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Dear colleagues,
We ask for your advice in the following clinical case:
21-year old female (165 cm /52kg) with invalidating complications of portal hypertension with extreme splenomegaly, due to an umbilical vein catheter in infancy. The past two years she developed a series of spontaneous splenic infarctions, and has invalidating problems:
– chronic abdominal pain in the splenic region (spleen size 32 cm) after a series of splenic infarctions; needs high dosages of opioids.
– fully dependent on enteral nutrition using a nasogastric tube, to prevent weight loss and nausea, fullness, and pain after eating, due to extreme splenomegaly and intestinal venous congestion
– grade 1 hepatic encephalopathy, WHO performance score 2.
– severe pancytopenia due to splenic poolingLiver transplantation (LTX) followed bij splenectomy seems the only effective and durable option to treat the portale hypertension, however she was not granted exception points on the national waiting list. We are now exploring living related donation with a donor vascular toolkit or grafting of veins for portal reconstruction.
Mesorex is not possible (portography was done) and TIPS is not possible; a splenorenal shunt already exists spontaneously, splenic embolization or splenectomy without LTX seems too risky and may hamper future LTX in case of complications.Our questions to the experts:
– Are there other/better options that LTX?
– Should splenectomy be performed simultaneously or after LTX?Kind regards,
Dr. Frederike GI van Vilsteren, gastroenterologist/hepatologist
University Medical Center Groningen, Netherlands. -
I may have missed it, but in addition to her pre-hepatic portal hypertension, what does the liver histology show or imaging ? Any ascites?
Her MELD score must be very low and you will not get exceptions.We have cases of extreme splenomegaly even after liver transplant. We once considered spleen radiation even Y90, but there are no good results.
May be you can submit this case with films to Professor Riad Salem here at Northwestern.
Daniel R. Ganger, MD
Professor of Medicine and Surgery
Northwestern University
ChicagoDaniel R. Ganger, MD
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Dear colleague,
Thank you for sharing with us this case.
1. Has a myeloproliferative neoplasm been firmly ruled out? With such a large spleen, it is important.
2. I would be reluctant to splenectomy or to any procedure like splenic artery embolization.
3. I agree with Dr Daniel R. Ganger than portal vein recalization should be considered.
4. Liver transplantation is indeed the option if portal vein recanalization is not feasible/fails and if there is a patent superior mesenteric vein.
Best regards
Pierre-Emmanuel RautouHôpital Beaujon, Clichy, France
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@ Dr Ganger: there is no severe fibrosis or cirrhosis upon elastography, and the liver function is normal. Howover the liver surface seems slighty irregular on MRI, and there are two hypervascular lesions on MRI, not suspicious for HCC. There is no ascites. She underwent several band ligations for esophageal variceal bleeding in her early years. Current MELD is 9 points. I would by happy to receive the contact info of Professor Riad Salem (f.g.i.van.vilsteren@umcg.nl).
@ Dr Rautou: 1) bone marrow study showed no abnormalities.
Thanks for your advice,
Kind regards,
Dr. Frederike GI van Vilsteren, gastroenterologist/hepatologist
UMC Groningen, Netherlands.
f.g.i.van.vilsteren@umcg.nl -
Dear Frederike,
Does liver biopsy show any form of porto-sinusoidal vascular disease? Nodular regenerative hyperplasia?
If the liver function is fine, there is basically no need for OLT, because TIPS will decrease portal pressure, however under the condition that the splanchnic veins are open or recanalization will be possible.
Have the advice of a competent interventional radiologist in this case.
Andrea De Gottardi
USI, Lugano, South Switzerland
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