gilani_iliocaval reconstruction in a patient with hereditary hemorrhagic telangiectasia_090115

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ILIOCAVAL RECONSTRUCTION IN A PATIENT WITH HEREDITARY HEMORRHAGIC TELANGIECTASIA Fellow: Saba Gilani, MD Attendings: Ian J. Wilson, MD; David L. Waldman MD, FSIR Program/Dept: Vascular and Interventional Radiology, University of Rochester

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Page 1: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

ILIOCAVAL  RECONSTRUCTION   IN  A  PATIENT  WITH  HEREDITARY  HEMORRHAGIC  

TELANGIECTASIAFellow:  Saba  Gilani,  MD  

Attendings:  Ian  J.  Wilson,  MD;  David  L.  Waldman  MD,  FSIR  

Program/Dept:  Vascular  and  Interventional  Radiology,  University  of  Rochester

Page 2: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

CHIEF  COMPLAINT  &  HPI

▪Chief  Complaint  and/or  reason  for  consultation  ▪57  year  old  female  with  history  of  hereditary  hemorrhagic  telangiectasia  (HHT)  with  recurrent  epistaxis,  prior  history  of  stroke,  and  known  pulmonary  AVMs  admitted  with  headache,  worsening  confusion,  back  pain,  and  bilateral  leg  swelling.  ▪VIR  was  consulted  for  possible  venous  thrombolysis.

Page 3: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

RELEVANT  HISTORY

▪ Past  Medical  History  ▪ HHT  

▪ manifesting  with  recurrent  epistaxis  ▪ multiple  pulmonary  AVMs  

▪ Patent  foramen  ovale  ▪ Pulmonary  Embolism  

▪ prior  IVC  filter  placement  ▪ CVA  

▪ HTN,  DM,  hypercholesterolemia,  hypothyroidism  

▪ Past  Surgical  History  ▪ Cauterization  for  epistaxis

▪ Family  &  Social  History  ▪ Both  parents  and  2  sisters  with  HTN.  ▪ 2  deceased  brothers  “sudden  death  from  brain  bleed.”  

▪ Former  smoker  (quit  2006)  

▪ Review  of  Systems  ▪ positive  for  headache,  back  pain,  leg  swelling  

▪ negative  for  chest  pain,  SOB  

▪ Medications  ▪ Levothyroxine,  lisinopril,  simvastatin  

▪ Allergies  ▪ NSAIDs  -­‐  contraindicated

Page 4: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

DIAGNOSTIC  WORKUP

▪ Physical  Exam  ▪ Positive  for  skin  telangiectasias,  severe  bilateral  lower  extremity  leg  swelling  with  edema,  patchy  areas  of  reddish  discoloration,  and  blistering.  

▪ Laboratory  Data  ▪ INR  1.2,  PTT  43.3,  Platelets  88  ▪ CK  27498  

▪ Non-­‐Invasive  Imaging  

▪ CT  head:  acute  on  chronic  right  frontoparietal  infarct.  ▪ CTA  chest:  showed  segmental  pulmonary  embolus  and  multiple  bilateral  AVMs  (largest  measuring  1.3  cm).  

▪ Doppler  US  of  the  lower  extremities:  thrombus  from  the  calf  veins  to  the  common  femoral  veins  bilaterally.

Page 5: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

DIAGNOSIS

▪Extensive  deep  venous  thrombus  with  developing  post  thrombotic  syndrome  and  phlegmasia  cerulea  dolens.

Page 6: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

CLINICAL  QUESTION

1)  What  is  post  thrombotic  syndrome  (PTS)?  

A:  CVA  following  DVT.  

B:  Chronic  pain  and  swelling  following  DVT.  

C:  Venous  sinus  thrombosis  following  DVT.  

D:  None  of  the  above.

Page 7: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

CORRECT!

1)  What  is  post  thrombotic  syndrome  (PTS)?  

A:  CVA  following  DVT.  

B:  Chronic  pain  and  swelling  following  DVT.  PTS  is  a  constellation  of  debilitating  lower  extremity  symptoms  following  DVT  including  leg  swelling,  pain,  edema,  discoloration.  

C:  Venous  sinus  thrombosis  following  DVT.  

D:  None  of  the  above.

CONTINUE  WITH  CASE

Page 8: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

SORRY,  THAT’S  INCORRECT!

1)  What  is  post  thrombotic  syndrome  (PTS)?  

A:  CVA  following  DVT.  

B:  Chronic  pain  and  swelling  following  DVT.  PTS  is  a  constellation  of  debilitating  lower  extremity  symptoms  following  DVT  including  leg  swelling,  pain,  edema,  discoloration.  

C:  Venous  sinus  thrombosis  following  DVT.  

D:  None  of  the  above.

CONTINUE  WITH  CASE

Page 9: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

TREATMENT  QUESTION

2)  What  are  the  treatment  options  for  symptomatic  iliocaval  DVT?  

A:  Mechanical  thrombectomy.  

B:  Pharmacologic  thrombolysis.  

C:  Pharmacomechanical  thrombolysis.  

D:  All  of  the  above.

Page 10: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

CORRECT!

2)  What  are  the  treatment  options  for  symptomatic  iliocaval  DVT?  

A:  Mechanical  thrombectomy.  Yes,  can  use  devices  such  as  Angiojet  or  Trellis.  

B:  Pharmacologic  thrombolysis.  Yes,  can  use  catheter  directed  tPA  infusion  therapy.  

C:  Pharmacomechanical  thrombolysis.  Yes,  can  use  Angiojet  with  tPA.  

D:  All  of  the  above.

CONTINUE  WITH  CASE

Page 11: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

SORRY,  THAT’S  INCORRECT!

2)  What  are  the  treatment  options  for  symptomatic  iliocaval  DVT?  

A:  Mechanical  thrombectomy.  Yes,  can  use  devices  such  as  Angiojet  or  Trellis.  

B:  Pharmacologic  thrombolysis.  Yes,  can  use  catheter  directed  tPA  infusion  therapy.  

C:  Pharmacomechanical  thrombolysis.  Yes,  can  use  Angiojet  with  tPA.  

D:  All  of  the  above.

CONTINUE  WITH  CASE

Page 12: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

INTERVENTION

▪Venous  thrombolysis  contraindicated  due  to  CVA,  pulmonary  AVM/PFO.  

▪Mechanical  thrombectomy  offered  to  help  diminish  clot  burden.

Page 13: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

INTERVENTION:  DAY  1

▪Venogram  demonstrating  multiple  filling  defects  in  the  bilateral  common  iliac  veins  and  near  complete  occlusion  of  the  distal  IVC.  An  IVC  filter  is  noted  (circle).

Page 14: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

INTERVENTION:  DAY  1  (CONT.)

▪ Percutaneous  transluminal  venous  angioplasty  of  the  common  iliac  veins  and  distal  IVC  was  performed.  This  was  followed  by  mechanical  thrombectomy  using  Angiojet.

•  Post  venous  angioplasty  and  thrombectomy  venogram  demonstrated  minimally  increased  patency  of  the  IVC  and  bilateral  common  iliac  veins.    •  Access  into  the  common  femoral  veins  could  not  be  obtained  bilaterally.  •  Dialysis  catheter  was  placed.  •  Treatment  was  limited  due  to  contraindication  for  catheter  directed  thrombolysis  overnight.

Page 15: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

INTERVENTION:  DAY  2

•  Patient  returned  to  IR  for  repeat  intervention,  this  time  with  access  distal  to  the  bilateral  common  femoral  veins  was  achieved  and  extensive  thrombus,  left  greater  than  right,  was  seen.  

•  This  was  treated  with  percutaneous  angioplasty  and  mechanical  thrombectomy.

Post-­‐Intervention  AngiogramsPre-­‐Intervention  Angiograms

Page 16: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

INTERVENTION:  DAY  3

• A:  Cavagram  demonstrating  increased  patency  of  the  IVC.  

• B:  A  suprarenal  IVC  filter  was  placed  (red  circle).  

• C:  The  old  IVC  filter  was  removed  using  wire-­‐loop-­‐snare  technique  (arrow).

A

BC

Page 17: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

INTERVENTION:  DAY  3  (CONT.)

• Repeat  percutaneous  angioplasty  and  mechanical  thrombectomy  of  the  distal  IVC,  bilateral  common  iliac,  common  femoral,  and  superficial  femoral  veins  was  performed.  

•  This  was  supplemented  by  mechanical  thrombolysis  from  the  common  iliac  vein  to  the  popliteal  vein  bilaterally  using  Angiojet  with  tPA  in  pulse  spray  mode  during  which  the  patient  developed  epistaxsis.

Page 18: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

INTERVENTION:  DAY  3  (CONT.)

• A:  After  thrombolysis,  iliocaval  reconstruction  was  performed  using  10  mm  self  expanding  stents.  

•  B:  Post  stent  deployment  venogram  demonstrated  significantly  increased  patency  in  the  IVC  and  bilateral  common  iliac  veins.

A B

Page 19: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

QUESTION  SLIDE

3)  What  type  of  filter  did  this  patient  have?  

A:  Bird’s  Nest.  

B:  Greenfield.  

C:  TrapEase.  

D:  G2.

Page 20: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

CORRECT!

3)  What  type  of  filter  did  this  patient  have?  

A.  Bird’s  Nest.  

B:  Greenfield.  

C:  TrapEase.  Permanent  filter;  high  rate  of  thrombosis  (25.2  %,  Kalva  SP,  et  al.  2006)  

D:  G2.

CONTINUE  WITH  CASE

Page 21: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

SORRY,  THAT’S  INCORRECT!

3)  What  type  of  filter  did  this  patient  have?  

A.  Bird’s  Nest.  

B:  Greenfield.  

C:  TrapEase.  Permanent  filter;  high  rate  of  thrombosis  (25.2  %,  Kalva  SP,  et  al.  2006)  

D:  G2.

CONTINUE  WITH  CASE

Page 22: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

QUESTION  SLIDE

4)  What  is  the  most  common  complication  of  IVC  filters?  

A.  Occlusion.  

B:  Migration.  

C:  Fracture.  

D:  IVC  penetration.

Page 23: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

CORRECT!

4)  What  is  the  most  common  complication  of  IVC  filters?  

A:  Occlusion.  Long  term  studies  estimate  13-­‐30%  rates  of  IVC  thrombosis  after  filter  placement  attributable  to  three  factors:  1.  thrombogenicity  of  the  filter,  2.  trapped  embolus  from  a  distal  source,  3.  cephalad  extension  of  distal  DVT.  

B:  Migration.  

C:  Fracture.    

D:  IVC  penetration.

CONTINUE  WITH  CASE

Page 24: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

SORRY,  THAT’S  INCORRECT!

4)  What  is  the  most  common  complication  of  IVC  filters?  

A:  Occlusion.  Long  term  studies  estimate  13-­‐30%  rates  of  IVC  thrombosis  after  filter  placement  attributable  to  three  factors:  1.  thrombogenicity  of  the  filter,  2.  trapped  embolus  from  a  distal  source,  3.  cephalad  extension  of  distal  DVT.  

B:  Migration.  

C:  Fracture.    

D:  IVC  penetration.

CONTINUE  WITH  CASE

Page 25: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

CLINICAL  FOLLOW  UP

▪Patient  returned  to  the  MICU,  was  extubated,  and  had  decreased  lower  extremity  swelling  over  time.  

▪She  developed  acute  renal  failure  requiring  dialysis.  ▪Bivalirudin  (specific  and  reversible  direct  thrombin  inhibitor)  was  used  for  anticoagulation.  

▪She  began  receiving  inpatient  physical  therapy  and  was  able  to  ambulate  8  days  post  IR  procedure.  

▪22  days  post  procedure  lower  extremity  doppler  US  demonstrated  resolved  lower  extremity  thrombus.

Page 26: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

SUMMARY  &  TEACHING  POINTS

▪A  57  year  old  female  with  HHT  developed  extensive  iliocaval  thrombus  with  manifestations  of  post  thrombotic  syndrome.  Her  management  was  complicated  by  the  presence  of  a  thrombogenic  IVC  filter  and  the  inability  to  anticoagulate  due  to  acute  CVA,  recurrent  epistaxsis,  and  multiple  pulmonary  AVMs.  

▪Mechanical  thrombectomy  without  thrombolytics  may  have  limited  value  when  there  is  a  large  clot  burden.  

▪ Iliocaval  reconstruction  can  result  in  successful  venous  recanalization  and  may  prevent  the  progression  of  post  thrombotic  syndrome.

Page 27: Gilani_Iliocaval Reconstruction in a Patient With Hereditary Hemorrhagic Telangiectasia_090115

REFERENCES  &  FURTHER  READING

Kalva  SP,  et  al.  TrapEase  vena  cava  filter:  experience  in  751  patients.  J  Endovasc  Ther.  2006  Jun;13(3):365-­‐72.    

Nayak  L,  et  al.  Postthrombotic  Syndrome:  Feasibility  of  a  Strategy  of  Imaging-­‐guided  Endovascular  Intervention.  JVIR.  23(9):1165  –  1173.  

Patel  RT,  et  al.  Endovascular  treatment  of  iliocaval  DVT.  Endovascular  Today.  2013  Sept:  36-­‐43.  

Williams  DM.  Iliocaval  reconstruction  in  chronic  deep  vein  thrombosis.  Tech  Vasc  Interv  Radiol.  2014  Jun;17(2):109-­‐13.