title: Perioperative respiratory complications caused by cystic lung malformation in Proteus syndrome.
abstract: A 7-year-old girl with Proteus syndrome, complicated with severe kyphoscoliosis and cystic lung malformation, developed respiratory complications during and after posterior spinal fusion under general anesthesia. We speculated that low oxygenation while in the prone position contributed to ventilation perfusion mismatch owing to the cystic lung malformation that existed predominantly in the right lung, and that postoperative respiratory failure was caused by initially increased bronchial mucous secretions and secondary persistent bacterial pneumonia. Cystic lung malformation rather than reduced respiratory function, although both can be a cause of mucous clearance impairment, should be considered as one of the predictive factors for perioperative respiratory failure in Proteus syndrome.
authors.affiliation: Department of Anesthesiology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima 960-1295, Japan.
authors.forename: Masaki
authors.initials: M
authors.lastname: Nakane
authors.forename: Mikako
authors.initials: M
authors.lastname: Sato
authors.forename: Hisashi
authors.initials: H
authors.lastname: Hattori
authors.forename: Yukio
authors.initials: Y
authors.lastname: Matsumoto
authors.forename: Manabu
authors.initials: M
authors.lastname: Otsuki
authors.forename: Masahiro
authors.initials: M
authors.lastname: Murakawa
mesh_terms: D002648:Child; D003560:Cysts / Q000150:complications*; D005260:Female; D006801:Humans; D007431:Intraoperative Complications / Q000209:etiology*; D008171:Lung Diseases / Q000150:complications*; D016715:Proteus Syndrome / Q000150:complications* / Q000601:surgery; D012131:Respiratory Insufficiency / Q000209:etiology*; D013123:Spinal Fusion; D014057:Tomography, X-Ray Computed