title: Congenital extrahepatic portosystemic shunt: an underdiagnosed but treatable cause of hepatopulmonary syndrome.
abstract: Congenital extrahepatic portosystemic shunt (CEPS) is a rare malformation of the mesenteric vasculature, which may lead to severe complications. In this report, we describe a case series of three children with type II CEPS (presenting as hypoxemia) and hepatopulmonary syndrome (HPS). The first patient was a 4-year-old male who did not receive any specific treatment and subsequently died of brain abscess 5 years after the diagnosis. The second patient was a 10-year-old female with a 5-year history of cyanosis and dyspnea on exertion. She had partial regression of hypoxemia and improved exercise tolerance at 8 months after a surgical shunt closure. The third patient was a 4-year-old male with a 3-year history of cyanosis and decreased exercise tolerance. He had full regression of hypoxemia at 3 months after a transcatheter shunt closure.
  
  
  CONCLUSIONS
  These results indicate that CEPS may present in children with unexplained hypoxemia, which may lead to devastating clinical consequences. Closure of portosystemic shunts may result in resolution of HPS in type II CEPS and the length of period for resolution varies depending on the severity of HPS.
  
  
  BACKGROUND
  Congenital extrahepatic portosystemic shunt (CEPS) is a rare cause of hepatopulmonary syndrome (HPS). There have been few reports in the literature about the management and outcome of HPS in children with CEPS.
  
  
  BACKGROUND
  CEPS may present in children with unexplained hypoxemia, which may lead to devastating clinical consequences. Closure of portosystemic shunts may result in resolution of HPS in type II CEPS.
authors: Department of Cardiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, No. 1678 Dongfang Road, Shanghai, 200127, China. fulijun@scmc.com.cn.
authors: Lijun
authors: L
authors: Fu
authors: Department of Radiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China. wangqian@scmc.com.cn.
authors: Qian
authors: Q
authors: Wang
authors: Department of Cardiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, No. 1678 Dongfang Road, Shanghai, 200127, China. wujinjin@scmc.com.cn.
authors: Jinjin
authors: J
authors: Wu
authors: Department of Cardiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, No. 1678 Dongfang Road, Shanghai, 200127, China. guoying@scmc.com.cn.
authors: Ying
authors: Y
authors: Guo
authors: Department of Cardiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, No. 1678 Dongfang Road, Shanghai, 200127, China. huangmeirong@scmc.com.cn.
authors: Meirong
authors: M
authors: Huang
authors: Department of Cardiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, No. 1678 Dongfang Road, Shanghai, 200127, China. liutingliang@scmc.com.cn.
authors: Tingliang
authors: T
authors: Liu
authors: Department of Surgery, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, No. 1678 Dongfang Road, Shanghai, 200127, China. chenqimin@scmc.com.cn.
authors: Qimin
authors: Q
authors: Chen
authors: Department of Cardiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, No. 1678 Dongfang Road, Shanghai, 200127, China. lifen@scmc.com.cn.
authors: Fen
authors: F
authors: Li
mesh_terms: D000792:Angiography; D002648:Child; D002675:Child, Preschool; D005260:Female; D020065:Hepatopulmonary Syndrome / Q000209:etiology* / Q000601:surgery; D006801:Humans; D000860:Hypoxia / Q000209:etiology; D008297:Male; D011169:Portal Vein / Q000002:abnormalities* / Q000601:surgery; D054079:Vascular Malformations / Q000150:complications* / Q000601:surgery
keywords: Abernethy malformation; Congenital extrahepatic portosystemic shunt (CEPS); Portosystemic shunt; hepatopulmonary syndrome (HPS)