Samuel M. Lam, M.D* and Edwin F. Williams, III, M.D.**
*Lam Facial Plastic Surgery Center,
**Clinical Associate Professor, Division of Otolaryngology, Department of Surgery,
Albany Medical College,
Albany, New York
Chief of Division,
Facial Plastic & Reconstructive Surgery,
Albany Medical College,
Albany, New York
Port-wine stains represent the most common type of vascular malformation. With the advent of pulse-dye laser therapy, treatment of these lesions has become achievable with minimal prospect of adverse scarring. Although many scientific articles have heralded the favorable outcomes in patients who have undergone pulse-dye laser treatments, few papers address the practical considerations (preoperative, intraoperative, and postoperative) that must be followed in order to ensure success. This paper presents the ten-year experience of the senior author (EFW) in the management of port-wine malformations and the clinical insights that have been gleaned from that extensive experience. Introductory remarks are made as to the nature of port-wine stains, related syndromes, and the evolution of treatment; and practical management guidelines are then discussed.
Port-wine stains (PWS), or more correctly, capillary vascular malformations, represent the most common type of vascular malformation. They occur in 3 of 1000 births, arise predominantly in the head and neck region (80%), and exhibit an equal sex distribution.1 PWS manifests initially as a flat lesion with a red to pink hue which may lighten during the first year but then tends to darken throughout life turning a deeper shade of red or blue and may even become thicker or more nodular as the individual matures.2 Two related syndromes have been linked with PWS: Sturge-Weber (SWS) and Klippel-Trenaunay (KTS).
Sturge-Weber, or encephalotrigeminal angiomatosis, refers to a PWS that is distributed in the first trigeminal division (V1), with or without V2 or V3 involvement, and with central nervous system (CNS) abnormalities. CNS defects include cerebral atrophy, leptomeningeal angiomas, and cortical calcifications that may lead to seizures, mental retardation and hemiparesis. Magnetic resonance imaging (MRI) should be conducted to screen the high-risk infant. In addition, ocular examinations should be undertaken to determine whether glaucoma is present with the syndrome. Klippel-Trenaunay syndrome, or angio-osteohypertrophy, is characterized by a PWS that usually involves a unilateral, lower extremity marked by hypertrophy, varicose veins, lymphedema and phleboliths. 3
Prior to the introduction of laser therapy, the only method of treatment for PWS was simply cosmetic camouflage. Initially, the argon laser showed promise in the treatment of vascular diseases, but the incidence of scarring and the advent of the pulse-dye laser have largely relegated the argon to historical interest. Now with the pulse-dye laser that selectively targets the vascular chromophore in PWS, patients have found a new hope in minimizing their deformity with little morbidity. Numerous studies have documented the proven reliability of the pulse-dye laser in treating PWS.4,5Some studies have investigated the role of a 532-nm KTP laser in the treatment of resistant PWS lesions or of mature PWS lesions that manifest nodularity and found clinical efficacy6, but scarring has been reported with this laser type.6,7 Intense pulsed light has also proven to be highly effective and safe for the treatment of PWS according to one study.8 However, the pulse-dye laser remains the proven gold standard for treatment of PWS.
Recent studies have emphasized the psychological aspect as much as the efficacy of treatment.9-12 Most studies have demonstrated the ostracizing effects of PWS and the benefit of intervention to the psychological welfare of the afflicted individual. One small study contended that the patients in the series (n=9) revealed no subjective benefit to treatment despite objective physical improvement.10 As the child matures, he or she not only confronts the psychosocial trauma of bearing the unsightly mark but also may develop a lesion that is more difficult to treat as age and hormonal factors darken and thicken the PWS. Proper counseling and early treatment should be initiated to avoid these adverse outcomes.
The first step in preoperative assessment is a thorough history and physical examination. The detailed history should address pertinent birth history, change in color or texture of the lesion, prior workup for relevant syndromes (Sturge-Weber or Klippel-Trenaunay), psychological effects of bearing the disfigurement, previous treatments, and standard inquiries into the past medical, surgical, social, and family history. More specifically, the patient should be asked if any alteration in color or texture of the lesion occurred with puberty, pregnancy (or other hormonal factors), prior trauma, or simply due to gradual maturation of the lesion.
If the lesion involves the V1 distribution with or without V2 or V3 involvement, we recommend an appropriate evaluation for possible Sturge-Weber syndrome. However, an isolated V1 manifestation that does not encroach on either eyelid is rarely indicative of SWS, and we do not compel patients to seek further workup. In fact, the extent of eyelid involvement (i.e., only upper versus both upper- and lower-eyelid extension) may reflect the likelihood that glaucoma may be present (personal observation & communication with other specialists). As part of the workup for SWS, an MRI scan should be performed after the first 6 months of life, as the abnormal cerebral vascular findings usually are not radiographically apparent prior to this time. Any abnormal findings would be conclusive of SWS and would warrant medical prophylaxis for seizures. We refer these individuals to their pediatrician for further counseling and management of this condition. As mentioned, part of SWS also involves the potential for the development of glaucoma in the ipsilateral eye. At the time of diagnosis of the PWS, we refer appropriate candidates to an ophthalmologist for evaluation and further care. Furthermore, patients may only have ocular and cutaneous manifestations and warrant treatment for both conditions but not constitute SWS for lack of cerebral findings. After two years of age, if the child with V1 PWS has not exhibited any central manifestations of SWS, namely seizures, hemiparesis, etc., then the likelihood for any syndromic abnormality is rare. However, risk of glaucoma is still present and should be assessed. All of these ocular and cerebral examinations should be carefully documented in the patient’s medical record.
Unlike Sturge-Weber syndrome, Klippel-Trenaunay syndrome does not carry commensurately grave morbidity. If a child with PWS of the trunk or limbs, particularly involving the lower extremities, presents with pain in the affected limb or limb disparity (of length or circumference vis-à-vis the contralateral extremity) then the diagnosis of KTS may be entertained. KTS extends beyond the superficial cutaneous dimensions of typical PWS to involve the deeper tissues; and this extension may be readily apparent on MRI scanning. If the underlying soft-tissue appears to be involved, then pressure application with support stockings may alleviate some discomfort and retard the progress of vascular expansion. Also, deep extension of this type of PWS is not readily amenable to pulse-dye therapy and may be more properly addressed with surgical excision with or without preoperative tissue expansion, if conservative measures, e.g., pulse-dye laser and pressure application, fail.
A physical examination should address the color, texture (presence or absence of nodularity), and extent of the lesion in addition to the formal inspection of the entire body per routine. Photographic documentation is critical to assess clinical improvement, document findings, secure insurance approval, and promote scientific communication. If the child is mature enough to cooperate, we attempt to obtain a full set of standardized photographs (frontal, oblique, lateral, and close-up frontal) prior to any procedure. If the lesion appears in the head and neck region, we consider this appropriate for submission to the insurance carrier for compensation. However, if the PWS is situated elsewhere, particularly the trunk or lower extremities, we believe that such a location would be more properly deemed cosmetic in nature (unless an underlying syndrome such as KTS may be giving rise to limb disparity or concomitant pain). Also, PWS that affects the trunk or extremity may be more refractory to treatment and has a proclivity to recur. For these reasons, we discourage treatment of these types of lesions; and, therefore, we also feel an ethical responsibility not to submit uncomplicated trunk or limb PWS lesions to the insurance carrier for compensation.
Part of the preoperative consultation requires education of the patient about the natural course of this disease and the need for repeated treatments to effect an improvement. The patient should fully understand that the PWS is a progressive disorder and may become darker and more nodular in nature if left untreated. Also it should be clearly stated that the PWS cannot be entirely eradicated but only lightened and perhaps flattened by pulse-dye laser therapy. Despite treatments, the lesion may continue to progress but usually at a considerably reduced rate. Studies have shown that 50% of PWS tend to return over time to their initial pretreatment appearance, and this unfortunate tendency should be honestly communicated to the patient. 13 On the other hand, some scholars contend that early and aggressive laser therapy may fully arrest, and reverse, the evolution of PWS. We advocate the first treatment session to begin no earlier than 6 months of age to ensure that the infant has the fortitude to undergo anesthesia safely (Figure 3). In addition, we tend to favor earlier laser therapy, as mature lesions may be more advanced or recalcitrant to laser intervention. However, we tailor our treatment to patients’ desire and motivation. Further, we inform patients that the degree of response after the first laser-treatment session may prognosticate the likelihood of the lesion to respond equally well, or poorly, after future sessions. Usually, we outline a conservative plan for 3 to 5 treatment sessions separated by a minimum of 6-week intervals as needed to achieve a desired endpoint. If that objective is not met after the projected treatment timeframe, then further sessions are designed accordingly.
Prior to an in-depth discussion concerning technical aspects of intraoperative care, we should clearly state our philosophy that supports judicious and appropriate treatment of PWS. We disagree with the need to perform “test spots” before treatment of the PWS, as the safety of the pulse-dye laser, if used correctly, has been well established. Further, segmental treatment of the PWS only prolongs the unnecessary misery of the patient and his or her family, who already must return on numerous occasions even when the entire lesion is treated at once. Usually, segmental laser therapy is performed to minimize patient discomfort. However, as we rely on sedated or general anesthesia to perform all of our PWS treatment sessions, we have not encountered any reports of patient discomfort. In our experience, younger children who have undergone laser therapy without any modicum of anesthesia are severely psychologically crippled from the experience and present to us less trusting and more fearful of any further intervention. Even older individuals are more favorably inclined to undergo further sessions, as they do not harbor any anxiety about potential pain or discomfort. Ethically, we also contend that treatment of the entire lesion at each session is less costly to the insurance carrier, to whom we should be responsible.
We will now discuss the myriad details to which one should adhere to optimize a successful outcome. The patient’s photographic record should be brought into the operative suite to confirm the extent of the actual lesion. After anesthesia is administered, when the patient lies supine, or when the child cries, the adjacent, normal tissue becomes engorged, and the delineation between PWS and normal skin is diminished. Further, a surgical marking pen should be used to outline the lesion before induction of anesthesia to ensure that the pulse-dye laser adequately treats the entire lesion and nothing else (Figure 1). The particular safety goggles worn for pulse-dye laser administration renders the PWS less visually distinct, and the marking pen facilitates ease of determining the perimeter of the lesion during the procedure. All staff must also don appropriate protective goggles prior to commencement of laser therapy. In addition, the patient should wear corneal shields to protect against inadvertent retinal injury (Figure 1). If the child’s frame is too small to accommodate corneal shields, he or she should have the eyes held shut by the staff and/or surgeon during the procedure. We have found that the fluences required to effect a favorable change are sufficiently high to cause some patient discomfort. Therefore, we advocate use of monitored anesthesia care in which the patient is sedated but spontaneously respiring. In the younger child (less than 2 years of age), general mask anesthesia is routinely required to ensure cooperation and to avoid the aforementioned unfavorable traumatic stress induced by lack of anesthesia and sedation. The anesthetic mask is temporarily removed as the laser passes over the area covered by the mask. The affected eyebrow is coated with surgical lubricating gel in order to avoid damage to the hair follicles but which still permits treatment of the underlying skin.
After the initial laser spot is administered, the surgeon pauses to inspect the tissue for appropriate response, i.e., the tissue should appear a dark purple but not assume a grayish hue, the latter of which portends potential over-treatment that may engender cicatricial formation. We then adjust the fluence accordingly to achieve the desired purpuric endpoint. However, in older individuals who have resistant PWS, we do attempt to attain a grayish endpoint in order to ensure a lightening of color. We tend to use higher fluences (8 to 12 J/cm2) in older (> 2 years old) patients particularly along the perimeter of the lesion (vide infra), whereas we rely on lower fluences (5 to 7 J/cm2) in infants who have more sensitive skin and may be more prone to scarring. We have found that the 5-mm spot-size handpiece allows us to achieve higher fluences and more precise control when addressing the periphery of the lesion. The 7-mm handpiece permits a wider arc of coverage for the more central areas when lower fluences are preferred. However, the 5-mm handpiece is often adequate when treating the central areas of an infant with a smaller lesion when the fluence is adjusted downward from that used on the periphery. These aforementioned fluences and spot-size parameters are only meant to serve as guidelines for the reader, and clinical judgment must be exercised. In addition, we have determined that these fluences and spot sizes are optimal for our laser device (ScleroPlus, Candela Corp., Wayland, MA) and safe when used with the proprietary Dynamic Cooling Device (DCD) spray, which ejects a pre-laser cryogen burst to protect the epidermis. For subsequent treatment sessions, we usually adhere to the same parameters if the patient has noticed a favorable change.
We target the perimeter of the lesion first with a direct, perpendicular aim of the laser at a higher fluence in order to soften the transition between normal and abnormal skin. We have found that this method effectively obscures the PWS by attenuating the border of demarcation 14 (Figure 1,2). It is imperative that the edge of the laser beam not overlap at all with normal adjacent skin because the unaffected skin will lighten and form a halo around the lesion (particularly in darker complected patients), which in turn only accentuates the border. The central areas of the lesion are then treated with either the same or lower fluence of the laser aimed tangentially to the skin surface in order to avoid the uneven, lattice-like appearance of partially treated areas (Figure 1,2). We also aim the laser beam obliquely over the eyelid and perform at most one pass of the laser, as the eyelid skin is very thin. At the end of the treatment, we apply a thin coating of Bacitracin ointment to facilitate healing, which the patient or patient’s family maintains twice daily for the first postoperative week.
We routinely see patients six weeks after the treatment session, as any time earlier is too premature to gauge whether the effected change has occurred. Further treatment sessions depend on patient satisfaction with prior sessions and motivation to pursue another cycle of therapy. Some patients desire intermittent treatments to accommodate their work or school schedule and at times express the need to abstain from treatments for awhile until they are psychologically ready to continue with therapy.
Potential complications from pulse-dye laser therapy are rare and include scarring, uneven pigmentary ablation, hypopigmentation of surrounding normal skin, ocular injuries, transient alopecia, herpetic outbreaks, and cutaneous infections. An area of scarred skin should be avoided on subsequent treatment sessions and hopefully allowed to heal over time. Uneven pigmentary changes can be addressed by focusing the laser on the residual darker patches and feathering the laser gently around these areas. Hypopigmentation may resolve with time, but frank depigmentation may be more recalcitrant. Ocular injuries are very rare, as the laser’s efficacy is significantly reduced after a 0.5-mm distance and as diligent protective measures, e.g., use of corneal shields, should be undertaken during every procedure. Alopecia, even in the eyebrow area, is temporary, and the patient should be reassured of this fact. We do not routinely treat patients prophylactically with antiviral medication but would initiate treatment if a herpetic outbreak should manifest itself. Cutaneous infections, albeit unusual, may be adequately treated with antibiotics aimed at staphylococcus aureusand/or pseudomonas aeruginosa. Fortunately, complications are very infrequent and usually readily amenable to treatment.
Pulse-dye laser therapy for PWS is a safe and effective treatment that may lighten but not eliminate the stigma of the hyperpigmented lesion. Due to the paucity of practical treatment information that exists in the literature, we have endeavored to provide a succinct, but comprehensive, management outline that addresses pertinent pre-, intra-, and postoperative care for the PWS patient so as to maximize benefit and minimize morbidity.
Figure 1: Illustration highlighting the pertinent intraoperative considerations that should be respected to ensure a favorable outcome.
Figure 2: Illustration demonstrating the tangential angle that the laser beam should be directed to treat the central areas of the PWS evenly and to avoid the lattice-like appearance that arises from a direct, perpendicular aim of the laser (A), and the perpendicular angle to the skin that the laser should be aimed at the periphery of the lesion to diminish the abrupt transition between the PWS lesion and normal adjacent skin with the use of a high-fluence setting and the laser maximally focused on the skin (B).