Palatoglossal fusion with cleft palate and hypoplasia of cerebellar vermis

Abstract

A new-born male presented within 12 h of birth with respiratory distress. On examination and workup, he had palatoglossal fusion, cleft palate and hypoplasia of the cerebellar vermis. A 2.5 Fr endotracheal tube was inserted into the pharynx through nostril as a nasopharyngeal stent, following which his respiratory distress improved. Once child was optimised, then feeding was started by nasogastric tube and feeds were tolerated well. Elective tracheostomy and gastrostomy were done, followed by release of adhesions between the tongue and palate at a later stage. Review of literature suggests that palatoglossal fusion is uncommon and presents as an emergency. Mostly, these oral synechiae are associated with digital and/or cardiac anomaly. Other disorders associated with intra-oral synechiae include congenital alveolar synechiae, van der Woude syndrome, popliteal pterygium syndrome and oromandibular limb hypogenesis syndrome. The authors report a hitherto undescribed association of palatoglossal fusion with cleft palate and hypoplasia of the cerebellar vermis.

Combined Tongue-Palate Fusion With Alveolar Bands in a Patient With Pierre Robin Sequence and Van der Woude Syndrome

“…Intraoral synechiae have been associated with several syndromes including CPLS, VWS, and PPS (Naidoo and Bütow, 2015). While there are a few case reports of tongue-palate adhesions, none report the type of near-circumferential adhesion between the tongue and cleft palate in the setting of VWS as seen in the individual described here (Gartlan et al, 1993; Garca et al, 2012; Rai et al, 2012; Lima et al, 2016; Solanki et al, 2016; Dongol et al, 2017).…”

Oral synechia with complete obstruction of the oral cavity and laryngopharynx

“…Oral synechia are rare congenital deformities that require early intervention to reduce complications regarding the airway and feeding 1–3 . They can be found at various adhesion sites, such as between the maxillary and mandibular alveoli, between the oral cavity floor and hard palate, and between the posterior part of the tongue and soft palate.…”

Airway management of palatoglossal bands – A challenge to an anaesthesiologist

“…They are usually associated with a variety of congenital defects such as cleft lip and palate, microglossia, micrognathia and temporomandibular disorders. [2]…”