(003) A Case of Ankyloglossia Superior Syndrome in a Premature Neonate

Introduction: Palatoglossal adhesion is a rare congenital anomaly with fewer than 20 reported cases. This anomaly can be found with a variety of other congenital malformations including cleft palate and limb malformations and this constellation of findings is known as ankyloglossia superior syndrome. The palatoglossal adhesion requires special consideration given the difficulties it presents with orotracheal intubation.

Methods: We present a case of an ex-25-week-old male who was born with palatoglossal fusion and limb abnormalities concerning for ankyloglossia superior syndrome. Given his extreme prematurity status careful planning of resection was needed to balance the needs for a safe airway with the risks of temperature shifts and blood loss in the operating room. We also reviewed the literature discussing prior presentations of superior ankyloglossia syndrome and surgical approaches for tongue release.

Results: We found 20 articles discussing palatoglossal adhesion or superior ankyloglossia syndrome. None of these cases reported this anomaly in an extremely premature neonate. Patients often presented with concomitant limb anomalies, cleft or palatal defects, micrognathia or macroglossia. Repairs were described under both local and general anesthesia. A variety of potential causative mechanisms

Conclusions: Ankyloglossia superior syndrome presents difficulties in airway management as it precludes the ability to perform direct laryngoscopy and makes orotracheal intubation difficult. Despite the associated anomalies that have been reported with this syndrome, laryngeal anomalies are not common. In a premature neonate these airway concerns become more critical, as positive pressure ventilation and intubation may be temporarily necessary as lungs develop.