Vascular Anomalies

Arin K. Greene

Jeremy Goss

ANATOMY

Vascular anomalies most commonly involve the integument (although they can affect any anatomical structure).
The most common morbidity is lowered self-esteem because of a deformity.
Lesions involving the head and neck are more likely to be problematic.

NATURAL HISTORY

The somatic mutations responsible for most vascular anomalies recently have been identified.
Some vascular tumors improve over time (infantile hemangioma, rapidly involuting congenital hemangioma, kaposiform hemangioendothelioma).²
Vascular malformations slowly worsen, particularly during adolescence.
Following treatment of vascular malformations, recurrence is common and patients often require repeated procedures.

PATIENT HISTORY AND PHYSICAL FINDINGS

Ninety percent of vascular anomalies can be diagnosed by history and physical examination.
Before considering management, the type of vascular anomaly must be identified because lesions have different natural histories and treatments.³
The most common vascular anomalies encountered in clinical practice are (in order of frequency) pyogenic granuloma, infantile hemangioma, and capillary malformation.
A handheld Doppler can determine whether the lesion has fast flow, which aids the diagnosis.
Lesions with fast flow include infantile hemangioma, congenital hemangioma, kaposiform hemangioendothelioma, and arteriovenous malformation. Slow-flow anomalies are capillary malformation, lymphatic malformation, and venous malformation.

IMAGING

Less than 10% of patients require imaging to diagnose their vascular anomaly.
If the diagnosis is unclear by history and physical examination, ultrasound is the first-line imaging study because it is easy to perform and does not require sedation in children.
If the diagnosis remains uncertain after ultrasonography, then MRI with contrast is obtained.
Imaging is not required prior to resection of hemangiomas, pyogenic granulomas, or capillary malformations.
Before operative intervention for a venous malformation, lymphatic malformation, or arteriovenous malformation, MRI typically is obtained to determine the extent of disease.

NONOPERATIVE MANAGEMENT

Nonoperative intervention is the mainstay of treatment for vascular anomalies; lesions are benign and often can be observed.
Less than 10% of infantile hemangiomas require treatment. Problematic lesions during the proliferative phase can be managed with topical timolol, corticosteroid injection, prednisone, propranolol, or rarely, resection.
Kaposiform hemangioendothelioma is treated with vincristine or sirolimus.
First-line intervention for problematic vascular malformations typically is nonsurgical: capillary malformation (pulseddye laser), venous malformation (sclerotherapy), lymphatic malformation (sclerotherapy), arteriovenous malformation (embolization).⁴^,⁵^,⁶^,⁷

SURGICAL MANAGEMENT

Operative treatment of vascular anomalies generally is performed for symptomatic patients who are not candidates or have failed nonoperative interventions.
Residual infantile or congenital hemangiomas causing a deformity can only be improved with resection (or pulseddye laser for telangiectasias).
The primary treatment of pyogenic granuloma is full-thickness skin excision or cautery (curettage, laser, and cryotherapy have a recurrence rate as high as 50%).
Capillary malformation can cause overgrowth of tissues beneath the stain, which can be improved by resection.
Operative intervention for venous malformation is reserved for small lesions that may be removed for cure or for a residual deformity following sclerotherapy.
Macrocystic lymphatic malformation may have redundant skin following sclerotherapy that can be resected. Microcystic lesions are not amenable to sclerotherapy, and thus, excision often is first-line treatment.
Arteriovenous malformations generally are removed if they remain symptomatic following embolization.

Preoperative Planning

A critical principle is that vascular anomalies are benign and thus do not require radical resection; the scar and reconstruction should not cause a worse deformity than the appearance of the lesion.
Vascular anomalies typically are diffuse and involve multiple tissue planes; consequently, most resections are subtotal.
The goal of operative management is to improve the patient’s symptoms by removing enough of the lesion to reduce pain, bleeding, and/or ulceration and/or improve appearance.
Symptomatic infantile hemangiomas are best excised between 3 and 4 years of age after they have completed involution.
If possible, resection should be postponed until the child is at least 6 months of age to reduce the anesthetic risk.
Consideration should be given to improving deformities before 4 years of age to minimize impact on self-esteem.
Venous, lymphatic, and arteriovenous malformations usually are removed after they have been treated with sclerotherapy or embolization; these interventions reduce the size of the lesion, decrease bleeding, create fibrosis, and facilitate the operation.

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