Skip to main content
NCBI home page
Annals of Surgery logoLink to Annals of Surgery
. 1986 Jul;204(1):72–77. doi: 10.1097/00000658-198607000-00010

Segmental portal hypertension.

M S Madsen, T H Petersen, H Sommer
PMCID: PMC1251225  PMID: 3729585

Abstract

Isolated obstruction of the splenic vein leads to segmental portal hypertension, which is a rare form of extrahepatic portal hypertension, but it is important to diagnose, since it can be cured by splenectomy. In a review of the English literature, 209 patients with isolated splenic vein obstruction were found. Pancreatitis caused 65% of the cases and pancreatic neoplasms 18%, whereas the rest was caused by various other diseases. Seventy-two per cent of the patients bled from gastroesophageal varices, and most often the bleeding came from isolated gastric varices. The spleen was enlarged in 71% of the patients. A correct diagnosis in connection with the first episode of bleeding was made in only 49%; 22% were operated on because of gastrointestinal bleeding, but the cause of bleeding was not found. The diagnosis should be suspected in patients with gastroesophageal varices, but without signs of a liver disease, especially if isolated gastric varices are found. The diagnosis is confirmed by portography.

Full text

PDF
72

Images in this article

74FIG. 1.
on p.74
74FIG. 2.
on p.74

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Alwmark A., Gullstrand P., Ihse I., Joelsson B., Owman T. Regional, portal hypertension in chronic pancreatitis. Acta Chir Scand. 1981;147(2):155–157. [PubMed] [Google Scholar]
  2. Amarnani N. H., Broor S. L., Varma R. R., Maddison F., Geenen J. E. Splenic vein thrombosis: a cause of gastric varices and upper gastrointestinal bleeding. Gastrointest Endosc. 1977 Nov;24(2):83–85. doi: 10.1016/s0016-5107(77)73460-1. [DOI] [PubMed] [Google Scholar]
  3. Aron R., Zapolanski A., Cooperman A. M., Hermann R. E. Regional portal hypertension. Cleve Clin Q. 1979 Spring;46(1):1–5. doi: 10.3949/ccjm.46.1.1. [DOI] [PubMed] [Google Scholar]
  4. Babb R. R. Editorial: Splenic vein obstruction: a curable cause of variceal bleeding. Am J Dig Dis. 1976 Jun;21(6):512–513. doi: 10.1007/BF01072140. [DOI] [PubMed] [Google Scholar]
  5. Belli L., Puttini M., Marni A. Extrahepatic portal obstruction: Clinical experience and surgical treatment in 105 patients. J Cardiovasc Surg (Torino) 1980 Jul-Aug;21(4):439–448. [PubMed] [Google Scholar]
  6. Ben-Ari G., Mitty H., Kark A. E., Rudick J. Segmental portal hypertension due to congenital cavernous malformation of splenic vein. Mt Sinai J Med. 1972 May-Jun;39(3):271–278. [PubMed] [Google Scholar]
  7. Bok E. J., Cho K. J., Williams D. M., Brady T. M., Weiss C. A., Forrest M. E. Venous involvement in islet cell tumors of the pancreas. AJR Am J Roentgenol. 1984 Feb;142(2):319–322. doi: 10.2214/ajr.142.2.319. [DOI] [PubMed] [Google Scholar]
  8. Bunt T. J., Hackler M. T., Greene F. L. Isolated splenic vein thrombosis: the curable variceal hemorrhage. South Med J. 1983 Jul;76(7):936–938. doi: 10.1097/00007611-198307000-00036. [DOI] [PubMed] [Google Scholar]
  9. Burbige E. J., Tarder G., Carson S., Eugene J., Frey C. F. Colonic varices. A complication of pancreatitis with splenic vein thrombosis. Am J Dig Dis. 1978 Aug;23(8):752–755. doi: 10.1007/BF01072365. [DOI] [PubMed] [Google Scholar]
  10. Burcharth F., Nielbo N., Andersen B. Percutaneous transhepatic portography. II. Comparison with splenoportography in portal hypertension. AJR Am J Roentgenol. 1979 Feb;132(2):183–185. doi: 10.2214/ajr.132.2.183. [DOI] [PubMed] [Google Scholar]
  11. Burcharth F. Percutaneous transhepatic portography. I. Technique and application. AJR Am J Roentgenol. 1979 Feb;132(2):177–182. doi: 10.2214/ajr.132.2.177. [DOI] [PubMed] [Google Scholar]
  12. Byerly W. G. Thrombosis of the splenic vein with secondary rupture of the spleen. N C Med J. 1975 Jun;36(6):352–354. [PubMed] [Google Scholar]
  13. Cho K. J., Martel W. Recognition of splenic vein occlusion. AJR Am J Roentgenol. 1978 Sep;131(3):439–443. doi: 10.2214/ajr.131.3.439. [DOI] [PubMed] [Google Scholar]
  14. Codd J. E. Splenic vein thrombosis. Case report. Mo Med. 1976 Jan;73(1):22–24. [PubMed] [Google Scholar]
  15. Cope J. R., Clendinnen D. G., Jacquet N. Malignant splenic vein obstruction. Br J Radiol. 1972 Nov;45(539):855–857. doi: 10.1259/0007-1285-45-539-855. [DOI] [PubMed] [Google Scholar]
  16. Cosgrove H., Legge D., O'Connell F. X., Weir D. Angiographic evaluation of gastrointestinal haemorrhage complicating pancreatic disease. Clin Radiol. 1978 May;29(3):289–293. doi: 10.1016/s0009-9260(78)80066-x. [DOI] [PubMed] [Google Scholar]
  17. Cummings R., Raju S. Splenic vein thrombosis: a curable form of portal hypertension. J Miss State Med Assoc. 1982 May;23(5):137–138. [PubMed] [Google Scholar]
  18. Dewar G. Regional portal hypertension. S Afr J Surg. 1983 Mar;21(1):69–71. [PubMed] [Google Scholar]
  19. Fitzer P. M., Stephens J. R. Splenic vein obstruction with bleeding gastric varices: a diagnostic approach. Va Med. 1978 Dec;105(12):850–853. [PubMed] [Google Scholar]
  20. Gallardo-Navarra V., Phillips E., Neyfeld P., Sakwa S. Letter: Segmental portal hypertension and islet cell adenoma of the pancreas. JAMA. 1973 Dec 17;226(12):1466–1466. [PubMed] [Google Scholar]
  21. Goldberg S., Katz S., Naidich J., Waye J. Isolated gastric varices due to spontaneous splenic vein thrombosis. Am J Gastroenterol. 1984 Apr;79(4):304–307. [PubMed] [Google Scholar]
  22. Goldstein G. B. Splenic vein thrombosis causing gastric varices and bleeding. Am J Gastroenterol. 1972 Sep;58(3):319–325. [PubMed] [Google Scholar]
  23. Haff R. C., Page C. P., Andrassy R. J., Buckley C. J. Splenectomy: its place in operations for inflammatory disease of the pancreas. Am J Surg. 1977 Nov;134(5):555–557. doi: 10.1016/0002-9610(77)90432-9. [DOI] [PubMed] [Google Scholar]
  24. Hamilton D. W., Hunt A. H. Extrahepatic portal obstruction. Med J Aust. 1970 Mar 7;1(10):493–499. doi: 10.5694/j.1326-5377.1970.tb78007.x. [DOI] [PubMed] [Google Scholar]
  25. Harnar T., Johansen K., Haskey R., Barker E. Left-sided portal hypertension from pancreatic pseudotumor. Am J Gastroenterol. 1982 Sep;77(9):639–641. [PubMed] [Google Scholar]
  26. Hasan A., Ahmed M. Isolated splenic vein occlusion: a report of two cases. J Pak Med Assoc. 1982 Mar;32(3):79–80. [PubMed] [Google Scholar]
  27. Hazenberg H. J., Gips C. H. 13N-ammonia test and urea index in a patient with splenic vein obstruction and cirrhosis of the liver. Neth J Med. 1976;19(3):127–134. [PubMed] [Google Scholar]
  28. Herlinger H. Arterioportography. Clin Radiol. 1978 May;29(3):255–275. doi: 10.1016/s0009-9260(78)80062-2. [DOI] [PubMed] [Google Scholar]
  29. Honda Y., Ueda M., Kyoi M., Mizunuma T., Takeda R. An unusual case of portasystemic encephalopathy caused by splenic vein occlusion following gastrectomy. Am J Gastroenterol. 1978 May;69(5):590–593. [PubMed] [Google Scholar]
  30. Itzchak Y., Glickman M. G. Duodenal varices in extrahepatic portal obstruction. Radiology. 1977 Sep;124(3):619–624. doi: 10.1148/124.3.619. [DOI] [PubMed] [Google Scholar]
  31. Itzchak Y., Glickman M. G., Gottschalk A., Lange R., Downing S. E. Hemodynamic and morphologic evaluation of the spleen after splenic vein ligation in the dog. Invest Radiol. 1978 Mar-Apr;13(2):155–160. doi: 10.1097/00004424-197803000-00010. [DOI] [PubMed] [Google Scholar]
  32. Itzchak Y., Glickman M. G. Splenic vein thrombosis in patients with a normal size spleen. Invest Radiol. 1977 Mar-Apr;12(2):158–163. doi: 10.1097/00004424-197703000-00010. [DOI] [PubMed] [Google Scholar]
  33. Jeanty P., Brion J. P., Van Gossum A., Struyven J. Portal and splenic vein thrombosis: ultrasonic demonstration. J Belge Radiol. 1982;65(1):45–47. [PubMed] [Google Scholar]
  34. Johnston F. R., Myers R. T. Etiologic factors and consequences of splenic vein obstruction. Ann Surg. 1973 Jun;177(6):736–739. doi: 10.1097/00000658-197306000-00012. [DOI] [PMC free article] [PubMed] [Google Scholar]
  35. Jones K. B., de Koos P. T. Postembolization splenic abscess in a patient with pancreatitis and splenic vein thrombosis. South Med J. 1984 Mar;77(3):390–393. doi: 10.1097/00007611-198403000-00033. [DOI] [PubMed] [Google Scholar]
  36. Keith R. G., Mustard R. A., Jr, Saibil E. A. Gastric variceal bleeding due to occlusion of splenic vein in pancreatic disease. Can J Surg. 1982 May;25(3):301–304. [PubMed] [Google Scholar]
  37. Khan A. H., O'Reilly C. J., Avakian V. A., Lucina P. A. Splenic vein thrombosis: an unusual case of gastric bleeding. Angiology. 1977 Oct;28(10):725–727. doi: 10.1177/000331977702801010. [DOI] [PubMed] [Google Scholar]
  38. Koehler R. E. Case: splenic vein obstruction due to metastatic hypernephroma. Gastrointest Radiol. 1981;6(4):365–370. [PubMed] [Google Scholar]
  39. Lane F. C., Amin P. Acute pancreatitis--at what point should you consider surgery? Med Times. 1980 Jun;108(6):38–43. [PubMed] [Google Scholar]
  40. Larmi T. K., Mokka R. E., Kairaluoma M. I., Huttunen R., Klintrup H-E Gastric bleeding due to segmental portal hypertension. A case report. Acta Chir Scand. 1976;142(8):609–610. [PubMed] [Google Scholar]
  41. Lavender S., Lloyd-Davies R. W., Thomas M. L. Retroperitoneal fibrosis causing localized portal hypertension. Br Med J. 1970 Sep 12;3(5723):627–628. doi: 10.1136/bmj.3.5723.627. [DOI] [PMC free article] [PubMed] [Google Scholar]
  42. Law D. K., Moore E. E. Compartmentalized gastrosplenic and mesenteric venous hypertension after distal splenorenal shunt occlusion: response to mesocaval shunt and splenectomy. Surgery. 1979 May;85(5):579–582. [PubMed] [Google Scholar]
  43. Leger L., Lenriot J. P., Lemaigre G. L'hypertension et la stase portales segmentaires dans les pancréatites chroniques. A propos de 126 cas examinés par spléno-portographie et spléno-manométrie. J Chir (Paris) 1968 May-Jun;95(5):599–608. [PubMed] [Google Scholar]
  44. Little A. G., Moossa A. R. Gastrointestinal hemorrhage from left-sided portal hypertension. An unappreciated complication of pancreatitis. Am J Surg. 1981 Jan;141(1):153–158. doi: 10.1016/0002-9610(81)90029-5. [DOI] [PubMed] [Google Scholar]
  45. Longstreth G. F., Newcomer A. D., Green P. A. Extrahepatic portal hypertension caused by chronic pancreatitis. Ann Intern Med. 1971 Dec;75(6):903–908. doi: 10.7326/0003-4819-75-6-903. [DOI] [PubMed] [Google Scholar]
  46. Looby W. E., Bennett J. R., Rehman A. Splenic hypertension in pancreatitis. Rocky Mt Med J. 1969 Aug;66(8):29–32. [PubMed] [Google Scholar]
  47. Lynch C. T., Jr, Maunz D. L. Gastroduodenal varices secondary to splenic vein thrombosis. J Maine Med Assoc. 1977 Dec;68(12):455–458. [PubMed] [Google Scholar]
  48. Maclean N., Falconer C. W., Gilmour I. E., Webb F. N. Islet cell tumours of the pancreas with portal varices and gastrointestinal haemorrhage. J R Coll Surg Edinb. 1970 Jul;15(4):206–212. [PubMed] [Google Scholar]
  49. Manenti A. Splenic vein obstruction secondary to pancreatic carcinoma. Acta Chir Belg. 1981 Sep-Oct;80(5):245–248. [PubMed] [Google Scholar]
  50. Marshall J. P., 2nd, Smith P. D., Hoyumpa A. M., Jr Gastric varices. Problem in diagnosis. Am J Dig Dis. 1977 Nov;22(11):947–955. doi: 10.1007/BF01076192. [DOI] [PubMed] [Google Scholar]
  51. Muhletaler C., Gerlock A. J., Jr, Goncharenko V., Avant G. R., Flexner J. M. Gastric varices secondary to splenic vein occlusion: radiographic diagnosis and clinical significance. Radiology. 1979 Sep;132(3):593–598. doi: 10.1148/132.3.593. [DOI] [PubMed] [Google Scholar]
  52. Negus D., Cotton P. Splenic vein thrombosis with pancreatic pseudo-cyst. Proc R Soc Med. 1973 Jul;66(7):649–650. [PMC free article] [PubMed] [Google Scholar]
  53. Novis B. H., Duys P., Barbezat G. O., Clain J., Bank S., Terblanche J. Fibreoptic endoscopy and the use of the Sengstaken tube in acute gastrointestinal haemorrhage in patients with portal hypertension and varices. Gut. 1976 Apr;17(4):258–263. doi: 10.1136/gut.17.4.258. [DOI] [PMC free article] [PubMed] [Google Scholar]
  54. Porter B. A., Frey C. F., Link D. P., Lantz B. M., Pimstone N. R. Splenic embolization monitored by the video dilution technique. AJR Am J Roentgenol. 1983 Nov;141(5):1063–1065. doi: 10.2214/ajr.141.5.1063. [DOI] [PubMed] [Google Scholar]
  55. Probst P., Rysavy J. A., Amplatz K. Improved safety of splenoportography by plugging of the needle tract. AJR Am J Roentgenol. 1978 Sep;131(3):445–449. doi: 10.2214/ajr.131.3.445. [DOI] [PubMed] [Google Scholar]
  56. Rice R. P., Thompson W. M., Kelvin F. M., Kriner A. F., Garbutt J. T. Gastric varices without esophageal varices. An important preendoscopic diagnosis. JAMA. 1977 May 2;237(18):1976–1979. [PubMed] [Google Scholar]
  57. Richman L. S., Boehnke M. Letter: Mesothelioma of the pericardium with cholesterol pericarditis. Angiology. 1974 Apr;25(4):300–303. doi: 10.1177/000331977402500409. [DOI] [PubMed] [Google Scholar]
  58. Rignault D., Mine J., Moine D. Splenoportographic changes in chronic pancreatitis. Surgery. 1968 Apr;63(4):571–575. [PubMed] [Google Scholar]
  59. Ruzicka F. F., Jr, Rossi P. Arterial portography: patterns of venous flow. Radiology. 1969 Mar;92(4):777–787. doi: 10.1148/92.4.777. [DOI] [PubMed] [Google Scholar]
  60. Rösch J. Die Splenoportographie in der Diagnostik der Pankreaserkrankungen. Radiologe. 1965 Aug;5(8):274–281. [PubMed] [Google Scholar]
  61. Røder O. C. Splenic vein thrombosis with bleeding gastroesophageal varices. Reports of two splenectomized cases and review of the literature. Acta Chir Scand. 1984;150(3):265–268. [PubMed] [Google Scholar]
  62. Salam A. A., Goldman M., Smith D., Jr, Hill H. L. Gastric, intestinal, and gallbladder varices: hemodynamic and therapeutic considerations. South Med J. 1979 Apr;72(4):402–408. doi: 10.1097/00007611-197904000-00010. [DOI] [PubMed] [Google Scholar]
  63. Salam A. A., Warren W. D., Tyras D. H. Splenic vein thrombosis: a diagnosable and curable form of portal hypertension. Surgery. 1973 Dec;74(6):961–972. [PubMed] [Google Scholar]
  64. Scott R. L., Himmelfarb E. H. X-ray of the month: splenic vein thrombosis. J Tenn Med Assoc. 1976 Mar;69(3):197–198. [PubMed] [Google Scholar]
  65. Sheers R. A pancreatic cystadenoma complicated by varices: case report. Br J Surg. 1980 Feb;67(2):144–145. doi: 10.1002/bjs.1800670225. [DOI] [PubMed] [Google Scholar]
  66. Smith-Laing G., Camilo M. E., Dick R., Sherlock S. Percutaneous transhepatic portography in the assessment of portal hypertension. Clinical correlations and comparison of radiographic techniques. Gastroenterology. 1980 Feb;78(2):197–205. [PubMed] [Google Scholar]
  67. Smith S. J., Wilson J. H. Splenic vein obstruction. Neth J Med. 1974;17(2):54–57. [PubMed] [Google Scholar]
  68. Smulewicz J. J., Clemett A. R. Torsion of the wandering spleen. Am J Dig Dis. 1975 Mar;20(3):274–279. doi: 10.1007/BF01070730. [DOI] [PubMed] [Google Scholar]
  69. Stahl Madsen M., Sommer H., Petersen T. H., Friis J., Moesgaard F. Variceal bleeding caused by segmental portal hypertension in association with situs inversus and malrotation. Ann Chir Gynaecol. 1985;74(4):185–187. [PubMed] [Google Scholar]
  70. Stone R. T., Wilson S. E., Passaro E., Jr Gastric portal hypertension. Am J Surg. 1978 Jul;136(1):73–79. doi: 10.1016/0002-9610(78)90203-9. [DOI] [PubMed] [Google Scholar]
  71. Sutton J. P., Yarborough D. Y., Richards J. T. Isolated splenic vein occlusion. Review of literature and report of an additional case. Arch Surg. 1970 May;100(5):623–626. doi: 10.1001/archsurg.1970.01340230089024. [DOI] [PubMed] [Google Scholar]
  72. Turrill F. L., Mikkelsen W. P. "Sinistral" (left-sided) extrahepatic portal hypertension. Arch Surg. 1969 Sep;99(3):365–368. doi: 10.1001/archsurg.1969.01340150073014. [DOI] [PubMed] [Google Scholar]
  73. Tylén U., Arnesjö B. Angiographic diagnosis of inflammatory disease of the pancreas. Acta Radiol Diagn (Stockh) 1973 Mar;14(2):215–240. doi: 10.1177/028418517301400209. [DOI] [PubMed] [Google Scholar]
  74. Vos L. J., Potocky V., Bröker F. H., de Vries J. A., Postma L., Edens E. Splenic vein thrombosis with oesophageal varices: a late complication of umbilical vein catheterization. Ann Surg. 1974 Aug;180(2):152–156. doi: 10.1097/00000658-197408000-00004. [DOI] [PMC free article] [PubMed] [Google Scholar]
  75. Warshaw A. L., Chesney T. M., Evans G. W., McCarthy H. F. Intrasplenic dissection by pancreatic pseudocysts. N Engl J Med. 1972 Jul 13;287(2):72–75. doi: 10.1056/NEJM197207132870204. [DOI] [PubMed] [Google Scholar]
  76. Webb L. J., Berger L. A., Sherlock S. Grey-scale ultrasonography of portal vein. Lancet. 1977 Oct 1;2(8040):675–677. doi: 10.1016/s0140-6736(77)90492-5. [DOI] [PubMed] [Google Scholar]
  77. Wexler M. J., MacLean L. D. Massive spontaneous portal-systemic shunting without varices. Arch Surg. 1975 Aug;110(8):995–1003. doi: 10.1001/archsurg.1975.01360140139027. [DOI] [PubMed] [Google Scholar]
  78. Wiechel K. L. Direct selective catheterization of the portal venous system. Scand J Gastroenterol Suppl. 1979;53:123–130. [PubMed] [Google Scholar]
  79. Wilson A. J. Isolated splenic vein thrombosis. Br J Clin Pract. 1978 Jan;32(1):27–29. [PubMed] [Google Scholar]
  80. Wolf J. H., Long R. J., Miller F. J., Jr, Jeffries G. H. Pancreatic islet cell tumor presenting as bleeding gastric varices secondary to splenic vein occlusion. Am J Dig Dis. 1977 Jul;22(7):652–655. doi: 10.1007/BF01073088. [DOI] [PubMed] [Google Scholar]
  81. Wolloch Y., Chaimoff C., Lubin E., Dintsman M. Splenic vein thrombosis, segmental portal hypertension and bleeding esophageal varices produced by a congenital pancreatic cyst. Isr J Med Sci. 1974 Jun;10(6):670–673. [PubMed] [Google Scholar]
  82. Yale C. E., Crummy A. B. Splenic vein thrombosis and bleeding esophageal varices. JAMA. 1971 Jul 19;217(3):317–320. [PubMed] [Google Scholar]
  83. Zannini G., Masciariello S., Pagano G., Sangiuolo P., Iaccarino V. Prehepatic portal hypertension: experience with 88 cases. Int Surg. 1982 Oct-Dec;67(4):311–315. [PubMed] [Google Scholar]

Articles from Annals of Surgery are provided here courtesy of Lippincott, Williams, and Wilkins

RESOURCES