Intervention for a VM is not mandatory. Treatment is reserved for lesions that lower self-esteem, cause pain, bleed, or obstruct/destroy tissues. Resection and reconstruction should not leave a deformity worse than the VM. The primary variable that determines whether a lesion should be resected is the type of VM; some can only be managed with excision, whereas others should be treated with sclerotherapy. Small, localized VMs are more amenable to resection, because they can potentially be removed for cure (see Video 9-1, Venous Malformation).

VENOUS MALFORMATION

Approximately 95% of VMs are nonfamilial, solitary, and result from a somatic mutation. Lesions can be small and localized or large and diffuse. Patients who have psychosocial morbidity, pain, or reduced function are candidates for treatment. Generally individuals are managed with sclerotherapy, because it has better efficacy and is safer than excision. Surgical treatment of VMs is reserved for:

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  Small lesions that can be completely removed for cure

  
  
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  Malformations that remain symptomatic but can no longer be treated with sclerotherapy because all of the channels have been obliterated

I prefer to resect large VMs only after they have been treated with sclerotherapy because:

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  The lesion becomes smaller.

  
  
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  Veins are converted to fibrotic tissue, which facilitates excision.

  
  
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  The sclerosant permeates small vessels at the periphery of the lesion and theoretically reduces the recurrence rate.

VENOUS MALFORMATION SUBTYPES

Blue Rubber Bleb Nevus Syndrome

Patients with this condition can have multifocal lesions involving the gastrointestinal tract. The major indication for intervention is chronic gastrointestinal bleeding requiring transfusions. Although VMs can be treated with a sclerosant, resection of individual lesions is the preferred treatment and gives favorable outcomes.

Cerebral Cavernous Malformation

Patients have small, localized cerebral VMs and may have associated hyperkeratotic skin lesions. Cerebral VMs are at risk of bleeding and seizures and may require resection if symptomatic. Bleeding skin lesions can be treated with a carbon dioxide laser. However, resection is preferred, because it gives more definitive improvement and a better aesthetic outcome.

Cutaneomucosal Venous Malformation

VMs associated with this multifocal, familial condition are typically small and well localized. Consequently, resection is usually the preferred first-line treatment instead of sclerotherapy.

This eponym is used for a diffuse VM of an extremity affecting all tissues (skin, subcutis, muscle, and bone). Patients have significant morbidity, including reduced function of the extremity. First-line treatment is targeted sclerotherapy to improve localized problematic areas. Resection is reserved for patients with significant symptoms who have failed or cannot be treated with sclerotherapy.

Fibroadipose Vascular Anomaly

This lesion shares features with an intramuscular VM but is associated with more pain, contractures, and fat. A fibroadipose vascular anomaly does not respond to sclerotherapy, because it is solid. However, adjacent phlebectatic veins may be injected. Initial management can include sclerotherapy of peripheral phlebectasia, corticosteroid injection, and/or cryotherapy. However, most individuals ultimately undergo resection of the lesion, which is definitive therapy.

Glomuvenous Malformation

This type of VM is typically multifocal, small, and isolated to the skin and subcutaneous tissue. Because lesions are usually localized and do not respond well to sclerotherapy, first-line treatment is resection. Sclerotherapy is reserved for large, diffuse lesions that could be difficult to remove surgically.

Phlebectasia

The abnormal dilation of a vein can cause a deformity and lowered self-esteem. Less commonly the vein may be susceptible to clotting and pain. Phlebectatic veins are rarely resected. Instead symptomatic areas are treated with sclerotherapy or an endovascular laser.

Sinus Pericranii

Sinus pericranii is a venous anomaly that extends from superficial tissues, through the cranium, to the dura. Veins are obliterated surgically or endovascularly to prevent intracranial thrombosis. Resection of an overlying soft tissue VM is performed after the transcranial veins have been closed.

Verrucous Venous Malformation

This type of VM is not amenable to sclerotherapy. A carbon dioxide laser can reduce cutaneous bleeding. The definitive treatment is resection.

SURGICAL MANAGEMENT

TIMING OF INTERVENTION

Milestones for the resection of VMs are: (1) 6 months, (2) 3 years, and (3) late childhood/early adolescence. If possible, a VM should not be removed before 6 months of age. Before this time the patient’s risk from anesthesia is greater than in an adult. In addition, an infant is less able to tolerate a surgical procedure. If a large lesion is located on the scalp, the surgeon should consider removing it before 6 months of age to take advantage of scalp laxity in infancy.

A VM causing significant symptoms requires intervention, regardless of the age of the patient. Generally, if the lesion is amenable to sclerotherapy, this intervention should be done first. Resection is performed for:

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  Symptomatic patients who have failed sclerotherapy

  
  
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  Small/localized lesions

  
  
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  VMs that are not amenable to sclerotherapy (for example, verrucous venous malformations)

VENOUS MALFORMATION

Localized lesions that are small can be removed without preoperative sclerotherapy. Large, diffuse VMs should be treated with sclerotherapy before resection. Sclerotherapy facilitates resection by decreasing the size of the lesion, making the VM fibrous, and reducing the recurrence rate. Sclerotherapy is continued until all veins have been obliterated, which may require several treatments at 6-week intervals. When venous targets are no longer present on ultrasound imaging, excision is performed at least 3 months after the last treatment to allow adequate fibrosis to occur. Removal of a scarred VM after sclerotherapy facilitates the procedure and reduces bleeding. An exception to the use of preoperative sclerotherapy is if a diffuse VM is located adjacent to important nerves (for example, a facial nerve or digital nerves). If a resection is planned for an area in which nerve dissection is necessary, preoperative sclerotherapy should be avoided, because scarring from the procedure will complicate the separation of neurovascular structures from the VM.

Excision of a VM can be associated with significant bleeding. Blood loss is reduced by:

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  Giving preoperative sclerotherapy

  
  
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  Infiltrating the operative area with epinephrine

  
  
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  Using epinephrine-soaked sponges during the procedure

  
  
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  Using a tourniquet for extremity lesions

  
  
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  Applying iced saline solution intraoperatively to promote vasoconstriction

  
  
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  Keeping the surgical area elevated and the patient’s blood pressure low

  
  
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  Incorporating clips to prevent skin edge bleeding (particularly scalp lesions)

If the volume of local anesthetic with epinephrine is limited by the amount of anesthetic that can be given based on the weight of the child, I will infiltrate an epinephrine-only solution to ensure maximum vasoconstriction of the surgical site (1 ml of 1:1000 epinephrine in 200 ml of normal saline = 1:200,000 solution). The extirpation proceeds carefully by incising a localized area and controlling bleeding before continuing. Long, deep incisions should not be performed because of the risk of significant blood loss. None of the patients with a VM on whom I have operated has required a blood transfusion.

Diffuse VMs are unable to be entirely removed without causing significant morbidity. Patients are managed with: (1) sclerotherapy for palliation or (2) subtotal excisions of symptomatic areas. Surgical intervention for a diffuse facial VM should be focused on improving the patient’s appearance or pain with localized, staged procedures without causing a significant deformity. Diseased tissue should not be removed if there is a risk of iatrogenic morbidity. It is critical to avoid facial nerve injury. Facial nerve dissections or parotidectomy should not be performed. When the lesion is throughout all structures of the face, strategies to improve the area include: (1) excision of excess skin and subcutaneous tissue, (2) removal of the buccal fat pad, and/or (3) contour burring of the zygoma. If overgrowth is located on the lateral cheek, a preauricular incision is used. Medial fullness is resected through a mesolabial incision. Alternatively, a circular excision can be used to access the overgrown tissue, and the purse-string closure can be revised at a second stage. Lip overgrowth is improved with a transverse mucosal resection along the keratinized and nonkera-tinized border of the vermilion. Vertical resections of the lip should be avoided.

Subtotal resections of diffuse VMs are closed with adjacent tissues. I have never required a skin graft or free tissue transfer to close a defect after extirpation of a VM. Large, cutaneous VMs can be removed with serial excision. If the skin has minor telangiectasias/dilated venules causing bluish discoloration and located in an unfavorable location (for example, the nasal tip or philtrum) where skin resection would cause a significant deformity, the area can be treated with sclerotherapy or a laser. I inject 1% sodium tetradecyl sulfate (1 ml of 3% solution mixed with 2 ml of saline) with a 30-gauge needle and 1 ml syringe. Repeat injections can be performed at 6-week intervals.

Skin grafts should be avoided, because they can cause a worse deformity than the appearance of the malformation and are more likely to fail if placed on an ischemic recipient site containing a VM. Tissue expansion should also be used with caution if adjacent to a VM, because the risk of infection, extrusion, stimulation of the lesion, and skin necrosis is much higher compared with reconstruction of an area that does not contain a vascular anomaly.

VENOUS MALFORMATION SUBTYPES

Cutaneomucosal Venous Malformation

Because these lesions are small and localized, they do not require sclerotherapy and can be excised and closed linearly.

Fibroadipose Vascular Anomaly

Lesions that have failed sclerotherapy, corticosteroid injection, and/or cryotherapy require resection. The muscle containing the fibroadipose vascular anomaly is usually nonfunctional, causes significant pain, and results in contractures. Consequently, subtotal or complete extirpation of the muscle is typically required. Because this type of VM can cause significant pain, as much of the lesion as possible should be removed while maintaining important structures.

First-line intervention for this type of VM is typically resection, because the lesions are localized and do not respond well to sclerotherapy. After excision wounds are closed linearly.

Verrucous Venous Malformation

A verrucous venous malformation most commonly affects the lower extremity and is not amenable to sclerotherapy, because it is primarily composed of fibroadipose tissue. Large lesions can be removed with serial excisions at 6-week intervals. Patients are often placed into a knee immobilizer to reduce the risk of wound dehiscence.

PATIENT EXAMPLES

LOCALIZED VENOUS MALFORMATION: COMPLETE RESECTION

Patient A