An 18-year experience in the management of congenital nevomelanocytic nevi

Background: Children with giant congenital nevomelanocytic nevi (CNN) are referred to our pediatric burn center for the surgical management of this disfiguring and potentially malignant skin disorder. Use of tissue expanders has contributed significantly in limiting donor site morbidity associated with treatment of giant CNN. Cultured skin substitutes (CSS) have also shown promise as an alternative wound coverage. With recent controversy regarding the effectiveness of excision in preventing melanoma risk, we wished to review our surgical management of giant CNN and to determine the incidence of malignancy in these patients. Methods: A retrospective chart review of patients with giant CNN was performed from 1985 to 2003. Charts were reviewed for age, sex, percentage total body surface area (TBSA) involved, age at initiation and completion of treatment, surgical treatment, complications, histopathology, and length of follow-up. Results: Of the 40 patients treated at our facility, the mean extent of skin involvement was 10% TBSA (range: 0.5%-75%). The mean age at initial operation was 5.1 years, and the majority of surgical interventions were completed within a mean of 1.3 years. Twenty-two patients (55%) required more than I surgical procedure. Excision and split-thickness skin grafting was the most common surgical procedure (n = 22) followed by excision with primary closure (n = 18). Ten patients were treated with tissue expansion, while 4 received cultured skin replacements. One patient died of extracutaneous melanoma during the course of surgical treatment. Three patients demonstrated histopathologic evidence of cytoatypia but remained clinically free of malignancy during a mean follow-up of 11 years. Conclusions: Giant CNN are both important cosmetic and medical problems. With an associated lifetime risk of melanoma in 4%-10% of patients, excision of CNN is recommended despite the fact that 50% of melanomas arise extracutaneously. Depending on the extent of body surface area involvement, wound closure can be obtained with conventional split- or full-thickness skin grafts, tissue expansion, and/or cultured autologous cultured skin substitutes. The latter 2 modalities provide improved cosmetic results, with minimal donor site morbidity.