Treatment of Congenital Vascular Malformations in Children and Adults



Treatment of Congenital Vascular Malformations in Children and Adults



B.B. Lee, J. Laredo and R. Neville


Since the 1990s, new concepts based on sound knowledge and a new classification system have led to a better understanding of the pathophysiology, anatomy, and embryology of congenital vascular malformations (CVMs). Recognition of the fundamental differences between extratruncular and truncular lesions has led to the development of better management principles.


A proper understanding of the crucial differences between an extratruncular lesion that has origins from an earlier stage of embryogenesis and a truncular lesion that has origins from a later stage is required for the advanced management of the entire group of congenital vascular malformations.


A fresh approach based on this newly acquired knowledge and a multidisciplinary team approach has resulted in better treatment outcomes and allows delivery of highly specialized care. The current multidisciplinary team approach requires the participation and coordination of many highly specialized disciplines. Every patient and every CVM lesion should be reviewed by the multidisciplinary team. The treatment plan should be derived by consensus of the team, following the appropriate diagnostic workup. The consensus should support the treatment decision based on treatment indication criteria (Box 1).



BOX 1   Indications for Treatment of Congenital Venous Malformations





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.


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FIGURE 1 A combined endovascular and open surgical approach in the treatment of a large right flank arteriovenous malformation (AVM) using preoperative embolotherapy, followed by surgical resection and postoperative sclerotherapy. A, A patient with a pulsating mass on his back and right flank. B, The lesion was confirmed to be a localized AVM with minimal involvement of the surrounding tissues as demonstrated on conventional abdominal computed tomography (CT). C, The AVM is also seen on CT angiography (CTA) with three-dimensional (3-D) reconstruction. D, Subsequent arteriography was performed, confirming the presence of an extensive AVM with multiple feeding arteries. The massively dilated venous outflow seen is as a result of the fistulous lesion producing a hemodynamically advanced condition. E, Direct puncture and transvenous embolization using 0.035-inch Bentsen wires was performed preoperatively for subsequent open resection of the lesion to reduce intraoperative risk (e.g., bleeding). F, The lesion was completely excised with coils, leaving a minor residual lesion that was treated with conventional sclerotherapy. G, Follow-up CTA and 3-D reconstruction demonstrates an excellent result.

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Aug 25, 2016 | Posted by in CARDIOLOGY | Comments Off on Treatment of Congenital Vascular Malformations in Children and Adults

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