Subglossopalatal synechia in association with cardiac and digital anomalies.
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.
Oh
Albert K
AK
Craniofacial Centre, Division of Plastic Surgery, Children's Hospital, Harvard Medical School. aoh@lifespan.org
Thakuria
Joseph
J
Kimonis
Virginia
V
Mulliken
John B
JB
D000015:Abnormalities, Multiple; D001017:Aortic Coarctation / Q000473:pathology; D004374:Ductus Arteriosus, Patent / Q000473:pathology; D005385:Fingers / Q000002:abnormalities*; D006330:Heart Defects, Congenital / Q000473:pathology*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009060:Mouth Floor / Q000002:abnormalities*; D010160:Palate, Soft / Q000002:abnormalities*; D012131:Respiratory Insufficiency / Q000209:etiology; D014034:Toes / Q000002:abnormalities*; D014683:Vena Cava, Superior / Q000002:abnormalities