M. Bigorre

Classification and pathogenic models of unintentional postural cranial deformities in infants: plagiocephalies and brachycephalies.

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 infants 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.

Speech outcomes of early palatal repair with or without intravelar veloplasty in children with complete unilateral cleft lip and palate

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.

New Neonatal Classification of Unilateral Cleft Lip and Palate Part 2: To Predict Permanent Lateral Incisor Agenesis and Maxillary Growth

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.

New Neonatal Classification of Unilateral Cleft Lip and Palate—Part 1: To Predict Primary Lateral Incisor Agenesis and Inherent Tissue Hypoplasia

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.

The scalp hair collar and tuft signs: A retrospective multicenter study of 78 patients with a systematic review of the literature

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.

Bilateral idiopathic calf muscle hypertrophy: an exceptional cause of unsightly leg curvature.

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 patients 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.

La chirurgie des oreilles décollées chez l’enfant

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