Computed tomography and magnetic resonance imaging of multiple focal nodular hyperplasias of the liver with congenital absence of the portal vein in a Chinese girl: case report and review of the literature

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• 作者：Kun Zhang (1)
  Qingjun Wang (1)
  Haiyi Wang (1)
  Huiyi Ye (1)
  Aitao Guo (2)
  Weidong Duan (3)
  
  1. Department of Radiology ; PLA General Hospital ; #28 Fuxing Road ; Beijing ; 100853 ; China
  2. Department of Pathology ; PLA General Hospital ; #28 Fuxing Road ; Beijing ; 100853 ; China
  3. Department of Hepatobiliary Surgery ; PLA General Hospital ; #28 Fuxing Road ; Beijing ; 100853 ; China
  
• 关键词：congenital absence of the portal vein ; focal nodular hyperplasia ; computed tomography ; magnetic resonance imaging
• 刊名：European Journal of Medical Research
• 出版年：2014
• 出版时间：December 2014
• 年：2014
• 卷：19
• 期：1
• 全文大小：3,624 KB
• 参考文献：1. Hu, GH, Shen, LG, Yang, J, Mei, JH, Zhu, YF (2008) Insight into congenital absence of the portal vein: is it rare?. World J Gastroenterol 14: pp. 5969-5979 CrossRef
  2. Goo, HW (2007) Extrahepatic portosystemic shunt in congenital absence of the portal vein depicted by time-resolved contrast-enhanced MR angiography. Pediatr Radiol 37: pp. 706-709 CrossRef
  3. Maekawa, S, Suzuki, T, Mori, K, Ichii, O, Kanda, K, Tai, M, Ochiai, H, Ejiri, Y, Wakatsuki, S, Takagi, T, Ohira, H, Tashiro, M, Otsuki, M (2007) Congenital absence of the portal vein in an adult woman with liver tumor. Nippon Shokakibyo Gakkai Zasshi 104: pp. 1504-1511
  4. Turkbey, B, Karcaaltincaba, M, Demir, H, Akcoren, Z, Yuce, A (2006) Multiple hyperplastic nodules in the liver with congenital absence of portal vein: MRI findings. Pediatr Radiol 36: pp. 445-448 CrossRef
  5. Gaetano, AM, Gui, B, Macis, G, Manfredi, R, Stasi, C, Manfredi, R, Stasi, C (2004) Congenital absence of the portal vein associated with focal nodular hyperplasia in the liver in an adult woman: imaging and review of the literature. Abdom Imaging 29: pp. 455-459 CrossRef
  6. Joyce, AD, Howard, ER (1988) Rare congenital anomaly of the portal vein. Br J Surg 75: pp. 1038-1039 CrossRef
  7. Noe, JA, Pittman, HC, Burton, EM (2006) Congenital absence of the portal vein in a child with Turner syndrome. Pediatr Radiol 36: pp. 566-568 CrossRef
  8. Hino, T, Hayashida, A, Okahashi, N, Wada, N, Watanabe, N, Obase, K, Neishi, Y, Kawamoto, T, Okura, H, Yoshida, K (2009) Portopulmonary hypertension associated with congenital absence of the portal vein treated with bosentan. Intern Med 48: pp. 597-600 CrossRef
  9. Venkat-Raman, N, Murphy, KW, Ghaus, K, Teoh, TG, Higham, JM, Carvalho, JS (2001) Congenital absence of portal vein in the fetus: a case report. Ultrasound Obstet Gynecol 17: pp. 71-75 CrossRef
  10. Borgne, J, Paineau, J, Hamy, A, Dupas, B, Lerat, F, Raoul, S (2000) Interruption of the inferior vena cava with azygos termination associated with congenital absence of portal vein. Surg Radiol Anat 22: pp. 197-202 CrossRef
  11. Abernethy, J (1973) Account of two instances of uncommon formation in the viscera of the human body. Philos Trans R Soc Lond B Biol Sci 83: pp. 295-299
  12. 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: pp. 1239-1241 CrossRef
  13. Nakasaki, H, Tanaka, Y, Ohta, M, Kanemoto, T, Mitomi, T, Iwata, Y, Ozawa, A (1989) Congenital absence of the portal vein. Ann Surg 210: pp. 190-193 CrossRef
  14. Barton, JW, Keller, MS (1989) Liver transplantation for hepatoblastoma in a child with congenital absence of the portal vein. Pediatr Radiol 20: pp. 113-114 CrossRef
  15. Kim, SZ, Marz, PL, Laor, T, Teitelbaum, J, Jonas, MM, Levy, HL (1998) Elevated galactose in newborn screening due to congenital absence of the portal vein. Eur J Pediatr 157: pp. 608-609 CrossRef
  16. Peker, A, Ucar, T, Kuloglu, Z, Ceyhan, K, Tutar, E, Fitoz, S (2009) Congenital absence of portal vein associated with nodular regenerative hyperplasia of the liver and pulmonary hypertension. Clin Imaging 33: pp. 322-325 CrossRef
  17. Tsutsui, M, Sugahara, S, Motosuneya, T, Wada, H, Fukuda, I, Umeda, E, Kazama, T (2009) Anesthetic management of a child with Costello syndrome complicated by congenital absence of the portal vein: a case report. Paediatr Anaesth 19: pp. 714-715 CrossRef
  18. Kasahara, M, Nakagawa, A, Sakamoto, S, Tanaka, H, Shigeta, T, Fukuda, A, Nosaka, S, Matsui, A (2009) Living donor liver transplantation for congenital absence of the portal vein with situs inversus. Liver Transpl 15: pp. 1641-1643 CrossRef
  19. Lundstedt, C, Lindell, G, Tranberg, KG, Svartholm, E (2001) Congenital absence of the intrahepatic portion of the portal vein in an adult male resected for hepatocellular carcinoma. Eur Radiol 11: pp. 2228-2231 CrossRef
  20. Marois, D, Heerden, JA, Carpenter, HA, Sheedy, PF (1979) Congenital absence of the portal vein. Mayo Clin Proc 54: pp. 55-59
  21. Kawano, S, Hasegawa, S, Urushihara, N, Okazaki, T, Yoshida, A, Kusafuka, J, Mimaya, J, Horikoshi, Y, Aoki, K, Hamazaki, M (2007) Hepatoblastoma with congenital absence of the portal vein - a case report. Eur J Pediatr Surg 17: pp. 292-294 CrossRef
  22. Kamiya, S, Taniguchi, I, Yamamoto, T, Sawamura, S, Kai, M, Ohnishi, N, Tsuda, M, Yamamura, M, Nakasaki, H, Yokoyama, S (1993) Analysis of intestinal flora of a patient with congenital absence of the portal vein. FEMS Immunol Med Microbiol 7: pp. 73-80 CrossRef
  23. Wojcicki, M, Haagsma, EB, Gouw, AS, Slooff, MJ, Porte, RJ (2004) Orthotopic liver transplantation for portosystemic encephalopathy in an adult with congenital absence of the portal vein. Liver Transpl 10: pp. 1203-1207 CrossRef
  24. Tateishi, Y, Furuya, M, Kondo, F, Torii, I, Nojiri, K, Tanaka, Y, Umeda, S, Okudela, K, Inayama, Y, Endo, I, Ohashi, K (2013) Hepatocyte nuclear factor-1 伪 inactivatedhepatocellular adenomas in patient with congenital absence of the portal vein: a case report. Pathol Int 63: pp. 358-363 CrossRef
  25. Asran, MK, Loyer, EM, Kaur, H, Choi, H (2012) Case 177: Congenital absence of the portal vein with hepatic adenomatosis. Radiology 262: pp. 364-367 CrossRef
  26. Grazioli, L, Alberti, D, Olivetti, L, Rigamonti, W, Codazzi, F, Matricardi, L, Fugazzola, C, Chiesa, A (2000) Congenital absence of portal vein with nodular regenerative hyperplasia of the liver. Eur Radiol 10: pp. 820-825 CrossRef
  27. Takeichi, T, Okajima, H, Suda, H, Hayashida, S, Iwasaki, H, Ramirez, MZ, Ueno, M, Asonuma, K, Inomata, Y (2005) Living domino liver transplantation in an adult with congenital absence of portal vein. Liver Transpl 11: pp. 1285-1288 CrossRef
  28. Tsuji, K, Naoki, K, Tachiyama, Y, Fukumoto, A, Ishida, Y, Kuwabara, T, Sumida, T, Tatsugami, M, Nagata, S, Ohgoshi, H, Uraki, S, Hidaka, T, Akagi, Y, Ishine, M, Saeki, S, Chayama, K (2005) A case of congenital absence of the portal vein. Hepatol Res 31: pp. 43-47 CrossRef
  29. Takagaki, K, Kodaira, M, Kuriyama, S, Isogai, Y, Nogaki, A, Ichikawa, N, Kajimura, M (2004) Congenital absence of the portal vein complicating hepatic tumors. Intern Med 43: pp. 194-198 CrossRef
  30. Cheung, KM, Lee, CY, Wong, CT, Chan, AK (2005) Congenital absence of portal vein presenting as hepatopulmonary syndrome. J Paediatr Child Health 41: pp. 72-75 CrossRef
  31. Wakamoto, H, Manabe, K, Kobayashi, H, Hayashi, M (1999) Subclinical portal-systemic encephalopathy in a child with congenital absence of the portal vein. Brain Dev 21: pp. 425-428 CrossRef
  32. Guariso, G, Fiorio, S, Altavilla, G, Gamba, PG, Toffolutti, T, Chiesura-Corona, M, Tedeschi, U, Zancan, L (1998) Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and cystic dysplasia of the kidney. Eur J Pediatr 157: pp. 287-290 CrossRef
  33. Morse, SS, Taylor, KJ, Strauss, EB, Ramirez, E, Seashore, JH (1986) Congenital absence of the portal vein in oculoauriculovertebral dysplasia (Goldenhar syndrome). Pediatr Radiol 16: pp. 437-439 CrossRef
  34. Matsuoka, Y, Ohtomo, K, Okubo, T, Nishikawa, J, Mine, T, Ohno, S (1992) Congenital absence of the portal vein. Gastrointest Radiol 17: pp. 31-33 CrossRef
  35. Ringe, K, Schirg, E, Melter, M, Flemming, P, Ringe, B, Becker, T, Galanski, M (2008) Congenital absence of the portal vein (CAPV). Two cases of Abernethy malformation type 1 and review of the literature. Radiologe 48: pp. 493-502 CrossRef
  36. Schmidt, S, Saint-Paul, MC, Anty, R, Bruneton, JN, Gugenheim, J, Chevallier, P (2006) Multiple focal nodular hyperplasia of the liver associated with congenital absence of the portal vein. Gastroenterol Clin Biol 30: pp. 310-313 CrossRef
  37. Tanaka, Y, Takayanagi, M, Shiratori, Y, Imai, Y, Obi, S, Tateishi, R, Kanda, M, Fujishima, T, Akamatsu, M, Koike, Y, Hamamura, K, Teratani, T, Ishikawa, T, Shiina, S, Kojiro, M, Omata, M (2003) Congenital absence of portal vein with multiple hyperplastic nodular lesions in the liver. J Gastroenterol 38: pp. 288-294 CrossRef
  38. Kinjo, T, Aoki, H, Sunagawa, H, Kinjo, S, Muto, Y (2001) Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital choledochal cyst: a case report. J Pediatr Surg 36: pp. 622-625 CrossRef
  39. Motoori, S, Shinozaki, M, Goto, N, Kondo, F (1997) Case report: congenital absence of the portal vein associated with nodular hyperplasia in the liver. J Gastroenterol Hepatol 12: pp. 639-643 CrossRef
  40. Chandler, TM, Heran, MK, Chang, SD, Parvez, A, Harris, AC (2011) Multiple focal nodular hyperplasia lesions of the liver associated with congenital absence of the portal vein. Magn Reson Imaging 29: pp. 881-886 CrossRef
  41. Morotti, RA, Killackey, M, Shneider, BL, Repucci, A, Emre, S, Thung, SN (2007) Hepatocellular carcinoma and congenital absence of the portal vein in a child receiving growth hormone therapy for turner syndrome. Semin Liver Dis 27: pp. 427-431 CrossRef
  42. Scheuermann, U, Foltys, D, Otto, G (2012) Focal nodular hyperplasia precedes hepatocellular carcinoma in an adult with congenital absence of the portal vein. Transpl Int 25: pp. e67-e68 CrossRef
  43. Attal, P, Vilgrain, V, Brancatelli, G, Paradis, V, Terris, B, Belghiti, J, Taouli, B, Menu, Y (2003) Telangiectatic focal nodular hyperplasia: US, CT, and MR imaging findings with histopathologic correlation in 13 cases. Radiology 228: pp. 465-472 CrossRef
  44. Grazioli, L, Morana, G, Federle, MP, Brancatelli, G, Testoni, M, Kirchin, MA, Menni, K, Olivetti, L, Nicoli, N, Procacci, C (2001) Focal nodular hyperplasia: morphological and functional information from MR imaging with gadobenate dimeglumine. Radiology 221: pp. 731-739 CrossRef
  45. Grazioli, L, Morana, G, Kirchin, MA, Schneider, G (2005) Accurate differentiation of focal nodular hyperplasia from hepatic adenoma at gadobenate dimeglumine 鈥?enhanced MR imaging: prospective study. Radiology 236: pp. 166-177 CrossRef
  46. Laverdiere, JT, Laor, T, Benacerraf, B (1995) Congenital absence of the portal vein: case report and MR demonstration. Pediatr Radiol 25: pp. 52-53 CrossRef
  47. 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: pp. e1-e3 CrossRef
  48. Gocmen, R, Akhan, O, Talim, B (2007) Congenital absence of the portal vein associated with congenital hepatic fibrosis. Pediatr Radiol 37: pp. 920-924 CrossRef
  49. Sanada, Y, Mizuta, K, Kawano, Y, Egami, S, Hayashida, M, Wakiya, T, Mori, M, Hishikawa, S, Morishima, K, Fujiwara, T, Sakuma, Y, Hyodo, M, Yasuda, Y, Kobayashi, E, Kawarasaki, H (2009) Living donor liver transplantation for congenital absence of the portal vein. Transplant Proc 41: pp. 4214-4219 CrossRef
  50. Sumida, W, Kaneko, K, Ogura, Y, Tainaka, T, Ono, Y, Seo, T, Kiuchi, T, Ando, H (2006) Living donor liver transplantation for congenital absence of the portal vein in a child with cardiac failure. J Pediatr Surg 41: pp. e9-e12 CrossRef
  51. Uchida, H, Sakamoto, S, Shigeta, T, Hamano, I, Kanazawa, H, Fukuda, A, Karaki, C, Nakazawa, A, Kasahara, M (2012) Living donor liver transplantation with renoportal anastomosis for a patient with congenital absence of the portal vein. Case Rep Surg 2012: pp. 670289
  52. Matsuura, T, Soejima, Y, Taguchi, T (2010) Auxiliary partial orthotopic living donorliver transplantation with a small-for-size graft for congenital absence of the portal vein. Liver Transpl 16: pp. 1437-1439 CrossRef
  
• 刊物主题：Medicine/Public Health, general;
• 出版者：BioMed Central
• ISSN：2047-783X

文摘

Background Patients with congenital absence of the portal vein (CAPV) often suffer from additional medical complications such as hepatic tumors and cardiac malformations. Case presentation Congenital absence of the portal vein (CAPV) is a rare malformation. We present a case of a 16-year-old Chinese girl with CAPV with multiple pathology-proven hepatic focal nodular hyperplasias (FNHs) and ventricular septal defect (VSD). The CT and MRI features of this case are described, and previously reported cases are reviewed. Conclusions CAPV is a rare congenital anomaly and in such patients, clarifying the site of portosystemic shunts, liver disease, and other anomalies is critical for appropriate treatment selection and accurate prognosis determination. Close follow-up, including laboratory testing and radiologic imaging, is recommended for all CAPV patients.