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Table 1 Summary of findings in patients with Abernethy Malformation

From: Diagnostic accuracy of multi-slice computed tomography in children with Abernethy malformation

Case No.

Age (y)/Sex

Clinical Features

Fistula classification and anatomy

Treatment

1

11 years/Female

VSD, Hyperammonemia, Abnormal liver function

Type Ib(PV-IVC)

Lost to follow-up

2

6 years/Female

PH,

Nodular liver lesions

Type Ib(PV-IVC)

Liver transplantation

3

12 years/Male

Hematochezia, PH,

Hyperammonemia

Type Ib(SV-iliac vein-IVC)

Lost to follow-up

4

11 years/Female

Cyanosis, Pulmonary arteriovenous fistula, Hyperammonemia

Type II(PV-IVC)

Surgical shunt occlusion

5

9 years/Male

Fatigue, PDA, Nodular liver lesions

Type II(PV-IVC)

Surgical shunt occlusion

6

4 years/Male

PH, Hyperammonemia

Type II(PV-IVC)

Surgical shunt occlusion

7

9 years/Male

Fatigue, PH,

Hepatic encephalopathy, Heart insufficiency, Hyperammonemia

Type II(PV-IVC)

Surgical shunt occlusion

8

14 years/Male

Fatigue, VSD,

Hyperammonemia, Nodular liver lesions

Type II(PV-IVC)

Lost to follow-up

9

5 years/Male

Cyanosis, Pulmonary arteriovenous fistula, Hyperammonemia

Type II(PV-IVC)

Interventional portocaval shunt occlusion under DSA

10

10 years/Female

Hematuria, Renal vascular malformation, Hyperammonemia

Type II(SV-iliac vein-IVC)

Surgical shunt occlusion

11

3 years/Female

ASD,PH,

Abnormal liver function

Type II(PV-IVC)

Interventional portocaval shunt occlusion under DSA

12

4 years/Male

Fatigue, ASD/PAPVC, Pulmonary arteriovenous fistula, Hyperammonemia, PH

Type II(PV-LRV-IVC)

Surgical shunt occlusion

13

7 years/Male

PH, Hyperammonemia

Type II(PV-IVC)

Surgical shunt occlusion

14

1 year/Female

Cyanosis, ASD, Polysplenia syndrome, Pulmonary arteriovenous fistula

Type II(SMV-LRV-IVC)

Interventional portocaval shunt occlusion under DSA

  1. PV = portal vein; IVC = inferior vena cava; SMV = Superior mesenteric vein; LRV = left renal vein; ASD = atrial septal defect; VSD = ventricular septal defect; PDA = patent ductus arteriosus; PAPVC = partial anomalous pulmonary venous connection; PH = pulmonary hypertension