AN  UNCOMMON  CAUSE  OF  PORTAL  

HYPERTENSION  Resident(s):  Bryan  I.  Hartley,  MD  

Attending(s):  Leann  S.  Stokes,  MD  

Program/Dept(s):  Vanderbilt  University  Medical  Center  

 

   

CHIEF  COMPLAINT  &  HPI  

   Chief  Complaint  

   “My  stomach  hurts.”  

   History  of  Present  Illness     A  55-­‐year-­‐old  man  presented  with  complaints  of  abdominal  swelling,  discomfort  and  associated  shortness  of  breath.  

RELEVANT  HISTORY  

   Past  Medical  History     Gastroesophageal  reflux     Denies  history  of  liver  disease,  liver  biopsy  or  trauma,  retrograde  or  transhepatic  cholangiography  or  hepatobiliary  operation  

   Past  Surgical  History     Splenectomy  

  Medications     Aspirin  81  mg  and  Esomeprazole  

   Allergies      NKDA  

 

DIAGNOSTIC  WORKUP  –  CT  ANGIOGRAM  

Figure  A:  There  was  marked  hypertrophy  of  the  celiac,  common  hepatic,  proper  hepatic  and  right  hepatic  arteries.  The  right  hepatic  artery  branch  directly  communicates  with  a  branch  of  the  right  portal  vein.  Note  atrophy  of  the  right  hepatic  lobe.  

Figure  B:  Reformatted  image  from  CT  angiogram  shows  opacification  of  the  portal  vein  (arrows)  on  arterial  phase  imaging.  

A   B  

DIAGNOSIS  

  Congenital  high  flow  arteriovenous  fistula  between  a  peripheral  branch  of  the  right  hepatic  artery  and  a  subcapsular  branch  of  the  right  portal  vein.  

QUESTION  

  True  or  false:  Most  congenital  arterioportal  fistulas  are  commonly  diagnosed  in  adulthood.    

   A.  True  B.  False  

CORRECT!  

  True  or  false:  Most  congenital  arterioportal  fistulas  are  commonly  diagnosed  in  adulthood.    

   A.  True  B.  False  

CONTINUE  WITH  CASE  

SORRY,  THAT’S  INCORRECT.  

  True  or  false:  Most  congenital  arterioportal  fistulas  are  commonly  diagnosed  in  adulthood.    

   A.  True  B.  False  

CONTINUE  WITH  CASE  

INTERVENTION    

A  5-­‐F  Cobra  II  catheter  (Angiodynamics,  Latham  NY)  was  used  to  select  the  hypertrophied  right  hepatic  artery.  

INTERVENTION    

•  The  Cobra  II  catheter  was  exchanged  over  a  wire  for  a  5-­‐F  vertebral  catheter  (Angiodynamics,  Latham,  NY).    

•  A  10  mm  x  14  cm  Nester  coil  (Cook  Medical,  Bloomington,  Indiana)  was  deployed  proximal  to  the  tapered  portion  of  the  distal  hepatic  arterial  branch.    

•  The  coil  (circle)  crossed  the  fistula  and  embolized  into  a  right  portal  vein  branch.  Subsequent  injections  demonstrated  no  disruption  of  flow  in  the  main  or  left  portal  systems.  

•   A  decision  was  made  to  proceed  with  Amplatzer  II  plug  (St.  Jude  Medical,  St.  Paul,  MN)  placement.  

•  The  vertebral  catheter  was  replaced  with  a  6-­‐F  MDC  guiding  catheter  (Boston  Scientific,  Natick,  MA).    

•  A  12  mm  Amplatzer  II  plug  (arrow)  was  deployed  in  the  right  hepatic  arterial  branch  through  the  guiding  catheter.  Final  injection  of  contrast  demonstrated  occlusion  of  the  AV  fistula.    

INTERVENTION    

•  48  hours  after  embolization  

•  Repeat  CT  angiogram  shows  occlusion  of  the  AV  fistula  

QUESTION  

  The  arrows  point  to  which  of  the  following  structures?  

   A.  Splenic  vein  B.  Superior  mesenteric  artery  C.  Celiac  artery  D.  Portal  vein  E.  Superior  mesenteric  vein  

CORRECT!  

  The  arrows  point  to  which  of  the  following  structures?  

   A.  Splenic  vein  B.  Superior  mesenteric  artery  C.  Celiac  artery  D.  Portal  vein  E.  Superior  mesenteric  vein  

CONTINUE  WITH  CASE  

SORRY,  THAT’S  INCORRECT.  

  The  arrows  point  to  which  of  the  following  structures?  

   A.  Splenic  vein  B.  Superior  mesenteric  artery  C.  Celiac  artery  D.  Portal  vein  E.  Superior  mesenteric  vein  

CONTINUE  WITH  CASE  

SUMMARY  &  TEACHING  POINTS  

•  Congenital  arterioportal  fistulas  are  rare  entities  and  uncommon  causes  of  portal  hypertension.  

•  Treatment  goals  include  relieving  the  sequelae  of  portal  hypertension.  

•  Endovascular  options  for  occlusion  include  stainless  steel  coils,  detachable  coils,  or  Amplatzer  occlusion  devices.  

•  Factors  to  consider:  diameter  of  feeding  vessel,  length  of  the  vessel  that  can  be  occluded  without  disruption  of  flow  to  normal  parenchymal  branches,  and  the  type  of  delivery  system  that  can  be  successfully  advanced  to  the  arteriovenous  communication.  

•  Cross  sectional  imaging  findings  that  support  the  diagnosis  of  a  high  flow  arterioportal  fistula  in  this  patient  include:  direct  communication  between  right  hepatic  artery  branch  and  right  portal  vein,  hypertrophy  of  the  celiac,  common  hepatic,  proper  hepatic  and  right  hepatic  arteries,  and  relative  atrophy  of  the  right  lobe  of  the  liver.  

•  The  benefits  to  using  an  Amplatzer  plug  for  occlusion  of  an  AV  fistula:  correct  size  can  be  determined  prior  to  deployment,  less  risk  of  distal  embolization,  decreased  time  and  radiation  exposure  required  for  complete  embolization  compared  with  coils.  

REFERENCES