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Noncirrhotic Extrahepatic Portosystemic Shunt Causing Adult-Onset Encephalopathy Treated with Endovascular Closure.

Elnekave E, Belenky E, Van der Veer L - Case Rep Radiol (2015)

Bottom Line: Lactulose and rifaximin therapy failed to normalize serum ammonia levels.The diagnosis of congenital extrahepatic portosystemic shunt was made and endovascular shunt closure was performed using a 22 mm Amplatzer II vascular plug.Within a day, serum ammonia levels normalized.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology and Oncology, Rabin Medical Center, 49100 Petah Tikva, Israel.

ABSTRACT
A 54-year-old woman presented with a six-month history of episodic confusion and progressive ataxia. A comprehensive metabolic panel was notable for elevated values of alkaline phosphatase (161 U/L), total bilirubin (1.5 mg/dL), and serum ammonia of 300 umol/L (normal range 9-47). Hepatitis panel, relevant serological tests, tumor markers (CA-19-9, CEA), and urea cycle enzyme studies were unrevealing. Lactulose and rifaximin therapy failed to normalize serum ammonia levels. Imaging revealed a structural vascular abnormality communicating between an enlarged inferior mesenteric vein and the left renal vein, measuring 16 mm in greatest diameter. The diagnosis of congenital extrahepatic portosystemic shunt was made and endovascular shunt closure was performed using a 22 mm Amplatzer II vascular plug. Within a day, serum ammonia levels normalized. Lactulose and rifaximin were discontinued, and confusion and ataxia resolved.

No MeSH data available.


Related in: MedlinePlus

(a) Visceral phase angiography following injection of the splenic artery after occlusion of the portosystemic shunt with Amplatzer vascular plug (circled) demonstrates splenic vein (white arrows) draining antegrade into the portal vein (black arrows). (b) VR CT image shows the position of the Amplatzer plug (arrow) and successful embolization of the shunt (no longer visualized). Portal venous flow is now seen to and within the liver.
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Related In: Results  -  Collection


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fig2: (a) Visceral phase angiography following injection of the splenic artery after occlusion of the portosystemic shunt with Amplatzer vascular plug (circled) demonstrates splenic vein (white arrows) draining antegrade into the portal vein (black arrows). (b) VR CT image shows the position of the Amplatzer plug (arrow) and successful embolization of the shunt (no longer visualized). Portal venous flow is now seen to and within the liver.

Mentions: Following embolization, antegrade hepatopetal flow was documented in the splenic and inferior mesenteric veins (Figure 2). In anticipation of thrombogenic slow antegrade flow within the hypertrophied inferior mesenteric vein (which had served as a conduit for retrograde splenic outflow), prophylactic anticoagulation was initiated using low molecular weight heparin overlapping with warfarin continued as outpatient therapy (target INR 2.0–3.0). Follow-up imaging demonstrated partial thrombus in the hypertrophied inferior mesenteric vein without extension into the portal vein. There was no evidence of submucosal colonic edema or ascites.


Noncirrhotic Extrahepatic Portosystemic Shunt Causing Adult-Onset Encephalopathy Treated with Endovascular Closure.

Elnekave E, Belenky E, Van der Veer L - Case Rep Radiol (2015)

(a) Visceral phase angiography following injection of the splenic artery after occlusion of the portosystemic shunt with Amplatzer vascular plug (circled) demonstrates splenic vein (white arrows) draining antegrade into the portal vein (black arrows). (b) VR CT image shows the position of the Amplatzer plug (arrow) and successful embolization of the shunt (no longer visualized). Portal venous flow is now seen to and within the liver.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4496491&req=5

fig2: (a) Visceral phase angiography following injection of the splenic artery after occlusion of the portosystemic shunt with Amplatzer vascular plug (circled) demonstrates splenic vein (white arrows) draining antegrade into the portal vein (black arrows). (b) VR CT image shows the position of the Amplatzer plug (arrow) and successful embolization of the shunt (no longer visualized). Portal venous flow is now seen to and within the liver.
Mentions: Following embolization, antegrade hepatopetal flow was documented in the splenic and inferior mesenteric veins (Figure 2). In anticipation of thrombogenic slow antegrade flow within the hypertrophied inferior mesenteric vein (which had served as a conduit for retrograde splenic outflow), prophylactic anticoagulation was initiated using low molecular weight heparin overlapping with warfarin continued as outpatient therapy (target INR 2.0–3.0). Follow-up imaging demonstrated partial thrombus in the hypertrophied inferior mesenteric vein without extension into the portal vein. There was no evidence of submucosal colonic edema or ascites.

Bottom Line: Lactulose and rifaximin therapy failed to normalize serum ammonia levels.The diagnosis of congenital extrahepatic portosystemic shunt was made and endovascular shunt closure was performed using a 22 mm Amplatzer II vascular plug.Within a day, serum ammonia levels normalized.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology and Oncology, Rabin Medical Center, 49100 Petah Tikva, Israel.

ABSTRACT
A 54-year-old woman presented with a six-month history of episodic confusion and progressive ataxia. A comprehensive metabolic panel was notable for elevated values of alkaline phosphatase (161 U/L), total bilirubin (1.5 mg/dL), and serum ammonia of 300 umol/L (normal range 9-47). Hepatitis panel, relevant serological tests, tumor markers (CA-19-9, CEA), and urea cycle enzyme studies were unrevealing. Lactulose and rifaximin therapy failed to normalize serum ammonia levels. Imaging revealed a structural vascular abnormality communicating between an enlarged inferior mesenteric vein and the left renal vein, measuring 16 mm in greatest diameter. The diagnosis of congenital extrahepatic portosystemic shunt was made and endovascular shunt closure was performed using a 22 mm Amplatzer II vascular plug. Within a day, serum ammonia levels normalized. Lactulose and rifaximin were discontinued, and confusion and ataxia resolved.

No MeSH data available.


Related in: MedlinePlus