[Secondary nasal surgery with cleft palates]. / Les rhinoseptoplasties secondaires des fentes labiopalatines.

Residual nasal deformity in patients with cleft palate remains the main demand of these patients. Performing primary nasal surgery has significantly improved the results. However, it is still often necessary to improve the nasal morphology. Respect of tissues during primary surgery is essential and allows easier secondary corrections. Anatomical reconstruction greatly facilitates the treatment of secondary deformities. Do not hesitate in case of major labionasales sequelae, to make revision, according to the rules of primary surgery, of the entire lip and nose.

[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.

[Craniofacial strategy for syndromic craniosynostosis]. / Stratégie craniofaciale pour les faciocraniosténoses.

The complexity of treatment of faciocraniosynostosis justifies the treatment in a reference center for rare diseases. The growth disturbances in the skull and face being variable according to the type of mutation in the FGFr (Crouzon, Pfeiffer, Apert), the strategy is adapted to the phenotype according to the following principles: posterior expansion with or without distraction around 6 months to limit the descent of the cerebellum tonsils and to prevent the turricephalic development; fronto-facial monobloc advancement with internal distraction around the age of 18 months in case of severe exorbitism or breathing impairment. The dissociated strategy (fronto-orbital advancement first, followed by facial osteotomy of Le Fort 3 type). The growing evolution dictates the sequence of subsequent surgeries according to the monitoring of intracranial pressure by fundus examination and of the respiration by polysomnography. Le Fort 3 and transversal maxillary distraction may be repeated if necessary. Orthognathic surgery is almost always compulsory after the age of 14, before the aesthetic refinements which can be undertaken ultimately (rhinoplasty, genioplasty, canthopexies, fat grafting…).

[Tissular expansion in giant congenital nevi treatment]. / Nævus pigmentaires congénitaux géants : quelle place pour l'expansion cutanée.

UNLABELLED: Surgical management of giant melanotic naevi remains a surgical challenge. Tissue expansion provides tissue of the same quality for the repair of defects. The aim of this study is to review tissular expansion for giant melanotic naevi. MATERIALS AND METHODS: We conducted a retrospective study from 2000 to 2012. All children patients who underwent a tissular expansion for giant congenital naevi had been included. Epidemiological data, surgical procedure, complication rate and results had been analysed. RESULTS: Thirty-tree patients had been included; they underwent 61 procedures with 79 tissular-expansion prosthesis. Previous surgery, mostly simple excision had been performed before tissular expansion. Complete naevus excision had been performed in 63.3% of the cases. Complications occurred in 45% of the cases, however in 50% of them were minor. Iterative surgery increased the complication rate. CONCLUSION: Tissular expansion is a valuable option for giant congenital naevus. However, complication rate remained high, especially when iterative surgery is needed.

Experimental review of PEI electrodeposition onto copper substrates for insulation of complex geometries.

Polyetherimide (PEI) was used for coating copper substrates via electrophoretic deposition (EPD) for electrical insulation. Different substrate preparation and electrical field application techniques were compared, demonstrating that the use of a pulsed voltage of 20 V allowed for the best formation of insulating coatings in the 2-6 µm thickness range. The results indicate that pulsed EPD is the best technique to effectively coat conductive substrates with superior surface finish coatings that could pass a dielectric withstand test at 10 kV mm-1, which is of importance within the EV automotive industry.

Dynamic orthognathic surgical procedure (DOSP) in asymmetric maxillomandibular dysmorphism secondary to unilateral micrognathia: Outcomes of 12 consecutive cases.

This study aimed to evaluate the outcomes following a dynamic orthognathic surgical procedure performed at the end of growth to treat asymmetric maxillomandibular deformities linked to unilateral micrognathia when conventional orthognathic surgery was not feasible. The dynamic orthognathic surgical procedure (DOSP) combined concomitant mandibular distraction osteogenesis with contralateral poorly stabilized sagittal split osteotomy and Le Fort I osteotomy. Cephalometric studies were retrospectively conducted on pre- and postoperative lateral and frontal cephalographs, and maxillomandibular movements were calculated. Outcome scores were computed by both experts and laypersons based on photographic analyses. There was a significant postoperative increase in height of the micrognathic ramus in all patients (n = 12; p = 0.002). The angle between the occlusal cant and horizontal reference plane decreased significantly in all of the patients, as did the angle between the midline sagittal plane and mandibular tilt (p < 0.001). Postoperative outcome scores showed significant improvements in all cases, according to both expert and layperson groups. This procedure allows correction of maxillomandibular asymmetries linked to micrognathia. However, it cannot resolve all the factors participating in facial asymmetry, such as those originating in the oculo-auriculo-ventricular spectrum or complex tumor sequelae, and second-step procedures may be required.

Gnathodiaphyseal dysplasia with a novel R597I mutation of ANO5: Mandibular reconstruction strategies.

Gnathodiaphyseal Dysplasia (GDD) is a rare, often misdiagnosed, autosomal-dominant disorder due to point mutations in the ANO5 gene. GDD combines craniofacial fibro-osseous lesions, dental loss and progressive curvature and cortical thickening of long bones and vertebra, causing pathological fractures. Diagnosis is based on bone pathology and mutation screening. Here we report three GDD cases within a single family with a novel ANO5 mutation: c.1790 G > T (p.Arg597Ile, i.e. R597I) on exon 16. Microsurgical mandibular reconstructions were performed in the three cases. We reviewed the literature on jaw reconstruction in this condition and discussed the challenges of craniofacial reconstruction in GDD due to the diffuse bone anomalies affecting potential flap donor zones and a specific risk for jawbone osteomyelitis.

Feasibility of magnetic activation of a maxillofacial distraction osteogenesis, design of a new device.

PURPOSE: Distraction osteogenesis is a technique of bone lengthening which uses the bone's natural healing process. Current devices for craniofacial distraction require a transmucosal or transcutaneous activator and are associated with numerous complications. The aim of this study was to evaluate the feasibility of a rodless magnetic activation device that could be used in craniofacial distraction. METHODS: The method is based on the torque applied between two unaligned permanent magnets. This torque depends on magnet size, shape, composition, magnetization and distance between the two magnets. Using a configuration close to that which would be applied in actual distraction osteogenesis (in terms of the distance between the two magnets), we performed an analytical study and evaluated the results. RESULTS: We observed good agreement between the model and the experimental results, finding that the transmitted force value is comparable to the force required in mandibular distraction. Thus, we proposed a design of a new distracting device consisting of a cylindrical permanent magnet diametrically magnetized and fixed to an endless screw along its main axis. Activation of the distraction motion is achieved through interaction of the first magnet with a second cylindrical magnet whose magnetization is orthogonal to its main axis and to the device's endless screw. CONCLUSION: This preliminary study demonstrates that magnetic activation for mandibular osteogenic distraction is feasible and that device size is not a constraint. We propose a prototypic device.

Patients' responses to distraction osteogenesis: a multi-centre study.

This investigation aimed to compare and contrast practical difficulties experienced by 54 children from three different centres (Mexico City, New York, Paris) treated with either extra-oral or intra-oral distraction devices. The possible effect of distraction osteogenesis on pain/sleeping difficulty, speech and eating problems and disturbance of recreational activities and alteration in sensation were investigated. It was expected that intra-oral devices would reduce the difficulties associated with the distraction process and their psychological effect on the child. Some benefits resulting from the use of intra-oral devices were identified, these included fewer sleeping problems (P = 0.006) and less disturbance of recreational activities (P = 0.002). However, eating and maintaining oral hygiene were more problematic with intra-oral devices the differences between the intra-oral and extra-oral groups was approaching significance at P = 0.07. A major disadvantage of the extra oral device was scarring. In both groups the alteration of lip sensation was temporary, pain was limited to the time of activation of the distraction device. A high level of patients' cooperation was mandatory for successful completion of the treatment.

Orbital soft tissue surgery for patients with Treacher-Collins or Nager syndrome. A new surgical approach with early correction of soft tissue: prospective study.

Orbitopalpebral anomalies in mandibulofacial dysostosis (Treacher-Collins syndrome) can be difficult to correct surgically and most authors recommend correction of the malar bone after the age of 8 years. We propose a new, early surgical approach for periorbital defects that involves initial implantation of autologous fat and subperiosteal malar lift with a pedicled upper eyelid flap. We prospectively studied 5 children, 3 of whom had previously had orbital reconstruction. Initial fat graft into the periorbital area was followed by a subperiosteal malar lift with lateral canthopexy, and a pedicled upper eyelid flap if needed. Two surgeons independently assessed the patients' characteristics including scleral show before and after operation, antimongoloid palpebral fissures, canthal dystopia, number 6 cleft (Tessier classification), skin quality, and surgical and ophthalmic complications. Before operation 3 patients had had ophthalmic problems. Postoperative evaluation showed improvements in scleral show, canthal dystopia, and skin quality, and correction of antimongoloid palpebral fissures and subcutaneous number 6 clefts. There were no postoperative complications. The proposed protocol gave satisfactory aesthetic and functional results in children with Treacher-Collins syndrome. We recommend that it is considered for periorbital reconstruction in all patients with a mandibulofacial deformity.

Anatomical study of the course of the inferior alveolar nerve in craniofacial microsomia using three-dimensional computed tomography: correlation with the Pruzansky classification.

Dysmorphogenesis of the mandible complicates assessment of the course of the inferior alveolar nerve in patients with craniofacial microsomia. Our aim in the present study was to correlate the anatomical description of the course with the Pruzansky classification (which indicates the severity of hemifacial microsomia), in the mandibles of 22 affected patients using 3-dimensional computed tomography (CT). We measured the distance between fixed landmarks on the normal and the microsomic sides. The normal sides served as controls. In the group of five patients with type I disease, we found no significant differences between the unaffected and the microsomic side. In the nine patients with Pruzansky type II disease morphological views of the course showed a between-side difference in the length of the bony canal and the height of the mandibular ramus. In the five patients with Pruzansky type III disease, there was no bony canal. Three-dimensional CT analysis may be of value in plotting the course of the inferior alveolar nerve and assisting the surgeon in mandibular osteotomy or distraction osteogenesis.

Long face: new proposals for taxonomy, diagnosis, treatment.

The authors suggest a taxonomy and a specific terminology from Greek etymology for vertical maxillo/mandibular excess dysmorphies, "long face" or "high face" (hypsoprosopia) to be more exact, so as to make their diagnosis and their therapeutic approach easier. They distinguish three anomalies which may, separate or in association, cause an increased vertical dimension of the face: hypsomaxillia, hypsogenia and inter incisal anterior vertical gap or hyposostomy, which may itself be differentiated into 3 aetiological sites (vertical posterior maxillary dento-alveolar excess, vertical anterior mandibular dento-alveolar deficiency, excessive gonial angle opening or amblygonia). They submit a clinical and teleradiographic table specific for each anomaly, relying on patients having achieved their full dento-osseous growth. When confronted with associated cases, they can therefore more systematically apply the various osteotomies, partial or total, maxillary or mandibular, or even combined operations.

Submerged intraoral device for mandibular lengthening.

The authors report a new technique for mandibular distraction. Lengthening of the mandible by gradual intraoral distraction was obtained in nine young patients. An intraoral device was used in order to avoid external scars. Seven patients had hemifacial microsomia, one patient had a ramus hypoplasia after TMJ ankylosis and one patient had the Treacher-Collins syndrome. The amount of mandibular lengthening ranged from 12 to 28 mm depending on the duration of expansion. Retention after expansion, to allow ossification to take place, lasted for 3 weeks on average. The follow-up period ranged from a minimum of 5 months to a maximum of 44 months.

Intraoral distraction for mandibular lengthening: a technical innovation.

An intraoral distraction device for mandibular lengthening is reported. Correction of vertical deficiency of the ramus was associated with expansion of the soft tissue of the jaw, without any visible scars.

[Surgical treatment of superficial vascular malformations. Indications for tissue expansion]. / Traitement chirurgical des malformations vasculaires superficielles. Intérêt de l'expansion tissulaire.

The aim of this work was to demonstrate the feasibility of surgical exeresis of superficial vascular malformations using tissue expansion. A retrospective analysis of data from 15 patients who underwent surgery over a 9 year period for arteriovenous (n = 6) and venous malformations of the trunk and limbs was made. Indications for treatment were pain in six patients or complications of an arteriovenous malformation. Twenty-eight expanders were used in 15 patients. Most of the complications observed (25% of the cases) were minor. The program had to be interrupted due to complications in only one case (7%). Mean duration of tissue expansion was 105 days (30-165). Mean delay to cicatrization was 40 days and mean duration of the treatment program was 156 days. Indication for surgical exeresis of superficial vascular malformations can be widened due the contribution of tissue expansion. With acquired experience, the risk of complications has been reduced. The duration of the treatment protocol is the main drawback.

Intraoral mandibular distraction: indications, technique and long-term results.

This report describes the experience of the Trousseau Hospital, Paris, France, with distraction osteogenesis of the mandible using an intraoral distraction device. From 1993 to 1998, 26 paediatric patients with mandibular hypoplasia underwent distraction of the mandible using the Leibinger Intraoral Distractor. The majority of the patients had hemifacial microsomia. Distraction was performed at a rate of 1 mm a day following complete osteotomy of the mandible. A mean of 15 mm of distraction was obtained. In conjunction with combined orthodontic management, satisfactory morphologic results were achieved in the majority of patients with good facial symmetry, adequate occlusal relationships and balanced maxillary mandibular relationships. Radiographic evaluation revealed that substantial new bone formation and remodelling was induced by the intraoral distraction along the entire hemimandible on the distracted side and that this contributed significantly to the aesthetically pleasing clinical results.

[Amblyopia and peri-ocular capillary hemangioma of infancy: screening and clinical course before and after surgery]. / Amblyopie et hémangiome orbito-palpébral capillaire chez le jeune enfant: dépistage et évolution pré et post-chirurgicale.

PURPOSE: The analysis of visual complications provoked by infantile periocular hemangiomas and the understanding of their evolution before and after surgery. MATERIAL: and methods: A retrospective study including 30 children (31 eyes) who were operated for a periocular hemangioma with a surgical technique with the aid of ultrasonic scalpel Cavitron. Ophthalmological evaluation before and after surgery included: appreciation of the palpebral occlusion, strabismus, ocular motility, objective refraction and amblyopia. RESULTS: Surgical operation was performed within an average age of 9.5 months. The excision of periocular hemangiomas was effective on the release of the visual axis and on the amblyopia: 51.7% (16 eyes) of palpebral occlusion before surgery against 6.4% (2 eyes) after surgery and 77.4% (24 eyes) of amblyopia before surgery against 38.7% (12 eyes) after surgery. The anisometropia decreased after surgery. The ocular motility and the strabismus also improved. CONCLUSION: Surgical excision of periocular hemangiomas with the aid of ultrasonic scalpel is an effective technique presenting few complications. This surgery can be suggested early in infancy. It has a very good result in the release of the visual axis and the astigmatism which aided the reeducation of the amblyopia.

[Anal plasty after excision of giant condyloma acuminata]. / Plastie anale après exérèse de condylomes acuminés géants.

Giant condyloma acumination, also called Bürschke-Loewenstein disease, is a pseudo-tumoral epithelial proliferation of viral origin (human papilloma virus). Surgery is the most effective of all treatments, but it results in loss of all tissues covering the anal canal. We suggest a reconstruction technique, performed under colostomy, in which the rectal mucosa is brought down and three sliding flaps are constructed from the skin of the ischio-anal fossae.

[Cranial fasciitis of childhood]. / Fasciite du cuir chevelu (cranial fasciitis) de l'enfant.

INTRODUCTION: A nodule of the scalp in a child of less than eleven years should evoke a cranial fasciitis among other serious diagnoses. OBSERVATION: A four-month old infant had a firm and pink nodule at the left parietal level, exhibiting a slow growth since two months. It was excised. The pathologic sample showed spindle-shaped cells within a myxoïde matrix, with a strong reactivity for smooth muscle actin (immunohistochemical analysis). Diagnosis of cranial fasciitis was made. Due to the results of pathology, it was possible to rule out the diagnosis of sarcoma, therefore, no complementary work-up was performed. Evolution was favorable. DISCUSSION: Cranial fasciitis is a diagnosis to be considered when confronted with a firm nodule of the scalp in a infant or a young child, with or without bone involvement. This is a benign lesion but worrying pathological signs may exist, making diagnosis of benignity difficult. Exeresis of the lesion, even incomplete, protects the child from possible recurrence. Evolution is always good.