Table 3 Characteristics of those with breakthrough bleeding despite primary prophylaxis.

Total patients

18

100

Underlying diagnosis

–

–

Alpha-1-antitrypsin deficiency

1

6

Autoimmune hepatitis

2

11

Biliary atresia

3

17

Cavernous transformation/thrombosis of the portal vein

1

6

Congenital hepatic fibrosis

1

6

Cystic fibrosis related liver disease

4

22

Primary sclerosing cholangitis

3

17

Other

3

17

Mean age at first endoscopy (SD)

8.39 (5.61)

–

Age range at first endoscopy

0.76–17.71

Variceal grade prior to breakthrough bleeding

–

–

None

2

11

Grade 1/small/mild

10

56

Grade 2/medium/moderate

1

6

Grade 3/large/severe

5

28

Patient received ligation/sclerotherapy in endoscopy prior to breakthrough bleeding

3

17

Presenting symptom

–

–

Hematemesis

3

17

Melena/bloody stools

10

56

Anemia

1

6

Hemoptysis

1

6

Other

3

17

Variceal grade at the time of breakthrough bleeding

–

–

None

2

11

Grade 1/small/mild

6

33

Grade 2/medium/moderate

3

17

Grade 3/large/severe

6

33

Visualized but not graded

1

6

Nonvariceal cause of bleeding identified

7

39

Ulcer or gastritis

4

22

Anal fissure

1

6

Inflammatory bowel disease

1

6

H. pylori

1

6

Treatment at the time of bleeding

–

–

None

7

39

Secondary ligation

10

6

Secondary sclerotherapy

1

6

Mean years from first endoscopy to breakthrough bleeding (SD)

2.25 (2.28)

–

Range of years from first endoscopy to breakthrough bleeding

0.11–7.56

–

Mean years from previous endoscopy to breakthrough bleeding (SD)

0.70 (0.64)

–

Range of years from previous endoscopy to breakthrough bleeding

0.04–2.35

–

Outcomes

–

–

Portosystemic shunt

2

11

Liver transplant

6

33

Death

3

17

Shunt and transplant free survival

7

39