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Arterio-Portal Fistula Syndrome
Case report & review of the literature
Samir Haffar M.D.
Assistant Professor of Gastroenterology
Clinical History
• 60 year-old female – 7 children
• 2months ago:
Alimentary & biliary vomiting
Diarrhea 5 – 6 / day with tenesmus
Abdominal pain especially epigastric
Relieved by defecation
Clinical examination
• BP 105/70 mmHg
• Pulse 90/min
• Temperature 37.5 C
• Generalized abdominal tenderness
• Hepatomegaly (3 fingers width)
• Ascites
• Continuous murmur in the left fifth inter-costal space
Laboratory studies
• Hemoglobin: 10.7
• Total bilirubin 1.5 (direct 0.7)
• Alkaline Phosphatase x 4 ULN
• ALAT 43 (N 40)
• ASAT 25 (N 38)
• PT 60 %
• Albumin 3.1
• HBs Ag +
• HBe Ag –
• Anti HCV –
Ascitic fluid examination
• Total protein 1.3
• Albumin 0.5
• WBC 1 130 Lymphocytes 90%
Neutrophils 7 %
• RBC 760
• Abnormal cells Negative
• KB Negative
Serum Ascites Albumin Gradient
SAAG
3.1 – 0.5 = 2.6
≥ 1.1 → Portal hypertension
Endoscopic studies
• UGI endoscopy Esophageal varices 1st degree
• Colonoscopy Recto-sigmoidal erythema
Ultrasound exam
• Ascites in moderate amount
• Splenomegaly 150 mm
• Large vessel in splenic hilum
• Dilated splenic vein 28 mm
• Dilated portal vein 21 mm
• Dilated intra-hepatic portal tract
• GB sludge
• Heterogeneous hepatic echostructure
Splenic hilum
Doppler Sonogram
• Venous circulation in vessel of splenic hilum
• Dilated splenic artery 14 mm
• Low RI in SA in hilum 0.42
• RI in intra-splenic arteries 0.61
• Arterialisation of flow in splenic vein
• Mean velocity in portal vein 12 cm/sec
• Normal hepatic veins
Diagnosis: Splenic arterio-venous fistula
Splenic artery from the celiac trunk
Splenic artery in the hilum
Splenic artery in the hilum
Intra-splenic artery
Splenic artery with arterialisation
Splenic vein behind the pancreas
Arterigraphy – Splenic artery
Arteriography – Splenic vein
Arteriography
Splenic Arterio-Venous Fistula
Arteriography – Renal arteries
Surgical intervention
• Great amount of ascites
• Thrill in the splenic hilum
• Severe dilatation of splenic vein in hilum
• Ligation of splenic vein proximal to dilatation
• Splenectomy
• Liver biopsy
• Drainage & closure
Pathological report
• Spleen Augmented in volume - WNL
• Liver biopsy Chronic hepatitis B G2 - S1
Post-operative course
• Abdominal pain & low grade fever (38.5)
• WBC 12 800 (N 77 %)
• Abdominal US 1 week post-op:
Thrombosis of SV & PV
• IV heparin by pomp (1 000 U/h)
• Warfarin per os
Arterio-Portal Fistula Syndrome
APFS
• Fistula involving one or several arteries & the portal
vein or one of its tributaries
• Hepatic artery: 65% of cases
Splenic artery: 11% of cases
SMA or IMA: 24% of cases
• Up to 1996, 75 cases of splenic arterio-venous fistula
reported in the medical litterature
Z Gastroenterol 1996 ; 34 : 234 – 249.
APFS – Clinical presentation
• Asymptomatic
• Heart failure Rare
Protection of heart by liver
• Intestinal ischemia 20 %
Steal phenomenon
Abd pain- diarrhea - bleeding
• Portal hypertension 20 – 40%
Splenomegaly – varices – ascites
APFS – Treatment
• Recommended even in asymptomatic patient
• Depends on Cause – size of vessels – facilities
• Embolization Procedure of choice now
Small – intrahepatic – iatrogenic
Different materials (Gelfoam-Ballons)
• Surgery Large – other traumatic injuries
Resection – legation
Conclusions
• When PV > 2 cm we should suspect APFS
• Doppler sonogram is a good modality for diagnosis
• Splenectomy is an alternative to embolisation in large
fistula located in the splenic hilum
• Portal hypertention caused by APFS is a curable
disorder unlike many of the other causes of PH
Arterio-Portal Fistula Syndrome (APFS)

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Arterio-Portal Fistula Syndrome (APFS)

  • 1. Arterio-Portal Fistula Syndrome Case report & review of the literature Samir Haffar M.D. Assistant Professor of Gastroenterology
  • 2. Clinical History • 60 year-old female – 7 children • 2months ago: Alimentary & biliary vomiting Diarrhea 5 – 6 / day with tenesmus Abdominal pain especially epigastric Relieved by defecation
  • 3. Clinical examination • BP 105/70 mmHg • Pulse 90/min • Temperature 37.5 C • Generalized abdominal tenderness • Hepatomegaly (3 fingers width) • Ascites • Continuous murmur in the left fifth inter-costal space
  • 4. Laboratory studies • Hemoglobin: 10.7 • Total bilirubin 1.5 (direct 0.7) • Alkaline Phosphatase x 4 ULN • ALAT 43 (N 40) • ASAT 25 (N 38) • PT 60 % • Albumin 3.1 • HBs Ag + • HBe Ag – • Anti HCV –
  • 5. Ascitic fluid examination • Total protein 1.3 • Albumin 0.5 • WBC 1 130 Lymphocytes 90% Neutrophils 7 % • RBC 760 • Abnormal cells Negative • KB Negative
  • 6. Serum Ascites Albumin Gradient SAAG 3.1 – 0.5 = 2.6 ≥ 1.1 → Portal hypertension
  • 7. Endoscopic studies • UGI endoscopy Esophageal varices 1st degree • Colonoscopy Recto-sigmoidal erythema
  • 8. Ultrasound exam • Ascites in moderate amount • Splenomegaly 150 mm • Large vessel in splenic hilum • Dilated splenic vein 28 mm • Dilated portal vein 21 mm • Dilated intra-hepatic portal tract • GB sludge • Heterogeneous hepatic echostructure
  • 10. Doppler Sonogram • Venous circulation in vessel of splenic hilum • Dilated splenic artery 14 mm • Low RI in SA in hilum 0.42 • RI in intra-splenic arteries 0.61 • Arterialisation of flow in splenic vein • Mean velocity in portal vein 12 cm/sec • Normal hepatic veins Diagnosis: Splenic arterio-venous fistula
  • 11. Splenic artery from the celiac trunk
  • 12. Splenic artery in the hilum
  • 13. Splenic artery in the hilum
  • 15. Splenic artery with arterialisation
  • 16. Splenic vein behind the pancreas
  • 21. Surgical intervention • Great amount of ascites • Thrill in the splenic hilum • Severe dilatation of splenic vein in hilum • Ligation of splenic vein proximal to dilatation • Splenectomy • Liver biopsy • Drainage & closure
  • 22. Pathological report • Spleen Augmented in volume - WNL • Liver biopsy Chronic hepatitis B G2 - S1
  • 23. Post-operative course • Abdominal pain & low grade fever (38.5) • WBC 12 800 (N 77 %) • Abdominal US 1 week post-op: Thrombosis of SV & PV • IV heparin by pomp (1 000 U/h) • Warfarin per os
  • 24. Arterio-Portal Fistula Syndrome APFS • Fistula involving one or several arteries & the portal vein or one of its tributaries • Hepatic artery: 65% of cases Splenic artery: 11% of cases SMA or IMA: 24% of cases • Up to 1996, 75 cases of splenic arterio-venous fistula reported in the medical litterature Z Gastroenterol 1996 ; 34 : 234 – 249.
  • 25. APFS – Clinical presentation • Asymptomatic • Heart failure Rare Protection of heart by liver • Intestinal ischemia 20 % Steal phenomenon Abd pain- diarrhea - bleeding • Portal hypertension 20 – 40% Splenomegaly – varices – ascites
  • 26. APFS – Treatment • Recommended even in asymptomatic patient • Depends on Cause – size of vessels – facilities • Embolization Procedure of choice now Small – intrahepatic – iatrogenic Different materials (Gelfoam-Ballons) • Surgery Large – other traumatic injuries Resection – legation
  • 27. Conclusions • When PV > 2 cm we should suspect APFS • Doppler sonogram is a good modality for diagnosis • Splenectomy is an alternative to embolisation in large fistula located in the splenic hilum • Portal hypertention caused by APFS is a curable disorder unlike many of the other causes of PH