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Published on: September 19, 2015
Subglossopalatal synechia in association with cardiac and digital anomalies
Albert K Oh1, Joseph Thakuria, Virginia Kimonis
1Craniofacial Centre, Division of Plastic Surgery, Children's Hospital, Harvard Medical School. aoh@lifespan.org
Insights
Congenital oral synechiae, a rare condition causing respiratory distress in infants, involves a midline band. This case highlights its association with cardiac and digital anomalies, suggesting a link to oromandibular-limb hypogenesis spectrum.
Area of Science:
- Pediatric Surgery
- Medical Genetics
- Neonatology
Background:
- Congenital oral synechiae are rare malformations.
- These can lead to significant respiratory distress and feeding difficulties in neonates.
Observation:
- A 1-day-old infant presented with respiratory distress due to a midline subglossopalatal band.
- Associated anomalies included a cleft palate, cardiac defects, and digital hypoplasia.
Findings:
- Initial surgical excision of the band provided temporary airway improvement.
- The infant required further interventions for persistent desaturations, including tongue-lip adhesion, aortic coarctation repair, and management of other cardiac anomalies.
Implications:
- Midline subglossopalatal synechia with systemic anomalies may represent a variant of the oromandibular-limb hypogenesis spectrum.
- Early diagnosis and intervention are crucial for managing respiratory and feeding issues in affected infants.
Abstract:
A 1-day-old boy in respiratory distress had a midline soft tissue band between the floor of the mouth and the posterior edge of the hard palate. There was also a soft palatal cleft, cardiac anomalies, and a hypoplastic right fifth finger and toe. Although his airway initially improved following urgent excision of the subglossopalatal band, he continued to have episodic desaturations. A tongue-lip adhesion opened his airway, and he subsequently underwent resection of juxtaductal aortic coarctation and ligation of patent ductus arteriosus and left superior vena cava. Congenital oral synechiae are uncommon. Affected infants often require prompt intervention secondary to respiratory distress and feeding difficulty. Review of the literature indicates that midline subglossopalatal synechia with cardiac and digital anomalies may be in the oromandibular-limb hypogenesis spectrum.
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