[Orbital congenital nevi: Principles of treatment about 51 cases]. / Nævi orbitaires congénitaux: principes de prise en charge thérapeutique à propos de 51 cas.

INTRODUCTION: The incidence of congenital nevi is one over 20,000 newborns per year, 14 % of them are located in the head and neck area. Nevi of the orbital region are particularly difficult to handle on the aesthetic and functional side. The objectives of this study were to conduct an analysis of different clinical presentations of congenital nevi of the eyelid orbital region in children to establish a treatment algorithm. MATERIEL AND METHODS: We realised a bi-centric retrospective study including 51 children with orbito-palpebral congenital nevi. We analysed the different clinical presentations, their treatments and their results. RESULTS: Nineteen underwent direct suture excision; three a total skin graft; 15 a combination of treatments, among them four underwent tissular expansion and 14 patients were not operated and clinically followed-up. The average follow-up time was 6.6 years. In 33 cases residual nevic area was still present. The postoperative sequelae were: dyschromia (n=17), anatomical deformation of the eye (n=10), nevi outbreaks (n=8), internal canthus deformation (n=5) and ectropion (n=1). CONCLUSION: The results of our study show that therapeutic abstention is preferred when the aesthetic wrong is accepted by the patient and when there is not a higher risk of malignant degeneration. In order to minimise the risk of postoperative sequelae, we propose a therapeutic algorithm for the management of congenital orbital nevi.

[Melanotic neurofibroma]. / Neurofibrome mélanotique.

BACKGROUND: Melanotic neurofibromas are rare tumours. The clinical and histological diagnosis is often difficult to make. CASE REPORT: A 41 year-old woman with type-1 neurofibromatosis presented with an old, large (16 cm by 6 cm) pigmented tumour on her left arm. It was initially considered to be a congenital naevus. Partial surgical resection was performed. Histological examination showed a loose proliferation of spindle-cells within the dermis and subcutaneous layers, with multiple foci of melanin-laden cells but no mitotic figures or atypical cells. There was no melanocytic theca. The tumour had immunoreactivity for the S-100 protein, neuron-specific-enolase, neurofilaments, synaptophysin, A-103 and HMB-45. The association of a benign pigmented tumour producing melanin and the presence of Schwann cells and nervous cells, led to the diagnosis of diffuse melanotic neurofibroma. DISCUSSION: Melanotic neurofibromas can occur on their own or be associated with neurofibromatosis. They must be distinguished from classical neurofibromas when pigmentation occurs in the latter. Melanotic neurofibromas usually appear in the second or third decade of life and rarely in childhood. It is worth noting that hairs may overlie a melanotic neurofibroma, mimicking a giant naevus or a neurocristic cutaneous hamartoma. These are the two main differential diagnoses among children. Among adults, the main difficulty is to distinguish melanotic neurofibroma from pigmented dermatofibrosarcoma, because of the clinical and histological similarities between these two.

[The importance of early surgery in preventing the esthetic sequelae of cervicofacial mucocutaneous hemangiomas in children]. / Intérêt de la chirurgie précoce dans la prévention des séquelles esthétiques des hémangiomes cutanéo-muqueux cervico-faciaux de l'enfant.

Infants with cutaneous hemangiomas are classically managed medically, well-defined surgery being planned for esthetic correction at the age of 8 to 10 years. We present a series of 65 cases of early surgery in children with head and neck cutaneous or mucosal hemangiomas where irreversible and unesthetic scars were predictable. The surgical procedure was simple and the cosmetic result was better than could be expected after late surgery, limiting psychological consequences. In our opinion, the abstention rule should be changed. A multidisciplinary check-up at 2 years to identify cases with a predictably unesthetic scar after complete resolution of the angioma would help select cases where early surgical correction, taking advantage of the exceptional quality of skin in these young children, would be most beneficial.

ICAM-3 and E-selectin endothelial cell expression differentiate two phases of angiogenesis in infantile hemangiomas.

Cellular adhesion molecules are newly identified mediators of angiogenesis. Infantile hemangiomas, characterized in the early stages by a proliferation of poorly differentiated vessels followed in the late stages by a vascular differentiation and regression of the tumor, represent an interesting model to study angiogenesis. We studied by immunohistochemistry the distribution of HLA-DR and three adhesion molecules ICAM-3, E-selectin and VCAM-1 on endothelial cells in different stages of vessel differentiation in infantile hemangiomas. We found high levels of ICAM-3 expression on proliferating vessels, while its expression was low or undetectable on well differentiated vessels. A different set of E-selectin antibodies showed a more heterogenous pattern of distribution and VCAM-1 antigens were found in both proliferating and differentiated vessels. HLA-DR expression on endothelial cells was inversely correlated to the vascular differentiation. Our results are consistent with the hypothesis that ICAM-3 plays a role in the early stages of vessel formation. Our results also suggest that variation of E-selectin and HLA-DR expression may be related either to vessel differentiation or may reflect the acquisition of an activated endothelial cell status.

Description of cutaneous excision and suture using a mathematical model.

Mathematical models are being developed at a fast rate in industry thanks to computer technology; they are used to simulate motion and deformation over time to test materials and objects in a virtual manner. These modeling techniques are being developed in medicine, but they remain, at this time, in the domain of biomechanical research. We report a mathematical model for cutaneous excision and suture, which we have used to predict the deformations and tensions that result when using four different forms of excision. The results are expressed in numerical and graphic form. The results obtained corresponded with our experience in dermatologic surgery. Uses and limits of this model are discussed.

Advantage of the presence of living dermal fibroblasts within in vitro reconstructed skin for grafting in humans.

Methods for serial cultivation of human keratinocytes can provide large quantities of epidermal cells, which have the potential of restoring the vital barrier function of the epidermis in extensive skin defects such as burns. To investigate the value of combining an epidermis with a dermal component, fibroblasts originated from the superficial dermis were used to seed a collagen lattice as described by E. Bell (dermal equivalent). Beginning in 1981, we grafted 18 patients (burns and giant nevi) using 35 grafts 10 x 10 cm in size. In the course of this work, the original technique was modified and improved as experience was gained. We began by using small skin biopsy samples as a source of keratinocytes cultured on a dermal equivalent before grafting in a one-step procedure, but this gave poor cosmetic results, because of a nonhomogeneous epidermalization. We then chose to cover the graft bed using a two-step procedure. The first step consisted of grafting a dermal equivalent to provide a dermal fibroblast-seeded substrate for subsequent in vivo epidermalization by cultured epidermal sheets. Whatever the epidermalization technique used, a living dermal equivalent applied to the graft bed was found to reduce pain, to provide good hemostasis, and to improve the mechanical and cosmetic properties of the graft. A normal undulating dermal-epidermal junction reappeared by 3 to 4 months after grafting and elastic fibers were detectable 6 to 9 months after grafting. As a result of the biosynthesis of these products, the suppleness (e.g., elasticity) of the grafts was closer to that of normal skin than the cicatricial skin usually obtained with epidermal sheets grafted without the presence of living dermal cells. This rapid improvement of the mechanical properties of the graft could be attributed to the presence of fibroblasts cultured from the dermis and seeded into the collagen matrix.

[Congenital giant blue nevus. Apropos of a case]. / Naevus bleu congénital géant. A propos d'un cas.

A case of a three years old girl showing a congenital giant blue nevus, in the neck and the occipital region, is presented. The giant blue nevus was associated to an important cutaneous nevomatosis. The treatment has consisted of an surgical excision of an surgical excision of the main lesion, and operative continuations have been simple. The literature study showed 11 cases of congenital giant blue nevus and demonstrate the high risk of malignancy, and the importance of an early surgical treatment.

Staphylococcal necrotizing fasciitis in the mammary region in childhood: a report of five cases.

OBJECTIVE: Necrotizing fasciitis is a highly lethal soft tissue infection rarely reported in childhood. The initiating site is usually a local trauma or a surgical wound. We observed five cases of necrotizing fasciitis the initiating site for which was the mammary region and discuss their management. STUDY DESIGN: We describe these five patients and review the clinical characteristics of their presentation. RESULTS: Staphylococcal necrotizing fasciitis was observed in the mammary region in all five cases. Four children were newborn infants with a mammitis preceding the onset of necrotizing fasciitis. Surgical débridement was done only after the fourth day from onset of illness. All children were discharged in good condition after 1 month. Two have been followed until puberty, with destruction of the mammary gland in one case and good development in the other one. CONCLUSION: Mammitis may be the initiating event for necrotizing fasciitis in neonates. Necrotizing fasciitis is a life-threatening disease; patients require early intensive care, parenteral antibiotic therapy, and surgical débridement. In a few instances surgery can be carefully delayed until the necrotic area is more delineated if the condition is diagnosed early during disease evolution and appropriate treatment is instituted in intensive care units.

Surgical treatment of large cutaneous lesions of the back in children by concentric cutaneous mobilization.

Excision of large cutaneous lesions of the back, such as giant nevi in children, represents a major surgical challenge. Actually, many authors use tissue expanders. Recently, we have proposed, through a better evaluation of the biomechanical properties of the skin, a natural expansion mode using the postoperative tension. This expansion is natural and delayed. In the back the natural expansion can be created by concentric excision, which takes maximum advantage of the possibilities of cutaneous mobilization of the back. The concentric excision has been used in 12 children presenting with cutaneous lesions of the back involving more than 30 percent of the dorsal anatomic unit. Nine patients underwent total excision; two patients were treated in one stage, four patients in two stages, and three patients in three stages. Three patients could not have total excision. The follow-up was uneventful, except in one patient who presented with wound dehiscence. The follow-up was 1 to 2 years and showed a modest scar widening (< 4 mm) in three patients, moderate widening (> 4 min, < 7 mm) in three patients, and significant widening (> 10 mm) in three patients. The systematization of the cutaneous mobilization for each region of the back has allowed us to optimize the utilization of peripheral normal tissue in the surgical treatment of large cutaneous lesions of the back. In some circumstances, the gain in tissue has been superior to that we usually obtain with skin expanders and with lesser cost, lesser pain, and fewer risks.

[Complex facial nevus in children treated by combination of prosthetic and deferred natural expansions]. / Naevus complexe facial de l'enfant traité par combinaison des expansions prothétique et naturelle différée.

Congenital pigmented naevi of the face in children represent indications for early excision and the search for the best aesthetic result has led the authors to prefer the use of skin expansion techniques. The case presented here illustrates the combined use of two expansion techniques: prosthetic expansion and natural differed expansion. This procedure gives good cosmetic results at a moderate overall cost.

Dog ear: true and false. A simple surgical management.

The rise of the utilization of tissue expanders and procedures like liposuction have brought us a better understanding of the biomechanical properties of the skin as a living organ. This understanding has helped us to appreciate better the etiopathology of a dog ear deformity and to utilize the same properties for its correction by simple methods and thus avoid complex plasties and additional scars.

[Use of deformities in the treatment of facial skin defects in children: "deforming resections". Report of 35 cases]. / L'utilisation des déformations dans le traitement des pertes de substance cutanées faciales de l'enfant: "les exérèses déformantes". A propos de 35 cas.

Spontaneous resolution of deformities after excision of facial skin lesions has been known and used for a long time by plastic surgeons. The resorption mechanism of deformity is based on natural skin expansion, and seems to be directly related to the action of the muscles of facial expression and their skin relations. Natural expansion has been shown to be effective in children. Between 1990 and 1994 excision of skin lesions, including congenital nevi, leading to postoperative deformity of the cheek, labial commissure, nose, eyelid and forehead, was performed on 35 patients, aged from 3 month to 12 years. The average follow-up is 24 months. For 26 patients (74%), natural resorption was observed 4 or 6 weeks later with good aesthetic results. For 6 patients (17%), the deformity persisted 3 or 6 months later, but did not require any further surgery. For 3 patients (9%), a second operation was necessary. Using the skins natural capacity for expansion in the treatment of facial skin defects in children is a method of reconstruction which has already been used for excision in enforced position. The platysma and muscles of facial expression by their action on skin mobilisation, allow natural expansion. A better knowledge of cutaneous biomechanical properties enables plastic surgeons to find an alternative to other classical methods.

[Deferred natural expansion]. / L'expansion naturelle différée.

Adaptation of the skin to the oriented and directed tension stresses can allow, under certain specific conditions, considerable expansion for the subsequent cover of large skin defects in children and adults. This paper describes the concept of natural deferred expansion, illustrated by two clinical examples. Its theoretical bases, as well as a computerized biological model are discussed. A classification of the various types of cutaneous mobility and the biomechanical properties of skin is proposed.

Antagonism between estradiol and progestin on Bcl-2 expression in breast-cancer cells.

Bcl-2 is a key protein involved in the control of apoptosis. Our previous studies on breast and endometrium indicated hormonal regulation of bcl-2 in these tissues. In the present work we have analyzed Bcl-2 and Bax protein expressions in MCF-7 and T47-D, 2 hormone-dependent breast-cancer cell lines, by immunoblots. Estradiol markedly increased Bcl-2 protein content, both in short- and in long-term treatments of MCF-7 cells. Two types of anti-estrogens (4-hydroxytamoxifen and RU 58668) were able to reverse this effect. Also, a synthetic progestin (ORG 2058) was able to decrease the Bcl-2 level in T47-D cells. The level of Bax protein, however, was not affected in the same conditions of hormonal treatments. The level of Bcl-2 expression was 4.5-fold higher in MCF-7 than in MDA-MB 231 (an estradiol-independent cell line). From these results, we infer the existence of hormonal regulation of Bcl-2 expression and evoke a novel role for estradiol and progestin in the genesis of breast cancer.

[Natural skin expansion. Applications to the surgery of nevus of the head and neck in children]. / Expansion cutanée naturelle différée. Applications à la chirurgie naevique cervico-céphalique de l'enfant.

The surgical treatment of congenital naevi of the head and neck often require the use of expansion prostheses. The high risk of complications in children such as infection, necrosis, prosthetic exposure, has led the authors to propose a new therapeutic approach, consisting of deferred cutaneous expansion, which uses the skin tension as an expansion motor. This allows repair in one or several surgical phases, of large defect using the skin of the same anatomical unit, with good esthetic results. The authors describe the three main techniques applied to surgery of naevi of the head and neck: simple excision-suture, excision-unfolding and deforming excision.

[Giant congenital blue nevus of the scalp]. / Naevus blue géant congénital de l'extrémité cervico-céphalique.

INTRODUCTION: Blue nevi are small acquired melanocytic tumors. Two clinical forms are described, a fibrous form and a cellular form. Giant nevi are exceptional. CASE REPORT: We describe a patient with a giant blue nevus of the scalp. In addition to this congenital, noninvasive tumor the patient presented numerous cutaneous melanocytic nevi. The main lesion was removed by surgical exeresis followed later with reconstruction plasty. The histological examination of the surgical specimen showed an infiltrating blue cell nevi. After a 4-year follow-up, there has been no recurrence and no metastasis. DISCUSSION: There have been 11 cases of giant blue nevi reported in the literature. All were congenital lesions with polymorphous clinical and histological aspects. Fibrous forms are noninvasive and cellular forms have a potential for local invasion, whether shortly after birth or later with invasion of muscles, bone and meninges without intracerebral extension. Congenital blue nevi require early surgical exeresis.

Use of distortions in the treatment of skin lesions of the face: the distorting excision.

Reconstruction after excision of a cutaneous lesion of the face is done by various complex old and new flaps to avoid any distortion. The distortions following primary closure of defects in the face are given undue importance. We have observed and used the capacity of self-correction of these distortions, which is partially due to the muscular actions. We show our experience in various distorting reconstructions of the face in 36 patients. Because of our partial presence in a children's hospital, most of our patients were children.

Rapid intraoperative distension using isotonic saline solution.

Rapid intraoperative expansion can provide a solution to certain losses of cutaneous substance. In some cases where this method proves useful, I have been able to obtain interesting results by using distension with saline. I would like to show the importance of this operating technique, as well as its limits and advantages.

Cutaneous expansion using enforced position in the treatment of large skin defects.

This paper presents a new method of reconstruction of a large skin defect, e.g., following excision of a giant pigmented nevus. By taking advantage of the flexibility of the joints and the extensibility of skin in the children, the large defects were closed in one stage by an enforced position that allows an easy closure of the wound. The scar was protected against stretching for 6 weeks to obtain sufficient strength. Later, free movements across the joints were permitted to allow gradual tension to the skin surrounding the scar. The final result is a permanent gain of the tissue replacing the defect.