Congenital Oral Synechiae

Abstract

Major congenital synechiae of the oral cavity constitute a clinically confusing spectrum of abnormalities. On the basis of clinical data, we propose two categories: 1) abnormalities secondary to persistence of the buccopharyngeal membrane and 2) abnormalities secondary to formation of ectopic membranes. An ectopic membrane results from abnormal fusion and can be subclassified as a subglossopalatal membrane, glossopalatal ankylosis, or syngnathia. This classification is supported by embryologic studies and is used to reclassify all cases reported since 1900. Distinct differences, such as the presence of associated limb anomalies, emerge; these are reviewed and add support to the proposed classification.

A patient with Charlie M Syndrome: Differential diagnosis of Oromandibular Limb Hypogenesis Syndromes

“…There have been several attempts to explain the emergence of OLHS, Charlie M syndrome and glossopalatine ankylosis [4][5][6]13,[16][17][18] . Generally, defects in facial and limb differentiation must be attributed to days 28-63 of the embryologic period 4,6 .…”

“…The first theory hypothesizes the appearance of intraoral bands by the persistence of the buccopharyngeal membrane (BPM) (ref. 4,[16][17][18] . The BPM develops when the intraembryonic mesoderm does not invade all parts of the intraembryonic disc which results in separation of the stomatodeum entoderm and the foregut ectoderm up to day 26 (ref.…”

“…Afterwards, tension of the BPM becomes greater by rapid growth of the pharynx, tongue and facial structures, which results in breakdown of the BPM (ref. [17][18] ). The second theory assumes the persistence of a subglossopalatal membrane or ectopic membranes, which can be present between the sixth and eighth week of the embryonic development and normally disappear by the dropdown of the tongue around the ninth week 4,[16][17][18] .…”

“…[17][18] ). The second theory assumes the persistence of a subglossopalatal membrane or ectopic membranes, which can be present between the sixth and eighth week of the embryonic development and normally disappear by the dropdown of the tongue around the ninth week 4,[16][17][18] . Therefore, either the persistence of membranes, failure of the tongue to drop down or both can cause membranous fusion between the upper and lower jaw.…”

Congenital Maxillomandibular Synechiae: Anesthetic Approach with Ketamine and Dexmedetomidine Sedation for Surgical Division of Synechiae and Tracheostomy

“…It can be found in any part of oral cavity and generally includes fibrous and supportive tissue such as muscle or bone and membranes or bands of epithelium (2). It can be associated with microglossia, micrognathia, cleft palate or syndromes like Vander de Woude, and Pierre-Robin (1, 2).…”