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Objectives The aim of this study was to define the prenatal ultrasound semiology of cleft palate without cleft lip using 3D visualization of the fetal palate. Methods A prospective longitudinal study was performed in our University Hospital Center from 2011 to 2018. The fetal secondary palate was studied in 3D, starting with 2D axial transverse ultrasound view. We defined a cleft palate as a disruption of the horizontal plate of the palatine bone of the secondary palate. Prenatal findings were correlated to anatomic postnatal examinations performed by a paediatric plastic surgeon. Results Forty-three cases of cleft palate without cleft lip were prenatally diagnosed, of whom 34 were associated with malformations. We defined four types of disruptive appearances: isolated nonvisualization of the posterior nasal spine; partial-disruption or cleft velum; complete disappearance or V-shaped cleft palate; and complete disappearance or U-shaped cleft palate. The adjusted kappa coefficient, between prenatal and postnatal evaluation, was 0.88 (95% CI: 0.79-0.97), corresponding to an excellent agreement. Conclusions Using a strictly axial transverse ultrasound view, visualization of the secondary fetal palate enables to diagnose a cleft palate without cleft lip. This method offers a prenatal anatomic classification of cleft palate with a high level of concordance to postnatal findings.

BACKGROUND: Sebaceous nevus is a congenital malformation of the skin that usually occurs on the scalp or face. Syndromic forms do rarely exist with associated cerebral and ocular malformations. The skin lesions are pale at birth and become irregular by puberty. In the adult patient, tumors (usually benign) develop from sebaceous nevus. Their surgical excision during childhood can give a better result in terms of the definitive scar. OBJECTIVES: The aim of this study is to analyze our cases of syndromic sebaceous hamartoma, perform a review of the existing literature, and propose guidelines for the therapeutic plan. METHODS: This is a retrospective study reviewing the cases of syndromic sebaceous nevus treated in the Department of Orthopedic Plastic Pediatric Surgery in Montpellier, France, and the Department of Pediatric Surgery in Lausanne, Switzerland, between 1994 and 2016. RESULTS: The files of six patients with syndromic sebaceous nevus were analyzed. The average age at the first consultation was 4 months. The location was craniofacial in all cases. Cerebral radiological imaging was performed on all patients; two showed abnormal findings. Four patients underwent ophthalmic examination, which all revealed abnormalities. Three patients had other associated malformations. Three patients presented with epilepsy or learning difficulties in the course of follow-up. CONCLUSION: All patients presenting with extensive sebaceous nevus of the craniofacial region should benefit from cerebral imagery and ophthalmic examination since there is a very high probability of associated abnormalities. The developmental problems encountered could not be definitively associated with the skin malformations. © 2018 The International Society of Dermatology.

International audience; Cephalohematoma is a common pathology in newborns. Observation is the primary treatment for most patients with small uncomplicated cephalohematoma. Conversely, a large cephalohematoma can lead to calcification with unesthetic local deformation or deformational plagiocephaly. The objective of the study was to evaluate the iatrogenic risk associated with early puncture under local anesthesia and oral sucrose. This is a retrospective study of 67 consecutive newborns followed at Montpellier University Hospital, France, between 2010 and 2017. Large cephalohematoma was defined on the basis of the bump projection. Due to the uncertainty of the spontaneous resorption and the risk of calcification after 4 weeks which render the needle aspiration ineffective, puncture was performed between 2 and 4 weeks of life after coagulation evaluation and ultrasound of the skull and scalp. Puncture was performed in 43 boys (64%) and 24 (36%) girls between day 15 and day 30 after birth. The cephalohematoma maximal projection measured by ultrasound ranged from 9 to 13 mm (Q1,Q4) with a median value of 12 mm. No puncture-related complication was recorded during the intervention and at the 1-month follow-up visit.Conclusion: In newborns with large and persistent unesthetic cephalohematoma, puncture under local anesthesia with oral sucrose can be safely proposed between day 15 and day 30 after birth.What is Known:• Infant cephalohematoma is a frequent pathology of newborns, consisting of a traumatic subperiosteal hematoma of the skull. Most cephalohematomas are small and require no treatment because they spontaneously disappear within the first month.• Large and non-resorptive cephalohematomas may have significant esthetic and functional consequences.What is New:• Early puncture under local anesthesia is a safe, effective, and rapid procedure, decreasing the risk of persistent skull deformities.• Puncture can be proposed for newborns with a large (high projection and/or high angle connection) persistent anesthetic cephalohematoma, between day 15 and day 30, before spontaneous calcification.

Background In patients with alveolar cleft, alveolar bone graft is usually performed before the permanent maxillary canine or incisor eruption and using cancellous bone harvested from the iliac crest. The authors sought to compare the early complication rate in patients who received cancellous bone graft alone and cortical bone graft in addition to cancellous bone to reconstruct the nasal floor. Methods For this observational, retrospective, monocentric study, patients with alveolar cleft who underwent autologous alveolar bone graft and gingivoperiosteoplasty at Montpellier Hospital were divided into two groups: group A, who had cortical and cancellous bone graft; and group B, who had only cancellous bone graft. Both groups were followed for 6 months after surgery, and complications were recorded. Results In total, 319 grafts (group A, 163; group, 156) were performed from June 1999 to May 2016. The major complication rates were 8% and 5.8% in group A and B, respectively, and were not significantly different (p = 0.52). Similarly, the rates of minor complications and of donor site morbidity were not different between groups. Conclusions This preliminary study shows that adding a cortical bone graft for the nasal floor reconstruction does not increase the risk of complications in 6 months following the graft. Long-term benefits such as alveolar height and a piriform aperture symmetrisation will have to be analysed to study the contribution of the technique.

International audience; Median cleft of the upper lip (MCL) is a specific and rare entity on the spectrum of facial clefts. MCL have different clinical expressions and can be either isolated or part of multiple malformations. Confusion still exists regarding the explanation and classification of MCL; some cases have been reported in the literature, but no studies carried out a complete review of the literature. This study reviewed cases of MCL in 2 French units and conducted a systematic review of the literature, in order to derive a new classification. Fourteen patients with MCL in the 2 units and 195 cases in the literature were reviewed. They involved complete (42%), incomplete (49%), and minor forms (9%). Epidemiological and clinical data were collected, from which a classification was derived, based on the type of cleft and its belonging to other syndrome(s). Three main groups were distinguished, namely, isolated MCL, MCL within craniofacial malformations, and MCL with extrafacial malformations. Each group and subgroup was associated with a prognosis and led to specific management. This study reviewed all of the various forms of MCL and their associated anomalies, in order to have a global view of MCL and to derive a useful classification scheme to guide management of care.

The authors present the management of a young female patient who presented with longstanding bilateral calf muscle hypertrophy, with no known cause. Taking into account the patient's wishes and the fact that the hypertrophy was mainly located in the posteromedial compartment, we chose to carry out a subtotal bilateral resection of medial gastrocnemius muscles. This procedure was performed with an harmonic scalpel, permitting a excellent cosmetic result while avoiding complications or functional impairment. After a reviewing of the commonly used techniques, the authors discuss the chosen surgical approach taking into account its clinical particularity.

International audience; The risk of maxillary permanent canine retention is elevated in children with cleft lip and palate (CLP). This study compared this risk in 10 ± 1 year-old patients with unilateral CLP (UCLP) who underwent or not early maxillary bone grafting at the age of 6 ± 1 years.METHODS:Retrospective, controlled monocentric and observational study with the following inclusion criteria: patients with UCLP, isolated cleft, pre-surgery orthodontic maxillary expansion, same surgical protocol, same surgical team. The risk of maxillary canine retention was evaluated using a dental panoramic radiograph, on the basis of the canine root development stage and of the angle (> or

Objectives To bring a neonatal classification system of unilateral cleft lip and palate and to correlate this classification with the distribution of the permanent lateral incisor and maxillary growth. Design Retrospective with longitudinal follow-up. Setting Tertiary. Patients A total of 112 individuals with treated unilateral cleft lip and palate and 30 controls. Main Outcome Measures Unilateral cleft lip and palate neonatal casts were classified anatomically in four categories, in which Class 1 corresponds to a maxillary arch with a narrow alveolar cleft; Class 2 corresponds to a balanced form; Class 3 corresponds to a wide cleft and short maxilla; and Class 4 corresponds to a wide cleft and long maxilla. The classification was correlated with the distribution of the permanent lateral incisor. Maxillary growth was evaluated using a cephalometric analysis after the age of 10 years. Results Clinical classification of unilateral cleft lip and palate found 10 cases of Class 1 (8.9%), 34 cases of Class 2 (30.4%), 46 cases of Class 3 (41.1%), and 22 cases of Class 4 (19.6%). The permanent lateral incisor was most often present in narrower clefts (Classes 1 and 2); whereas, large clefts (Classes 3 and 4) were relatively more frequently associated with an agenesis of the permanent lateral incisor ( P = .019). Maxillary growth impairment was most severe in Class 3, with a mean sella-nasion-A point angle at 71.9° ± 4.6° ( P < .001). Conclusions Using the cleft width, arch form, and shape of the nasal septum, unilateral cleft lip and palate can be classified into four different classes at birth, which can all give information about permanent lateral incisor agenesis and maxillary growth.

Introduction Nous rapportons 2 observations d’hemangiomes infantiles (HI) multiples de disposition Blaschko-lineaire. Observations Un garcon âge de 16 jours etait vu pour des HI multiples presents depuis la naissance. L’examen clinique relevait 9 HI plans, rouges avec halo clair peripherique d’une taille de 4 a 15 mm de grand axe. Les lesions siegeaient sur la face laterale gauche du cou, la region temporale et parietale inferieure homolaterale suivant une disposition en courbure blaschkoide ( Fig. 1 ). L’IRM encephalique et l’echographie hepatique etaient normales. Les lesions d’HI palissaient progressivement les 6 mois suivants. Un garcon âge de 2 mois etait adresse pour des HI multiples du membre superieur gauche (G) developpes depuis l’âge de 8 jours. L’examen clinique attestait de la presence de 11 HI superficiels, tubereux, d’une taille variable entre 1 a 2 cm de grand axe. Ils siegeaient sur la face externe de l’avant-bras G, le coude et la face posterieure du bras homolateral suivant une disposition Blaschko-lineaire en forme de « S » inverse. Les explorations complementaires suivantes etaient normales : echographie cardiaque, ECG, radiographie thoracique et echographies hepatique et cerebrale avec doppler ( Fig. 2 ). Les lesions d’HI s’affaissaient et palissaient progressivement les 6 mois suivants. Discussion Nous rapportons les observations d’HI distribues de facon lineaire suivant les lignes de Blaschko. Dans le premier cas, le caractere plan des lesions et leur regression progressive plaidaient en faveur d’HI a croissance minime malgre leur presence des la naissance. La disposition lineaire sur le cou et la region temporo-parietale ne correspondait pas a une distribution anatomique neurologique, vasculaire ou suivant les lignes de fusion des placodes faciales. En revanche, elle suivait strictement les lignes de Blaschko de l’extremite cephalique. Cette observation questionne la validite de l’hypothese physiopathologique uniciste d’une disposition des HI faciaux segmentaires et focaux en regard des placodes faciales ou de leurs lignes de separation embryologique, ces dernieres etant relativement superposables en direction avec les lignes de Blaschko cephalique. Dans le second cas, la disposition en courbure en « S » inverse suivait egalement strictement les lignes de Blaschko et ne correspondait pas a au trajet anatomique de l’artere brachiale et de ses branches radiale et ulnaire, ni a des aires de suppleance vasculaire embryologique en l’absence de caractere segmentaire. Ces observations accreditent le role predominant d’une anomalie en mosaique des cellules ectodermiques issues de la crete neurale et de leur role direct dans la proliferation des angioblastes d’origine mesodermique et la migration des HI. Conclusion Nous rapportons les premieres observations d’HI multiples suivant les lignes de Blaschko accreditant le role potentiel predominant des cellules de la crete neurale dans la genese des HI.

Plexiform neurofibroma is a rare and benign tumor often associated with type 1 neurofibromatosis (NF1) or Von Recklinghausen's disease. Present in one third of cases of NF1, there are isolated forms where the diagnosis should remain a diagnosis of exclusion. We report four cases of isolated plexiform neurofibromas found in children from topographies and we discuss the pretherapeutic assessment, the surgical management and the long-term follow-up.

International audience; BACKGROUND:Hair collar sign (HCS) and hair tuft of the scalp (HTS) are cutaneous signs of an underlying neuroectodermal defect, but most available data are based on case reports.OBJECTIVE:We sought to define the clinical spectrum of HCS and HTS, clarify the risk for underlying neurovascular anomalies, and provide imaging recommendations.METHODS:A 10-year multicenter retrospective and prospective analysis of clinical, radiologic, and histopathologic features of HCS and HTS in pediatric patients was performed.RESULTS:Of the 78 patients included in the study, 56 underwent cranial and brain imaging. Twenty-three of the 56 patients (41%) had abnormal findings, including the following: (1) cranial/bone defect (30.4%), with direct communication with the central nervous system in 28.6%; (2) venous malformations (25%); or (3) central nervous system abnormalities (12.5%). Meningeal heterotopia in 34.6% (9/26) was the most common neuroectodermal association. Sinus pericranii, paraganglioma, and combined nevus were also identified.LIMITATIONS:The partial retrospective design and predominant recruitment from the dermatology department are limitations of this study.CONCLUSIONS:Infants with HCS or HTS are at high risk for underlying neurovascular anomalies. Magnetic resonance imaging scans should be performed in order to refer the infant to the appropriate specialist for management.Copyright © 2016 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

Objective To investigate the accuracy of prenatal axial three-dimensional (3D) ultrasound in predicting the absence or presence of cleft palate in the presence of cleft lip. Methods Between March 2005 and January 2009, there were 81 cases with a prenatal two-dimensional (2D) ultrasound screening diagnosis of unilateral or bilateral cleft lip at 22–25 weeks of gestation referred to our tertiary care center. Of these, 79 fetuses were included in this prospective study and two were excluded. Axial 3D ultrasound imaging of the fetal palate was performed and the diagnoses were compared with clinical findings at delivery. The frequencies of intact and cleft palate, the degree of association between the prenatal predictions and postnatal findings and the probability of detection of cleft lip and palate were determined. Results Of 79 prenatal predictions, 77 (97%) were correct and the association between the prenatal predictions and postnatal findings was strong. The sensitivity for detection of cleft lip and palate within this high-risk population was 100% and the specificity was 90%. In one of the excluded cases, the palate could not be visualized due to a fetal prone position. There were chromosomal anomalies in 4% of cases and associated structural or growth anomalies in 23%, termination of pregnancy was carried out in 4% and intrauterine fetal demise occurred in 3%. Conclusion Axial 3D ultrasound of the fetal palate has high accuracy in identifying prenatal cleft palate when cleft lip is diagnosed at mid-trimester 2D ultrasound screening. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd.

Introduction Depuis 2015, le propranolol oral (Pr) est le traitement de 1re intention des hemangiomes infantiles (HI). Il existe quelques rares cas d’HI resistants a 3 mg/kg/j (IHRPr3), pour lesquels il peut etre necessaire d’augmenter la dose a > 3 mg/kg/j. L’interet de cette dose augmentee n’a jamais ete evalue. Materiel et methodes Etude observationnelle, retrospective, multicentrique. Inclusion de tous les IHRPr3 traites a > 3 mg/kg/j entre 2008 et 2018. Recueil des informations cliniques par questionnaire standardise et photographies. Reponse de l’HI a 3 et > 3 mg/kg/j evaluee par son volume, cote par IGA (Improvement Global Assessment) : −1 = augmentation, 0 = stabilite, 1 = diminution mediocre, 2 = diminution significative. Objectifs Estimer la prevalence des IHRPr3 (definis par IGA de −1 a 1 apres ≥ 5semaines a 3 mg/kg/j), estimer le pourcentage de IHRPr3 devenant repondeurs a > 3 mg/kg/j (IGA 2), identifier des facteurs predictifs de reponse favorable (IGA 2 vs IGA de −1 a 1 a > 3 mg/kg/j, Fischer & Mann Whitney, α = 5 %), decrire la tolerance a > 3 mg/kg/j. Resultats Quinze patients (12 F/3 H) inclus (9 centres). Prevalence : 0,45 %. 3 profils de IHRPr3 : (A) 47 % HI segmentaires du visage, mixtes ou superficiels (71 % sur la zone de la barbe, 29 % avec HI sous-glottique [HSG]) ; (B) 40 % HI du visage, localises, mixtes ou profonds ; (C) 13 % HI du corps, localises, mixtes, avec ulceration ne cedant pas sous traitement. Dose > 3 mg/kg/j utilisee : de 3,5 a 4 mg/kg/j. IGA 2 a > 3 mg/kg/j : 43 %. Facteurs predictifs identifies : IGA 1 a 3 mg/kg/j (p = 0,026) et duree de traitement prolongee (totale : p = 0,029, par palier de dose : p = 0,043). Un cas particulier : HI segmentaire du visage + HSG avec IGA −1 a 3 et > 3 mg/kg/j, malgre un long traitement (20,5 mois). Bonne tolerance a > 3 mg/kg/j, EI connus et de faible intensite. Discussion Premiere serie de IHRPr3 traites a > 3 mg/kg/j (7 cas de IHRPr3 dans la litterature, pas de donnees a > 3 mg/kg/j). L’augmentation a > 3 mg/kg/j a permis 43 % de reponse favorable, avec un profil de tolerance correct, des lors que les IHRPr3 etaient traites longtemps et repondaient deja partiellement a 3 mg/kg/j. Hypotheses pour les IHRPr3 restant en echec (partiel ou total) a > 3 mg/kg/j : phase de proliferation explosive et prolongee, insuffisamment controlee par Pr, meme a dose augmentee ? Diagnostic errone ? Differentiation ou composante adipeuse precoce ? HI totalement resistant au Pr ? Perturbation d’une etape pharmacocinetique (effet de 1er passage hepatique) ? Probleme d’observance ? Conclusion Une dose > 3 mg/kg/j semble une option a envisager en cas de IHRPr3. Ces IHRPr3 sont tres rares (prevalence de 0,45 %). Des etudes ulterieures prospectives avec donnees echo-doppler (quantification de l’activite vasculaire des IHRPr3) et dosages plasmatiques de Pr (relation PK/PD) pourraient etre conduites pour approfondir les connaissances au sujet des IHRPr3 restant en echec de traitement.

Objectives To describe a three-dimensional (3D) ultrasound technique for assessing the fetal soft palate. Methods A prospective study conducted from April to December 2006 included 87 fetuses in a low-risk population. Fetal ultrasound scans were performed between 21 and 25 weeks of gestation to determine the normal 3D ultrasound view of the fetal soft tissues of the palate. The sonographers used a 30°-inclined axial 3D view of the fetal palate. Ultrasound images obtained in this view were compared with fetopathological specimens of the same gestational age by two observers, both pediatric surgeons. Each observer indicated whether they thought that the uvula or the velum could be detected, and the differences in responses between the observers were assessed. Results The frequencies of detection of the uvula and velum of each observer varied between 80% and 90%. The observers showed a significant difference in judging the visualization of the uvula, but not in judging the velum. Conclusions A 30°-inclined axial 3D ultrasound view seems to be effective in assessing the integrity of the fetal soft palate. Copyright © 2008 ISUOG. Published by John Wiley & Sons, Ltd.

The objective of this case report is to evaluate the faculty of a recently described original three-dimensional ultrasound technique to detect pathological processes of the fetal palate. The palate of the fetus of a patient with a personal history of isolated incomplete cleft palate is evaluated by three-dimensional ultrasound at 34 weeks of gestation. The postnatal findings are compared to the prenatal investigation. The used three-dimensional ultrasound technique provides selective visualization of the total hard palate which permits the prenatal diagnosis and multidisciplinary approach of an incomplete cleft palate. These prenatal findings might encourage further studies to confirm the value of this recently described innovative technique in the diagnosis and surgical prenatal counselling of fetal cleft palate.

Resume Sujet. – La scaphocephalie est une craniostenose frequente secondaire a la synostose de la suture sagittale. Malgre cette pathogenie univoque il existe un grand nombre de variations morphologiques dont la systematisation reste imprecise. Le but de cette etude est d'analyser les differentes formes morphologiques de scaphocephalies a l'aide du scanner avec reconstruction tridimensionnelle. Materiel et methode. – Cette etude retrospective a ete realisee a partir de l'analyse morphometrique de 42 scanners 3D (dix filles et 32 garcons). Les scaphocephalies ont ete classees en deux groupes suivant la systematisation de Stricker et Montaut : les sphenocephalies (front large) et les leptocephalies (front etroit). En plus des variations de forme de la calvaria, plusieurs index crâniens ont ete calcules. Resultats. – Il y avait 30 sphenocephalies et 12 leptocephalies. En dehors de la deformation du front il existait une difference morphometrique du pole posterieur qui etait plus bascule en arriere dans les leptocephalies. Dans les deux cas on retrouvait des zones de striction parietale retrocoronale (40,5 %) ou prelambdoide (9,5 %) independamment du degre de synostose sagittale. Conclusions. – Les donnees morphometriques confirment que la deformation du crâne secondaire a la synostose prematuree peut etre differente au niveau du front et de l'occiput. Ces deformations peuvent etre harmonieuses ou s'accompagner d'une striction retrocoronale responsable d'un retrecissement bitemporal ou d'une striction prelambdoide responsable d'un chignon occipital. Cela amene a nuancer la correction chirurgicale pour s'adapter aux differentes morphologies.

Congenital nasal pyriform aperture stenosis may be isolated or associated with other midline anomalies. The aim of the study was to describe the measurements and features of the interorbital structure and midface in congenital nasal pyriform aperture stenosis. The computed tomography scans of eight patients (two girls and six boys) were retrospectively reviewed. Several distances were obtained at the orbital and midface levels and compared with normative data. The average width of the pyriform aperture was 5.5 mm +/- 1.6, and there was overgrowth of the pyriform aperture rim (nasal process of the maxilla). No skeletal anomalies were present other than the congenital nasal pyriform aperture stenosis. It was associated with a single median maxillary central incisor in two cases. The anterior and lateral interorbital distances were normal (17.4 mm and 65.4 mm, respectively). The midface was not hypoplastic. There was no brain malformation. Congenital nasal pyriform aperture stenosis is a localized dysostosis of the pyriform aperture rim without interorbital or midface hypoplasia. It may be associated with a single median maxillary central incisor but cannot be viewed as a minor form of holoprosencephaly.

Resume Le but de cette etude etait d'analyser les mecanismes de la deformation crânienne rencontree dans les plagiocephalies frontales et occipitales sans synostose. Population et methodes. – De 1987 a 2001, 96 cas de plagiocephalies sans synostose touchant 72 garcons et 24 filles, ont ete examines. Il s'agissait de 72 plagiocephalies frontales (PF) et de 24 plagiocephalies occipitales (PO). L'examen clinique des muscles du cou a ete realise a la recherche d'un torticolis avec ou sans retraction. Tous les patients ont eu un scanner 3D du crâne pour eliminer une craniostenose. L'index cephalique (IC) etait mesure. Resultats. – Un torticolis etait retrouve dans 89,3 % des PF. Il etait associe a une retraction musculaire (54,8 %), a une asymetrie de tonus (16,6 %) ou aux deux (28,6 %). Le crâne avait une deformation parallelepipedique de grand axe oblique et l'IC etait egale a 0,85. Un torticolis etait present dans une PO sur deux, associe a une retraction musculaire (28,6 %), a une asymetrie de tonus (57,1 %) ou aux deux (14,3 %). Le crâne etait carre avec un meplat occipital et l'IC etait de 0,95. Discussion. – Les PF et PO sont deux deformations dont les mecanismes sont differents. Les PF correspondent a une asymetrie du crâne dans les trois plans de l'espace secondaire a des forces de tractions musculaires exercees sur la base du crâne et les PO correspondent a une asymetrie localisee de l'arriere crâne secondaire a des forces de compression. Conclusion. – La deformation par traction et/ou compression s'installe in utero ou chez le nouveau-ne et le decubitus dorsal est un facteur positionnel aggravant.

Resume Introduction. – Le but de cette etude est de revoir les modalites techniques et les indications de la greffe osseuse secondaire dans les fentes labio-maxillo-palatines (FLMP). Materiel et methode. – Soixante-deux greffes osseuses secondaires ont ete revus retrospectivement de 1993 a 2000. Il y avait 50 FLP unilaterales et 12 FLP bilaterales. L'âge au moment de la greffe osseuse va de 9 ans a 25,5 ans, avec une moyenne de 14,34 ± 2,9 ans. Le meme operateur a pratique une GingivoPeriOsteoplastie (GPO) dans tous les cas et le greffon utilise etait de l'os spongieux de crete iliaque dans tous les cas. Resultats. – Il existe trois types d'indications de la greffe osseuses. Premierement la greffe osseuse interceptive (50 %) : elle a ete realisee avant le traitement orthodontique a 12,8 ± 2,3 ans. Elle apporte un support osseux pour les dents de bord de fentes. Deuxiemement la greffe de stabilisation (33,8 %) : elle stabilise le traitement orthodontique et/ou pour preparer une chirurgie orthognathique (15,2 ± 1,6 ans). Troisiemement la greffe de comblement (16,2 %) : c'est une greffe secondaire tardive en fin de croissance (17,3 ± 3,6 ans) de correction des sequelles. Elle restaure la continuite du maxillaire, stabilise les dents de bord de fente et prepare une rehabilitation prothetique. Conclusion. – Les avantages de la greffe osseuse secondaire avec GPO sont nombreux et elle fait partie du protocole de traitement des patients porteurs d'une FLMP. Un suivi multidisciplinaire est necessaire pour poser l'indication.

Management of labio-maxillo-palatine clefts has two major requirements: to constitute a multidisciplinary staff (surgical, phonological, orthodontical) intervening as soon as possible and determination of a precise therapeutical chronology not only for primary surgery but also for sequellae. Primary surgical protocol is in cases of total clefts these defined by Malek and Psaume; and for pure labial or incomplete clefts, we perform a neonatal surgery. Integration of interceptive correction of sequellae into thus protocol appears basic: correction of alveolar cleft by gingivoplasty (5 to 7 years) associated with secondary home-graft between 11 to 13 years; early nasal revision since 2 years for functional and aesthetic reasons. Early control of speech development, otologic problems and their management appears a very important point. Introduction of the concept of maxillary distraction appears to us a very important improvement for correcting orthognatic cases with major problems of squeletical growth. Recent introduction of the antenatal diagnosis introduces a new concept in psychological approach of these cases. It is necessary to establish a network for managing these cases since the antenatal period.

International audience; BACKGROUND:Reticular infantile hemangioma (RIH) with minimal or arrested growth (MAG) is an underrecognized variant of infantile hemangioma (IH).OBJECTIVE:We describe a new clinical subtype of RIH-MAG associated with lipoatrophy.METHODS:The medical charts and serial clinical photographs and imaging studies of 53 children given a diagnosis between 2004 and 2013 labeled as "abortive," "minimal or arrested growth," "reticular," or "congenital" hemangiomas were reviewed in the departments of dermatology and infantile plastic surgery of 2 French university hospital centers.RESULTS:Seven children were identified with RIH-MAG associated with lipoatrophy. There were 3 main patterns: (1) a focal cutaneous depression; (2) semicircular lipoatrophy with a horizontal bandlike depression; and (3) segmental soft-tissue atrophy. One case of segmental RIH-MAG of the back of the foot was associated with genital abnormalities reminiscent of perineal hemangioma, external genitalia malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, and skin tags (PELVIS) syndrome.LIMITATIONS:The limitations were the retrospective study design, the small number of cases, and selection bias based on recall and photography.CONCLUSION:RIH-MAG with lipoatrophy is an uncommon variant of IH that may be confused with other vascular anomalies, particularly capillary malformation, focal and atrophic cutis marmorata congenita telangiectatica, and rapidly involuting congenital hemangioma with lipoatrophy as a sequela.

International audience; Maxillary distraction osteogenesis (DO) is a reliable treatment for severe maxillary deficiency in cleft lip and palate (CLP). The objective was to analyze its long-term effects on the mandible. A retrospective study of 24 CLP treated with maxillary DO using the Polley and Figueroa technique was done; patients were followed for more than 4 years. Preoperative (T0), 6–12 months postoperative (T1), and ≥4 years postoperative (T2) cephalometric radiographs were evaluated. A classical cephalometric analysis was used to assess treatment stability, and a Procrustes superimposition method was used to assess local changes in the shape of the mandible. The mean age of patients at T0 was 15.4 ± 4.1 years. SNA increased at T1 and T2 (P < 0.001), with no significant relapse between T1 and T2, indicating stability at 1 year after treatment (T0 = 72.4 ± 5.3°; T1 = 81.3 ± 6.2°; T2 = 79.9 ± 6.1°). SNB, facial angle, gonial angle, and symphyseal angle remained stable. Long-term analysis of the mandible demonstrated a minimal counter-clockwise rotation of the body (mandibular plane = −0.2 ± 3.2°) and ramus (−0.6 ± 4.3°). Maxillary DO in CLP had no significant effect on the shape or rotation of the mandible. The maxillary advancement remained stable after 1 year.

Resume L'imagerie moderne a ouvert une possibilite d'exploration precise des syndromes oto-mandibulaires. Les techniques d'exploration par IRM et CT Scan et les logiciels de traitement d'images qui sont adjoints a ces techniques (tridimensionnel, superposition ou effacement des structures) ont permis de realiser une veritable dissection anatomique de l'ensemble de ces syndromes malformatifs. L'etude des anomalies squelettiques gagne en precisant les troubles de developpement des divers elements de la mandibule : articulation temporomandibulaire, branches verticale et horizontale, ainsi que des elements crânio-maxillaires : structures orbitaires, base du crâne enfin, l'etude des muscles montre des atteintes majeures correlees aux troubles squelettiques et realisant un gradient de gravite morphologique et fonctionnelle. Cette etude analytique permet de degager des axes malformatifs : axe malaire pour les dysostoses oto-mandibulaires, axe temporo-mandibulaire dans les microsomies hemifaciales. L'imagerie moderne va, par l'introduction d'analyse cephalometrique tridimensionnelle, permettre une appreciation precise des troubles de la croissance et des resultats post-therapeutiques. Enfin, l'utilite de l'imagerie dans la modelisation du traitement et de l'ouverture vers la chirurgie aidee par ordinateur va permettre d'apporter une base solide aux techniques d'osteotomie et surtout de distraction.

International audience; The objective was to analyze the effects of growth on the long-term result of maxillary distraction osteogenesis (DO) in cleft lip and palate (CLP). Retrospective study of 24 CLP cases with long-term follow-up operated for maxillary DO using the Polley and Figueroa technique: 10 patients were distracted during growth, while 14 patients were operated after their growth spurt. Preoperative (T0), 6-12 months postoperative (T1), and ≥4 years postoperative (T2) cephalometric radiographs were evaluated. A classical cephalometric analysis was used to assess the treatment stability, and a Procrustes superimposition method was performed to assess local changes in the maxilla and the mandible. At T0, the mean age was of 11.9 ± 1.4 years for growing patient, and 17.9 ± 3.5 years for patient treated after their growth spurt (P

Objectives To bring a neonatal classification system of unilateral cleft lip and palate (UCLP) and to correlate this classification with the distribution of the primary lateral incisor. Design Retrospective with longitudinal follow-up. Setting Tertiary. Patients One hundred twenty-one patients with treated UCLP. Thirteen plaster casts were used as controls. Main Outcome Measures The UCLP patients were classified anatomically into four categories: class 1 corresponds to a maxillary arch with a narrow alveolar cleft, class 2 corresponds to a balanced form, class 3 corresponds to a wide cleft and short maxilla, and class 4 corresponds to a wide cleft and long maxilla. Clinical validity was evaluated with a concordance analysis (intra- and interexaminer). This anatomical classification was also corroborated with an automatic classification determined by morphometric parameters measured on neonatal maxillary plaster casts. The class was finally correlated with the distribution of the primary lateral incisor. Results Clinical classification of UCLP found 12 cases of class 1 (9.9%), 36 cases of class 2 (29.8%), 47 cases of class 3 (38.8%), and 26 cases of class 4 (21.5%). The clinical classification was validated with a good intra- and interexaminer concordance analysis (κ > .6). The automatic classification was close to the clinical classification in 84%. The correlation was ideal in class 1 (100%), almost perfect in class 4 (92%), but lower for class 2 (74%) and class 3 (70%). The primary lateral incisor was usually duplicated in class 1, whereas class 2 and class 4 were correlated with a primary lateral incisor located on the lateral palatal segment. Class 3 was associated with an agenesis of the primary lateral incisor ( P < .001). Conclusions UCLP can be classified into four different classes at birth, which can all give information about the inherent tissue hypoplasia and the distribution of the primary lateral incisor.

We compared the early speech outcomes of 40 consecutive children with complete unilateral cleft lip and palate (UCLP) who had been treated according to different 2-stage protocols: the Malek protocol (soft palate closure without intravelar veloplasty at 3 months; lip and hard palate repair at 6 months) (n = 20), and the Talmant protocol (cheilorhinoplasty and soft palate repair with intravelar veloplasty at 6 months; hard palate closure at 18 months) (n = 20). We compared the speech assessments obtained at a mean (SD) age of 3.3 (0.35) years after treatment by the same surgeon. The main outcome measures evaluated were acquisition and intelligibility of speech, velopharyngeal insufficiency, and incidence of complications. A delay in speech articulation of one year or more was seen more often in patients treated by the Malek protocol (11/20) than in those treated according to the Talmant protocol (3/20, p = 0.019). Good intelligibility was noted in 15/20 in the Talmant group compared with 6/20 in the Malek group (p = 0.010). Assessment with an aerophonoscope showed that nasal air emission was most pronounced in patients in the Malek group (p = 0.007). Velopharyngeal insufficiency was present in 11/20 in the Malek group, and in 3/20 in the Talmant group (p = 0.019). No patients in the Talmant group had an oronasal fistula (p < 0.001). All other outcomes were similar. Despite later closure of the soft and hard palate, early speech outcomes were better in the Talmant group because intravelar veloplasty was successful and there were no fistulas after closure of the hard palate in 2 layers. © 2013 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

International audience; Klippel-Trenaunay syndrome (KTS) is characterized by the triad of cutaneous capillary malformations (port-wine stains), asymmetrical disturbed growth of soft tissues and/or bone, and venous and lymphatic malformations. Prolonged and recurrent cutaneous bleeding from ulceration of the capillary and/or venous and/or lymphatic malformation can be life-threatening. We report for the first time to our knowledge a case of Klippel-Trenaunay syndrome treated by oral sirolimus.

Introduction: Treacher Collins syndrome (TCS) is a severe and complex craniofacial malformation affecting the facial skeleton and soft tissues. The palate as well as the external and middle ear are also affected, but his prognosis is mainly related to neonatal airway management. Methods of zygomaticoorbital reconstruction are numerous and currently use primarily autologous bone, lyophilized cartilage, alloplastic implants, or even free flaps. This work developed a reliable “customized” method of zygomatico-orbital bony reconstruction using a generic reference model tailored to each patient. Methods: From a standard computed tomography (CT) acquisition, we studied qualitatively and quantitatively the skeleton of four individuals with TCS whose age was between 6 and 20 years. In parallel, we studied 40 controls at the same age to obtain a morphometric database of reference. Surgical simulation was carried out using validated software used in craniofacial surgery. Results: The zygomatic hypoplasia was very important quantitatively and morphologically in all TCS individuals. Orbital involvement was mainly morphological, with volumes comparable to the controls of the same age. The control database was used to create three-dimensional computer models to be used in the manufacture of cutting guides for autologous cranial bone grafts or alloplastic implants perfectly adapted to each patient’s morphology. Presurgical simulation was also used to fabricate custom positioning guides permitting a simple and reliable surgical procedure. Conclusions: The use of a virtual database allowed us to design a reliable and reproducible skeletal reconstruction method for this rare and complex syndrome. The use of presurgical simulation tools seem essential in this type of craniofacial malformation to increase the reliability of these uncommon and complex surgical procedures, and to ensure stable results over time.

Treacher Collins syndrome (TCS) is a severe and complex craniofacial malformation affecting the facial skeleton and soft tissues. The palate as well as the external and middle ear are also affected, but his prognosis is mainly related to neonatal airway management. Methods of zygomatico-orbital reconstruction are numerous and currently use primarily autologous bone, lyophilized cartilage, alloplastic implants, or even free flaps. This work developed a reliable "customized" method of zygomatico-orbital bony reconstruction using a generic reference model tailored to each patient.From a standard computed tomography (CT) acquisition, we studied qualitatively and quantitatively the skeleton of four individuals with TCS whose age was between 6 and 20 years. In parallel, we studied 40 controls at the same age to obtain a morphometric database of reference. Surgical simulation was carried out using validated software used in craniofacial surgery.The zygomatic hypoplasia was very important quantitatively and morphologically in all TCS individuals. Orbital involvement was mainly morphological, with volumes comparable to the controls of the same age. The control database was used to create three-dimensional computer models to be used in the manufacture of cutting guides for autologous cranial bone grafts or alloplastic implants perfectly adapted to each patient's morphology. Presurgical simulation was also used to fabricate custom positioning guides permitting a simple and reliable surgical procedure.The use of a virtual database allowed us to design a reliable and reproducible skeletal reconstruction method for this rare and complex syndrome. The use of presurgical simulation tools seem essential in this type of craniofacial malformation to increase the reliability of these uncommon and complex surgical procedures, and to ensure stable results over time.

Unintentional postural deformities of the skull have increased in a pseudoepidemic manner in the last 15 years. Although dorsal decubitus and prenatal risk factors can play a role in the genesis of such deformities, we think that a crucial determinant is a postnatal defect of cervical mobility responsible for the infant's posture (ie, positional preference) when supine. Indeed, muscular factors, which limit the range of head and neck movements, have been underestimated in the genesis of skull deformities. Here, we have retrospectively analyzed data from 181 infants with unintentional skull deformities and propose a classification of these deformities into 3 types based on their pathogenic model and clinical appearance: fronto-occipital plagiocephalies due to severe muscle hypertonia in which the myogenic component is the first implicated, occipital plagiocephalies with muscle imbalance due to neurogenic muscle hypertonia, and posterior brachycephalies with neurogenic muscle hypertonia of the suboccipital muscles due to trauma to the occipitovertebral junction. Future studies on the size and density of specific muscles or group of muscles should help us to better understand their involvement in the pathogenesis of postural deformities. Our findings also highlight the importance of carefully assessing cervical mobility during the first week of life to detect possible limitations and to prescribe (if needed) an adapted rehabilitation. Rehabilitation should be associated with postural measures put in place when infants sleep supine to prevent the appearance of skull deformations.

Background Research on the skull base is important to improve our understanding of the growth and development of the modern human skull. To study the growth of the human fetal skull base, we assessed a new geometric morphometric tool, which does not require the use of bone landmarks. Material and methods Seven dry fetal skulls of an estimated gestational age ranging from 15 to 27 weeks were studied. Each skull was scanned using a standard CT scan and the image sets were post-processed to extract volumetric data by segmenting the skull base into predefined regions of interest. Our method of analysis was based on the inertial properties of reconstructed volumes. Results The volumetric study of the skulls highlighted an asynchronous speed of growth between the pre and post-chordal parts of the skull base whose preferential growth are in the vertical and horizontal planes. We also found different speeds of growth in the pre-chordal part depending on the type of ossification (endochondral or membranous). The overall shape of the skull base bones were preserved during the period studied except for the petrous pyramids. The expansion of bone parts was isometric with reference to a central point that was located at the intrasphenoidal synchondrosis. Finally, the analysis of the basicranial angles corroborated data from the literature in the sagittal plane and allowed their study also in the frontal and horizontal planes. Conclusions This three-dimensional volumetric approach is a necessary complement to studies that are performed in the sagittal plane and are based on the identification of landmarks. The geometric morphometric method used by authors permitted to obtain original informations on the growth kinetics and bone tridimensional movements of the human fetal skull base.

The successful management of subglottic hemangioma with propranolol has been reported. We report three cases of subglottic hemangioma treated with the cardioselective beta-blocker acebutolol, 8 mg/kg/ day. Treatment was efficient in two cases while an open procedure was necessary in the third child. In our experience, acebutolol could be easily administered in oral form twice-a-day only with a dose that was adaptable according to the growth of the child and showed no side effects. We also report a case of rebound growth after beta-mimetic drug use and the efficiency of propranolol treatment in such a recurrence. Considering the lack of side effects and the advantages in terms of administration, we suggest acebutolol as a first-line treatment of subglottic hemangiomas for which intervention is required.

Objective To document the epidemiological, clinical, histological and radiological characteristics of aggressive vascular abnormalities of bone in children. Study design Correspondents of the French Society of Childhood Malignancies were asked to notify all cases of aggressive vascular abnormalities of bone diagnosed between January 1988 and September 2009. Results 21 cases were identified; 62% of the patients were boys. No familial cases were observed, and the disease appeared to be sporadic. Mean age at diagnosis was 8.0 years [0.8-16.9 years]. Median follow-up was 3 years [0.3-17 years]. The main presenting signs were bone fracture (n = 4) and respiratory distress (n = 7), but more indolent onset was observed in 8 cases. Lung involvement, with lymphangiectasies and pleural effusion, was the most frequent form of extraosseous involvement (10/21). Bisphosphonates, alpha interferon and radiotherapy were used as potentially curative treatments. High-dose radiotherapy appeared to be effective on pleural effusion but caused major late sequelae, whereas antiangiogenic drugs like alpha interferon and zoledrenate have had a limited impact on the course of pulmonary complications. The impact of bisphosphonates and alpha interferon on bone lesions was also difficult to assess, owing to insufficient follow-up in most cases, but it was occasionally positive. Six deaths were observed and the overall 10-year mortality rate was about 30%. The prognosis depended mainly on pulmonary and spinal complications. Conclusion Aggressive vascular abnormalities of bone are extremely rare in childhood but are lifethreatening. The impact of anti-angiogenic drugs on pulmonary complications seems to be limited, but they may improve bone lesions.

Large perineal hemangiomas can be associated with congenital anomalies, including anorectal, urinary tract, spine, and external genitalia malformations.We describe 2 infants with large sacral and perineal hemangiomas, with severe malformation of the external genitalia with ambiguous genitalia in one case and urinary tract malformations and imperforate anus in the other case. These cases are discussed, along with 9 previously reported cases with similar findings.Large perineal hemangiomas may constitute a distinctive group of associated anorectal, neurologic, renal or urinary tract, and genital defects. We propose the acronym PELVIS syndrome to emphasize the characteristic findings of this syndrome: perineal hemangioma, external genitalia malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, and skin tag.

Dermal substitutes such as Integra® have been used in surgery since 1981. This product was developed to cover large skin burns but it may have a role in reconstructive upper limb surgery. In upper extremity avulsion, the choice of skin cover depends on many variables. Dermal substitute is an alternative when split-thickness skin grafting is appropriate. We present a case of severe upper limb avulsion, or ‘degloving’, in a child of 9 years old which was treated successfully with Integra. Integra® is particulary useful in growing children to cover large skin losses.

lescents souffrent pour la plupart de troubles anxio- depressifs au debut de la prise en charge [9]. La gynecomastie est source d’exclusion sociale et parfois de trouble de l’identite sexuelle. Il parait donc indispensable d’aborder la problematique du vecu des le debut de la prise en charge et de proposer une aide psy-chologique au cas par cas.

ou parce qu’il est « victime » de moqueries iteratives et vexatrices. Parfois la decision peut etre decalee dans le temps meme si la prise de conscience de ce probleme se situe souvent autour de 6 ans. La demande est justifiee par le chirurgien qui evalue le degre des oreilles decollees (minime, moyenne, severe) et le type anatomique de la deformation. Par ailleurs, il faut savoir que les oreilles atteignent entre 8590 % de leur taille adulte a l’âge de 7 ans [2]. En cas de forme minime, d’enfant tres timide, ou de demande emanant essentiellement des parents, il faut savoir refuser l’indication et demander une autre consultation pour reevaluer la demande. La chirurgie des oreilles decollees chez l’enfant

The aim of this study was to perform a morphometric analysis of the skull base to understand the mechanism of asymmetry between malformational and deformational plagiocephaly. Since 1988, we have carried out 102 CT scans with three-dimensional reconstruction of the endocranial side of the skull base. There were 20 cases of malformational plagiocephaly, consisting of 18 unicoronal synostoses (UCS) and two unilambdoid synostoses (ULS), and 82 cases of plagiocephaly without synostosis, made up of 60 cases of frontal plagiocephaly (FP) and 22 of occipital plagiocephaly (OP). The skull base asymmetry was analyzed in three ways: (1) the hemibases and the cranial fossa angles were compared with each other; (2) the structural asymmetry of the chondrocranium was calculated; and (3) the architectural asymmetry was measured in relation to the medio-labyrinthine plane. The skull base asymmetry of malformational plagiocephaly was located in the anterior and middle cranial fossae for UCS and in the posterior and middle cranial fossae for ULS. The asymmetry was the result of a localized structural anomaly of the chondrocranium. The asymmetry of deformational plagiocephaly corresponded to a distortion in relation to the medio-labyrinthine plane while the chondrocranium remained symmetric. The skull base asymmetry of malformational plagiocephaly is secondary to a localized malformation of the chondrocranium, and that of the deformational plagiocephaly is secondary to an architectural deformation.

Objectifs Evaluer les resultats de consultations multidisciplinaires pour des enfants adresses pour troubles de succion-deglutition. Materiels et methodes Analyse des resultats obtenus lors de consultations multidisciplinaires (Orl, orthophoniste +/− chirurgien maxillo-facial) pour 73 enfants adresses pour problemes de succiondeglutition de l’enfant. Resultats L’âge median des enfants etait de 2,5 ans. Les motifs de consultations etaient des difficultes de teter, des fausses routes, un bavage, des difficultes de diversification ou un sevrage de nutrition enterale. L’examen retrouvait des problemes velaires, une hypertrophie amygdalienne ou linguale, des anomalies du temps buccal, des anomalies de propulsion ou des troubles de coordination, des anomalies de la mobilite laryngee et des anomalies de la sensibilite pharyngee avec stases. Un contact a ete pris avec les acteurs de proximite pour 34 enfants. Trente-cinq enfants ont ete orientes (25 vers une orthophoniste, 5 vers un CAMSP et 5 en pedopsychiatrie). Une chirurgie a ete proposee pour 6 enfants. Conclusion La consultation multidisciplinaire permet une evaluation globale du trouble d’alimentation en fonction du developpement de l’enfant et peut orienter vers une prise en charge adaptee elle-meme multidisciplinaire.

Objectives: To evaluate usefulness of sagittal CDUS images for the differential diagnosis of fetal cleft lip (CL) and cleft palate (CP). Methods: We got coronal and axial images of upper lip and maxillary alveolar in 25 fetuses with CL and CP. Existence of defect and malalignment of alveolar was assessed. We assessed existence of hard palate defect on the mid-sagittal image and flow through the defect on CUDS during fetal swallowing. We compared the US findings with postnatal features. We assessed the accuracy of axial and sagittal images in the differential diagnosis of CL and CP. Results: 19 cases had CL and CP. 6 cases had CL without CP. 3 of 6 CL cases had cleft alveolar. Alveolar defect on axial image was detected in 18 of 19 cases with CP and 3 of 6 with CL. Alveolar malalignment was detected in 11 of 19 cases with CP, but in no case with CL. Hard palate defect on the sagittal image was detected in 15 of 19 cases with CP, but in no case with CL. CDUS evaluation of fetal swallowing was possible in 20 of 25 cases. Communicating flow through the palate defect was detected in 14 of 15 cases with CP, but in no case with CL. The accuracy of alveolar defect and malalignment on axial image were 84% and 68%, respectively. Accuracy of hard palate defect and flow on mid-sagittal image were 84% and 95%, respectively. Combined axial and sagittal images correctly diagnosed in all 6 cases with CL and 18 of 19 cases with CP. The accuracy was 96%. Conclusions: Sagittal US evaluation of the fetal hard palate may have additional value in the differential diagnosis of fetal CL and CP.

L’objectif etait de creer et de diffuser, a la demande des professionnels de la perinatalite (sages femmes, gynecologues echographistes, obstetriciens, pediatres, autres) des fiches d’information pratiques sur les principales malformations chirurgicales et leur prise en charge neonatale. Les fiches ont ete realisees dans le cadre du Reseau Perinatal Regional Naitre et Grandir en Languedoc Roussillon. Apres appel a participation des chirurgiens infantiles plusieurs reunions ont ete realisees pour definir les themes d’information et le contenu. Douze fiches d’information ont ete etablies pour servir de referentiel. Elles portent sur les malformations chirurgicales les plus frequentes ou celles qui posent le plus de probleme. Pour chaque fiche le meme plan a ete utilise pour faciliter la lecture. Si necessaire l’iconographie echographique prenatale et l’aspect clinique postnatal a ete insere. Apres validation les fiches seront accessibles de facon securisee sur la plate-forme internet du reseau. Les fiches d’information permettront aux professionnels de la perinatalite d’avoir une conduite a tenir pour mieux informer les familles et orienter la prise en charge du nouveau-ne. Cette demarche a ete realisee dans le cadre d’un reseau regional de soins et son utilite devra etre evaluee.

Sujet Les deformations du crâne sont un motif frequent de consultation en chirurgie plastique pediatrique. Le but est de proposer une classification pathogenique basee sur la limitation de la mobilite cervicale. Materiels et Methodes les donnees de 181 nourrissons atteints de deformations du crâne ont ete revus retrospectivement. Ils ont ete classes en trois types selon l’aspect clinique de la deformation: plagiocephalie fronto-occipitale (PFO), plagiocephalie occipitale (PO) et brachycephalie posterieure (BP). Les facteurs de risques perinataux et les troubles de la mobilite cervicale ont ete analyses. Resultats Dans les PFO il existait principalement une hypertonie musculaire de type myogenique (p Conclusions l’hypertonie musculaire qu’elle soit unilaterale ou bilaterale, de type myogenique ou neurogene semble etre associe preferentiellement a une type de deformation. L’approche preventive et therapeutique devra donc tenir compte du type d’hypertonie musculaire cervicale pour etre la plus efficace.

Objectives: To evaluate the assessment methods for fetal lip/palate on 3D ultrasound with various techniques and examination planes. Methods: Stored 3D volumes of fetuses with postnatal confirmation of cleft lip and/or palate were reviewed. The abnormalities at birth were compared with the antenatal 3D ultrasound findings in the following planes obtained on techniques previously described: 1. The axial plane 2. The sagittal plane 3. The coronal plane 4. Oblique axial plane 5. Oblique sagittal plane 6. Targeted reverse face view Results: There were limitations to each technique or examination plane in antenatal assessment of fetal lip/palate on 3D ultrasound. Different parts of the fetal lip and palate could be visualized with each of the above planes. The anterior part of the palate and the fetal lips are better seen in the axial, sagittal and coronal planes. The oblique axial, oblique sagittal planes and the targeted reverse face view allowed better visualization of the hard and the soft palates. The presence of facial cleft led to a distortion of the anatomy. The use of a combination of these planes allowed a more complete assessment of fetal lip and palate from various angles. Conclusions: The use of a combination of planes and techniques provides more information than the use of a single one in evaluation of fetal cleft lip/palate on 3D ultrasound.

Objective: Cleft palates are still considered as impossible or very difficult to study in 2D or in 3D, and impossible in routine examination. We described a new approach to identify normal palate and it’s pathology. Since 1 year, a new treatment of 3D acquisition can be applied with this method. Method: This easy 2D approach, has been realised with success on every fetuses seen, aged from 18 to 35 Weeks since 2002 (that is to say more than 7000 fetuses), sometimes completed with 3D view. Using precise guide marks, this approach allows a direct very echogenic visualisation of the osseous palate’s posterior wall, proving it’s integrity. Keeping the same guide lines, 3D thick view often visualise the whole palate. Results: With this approach the visualisation of the posterior wall has always been successful and registered. First we could definite criteria of a normal palate. We could also have cleft lips and we could demonstrate the associated cleft palate and the duplication of the uvula. Further we could diagnose cleft palate without cleft lips, short palate (helped with 3D view), and one suspected ogival palate, confirmed with a sagittal view (3D thick view wasn’t available). All these findings have been confirmed by postnatal examination. There hasn’t be any false negative or false positive cases about the integrity of the palate. We described the guidelines we used in this study with 2D and 3D view, the criteria of normal palate, it’s pitfalls, and the sonographic characteristics of cleft and in ogival forms. Conclusion: This study demonstrate than the palate is possible to visualise, even in routine, with a very good prediction of it’s normality or pathology. This will be a great help for parents avoiding the surprise of this pathology or eliminate the cleft palate’s fear. It will be also useful for the surgeon in case of cleft lip for example to explain the parents the more precisely possible what will occur after birth.