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.

Speech outcome after intravelar veloplasty.

OBJECTIVE: Cleft lip and palate is the main craniofacial malformation in France. Many surgical techniques had been described to restore cleft palate. In this study, we evaluate phonation in a homogeneous series of patient with isolated unilateral non-syndromic cleft lip and palate before (and after) alveolar cleft closure, operated according to our surgical protocol. METHODS: We included retrospectively 71 patients with isolated non-syndromic unilateral cleft lip and palate (UCLP), operated in our department from 2009 to 2013. All patients underwent the same surgical protocol: modified Millard cheilorhinoplasty (from 5 to 9-month-old); direct hard palatal closure (from 12 to 20-month-old); alveolar cleft closure with cancellous iliac bone graft (from 4 to 6-year-old). The phonation and clinical statute were evaluated before and after alveolar cleft closure. Fistula rate and speech evaluation were recorded. RESULTS: The rate of oronasal fistula was 12.7%. About phonation, 76% and 86% of patients were competent or borderline competent respectively before and after gingivoperiostoplasty. CONCLUSION: This surgical protocol provided speech results in patients with isolated unilateral non-syndromic cleft lip and palate. The gingivoperiostoplasty improved the speech intelligibility.

The use of three-dimensional reconstructions of CT scans to evaluate anomalies of hyoid bone in Pierre Robin sequence: A retrospective study.

AIM: The aim of the study was to investigate hyoid bone anomalies in patients with Pierre Robin sequence (PRS) compared to the control group, using computed tomography (CT) examination and three-dimensional reconstruction of the hyoid bone and mandible. METHODS: A retrospective study was performed of patients between birth and 12 months old with isolated PRS (i-PRS) and syndromic PRS (ni-PRS), who had undergone CT examination, and whose results were compared to the control group of the same age. DICOM data was processed to highlight bone tissue. The mandible and hyoid bones were the main targets of the three-dimensional reconstruction. The study outcomes were the analysis of hyoid bone ossification, volume, and position (distance between hyoid and mandibular symphysis). Univariate and multivariate statistical analyses were performed with α=0.05 as level of significance. RESULTS: The study sample included 29 i-PRS and 21 ni-PRS patients, while 43 infants in the control group. Hyoid ossification was present in 26/50 (52%) PRS patients (14 i-PRS; 12 ni-PRS) but in 31/43 controls (72%). Statistical analysis showed that absence of hyoid ossification was significantly associated with the diagnosis of PRS (P<0.05). Only ni-PRS patients showed a significant reduction of the distance between hyoid and mandible compared to the control group (P<0.001). Hyoid volume was significantly lower only in the ni-PRS group than in controls (P<0.001). CONCLUSION: I-PRS and ni-PRS patients differ both etiologically and clinically. Ni-PRS patients confirmed their worst clinical condition than i-PRS with severe anomalies of hyoid development, helping for their ontogeny classification.

[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.

Maxillary shape after primary cleft closure and before alveolar bone graft in two different management protocols: A comparative morphometric study.

AIM AND SCOPE: Result assessment in cleft surgery is a technical challenge and requires the development of dedicated morphometric tools. Two cohorts of patients managed according to two different protocols were assessed at similar ages and their palatal shape was compared using geometric morphometrics. MATERIAL AND METHODS: Ten patients (protocol No. 1) benefited from early lip closure (1-3 months) and secondary combined soft and hard palate closure (6-9 months); 11 patients (protocol No. 2) benefited from later combined lip and soft palate closure (6 months) followed by hard palate closure (18 months). Cone-Beam Computed Tomography (CBCT) images were acquired at 5 years of age and palatal shapes were compared between protocols No. 1 and No. 2 using geometric morphometrics. RESULTS: Protocols No. 1 and No. 2 had a significantly different timing in their surgical steps but were assessed at a similar age (5 years). The inter-canine distance was significantly narrower in protocol No. 1. Geometric morphometrics showed that the premaxillary region was located more inferiorly in protocol No. 1. CONCLUSION: Functional approaches to cleft surgery (protocol No. 2) allow obtaining larger inter-canine distances and more anatomical premaxillary positions at 5 years of age when compared to protocols involving early lip closure (protocol No. 1). This is the first study comparing the intermediate results of two cleft management protocols using 3D CBCT data and geometric morphometrics. Similar assessments at the end of puberty are required in order to compare the long-term benefits of functional protocols.

Diagnostic criteria in Pai syndrome: results of a case series and a literature review.

Pai syndrome was originally described as the association of a midline cleft lip, midline facial polyps, and lipoma of the central nervous system. However, only a few patients present with the full triad, and most exhibit a wide spectrum of phenotypic variability. The aim of this study was to phenotypically delineate Pai syndrome and to propose new criteria to facilitate a clinical diagnosis in the future. The study cohort consisted of seven case patients and an additional 60 cases diagnosed with Pai syndrome identified in a literature review. Only 23 of 67 patients presented the full triad as historically described by Pai et al. (1987). A congenital facial midline skin mass was always encountered, particularly affecting the nasal structures (60/67). A midline facial cleft was reported in 45 of 67 patients and a pericallosal lipoma in 42 of 67 patients. The proposed definition of Pai syndrome is the association of (1) a congenital nasal and/or mediofrontal skin mass and/or a mid-anterior alveolar process polyp as a mandatory criterion, and at least one of the following criteria: (2) midline cleft lip and/or midline alveolar cleft, and/or (3) a pericallosal lipoma or interhemispheric lipoma in the case of corpus callosum dysgenesis.

Pierre Robin sequence: A comprehensive narrative review of the literature over time.

Pierre Robin syndrome (PRS) is characterized of a triad of clinical signs: micrognathia, glossoptosis and obstruction of the upper airways frequently associated with palatal cleft. It is a heterogenic pathological entity and it can be found as isolated disease (nsPRS) or in association with other syndromes (sPRS), with more pronounced symptoms and systemic involvement. This review aims to summarize the principal features of PRS, analysing the different aspects of the disease. Epidemiological data highlight incidence, severity and mortality of PRS; pathophysiological mechanism reports the etiology and pathogenesis of the disease distinguishing between isolated and syndromic form. Because of the clinical importance of PRS, it's fundamental to describe the features of the Robin sequence to clearly define its primary and secondary clinical signs useful to diagnosis. A complete evaluation of the syndrome allows choosing the most appropriate therapeutic treatment, opting for conservative or surgical management, in order to improve the quality of life of the patient.

[Facial injections of hyaluronic acid-based fillers for malformations. Preliminary study regarding scar tissue improvement and cosmetic betterment]. / Injections d'acides hyaluroniques au niveau de visages atteints de malformations faciales. Étude préliminaire de l'assouplissement des zones cicatricielles et de l'amélioration esthétique.

BACKGROUND: Cross-linked hyaluronic acid-based fillers have gained rapid acceptance for treating facial wrinkles, deep tissue folds and sunken areas due to aging. This study evaluates, in addition to space-filling properties, their effects on softness and elasticity as a secondary effect, following injection of 3 commercially available cross-linked hyaluronic acid-based fillers (15mg/mL, 17,5mg/mL and 20mg/mL) in patients presenting with congenital or acquired facial malformations. PATIENTS ET METHODS: We started injecting gels of cross-linked hyaluronic acid-based fillers in those cases in 2013; we performed 46 sessions of injections in 32 patients, aged from 13-32. Clinical assessment was performed by the patient himself and by a plastic surgeon, 15 days after injections and 6-18 months later. RESULTS: Cross-linked hyaluronic acid-based fillers offered very subtle cosmetic results and supplemented surgery with a very high level of satisfaction of the patients. When injected in fibrosis, the first session enhanced softness and elasticity; the second session enhanced the volume. Cross-linked hyaluronic acid-based fillers fill sunken areas and better softness and elasticity of scar tissues. CONCLUSION: In addition to their well-understood space-filling function, as a secondary effect, the authors demonstrate that cross-linked hyaluronic acid-based fillers improve softness and elasticity of scarring tissues. Many experimental studies support our observations, showing that cross-linked hyaluronic acid stimulates the production of several extra-cellular matrix components, including dermal collagen and elastin.

Speech evaluation after intravelar veloplasty. How to use Borel-Maisonny classification in the international literature?

OBJECTIVE: Comparing functional outcomes after velar repair appeared to be difficult because of the absence of international standardized scale. Moreover most of the studies evaluating speech after cleft surgery present multiple biases. The aim of our study was to assess speech outcomes in a homogeneous group of patients, and to define an equivalence table between different speech scales. MATERIALS AND METHODS: Patients with isolated cleft lip and palate (CLP), operated in our unit by the same senior surgeon were included. All patient were operated according to the same protocol (cheilo-rhinoplasty and intravelar veloplasty at 6 months, followed by a direct closure of the hard palate at 15 months). Speech evaluation was performed after 3 year-old and before the alveolar cleft repair. Borel-Maisonny scale and nasometry were used for speech evaluation. RESULTS: Twenty-four patients were included: 17 unilateral CLP and 7 bilateral CLP. According to the Borel-Maisonny classifications, 82.5% were ranged phonation 1, 1-2 or 2b. Nasometry were normal in almost 60% of cases. CONCLUSIONS: This study showed the efficiency of our protocol, and intravelar veloplasty. Moreover we proposed an equivalence table for speech evaluation scale.

[Electromyographic assessment of the temporalis muscle prior to a lengthening myoplasty in patients with Moebius syndrome]. / Étude électromyographique du muscle temporalis avant une myoplastie d'allongement dans le syndrome de Moebius.

BACKGROUND/OBJECTIVES: Temporalis muscle lengthening myoplasty improves tightening of the lips and rehabilitates smile for patients with congenital facial palsies. Because Moebius syndrome is heterogeneous, a careful evaluation is mandatory before deciding to perform myoplasty. This series shows the role of electromyography for investigating temporalis muscle and trigeminal nerve motor functions. METHODS: We conducted a retrospective study of 18 patients with no upward movements of the labial commissure and absent or unsightly smile. Electromyography was used to study the temporalis muscle bilaterally. Analysis focused on the recruitment pattern of voluntary contraction and electrical silence or activity at rest. Traces were classified as normal, neurogenic, or low-amplitude. Functional outcomes of myoplasty were evaluated by measuring the upward movement of the commissure (mm), and qualified as high (≥10), medium (>5), or little (≤5). RESULTS: Surgery was cancelled for 5 patients with abnormal electromyographic signs, neurogenic (2) or low-amplitude (3). Myoplasty was performed in 7 patients (age: 8-17 years), unilaterally (3) or bilaterally (4). Preoperative electromyogram was normal (3), or showed moderate neurogenic (2) or low-amplitude (2) changes. Follow-up period after surgery was from 2 to 12 years; functional outcomes were high (5), medium (1), or little (1). CONCLUSION: Electromyographic study of the temporalis can detect muscle denervation or atrophy, or dyspraxia, and guide decision to encourage or discourage performing myoplasty, or enhance rehabilitation programme and make the patient aware of possibly modest outcome.

[Functional treatment of children subcondylar fractures: An axiographic assessment]. / Traitement fonctionnel des fractures sous-condyliennes de l'enfant : une évaluation axiographique.

INTRODUCTION: Subcondylar fractures are common in children. Occlusion disorders resulting from these fractures in deciduous or mixed dentition do not have as much impact as in adults due to alveolar adaption possibilities. Functional treatment allows for good functional results, but does not treat the dynamic shortening of the ramus. The objective of this study was to evaluate the axiographic condylar slope changes after subcondylar fracture in children. MATERIALS AND METHODS: A prospective study was conducted from 2010 to 2015, including all the under-18 patients presenting with a subcondylar fracture. Examination by mean of a Quick Axis axiograph measured the length of propulsion and the condylar inclination on both sides. The main evaluation criterion was the amount of condylar inclination decrease on the fractured side. RESULTS: Twelve patients (mean age: 10.42; 5-16) were included. Eleven children had a loss of condylar inclination on the fractured side without occlusion disorders at 33.2 months on average (3-144 months) after the initial trauma. Only one patient had symmetric axiographies without loss of condylar inclination on the fractured side. DISCUSSION: Dynamic shortening of the ramus on the side of the subcondylar fracture is consistent at short and medium terms in children. Surgical treatment may be the solution for avoiding this dynamic disorder of the mandible and should be evaluated.

[Facial trauma and injury in children]. / Plaies et traumatismes de la face de l'enfant.

Facial traumas are common in children but often unconsidered. Facial injury is responsible of impressive bleeding because of the rich vascularization of the face; this bleeding is often underestimated because of the immediate arterial vasoconstriction that is very strong for children. The blood volume is 80ml/kg for a newborn, with a total of 250ml, reaching 70ml/kg at one year of age. The evaluation must be rigorously performed due to the risk of a sudden decompensation. Regarding the wounds, the primary repair must be performed directly neat or optimal in case of damaged tissues. The rule is to keep maximum of the integrity and to limit debridement. Careful repair often requires general anesthesia, especially in young children, to facilitate a perfect joining of the edges and of the mucocutaneous lines. Losses of substance should be treated by directed cicatrization. Flaps are never performed in children as a first intention for reasons developed below. Given the elasticity of the facial skeleton, fractures require a brutal shock to occur, but the clinical signs can be misleading. For instance, too specific and sometimes ignored, fractures can show weakly symptomatic signs : the fractures of the condylar and the orbital floor, with their respective complication that are temporomandibular bone ankylosis and definitive diplopia. Possible children abuse should be suspected in case of different age lesions and discrepancies between the told story and types of injuries. Once the vital urgency is eliminated, the orbital emergency should be first considered in facial traumas within the ophthalmology specialty because wounds and contusions of the globe are often under-evaluated and threaten the vision. The second emergency is the orbital floor fracture in its 'trapdoor' type, specific to the child. Combined with a motionless eye and uncontrollable vomiting, this is the second true urgency because it involves the prognosis of the oculomotricity and requires emergency surgery. Finally, dental trauma should not be overlooked because of their functional and aesthetic consequences. Primary cicatrization is usually rapid but scars remain inflammatory during a long time. The risk of hypertrophy exists in case of contusions and lacerations associated with wounds but also during puberty and in some locations. Age interfere with the result because growth will either improve or worsen the initial result, depending on the location and mechanism. The secondary specialized and prolonged managing and monitoring is capital on the functional, aesthetic and psychological points of view.

[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.

Intraosseous haemangioma: semantic and medical confusion.

The literature is rich in case reports of intraosseous haemangioma, although most of these are actually cases of venous or capillary malformations. To illustrate this confusion in terminology, we present three cases of slow-flow vascular malformations misnamed as intraosseous haemangioma. A retrospective study of children diagnosed with intraosseous haemangioma was conducted. Clinical and radiological data were evaluated. Histopathological examinations and immunohistochemical studies were redone by three independent pathologists to classify the lesions according to the International Society for the Study of Vascular Anomalies (ISSVA) and World Health Organization (WHO) classifications. Three children who had presented with jaw haemangiomas were identified. Computed tomography scan patterns were not specific. All tumours were GLUT-1-negative and D2-40-negative. The lesions were classified as central haemangiomas according to the WHO, and as slow-flow malformations according to the ISSVA. The classification of vascular anomalies is based on clinical, radiological, and histological differences between vascular tumours and malformations. Based on this classification, the evolution of the lesion can be predicted and adequate treatment applied. The binary ISSVA classification is widely accepted and should be applied for all vascular lesions.

[Wiedemann-Beckwith syndrome: a new approach for reduction glossoplasty using Ultracision(®)]. / Syndrome de Wiedemann-Beckwith - glossectomie de réduction par Ultracision(®) : une approche novatrice.

INTRODUCTION: A reduction glossectomy may be complicated by tongue and mouth floor edema and extend the recovery time for a normal tongue function. We performed reduction glossectomy using Ultracision(®), an ultrasonic vibrating device, so as to limit these complications. TECHNICAL NOTE: We performed a keyhole glossoplasty under general anesthesia. The initial tongue incision was performed with a cold scalpel, then the glossectomy was continued with Ultracision(®) only. We also used CURVED SHEARS(®). We evaluated our preliminary results with 3 patients presenting with Wiedemann-Beckwith syndrome. CONCLUSION: Ultracision(®) is a useful tool for intra-oral surgery, and should soon be more frequently used. It is an alternative to electrocautery for this type of surgery.

[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.

Beckwith-Wiedemann syndrome: growth pattern and tumor risk according to molecular mechanism, and guidelines for tumor surveillance.

BACKGROUND: Beckwith-Wiedemann syndrome (BWS) is an overgrowth syndrome associated with an increased risk of pediatric tumors. The underlying molecular abnormalities may be genetic (CDKN1C mutations or 11p15 paternal uniparental isodisomy, pUPD) or epigenetic (imprinting center region 1, ICR1, gain of methylation, ICR1 GOM, or ICR2 loss of methylation, ICR2 LOM). AIM: We aimed to describe a cohort of 407 BWS patients with molecular defects of the 11p15 domain followed prospectively after molecular diagnosis. RESULTS: Birth weight and length were significantly higher in patients with ICR1 GOM than in the other groups. ICR2 LOM and CDKN1C mutations were associated with a higher prevalence of exomphalos. Mean adult height (regardless of molecular subtype, n = 35) was 1.8 ± 1.2 SDS, with 18 patients having a final height above +2 SDS. The prevalence of tumors was 8.6% in the whole population; 28.6 and 17.3% of the patients with ICR1 GOM (all Wilms tumors) and 11p15 pUPD, respectively, developed a tumor during infancy. Conversely, the prevalence of tumors in patients with ICR2 LOM and CDKN1C mutations were 3.1 and 8.8%, respectively, with no Wilms tumors. CONCLUSION: Based on these results for a large cohort, we formulated guidelines for the follow-up of these patients according to the molecular subtype of BWS.