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Surgery Today

, Volume 44, Issue 8, pp 1561–1564 | Cite as

Massive hemorrhage after Kasai portoenterostomy in a patient with a congenital extrahepatic portosystemic shunt, malrotation and a double aortic arch: report of a case

  • Shinya Takazawa
  • Hiroo UchidaEmail author
  • Hiroshi Kawashima
  • Yujiro Tanaka
  • Kaori Sato
  • Takahiro Jimbo
  • Kyoichi Deie
  • Kazuki Koiwai
  • Koji Nomura
  • Tadashi Iwanaka
Case Report
  • 199 Downloads

Abstract

A newborn female was transferred to our hospital presenting with severe respiratory distress. She underwent tracheal intubation and nasogastric tubing. Investigations revealed a congenital extrahepatic portosystemic shunt (CEPS) type 1, biliary atresia, heterotaxia, polysplenia, malrotation and a double aortic arch (DAA). She underwent the Kasai portoenterostomy and the Ladd procedure when she was 29 days old. On postoperative day 20, she developed sudden hematemesis with bright red blood. Endoscopy showed massive bleeding from an esophageal ulcer, and endoscopic therapy was performed successfully. During left thoracotomy, an aortoesophageal fistula (AEF) was detected and repaired by direct suturing. The postoperative course was uneventful. CEPS type 1 is commonly associated with other congenital malformations; however, there have been no previous reports of an association between CEPS and DAA. Nasogastric tube insertion in a patient with DAA can result in catastrophic AEF. The treatment strategy should be carefully considered in patients with CEPS type 1 and multiple congenital fetal anomalies.

Keywords

Congenital extrahepatic portosystemic shunt Biliary atresia Double aortic arch Nasogastric tube Aortoesophageal fistula 

Notes

Conflict of interest

The author declares that no conflicts of interest exist.

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Copyright information

© Springer Japan 2013

Authors and Affiliations

  • Shinya Takazawa
    • 1
    • 3
  • Hiroo Uchida
    • 1
    Email author
  • Hiroshi Kawashima
    • 1
  • Yujiro Tanaka
    • 1
  • Kaori Sato
    • 1
  • Takahiro Jimbo
    • 1
  • Kyoichi Deie
    • 1
  • Kazuki Koiwai
    • 1
  • Koji Nomura
    • 2
  • Tadashi Iwanaka
    • 3
  1. 1.Department of Pediatric SurgerySaitama Children’s Medical CenterSaitamaJapan
  2. 2.Department of Cardiovascular SurgerySaitama Children’s Medical CenterSaitamaJapan
  3. 3.Department of Pediatric SurgeryThe University of Tokyo HospitalTokyoJapan

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