ORAL‐MOTOR DYSFUNCTION AND FEEDING DISORDERS OF INFANTS WITH TURNER SYNDROME

The oral‐motor function of 10 infants with Turner syndrome and their age‐ and sex‐matched controls were assessed during feeding. In addition to well‐recognised dysmorphic features, including oral anomalies and high‐arched palates, index infants had marked hypotonia of the cheeks and lips, dysfunctional tongue movements and poorly developed chewing skills. Their meal‐times were significantly shorter than those of the controls and they weighed significantly less at six, 12 and 15 months. All mothers of infants with Turner syndrome complained of difficulties feeding their children and these problems often had been present since birth. Dysfonction oro‐motrice el troubles alimentaires chez les enfants présentant un syndrome de Turner Les fonctions oro‐motrices de 10 nourrissons présentant un syndrome de Turner et des contrôles appariés pour l'âge et le sexe ont étéétudiés durant l'alimentation. En plus des caractères dysmorphiques bien connus incluant des anomalies orales et un palais à voute élevée, les nourrissons index présentaient une hypotonie des joues et des lèvres, des mouvements de dysfonction de la langue et peu d'habiletéà mâcher. Les temps de repas étaient significativement plus courts que ceux des contrôles et les nourrissons pesaient significativement moins, à six, 12 et 15 mois. Toutes les mères de nourrissons avec syndrome de Turner se plaignaienf des difficultés d'alimentation de leur enfant et ces problèmes existaient souvent depuis la naissance. Oralmotorische Dysfunktion und Fütterungsschwierigkeiten bei Kindern mit Turner Syndrom Bei 10 Kindern mit Turner Syndrom und ihren Kontrollen gleichen Alters und Geschlechts wurde die oralmotorische Funktion beim Füttern untersucht. Zusätzlich zu den gut bekannten Dysmorphiezeichen, einschließlich oraler Anomalien und hohem Gaumen, hatten die Index‐Kinder eine ausgeprägte Hypotonie im Wangen‐ und Lippenbereich, Dysfunktionen der Zungenbewegungen und eine schwach entwickelte Kaufähigkeit, Ihre Mahlzeiten waren signifikant kürzer als die der Kontrollen und sie wogen im Alter von sechs, 12 und 15 Monaten signifikant weniger. Alle Mütter von Turner Kindern klagten über Fütterungsschwierigkeiten und diese Probleme bestanden häufig von Geburt an. Disfunción motora oral y alleraciones de la alimentación en lactantes con síndrome de Turner Se evaluó durante la alimentación el funcionamiento oral motor en 10 nin̄os con síndrome de Turner y en nin̄os control correlacionados por edad y sexo. Además de las caracteristicas dismórficas bien conocidas, incluyendo anomalias orales y paladar ojival, los nin̄os indice tenian una marcada hipotonia de mejillas y labios, movimientos de lengua disfuncionales y una capacidad de masticar pobremente desarrollada. Sus tiempos de comida eran significativamente más cortos que los controles y pesaban menos a los seis, 12 y 15 meses. Todas la madres de nin̄os con síndrome de Turner se quejaban de dificultades para alimentar a sus nin̄os y manifestaban que estos problemas a menuda estaban presentes desde el nacimiento

The objective rating of oral-motor functions during feeding

The Schedule for Oral Motor Assessment (SOMA) was developed to record oral-motor skills objectively in infants between ages 8 and 24 months postnatal. Its aim is to identify areas of dysfunction that could contribute to feeding difficulties. The procedure takes approximately 20 min to administer, and is intended to be rated largely from a videorecording of a structured feeding session. A series of foodstuffs of varying textures, including liquids, is presented to the child in a standardized manner. Oral-motor skills are evaluated in terms of discrete oral-motor movements. The schedule distinguishes these from skills at more aggregated levels of functioning such as jaw, lip, and tongue control. A total of 127 children have been studied with the instrument, including normal healthy infants and samples with nonorganic failure to thrive, and cerebral palsy. Interrater and test-retest reliabilities were determined on a subset of 10 infants who each took part in three trials rated by 2 therapists. Excellent levels of interrater reliability (kappa >0.75) were obtained for the presence/absence of 69% of discrete oral-motor behaviors. Test-retest reliability was similarly excellent for 85% of ratable behaviors. For the first time an assessment of oral-motor functioning has been shown to have adequate reliability for children aged 8-24 months. The validation of the SOMA on a large sample of normally developing infants and its application to clinical groups is presented in an accompanying paper [1]