Antenatal ear examination is an integral part of the thorough examination of the fetal face. The discovery of an anomaly, whether it is made by chance or during a complementary in-depth examination, leads the practitioner to determine its isolated or associated character, in order to characterise its possible belonging to a syndromic entity. In this context, the realization of genetic analysis more precise and wider allowing a return of the results in a time compatible with an evolutive pregnancy, gives to the geneticist a central role in the management of these couples. The main challenge lies in obtaining a set of concordant clinical and biological clues, enabling the genetic results identified to be interpreted correctly, the optimised functioning of the ultrasound practitioner - geneticist duo is therefore fundamental. This results in a complex information to deliver, in the fact that the clinical translation of an ear anomaly in antenatal can go from an isolated aesthetic anomaly to a genetic syndrome with neurodevelopmental disorder. The objective of this work is to describe, from a methodological analysis of antenatal ears, the accessible malformative entities, isolated or associated, and to discuss the problems in the need or not to propose their screening. (c) C 2022 Elsevier Masson SAS. All rights reserved.

L’examen des oreilles en anténatal fait partie intégrante de l’examen approfondi de la face fœtale. La découverte d’une anomalie, qu’elle soit faite de manière fortuite ou lors d’un examen approfondi complémentaire, amène l’échographiste à déterminer son caractère isolé ou associé, pour ainsi caractériser son éventuelle appartenance à une entité syndromique. Dans ce contexte, la réalisation d’analyses génétiques maintenant plus précises et plus larges permettant un rendu des résultats dans un délai compatible avec une grossesse évolutive, donne au couple prénataliste – généticien un rôle central dans la prise en charge de ces couples. Le principal challenge réside dans l’obtention d’un faisceau d’indices cliniques et biologiques concordants, permettant d’interpréter correctement les résultats génétiques identifiés. Il en découle alors une information complexe à délivrer, dans le fait où la traduction clinique d’une anomalie des oreilles en anténatal peut être envisagé depuis une anomalie esthétique isolée jusqu’à un potentiel syndrome génétique avec trouble du neuro-développement. L’objectif de ce travail est de décrire, à partir d’une analyse méthodologique des oreilles en anténatal, les entités malformatives accessibles, isolées ou associées, et de discuter des problématiques inhérentes à la nécessité ou non de proposer leur dépistage.

ObjectivesCraniofacial deformities have a high psychosocial impact. The aim of this paper is to improve obstetric ultrasonography and prenatal detection of facial anomalies by providing a new fetal dental panorama.Material and methodsThe present study describes a new modality to visualize the fetal tooth germs and an easy step-by-step diagnostic approach. Image acquisition was performed between 23 and 32weeks of gestation using a Voluson E10 GE ultrasound machine with an RM6C transducer (GE Medical Systems, Zipf, Austria). Reconstruction was performed using Omniview from the axial image. Volume contrast imaging (VCI) was used with a thickness of 20mm, and a render mode that combined Rx mode and surface texture.ResultsThe resulting imaging allows a more precise visualization of the fetal dental arch and can be obtained between 14 and 28weeks of gestation. The presence of dental anomalies can be a clue for the diagnosis of various congenital defects, in particular conditions with a shortage of other physical abnormalities, such as ectodermal dysplasia and Binder syndrome.ConclusionsThe creation of a precise fetal dental panorama allows an improved detection of facial deformities.Clinical relevanceWith the current paper, we want to increase prenatal diagnostics facial anomalies, and help to establish a tailored multidisciplinary treatment plan. This paper should be of interest to readers who are currently treating patients with craniofacial malformations and readers who are performing diagnostic prenatal sonography.

Three-dimensional (3D) ultrasound has significantly improved prenatal screening and perinatal care in the area of cleft lip/palate and other deformities, providing essential preoperative information to the surgical team. However, current 3D reconstruction modalities are limited primarily to display on a two-dimensional surface. In contrast, a 3D printed haptic model allows both the surgeon and the parents to develop a better understanding of the anatomy and the surgical procedure through the ability to interact directly with the printed model. The production of a 3D printed haptic model of cleft lip and palate obtained from a surface-rendered oropalatal sonographic view is presented here. The development of this 3D printed haptic model will allow the surgical team to perform preoperative planning with a highly accurate medical model, and it therefore represents a new tool in the management of cleft lip/palate. It also provides better prenatal information for the parents.

We propose a sonographic method to identify the fetal hyoid bone in an axial or sagittal way. Visualisation of the fetal hyoid bone could be an interesting anatomic and functional marker in fetal neck and face malformations such as lymphangioma or Pierre Robin syndrome. Thanks to pathological exams, we know that fetal hyoid bone is located at the height of the first and second cervical vertebrae (C1, C2) before birth whereas in adult it's located at the fourth cervical vertebrae (C4). It's a circular arc which is back opened, firstly made of cartilage before getting bony at the 3rd trimester. It's a vertical deglutitive mobile structure which is balanced and supported thanks to mandibular and basi-cranial muscles. These anatomic hyoid complex carries, above, all the tongue muscles, below, the laryngeal structures and posteriorly the pharynx. It appears as an important centre for swallowing efficiency and ventilatory regulation. The sonographic identification of hyoid bone needs a fetal head deflection, an axial view with a lateral approach of the neck. The ultrasounds have to be directed below the mandible. A hypoechogen circular arc are easily identify in front of the vocal cords in the larynx area, the hyoid bone is located in front of this hypoechogenic arc and can be seen as a hyperechogenic circular behind the myohyoideus muscle. The cervical spine is at the back of the view. When the fetus is swallowing the pharynx appears as an anechogenic area between the larynx and the spine. To identify the fetal hyoid bone by ultrasound shows a good feasibility when using our method. This anatomic landmark could be a precious help is fetal malformation such as fetal lymphangioma of the neck and face, or to predict the respiratory disorder in fetus affected by Pierre Robin sequence.

To present the usefulness of a fetal dental panoramic in the prenatal diagnosis of orofacial anomalies or syndromes. Technological advances in 3D sonography allow us to focus on some previously invisible elements such as the dental organ. The Fetal Dental Panoramic acquisition requires a fetus with a head in a deflective position. With the Voluson E10 General Electric ultrasound machine and the RM6C Transducer, we perform an axial initial section, objectivising the lip, maxillary bone and the anterior palatal canal. This acquisition is performed with slow sweep to obtain a maximum amount of information. Reconstruction is performed by Omniview from the axial acquisition. The Polyline function permits reconstruction in a continuous line that is positioned on the external ridge of the maxillary bone. It can be performed between 14–15 and 28 weeks of gestation. The interpretation of this sonographic image requires the knowledge of the dental growth during the fetal stage. The 20 deciduous teeth start their enamel-dentine mineralisation between the 14th and the 18th weeks and they grow up according to a precise chronology. Visualisation in 3D sonography of missing teeth allows us to identify precociously hypodontia or tooth agenesis, which are the most frequent developmental malformations of the orofacial complex. The image can be compared with a panoramic dental X-ray obtained in older children and adults. After presenting the normal appearance of this Fetal Dental Panoramic, we propose to illustrate its interest in fetal pathology. We will present its application in orofacial syndromes and in Cleft Lip and Palate. The visualisation of the dental organ could be a precocious diagnostic element in oro facial anomalies such as ectodermal dysplasia or clefts lip and palate.

Ultrasound in Obstetrics & GynecologyVolume 47, Issue 2 p. 244-246 Picture of the MonthFree Access Visualization of fetal lips and palate using a surface-rendered oropalatal (SROP) view in fetuses with normal palate or orofacial cleft lip with or without cleft palate D. Rotten, Corresponding Author D. Rotten Department of Obstetrics and Gynecology, Delafontaine General Hospital, Saint-Denis, FranceCorrespondence to: Dr D. Rotten, 1 rue Dante, 75005 Paris, France (e-mail: [email protected])Search for more papers by this authorJ.-M. Levaillant, J.-M. Levaillant Centre for Woman and Fetal Imaging, Créteil, FranceSearch for more papers by this authorL. Benouaiche, L. Benouaiche Department of Maxillofacial Surgery, Antony Private Hospital, Antony, FranceSearch for more papers by this authorR. Nicot, R. Nicot Centre for Woman and Fetal Imaging, Créteil, FranceSearch for more papers by this authorG. Couly, G. Couly Department of Maxillofacial Surgery, Necker Enfants Malades University Hospital, Paris, FranceSearch for more papers by this author D. Rotten, Corresponding Author D. Rotten Department of Obstetrics and Gynecology, Delafontaine General Hospital, Saint-Denis, FranceCorrespondence to: Dr D. Rotten, 1 rue Dante, 75005 Paris, France (e-mail: [email protected])Search for more papers by this authorJ.-M. Levaillant, J.-M. Levaillant Centre for Woman and Fetal Imaging, Créteil, FranceSearch for more papers by this authorL. Benouaiche, L. Benouaiche Department of Maxillofacial Surgery, Antony Private Hospital, Antony, FranceSearch for more papers by this authorR. Nicot, R. Nicot Centre for Woman and Fetal Imaging, Créteil, FranceSearch for more papers by this authorG. Couly, G. Couly Department of Maxillofacial Surgery, Necker Enfants Malades University Hospital, Paris, FranceSearch for more papers by this author First published: 16 July 2015 https://doi.org/10.1002/uog.14946Citations: 20AboutSectionsPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL In the presence of an orofacial cleft detected at routine midtrimester antenatal screening, precise characterization of the malformation is necessary, as this will affect the medical work-up and help in informing the parents1. The best method with which to analyze the palate is still under debate. Evaluation is usually based on multiplanar or tomographic reconstructions obtained with three-dimensional (3D) ultrasonography. Analysis of the posterior palate is impeded by artifacts due to acoustic shadowing by the anterior bony structures of the maxilla2. Various specific views have been advocated to overcome this difficulty, such as the 'reverse face' view, the intraoral 'en face' view, the 'flipped face' view, 'angled insonation', the 'axial underside' view and the 'oblique face' view (reviewed by To3). However, none has received general agreement. Our group has shown that the use of all three traditional orthogonal planes is necessary to examine thoroughly the different landmarks, in order to differentiate the involvement of the lip, alveolus and posterior hard palate. Lip analysis necessitates visualization of the coronal planes, and, in the case of bilateral clefts, the midsagittal plane. The palate (alveolus, maxilla, secondary palate) is best analyzed using coronal and axial planes4, 5. However, 3D multiplanar reconstructions are impeded by certain drawbacks: to convey the information necessitates multiple scans; they focus on the bony defect of the palate; and they are difficult to interpret, in particular by lay people, in contrast with the more readily understood surface-rendered views6. To overcome these flaws, we have developed a surface-rendered representation, corresponding to the submental intraoral photograph of the neonatal palate that is used by orofacial surgeons to visualize clefts7, 8 : the surface-rendered oropalatal (SROP) sonographic view. The SROP sonographic view is oriented in an oblique direction, transoral and directed upwards, from cephalad to caudal. Image reconstruction utilizes the surface rendering mode, which combines representation of the surface itself and that of the subjacent muscles and bony structures. The virtual lighting (in HDlive mode (GE Medical Systems, Zipf, Austria)) is focused on the frontal view of the palate. This view allows simultaneous visualization of the lips, the alveolar ridge and the secondary palate, thus synthesizing the essential information that must be communicated to the orofacial team about the bony and soft tissue defects, and helping the parents to understand the malformation. In the case of a normal palate, the SROP view visualizes, from front to back, the following structures (Figure 1): the cutaneous and mucous structures that cover the perioral muscle, the maxilla and the palatal processes. The posterior border of the horizontal plates of the palate bone is imaged at the level of the pterygoid processes. The integrity of the palate at this level constitutes a reliable landmark for excluding a cleft of the bony palate. The velum is also apparent. Figure 1Open in figure viewerPowerPoint Typical surface-rendered oropalatal (SROP) view in a 22-week fetus with normal palate, showing: alveolar ridge with tooth buds (A), lip (L), nares (N), palate bone (P) and synostosis of palatine processes. In the case of unilateral cleft lip with or without cleft palate (CL ± P), the SROP view visualizes, from front to back, the following anatomical landmarks relevant for description of the defect (Figure 2): the nostril rim, allowing description of its deformation and splaying; the thickness of the soft tissues of the internal and external banks of the separated lip; and the size, form and position of both the larger and lesser fragments that constitute the bony lining of the alveolopalatal cleft. Figure 2Open in figure viewerPowerPoint Surface-rendered oropalatal (SROP) views showing characteristic landmarks of unilateral cleft lip with or without cleft palate, in: (a) a 24-week fetus with narrow unilateral cleft; and (b) a 24-week fetus with large unilateral cleft. A, alveolar ridge and tooth buds; C, cleft; L, lip; N, nares; P, palate bone. Similarly, in the presence of a bilateral CL ± P, the following structures relevant to describe the defect are imaged, from front to back (Figure 3): the protruding premaxillary prolabium, with the possibility of characterizing the degree of its protrusion, and the width and length of both its bony and its soft tissue components; the lips on both sides of the premaxillary prolabium; and the bony parts of the alveolopalatal cleft (thickness and spacing of the banks). Figure 3Open in figure viewerPowerPoint Surface-rendered oropalatal (SROP) views showing characteristic landmarks of bilateral cleft lip with or without cleft palate, in: (a) a 24-week fetus with narrow bilateral cleft; and (b) a 25-week fetus with large bilateral cleft. A, alveolar ridge; C, cleft; CFb, central fragment, bony part; CFs, central fragment, soft part; L, lip; N, nares; P, palate; V, septovomer. Thus, the SROP view is a comprehensive ultrasonographic view of the fetal perioral region. It allows visualization, in a single view, of the lips, the alveolar ridge and the hard palate, condensing the basic information on a cleft as proposed by Mulliken and Benacerraf: side, type and extent7. As the SROP view originates from the same acquired volumes as do the three standard orthogonal planes, it provides the same basic information about the anatomical defects as does traditional 3D multiplanar reconstruction. However it has several advantages. First, thanks to the combination of the surface rendering mode with virtual lighting, it allows visualization of the surface itself and that of the subjacent muscles and bony structures. Adding an analysis of the soft tissues enables more precise prediction of the severity of the defect to be corrected, providing the surgeon with information that can help him to predict with more precision both the technical difficulties that he will encounter and the expected final result. Accurate nasal and lip reconstruction is essential to the final esthetic result9. Second, it combines in a single view the essential information that needs to be communicated to various members of the orofacial team. Finally, it provides the parents with a representation of the defect affecting their child, facilitating their understanding of the malformation and of the information provided by the orofacial team regarding the nature of the defect and the sequence of therapeutic steps required. References 1Maarse W, Rozendaal AM, Pajkrt E, Vermeij-Keers C, Mink van der Molen AB, van den Boogaard MJ. A systematic review of associated structural and chromosomal defects in oral clefts: when is prenatal genetic analysis indicated? J Med Genet 2012; 49: 490– 498. 2Campbell S. Prenatal ultrasound examination of the secondary palate. Ultrasound Obstet Gynecol 2007; 29: 124– 127. 3To WWK. Prenatal diagnosis and assessment of facial clefts: where are we now? Hong Kong Med J 2012; 18: 146– 152. 4Rotten D, Levaillant JM. Two- and three-dimensional sonographic assessment of the fetal face. 2. Analysis of cleft lip, alveolus and palate. Ultrasound Obstet Gynecol 2004; 24: 402– 411. 5Rotten D, Levaillant JM. Prenatral diagnosis of facial clefts. In Comprehensive Cleft Care, JE Losee, RE Kirschner (eds). McGraw-Hill Medical: New York, 2009. 6Sepulveda W, Wong AE, Castro F, Adiego B, Martinez-Ten P. Feasibility of 3-dimensional sonographic examination of the fetal secondary palate during the second-trimester anatomy scan. J Ultrasound Med 2011; 30: 1619– 1624. 7Mulliken JB, Benacerraf BR. Prenatal diagnosis of cleft lip: what the sonologist needs to tell the surgeon. J Ultrasound Med 2001; 20: 1159– 1164. 8Schaaf H, Streckbein P, Ettorre G, Lowry JC, Mommaerts MY, Howaldt HP. Standards for digital photography in cranio-maxillo-facial surgery--part II: additional picture sets and avoiding common mistakes. J Craniomaxillofac Surg 2006; 34: 366– 377. 9He X, Shi B, Kamdar M, Zheng Q, Li S, Wang Y. Development of a method for rating nasal appearance after cleft lip repair. J Plast Reconstr Aesthet Surg 2009; 62: 1437– 1441. Citing Literature Volume47, Issue2February 2016Pages 244-246 FiguresReferencesRelatedInformation

We present a new way to diagnose orofacial cleft in order to improve fetal ultrasound screening. The Levaillant view enables to explore the labial, labiomaxilla and velopalatin cleft in a better plan. This technical consists in accessing by tangential transbuccal way the lips, maxilla and palate. Using this view offers good information from the 2nd trimester screening to prenatal teams and especially surgeons as it gives anatomic specific details and measurements. These prognostic factors are helpful for prenatal consulting and for planning postnatal repair strategy.

Los traumatismos faciales de la población pediátrica requieren un tratamiento adecuado. Debido a las peculiaridades fisiológicas y anatómicas propias de esta población, este tratamiento puede diferir del aplicado en los adultos. La exploración física será suave y hay que evitar cualquier dolor inútil. Las exploraciones radiológicas que pueden realizarse en los adultos no siempre son factibles en los niños, lo que puede aumentar la frecuencia de exploraciones mediante tomografía computarizada (TC), a pesar de la sensibilidad pediátrica a los rayos X. La exploración física adecuada permite orientar mejor las pruebas de imagen en función de los signos clínicos. El tratamiento variará tanto en lo referente a sus modalidades técnicas (adaptación de las osteosíntesis, prioridad de los tratamientos funcionales) como a la menor duración de la inmovilización. En los traumatismos de los tejidos blandos, se establece una diferencia entre las heridas simples y aquéllas con riesgo de lesión de los órganos nobles. Los traumatismos dentales requieren una descripción perfecta, debido a su repercusión futura social y económica. En este artículo se describen las distintas fracturas, enumeradas por orden de frecuencia: fracturas de los huesos propios nasales, fracturas alveolodentales, fracturas mandibulares, fracturas del hueso cigomático, fracturas del piso orbitario, etcétera. Se describen desde los puntos de vista etiopatogénico, clínico y de las pruebas complementarias. En cada caso, se indica el tratamiento. La iconografía permite apreciar el aspecto radiográfico de las distintas fracturas.

Studies carried out in the avian embryo and based on the construction of quail-chick chimeras have shown that most of the skull and all the facial and visceral skeleton are derived from the cephalic neural crest (NC). Contribution of the mesoderm is limited to its occipital and (partly) to its otic domains. NC cells (NCCs) participating in membrane bones and cartilages of the vertebrate head arise from the diencephalon (posterior half only), the mesencephalon and the rhombencephalon. They can be divided into an anterior domain (extending down to r2 included) in which genes of the Hox clusters are not expressed (Hox-negative skeletogenic NC) and a posterior domain including r4 to r8 in which Hox genes of the four first paraloguous groups are expressed. The NCCs that form the facial skeleton belong exclusively to the anterior Hox-negative domain and develop from the first branchial arch (BA1). This rostral domain of the crest is designated as FSNC for facial skeletogenic neural crest. Rhombomere 3 (r3) participates modestly to both BA1 and BA2. Forced expression of Hox genes (Hoxa2, Hoxa3 and Hoxb4) in the neural fold of the anterior domain inhibits facial skeleton development. Similarly, surgical excision of these anterior Hox-negative NCCs results in the absence of facial skeleton, showing that Hox-positive NCCs cannot replace the Hox-negative domain for facial skeletogenesis. We also show that excision of the FSNC results in dramatic down-regulation of Fgf8 expression in the head, namely in ventral forebrain and in BA1 ectoderm. We have further demonstrated that exogenous FGF8 applied to the presumptive BA1 territory at the 5-6-somite stage (5-6ss) restores to a large extent facial skeleton development. The source of the cells responsible for this regeneration was shown to be r3, which is at the limit between the Hox-positive and Hox-negative domain. NCCs that respond to FGF8 by survival and proliferation are in turn necessary for the expression/maintenance of Fgf8 expression in the ectoderm. These results strongly support the emerging picture according to which the processes underlying morphogenesis of the craniofacial skeleton are regulated by epithelial-mesenchymal bidirectional crosstalk.

La prise de conscience de la dangerosité par les opérateurs(trices) des interventions grandes ou petites de chirurgie maxillofaciale se construit grâce à un savoir anatomique irréprochable, concernant les sites et régions abordées, seul facteur d'aisance technique et ainsi de maîtrise du risque. La dangerosité garde encore toute son acuité dans les suites opératoires, dominées par les accidents hémorragiques, facteurs d'hypovolémie et de détresse respiratoire parfois mortelles. L'hémostase peropératoire et le drainage aspiratif postopératoire ne se discutent pas. La dangerosité demeure encore bien réelle pour les actes dont les indications sont fictives.

La cavité buccale du nourrisson et du nouveau-né est le siège de multiples manifestations tumorales ou pseudotumorales. Les plus fréquentes sont les kystes épithéliaux d'évolution parfaitement bénigne. À l'opposé, les sarcomes embryonnaires sont des lésions exceptionnelles mais de pronostic réservé. Entre les deux se situent une multitude de lésions bénignes. Certaines sont de diagnostic clinique, confirmé par le geste chirurgical (grenouillette, épulis congénitale, hamartome…). Certaines sont de diagnostic clinique mais nécessiteront un bilan d'imagerie avant exérèse (angiomes). Certaines pourront bénéficier dans certains cas d'une abstention thérapeutique (angiome plan, hémangiome, papillome, nævus, petit kyste mucoïde) sous couvert d'une surveillance. Certaines peuvent avoir une valeur prédictive (névromes myéliniques de l'apudomatose IIb). D'autres lésions plus rares sont possibles et sont alors le plus souvent de diagnostic histologique. The most frequently encountered lesions are epithelial cysts which are absolutely benign. On the other side, sarcomas are uncommon but have a poor prognosis. Many other benign lesions may be identified by clinical examination. Diagnosis will be confirmed by histology. Most lesions will need surgical treatment. In some cases, no treatment is required (haemangioma, papilloma, naevus, small mucous retention cyst, capillary or lymphatic malformation…). Some lesions may be of prognostic value (nevromas of the phacomatosis). The oral cavity of the infant or the new-born may be affected by various tumours or tumour-like lesions.

Cette étude a pour but de décrire les caractéristiques psychologiques, sociodémographiques et somatiques de tous les enfants (âgés de 8 à 12 ans) qui ont consulté pour un problème alimentaire sur une unité de soins pédiatriques d’un centre hospitalier universitaire pendant une période de 15 ans.Nous avons revu les dossiers médicaux de 215 enfants à l’aide d’une grille détaillée élaborée pour cette étude. Des statistiques descriptives (tests-t, Chi2) ont été effectuées.L’échantillon total est composé majoritairement de filles (92 %, n = 197), 4 % d’entre elles sont des jumelles. Des 215 enfants, 52 % ont été hospitalisés au moins une fois et 48 % ont été soignés en service ambulatoire. Dans l’échantillon, 82 % expriment des préoccupations à l’égard de la nourriture et du poids, 69,4 % ont peur de prendre du poids, 57,5 % désirent encore en perdre et 46,6 % ont des préoccupations par rapport à leur image corporelle. La majorité des enfants, soit 95 %, ont des comportements restrictifs et 13,3 % ont des comportements boulimiques. Des difficultés alimentaires durant l’enfance sont présentes chez 15,9 % des enfants. Un peu plus de la moitié des enfants (55,8 %) a au moins un autre trouble comorbide et la présence d’antécédents familiaux de maladies psychiatriques est notée chez 36,3 % des enfants de l’échantillon. Les filles et les garçons obtiennent des résultats similaires sur la majorité des variables de l’étude. On note une seule différence significative entre eux : les garçons sont plus isolés socialement. Des différences significatives sont observées entre les enfants plus jeunes (âgés de 8 à 10 ans) et les plus âgés (10–12 ans) constituant l’échantillon.Les résultats soulèvent des questionnements sur l’étiologie du trouble et sur son éventuelle évolution. Des études sur le devenir de ces enfants sont essentielles afin de mieux les comprendre et nous aider à mieux adapter nos stratégies d’interventions auprès d’eux.This study aimed to describe psychological, sociodemographic and somatic characteristics of all children aged 8–12 years who received treatments for eating disorders on a pediatric unit of a University Health Centre during a period of 15 years.We reviewed the medical records of 215 children with a detailed grid. Descriptive statistics (t-test, Chi2) were performed.The overall sample was comprised largely (92%) of girls (n = 197); 4% of whom were twins. Out of the 215 children, 52% were hospitalized at least one time and 48% were seen and treated as outpatients. In the sample, 82% expressed concerns towards food/weight, 69.4% were afraid of gaining weight, 57.5% still want to lose weight and 46.6% had body image preoccupations. Most of the children, e.g. 95%, had restrictive eating behaviors and 13.5% had bulimia symptoms. Eating problems during infancy were present in 15.9% of the sample. More than half of the children (55.8%) had at least one comorbid condition and family history of psychiatric problems was observed for 36.3% of the children. Boys and girls obtained very similar results on all variables of the study. Only one significant difference had been observed between them: boys were more socially withdrawn. Some significant differences were found between younger children (8–10 years old) and the older one (10–12 years old) of the sample.These results bring out questioning on the etiology of ED in children and on their outcome. Follow-up studies of children presenting ED are essential to better understand these problems and their evolution in order to adapt our strategies for treating them.

The neural crest (NC) yields pluripotent cells endowed with migratory properties. They give rise to neurons, glia, melanocytes and endocrine cells,and to diverse `mesenchymal' derivatives. Experiments in avian embryos have revealed that the differentiation of the NC `neural' precursors is strongly influenced by environmental cues. The reversibility of differentiated cells(such as melanocytes or glia) to a pluripotent precursor state can even be induced in vitro by a cytokine, endothelin 3. The fate of `mesenchymal' NC precursors is strongly restricted by Hox gene expression. In this context,however, facial skeleton morphogenesis is under the control of a multistep crosstalk between the epithelia (endoderm and ectoderm) and NC cells.

Hyoid bone is a part of the visceral skeleton which arises from both Hox ‐expressing ( Hox +) and Hox ‐nonexpressing ( Hox ‐) cephalic neural crest cells. In a previous work, we have demonstrated that the Hox ‐ neural crest domain behaves as a naïve entity to which the ventral foregut endoderm confers patterning cues to specify the shape and orientation of the nasal and mandibular skeleton. By using ablation and grafting approaches, we have extended our study to the formation of the hyoid bone and tested the patterning ability of more caudal levels of the lateroventral foregut endoderm in the chick embryo at the early neurula stage. In this study, endodermal stripes have first been delineated according to the projection of mid‐ and posterior rhombencephalic structures. The extirpation of endodermal transverse stripes along the anteroposterior axis selectively hampers the formation of the ceratobranchials and epibranchials. Thus defined, the patterning ability of the endodermal stripes was further explored in their medial and lateral parts. When homotopically engrafted on the migration pathway of cephalic neural crest cells, ventromedial zones of endoderm lead to the formation of supernumerary basihyal and basibranchial, while lateral zones generate additional cartilaginous pieces recognizable as ceratobranchial and epibranchial. Taken together, our data demonstrate that, early in development, the ventral foregut endoderm exerts a regionalized patterning activity on the cephalic neural crest to build up the primary facial and visceral skeleton in jaws and neck and enable a map of the endodermal skeletogenic areas to be drawn. This map reveals that a cryptic metamerization of the anterior foregut endoderm precedes the formation of the branchial arches. Developmental Dynamics 228:239–246, 2003. © 2003 Wiley‐Liss, Inc.

Diencephalic, mesencephalic and metencephalic neural crest cells are skeletogenic and derive from neural folds that do not express Hox genes. In order to examine the influence of Hox gene expression on skull morphogenesis, expression of Hoxa2, Hoxa3 and Hoxb4 in conjunction with that of the green fluorescent protein has been selectively targeted to the Hox-negative neural folds of the avian embryo prior to the onset of crest cell emigration. Hoxa2 expression precludes the development of the entire facial skeleton. Transgenic Hoxa2 embryos such as those from which the Hox-negative domain of the cephalic neural crest has been removed have no upper or lower jaws and no frontonasal structures. Embryos subjected to the forced expression of Hoxa3 and Hoxb4 show severe defects in the facial skeleton but not a complete absence of facial cartilage. Hoxa3 prevents the formation of the skeleton derived from the first branchial arch, but allows the development (albeit reduced) of the nasal septum. Hoxb4, by contrast, hampers the formation of the nasal bud-derived skeleton, while allowing that of a proximal (but not distal) segment of the lower jaw. The combined effect of Hoxa3 and Hoxb4 prevents the formation of facial skeletal structures, comparable with Hoxa2. None of these genes impairs the formation of neural derivatives of the crest. These results suggest that over the course of evolution, the absence of Hox gene expression in the anterior part of the chordate embryo was crucial in the vertebrate phylum for the development of a face, jaws and brain case, and, hence, also for that of the forebrain.

The vertebrate face contains bones that differentiate from mesenchymal cells of neural crest origin, which colonize the median nasofrontal bud and the first branchial arches. The patterning of individual facial bones and their relative positions occurs through mechanisms that remained elusive. During the early stages of head morphogenesis, an endodermal cul-de-sac, destined to become Sessel's pouch, underlies the nasofrontal bud. Reiterative outpocketings of the foregut then form the branchial pouches. We have tested the capacity of endoderm of the avian neurula to specify the facial skeleton by performing ablations or grafts of defined endodermal regions. Neural crest cells that do not express Hox genes respond to patterning cues produced regionally in the anterior endoderm to yield distinct skeletal components of the upper face and jaws. However, Hox-expressing neural crest cells do not respond to these cues. Bone orientation is likewise dependent on the position of the endoderm relative to the embryonic axes. Our findings thus indicate that the endoderm instructs neural crest cells as to the size, shape and position of all the facial skeletal elements, whether they are cartilage or membrane bones.

Background. Paragangliomas are unusual tumors in the head and neck originating from the paraganglia or glomus cells of neural crest origin.Methods. We describe the first case of a primitive paraganglioma of the floor of the mouth presenting in childhood.Results. Complete surgical removal was performed after embolization of the left lingual artery. There was no evidence of either persistent or recurrent disease 5 years after surgery. The embryologic and anatomic origins of head and neck paragangliomas are reviewed.Conclusions. An embryologic theory based on the common neural crest origin and migration pathways of both autonomic viscerocranium appended ganglias and paragangliomas is proposed that unifies the topographically heterogeneous group of viscerocranium-appended paragangliomas. (C) 2001 John Wiley & Sons, Inc.