Contemporary Management of Port Wine Malformations

Edward D. Buckingham, M.D.

Edwin F. Williams, M.D., F.A.C.S. *

Williams’ Center for Facial Plastic and Reconstructive Surgery

1072 Troy Schenectady Road

Latham, NY 12110

edbuckin@hotmail.com

edwilliams@nelasersurg.com

518-786-7000 (phone) 518-786-1160(fax)

*Corresponding author

Port-wine malformations also referred to as port-wine stains (PWS) are more accurately described as postcapillary venule malformations. In the past the classifications of vascular malformations proved confusing, however the landmark work of Mullikan and Glowicki elucidated the difference and provided a classification scheme that clearly differentiated the different vascular malformations and hemangiomas. (1) The incidence of PWS is approximately 0.3% with an equal sex distribution and 80% presenting in the head and neck region. (2) Like all true vascular malformation, port-wine lesions are always present at birth but not always evident, as post-delivery erythema or anemia of infancy may camouflage them. Port-wine malformations present as a smooth pink or salmon colored area without a well demarcated border. It is believed that they result from a relative or absolute deficiency of autonomic innervation of the precapillary sphincter.(3) (Fig. 1) As with all malformations, PWS will continue to grow throughout life although at variable rates. The primary treatment for PWS today is the tunable pulsed dye laser. The following discussion will be primarily focused around this modality of therapy and the nuances of its use. Indications

PWS should not be considered only as a cosmetic dilemma. As these lesions progress throughout life they will take on a more red appearance and often, approximately, 20-30% of lesions by age 40, progress to a dark purple lesion with raised nodularity. (Fig. 2) Troublesome bleeding and pyogenic granulomas may occur in the mature malformation. It is therefore important to educate the patient, and/or parents depending on the age at presentation, as to the natural course of the lesion and the possible consequences of lack of treatment. Additionally, the psychological consequences of these malformations and the psychological benefit of treatment has been well described, therefore it is recommended to offer treatment to all patients with a PWS who are willing to accept treatment and the potential although minimal complications of such treatment. While infants may begin treatment in the early weeks of life there is really no benefit to beginning treatment this early and the anesthetic risk is much greater. Treatment is therefore offered to patients beginning at one year of age.

Contraindications

No contraindications exist to treatment with the pulsed-dye laser itself. A relative contraindication exists for those patients who are unable to accept the limitations of treatment to not cure the malformation. However, with careful discussion this is not a problem. Additionally, patients with mature PWS must understand and accept the limited improvement that can be obtained with pulse dye laser treatment. Once the lesion has progressed so that it is thickened and nodular, the pulsed-dye laser has limited penetration and therefore cannot significantly reduce the thickness. The only other consideration is anesthesia risk, which is no different than other surgical procedures and can be avoided altogether in the highly motivated patient if necessary.

Alternative Techniques

While other laser and surgical therapies have been used in the past, an unacceptably high incidence of scarring and poor results has lead to their demise. The concept of selective photothermolysis and the introduction of the pulsed-dye laser has revolutionized treatment for these lesions. The carbon dioxide laser does have limited application for the highly motivated patient with severe epidermal surface irregularity whose PWS has reached the point of diminishing return with the pulsed-dye laser or is already advanced with surface irregularity and nodularity. Additionally in the rare patient with a significant subcutaneous component usually associated with Sturge-Weber Syndrome, limited surgical resection of the subcutaneous portion beyond the reach of the laser may be offered. (3) As an adjuvant to pulse dye laser, patients with advanced lesions exhibiting nodularity or pyogenic grannulomas are offered simple excision of the nodules with or without additional pulse dye therapy.

Preoperative Considerations

Port-Wine Malformations are associated with two distinct syndromes. Sturge-Weber syndrome is a condition where a PWS of the V₁ distribution of the trigeminal nerve with or without involvement of V₂ is associated with involvement of the choroid plexus of the eye and/or leptomeningeal involvement. Leptomeningial involvement may lead to calcifications of the dura or brain and subsequent seizure disorder; therefore, young children with significant V₁ with or without V₂ involvement should be referred for MRI evaluation. However, in children presenting after age two who have not yet developed a seizure disorder, the yield of MRI is extremely low and so therefore these children are merely observed for the development of CNS symptoms. Children with either involvement of the eyelids or V₁ and V₂ involvement are also at significant risk of developing glaucoma and therefore should be evaluated by an ophthalmologist with follow-up visits every 6 months or as recommended by the eye physician. (3) Klippel-Trenaunay syndrome is characterized by a PWS that usually involves a unilateral, lower extremity with deep tissue involvement manifested clinically by unilateral limb enlargement, varicose veins, lyphedema, and phleboliths. The treatment of this syndrome is usually conservative and consists of compressive garments, elevation, and swimming

exercises. (2)

Because PWS will progress throughout life without treatment and become more difficult to treat as they progress, early intervention is preferred. (Fig. 3) All patients should be counseled thoroughly as to the natural course of the disease and offered treatment. While treatment is not a cure, early intervention and diligent continuation of treatment as indicated is very effective in limiting the progression of disease in most individuals. Several prognostic indicators exist that are fairly reliable in predicting response to treatment. Lesions that progress rapidly prior to the first treatment are more difficult. Lesions of the forehead and neck tend to respond better, whereas lesions of the central face and extremities respond more poorly. Lesions that are coalescent also tend to respond more slowly. It is theorized lesions that progress more rapidly and are coalescent tend to have a relative greater lack of innervation than their less coalescent more slowly progressing counterparts. Additionally, the lesions response to the first treatment is a good indication as to the future response. Those lesions with poor prognostic indicators will require more frequent aggressive treatment and the patient should be counseled appropriately.

Special surgical Requirements

The only requirements are a pulsed-dye laser preferably fitted with a dynamic cooling device, anesthesia services, and appropriate laser precautions and certification. While treatment could be preformed in the office using multiple limited procedures to treat the entire PWS, intravenous sedation in adults or mask general anesthesia in children allows the entire malformation to be treated in an aggressive fashion in one session. This prevents the discomfort in adults and eliminates the necessity of restraining devices in children therefore improving the surgical experience and likelihood of continued treatment. The added cost of anesthesia is well justified because the entire lesion is treated in one session; this practice is more cost effective for the patient over time than multiple smaller office procedures.

Preoperative Analysis

At initial presentation the most important item to consider is whether the PWS is part of a larger syndrome or if the vision is at risk from glaucoma. Appropriate referrals and imaging should then be obtained as discussed. Discussion with the patient and/or the parents should confirm that the lesion presented at birth or shortly thereafter, has been becoming progressively darker with time, and that the distribution of the malformation itself is relatively stable. This history and confirming physical exam is all that is necessary to diagnose the PWS and no further imaging is necessary unless a syndrome is suspected and the patient is age appropriate.

Once the diagnosis is verified the patient should be photographed. This is critical as a reference because once treatment begins it can become extremely difficult to determine the original borders of the PWS. The lesion should then be classified by describing the dermatome divisions involved and laterality. Additionally, it is helpful to describe whether the lesion is noncoalescent or confluent. Waner and Suen (4) proposed a grading system with a continuum from grade 1 to 4 based on the diameter of the vessels. Grade 1 lesions are the earliest lesions, have the smallest vessels, and are pink macules: whereas grade 4 lesions are the most advanced and can be described as large dilated vascular spaces with the vascular lesion described as thick, purple, and palpable with considerable nodularity or “cobblestoning”. (Fig. 4) It is important to realize that this grading system represents a continuum and that an early lesion left untreated may progress to a more advanced grade. Additionally, different areas within the PWS of the same patient may display different grades. (Fig. 5)

Surgical Technique

The pulsed-dye laser introduced in 1990 produced a wavelength of 585 nm; this wavelength corresponds to the second absorption peak of hemoglobin and the third absorption peak of oxyhemoglobin allowing for selective photothermolysis. At this wavelength, most of the energy is absorbed by the first 0.5 mm of tissue thereby limiting the response in port-wine malformation that has a significant deeper component. As the wavelength is increased from 585 to 600 nm, the incident of laser light will reach almost twice the depth, theoretically resulting in amore favorable response for PWS that have a slightly deeper component. Second generation lasers had the ability to adjust or “tune” the wavelength slightly from 585 nm to 600 nm, therefore increasing the ability to treat deeper into the tissue. Currently, a wavelength of 595 nm is used to treat most lesions. Occasionally for refractory lesions 600 nm is used in an attempt to achieve additional results. First generation lasers used a pulse length of 450 to 500 microseconds, significantly shorter than the thermal relaxation time of skin of 700 to 900 microseconds, and therefore very safe. Second generation lasers increased the pulse width to 1500 microseconds in an effort to increase clinical efficacy for larger vessel malformations. More recently, Candela Corporation has introduced the V beam pulse-dye laser. This laser allows treatment of a pulse width of up to 40 msecs in an attempt to increase the depth of penetration. Peer reviewed data on improved response rates with the longer pulse width are lacking and requires further investigation, clinically the longer pulse width does not appear to improve response (3)

Addition of a cryogen cooling spray to the pulse-dye laser allows cooling of the superficial epidermis, while allowing continued penetration of laser energy to the lesion and effective thermal ablation. The dynamic cooling device (DCD) is attached to the hand-piece of the pulse-dye laser and delivers a brief (20-30 msec) liquid cryogen pulse. Theoretically, the DCD allows cooling of the epidermis and therefore less thermal injury resulting in reduced risk for scarring and decreased discomfort when used in the office setting without anesthetic. Clinically, when using the device the purpora produced by the safe-start parameters is greatly reduced allowing treatment at fluences 2 to 3 J/cm² higher than start-safe parameters, and anecdotally improving results by allowing greater energy application to the deeper tissues. (5)

As with any laser procedure laser safety precautions are the first step. One of the members of staff should accept the responsibility as laser safety officer during the operation of the laser because most accidents and injuries occur with the casual use of a laser in the setting of well-trained and credentialed professionals. When an anesthetic is used, it is important to test or calibrate the laser before anesthetizing the patient because this practice will eventually save the clinician the embarrassment and possibly even litigation associated with inevitable malfunction of the laser.

The two greatest laser safety hazards include eye safety for staff and patients and the potential for fire combustion associated with laser use in an oxygen-enriched environment. For staff, the use of laser safety glasses of the appropriate wavelength is sufficient. Opaque eye shields are placed over the anesthetized patient to ensure that the eye is always protected from inadvertent exposure. The eyelids often need to be treated, and the use of opaque corneal shields is a safer way to protect the eye. Extreme caution needs to be exercised when treating near the oxygen-enriched environment associated with providing anesthesia. In children, the authors prefer a mask general anesthesia while treating the PWS. Cooperation between the anesthesiologist and treating physician allows for the simultaneous delivery of anesthesia and pulse-dye laser treatment. When treatment is required for the perioral area and nose, a brief interruption of the mask anesthesia makes treatment possible. If treatment of the perioral area and nose is extensive, the anesthesiologist and treating clinician can alternate masking and treatment to avoid intubation. During mask anesthesia, it is imperative that the flow of oxygen is observed cautiously and it is not allowed to flow over the operative filed indiscriminately.

For the adult patient, the authors prefer monitored anesthesia care with oxygen supplementation provided by way of an 8 F catheter placed through the nasal cavity, into the nasopharynx, and running at a rate of 2 L per minute. This approach assures that oxygen is delivered to the patient without increasing the concentration of ambient oxygen in the operative field.

In the cooperative patient the border of the PWS is marked out prior to anesthesia induction because anesthetic gases, vasoactive changes, and facial skin compression from bag masking all affect the appearance of the lesion. Then once a satisfactory level of anesthesia is achieved, the entire lesion is treated using the start safe fluence parameters provide by the laser company. The intra-operative use of good quality preoperative photographs compliments the surgical marking guiding treatment especially in the areas of lower grade. A systematic approach is recommended to minimize the potential for treating an area more than once or more commonly by missing treatment of an involved area. No attempt is made to treat hair-bearing scalp tissue except on the rare occasion to specifically treat an area of nodularity associated with recurrent bleeding. Malformation of the skin beneath the hair of the eyebrow can be safely treated without damaging the hair follicle by applying Surgilube â to the area and treating through the fluid. The laser energy passes with little if any diffusion through the hair, but adequately treats the skin, thus preventing combustion of the hair while allowing treatment of the malformation.

The authors prefer to treat the outline of the PWS first followed by treatment on the central portion for two reasons. Studies attempting to quantify perception of PWS size have determined that a softening of the outer border or line of demarcation is associated with the perception of a smaller PWS and has a better response. Furthermore, the effects of anesthesia over time often reduces the visibility of the PWS to the treating clinician’s eye, making the border of the PWS more difficult to follow.

The 7-mm hand-piece is used for larger areas, and the 5-mm hand-piece is used around the eyelids, nose, and mouth where a smaller spot size is needed. Because the energy striking the tissue follows the Gaussian distribution curve, the pulses should overlap approximately 10% to achieve uniform tissue fluence. The desired tissue response is between a purpora and a whitish appearance. (Fig. 6) A tissue response appearing whitish-gray is consistent with over-treatment, and the fluence should be reduced slightly to lessen the risk of scarring. Similarly, after a few pulses, the clinician should stop to assure a sufficient and appropriate tissue response. No or little lightening of the PWS will be observed if the lesions are not treated with an adequate fluence. With experience, the clinician will become comfortable adjusting the fluence away from the start safe parameters as appropriate, given the particular patient situation (i.e., treat more aggressively as age increases, confluent red lesions need to be treated more aggressively than noncoalescent pink lesions). Post-treatment care consists of a topical antibiotic petroleum-based ointment for 5 to 7 days or until the purpora resolves and ibuprofen is used on an as-required basis for discomfort. (3)

Postoperative Follow-up

A follow-up is recommended at 4 to 5 weeks because the clinical improvement continues to be noticed for 3 to 4 weeks. The response rate varies varies widely with all patients anywhere from a 5% to 50% improvement, depending on the factors of grade, rate of progression, and response to first treatment. (Fig. 7) For most patients, the recommended treatment plans discussed will usually involve two to four sessions separated by a 6 to 8 week interval. Treatment is usually continued until the lesion is minimally perceptible, the point of diminishing response, or according to patient desires and fatigue for further treatment. There are some reports of complete long-term clearance and eradication of the facial PWS, especially when treatment is initiated early and at a higher fluence. This, however, is somewhat controversial, and Waner has reported recurrence with sufficient time in as many as 50% of patients treated. (4)

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Management of Complications

Hypertrophic scar associated with a full-thickness injury is extremely rare in experienced hands but may occur with indiscriminant use of the laser, inexperience, or an overzealous effort. The tissue response to treatment should always be observed to avoid over-treatment and the potential for a hypertrophic scar. If this occurs topical, injectable, or oral steroids are the preferred method of treatment with surgical excision reserved for refractory cases.

The occurrence of an atrophic scar is also very uncommon and less problematic. The greatly reduced morbidity and recovery period has almost eliminated the possibility of developing an atrophic scar as it has been the authors’ experience that this is associated with a crust of scab that developed during the post-treatment period that was “picked” or removed by the patient. The use of the dynamic cooling device and appropriate post-operative topical placement of an antibiotic ointment should eliminate the potential risk for occurrence of an atrophic scar. (Fig.8) Temporary post-inflammatory hyperpigmentation may occur with any irritation or inflammation of the dermis; however, the presence of the malformation generally overshadows the perception or presence of hyperpigmentation. Permanent hypopigmentation of the adjacent noninvolved tissue is much more common and clinically significant with multiple treatments. This may actually accentuate or highlight the port-wine malformation along the perimeter and create a “halo” effect. This can occur because the absorption curve for melanin ascends through the treatment range of 585 nm to 600 nm. With repeated indiscriminant treatment of normal tissue the melanocyte population will be diminished permanently, resulting in hypopigmentation. This can be prevented by precise and careful use of the laser with minimal or no overlap onto normal tissue. General anesthetic in children assists in accomplishing precise treatment without the potential for movement while the surgeon is treating along the perimeter of the lesion. (3) Postoperative Analysis

The authors’ goal with treatment is to reduce the deformity associated with the PWS, to be cosmetically acceptable, and to help with preventing the progression of the malformation and the known sequelae of nodularity. Once the initial series of treatments is completed, most patients are recommended for treat3ment on an annual basis to prevent further progression and to assist in keeping the PWS cosmetically acceptable

Key Technical Points

1. Mark out border prior to initiation of anesthesia, use preoperative photographs

2. Laser safety, using corneal protectors for patient if periorbital area treated

3. Cooperation with the anesthesiologist especially regarding oxygen delivery and safety

4. Systematic approach to make sure and treat the entire malformation without duplication; overlap spots about 10% to allow even treatment

5. Treat perimeter first avoiding normal tissue to prevent hypopigmentation and “halo” effect

6. Use lower fluence for immature pink lesions and higher for mature darker lesions

7. Endpoint of treatment even purpora to whitish coloration

References

1. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: A classification based on endothelial characteristics. Plast Reconstr Surg 1982;69:412-422.
2. Lam SM, Williams EF III. Vascular anomalies: review and current therapy. Curr Opin Otolaryngol Head Neck Surg 2002;10:309-315.
3. Williams EF III, Hove C, Hochman M. The treatment of vascular malformations: Port-wine stains. Facial Plast Surg Clin North Am 2001;9(4):609-619.
4. Waner M, Suen JY. Hemangiomas and vascular malformations of the head and neck. New York: Wiley-Liss, 1999.
5. Cheng-Jen C, Nelson JS, Cryogen spray cooling and higher fluence pulsed dye laser treatment improve port-wine stain clearance while minimizing epidermal damage. Dermatol Surg 1999;25:767-772.