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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 before occlusion of the portosystemic shunt shows retrograde flow from the splenic vein (white arrows) via an enlarged inferior mesenteric vein (black arrows) via shunt (three dashed arrows) into the systemic venous system. (b) Venous phase volume-rendered (VR) image demonstrates a serpentine vascular shunt (three small arrows) connecting an enlarged IMV (large arrow heads) to the left renal vein via a tortuous shunt (three small arrows). The splenic vein is marked with three two large arrows. Normal appearance of the SMV (dashed arrows). Streak artifact in the portal confluence is due to clips from prior Whipple procedure.
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fig1: (a) Visceral phase angiography following injection of the splenic artery before occlusion of the portosystemic shunt shows retrograde flow from the splenic vein (white arrows) via an enlarged inferior mesenteric vein (black arrows) via shunt (three dashed arrows) into the systemic venous system. (b) Venous phase volume-rendered (VR) image demonstrates a serpentine vascular shunt (three small arrows) connecting an enlarged IMV (large arrow heads) to the left renal vein via a tortuous shunt (three small arrows). The splenic vein is marked with three two large arrows. Normal appearance of the SMV (dashed arrows). Streak artifact in the portal confluence is due to clips from prior Whipple procedure.

Mentions: A computed tomography (CT) scan of the abdomen demonstrated a smooth hepatic contour and was without splenomegaly, perigastric varices, splenorenal varices, or ascites or other stigmata of portal hypertension. The extrahepatic portal vein was normal in caliber and contrast opacification. A serpentine vascular structure in the right lower abdominal quadrant communicated between a markedly enlarged inferior mesenteric vein (IMV) and the left renal vein (Figure 1). The vascular structure could be identified retrospectively on CT scans dating at least ten years earlier. The shunt progressively increased in size from a maximal diameter of 7 mm in 2003 to 16 mm in 2013. Doppler evaluation demonstrated low flow in the portal vein with a Time Averaged Mean Velocity (TAMV) of 10 cm/s (normal 15–18 cm/s). Transjugular liver biopsy showed fatty change and mild chronic portal triad inflammation and normal appearance of the portal vein within the triad. There was no pathologic evidence of cirrhosis and the corrected mean portosystemic venous gradient measured 3 mmHg.


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 before occlusion of the portosystemic shunt shows retrograde flow from the splenic vein (white arrows) via an enlarged inferior mesenteric vein (black arrows) via shunt (three dashed arrows) into the systemic venous system. (b) Venous phase volume-rendered (VR) image demonstrates a serpentine vascular shunt (three small arrows) connecting an enlarged IMV (large arrow heads) to the left renal vein via a tortuous shunt (three small arrows). The splenic vein is marked with three two large arrows. Normal appearance of the SMV (dashed arrows). Streak artifact in the portal confluence is due to clips from prior Whipple procedure.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig1: (a) Visceral phase angiography following injection of the splenic artery before occlusion of the portosystemic shunt shows retrograde flow from the splenic vein (white arrows) via an enlarged inferior mesenteric vein (black arrows) via shunt (three dashed arrows) into the systemic venous system. (b) Venous phase volume-rendered (VR) image demonstrates a serpentine vascular shunt (three small arrows) connecting an enlarged IMV (large arrow heads) to the left renal vein via a tortuous shunt (three small arrows). The splenic vein is marked with three two large arrows. Normal appearance of the SMV (dashed arrows). Streak artifact in the portal confluence is due to clips from prior Whipple procedure.
Mentions: A computed tomography (CT) scan of the abdomen demonstrated a smooth hepatic contour and was without splenomegaly, perigastric varices, splenorenal varices, or ascites or other stigmata of portal hypertension. The extrahepatic portal vein was normal in caliber and contrast opacification. A serpentine vascular structure in the right lower abdominal quadrant communicated between a markedly enlarged inferior mesenteric vein (IMV) and the left renal vein (Figure 1). The vascular structure could be identified retrospectively on CT scans dating at least ten years earlier. The shunt progressively increased in size from a maximal diameter of 7 mm in 2003 to 16 mm in 2013. Doppler evaluation demonstrated low flow in the portal vein with a Time Averaged Mean Velocity (TAMV) of 10 cm/s (normal 15–18 cm/s). Transjugular liver biopsy showed fatty change and mild chronic portal triad inflammation and normal appearance of the portal vein within the triad. There was no pathologic evidence of cirrhosis and the corrected mean portosystemic venous gradient measured 3 mmHg.

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