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Dear friends,
We would like to know your opinion in the case of a patient with diffuse colonic varices. We wonder if some of you have seen a patient like that and what is your potential explanation for varix formation in this patient and options of treatment.
34 year old woman, smoker, with no other past medical history, that first presented in 2004 with lower gastrointestinal bleeding. She underwent a colonoscopy that showed big varices through the entire colon although they seemed to be bigger in the rectum, transverse and right colon. The study was completed with an angioCT and an arteriography that showed collateral circulation in the intestinal wall of distal ileon, cecum, right and transverse colon. The gastroepiploic and pancreaticoduodenal artery seem to be congested while the ileocolic vein appears to be slightly diminished with no clear change in the vessel diameter.
Intrahepatic Portal hypertension has been ruled out with a hemodynamic study that obtained a HVPG of 1mmHg, transient elastography 3,2kPa, normal liver appearance at imaging studies, no gastroesophageal varices, normal liver tests and negative thrombophilia study.
Splanchnic venous thrombosis was not identified (at several angioCTs, and arteriography) but we still think that maybe some unidentified proximal and extense thrombosis was present causing portal hypertension at the level of gut and that’s why propranolol was initiated. However, despite Betablockers, during the lasts months she has been admitted several times because of lower gastrointestinal bleeding and the hemoglobin has dropped to 7-8g/L.
The patient, until now, has been always hemodynamically stable and tolerates very well the Hb level and has not been transfused. Several colonoscopies have confirmed the presence of severe varices all over the colon, rectum and ileocecal area. In the ileocecal area the varices look more prominent although we have never been able to identify the varix that was bleeding.Thanks a lot
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dear Juan Carlos,
my first impression is the same that you have had, namely that a local outflow tract thrombosis, although recanalized, may have caused a regional pre-hepatic portal hypertension.
However, I wonder whether ultrasonography with Doppler can detect some of the colonic varices or collaterals to test whether the flow is flat and venous rather than phasic / arterialized. The latter instance may suggest an arteriovenous fistula, causing local portal hypertension.
Would it be possible to consider arteriography to evaluate separately the vascular beds deriving for the inferior mesenteric artery and superior mesenteric artery, with venous phases too, to confirm whether signs of long standing venous thrombosis or rather arterio-venous fistula exist?Fabio
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Dear Dominique, Federica and Fabio,
thanks for your interest in our patient. We indeed have already done a direct (superior and inferior mesenteric arteriography. No data on arteriovenous fistula was seen).We are considering to do a direct portal punction and measure pressure to discard any prehepatic component of portal hypertension that we have been unable to identify by imaging. I will keep pou all informed aout the evolution. If there are other advices…
Thanks VALDIG consortia
Best and seen you soon in Ascona -
Dear Juan Carlos,
Thank you for sharing this interesting case with us. I cannot remember a similar case in Vienna… Does splenomegaly and/or thrombocytopenia suggest (prehepatic/presinusoidal) portal hypertension? EUS-guided portal vein pressure measurement might be an option to directly measure portal pressure. Any family history of GI-bleeding in this patients’ family? Are there other (muco)cutaneous signs of vascular malformations in this patient?
In terms of management and treatment i do not have a good idea at the moment.All the best and i wish you and all the VALDIG team a great meeting in Ascona, which i am unfortunately not able to attend!
Best wishes,
Thomas -
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Dear Juan Carlos
We have used spleen pulp pressure measurement as a mean to determine the non-cirrotoc portal hypertension, which must be found in your patient.
Did you perform a liver biopsy and did it reveal any changes. Despite the normal elstography there may be changes in the liver suggesting pre-sinusoidal portal hypertension. Has sarcoidosis been ruled out?
Are liver enzymes normal?
For treatment long-acting soamtostatin analogue may help – though evidence is scarce?
Is the patient European? No schistosomiasis?Looking forward to have the diagnosis
best wishes
Henning Grønbæk -
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