Arterio-Portal Fistula Syndrome (APFS)

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Arterio-Portal Fistula Syndrome (APFS)

  1. 1. Arterio-Portal Fistula Syndrome Case report & review of the literature Samir Haffar M.D. Assistant Professor of Gastroenterology
  2. 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. 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. 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. 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. 6. Serum Ascites Albumin Gradient SAAG 3.1 – 0.5 = 2.6 ≥ 1.1 → Portal hypertension
  7. 7. Endoscopic studies • UGI endoscopy Esophageal varices 1st degree • Colonoscopy Recto-sigmoidal erythema
  8. 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
  9. 9. Splenic hilum
  10. 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. 11. Splenic artery from the celiac trunk
  12. 12. Splenic artery in the hilum
  13. 13. Splenic artery in the hilum
  14. 14. Intra-splenic artery
  15. 15. Splenic artery with arterialisation
  16. 16. Splenic vein behind the pancreas
  17. 17. Arterigraphy – Splenic artery
  18. 18. Arteriography – Splenic vein
  19. 19. Arteriography Splenic Arterio-Venous Fistula
  20. 20. Arteriography – Renal arteries
  21. 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. 22. Pathological report • Spleen Augmented in volume - WNL • Liver biopsy Chronic hepatitis B G2 - S1
  23. 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. 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. 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. 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. 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

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