A veil in the oral cavity: report of two cases of oral synechiae

This report describes the presentation of a newborn male with circumferential tongue-palate fusion associated with cleft palate and alveolar bands. After intraoral adhesions lysis, the patient was diagnosed with Pierre Robin sequence. A family history of cleft lip and palate was noted, and interferon regulatory factor 6 ( IRF6) sequencing revealed a heterozygous variant, confirming the diagnosis of van der Woude syndrome. The disruption of IRF6 resulted in abnormal orofacial development including micrognathia and intraoral adhesions as well as tongue-palate fusion, then resulting in glossoptosis with airway obstruction and cleft palate.

Section: Discussionmentioning

confidence: 78%

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

Robbins

Zárate

Hartzell

2018

“…Congenital maxillomandibular fusion or glossopalatine adhesion without the tip of tongue is also rare and is recognized as another entity that has been reported in association with disorders such as Van der Woude syndrome (Johnsson and Robinow, 1978), oromandibular-limb hypogenesis syndrome (Cohen, 1978), or with other craniofacial anomalies, including cleft palate (Gartlan et al, 1993). In many affected patients, bilateral soft tissue bands or membranes were connected to lateral portions of the oral cavity (Cronin et al, 2001;Parkins and Boamah, 2009;Salahuddin et al, 2014;Dongol et al, 2017).…”

Section: Discussionmentioning

confidence: 99%

“…Ankyloglossia superior, as well as other types of oral synechiae, can be easily diagnosed on the basis of the restricted mouth opening and suckling disturbance caused by an inability to move the tongue immediately after the birth (Bolling et al, 2007;Dongol et al, 2017). In addition, especially in the cases of micrognathia or cleft palate, or both, as in the Pierre Robin sequence, respiratory disturbance can occur, and earlier surgical intervention should be considered.…”

Section: Discussionmentioning

confidence: 99%

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

Hara

Tanaka

Kogo

2020

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.

“…Intubation for anesthesia should be performed with care and caution (Alfery et al, 1979; Dinardo et al, 1989; Cronin et al, 2001; Puvabanditsin et al, 2003; Dongol et al, 2017). In severe cases with multiple anomalies or cases that have failed using other techniques, tracheostomy should be considered (Snijman and Prinsloo, 1966; Valnicek and Clarke, 1993).…”

Section: Discussionmentioning

confidence: 99%

Patients With Cleft Lip and Palate Associated With Intraoral Fibrous Bands: A Report of 3 Cases and Review of Literature

Vamvanij

Chen

2019

In rare instances, cleft lip and palate occur in association with synechia, intraoral fibrous bands connecting the maxilla and mandible. The main concern in synechia is a restricted mouth opening that leads to airway and feeding problems. This study reports our experience in the treatment of 3 cases and includes a review of the literature. Three patients with intraoral fibrous bands received treatment and follow-ups in our center. Division of the bands and reconstruction of the lip and palate were successfully performed. All reported cases in the literature were collected for a summary of clinical presentations and as references for management. Early management of the synechia improves the mouth opening and facilitates the treatment of associated anomalies.