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A syndrome characterized by multiple intrabuccal synechiae associated with craniofacial malformations.

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CPLS Syndrome.

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Extremely rare genetic disorder (fewer than 10 cases have been described) with autosomal dominant inheritance.

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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.

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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 15:241, 1979.  [PubMed: 421363]
Haramis HT, Apesos J: Cleft palate and congenital lateral alveolar synechia syndrome: Case presentation and literature review. Ann Plast Surg 34:424, 1995.  [PubMed: 7793792]
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 47:330, 1993.  [PubMed: 8135276]

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