Compression. Compression garments are the first line of treatment for venous, lymphatic, and mixed malformations and are used to reduce blood stagnation, symptomatic expansion, phlebolith formation, and the chance of localized intralesional coagulopathy (LIC). Children who experience relief of their symptoms with compression can become surprisingly compliant and active in daily life.

Pharmacologic. Pain is often associated with intralesional thrombi or phlebothrombosis of vascular malformations. Daily low-dose aspirin helps to prevent thrombus formation. More definitive anticoagulation and placement of an intraluminal inferior vena caval filter are used selectively in the patient at high risk for thromboembolism. Although long-term anticoagulation intralesional bleeding, with or without nerve compression or muscle compartment swelling, may require appropriate decompression.

Sclerotherapy. Direct-puncture or catheter-introduced sclerotherapy is the next step for venous and lymphatic malformations that cause functional impairment, nerve compression, mass effect, pain, or major contour problems if conservative measures fail. This intervention reduces the size of the malformation, but generates scar and does not remove the malformation. The secondary scar generated by sclerotherapy can be detrimental to nerve function, tendon gliding, and joint motion especially in the distal forearm, hand, and digits. In the upper arm, elbow, and proximal forearm, sclerotherapy is safer and often more effective than resection (Burrows et al. 2004; Smith et al. 2009). Sclerotherapy is preferred for diffuse lesions, except for those with multiple intralesional thromboses, compressive symptoms, or lesions which involve the distal forearm, wrist, hand, and digits. Figure 1 shows the radiographic work-up and injection of a proximal third forearm VM which is ideal for sclerotherapy.

Sclerosant agents include doxycycline, sodium tetradecyl sulfate (STS), ethanol, bleomycin, and OK-432. Ethanol has the highest complication rate; OK-432, although very effective, is not widely available (Burrows et al. 2004). Direct injection of sclerosant near neurovascular structures within the palm of the hand, volar wrist flexion crease, antecubital fossa, and axilla carries a considerable potential risk. The most common complications are skin ulceration (10–15 %), local extravasation, compartment syndrome, and secondary contracture. Multiple sessions are often required.

Although nonoperative therapy is initially preferred, surgery is mandated when these previous modalities have failed to correct or even control functional loss, compression neuropathies, recurrent infections, and pain. In the adolescent, aesthetics is not an irrelevant consideration.

The advantages of surgical resection include the use of a pneumatic tourniquet, the identification of all anatomic structures, direct removal of the malformation and involved tissues, and sparing of vital structures. Compared to the other modalities, however, surgical resection imparts a longer recovery time with the need for more rigorous rehabilitation. It should be stated from the outset that unless amputated, these lesions can never be absolutely eradicated.

The excision of a vascular malformation is always beset with potential pitfalls, and complications include compartment syndrome, bleeding, wound dehiscence, infection, devascularization of a limb or digit, secondary contracture, tendon adherence, and chronic pain secondary to nerve injury. Adherence to basic surgical principles will minimize these potential problems.

Vascular anomalies in the upper arm and axillary areas are usually in continuity with anomalies extending into the supraclavicular fossa, neck, and not uncommonly the mediastinum. The inability to use a pneumatic tourniquet makes resection in this region hazardous – intraoperative blood loss can be life-threatening. Interventional radiologic and endovascular laser approaches are therefore preferred.

If resection is required, careful preoperative planning, coordination with the anesthesiologist, and careful dissection with cell savers, blood products, and hypotensive anesthesia available should ensure a successful outcome. Quilting sutures and tissue sealants are helpful during closure, and drains may need to remain in place for weeks or months postoperatively. The child in Fig. 2 required all these considerations for his complicated upper limb and mediastinal LM. It is impossible to remove all pathologic tissue in this region; however, initial resection should be thorough because reentry into these areas is always accompanied by major bleeding, scarring, and subsequent contracture.

Large lymphatic channels or extensive VMs are often intertwined with the brachial plexus. The brachial plexus is easily damaged if one is not familiar with the anatomy, has inadequate hemostasis, or suboptimal assistance. Fortunately, an areolar/fat connective tissue plane is present around these nerves and serves as a guide for dissection.

All of these patients with large proximal lesions are at risk for pulmonary embolism even in the context of a normal preoperative coagulation work-up, since the vascular channels within the lesion – especially inflamed portions – may be locally coagulopathic.

The lower arm and elbow region is much more amenable to surgical approaches than the axilla and upper arm because a pneumatic tourniquet can be used. Excision of an LM in this area is much easier than other types of malformation because LM is primarily confined to the subcutaneous tissue planes.

The neurovascular structures must be identified before the resection begins. They are generally in their normal location though they may course directly through extensive areas of malformation. The biceps tendon is the best reference point anteriorly, as the median nerve and brachial artery are always medial to it. Both these structures can be difficult to identify in the presence of an extensive vascular malformation, especially in combined lesions such as CLVM and LVM. It is very easy to inadvertently remove the brachial artery in the midst of large, thick-walled VM. Epineural fat is usually a different color from the dysplastic adipose tissue in the vascular malformation and is therefore a useful marker. The ulnar nerve should be transposed following debulking and decompression.

Sclerotherapy is preferred in this location and remains the first line of intervention (Burrows and Mason 2004). If surgery is required, the forearm is ideally approached through a straight medial incision, extending from the wrist to the elbow. The proximal forearm presents one of the most difficult operative fields for the extremity surgeon due to the high density of important anatomic structures in a compact space. While LMs tend to stay between fascial planes, VMs in this region are usually diffuse and usually extend well beyond the fascial planes into the muscles. Intramuscular lesions are difficult to resect because of the potential for damage to adjacent muscle and injury to the many small motor branches of the median nerve. Commonly, vascular anomalies involve the dorsal interosseous system along the entire length of the interosseous membrane and may involve both the dorsal and the volar compartments.

The distal forearm and wrist regions are more amenable to surgical extirpation because there is less muscle and the major nerves have few arborizations. Surgical outcomes in this area are more predictable with less morbidity than outcomes following sclerotherapy (Upton et al. 1999; Upton and Marler 2006; Upton and Taghinia 2011). Frequently, what appears to be a superficial lesion in the distal forearm and wrist extend through the interosseous membrane and involve the rete system. Removal of the membrane may be required, but it is important to retain as much of the normal interosseous arterial anatomy as possible. The median and ulnar nerves are frequently involved in this region and may become heavily studded with nests of VM with intralesional thrombosis. Denervation of both anterior and posterior interosseous nerves may help alleviate postoperative wrist pain.

Sclerotherapy in this portion of the hand is highly discouraged because of the many potential complications due to the high concentration of neurovascular structures, gliding tendons, and delicate intrinsic muscles. Because of its complex anatomy, the hand is the most difficult region to resect any type of vascular malformation (Upton and Marler 2006). Table 5 outlines key principles to follow in the approach to surgical resection of palmar lesions.

These procedures are tedious and lengthy, often requiring two or even three tourniquet runs. What appears to be localized lesions in the palm and dorsum of the hand is always found to be much more extensive intraoperatively. Technique must be meticulous, preserving all sensory and motor nerves, common digital arteries, and the palmar arch. Thorough exsanguination and compulsive hemostasis are mandated. Once blood staining occurs, the connective tissue planes become blurred. In Fig. 3, a typical palmar VM is presented with its preoperative bulk and blemish, an MR investigation of its extent, a precise dissection, and its immediate postoperative appearance and management with closed-suction drains.