Tumescent local anaesthesia for the surgical therapy of congenital nevi in the first year of life.

BACKGROUND: The indication for surgical treatment of congenital nevi must be made after individual consideration of the expected benefit and risk and requires careful information of the parents as well as interdisciplinary psychological support. In addition to suspected malignancy, a relevant indication is the risk of stigmatization depending on the size and localization of the nevus. Objective was to show which size reduction of the congenital nevus can be achieved by surgical therapy under tumescent local anaesthesia (TLA) in infancy and how often complications of anaesthesia or surgery occur. METHODS: All infants up to 12 months of age who underwent surgery for a congenital nevus under TLA at the University Dermatological Clinic Tuebingen between January 2015 and December 2021 were included; surgeries were performed using serial excisions (powerstretching technique), whereby the incisions were made inside the nevus and mobilization was strictly limited to the skin side without nevus. RESULTS: Overall, a cumulative total area of 38.65 cm2 could be removed on average within the first year of life (trunk: 67 cm2 , head: 21.2 cm2 , legs: 21.6 cm2 , arms: 13.2 cm2 ). A cumulative maximum area reduction of 406.9 cm2 could be achieved. We evaluated 363 surgical sites (123 children) on all body regions. The median age of the children at the first surgery was 3.5 months (0.46-10.7 months). Complications occurred in 2.3% (seven procedures). All these complications were reversible in the course of the operation and did not lead to a prolonged hospital stay. No anaesthesia-related complications occurred. CONCLUSION: We were able to show that a reduction of large areas of congenital nevi is possible in the first year of life with the combination of serial excisions using powerstretching technique, TLA, and intracutaneous butterfly sutures.

[Naevus unius lateris: a confusing entity (a case report)]. / Nævus unius lateris: un nævus déroutant (à propos d´un cas).

Naevus unius lateris is a rare congenital hamartoma, originating from the ectoderm. It is considered as a verruquous variant of the epidermal naevus. Given its extensive unilateral distribution, it is frequently associated with neurological, musculoskeletal, auditory and visual abnormalities. We here report the case of a 9-year-old child with nevus unius lateris associated with neurological and eye abnormalities.

Lower Extremity Reconstruction in the Pediatric Population.

Indications for lower extremity reconstruction in children are unique because most result from congenital conditions (eg, constriction ring, lymphedema, syndactyly, nevi, vascular anomalies). Like adults, pediatric patients also suffer from effects following extirpation and trauma. Principles of reconstruction are based on the condition and type of deformity. The pediatric population typically has fewer comorbidities than adults that can negatively affect outcomes (eg, diabetes, peripheral vascular disease), although children can be less compliant with postoperative care. Growth, development, appearance, and postoperative compliance are variables that especially influence operative management of children.

A study of dermal melanophages in childhood nevi. Reassessing so-called "pigment incontinence".

In inflammatory dermatoses, dermal melanophages (MLP) are ascribed to "pigment incontinence," with melanin "dropping down" from the epidermis. Although this is analogous to the "dropping down" of melanocytic nevus cells (Abtropfung), MLP in ordinary nevi have not been systematically studied-so "pigment incontinence" may not apply to MLP in nevi. A total of 31 childhood nevi identified by pediatricians and family practitioners were evaluated for the distribution of MLP. We tested the hypothesis that a dermal origin of the melanin in MLP is more likely than dropping down from the epidermis. In our cohort, 90.3% (28/31) of childhood nevi had dermal MLP, a significantly higher frequency, compared to 31/60 ordinary adult nevi (P < 0.0001). Superficial dermis was the most common location (P < 0.001). However, only six specimens had MLP restricted to the superficial dermis, significantly less than predicted by the theory that melanin drops down from the epidermis (P < 0.00001). We also evaluated perivascular MLP, since nerves run together with vessels in neurovascular bundles (NVB), and it has been showed that precursors of melanocytes migrate from the neural crest to the skin as nerve sheath stem cells. Superficial NVB MLP correlated with deep NVB bundle MLP (P < 0.05), suggesting that NVB MLP represent "tombstones" for superficial and deep dermal nevus cells. Deep dermal, deep NVB, and deep periadnexal MLP may be valid biological criteria for diagnosis of congenital type (prenatal) nevi. Viewing prenatal nevi in children as a neurocristopathy fits a major principle of pediatric pathology: childhood diseases should be studied and understood based on what happens during tissue development.

TERT and TERT promoter in melanocytic neoplasms: Current concepts in pathogenesis, diagnosis, and prognosis.

BACKGROUND AND OBJECTIVE: Located on chromosome locus 5p15.33, telomerase reverse transcriptase (TERT or hTERT) encodes the catalytic subunit of telomerase which permits lengthening and preservation of telomeres following mitosis. Mutations in TERT promoter (TERT-p) upregulate expression of TERT, allowing survival of malignant cells and tumor progression in wide variety of malignancies including melanoma. The objective of this review is to examine the roles of TERT and TERT-p in the pathogenesis, diagnosis, and prognostication of cutaneous melanoma. METHODS: All studies of TERT or TERT-p in cutaneous melanocytic neoplasms with the following inclusion criteria were reviewed: publication date between 2010 and 2019, English language, and series of ≥3 cases were reviewed for evidence supporting the role of TERT in pathogenesis, diagnosis, and prognosis. Studies with <3 cases or focused primarily on mucosal or uveal melanocytic tumors were excluded. RESULTS AND CONCLUSION: TERT-p mutations are frequent in chronic and non-chronic sun damage melanoma and correlate with adverse prognosis, inform pathogenesis, and may provide diagnostic support. While TERT-p mutations are uncommon in acral melanoma, TERT copy number gains and gene amplification predict reduced survival. Among atypical spitzoid neoplasms, TERT-p mutations identify biologically aggressive tumors and support the diagnosis of spitzoid melanoma. TERT-p methylation may have prognostic value in pediatric conventional melanoma and drive tumorigenesis in melanoma arising within congenital nevi. Finally, TERT-p mutations may aid in the differentiation of recurrent nevi from recurrent melanoma.

Choosing the right rectangular expander and maximising the benefits from expanded tissue.

OBJECTIVE: Tissue expanders are widely used in the reconstruction of tissue wounds. This study aims to demonstrate how to choose the correct size of rectangular expander in relation to wound size and the maximum advancement technique for wound coverage in order to achieve a successful outcome. METHODS: The present study included patients who were operated on between January 2013 and January 2017. The expander height chosen was more than half the length of the wound and the expander width was chosen to be as wide as possible, based on the width of the wound. The expander was placed in a site adjacent to the wound. Maximum advancement method was used to achieve coverage of the wound. RESULTS: A total of 19 patients were included in the study, mean age 17.5 (range: 11-25) years. Indication included burn scar (n=14) and congenital nevus (n=5). The tissue expander was inserted into the scalp in 17 patients and supraclavicular area in two patients. A successful wound repair was achieved with the planned flaps in all patients. CONCLUSION: In expansion using rectangular expanders, the required expanded skin is gained through the height of the expanded tissue. Thus, expander size should be preoperatively planned to ensure the height of expanded tissue would be, at least, half of the wound length. Maximum benefit will be achieved from the expanded tissue through the correct placement of expanded tissue lateral flaps.

Genetic variant in the NSDHL gene in a cat with multiple congenital lesions resembling inflammatory linear verrucous epidermal nevi.

BACKGROUND: The feline counterpart of human inflammatory linear verrucous epidermal nevus (ILVEN) has been described; however, the possible underlying developmental defect has not been investigated. OBJECTIVE: To report a case of multiple ILVEN-like lesions in a cat with a genetic variant in the NSDHL gene. ANIMALS: A 2-year-old, female, domestic short hair cat with a history of multiple alopecic, verrucous, hyperpigmented and erythematous skin lesions, following Blaschko's lines on the head, the limbs, the trunk and paw pads. METHODS AND RESULTS: According to the clinical and histopathological findings, a diagnosis of multiple ILVEN-like lesions was made. Genetic investigation revealed a heterozygous missense variant in the X-chromosomal NSDHL gene predicted to lead to a loss-of-function of the NSDHL protein. CONCLUSIONS AND CLINICAL IMPORTANCE: To the best of the authors' knowledge, this is the first case of feline ILVEN-like lesions in which a genetic cause has been proposed. Future studies to establish a causal relationship between NSDHL variants and skin lesions might lead to pathogenesis-directed treatments.

Congenital nevus comedonicus complicated by a hidradenitis suppurativa-like lesion: Report of a childhood case.

Nevus comedonicus is a rare, benign hamartoma of the pilosebaceous unit that may be isolated or part of the nevus comedonicus syndrome. Although rare in children, complication by hidradenitis suppurativa-like lesions has been described. We present a report of a 9-year-old girl in whom surgical excision was curative, with a review of the relevant literature.

Functional and aesthetic reconstruction of a large upper lip defect using combined three local flaps: A case report.

RATIONALE: A significant clinical issue for treating patients with large upper lip defects is how to reconstruct the lip functionally and aesthetically. Traditional methods usually lead to asymmetry of the nasal base, philtrum and the lips. PATIENT CONCERNS: A 22-year-old lady presented with a large congenital nevus on her upper lip which involved the cutaneous, vermilion, and the philtrum. Secondary deformity caused by previous partial excisions was also identified. DIAGNOSES: Patient was diagnosed as upper lip nevus with secondary deformity after partial excisions. INTERVENTIONS: We repaired the large upper lip defect by using combined nasolabial rotation flap and orbicularis oris myocutaneous flap. An additional small piece of mucosal flap was used to lengthen the vermilion. OUTCOMES: After the surgery, patient with large upper lip defects achieved satisfactory cosmetic and functional repair. LESSONS: Reconstruction of the upper lip has been successfully accomplished through the use of combined nasolabial rotation flap, orbicularis oris myocutaneous flap, and a small piece of mucosal flap.

Dermatologic surgery in children: an update on indication, anesthesia, analgesia and potential perioperative complications.

BACKGROUND: Children undergoing dermatosurgical procedures require, unlike adults, particular attention; the administration of various analgesics, anesthetics or sedatives requires a thorough knowledge of drug pharmacokinetics and pharmacodynamics. Furthermore, there are concerns that drugs used for sedation/general anesthesia may result in anesthetic/analgesic complications in children undergoing surgery, with a risk of impaired mental development. OBJECTIVES: Based on our clinical experience and a literature review, we illustrate the most commonly used analgesic, anesthetic and sedative drugs in pediatric dermatosurgery, and identify risk factors and complications following dermatosurgical procedures. RESULTS: Topical anesthetics can be used in children for superficial dermatologic procedures or prior to infiltration anesthesia. Maximum recommended doses based on body weight should be calculated in order to avoid overdosage of local anesthetics. General anesthesia in dermatosurgery is considered safe and has a low rate of side effects. However, caution is advised in children under the age of one due to potential long-term neurological side-effects. NSAIDs and opioids play a significant role in analgesia for children. CONCLUSIONS: This article reviews currently available data on analgesia, anesthesia and complications that may arise in pediatric dermatosurgery. These data may be useful in optimizing the safety and quality of care and in improving parent counseling.

Amelanotic naevoid melanoma in a 16-month-old albino infant.

Melanoma in individuals with oculocutaneous albinism has been reported in the literature to be rare compared with the more common occurrence of squamous cell carcinoma and basal cell carcinoma. We present a singular case of amelanotic naevoid melanoma arising from a small congenital naevus in a 16-month old albino boy, the youngest reported to date.

[Frontal and scalp expanded skin flap combined with laser hair removal for children congenital facial giant nevi].

Objective: To investigate the feasibility and therapeutic effect of frontal and scalp expanded skin flap combined with laser hair removal for children congenital facial giant nevi. Methods: From January 2013 to December 2015,6 cases with congenital facial giant nevi were treated with expanded frontal and scalp skin flap combined with laser hair removal in the department of plastic surgery, An Zhen Hospital, Capital Medical University. The process of treatment could be divided into three stages. Stage 1:The expander capacity was determined according to the size of lesion. Expander was implanted under the galea aponeurotica in front and scalp, and expanded regularly postoperatively. Stage 2:The expander was removed and facial giant nevi was excised. The expanded skin flap was designed according to the shape and size of nevi. Stage 3:After the expanded skin flap healed completely, laser hair removal was performed for 4-6 times with 6 weeks of interval time. Results: 300 ml to 350 ml expanders were selected with expanding frequency of 2 times a week. The expansion time lasted for 16 to 20 weeks(average,18.7 weeks).The complications such as leakage, angle and skin flap blood supply obstacle didnt occurr in the process of expansion. The size of frontal and scalp skin flap ranged from 12 cm × 11 cm to 20 cm × 17 cm. Venous drainage disorder happened in one flap and was treated by partial suture removal and acupuncturebleeding treatment. Skin flap necrosis didn't occurred. The defects at donor sites were sutured directly with inconspicious scar. The effect of semiconductor laser hair removal was good without recurrence during oneyear follow-up period. All expanded skin flaps had no obvious contraction with good match of color and texture. Conclusions: This method provides a new selection of donor site for larger facial defect with reliable effect. The facial giant nevi could be excised completely and repaired in one operation with short operation time. The scar in middle face and secondary deformities can be avoided.

Vascular Stains: Proposal for a Clinical Classification to Improve Diagnosis and Management.

Vascular stains are a common reason for consultation in pediatric dermatology clinics. Although vascular stains include all vascular malformations, the term is most often used to refer to capillary malformations, but capillary malformations include a wide range of vascular stains with different clinical features, prognoses, and associated findings. The discovery of several mutations in various capillary malformations and associated syndromes has reinforced these differences, but clinical recognition of these different types of capillary vascular stains is sometimes difficult, and the multitude of classifications and confusing nomenclature often hamper the correct diagnosis and management. From our own experience and a review of the most relevant literature on this topic, we propose categorizing patients with capillary vascular stains into seven major clinical patterns: nevus simplex, port-wine stain, reticulated capillary malformation, geographic capillary malformation, capillary malformation-arteriovenous malformation (CM-AVM), cutis marmorata telangiectatica congenita, and telangiectasia. We also discuss the differential diagnosis of vascular stains as well as other conditions that can closely resemble capillary malformations and thus may potentially be misdiagnosed.