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Rex Shunts:
the role of IR
James Donaldson, MD
Lurie Children’s Hospital of Chicago
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IR and Rex Shunts
Portal Hypertension
The Rex Shunt
Pre-operative evaluation
IR
Results
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Portal Hypertension in Children
…with progessive hepatic dysfunction
Biliary diseases
Parenchymal diseases
Hepatic venous outflow obstruction
…without progressive hepatic dysfunction
Hepatic fibrosis
Klatzkin’s disease (angiodysplasia)
Splenic vascular anomaly
Portal vein obstruction
Portal vein anomalies
Cavernous transformation
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Extra-hepatic portal vein
obstruction Etiology usually unknown
History of UVC
Prematurity
PV thrombosis
Gastroenteritis
Dehydration
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Management portal hypertension
without hepatic dysfunction
Serial sclerotherapy
Partial splenic embolization
Splenectomy
Surgical management
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Surgical Managaement
Selective Shunt
Warren (Spleno-renal)
Rex (Meso-Rex)
Non-selective
Mesocaval
Segura (gastric venous
devascularization)
J. de Ville de Goyet, et al J Pediatr Surg 33
(1998), pp. 597-601
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Rex Shunt
Decompress portal hypertension
Restores mesenteric/portal blood flow to liver
Growth increase (BMI)
Improve hepatic function Ammonia
Neurocognitive improvement
Lautz T. J Pediatr Surg 2009
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SAM Question #1
The Rex Shunt is appropriate for which of the
following:
1. Abernethy malformation Type I
2. A complication of umbilical venous
catheterization
3. Veno-occlusive disease
4. Budd Chiari
5. Alagille syndrome
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SAM Question #1
The Rex Shunt is appropriate for which of the
following:
1. Abernethy malformation Type I
2. A complication of umbilical venous
catheterization
3. Veno-occlusive disease
4. Budd Chiari
5. Alagille syndrome
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SAM Question #2
The following are advantages of the Rex
shunt over a Warren shunt except:
1. Reduced bleeding from esophageal varices
2. Reduced incidence of hepatopulmonary
syndrome
3. Improved portal venous blood flow
4. Improved cognition
5. Improved BMI
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SAM Question #2
The following are advantages of the Rex
shunt over a Warren shunt except:
1. Reduced bleeding from esophageal varices
2. Reduced incidence of hepatopulmonary
syndrome
3. Improved portal venous blood flow
4. Improved cognition
5. Improved BMI
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Pre-operative Evaluation US
CTV
MRV
Direct PV puncture
Transjugular wedged portal
venography
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Transjugular
Wedged catheter
Portal Vein
TJ wedged hepatic vein portography
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Multiple wedged sites
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Rex Shunt Surgery
Right IJ vein graft
Dissection of the Rex recessus
Access of the left PV
Appropriate mesenteric vein access site
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Patent shunt
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5 yrs Post
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Pre and Post
+ 12 months
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Increased liver volume
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1 day post-op - Occluded shunt
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17% patients develop stenosis at PV end
Lautz T, Kim S, Donaldson J, Superina R. JVIR 2012
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Surveillance of Rex Shunts
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Surveillance of Rex Shunts
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Surveillance of Rex Shunts
MRV
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Surveillance of Rex Shunts
Clinical
Splenomegaly
Thrombocytopenia
GI bleeding
Imaging
US
CTV
MRV
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Rex Interventions
Transhepatic PV access
Right-sided
US-guided
22 gauge needle
PV
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Introduction of vascular sheath
Heparin
Probe PV to find Rex
Identify stenosis
Pressure gradient
> 5 mm Hg
Rex
Stenosis
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Pre and post angioplasty
6 mm balloon
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Evaluation of shunt
SMV end
PV end
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Recurrent stenosis post stent
Stent
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Kissing stents
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IR managaement
15 patients with stenosis
9 (60%) primary venoplasty
3 (20%) required multiple procedures
3 (20%) failed interventions – Rex abandoned
27 Interventional procedures
17 venoplasties
4 cutting balloons
5 stents
1 venoplasty with thrombolysis
Lautz T, Kim S, Donaldson J, Superina R. Outcomes of percutaneous
interventions of Meso-Rex Shunts. JVIR 2012
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Results
Pressure gradient reduced 11 to 5 mm Hg
Platelets increased 97K to 165K
Ammonia reduced 44 to 24 µmol/L
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Back table demo
Parenchymal tract
Slowly withdraw sheath
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1 major complication
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Rex Shunts Lurie Children’s Chicago
1997- 2012
143 patients attempted Rex shunts
104 Patent to date
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SAM Question #3
Indications for IR investigation of a Rex shunt
include all except:
1. Thrombocytopenia
2. A suspected stenosis by US, CT, or MR imaging
3. Splenomegaly
4. Recurrent UGI bleeding
5. Lower extremity edema
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SAM Question #3
Indications for IR investigation of a Rex shunt
include all except:
1. Thrombocytopenia
2. A suspected stenosis by US, CT, or MR imaging
3. Splenomegaly
4. Recurrent UGI bleeding
5. Lower extremity edema
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Summary
The Rex shunt is an ideal surgical option for extra-
hepatic PV obstruction
Pre and post operative imaging is challenging
but vitally important for the success of a Rex
shunt program
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Summary
IR plays a significant role in the Rex shunt
program
Percutaneous interventions are challenging but
can be done safely and effectively in this patient
population
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References
J. de Ville de Goyet, et al. Direct bypassing of extrahepatic portal vein obstruction in children: A new technique for combined hepatic revascularization and managing portal hypertension. J Pediatr Surg 33 (1998), pp. 597-601
Dasgupta R, Roberts E, Superina RA, Kim PC Effectiveness of Rex shunt in treatment of portal hypertension. J Pediatr Surg 41 (2006), pp. 108-112
Lautz T, Kim ST, Donaldson JS, Superina R. Outcomes of percutaneous interventions for managing stenosis of meso-Rex bypass in patients with extrahepatic portal vein obstruction. JVIR 2012; 23:377-383