Three Cases of Congenital Portosystemic Shunt With Different Treatment Approaches Selected

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  • Murakami Masakazu
    Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University
  • Harumatsu Toshio
    Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University
  • Yano Keisuke
    Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University
  • Baba Tokuro
    Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University
  • Onishi Shun
    Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University
  • Yamada Koji
    Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University
  • Yamada Waka
    Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University
  • Masuya Ryuta
    Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University
  • Nakame Kazuhiko
    Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University
  • Ieiri Satoshi
    Department of Pediatric Surgery, Research Field in Medical and Health Sciences, Medical and Dental Area, Research and Education Assembly, Kagoshima University

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Other Title
  • 異なるアプローチで治療した先天性門脈体循環シャントの3例
  • 症例報告 異なるアプローチで治療した先天性門脈体循環シャントの3例
  • ショウレイ ホウコク コトナル アプローチ デ チリョウ シタ センテンセイ モンミャクタイ ジュンカン シャント ノ 3レイ

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<p>Congenital portosystemic shunt (CPSS) can cause hepatopulmonary syndrome and encephalopathy, among other complications. Interruption of the shunt blood flow is the definitive treatment and can prevent such complications. However, there is no standardized treatment approach for CPSS because of the varied pathophysiological states that can manifest. We treated three cases of CPSS with three different approaches. A one-year-old girl was treated for shunt vessel transection by laparoscopic liver resection. A 10-month-old boy with congenital heart disease underwent shunt vessel banding by laparotomy. A 17-year-old boy underwent transcatheter embolization. CPSS may cause severe complications, such as progressive pulmonary hypertension. Therefore, even when no clinical manifestations are apparent, CPSS should be treated as early as possible. If hypoplasia of the intrahepatic portal system (IHPS) is mild or moderate and the portal venous pressure (PVP) under the occlusion test is <25 mmHg, shunt closure can be performed in a single step. If hypoplasia of IHPS is severe or the PVP is ≥25 mmHg, the patient is a candidate for 2-step shunt closure. Endovascular closure is the first option for treatment, whereas surgical closure should be considered second. We should carefully select our approach on the basis of the preoperative evaluation of the anatomy of the shunt vessel and IHPS.</p>

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