Carbon Dioxide Laser Treatment of Cutaneous Neurofibromas

Yi Zhen Chiang 0 Firas Al-Niaimi 0 Janice Ferguson 0 Paul Jeffrey August 0 Vishal Madan 0 0 Y. Z. Chiang F. Al-Niaimi J. Ferguson P. J. August V. Madan (&) The Dermatology Centre, Salford Royal NHS Foundation Trust , Manchester, UK Introduction: Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder, with multisystem involvement, including cutaneous manifestations of hyperpigmentation and neurofibromas. Multiple cutaneous lesions are often disfiguring and lead to emotional distress and social isolation. Treatment of NF1 is predominantly surgical but alternative treatments should be considered for patients with large numbers of lesions as cold steel excision of multiple lesions can be cumbersome This study was presented at the British Society of Dermatological Surgery section of the 90th meeting of the British Association of Dermatologists' in London, July 2011. - multiple neurofibromas. Hypopigmentation or depigmentation at treatment sites were the only reported adverse effects. Conclusion: Based on current results, the authors feel that CO2 laser treatment achieves a high level of patient satisfaction with a low recurrence of treated lesions. INTRODUCTION Neurofibromatosis type 1 (NF1), formerly known as Von Recklinghausens disease, is an autosomal dominant inherited condition with an incidence of 1:2,5001:3,000, and a prevalence of 1:4,0001:5,000 [1]. Clinical manifestations of NF1 involve multiple systems, including the nervous system, bone, and skin, with patients developing manifestations of hyperpigmentation and neurofibromas [2]. The National Institute of Health (NIH) Consensus Development Conference have determined the diagnostic criteria for NF1, which requires two or more of the following: six or more cafe au lait macules; two or more cutaneous/subcutaneous neurofibromas, or one plexiform neurofibroma; axillary/groin freckling; optic glioma; two or more Lisch nodules in the eyes; bony dysplasia; and a first-degree relative with NF1 [3]. Cutaneous neurofibromas can affect any part of the body and can vary in size, number, and distribution. Neurofibromas usually appear around puberty and continue to grow in size and number [4]. Significant disfigurement can result from the growth of hundreds of cutaneous neurofibromas, leading to social isolation and emotional distress [5]. Treatment of cutaneous neurofibromas is predominantly surgical but alternative treatments need to be considered for patients with multiple lesions often in excess of 100, in whom surgical intervention may not be possible or desirable [6]. The CO2 laser has been shown to be effective in treating large numbers of small-to-medium sized neurofibromas with cosmetic outcomes equal to or even better than surgical excision [2, 7]. However, evidence for CO2 effectiveness, effect of treatment on patient satisfaction, and rate of recurrence post-treatment is scarce. The authors evaluated a series of patients with multiple neurofibromas that were successfully treated using CO2 laser treatment under general anesthetic, and assessed patient satisfaction with the treatment and rate of recurrence post-treatment. MATERIALS AND METHODS The authors inclusion criteria included patients who were diagnosed with NF1, fulfilled the NIH Consensus Development Conference NF1 diagnostic criteria, had more than 100 neurofibromas of varying sizes, and were treated with CO2 laser treatment over the last 4 years. Seven NF1 patients satisfied these inclusion criteria and were included. Informed consent was obtained from five patients (71% response rate) for a post-treatment telephone survey. All patients underwent test patches with the CO2 laser and were reviewed at 36 months when the results were assessed. If satisfactory, patients were then invited for CO2 laser treatment under general anesthetic. The Sharplan 40 C Silktouch laser (Laser Industries Ltd., Tel Aviv, Israel) was used in its freehand continuous mode (1020 W with rapid side-to-side hand movements) or in scanner settings (125 mm hand piece, 714 W, 3 mm spot) to ablate the small neurofibromas. Peripheries of larger lesions were first scored with the continuous laser beam. The incision was then extended as the neurofibroma was held with forceps and the entire dumbbell-shaped lesion was excised using the laser. Bleeding was rarely encountered and was easily controlled using the defocused laser beam or Vicryl (Johnson & Johnson, NJ, USA). Postoperatively, antibacterial ointment (mupirocin, Bactroban ; Smithkline Beecham Corporation, Philadelphia, PA, USA) was applied under hydrocolloid dressings as the laser wounds healed with secondary intention. Wounds that were considered large enough for delayed secondary intention healing were sutured with Vicryl. All patients were followed up 3 and 6 months later to review the wound, scarring, and cosmetic outcome of treated neurofibroma lesions. Five patients completed the post-treatment telephone survey, which was conducted by the same doctor. Patients were asked to rate their satisfaction of CO2 laser treatment (score 010) and whether they would recommend CO2 laser treatment to other patients. Retrospective data on CO2 laser treatments, follow-up, and recurrence following treatment was obtained. Clinical Outcomes All patients had more than 100 neurofibromas of varying sizes. The mean age of patients was 45.2 (age range 3656) with a male/female ratio of 3:2. Four patients had truncal neurofibromas and one had treatment for neurofibromas on facial, neck, and upper limb skin. Patients received a mean of 2.2 treatment sessions (range 14). The mean number of lesions per patient was 114 (range 20200 lesions). The mean follow-up period was 14.4 months (range 624 months). Three patients (60%) had no lesional recurrence 2 years post-CO2 laser treatment. Two patients (40%) had recurrence of a few of the treated lesions (B10% of treated lesions per patient). Preoperative and postoperative photos are shown in Figs. 1, 2 and 3. Healing took 34 weeks. The wounds were erythematous for approximately 36 months, and matured into hypopigmented and sometimes atrophic scars. There were no significant complications. All five patients reported that treatment-induced scarring was cosmetically preferable to neurofibroma lesions. Hypopigmentation or depigmentation at treatment sites were the only reported adverse effects. Patient Satisfaction The mean patient satisfaction score was 9.2 out of 10 (range 810). All five patients (100%) commented that they would recommend CO2 laser treatment to other patients with neurofibromas. Surgical excision is the standard procedure for removal of lesions, with advantages of a neat Fig. 1 Patient 1. Before (a) and 6 months after (b) excision of neurofibromas with the CO2 laser Fig. 2 Patient 2. Before (a) and 6 months after (b) excision of neurofibromas with the CO2 laser linear scar and a specimen for histology. The disadvantages are a longer procedure time and higher risk of bleeding (...truncated)