Congenital extrahepatic portosystemic shunt: an underdiagnosed but treatable cause of hepatopulmonary syndrome

详细信息    查看全文

• 作者：Lijun Fu ; Qian Wang ; Jinjin Wu ; Ying Guo ; Meirong Huang…
• 关键词：Abernethy malformation ; Congenital extrahepatic portosystemic shunt (CEPS) ; hepatopulmonary syndrome (HPS) ; Portosystemic shunt
• 刊名：European Journal of Pediatrics
• 出版年：2016
• 出版时间：February 2016
• 年：2016
• 卷：175
• 期：2
• 页码：195-201
• 全文大小：4,180 KB
• 参考文献：1.Abernethy J, Banks J (1793) Account of Two Instances of Uncommon Formation, in the Viscera of the Human Body. By Mr. John Abernethy, Assistant Surgeon to St. Bartholomew's Hospital. Communicated by Sir Joseph Banks, Bart. PRS. Philosophical Transactions of the Royal Society of London 83:59–66CrossRef
  2.Alvarez AE, Ribeiro AF, Hessel G, Baracat J, Ribeiro JD (2002) Abernethy malformation: one of the etiologies of hepatopulmonary syndrome. Pediatr Pulmonol 34:391–394. doi:10.​1002/​ppul.​10182 CrossRef PubMed
  3.Bernard O, Franchi-Abella S, Branchereau S, Pariente D, Gauthier F, Jacquemin E (2012) Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis 32:273–287. doi:10.​1055/​s-0032-1329896 CrossRef PubMed
  4.Degos B, Daelman L, Huberfeld G, Meppiel E, Rabier D, Galanaud D, Magis AS, Lyon-Caen O, Samuel D, Sedel F (2012) Portosystemic shunts: an underdiagnosed but treatable cause of neurological and psychiatric disorders. J Neurol Sci 321:58–64. doi:10.​1016/​j.​jns.​2012.​07.​050 CrossRef PubMed
  5.Franchi-Abella S, Branchereau S, Lambert V, Fabre M, Steimberg C, Losay J, Riou JY, Pariente D, Gauthier F, Jacquemin E, Bernard O (2010) Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr 51:322–330. doi:10.​1097/​MPG.​0b013e3181d9cb92​ PubMed
  6.Grace JA, Angus PW (2013) Hepatopulmonary syndrome: update on recent advances in pathophysiology, investigation, and treatment. J Gastroenterol Hepatol 28:213–219. doi:10.​1111/​jgh.​12061 CrossRef PubMed
  7.Howard ER, Davenport M (1997) Congenital extrahepatic portocaval shunts—the Abernethy malformation. J Pediatr Surg 32:494–497CrossRef PubMed
  8.Iida T, Ogura Y, Doi H, Yagi S, Kanazawa H, Imai H, Sakamoto S, Okamoto S, Uemoto S (2010) Successful treatment of pulmonary hypertension secondary to congenital extrahepatic portocaval shunts (Abernethy type 2) by living donor liver transplantation after surgical shunt ligation. Transpl Int 23:105–109. doi:10.​1111/​j.​1432-2277.​2009.​00964.​x CrossRef PubMed
  9.Morgan G, Superina R (1994) Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg 29:1239–1241CrossRef PubMed
  10.Morikawa N, Honna T, Kuroda T, Kitano Y, Fuchimoto Y, Kawashima N, Kawasaki K (2008) Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an Abernethy malformation. J Pediatr Surg 43:e35–38. doi:10.​1016/​j.​jpedsurg.​2007.​11.​001 CrossRef PubMed
  11.Ohnishi Y, Ueda M, Doi H, Kasahara M, Haga H, Kamei H, Ogawa K, Ogura Y, Yoshitoshi EY, Tanaka K (2005) Successful liver transplantation for congenital absence of the portal vein complicated by intrapulmonary shunt and brain abscess. J Pediatr Surg 40:e1–3. doi:10.​1016/​j.​jpedsurg.​2005.​02.​011 CrossRef PubMed
  12.Papagiannis J, Kanter RJ, Effman EL, Pratt PC, Marcille R, Browning IB 3rd, Armstrong BE (1993) Polysplenia with pulmonary arteriovenous malformations. Pediatr Cardiol 14:127–129. doi:10.​1007/​bf00796995 CrossRef PubMed
  13.Rabiller A, Nunes H, Lebrec D, Tazi KA, Wartski M, Dulmet E, Libert JM, Mougeot C, Moreau R, Mazmanian M, Humbert M, Herve P (2002) Prevention of gram-negative translocation reduces the severity of hepatopulmonary syndrome. Am J Respir Crit Care Med 166:514–517. doi:10.​1164/​rccm.​200201-027OC CrossRef PubMed
  14.Saulters K, Hittle K (2015) Hepatopulmonary syndrome in the presence of abernethy malformation: a pediatric case report. J Pediatr Health Care 29:104–107. doi:10.​1016/​j.​pedhc.​2014.​05.​005 CrossRef PubMed
  15.Sokollik C, Bandsma RH, Gana JC, van den Heuvel M, Ling SC (2013) Congenital portosystemic shunt: characterization of a multisystem disease. J Pediatr Gastroenterol Nutr 56:675–681. doi:10.​1097/​MPG.​0b013e31828b3750​ CrossRef PubMed
  16.Srivastava D, Preminger T, Lock JE, Mandell V, Keane JF, Mayer JE Jr, Kozakewich H, Spevak PJ (1995) Hepatic venous blood and the development of pulmonary arteriovenous malformations in congenital heart disease. Circulation 92:1217–1222CrossRef PubMed
  
• 作者单位：Lijun Fu (1)
  Qian Wang (2)
  Jinjin Wu (1)
  Ying Guo (1)
  Meirong Huang (1)
  Tingliang Liu (1)
  Qimin Chen (3)
  Fen Li (1)
  
  1. Department of Cardiology, Shanghai Children’s Medical Center, Shanghai Jiao Tong University School of Medicine, No. 1678 Dongfang Road, Shanghai, 200127, China
  2. Department of Radiology, Shanghai Children’s Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China
  3. Department of Surgery, Shanghai Children’s Medical Center, Shanghai Jiao Tong University School of Medicine, No. 1678 Dongfang Road, Shanghai, 200127, China
  
• 刊物主题：Pediatrics;
• 出版者：Springer Berlin Heidelberg
• ISSN：1432-1076

文摘

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. Conclusion: 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. What is Known: • 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. What is New: • 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.