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.

[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.

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.

[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.

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.

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.

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.

[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.

Melanotic neuroectodermal tumour of infancy involving the orbit and maxilla: surgical management and follow-up strategy.

Melanotic neuroectodermal tumour (MNET) of infancy is a rare benign but locally aggressive tumour. We describe our surgical treatment of MNET of the orbital region. There was osteogenic relapse involving the bone of the orbit, 20 days after macroscopically complete excision of the primary tumour when the patient was 12 weeks old. This is only the second report of osteogenic relapse in MNET. The relapse was treated by excision of the involved orbital floor, preserving the orbital periosteum. The tumour has not recurred in 23 months of follow-up. Residual tumour islets may regress spontaneously after incomplete excision of MNET, but the relapse rate is between 15% and 45%. In our opinion, excising a safety margin of a few mm of apparently healthy bone reduces the risk of relapse. In contrast, the orbital contents should be preserved if they are macroscopically normal. Follow-up consisted of frequent physical examinations and CT scans.

[Angiomatous lips]. / Les lèvres angiomateuses.

After defining vascular malformations and tumors, the authors approach specific problems of these lesions involving the lips. Careful planning and assessment are necessary throughout the clinical course and evolution. Therapeutic management concern the vascular anomaly but the functional, cosmetic and psychological repercussions as well. The rules of surgical treatment are discussed in this labial location. Capillary malformations can be treated by pulsed dye laser for the skin involvement, but sometimes by reconstructive surgery in case of soft tissue and bony overgrowth. Venous malformations require percutaneous sclerotherapy, partial or total removal surgery, reconstructive surgery, with or without previous embolization, according to the size and functional repercussions. Lymphatic malformations involving the lip are based upon conservative and observing treatment or surgery according to impairment and psychological impact. There is a strong tendency for these lymphatic microcystic malformations to invade and to recur after surgery. The new lasers (diode, Nd Yag) have to be assessed in this area. Arterio-venous malformations are the most severe anomaly. When the lesion is cosmetically and functionally acceptable, the authors propose conservative management waiting for therapeutic progress expected from genetics research. Otherwise management require embolization and complete surgical treatment with lip reconstruction. The first-line treatment of hemangiomas is medical and pharmacological (local medical care, corticosteroids, interferon, vincristine) but surgery may be indicated in three situations. In urgent cases with severe complications surgery is performed after failure of medical management. Early surgery is recommended to prevent functional or cosmetic disturbance or serious psychological distress. Ultrasound dissection (Dissectron) an significantly contribute to the surgical outcome. Late surgery retains its classical cosmetic and functional indications and techniques to treat the residual after-effects. Three key-words dominate the rules of therapeutic management of all types of vascular anomalies: multidisciplinary approach, experience and carefulness.

[Early surgery of immature hemangiomas with the aid of an ultrasonic scalpel. Apropos of 81 cases]. / Chirurgie précoce des hémangiomes immatures à l'aide d'un dissecteur à ultrasons. Etude à propos de 81 cas.

BACKGROUND: The natural course of immature hemangiomas in infants is well-known. A rapid phase of growth from 6 to 8 months is followed by a period of stability then regression. Since approximately 70% of these immature hemangiomas resolve spontaneously, abstention is generally the rule. The volume or localization of certain lesions may nevertheless have a serious functional or morphological impact. MATERIAL AND METHODS: This retrospective study included 81 children who underwent surgery between October 1994 and March 2000. The children were aged 2 to 38 months at the time of surgery. Orbital localizations predominated (33 children). The indication for surgery was based on two criteria: risk of a functional impairment or risk of morphological sequela. All children with orbital hemangiomas with a functional risk of amblyopia were initially treated with corticosteroids. Surgery was performed in case of failure. The CAVITRON was used for 77 children and DISSECTRON for 4. These two ultrasound devices allowed easy dissection with little hemorrhage. RESULTS: There were no peroperative hemorrhagic complications. Few postoperative complications were observed. After resection of the orbital hemangiomas there was little functional impact and the postoperative ophthalmologic examinations were normal within several weeks. Mean follow-up was 12 months after surgery. Use of an ultrasound dissector allowed early and safe treatment of immature hemangiomas. DISCUSSION: Certain voluminous or poorly localized hemangiomas, particularly on the face, can have a serious function, morphological or psychological impact. Medical treatment is not always active and surgical resection may be required before the development of definitive sequelae. Ultrasound dissection, not previously used in this indication, can contribute significantly to the surgical outcome as demonstrated in these children operated on early. This technique is safe and shortens operative time. In light of these results, we believe early resection of immature hemangiomas should be reevaluated. It should not be considered as a last resort but rather as a complementary treatment.

[Mandibular advancement: osteotomy or distraction osteogenesis? A case report]. / Avancement mandibulaire: ostéotomie ou distraction? A propos d'un cas clinique.

From an experience in osteogenic distraction techniques acquired since 1993 and facing a case of retromandible, the authors have tried to define the criteria leading them to suggest a distraction rather a classical surgical procedure. The selection of the patient is based upon the age, the significance of the advancement, the asymmetry of the advancement and the former existence of a temporomandibular joint degenerative disease. Monitoring the distraction, in particular with per- and post-distraction orthodontics, remains complicated. The overcorrection and the reduction of recurrences after advancement using distraction remain openly discussed.

"Distraction" of grafted alveolar bone in cleft case using endosseous implant.

OBJECTIVE: Presentation of a specific surgical technique of vertical alveolar ridge distraction performed on an alveolar bone graft 1 year after the primary grafting procedure designed to correct a labioalveolar cleft. The graft had not obtained the results desired and presented heavily scarred mucosal tissue. TECHNIQUE: A dental implant placed within the graft once it has been entirely freed by osteotomies functions as an endless screw. To avoid vascular risk, the alveolar bone graft is detached as a whole along its total height. Consequently, no bone remains above it to provide support for a conventional alveolar distractor. An osteosynthetic miniplate, fastened in a horizontal position beneath the nasal mucosae above the graft, is used to replace the missing bony support and to stabilize the implant, which activates the distraction process. PATIENT: This study is based on our first clinical case with a follow-up of 18 months at present. RESULT: A vertical displacement of the entire graft was achieved, including its attached mucosal layer, which repositioned both bone and mucosa on a far better level in the cleft area. Thus, a normal length of abutment was obtained permitting prosthetic rehabilitation based on the same endosseous implant, which was left in place within the graft. CONCLUSION: This technique may prove particularly helpful in certain cases in which a primary alveolar bone grafting procedure has produced borderline results. In such cases, on the one hand, neither the reasonably satisfactory volume of the bone graft itself nor the poor quality of its scarred mucosal tissue argue in favor of a secondary grafting procedure. On the other hand, it is impossible to resort to currently available alveolar distracters since our choice of techniques leaves no bone support above the graft.

[Oto-mandibular dysplasias: genetics and nomenclature of syndromes]. / Dysplasies oto-mandibulaires: génétique et nomenclature des formes syndromiques.

Otomandibular dysplasia are characterised by a combination of anomalies of the ear and the mandible. From the surgical point of vue, facial dysostosis is prominent and focus the attention. For the geneticist it is a group of different entities, familial or sporadic. Familial history, detailed clinical examination looking for extra-facial associated malformations, characteristics of the facial dysostosis, unilaterality or bilaterality and biological or radiological findings allow sometimes to identify a known syndrome. A bilateral and symetric dysostosis with predominant zygomatic and malar hypoplasia suggest the diagnosis of Treacher-Collins or Franceschetti syndrome or mandibulofacial dysostosis, particularly in the presence of positive familial history. Acral anomalies associated with facial dysostosis allow the distinction between Treacher-Collins syndrome and acrofacial dysostosis (Nager and Miller syndromes). Unilateral and bilateral asymmetrical anomalies, namely facioauriculovertebral syndrome, hemifacial microsomia, otomandibular dysostosis, no. 7 cleft, first branchial arch syndrome, Goldenhar syndrome were lumping together by Gorlin in 1990, who proposed to use the term "oculoauriculovertebral spectrum". This classification is the first step before genetic studies, who need homogeneous group of patients. Lastly recurrence risk can be evaluated and genetic counselling can be done only if a precise genetic diagnosis is known.

[Hemifacial microsomia treated with mandibular lengthening using intraoral distractors. On precise indications]. / Microsomies hémifaciales et distraction mandibulaire intra-orale. Vers des indications précises.

Mandibular hypoplasia in the hemifacial microsomia have largely benefited from distraction techniques especially intraoral. In fact, these techniques are possible in the child, replacing him in a better morphological, psychological and functional configuration. They can redynamise growth and, in any case, diminish secondary distant effects. The authors, from precise criteria, propose a surgical classification to facilitate the therapeutic decision. Intraoral and sometimes bidirectional distraction, whose results are equivalent to external distraction, present numerous advantages that, in the majority of cases, make it preferred now to external distraction. Its three-dimensional (3D) effect on bone and soft tissues and the long term follow-up must be evaluated more objectively, as pre and post-distraction stereolithographic or 3D photogrammetric may get. It must be considered in the child as the first step of skeletic surgery preparing to a facilitated second step orthopedic, orthodontic or orthognathic after puberty. In the young adult, it is in competition with conventional surgery but the slow progressive process, kind with the temporo-mandibular joints, allows in mandibular asymmetries an adaptation having avoided the need for controlateral osteotomy or a bone graft.