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 GradientSAAG
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 SyndromeAPFS
• Fistula involving one or several arteries & the portal vein or one of its tributaries
• Hepatic artery: 65% of casesSplenic artery: 11% of casesSMA or IMA: 24% of cases
• Up to 1996, 75 cases of splenic arterio-venous fistulareported 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 nowSmall – intrahepatic – iatrogenic Different materials (Gelfoam-Ballons)
• Surgery Large – other traumatic injuriesResection – 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