Left Sided

Ulster Med J 2006; 75 (3) 175-177
Sinistral portal hypertension
175
Clinical Review

Richard J Thompson, Mark A Taylor, Lloyd D McKie, Thomas Diamond
Accepted 4th May 2006
ABSTRACT
Sinistral, or left-sided, portal hypertension is a rare cause of upper gastrointestinal haemorrhage. There are many causes
of sinistral portal hypertension. The primary pathology usually arises in the pancreas and results in compression of the
pancreatic vein. This compression causes backpressure in the left portal venous system and subsequent gastric varices.
Management is usually surgical to treat the underlying pathology and splenectomy to decompress the left portal venous
system.
This paper presents four cases of sinistral portal hypertension followed by a literature review of the reported causes and
management issues.
KEYWORDS Left-sided portal hypertension, Sinistral portal hypertension, Upper gastrointestinal haemorrhage, Gastric
varices
INTRODUCTION
Sinistral, or left-sided, portal hypertension is a rare cause of

upper gastrointestinal haemorrhage. Isolated gastric varices
result from thrombosis or obstruction of the splenic vein
resulting in back pressure changes in the left portal system.
The primary pathology usually arises in the pancreas and
common aetiologies include pancreatitis and pancreatic
neoplasms. Four illustrative case histories from patients
with sinistral portal hypertension are discussed, followed
by a review of the literature highlighting the aetiology and
management of this condition.
CASE SERIES
Case 1
A 57 year old lady presented with upper gastrointestinal
bleeding. She had a past medical history of retroperitoneal
fibrosis and had a previous incidental finding of a cyst at
the splenic hilum, associated with splenomegaly. No active
bleeding source was identified at gastroscopy. Following
a rebleed, she underwent a laparotomy, which revealed
prominent veins around the gastric fundus and confirmed
the presence of splenomegaly. Two large actively bleeding
gastric varices were ligated. A second laparotomy, following
a further rebleed ten days post-operatively, revealed a cystic
inflammatory mass in the tail of the pancreas associated
with the other features previously noted. There was no
macroscopic evidence of liver cirrhosis. Deroofing of the cyst
and splenectomy were carried out. Histology revealed benign
chronic inflammatory features in keeping with a pancreatic
pseudocyst. She has remained well with no further bleeding.
Case 2
A 53 year old man presented with melaena. He had no history
of pancreatitis or alcohol excess. Gastroscopy revealed a mass
of varices in the gastric fundus but no oesophageal varices
The Ulster Medical Society, 2006.
or evidence of generalised portal hypertensive gastropathy.

CT scan showed a mass between the tail of the pancreas
and the splenic hilum. CA 19-9 was elevated at 624 IU/ml.
Laparotomy confirmed a mass in the tail of the pancreas
but it was unresectable due to infiltration of the posterior
abdominal wall. Grossly dilated veins were also noted
around the greater and lesser curves of the stomach. Biopsy
revealed a neuroendocrine (islet cell) tumour of the pancreas.
Immunohistochemistry confirmed a somatostatinoma. He has
remained symptomatically well with no further bleeding.
Case 3
A 42 year old woman presented with an upper gastrointestinal
bleed. She had no history of liver disease, alcohol excess
or peptic ulcer disease. She had a past history of breast
carcinoma. Gastroscopy failed to reveal an exact cause of the
bleeding. There were no oesophageal varices and no duodenal
ulceration. A laparotomy was undertaken for rebleeding and
this showed bleeding gastric varices, which were ligated
and decompressed by division of several of the short gastric
vessels. CT scan revealed a low-density lesion in the tail
of the pancreas associated with moderate splenomegaly
(Figure). CA 19-9 was elevated at 779 IU/ml. Subsequent
staging laparotomy revealed dilated vessels around the
greater curvature and multiple intraperitoneal deposits in
keeping with metastases. Histopathology indicated metastatic
pancreatic adenocarcinoma. Pancreatectomy and splenectomy
were not attempted in view of the advanced disease.
Surgical Unit, Mater Hospital, Crumlin Road, Belfast,

BT14 6AB.
Correspondence to Mr Diamond
E-mail: [email protected]
www.ums.ac.uk
176
The Ulster Medical Journal
Case 4
A 41 year old man had a past medical history of
alcoholic pancreatitis and had a previous pancreatic
pseudocystgastrostomy. He had multiple subsequent
admissions for upper gastrointestinal bleeding requiring
transfusion. Endoscopy revealed mild gastritis. Mesenteric
angiography was negative. Laparotomy was undertaken
following a massive rebleed. Dense adhesions were noted
around the pancreas and spleen in keeping with chronic
pancreatitis and the splenic vein was noted to be grossly
dilated and thrombosed. Gastrotomy revealed brisk
haemorrhage from the region of the gastrooesophageal
junction. Splenectomy and distal pancreatectomy were
undertaken followed by oesophageal transection to further
control the bleeding. He has had no further bleeds three years
following surgery.
Fig 1.
CT scan demonstrating a solid lesion in the tail of

pancreas with splenomegaly and enlargement of the
splenic vein (arrow).
DISCUSSION
Greenwald and Wasch first outlined the pathophysiology of

left-sided portal hypertension in 1939.1 The development
of this has been recognised as an important cause of upper
gastrointestinal bleeding resulting from gastric varices
secondary to pathology in the pancreas. The splenic vein
is susceptible in lesions of the pancreas due its close
anatomical course along the superior pancreatic surface.
The most common pathologies resulting in splenic vein
thrombosis or obstruction and leading to the phenomenon
of left-sided portal hypertension are chronic pancreatitis,2-4
pancreatic pseudocysts5 and pancreatic neoplasms. These
include benign neoplasms, adenocarcinoma and functioning
and non-functioning islet-cell (neuroendocrine) tumours.614
There are numerous other less common pathologies
reported. These include iatrogenic splenic vein injury,15
post-liver transplantation,16,17 a wandering/ectopic spleen,18,19
infiltration by colonic tumour,20 spontaneous splenic vein
thrombosis21 and perirenal abscess.22 This case series provides
examples of four distinct causes, a pancreatic pseudocyst, a
neuroendocrine tumour, an adenocarcinoma of the pancreas
and chronic pancreatitis.
Splenic vein occlusion results in back pressure which is
transmitted through its anastomoses with the short gastric and
gastroepiploic veins and subsequently via the coronary vein

into the portal system. This results in reversal of flow in these
veins and the formation of gastric varices. The hypertension is
confined to the left side of the portal system and is therefore
distinct from the common phenomenon of generalised portal
hypertension. Isolated gastric varices occur in left sided
and generalised portal hypertension,23 however, the more
common phenomenon of generalised portal hypertension
should initially be excluded. The diagnosis of sinistral portal
hypertension should be considered in all those with upper
gastrointestinal bleeding associated with splenomegaly and
normal liver function tests.24
Management of this condition traditionally involves surgical
removal of the primary cause if possible, combined with
splenectomy.2,5 Splenectomy decreases the arterial inflow
into the left portal system by ligation of the splenic artery,
resulting in decompression of the gastric varices. Prophylactic
splenectomy may not be necessary in all patients with
sinistral portal hypertension. The benefits of splenectomy
are obvious in the management of those with severe upper
gastrointestinal haemorrhage in order to rapidly reverse the
cause. However, it may be acceptable to undertake a more
conservative approach in those with more minor bleeds or in
asymptomatic patients.25 Embolisation of the splenic artery
has also been suggested as an alternative to splenectomy in
high-risk patients5 or as a preoperative measure to reduce
intra-operative blood loss.26
The overall prognosis for patients with sinistral portal
hypertension is clearly dependant on the primary pathology
but will invariably be poor in cases with a malignant primary
pathology, as involvement of the splenic vein implies
advanced infiltrative disease.27
CONCLUSION
Sinistral portal hypertension is an important cause

of potentially life threatening upper gastrointestinal
haemorrhage. Primary pancreatic pathology should be
considered in patients with isolated gastric varices and in
those with upper gastrointestinal haemorrhage associated
with splenomegaly in the absence of chronic liver disease.
Splenectomy is the appropriate management option when it
is associated with major gastrointestinal haemorrhage.
Conflict of interest: None declared
REFERENCES
1.
Greenwald HM, Wasch MG. The roentgenologic demonstration of

esophageal varices as a diagnostic aid in chronic thrombosis of the
splenic vein. J Pediatr. 1939;14:57-65.
2.
Sakorafas GH, Sarr MG, Farley DR, Farnell MB. The significance of
sinistral portal hypertension complicating chronic pancreatitis. Am J
Surg. 2000;179(2):129-33.
3.
Ruzicka M, Konecna D, Jordankova E. Portal hypertension as a

complication of chronic pancreatitis. Hepatogastroenterology.
1999;46(28):2582-4.
4.
Little AG, Moossa AR. Gastrointestinal haemorrhage from left-sided

hypertension. An unappreciated complication of pancreatitis. Am J Surg.
1981;141(1):153-8.
5.
Evans GR, Yellin AE, Weaver FA, Stain SC. Sinistral (left-sided) portal
hypertension. Am Surg. 1990;56(12):758-63.
www.ums.ac.uk
177
6.
Iwasaki T, Nagata Y, Watahiki H, Yamamoto H, Ogawa H. A rare case

of serous cystadenoma of the pancreas presenting with left-sided portal
hypertension. Surg Today. 1996;26(6):442-5.
17. Stevenson WC, Sawyer RG, Pruett TL. Recurrent variceal bleeding
after liver transplantation--persistent left-sided portal hypertension.
Transplantation. 1992;53(2):493-5.
7.
Chang CY. Pancreatic adenocarcinoma presenting as sinistral portal

hypertension: an unusual presentation of pancreatic cancer. Yale J Biol
Med. 1999;72(4):295-300.
18. Angeras U, Almskog B, Lukes P, Lundstam S, Weiss L. Acute

gastric hemorrhage secondary to wandering spleen. Dig Dis Sci.
1984;29(12):1159-63.
8.
Sahani D, Prasad SR, Maher M, Warshaw AL, Hahn PF, Saini S.

Functioning acinar cell pancreatic carcinoma: diagnosis on mangafodipir
trisodium (Mn-DPDP)-enhanced MRI. J Comput Assist Tomogr.
2002;26(1):126-8.
19. Melikoglu M, Colak T, Kavasoglu T. Two unusual cases of wandering

spleen requiring splenectomy. Eur J Pediatr Surg. 1995;5(1):48-9.
9.
Caggiano AV, Adams DB, Metcalf JS, Anderson MC. Neuroendocrine

tumor of the pancreas in a patient with pernicious anemia. Am Surg.
1990;56(6):347-51.
10. Dalvi AN, Rege SA, Bapat MR, Abraham P, Joshi AS, Bapat RD.
Nonfunctioning islet cell tumor presenting with ascites and portal
hypertension. Indian J Gastroenterol. 2002;21(6):227-8.
11. Watase M, Sakon M, Monden M, Miyoshi Y, Tono T, Ichikawa T, et al. A
case of splenic vein occlusion caused by the intravenous tumor thrombus
of nonfunctioning islet cell carcinoma. Surg Today. 1992;22(1):62-5.
12. Sheen-Chen SM, Eng HL, Wan YL, Chou FF. Acinar cell carcinoma
of the pancreas: a rare cause of left-sided portal hypertension. Am J
Gastroenterol. 1991;86(8):1070-2.
13. Metz DC, Benjamin SB. Islet cell carcinoma of the pancreas presenting
as bleeding from isolated gastric varices. Report of a case and review
of the literature. Dig Dis Sci. 1991;36(2):241-4.
14. Chellapa M, Yee CK, Gill DS. Left-sided portal hypertension from
malignant islet cell tumour of the pancreas: review with a case report.
J R Coll Surg Edinb. 1986;31(4):251-2.
15. Tsuchida S, Ku Y, Fukumoto T, Tominaga M, Iwasaki T, Kuroda Y.
Isolated gastric varices resulting from iatrogenic splenic vein occlusion:
report of a case. Surg Today. 2003;33(7):542-4.
16. Malassagne B, Soubrane O, Dousset B, Legmann P, Houssin D.
Extrahepatic portal hypertension following liver transplantation: a rare
but challenging problem. HPB Surg. 1998;10(6):357-63.
20. Seenu V, Goel AK, Shukla NK, Dawar R, Sood S. Hodgkins lymphoma
of colon: an unusual cause of isolated splenic vein obstruction. Indian
J Gastroenterol. 1994;13(2):70-1.
21. Goldberg S, Katz S, Naidich J, Waye J. Isolated gastric varices
due to spontaneous splenic vein thrombosis. Am J Gastroenterol.
1984;79(4):304-7.
22. Koklu S, Koksal A, Yolcu OF, Bayram G, Sakaogullari Z, Arda K, Sahin
B. Isolated splenic vein thrombosis: an unusual cause and review of the
literature. Can J Gastroenterol. 2004;18(3):173-4.
23. Nagral S, Shah S, Gandhi M, Mathur SK. Bleeding isolated gastric
varices: a retrospective analysis. Indian J Gastroenterol. 1999;18(2):6972.
24. Glynn MJ. Isolated splenic vein thrombosis. Arch Surg.1986; 121(6):723725.
25. Loftus JP, Nagorney DM, Illstrup D, Kunselman AR. Sinistral portal
hypertension. Splenectomy or expectant management. Ann Surg.
1993;217(1):35-40.
26. Adams DB, Mauterer DJ, Vujic IJ, Anderson MC. Preoperative control
of splenic artery inflow in patients with splenic venous occlusion. South
Med J. 1990;83(9):1021-4.
27. Hwang TL, Jan YY, Jeng LB, Chen MF, Hung CF, Chiu CT. The
different manifestation and outcome between pancreatitis and pancreatic
malignancy with left-sided portal hypertension. Int Surg. 1999;84(3):20912.
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Ulster Med J 2006; 75 (3) 175-177

Sinistral portal hypertension

Sinistral portal hypertension

Sinistral, or left-sided, portal hypertension is a rare cause of

The Ulster Medical Society, 2006.

or evidence of generalised portal hypertensive gastropathy.

Surgical Unit, Mater Hospital, Crumlin Road, Belfast,

The Ulster Medical Journal

CT scan demonstrating a solid lesion in the tail of

Greenwald and Wasch first outlined the pathophysiology of

The Ulster Medical Society, 2006.

gastroepiploic veins and subsequently via the coronary vein

Sinistral portal hypertension is an important cause

Greenwald HM, Wasch MG. The roentgenologic demonstration of

Ruzicka M, Konecna D, Jordankova E. Portal hypertension as a

Little AG, Moossa AR. Gastrointestinal haemorrhage from left-sided

Sinistral portal hypertension

Iwasaki T, Nagata Y, Watahiki H, Yamamoto H, Ogawa H. A rare case

Chang CY. Pancreatic adenocarcinoma presenting as sinistral portal

18. Angeras U, Almskog B, Lukes P, Lundstam S, Weiss L. Acute

Sahani D, Prasad SR, Maher M, Warshaw AL, Hahn PF, Saini S.

19. Melikoglu M, Colak T, Kavasoglu T. Two unusual cases of wandering

Caggiano AV, Adams DB, Metcalf JS, Anderson MC. Neuroendocrine

The Ulster Medical Society, 2006.

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