A well-balanced approach that incorporates calculated risk and cautious execution is necessary-one that can only be cultivated with experience. The surgeon whose practice is devoted to the treatment of these lesions is obliged to document meticulously and analyze critically; these are habits that will serve one well for lifelong learning.

These lesions can never be absolutely eradicated. Persistence of the vascular anomaly is the rule despite clean and thorough extirpative operations. This is an important consideration in preoperative planning, family/patient counseling, and future interventions.

SURGICAL INDICATIONS

The indications for surgical intervention in the upper extremity are similar to other areas of the body. Typical reasons to intervene are pain, ulceration, persistent growth, functional compromise, and even appearance (on occasion). A trial of conservative treatment is always worthwhile before surgical intervention. This may include compression garments, blood thinners (for example, aspirin), injections, splinting, or hand therapy. Many children with significant vascular anomalies are treated conservatively during childhood and present as teenagers requesting surgery. Lymphatic malformations (LMs) and VMs tend to become more symptomatic in adolescent females than in males. In contrast, fast-flow lesions are slow growing, indolent, and insidious. The surgeon should provide a clear explanation of all potential complications and expected short-term and long-term outcomes before any surgical treatment.

The risks and benefits of surgical intervention must be weighed carefully against minimally invasive intervention. During the past several decades, the treatment of most vascular anomalies has improved as the result of minimally invasive techniques such that most lesions can now be treated by interventional radiologists. As scientists further elucidate the molecular origin and structure of these conditions, medical therapy has continued to improve. More recently, sirolimus has been used more frequently with promising outcomes to treat larger, more difficult lesions. Nevertheless, surgery is the best treatment for some of these lesions. For example, most LMs are confined to the subcutaneous tissue planes and are frequently removed without the need to explore the deeper tissues.

The surgeon who has the courage to treat difficult fast-flow lesions should also be prepared to amputate a symptomatic nonfunctional or painful digit or limb after unsuccessful attempts at palliation. Patients with severe pain will often request amputation of the affected part. Other patients may wish to continue treatment despite a functionless, parasitic limb or digit. The most difficult decisions are those in young children who do not verbalize their pain and some teenagers who have minimal tolerance to pain and have not adapted well to their limb malformation. In difficult lesions, amputation should be considered and discussed before surgery. The reconstructive surgeon should not view amputation as a failure.

Surgical principles gain acceptance with time and must be periodically reassessed and refined, particularly in this rapidly advancing field. Principles should not be confused with techniques that change frequently and are modulated to a great extent by technology. Many of the following principles have evolved from the early surgical experience with vascular malformations (especially the fast-flow types), which was punctuated with complications.

PREOPERATIVE PLANNING

Preoperative planning is the cardinal principle of surgical treatment. Most poor outcomes can be avoided by adhering to this principle alone. Planning should include correlation of the size, extent, and involvement of structures with physical examination and imaging. Imaging modalities include plain radiographs, ultrasonography, MRIs, and angiography. Before surgery is performed on complex, fast-flow lesions, a step-by-step plan of resection, including the approach and extent of resection, must be determined and followed. Despite imaging, the extension of some anomalies may not be completely delineated, and intraoperative decision-making may be required after the true extent becomes visible. Despite this, the surgeon must abide (as much as possible) by the initial surgical plan; straying from the plan may lead to a violation of uninvolved areas, an inadvertent injury to neurovascular structures, a waste of precious tourniquet time, and an incomplete excision. For complex lesions, we have found it helpful to print the angiogram and label the nidus, shunts, and all large branches within that portion of the lesion to be excised. These scans should be displayed in the operating room for easy reference during the procedure. Other preparatory steps for fast-flow lesions include central venous and peripheral arterial monitoring, availability of warmers and blood products for transfusion, and ICU monitoring after surgery.

PLACEMENT OF INCISIONS

Placement of incisions is important, particularly in children. When planning an incision, the surgeon should consider exposure, blood supply, function, aesthetics, and future surgery. Incisions should be long enough to provide full exposure of the entire lesion. Small incisions provide no surgical advantage and risk injury to important structures, especially in the hand. In the digits, a high midaxial incision is preferred, because it provides excellent dorsal and palmar exposure, can be used again, and is typically well hidden. Dorsal longitudinal hand and digital incisions are avoided, if possible, because they provide less exposure and are more conspicuous. Palmar incisions can lead to contracture if poorly planned; zigzag incisions and those that use normal skin creases are recommended.

If multiple debulking procedures are contemplated in the upper arm or forearm, each incision should be planned carefully to avoid unnecessary scarring near neurovascular structures. The medial surface of the arm, elbow, and forearm is the least conspicuous. Scars in the web spaces or along eponychial and/or paronychial folds will contract and are likely to become problematic.

DISSECTION IN A BLOODLESS FIELD

Dissection in a bloodless field is critical for identification of important structures and delineation of the vascular lesion. Accordingly, a pneumatic tourniquet is essential for all but the simplest excisions. Limb exsanguination should be thorough before tourniquet inflation. If bleeding occurs in the wound within 5 to 10 minutes of tourniquet inflation, the arm should be reelevated and the process repeated. In small children we use the elastic Esmarch wrap as the tourniquet secured at the midhumeral level. Dissection of VMs, lymphaticovenous malformations (LVMs), and arteriovenous malformations (AVMs) with arteriovenous fistulas is most vulnerable to blood staining, whereas LMs are more forgiving. A blood-stained field within any dissection will decrease visibility, obliterate fascial planes, and set the stage for potential injury to important structures.

With a chest wall LM and capillary-LVM excisions or brachial plexus explorations, the tourniquet cannot be used. During these long procedures, blood loss can be insidious, especially in small children. Thus a premium is placed on meticulous hemostasis, which is best accomplished by careful dissection and preemptive ligation or clipping of vessels before they are cut. To further minimize surgical blood loss and blood staining, four types of cautery devices are used selectively: the monopolar cautery, the bipolar microsurgical cautery, the battery-operated handheld ophthalmic cautery, and the Aquamantys system (radiofrequency energy with saline coolant). Each device has a specific use. The monopolar cautery is used for subcutaneous dissection, the bipolar is used for small vessels, and the ophthalmic cautery is used for developing surgical planes, especially within muscles, which tend to fasciculate with the monopolar cautery. The Aquamantys system coagulates larger vessels and high venous pressure/variceal types of bleeding commonly encountered during excision of VMs. Tissue sealants have been a useful adjunct at the end of a procedure after the tourniquet is deflated. Excision of fast-flow lesions of the chest and axilla is a dreaded (but not impossible) procedure and accomplished with the aid of intraarterial balloon catheters, hypothermia, hypotensive anesthesia, cell savers, adequate blood and fluid resuscitation, and experienced anesthesiologists and cardiovascular surgeons.

SURGICAL INSTRUMENTS