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