A syndrome characterized by multiple intrabuccal
synechiae associated with craniofacial malformations.
Extremely rare genetic disorder
(fewer than 10 cases have been described) with autosomal dominant inheritance.
Patients present with mental and growth
retardation and hypotonia. Clinical features involve the head and neck with
microcephaly, cleft palate, multiple, “cord-like” adhesions between the
lateral aspects of the tongue, the floor of the mouth and the hard palate,
protruding lips, hypoplastic or bifid uvula, micrognathia/retrognathia,
beaked nose, short philtrum, malar hypoplasia, and lacrimal abnormalities.
Anesthesia in this condition has not
been described. The described features suggest that face-mask ventilation
may be difficult, and direct laryngoscopy and tracheal intubation may be
impossible because of severely restricted mouth opening. Spontaneous
ventilation should be preserved until the airway has been secured and alternative
airway management techniques (e.g., awake fiberoptic nasal intubation) have to be considered.
Gassner I, Muller W, Rossler H et al: Familial occurrence of syngnathia
congenita syndrome. Clin Genet
Haramis HT, Apesos J: Cleft palate and congenital lateral alveolar synechia
syndrome: Case presentation and literature review. Ann Plast Surg
Nakata NM, Guion-Almeida ML, Richieri-Costa A: Cleft palate-lateral
synechiae syndrome: Report on three new patients with additional findings
and evidence for variability and heterogeneity. Am J Med Genet