[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.

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.

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.

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.

Maxillary and mandibular distraction osteogenesis in growing patients: lessons learned after 14 years and update on indications.

In the past decade, distraction osteogenesis (DO) has become increasingly popular and has opened new therapeutic perspectives for the treatment of numerous congenital and acquired craniofacial skeletal anomalies. However, DO mechanisms still remain unclear and different treatment protocols are applied by different groups. Here the authors use their 14 years-clinical experience to evaluate DO parameters such as maxillary and mandibular DO stability over time, especially in growing patients, DO effects on soft tissues and the correlation between the bone gain and lengthening capabilities of the device. Based on these data, clinical indications and treatment protocols for mandibular and maxillary DO are suggested. The application of DO to the membranous bones of the craniofacial skeleton has opened a new chapter in the surgical treatment of several congenital and acquired craniofacial deformities. Based on this experience, the authors recommend DO indications guidelines in selected cases of hemifacial microsomia, maxillary hypoplasia and narrow mandible with anterior dental crowding.

Use of ultrasonic dissection in the early surgical management of periorbital haemangiomas.

PURPOSE: To evaluate the efficacy and safety of the early surgical excision of periorbital haemangiomas with an ultrasonic scalpel in infants at risk of visual impairment. STUDY: A retrospective analysis of 67 infants diagnosed to be at risk of amblyopia from periorbital haemangiomas, treated consecutively with the Dissectron between 1994 and 2005. Ophthalmic outcome parameters included the pre- and postoperative measurement of visual axis occlusion, strabismus, astigmatism, and degree of amblyopia. RESULTS: Visual performance showed an overall improvement of 30% following treatment. Seventy-six patients were found to have abnormal ophthalmic examinations preoperatively, compared to 46 following surgery. After surgery, visual axis occlusion decreased from 73 to 6%; amblyopia decreased from 67 to 22%, strabismus decreased from 26 to 18% and astigmatism (>onedioptre) decreased from 66 to 31%. Mean astigmatism values decreased from 3.5 to 1.9 dioptres. No new cases of astigmatism, strabismus or amblyopia were diagnosed postoperatively. Three minor complications resolved with conservative treatment. All patients were satisfied with the outcome of their surgery. CONCLUSION: Early surgical excision of periorbital haemangiomas using the Dissectron in infants with an established risk of visual impairment is a safe and effective alternative to pharmacological therapy. The use of the Dissectron is associated with reduced operative times and a shorter hospital stay.

[Maxillary sequelae in cleft patients. Causes of maxillary hypoplasia and possible prevention]. / Les séquelles maxillaires dans les fentes labioalvéolopalatovélaires. Analyse des causes de la rétromaxillie et réflexions sur sa prévention.

Maxillary hypoplasia is frequently observed in cleft patients. Although maxillary retrusion can be a syndromic outcome, the growth failure is also a consequence of the primary surgery of the palate, alveolar cleft, or lip. In this article the authors analyze the impact of primary surgery on the maxillary growth failure and discuss on how to prevent this complication.

[Maxillary sequels in labial-alveolar-velopalatine clefts. Role of distraction osteogenesis]. / Les séquelles maxillaires dans les fentes labioalvéolopalatovélaires. Place de la distraction ostéogénique.

A high rate of cleft patients present with maxillary hypoplasia. Most of the growth defects concern the anteroposterior axis of the maxilla. Before bone lengthening by distraction osteogenesis, orthognathic surgery was the only alternative treatment for maxillary hypoplasia. Several studies showed the lack of stability after conventional surgery. In this article reviewing the literature concerning all bone lengthening procedures, the authors discuss published data on maxillary distraction osteogenesis by external and internal devices. Indications of distraction in growing children as an interceptive step are discussed.

[Vertical maxillo-mandibular dysmorphias due to excessive growth. Terminology, diagnosis and treatment]. / Dysmorphies verticales maxillo-mandibulaires par excès. Propositions taxinomiques, diagnostiques et thérapeutiques.

The authors propose a system of terminology for vertical maxillo-mandibular dysmorphia, "long face" or rather "high face", and "hypsoprosopia" hypsomaxillia to facilitate the diagnosis and the therapeutic approach. They distinguish 3 abnormalities which are responsible, alone or in combination, for an increased height in the face: hypsomaxilla, hypsogenia and vertical anterior inter-incisive aperture (open bite), which can arise in three situations (supra erupted posterior alveolar maxillary segment, infra erupted anterior alveolar mandibular segment, exaggerated opening of the angle of the mandible or amblygonia). They present the clinical picture and the specific teleradiographic features of each abnormality, supported by cases of patients with definite dento-maxillary growth. On the basis of this classification, the authors propose a more systematized approach to the various segmental or total maxillary or mandibular osteotomies, which may be coupled or even combined with advancements or transverse movements in cases of associated dysmorphosis.

Unilateral congenital choanal atresia and maxillary sinus development.

Most authors feel that the maxillary sinus and dento-alveolar arch do not develop normally in case of choanal atresia. This as explained by respiratory deficiency on the atretic side. A series of 11 cases of unilateral choanal atresia, either untreated or treated after the period of maximum growth, have been studied by clinical examination, serial X-rays and cephalometric tracings in order to compare atretic and normal sides. No significant difference has been found, neither for size nor shape of the maxillary sinus. These observations are therefore in contradiction to earlier reported findings and require further study.