French updated recommendations in Stage I to III melanoma treatment and management.

As knowledge continues to develop, regular updates are necessary concerning recommendations for practice. The recommendations for the management of melanoma stages I to III were drawn up in 2005. At the request of the Société Française de Dermatologie, they have now been updated using the methodology for recommendations proposed by the Haute Autorité de Santé in France. In practice, the principal recommendations are as follows: for staging, it is recommended that the 7th edition of AJCC be used. The maximum excision margins have been reduced to 2 cm. Regarding adjuvant therapy, the place of interferon has been reduced and no validated emerging medication has yet been identified. Radiotherapy may be considered for patients in Stage III at high risk of relapse. The sentinel lymph node technique remains an option. Initial examination includes routine ultrasound as of Stage II, with other examinations being optional in stages IIC and III. A shorter strict follow-up period (3 years) is recommended for patients, but with greater emphasis on imaging.

[Update to the recommendations for management of melanoma stages I to III]. / Actualisation des recommandations de prise en charge du mélanome stades I à III.

As knowledge continues to develop, regular updates are necessary concerning recommendations for practice. The recommendations for the management of melanoma stages I to III were drawn up in 2005. At the request of the Société Française de Dermatologie, they have now been updated using the methodology for recommendations proposed by the Haute Autorité de Santé. In practice, the principal recommendations are as follows: for staging, it is recommended that the 7th edition of AJCC be used. The maximum excision margins have been reduced to 2cm. Regarding adjuvant therapy, the place of interferon has been reduced and no validated emerging medication has yet been identified. Radiotherapy may be considered for patients in stage III at high risk of relapse. The sentinel lymph node technique remains an option. Initial examination includes routine ultrasound as of stage II, with other examinations being optional in stages IIC and III. A shorter strict follow-up period (3years) is recommended for patients, but with greater emphasis on imaging.

[Advantages of the superior areolar approach for tuberous breast II and III correction with implants]. / Avantages de la voie hémi-aréolaire supérieure pour la correction par implants des seins tubéreux de types II et III.

Tuberous breast is a mammary malformation that remains difficult to treat in most cases. It results from an abnormal development of the mammary base, mainly at the lower pole of the breast. The superficialis fascia is adherent to the dermis and deep plane. For type II and III of Grolleau's classification, we were confronted by some difficulties with inferior areolar approach as in Puckett's or Ribeiro's techniques. We had frequently an insufficient glandular volume at the inferior part of the breast and an early look of a "double bubble" deformity. We propose a technical modification to these procedures using a superior hemiareolar approach to place the implant. Associated with an incision of the deep part of the superficialis fascia, it provides a good-shaped lower pole of the breast by preserving glandular volume at this part and reduces the apparition of "double bubble". We operated on 14 types II-III tuberous breasts on nine female patients and had no significant complication. The mean follow-up is 36 months and the results were very satisfying for the patient and her surgeon.

IRF6 Screening of Syndromic and a priori Non-Syndromic Cleft Lip and Palate Patients: Identification of a New Type of Minor VWS Sign.

Van der Woude syndrome (VWS), caused by dominant IRF6 mutation, is the most common cleft syndrome. In 15% of the patients, lip pits are absent and the phenotype mimics isolated clefts. Therefore, we hypothesized that some of the families classified as having non-syndromic inherited cleft lip and palate could have an IRF6 mutation. We screened in total 170 patients with cleft lip with or without cleft palate (CL/P): 75 were syndromic and 95 were a priori part of multiplex non-syndromic families. A mutation was identified in 62.7 and 3.3% of the patients, respectively. In one of the 95 a priori non-syndromic families with an autosomal dominant inheritance (family B), new insights into the family history revealed the presence, at birth, of lower lip pits in two members and the diagnosis was revised as VWS. A novel lower lip sign was observed in one individual in this family. Interestingly, a similar lower lip sign was also observed in one individual from a 2nd family (family A). This consists of 2 nodules below the lower lip on the external side. In a 3rd multiplex family (family C), a de novo mutation was identified in an a priori non-syndromic CL/P patient. Re-examination after mutation screening revealed the presence of a tiny pit-looking lesion on the inner side of the lower lip leading to a revised diagnosis of VWS. On the basis of this data, we conclude that IRF6 should be screened when any doubt rises about the normality of the lower lip and also if a non-syndromic cleft lip patient (with or without cleft palate) has a family history suggestive of autosomal dominant inheritance.

Techniques for coverage of infected vascular grafts in the groin.

Plastic surgeons expertise is requested for treatment of vascular graft exposures. Exposure within the femoral triangle (Scarpa) represents the majority of those cases. We intend to share our experience in the coverage of exposed vascular grafts: through the most common and safe procedures we are currently using, illustrated by some of our clinical cases. Our experience leaded us to formulate some guidelines and conclusions, to ensure vascular surgeons that coverage of a vascular graft can always be successfully done.

Immediate skin grafting of sub-acute and chronic wounds debrided by hydrosurgery.

A wound bed may be prepared by various non-surgical debridements using autolytic, biological or enzymatic techniques. These are all effective in selective wounds but tend to be time consuming. Surgical debridement is not selective since healthy collateral tissue is also removed. Physical debridement uses whirlpool therapy to slough off necrotic tissues - the saline which comes out of the hand piece if vapourized over the wound - and therefore disseminates contaminated droplets. Hydrosurgery combines physical and surgical debridement but does not have their drawbacks. Water dissection works by using a high-pressure jet of sterile saline that travels parallel to the wound and creates a Venturi effect, thus enabling the selective removal of necrotic tissues without dissemination of contaminants. In this study, the authors report on 167 sub-acute and chronic wounds from 155 patients treated under general anaesthesia by hydrosurgery (Versajet). Of these, 95% of the debrided wounds were immediately covered with an autologous meshed graft. Compared to other debridement techniques, hydrosurgery has two main advantages: namely its tissue selectivity and its high percentage of successful engraftment after immediate skin grafting.

Idiopathic scoliosis and breast asymmetry.

The aim of this study is to describe the semiology for the assessment of breast asymmetry in the presence of scoliosis. Twenty-four women with right idiopathic scoliosis treated with bracing alone (23 out of 24) or with bracing and spine surgery (1 out of 24) were evaluated by physical and morphological examinations and three-dimensional (3D) surface scan. Physical examination revealed a smaller right breast in 20 women. A left costal protrusion was observed in 18 patients. Anthropomorphic analysis revealed that the right breast was higher in 19 cases, and smaller in 18 cases. The calculations from 3D scan showed the right breast to be smaller in 19 women. A strong correlation is found between clinical parameters, anthropomorphic measurements and 3D scan analysis, suggesting that a meticulous clinical examination is sufficient to evaluate breast asymmetry in patients with idiopathic scoliosis. A patient who is properly diagnosed and informed of her skeletal deformity and breast asymmetry is more likely to have realistic expectations from breast surgery.

The versatility of the sub-mental flap: a case report.

The sub-mental flap has been used in four elderly patients (mean age: 83 years) for reconstruction of defects after oncological resection: three had basal cell carcinoma (cheek, temporal region and fronto-temporal region). One had a squamous cell carcinoma of the hard palate. We believe that the latter example is original and present it in this article. This case shows that the sub-mental flap in addition to its intrinsic qualities is a reliable flap which may be useful in difficult repairs. It can be used to repair wide palatal fistula which occurs after oncological resections.

[Labial sequels after uni- and bilateral cleft lip repair]. / Les séquelles labiales dans les fentes uni- et bilatérales.

Children born with labial-alveolar-velopalatine clefts must be managed by multidisciplinary teams in order to decrease the frequency and the importance of sequels, by implementing a true therapeutic strategy. It is indeed easier to avoid a secondary deformation than to correct it. Labial sequels are often associated to nasal sequels, and are managed in a single surgical intervention, with total revision of the cheilorhinoplasty. Some less important labial deformities can be corrected without total and simultaneous revision of the lip-nose complex. The goal of correction is functional and aesthetic, and the choice of the moment depends mainly on the psychological impact of the deformation for the child, and his motivation for reoperation.

[Vascular tumours and malformations, classification, pathology and imaging]. / Tumeurs et malformations vasculaires, classification anatomopathologique et imagerie.

The understanding of vascular anomalies (vascular tumours and vascular malformations) was obscured, for a long time, by confusion and uncertainties in nosology and terminology. The International Society for the Study of Vascular Anomalies (ISSVA) recently adopted a classification scheme, clearly separating vascular tumours (hemangiomas of different types) which result from active cell proliferation, from vascular malformations, which are inborn defects in vascular morphogenesis. These two types of lesions have different clinical behaviour and require different diagnostic and therapeutic strategies. The most frequent vascular tumour is infantile hemangioma. Its clinical aspects and evolution are well-known. New data have been recently obtained concerning the phenotype of tumour cells and its histogenesis. Of the numerous new vascular tumours, which have been recently described, only the congenital hemangiomas, the vascular tumours associated with the Maffucci syndrome and the tumours that may be complicated by a profound thrombocytopenia (Kasabach and Merritt phenomenon) will be considered. Vascular malformations can be classified according to the vessel(s) types they are composed of. A classification table is presented, separating the malformations of vascular trunks from tissular malformations which are more intimately embedded in the surrounding tissues. The different syndromes associated with vascular anomalies take also place in this table. The clinical, imaging and histological aspects of the most frequent malformations (capillary, venous, lymphatic and arteriovenous) are presented. This classification intend to clarify the nosology and terminology of the complex field of vascular tumours and malformation and to offer a common language to the different physicians and specialists contributing, preferably with a interdisciplinary approach, to the diagnosis and treatment of these difficult lesions.

[Medical and surgical treatment of venous malformations]. / Traitement médical et chirurgical des malformations veineuses.

Treatment of venous malformations (VM) is difficult as these lesions are ill defined and can infiltrate tissues. Moreover, no specific treatment related to their etiopathogenesis exists. Currently, VM can be treated conservatively or medically and, more aggressively with either sclerotherapy, or surgical resection. A multidisciplinary approach is needed to obtain the best result with minimal complications. Surgical resection, complete or partial, plays an important role in the treatment of symptomatic VM. In case of extensive VM, all techniques of plastic surgery are indicated.

[Arteriovenous malformations: clinical aspects and evolution]. / Les malformations artérioveineuses: aspects cliniques et évolution.

Arteriovenous malformations are rare high-flow malformations. As other malformations, they are present at birth but may remain quiescent for many years; their flow may speed up quickly consequently on an infection, traumatism or modification of the hormonal climate. A "nidus" appears in the heart of the malformation which acts as a real "turbo" on the blood flow; vascular shunts appear creating ulcerations (vascular steal phenomenon); cardiac failure may be observed. Among all vascular anomalies, arteriovenous malformation is the most dangerous in its evolution which may be fatal. Its control requires prudence and skill.

Orofacial clefting: update on the role of genetics.

INTRODUCTION: Cleft lip with or without cleft palate (CL/P) is one of the most common birth defects in the world. Prevalence varies between populations, with an average of 1/700. CL/P has a major clinical impact, requiring surgical, dental, orthodontic, speech, hearing and psychological management throughout childhood. The aetiology of CL/P is mostly unknown, and it is thought that both genetic and environmental factors play a role. Several causative genes for inherited syndromic forms of CL/P have been identified, and some recent studies have shown that these genes also contribute to the occurrence of isolated forms. Van der Woude syndrome (VWS) is one of the best models for non-syndromic CLP. It is an autosomal dominant disorder characterised by the presence of pits on the lower lip in addition to CL/P. Pits are the only feature distinguishing VWS from isolated clefts. Interestingly, in numerous VWS patients, the lip pits are very small and not readily diagnosed, thus mimicking isolated CL/P. Mutations in the IRF6 gene were shown to be the major genetic cause of VWS.' RESULTS: We performed direct sequence analysis of IRF6 on samples from a large European cohort and identified mutations in 27 (80%) families. This shows that IRF6 is the major causative gene of VWS in Europe also. Moreover, it is the gene to study when a seemingly isolated CL/P patient has minor signs, such as lip pits, since the identification of a mutation in IRF6 is associated with an increase in the risk of having a child with CL/P from 4-6%, the risk of transmission of an isolated cleft, to 50%, the risk of transmission of a dominant Mendelian disorder like VWS. Moreover, we studied the association of isolated CL/P with the IRF6 locus using two variants in a set of 195 patients from Belgium. As in an American study, a clear association was observed. This suggests that IRF6 also contributes to the occurrence of sporadic, isolated CL/P, even if no mutation in the gene can be identified in such patients. CONCLUSION: In conclusion, genes that are mutated in familial syndromic forms of CL/P may be predisposing genetic factors to sporadic isolated CL/P. Due to technological advances and the availability of the human genome sequence, we have now the opportunity to try and unravel the genetic factors behind the various forms of CL/P.

Review of the UCL management of patients with cleft lip and palate.

We describe the way our multidisciplinary team manages cleft lips and palates at our centre at the Catholic University of Louvain. Since 1987, we have opted for the neonatal repair of the cleft lip and nose, and closure of the cleft palate at three months of age. Multidisciplinary follow-up then takes place to detect and correct the sequellae. The children are seen once a year by a plastic surgeon, an otorhinolaryngologist, a maxillofacial surgeon, a speech therapist, an audiologist, and an orthodontist. Secondary corrections are scheduled depending on functional, aesthetic, and psychological requirements.

Bony syngnathia, vertebral segmentation defect, coloboma, microcephaly and mental retardation: confirmation of Dobrow syndrome and review of syndromal syngnathias.

Congenital bony fusion of the maxilla and mandible is a rare condition. Two classifications were previously proposed dealing exclusively with craniofacial malformations. Most of the reported cases to date represent either aglossia-adactylia or hemifacial microsomia syndromes. We report a young girl with bony syngnathia associated with multiple defects (severe microcephaly, coloboma, vertebral segmentation defects), growth and mental delay. This patient is very similar to the patient described by Dobrow in 1983 and confirms the existence of this extremely rare disorder.