Syngnathia is a rare anomaly involving soft tissue or bony adhesions between the maxilla and mandible. Two cases of congenital syngnathia secondary to interalveolar soft tissue synechia are presented. These children present problems with airway protection and feeding in the immediate neonatal period. Most have multiple associated anomalies requiring concurrent management. Less than 50 cases have been described, most commonly in association with popliteal pterygium syndrome. Surgical management involves division of the adhesions in the first few days of life. Depending on the severity, these patients may present formidable anesthetic challenges. The prognosis is generally favorable in cases of soft tissue adhesions with eventual restoration of normal mandibular motion and function.
BixlerD., PolandC., NanceW.E.. Phenotypic variation in the popliteal pterygium syndrome.Clin Genet1973; 4: 220–228.
5.
BurketL.W.. Congenital bony temporomandibular ankylosis and facial hemiatrophy: a review of the literature and report of a case.J Am Med Assoc1936; 106: 1719–1722.
DinardoN.M., ChristianJ.M., BennettJ.A.. Cleft palate lateral synechia syndrome. Review of the literature and a case report.Oral Surg Oral Med Oral Pathol1989; 68: 565–566.
10.
DobrowB.. Syngnathia and multiple defects.J Clin Dysmorphol1983; 1: 5–7.
11.
EscobarV., WeaverD.D.. The facio-genito-popliteal syndrome.Birth Defects1978; Vol. XIV, 6B: 185–192.
12.
Froster-IskeniusU.G.. Popliteal pterygium syndrome.J Med Genet1990; 27: 320–326.
13.
FuhrmannW., KochF., SchweckendickW.. Autosomal dominante Vererberung von Gaumenspalten und Synechien zwischen Gaumen und Mundboden oder Zünge.Humangenetik1972; 14: 196–203.
14.
GorlinJ.J., CohenN.M., LevinL.S.. Syndromes of the head and neck.3rd Ed.New York: Oxford University Press,1990: 738–740.
15.
HammerJ., KlauslerM., SchinzelA.. The popliteal pterygium syndrome: distinct phenotypic variation in two families.Helv Pediatr Acta1988; 43: 507–514.
16.
HochstetterF.. Über zwei Fälle epithelialer Syngnalhie bei menschlichen Keimlingen.DMW1948; 108–112.
17.
HogginsG.S.. Aglossia congenita with bony fusion of the jaws.Br J Oral Surg1969; 7: 63–65.
SeghersM.J.. Une malformation rare: l'imperforation oropharyngienne.Acta Paediat Belg1966; 20: 130–137.
31.
ShahR.M.. Palatomandibular and maxillo-mandibular fusion, partial aglossia and cleft palate in a human embryo. Report of a case.Teratology1977; 15: 261–272.
32.
ShawW.C., SimpsonJ.P.. Oral adhesions associated with cleft lip and palate and lip fistulae.Cleft Palate J1980; 7: 2–6.
33.
SimpsonJ.R., MavesM.D.. Congenital syngnathia or fusion of the gums and jaws.Otol Head Neck Surg1985; 93: 96–99.
34.
SnijmanP.C., PrinslooJ.G.. Congenital fusion of the gums.Am J Dis Child1966; 112: 593–595.
SristavaS., BangR.L.. Congenital sinuses of the lower lip: reappraisal of Van der Woude syndrome on the basis of nine patients.Ann Plast Surg1989; 22: 316–320.
37.
StenbergN., SagherU., GolanJ., EidelmanA.I., Ben-HurN.. Congenital fusion of the gums with bilateral fusion of the temporomandibular joints.Plast Reconstr Surg1983; 72: 385–387.
