title: Severe cyanosis after Kawashima operation resulting from congenital portosystemic shunt.
abstract: We describe an unusual case of a patient with polysplenia, inferior vena cava (IVC) interruption, and unrecognized congenital portosystemic venous connection (CPVC) in whom severe cyanosis developed in the acute postoperative stage after a Kawashima operation and required urgent surgical division of the CPVC. It has been stressed recently that CPVCs and other abdominal venous abnormalities have been underdiagnosed in patients with polysplenia and IVC interruption, and we think that a routine evaluation of abdominal venous connections should be mandatory before considering the Kawashima operation for these patients.
authors.affiliation: Department of Cardiovascular Surgery, Chiba Children's Hospital, Chiba, Japan. yukicvs@gmail.com
authors.forename: Yuki
authors.initials: Y
authors.lastname: Nakamura
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Aoki
authors.forename: Ikuo
authors.initials: I
authors.lastname: Hagino
authors.forename: Hiromichi
authors.initials: H
authors.lastname: Nakajima
mesh_terms: D000005:Abdomen; D003490:Cyanosis / Q000209:etiology*; D059446:Heterotaxy Syndrome / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D012082:Renal Veins / Q000002:abnormalities; D013152:Splanchnic Circulation; D013162:Splenic Vein / Q000002:abnormalities; D013270:Stomach / Q000098:blood supply; D054079:Vascular Malformations / Q000150:complications* / Q000601:surgery*; D014656:Vascular Surgical Procedures / Q000009:adverse effects*; D014680:Veins / Q000002:abnormalities*; D014682:Vena Cava, Inferior / Q000002:abnormalities*