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Partial Splenic Artery Embolization for Severe Hepatic Myelopathy in Cirrhosis
Cyriac Abby Philips1, Lijesh Kumar2, Philip Augustine 3
1 Hepatology and Liver Transplant Medicine, PVS Memorial Hospital Ltd, Cochin, Kerala
682018, India. Email:
2 Interventional and Diagnostic Radiology, PVS Memorial Hospital Ltd, Cochin, Kerala
682018, India. Email:
3 Gastroenterology, PVS Memorial Hospital Ltd, Cochin, Kerala 682018, India. Email:
Corresponding author
Cyriac Abby Philips MD DM
Philip Augustine Associates
35/194 B Symphony, Automobile Road
Palarivattom, Cochin 682025
Kerala, India
Phone: (91) 484 2535838, +91 9207745776
Sources of funding: None
Conflicts interests: None
Abbreviations: PHT – portal hypertension; HE – hepatic encephalopathy; UPDRS - Unified
Parkinson’s Disease Rating Scale; HIV – human immunodeficiency virus; LT – liver
transplantation; PSAE - partial splenic artery embolization;
This article has been accepted for publication and undergone full peer review but has not been
through the copyediting, typesetting, pagination and proofreading process which may lead to
differences between this version and the Version of Record. Please cite this article as
doi: 10.1002/hep.29597
This article is protected by copyright. All rights reserved.
Hepatic myelopathy is a devastating but rare complication of cirrhosis and portal
hypertension that profoundly affects quality of life and improves only with liver
transplantation. We present a case where progressive severe spastic paraparesis due to hepatic
myelopathy was substantially reversed with partial splenic artery emobilization.
Case presentation
A 48-year-old man with alcoholic cirrhosis, and portal hypertension (PHT) with recurrent
overt hepatic encephalopathy (HE) in the previous four years, without chronic toxin exposure
experienced progressive difficulty in walking for three years and was confined to bed for one
month. Clinical examination revealed asterixis, slurred speech, extreme psychomotor
slowing, hypokinesia, ataxia, severe hyperreflexia, spasticity, and rigidity of the lower limbs
without sensory deficit or sphincteric involvement. The patient was unable to walk
(Supplementary Video: pre-procedure), even with support. Blood tests revealed hemoglobin:
9.2 g/dl; total white blood cell count: 3,100 cells/mm3; platelet count: 68,000 cells/mm3; total
serum bilirubin: 4.2 mg/dl; direct bilirubin: 1.1 mg/dl; serum albumin: 2.8 g/dL; international
normalized ratio: 1.6; and ammonia: 98 mcg/dL. The work-up for viral hepatitis B and C,
retroviruses, cytomegalovirus, parvovirus, human-T-lymphotropic virus type-1 (HTLV-1),
and syphilis returned negative. Serum vitamin B-12 and folate levels were normal.
Abdominal imaging indicated cirrhosis, splenomegaly, multiple splenic hilar collaterals, and
dilated splenoportal confluence (Figure 1A-C). Magnetic resonance imaging of the brain
revealed bilateral hyperintensities in the internal capsule and thalamus, while imaging of the
whole spine was normal. Cerebrospinal fluid analysis and nerve conduction studies were
normal and the electroencephalogram was suggestive of hepatic encephalopathy. The patient
was diagnosed with severe spastic paraparesis secondary to hepatic myelopathy (HM). We
recommended liver transplantation (LT), but the family was unwilling due to long-term
financial constraints. Since PHT, portosystemic shunting, and hyperammonemia are central
to the development of HM, we postulated that portal pressure reduction through partial
splenic artery embolization (PSAE) could improve the patient’s condition. The patient
underwent PSAE uneventfully and post-procedure mild embolization syndrome was managed
conservatively. The slurring of speech improved from post-procedure day three. Postprocedure investigations are shown in Supplementary Table 1. Aggressive limb
physiotherapy was continued at home, while monthly video-based clinical assessments were
performed due to the distance of the patient. The patient showed a steady neurological
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improvement at two months post-procedure and could perform simple personal chores with
minimal support (Supplementary Video: post procedure). He continues to be rehabilitated.
HM is a rare and severe form of progressive spastic paraparesis seen in patients with cirrhosis
and most often with recurrent HE, regardless of the presence of large portosystemic shunts,
that does not respond to standard ammonia reduction therapies (1). Differential diagnoses
include amyotrophic lateral sclerosis, hereditary, Wernicke’s and toxic myelopathy, multiple
sclerosis, paraneoplastic syndromes, radiation myelopathy, infectious causes of myelopathy,
and vascular spinal cord disease, all of which have a distinct presentation, progression, and
outcomes. The occurrence of HM is thought to be due to nitrogenous toxins causing injury to
the axon cylinder, neuronal cell bodies, and myelin. (2) Treatment options include ammonia
lowering agents, benzodiazepine receptor antagonists, high dose B vitamins, gabapentin,
pentoxifylline, non-absorbable antibiotics, and portosystemic-shunt embolization, none of
which improves progressive gait disability. (3) In the early stages, LT is the only treatment
modality shown to improve neurological outcomes in this disabling disease (Figure 2). (4)
Splenic artery occlusion reduces portal pressures, improves hepatic function, and ameliorates
hypersplenism in patients with cirrhosis. Post-embolization syndrome occurs in
approximately 78% of cases. (5) We believe that PSAE improved liver function, decreased
PHT, lowered portosystemic shunting, and ameliorated neurological symptoms. In patients
with severe HM who are not eligible or waiting for LT, PSAE could be of benefit in
improving disability and hence quality of life.
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1. Yin YH, Ma ZJ, Guan YH, Ren YD, Zhang ZL. Clinical features of hepatic
myelopathy in patients with chronic liver disease. Postgrad Med J. 2009; 85:64-8.
2. Satyawali V, Singh Y, Khalil M, Kumar J. Hepatic myelopathy in a patient with
decompensated liver disease: An unusual complication. Int J Nutr Pharmacol Neurol
Dis 2013; 3:392-5
3. Feltracco P, Cagnin A, Carollo C, Barbieri S, Ori C. Neurological disorders in liver
transplant candidates: Pathophysiology and clinical assessment. Transplant Rev.
2017; pii: S0955-470X (16)30080-5
4. Baccarani U, Zola E, Adani GL, Cavalletti M, Schiff S, Cagnin A, et al. Reversal of
hepatic myelopathy after liver transplantation: fifteen plus one. Liver Transpl. 2010;
5. Yoshida H, Mamada Y, Taniai N, Yamamoto K, Kaneko M, Kawano Y et al. Longterm results of partial splenic artery embolization as supplemental treatment for
portal-systemic encephalopathy. Am J Gastroenterol. 2005; 100:43-7.
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Caption : Contrast enhanced abdominal computed tomography revealing A - cirrhotic liver (white arrows),
massive splenomegaly (dashed arrow) pushing and displacing the left kidney (black arrow); B – main splenic
artery at the hilum (dashed arrow) dividing into multiple intra-splenic superior and inferior branches (black
arrows); C – portal venography showing dilated portal vein (black arrow) and dilated splenic vein with hilar
collaterals (white arrow); D – fluoroscopy imaging of the celiac axis showing tortuous main splenic artery
(dashed arrow); E – Injection of polyvinyl alcohol foam particles (Contour TM PVA 300, Boston Scientific,
Marlborough, Massachusetts, United States) through Progreat microcatheter (Terumo, Shibuya, Tokyo,
Japan; black arrow) and F – post PVA embolization, splenic arteriogram in mid-arterial phase, demonstrating
attenuation of intra-splenic branches supplying the middle and inferior pole of the spleen (white arrows).
190x107mm (300 x 300 DPI)
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Pathophysiology of and therapeutic targets in hepatic myelopathy
162x91mm (300 x 300 DPI)
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