Treatment of Congenital Vascular Malformations in Children and Adults

Treatment Guidelines

The treatment strategy should first focus on the primary malformation, followed by treatment of secondary disorders associated with the vascular, musculoskeletal, and integumentary systems (e.g., leg length discrepancy). This is especially true for CVMs with significant hemodynamic consequences.

Correction of various hemodynamic derangements caused by the primary lesion should be given priority. This can involve either reconstructive surgery (e.g., venous bypass, venous aneurysmorrhaphy, free lymph node transplantation) or ablative or excisional surgery (e.g., removal of the marginal vein, removal of aneurysms, excision of the CVM). Corrective surgery for the sequelae of secondary hemodynamic consequences may follow.

The optimal combination of the various treatment modalities should be selected based on precise characterization of the embryologic and hemodynamic nature of the CVM. The potential for lesion recurrence following treatment remains a significant problem for extratruncular lesions, which is a hallmark of these embryologically immature lesions. The potential for recurrence should never be underestimated when formulating the treatment plan. Thus a diffusely infiltrating, extratruncular lesion requires special attention to its high tendency to progress, associated high morbidity and complications, and high likelihood of recurrence, as a result of the primitive nature of its mesenchymal cell origin. When treatment is indicated, destruction of the lesion’s nidus is required in order to prevent recurrence. However, a controlled aggressive approach must be coupled with a realistic assessment of the long-term results of the treatment regimen.

The traditional conservative approach is still recommended for the vast majority of CVMs. In contrast to an AVM lesion occurring in the pediatric age group, a typical venous malformation without bone involvement can usually be monitored until the age of 2 years or later, when the child is mature enough to tolerate the various procedures required for diagnosis and treatment. On the other hand, earlier intervention is required when the venous malformation produces the vascular bone syndrome, resulting in discrepancy of long bone growth, or when the lesion is located at an anatomic area threatening life or limb or vital functions (e.g., breathing, seeing, eating, hearing).

Surgical Treatment

Surgical resection has long been the only means of eradicating the CVM nidus and for decades has remained the gold standard for treating CVMs despite high rates of complication, morbidity, and recurrence. Complete eradication of the nidus of the CVM is required to achieve an effective cure and generally requires surgical excess (e.g., radical resection) with high morbidity (e.g., excessive blood loss). Incomplete resection of the lesion’s nidus is unavoidable in many cases as a result of the prohibitively high morbidity associated with radical surgical therapy and results in higher risks of recurrence.

Open surgical resection outcomes have also significantly improved when combined with endovascular therapy ( Figure 1 ). Embolotherapy and sclerotherapy improves the safety and effectiveness of surgical therapy when used as an adjunctive treatment. Preoperative embolization and sclerotherapy of the CVM lesion can reduce the morbidity and likelihood of complications (e.g., intraoperative bleeding) associated with surgical resection. In addition, postoperative supplemental endovascular therapy can also improve overall efficacy of surgical therapy while avoiding surgical excess.