アブストラクト
Japanese
| Title | 先天性門脈体循環シャントに対して外科的治療を施行した10例の検討 |
|---|---|
| Subtitle | [原著] |
| Authors | 木戸美織1,3, 岡島英明1,3, 田嶋哲也2, 鈴木久美子1, 金城昌克1, 園田真理1, 小川絵里1, 岡本竜弥1, 岡本晋弥1,4, 上本伸二1,2 |
| Authors (kana) | |
| Organization | 1京都大学医学部附属病院小児外科, 2京都大学医学部附属病院肝胆膵・移植外科, 3金沢医科大学小児外科, 4兵庫県立尼崎総合医療センター小児外科 |
| Journal | 日本小児外科学会雑誌 |
| Volume | 55 |
| Number | 6 |
| Page | 1037-1043 |
| Year/Month | 2019 / 10 |
| Article | 原著 |
| Publisher | 日本小児外科学会 |
| Abstract | 「要旨」【目的】先天性門脈体循環シャント(CPSS)症例では, 門脈血が直接体循環に流入することにより高アンモニア血症, 肺血管病変, 限局性結節性過形成(以下FNH)等の合併症をきたしうる. 外科的治療を行った自験例を検討した. 【方法】2004年1月から2018年10月までに京都大学病院小児外科で手術加療を行った10例について, 診断時の年齢, 診断契機, シャント形態, 臨床症状, 手術時の年齢, 手術適応, 術式, 予後を検討した. 【結果】診断時年齢は中央値で0か月(0か月〜5歳), 男児3例, 女児7例. 診断契機は6例が新生児マススクリーニング検査におけるガラクトース血症疑いで発見された. シャント形態は門脈本幹 - 下大静脈シャント5例, 静脈管開存5例であった. 5例に門脈肺高血圧症, 2例に肝肺症候群, 1例でFNHの急速な増大と門脈肺高血圧症, 1例でFNHの結節内出血による急速増大, 1例に成長障害と肝性脳症を認めた. 1例は無症状であった. 門脈肺高血圧症の3例に生体肝移植を, 2例(うち1例はFNH急速増大合併例)にシャント結紮術を施行した. 肝肺症候群1例に生体肝移植を, 1例にシャント結紮術を施行した. FNHの急速増大を認めた2例で外側区域切除術を施行した. 肝肺症候群に対しシャント結紮後の1例で術後に肝内に多発性の門脈肝静脈シャント再発とこれに起因する門脈肺高血圧症を呈し, 4年後に生体肝移植を施行した. 術後に肺血管病変は全例で改善し生体肝移植を施行した5例中4例では内科的治療から離脱した. 【結論】CPSSで有症状の症例に対し外科的治療は有効で, 病態やシャントの形態に応じた外科的治療を選択することが肝要である. |
| Practice | 臨床医学：外科系 |
| Keywords | 先天性門脈体循環シャント, 門脈肺高血圧症, 肝肺症候群, 限局性結節性過形成, 肝移植, congenital portosystemic shunt, portopulmonary hypertension, hepatopulmonary syndrome, focal nodular hyperplasia, liver transplantation |
English
| Title | Evaluation of Ten Pediatric Patients With Congenital Extrahepatic Portosystemic Shunt Surgically Treated |
|---|---|
| Subtitle | Originals |
| Authors | Miori Kido1,3, Hideaki Okajima1,3, Tetsuya Tajima2, Kumiko Suzuki1, Masakatsu Kaneshiro1, Mari Sonoda1, Eri Ogawa1, Tatsuya Okamoto1, Shinya Okamoto1,4, Sinji Uemoto1,2 |
| Authors (kana) | |
| Organization | 1Department of Surgery, Division of Pediatric Surgery, Kyoto University Hospital, 2Department of Surgery, Division of Hepato-Biliary-Pancreatic Surgery and Transplantation, 3Department of Pediatric Surgery, Kanazawa Medical University, 4Department of Pediatric Surgery, Hyogo Prefectural Amagasaki General Medical Center |
| Journal | Journal of the Japanese Society of Pediatric Surgeons |
| Volume | 55 |
| Number | 6 |
| Page | 1037-1043 |
| Year/Month | 2019 / 10 |
| Article | Original article |
| Publisher | Japanese Society of Pediatric Surgeons |
| Abstract | [Purpose]: A congenital portosystemic shunt (CPSS) may cause hyperammonemia, pulmonary vascular problems, and hepatic nodules such as focal nodular hyperplasia (FNH). We evaluated our CPSS patients treated surgically. [Methods]: Between January 2004 and October 2018, ten CPSS patients were surgically treated. Age and episode for diagnosis, type of shunt, clinical manifestations, indication, age at surgery, mode of surgery, and prognosis were evaluated. [Results]: There were three boys and seven girls with the median age of 0 months ranging from 0 to 5 years old. The most frequent reason for diagnosis was a positive result in neonatal mass screening for galactosemia. The types of shunts were port-caval shunt in five and patent ductus venosus in four. Regarding clinical manifestations, portopulmonary hypertension (PPHTN) was observed in five, hepatopulmonary syndrome (HPS) in two, and rapid growth of FNH in two. In patients with PPHTN, liver transplantation was performed in three and shunt ligation in two. In those with HPS, liver transplantation was performed in one, shunt ligation in one, and left lateral segmentectomy in two with rapid growth of FNH. One patient with HPS treated by shunt ligation showed recurrence of the intrahepatic portosystemic shunt after the ligation and PPHTN occurred. Liver transplantation was required four years after ligation. After surgical treatment, all the patients with pulmonary vascular problems improved, and four patients who received liver transplantation became free from medication. Two with FNH treated surgically have been doing well without recurrence. [Conclusions]: Surgical treatments were effective for CPSS patients. The indication and method of operation should be determined individually on the basis of the clinical status of patients and the morphology of the shunt. |
| Practice | Clinical surgery |
| Keywords | congenital portosystemic shunt, portopulmonary hypertension, hepatopulmonary syndrome, focal nodular hyperplasia, liver transplantation |
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