title: Popliteal pterygium syndrome (PPS) with intra-alveolar syngnathia: a discussion of anesthetic and surgical considerations.
abstract: Popliteal pterygium syndrome (PPS) is a rare genetic disorder that involves the association of a popliteal web with a combination of craniofacial, genitourinary and extremity malformations. In this article, we describe a patient with PPS complicated with multiple intra-alveolar syngnathia. We discuss the anesthetic and the surgical management of this case and review the literature regarding PPS and intra-alveolar syngnathia.
authors.affiliation: Department of ENT Surgery, Karolinska University Hospital, Stockholm, Sweden. caroline.gahm@karolinska.se
authors.forename: Caroline
authors.initials: C
authors.lastname: Gahm
authors.forename: Richard
authors.initials: R
authors.lastname: Kuylenstierna
authors.forename: Georgios
authors.initials: G
authors.lastname: Papatziamos
mesh_terms: D000015:Abnormalities, Multiple; D000539:Alveolar Process / Q000002:abnormalities*; D000758:Anesthesia / Q000379:methods*; D002680:Chin / Q000002:abnormalities* / Q000601:surgery*; D019465:Craniofacial Abnormalities / Q000601:surgery*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D035002:Lower Extremity / Q000601:surgery*; D011150:Popliteal Artery; D013514:Surgical Procedures, Operative / Q000379:methods*; D013577:Syndrome