an unusual cause of hepatic encephalopathy resident(s): rajesh bhavsar, md program/dept(s):...
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AN UNUSUAL CAUSE OF HEPATIC ENCEPHALOPATHY
Resident(s): Rajesh Bhavsar, MD
Program/Dept(s): Montefiore Medical Center, Albert Einstein College of Medicine
Originally Posted: June 01, 2014
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CHIEF COMPLAINT & HPI
Chief Complaint • 71 year-old female presents with persistent lethargy and failure to
thrive.
History of Present Illness• 71 year-old female with history of mitral insufficiency had mitral
valve repair about one month prior to presentation. She was transferred from rehabilitation facility to hospital with persistent lethargy and failure to thrive after the surgery.
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RELEVANT HISTORY
Past Medical History multiple episodes of mitral valve endocarditis resulting in mitral
valvular insufficiency bicuspid aortic valve with aortic valvular stenosis multiple infections and pneumonias of unclear etiology incidentally found “liver abnormality” which was deemed
asymptomatic on a CT from a few years ago. no reported prior episodes of encephalopathy
Past Surgical History aortic valve repair mitral valve repair one month before presentation
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DIAGNOSTIC WORKUP
Physical Exam Asterixis
Laboratory Data Elevated ammonia level of 162 umol/L
Non-Invasive Imaging CT
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DIAGNOSTIC WORKUP- QUESTION
The portal vein is marked by the letter:A. aB. bC. cD. No portal vein is pres
ent.
a
b
c
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CORRECT!
The portal vein is marked by the letter:A. a – this is an
anomalous vesselB. b – this is the
portal vein, which is severely atretic
C. c – this is the IVCD. No portal vein is
present. – a portal vein is present.
a
b
c
CONTINUE WITH CASE
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SORRY, THAT’S INCORRECT.
The portal vein is marked by the letter:A. a – this is an
anomalous vesselB. b – this is the
portal vein, which is severely atretic
C. c – this is the IVCD. No portal vein is
present. – a portal vein is present.
a
b
c
CONTINUE WITH CASE
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DIAGNOSTIC WORKUP- QUESTION
In this patient, the superior mesenteric vein drains into:A. A normal caliber portal veinB. The inferior vena cavaC. The left renal veinD. A splenorenal shunt
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CORRECT!
In this patient, the superior mesenteric vein drains into:A. A normal caliber portal veinB. The inferior vena cavaC. The left renal veinD. A splenorenal shunt
SMV
SMV
CONTINUE WITH CASE
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SORRY, THAT’S INCORRECT.
In this patient, the superior mesenteric vein drains into:A. A normal caliber portal veinB. The inferior vena cavaC. The left renal veinD. A splenorenal shunt
SMV
SMV
CONTINUE WITH CASE
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DIAGNOSIS
Hepatic encephalopathy in a patient with congenital portosystemic shunt (Abernathy Malformation) onset by mitral valve annuloplasty. The patient had a congenital portosystemic shunt which was
compensated before heart surgery and essentially asymptomatic. The right heart failure served as a point of resistance for the portosystemic shunt forcing more blood into the diminutive portal vein. Following mitral valve repair, the right heart pressures decreased, allowing more blood flow through the shunt resulting in increased portosystemic shunting and symptoms of encephalopathy.
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INTERVENTION
Image A: Venography through the SMV demonstrates preferential flow through the shunt with little antegrade flow through a diminutive portal vein (PV) (arrow). Intravascular US showed the smallest caliber of the shunt vein to be 14mm in diameter
Image B: It was felt that if the shunt was occluded abruptly, the small PV would not be adequate to take all of the splanchnic flow and reducing flow in the shunt would be more prudent. A 16 x 60mm Wallstent which was constricted with a suture (arrow) was deployed within the shunt to make a landing zone for a PTFE covered device that will restrict flow.
A B
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INTERVENTION
Images A and B: a 10mm x 38mm PTFE covered balloon-mounted stent (arrows) was positioned at the narrowed portion of the Wallstent and deployed in an hourglass shape using strategic balloon inflations.
A second balloon (*) was inflated to prevent stent migration during deployment (Image B).
A B
*
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INTERVENTION
Yellow arrow indicates the inner 10 x 38mm PTFE covered balloon-mounted stent.
White arrow indicates the outer 16 x 60mm Wallstent.
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INTERVENTION
Before (image A) and after (image B) venograms. Blood flow through the splenorenal shunt is reduced post-intervention.
A B
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CLINICAL FOLLOW UP
Following the reduction of flow in the shunt, the patient’s ammonia levels decreased to 43 umol/L (from 162 umol/L), and the patient became alert with resolution of symptoms.
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SUMMARY & TEACHING POINTS
Abernathy malformation is a rare congenital anomaly of the splanchnic vasculature secondary to defects in vitelline vein formation. It is common in dogs (Yorkshire terriers) but extremely uncommon in humans.
Two types are described: Type I (females) : absent or atretic portal vein with diversion of portal
blood into systemic circulation; end to side shunts. Often associated with other abnormalities such as VSD and aortic arch defects. type Ia : separate drainage of the superior mesenteric and splenic veins into
systemic veins. type Ib : superior mesenteric and splenic veins join to form a short extra-
hepatic portal vein which drains into a systemic vein.
Type II (males): hypoplastic portal vein with portal blood diversion into the vena cava through a side-to-side, extrahepatic communication.
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REFERENCES & FURTHER READING
1. Gallego C, Velasco M, Marcuello P, Tejedor D, De Campo L, Friera A. Congenital and acquired anomalies of the portal venous system. Radiographics. 2002;22(1):141–59. doi:10.1148/radiographics.22.1.g02ja08141.
2. Lee W-K, Chang SD, Duddalwar V a, et al. Imaging assessment of congenital and acquired abnormalities of the portal venous system. Radiographics. 2011;31(4):905–26. doi:10.1148/rg.314105104.