Ankyloglossia Superior Syndrome With Complex Craniofacial Anomalies: Case Report and Literature Review.

Ankyloglossia superior syndrome is an extremely rare entity in which centrally located glossopalatine ankylosis is a principal feature. Some cases are accompanied by cleft palate, micrognathia, or tongue hypoplasia, and affected patients need careful nutritional and respiratory support. We describe a newborn girl in whom ankyloglossia superior syndrome comprised complex craniofacial malformations, including cleft palate, micrognathia, microglossia, and natal teeth as well as limb anomalies. Surgical treatment entailed release of synechiae, and glossopexy was performed successfully to prevent postsurgical airway complications and to ensure adequate nutrition by nipple feeding during infancy.

Quantitative imaging of tongue kinematics during infant feeding and adult swallowing reveals highly conserved patterns.

Tongue motility is an essential physiological component of human feeding from infancy through adulthood. At present, it is a challenge to distinguish among the many pathologies of swallowing due to the absence of quantitative tools. We objectively quantified tongue kinematics from ultrasound imaging during infant and adult feeding. The functional advantage of this method is presented in several subjects with swallowing difficulties. We demonstrated for the first time the differences in tongue kinematics during breast- and bottle-feeding, showing the arrhythmic sucking pattern during bottle-feeding as compared with breastfeeding in the same infant with torticollis. The method clearly displayed the improvement of tongue motility after frenotomy in infants with either tongue-tie or restrictive labial frenulum. The analysis also revealed the absence of posterior tongue peristalsis required for safe swallowing in an infant with dysphagia. We also analyzed for the first time the tongue kinematics in an adult during water bolus swallowing demonstrating tongue peristaltic-like movements in both anterior and posterior segments. First, the anterior segment undulates to close off the oral cavity and the posterior segment held the bolus, and then, the posterior tongue propelled the bolus to the pharynx. The present methodology of quantitative imaging revealed highly conserved patterns of tongue kinematics that can differentiate between swallowing pathologies and evaluate treatment interventions. The method is novel and objective and has the potential to advance knowledge about the normal swallowing and management of feeding disorders.

Congenital lateral cleft palate with unilateral craniofacial microsomia and lateral ankyloglossia.

Craniofacial microsomia is a group of anomalies that occur due to developmental defects in the first and second branchial arches during the embryological period. The disease has various craniofacial and extracraniofacial presentation patterns. Lateral cleft palate is distinct from the most common palate clefts because it is located lateral to the uvula. The presence of a lateral cleft palate has been very rarely reported in the literature, and the aetiopathogenesis of the disease is still not well understood. We aimed to report a case of the coexistence of lateral cleft palate and craniofacial microsomia and discuss the aetiopathogenesis of these diseases.