizes the surgical plan employed for each type of lesion and the reconstruction used when required.
| Type I n = 38 |
Type II n = 44 |
Type III n = 12 |
Type IV n = 11 |
Type V n = 10 |
|
|---|---|---|---|---|---|
| CTC | 32 | 34 | 7 | 2 | 6 |
| MRI | 0 | 1 | 1 | 0 | 0 |
| Angiogram | 0 | 5 | 4 | 8 | 4 |
| No investigation | 6 | 4 | 0 | 1 | 0 |
| Type I n = 38 |
Type II n = 44 |
Type III n = 12 |
Type IV n = 11 |
Type V n = 10 |
|
|---|---|---|---|---|---|
| ECA control | 1 | 32 | 11 | 11 | 10 |
| Non-ECA control | 37 | 12 | 1 | Nil | Nil |
| Embolization | Nil | Nil | Nil | 1 a | 1 a |
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