Superficial Temporal Artery Island Flap Combined With Laser Hair Removal for Inadequate Skin Expansion Following Tissue Expansion: A Case Report of Congenital Melanocytic Nevus of the Forehead in an Adult.

Tissue expansion is a handy reconstructive technique for the head and neck region; however, its implementation requires careful planning and surgical experience. If tissue expansion is inadequate, forced closure results in wound tension and risks complications, such as postoperative deformity, wide scarring, and wound dehiscence. We report a case of adult forehead melanocytic nevus excision using a tissue expander (TE) where complications caused by insufficient tissue expansion were avoided by creating a flap using a dog ear. The patient was a male in his 20s who underwent surgery with a TE for a congenital melanocytic nevus sized 15 × 10 cm on the left forehead. Resection was performed by tissue expansion using two TEs; however, simple advancement flaps led to excessive wound tension, risk of elevation of the eyebrow on the affected side, and postoperative scarring. Hence, a superficial temporal artery fasciocutaneous island flap with left superficial temporal vessels as a pedicle was raised at the dog ear and moved to the site of strong tension, and the wound was closed without difficulty. Although postoperative laser hair removal was required, both the appearance and functional results were satisfactory. Using anatomical flaps obtained from the surroundings during tissue expansion helps avoid complications associated with forced wound closure.

Melanoma on congenital melanocytic nevi.

Among nevus-associated melanomas, which overall account for 20%-30% of all melanomas, those arising specifically in congenital melanocytic nevi are infrequent, but can be disproportionately frequent in childhood and adolescence. Congenital melanocytic nevi (CMNi) are common benign melanocytic tumors that are present at birth or become apparent in early childhood. They are classified based on the projected adult size. Small and medium-sized CMNi are frequent, whereas large/giant CMNi (over 20 cm in diameter) are rare, but can be associated with high morbidity due to marked aesthetic impairment and the risk of neurocutaneous syndrome or melanoma development. In this setting, melanomas can appear in early childhood and are very aggressive, while the risk of small-medium CMNi of developing melanoma is low and similar to non-congenital melanocytic nevi. Histologically, most melanomas on CMNi initiate their growth at the epidermal-dermal junction, but in large/giant CMNi they can develop entirely in the dermis, in deeper tissues, or in extracutaneous sites (especially in the central nervous system). Most CMNi harbour an NRAS mutation, but other genes are rarely involved, and gene translocations have recently been described. However, no prognostic implications have been associated with the CMN genotype. Melanomas developed on CMNi harbour additional molecular alterations to which the aggressive clinical course of these tumors has been attributed. This review covers the distinctive clinical and pathological aspects of melanomas on CMNi, and includes the epidemiology, etiopathogenesis, clinical and dermoscopic presentation, histological and molecular characteristics, as well as tumour behaviour.

Erbium: YAG laser treatment efficacy and association with histologic features for giant congenital melanocytic nevi management.

BACKGROUND: Limited research exists on laser treatment of giant congenital melanocytic nevus (GCMN). OBJECTIVE: We sought to elucidate the efficacy of the Erbium: YAG laser on GCMN and the histologic factors associated with a positive clinical response. METHODS AND MATERIALS: Between 2019 and 2022, we enrolled 30 medium-to-giant CMN patients who underwent Er: YAG laser treatment. All patients received biopsies before and after laser treatments. Clinical efficacy outcomes were evaluated by the investigator's global assessment (IGA), 5-point scale of depigmentation, and Vancouver Scar Scale (VSS) scores at least 6 months after treatment. RESULTS: Of the 30 cases, 18 (60.0%) showed improvement (IGA score ≥3). Eight (26.7%) patients showed repigmentation. Eight (26.7%) patients developed hypertrophic scars. The average IGA, depigmentation, and VSS scores were 2.93, 3.57, and 3.20. The IGA score was higher (3.24 ± 1.18 vs. 2.22 ± 0.97, p = 0.031) and a lower repigmentation rate (14.3% vs. 55.6%, p = 0.032) was observed in the cases with Grenz zone. The IGA score was higher (3.33 ± 1.24 vs. 2.13 ± 0.89, p = 0.023) and the repigmentation rate was lower (11.1% vs. 50.0%, p = 0.034) also in the cases with the melanocytes nests with aggregation of melanin. Lesions with superficial ablation resulted in less hypertrophic scar formation than those with deep ablation (5.9% vs. 53.8%, p < 0.05). CONCLUSION: The Er: YAG laser demonstrated effective clinical results for GCMNs. The grenz zone and the melanocytes nests with aggregation of melanin are promising predictors of laser efficacy.

Updates in the Management of Congenital Melanocytic Nevi.

Congenital melanocytic nevi (CMN) carry an increased risk of melanoma and may be disfiguring, and consensus regarding treatment recommendations is lacking. While clinical monitoring is the standard of care, many caregivers are interested in its removal to prevent psychosocial burden or to decrease risk. Although melanoma can occur regardless of CMN removal, there are a variety of treatments that may offer improved cosmesis or local symptom control, including surgical excision, laser therapy, and other superficially destructive techniques. Regardless of the selected management, these patients are monitored for ongoing melanoma risk. An extensive discussion with families regarding the risks and benefits of observation versus active intervention is essential. To facilitate these discussions, we herein summarize current CMN management strategies and considerations.

Ganglioneuroma Arising in Congenital Melanocytic Nevus in a Patient with Cardiac Anomalies: A Case Report.

A congenital melanocytic nevus is a benign melanocyte proliferation, that may be complicated by malignant transformation. We are reporting a three-year-old girl, who had a giant congenital melanocytic nevus on her back, that was treated by serial surgical excisions with tissue expander insertion. Histopathological examination confirmed the diagnosis of congenital melanocytic nevus with ganglioneuroma. Out of approximately 250 case reports on congenital melanocytic nevus, we identified only two reports of medium/large congenital melanocytic nevus with cutaneous ganglioneuroma. Due to the potential malignant transformation of congenital melanocytic nevus, reporting the features and characteristics of such rare findings may help in further understanding congenital melanocytic nevus, its associations, and prognosis.

Non-multiple medium-sized congenital melanocytic nevi: to excise or to follow up?

Introduction: Congenital melanocytic nevi (CMN) are benign lesions composed of clonal proliferations of melanocytes. Although medium-sized CMN are common and generally remain benign throughout a person's lifetime, they may be precursors of melanoma. There is a limited number of studies focused on the risk of melanoma in solitary, medium-sized, congenital melanocytic nevus; therefore, the incidence of malignant transformation and guidelines for treatment are not well established. Aim: Prompted by the limited data, we conducted this study to gather more information about medium-sized CMN, to optimize clinical care. We share our analysis of surgically removed medium-sized CMN. Material and methods: A total of 10 patients with non-multiple, medium-sized, congenital melanocytic nevus were included in this study. Lesions were removed using surgical procedures. Results: In most of the cases the reason for excision of the medium-sized CMN was evolution of the lesion or aesthetic considerations reported by the patients. In 2 cases, due to the large size of the lesions, serial excisions were performed, while other CMN were removed surgically using simple excision technique. Eight of 10 medium-sized CMN were histologically described as benign, and 2 cases of malignant transformations were reported. Conclusions: According to our clinical experience and knowledge, we recommend managing patients on an individual basis, taking into consideration multiple clinical attributes. In our opinion, long-lasting observation is the management of choice, and if there is need of surgery, we recommend total simple or staged excision depending on nevus size.

Giant Congenital Melanocytic Nevus with Congenital Neurofibroma: A Case Report.

BACKGROUND: Giant congenital melanocytic nevus (GCMN) is characterized by its large size and potential for transformation into melanoma. It can be associated with other neural cristopathies, including neurofibroma, however, it has not previously been described with a congenital neurofibroma. CASE REPORT: A newborn girl presented with a large congenital neurofibroma arising in a bathing trunk type of giant congenital melanocytic nevus. CONCLUSION: Congenital neurofibromas can be associated with (or a component of) a GCMN.

Genetic and phenotypic diversities of nevus spilus phenotypes: Case series and a proposed diagnostic algorithm.

Nevus spilus (NS) is composed of multiple types that characterized by a congenital hyperpigmented patch within variable even superimposed lesions originating from melanocytic lineage cells. The molecular mechanism and classification of diverse NS phenotypes remain unclear. Five children with a phenotype of NS were genotyped by the panel based on next-generation sequencing in this study. DNA from biopsies, blood samples and hair follicle were sequenced to confirm the presence of a somatic mutation. Sequencing results indicated somatic mutation in the gene of NRAS or HRAS in all biopsies from the nevi, and the pathogenic variants were not detected in the samples of blood and hair follicle. This study successfully identified the somatic mutation in five unrelated children with diverse NS phenotypes. Moreover, it provided typical images and differential diagnoses between variable NS phenotypes in clinical, pathological, and genetic features, and first proposed a clinical diagnostic algorithm that contributed to simplifying and optimizing the diagnoses and management of these overlapped diseases.

Ambiguous genitalia, giant congenital melanocytic nevus and subpulmonic outlet ventricular septal defect in an African child with Neurofibromatosis 1.

Neurofibromatosis type 1 is an autosomal dominant multisystemic disease caused by mutation of the neurofibromin (NF1) gene located on chromosome 17q11. We report a case of Neurofibromatosis 1 with ambiguous genitalia, giant congenital melanocytic nevus, and associated subpulmonic outlet ventricular septal defect, hitherto unreported in sub-Saharan Africa. In addition, a literature review of congenital heart diseases associated with Neurofibromatosis 1 is presented.

Impact of public exhibition on the perception of birthmarks.

BACKGROUND: The importance of photographs in social media, the steep rise in popularity of tattoos, and the prominence of individuals with visibly different skin in fashion are likely to be changing the landscape of self- and public perception of birthmarks. Study objectives were to assess the impact of a photoshoot and public exhibition on the self-perception of individuals with extensive birthmarks, and to explore the viewing public's reactions. METHODS: Thirty individuals with congenital melanocytic nevi (CMN) were recruited internationally. Each had a professional photoshoot portrait with their skin exposed, resulting in a public exhibition in London entitled "How do you C Me Now?" Participants/parents completed pre- and post-questionnaires relating to self-perception and the impact of their birthmarks on behavior. Over 8000 members of the public viewed the exhibition, 464 completing an on-site questionnaire on its effects. RESULTS: All participants/parents rated the experience as positive, valuable and helpful. Scores on self-appreciation and self-confidence were significantly higher after the photo shoot. Members of the general public overwhelmingly reported the exhibition increased their positive feelings towards people with birthmarks. The majority of public respondents also reported that the exhibition made them feel better about their own skin and about their looks in general. CONCLUSION: This unique exhibition and the associated research has provided a striking new perspective on potential psychological interventions for individuals with birthmarks.

Dynamic evolution of a scalp congenital melanocytic nevus with poliosis and cutis verticis gyrata.

Cutis verticis gyrata (CVG), characterized by cerebriform overgrowth of the scalp, is rarely observed in congenital melanocytic nevi (CMN). We describe a 13-year-old male with autism and a large CMN of the scalp with numerous satellite nevi whose scalp nevus exhibited evolution with poliosis and CVG. Given the potential association of CVG (independent of CMN) with seizures, neuropsychiatric, and ophthalmologic disorders, and nevus-associated CVG (cerebriform intradermal nevus) with melanoma, multidisciplinary evaluation of CMN patients with CVG is important to guide management and treatment.

Nevo Melanocítico Congénito Gigante con hemangioma hepático asociado. Reporte de caso clínico. Revisión bibliográfica

El nevo melanocítico congénito gigante es una lesión pigmentada de gran tamaño presente al nacimiento. Su incidencia es de 1/1.000-500.000 recién nacidos. La localización más frecuente es el tronco posterior, la cara, el cuero cabelludo y las extremidades. El objetivo que buscamos con la presentación de este caso clínico es ofrecer una revisión actualizada sobre la evaluación al nacimiento, conducta y tratamiento a seguir por los neonatólogos y pediatra de atención primaria ante la inesperada presencia de las dermatopatías no tan frecuentes como la que nos ocupa. Se trata de un recién nacido que nace con un "nevo melanocítico congénito gigante" y que además se asocia con un hemangioma hepático diagnosticado en el ingreso. Valoramos la importancia de esta patología que radica en los problemas impactantes desde el punto de vista estético, quirúrgico y emocional que pueden originar en el paciente y sus familiares, además de la posible asociación con otras malformaciones del sistema nervioso central y en algunos de ellos, el riesgo de ser el origen de un melanoma.

The giant congenital melanocytic nevus is a large pigmented lesion present at birth. Its incidence is 1 / 1,000-500,000 newborns. The most frequent location is the posterior trunk, the face, the scalp and the extremities. We decided with the presentation of this clinical case to offer an updated review on the evaluation at birth, behavior and treatment to be followed by neonatologists, primary care pediatrician before the unexpected presence of dermatopathies not as frequent as the one we are dealing with today, the "nevus" giant congenital melanocytic ". The importance of this pathology lies in the impactful problems from the aesthetic, surgical and emotional point of view that can originate in the patient and their relatives, in addition to the possible association with other malformations of the central nervous system and in some of them, the risk of being the origin of a melanoma.

O nevo melanocítico congênito gigante é uma grande lesão pigmentada presente ao nascimento. Sua incidência é de 1/1.000-500.000 recém-nascidos. A localização mais frequente é o tronco posterior, face, couro cabeludo e extremidades. O objetivo que buscamos com a apresentação deste caso clínico é oferecer uma revisão atualizada sobre a avaliação ao nascimento, comportamento e tratamento a ser acompanhado por neonatologistas e pediatras da atenção primária na presença inesperada de dermatopatias não tão frequentes quanto a em questão. É um recém-nascido nascido com um "nevo melanocítico congênito gigante" e também está associado a um hemangioma hepático diagnosticado na admissão. Valorizamos a importância dessa patologia que reside nos problemas chocantes do ponto de vista estético, cirúrgico e emocional que podem se originar no paciente e em seus familiares, além da possível associação com outras malformações do sistema nervoso central e, em algumas de las, o risco de ser a origem do melanoma.

Association of novel MUC16, MAP3K15 and ABCA1 mutation with giant congenital melanocytic nevus.

BACKGROUND: Giant congenital melanocytic nevus (GCMN) is the benign nevomelanocytic proliferation. Mutations in NRAS have been previously detected in GCMN, but mutations in BRAF are generally lacking in the Chinese population. Mutated genes in this disease can estimate the risk of malignant transformation in GCMN. Therefore, it is worth investigating the genetic information of GCMN. METHODS: Here, we presented two cases of GCMN of the upper extremities. The clinical and histological data were analyzed. The whole exome sequencing (WES) was performed to investigate the mutational profile of peripheral venous blood (PB), normal skin (NS), small melanocytic nevus (SMN), deep penetrating and non-penetrating GCMN (dPGCMN and nPGCMN). RESULTS: We showed a reduction in the circumference of involved upper extremities in both patients. The clinical and histopathological data indicated the reduction of adipose tissue associated with the invasion of GCMN. The WES data revealed that MUC16, MAP3K15 and ABCA1 were novel potential candidate genes for the disease as well as biomarkers for predicting malignant transformation. CONCLUSION: The MUC16, MAP3K15 and ABCA1 may serve as novel biomarkers for predicting malignant transformation and targets for the diagnoses and therapy for the GCMN.

Two-stage skin grafting using a basic fibroblast growth factor-impregnated artificial dermis.

For traditional artificial dermises, a waiting period of approximately three weeks is required after the first implantation before they are adequately vascularized. The objective of this retrospective case series was to investigate whether full-thickness skin defects, requiring surgical reconstruction, could be successfully treated by implantation of a basic fibroblast growth factor (bFGF)-impregnated artificial dermis and secondary skin grafting with a shorter waiting period. Between January 2019 and January 2021, 19 skin defects in 14 patients (7 male and 7 female) were treated with two-stage skin grafting using bFGF-impregnated collagen-gelatin sponge (CGS). All of them were included in this case series, and the waiting period for skin grafting, success rate of skin grafting, infection during the waiting period, and scar quality 6-12 months postoperatively were retrospectively investigated. As a result, all skin grafting surgeries were successfully performed with a waiting period of 13.3 ± 4.3 days. Infection during the waiting period was observed in three lesions (15.8%); however, all infections were controllable. Postoperative scar quality was acceptable (Vancouver Scar Scale score range, 1-8). In conclusion, compared to traditional artificial dermises, bFGF-impregnated CGSs have the potential to shorten the waiting period without decreasing the success rate of skin grafting. Further studies are required to confirm this finding.

Comparison of three different surgical approaches for the treatment of large/giant congenital melanocytic nevus.

OBJECTIVE: Treating large/giant congenital melanocytic nevus (L/GCMN) is challenging for surgeons. Operative approaches commonly used to remove L/GCMN include serial excision, tissue expansion, and skin grafting. Thus, we retrospectively compared these three operations' applications and therapeutic effects. METHODS: The clinical data of 97 L/GCMN patients from June 1, 2015, to June 1, 2019, were collected and divided into three groups according to the operations used: serial excision group (SE group, n = 18), tissue expansion group (TE group, n = 23), and skin grafting group (SG group, n = 56). The location and size of the lesion, the number of operations, duration of each operation, preoperative preparation time, postoperative hospital stay, complications, and clinical outcomes of all patients were collected and assessed. RESULTS: The SE group had the most times of operation (3.9 and 6.0 for LCMN and GCMN, respectively), the shortest surgery length (56.3 min), and the shortest postoperative hospital stay (10.0d). The SE and SG groups required much less time to prepare for surgery and had a lower rate of complications than the TE group. During the 11.9-month median follow-up period, the SE and TE groups had better postoperative outcomes than the SG group. CONCLUSION: Each of the three operations has different advantages and disadvantages, and the specific surgical strategy should be decided based on the patient's unique circumstances.