Biliary Atresia with Extrahepatic Biliary Cyst

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Biliary Atresia with Extrahepatic Biliary Cyst

Jae Hee Chung, M.D., Han Hong Lee, M.D., Seon Wook Cha, M.D., Young Tack Song, M.D.

Department of Surgery, College of Medicine, the Catholic University of Korea Seoul, Korea

Biliary atresia (BA) with extrahepatic biliary cysts (EHBC) is a rare disease.

^It

has been generally recognized as type I (correctable with cystic dilatation), which means a good prognosis. From a total of 73 patients with BA who underwent operation from September 1988 to September 2003 at our institute, 7 (9.6

%)

cases of type III BA with EHBC (uncorrectable with cystic dilatation) are reviewed.

Clinical findings, laboratory data, radiologic findings, treatment methods and outcomes were reviewed. Female was more prevalent (male to female ratio: 2:5).

All cases were type III with EHBC according to the intraoperative cholangiography, and underwent Kasai's portoenterostomy. The mean age was 57 days at operation.

Three patients(42.9

%)

are long term survivors. Further evaluation is needed to determine the correlation between prognostic factors and outcome for.

o

Kor Assoc Pediatr Surg 10(2):136-141), 2004.

Index Words: Biliary atresia, Extrahepatic biliary cyst, Uncorrectahle type

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Fig. 1. Ultrasonography (A and 8), MRCP (C) and intraoperative cholangiography (0) of the Case 1. A; arrow indicates the small atretic gall bladder. 8,C,D; arrows indicate the extrahepatic biliary cysts.

Table

1.

Age, Sex, Symptom, Sign and Preoperative Laboratory Findings

Age at Preoperative laboratory findings

Case NO. Sex Symtom & sign Total bilirubin Direct bilirubin operation (d)

(mg/dL) (mg/dL) AST (U/L) ALT (U/L)

F

21

Jaundice, acholic stool

10.9 3.9 111 15

2

M

58

Jaundice, acholic stool

8.4 5.6 360 160

3

F

65

Jaundice, acholic stool, fever

8.0 5.0 92 60

4

M

60

Jaundice, acholic stool

8.6 5.2 168 128

5

F

40

Jaundice, acholic stool,

abdominal mass

7.6 3.5 152 92

6

F

90

Jaundice, acholic stool

6.0 3.3 448 259

7

F

64

Jaundice, acholic stool

9.9 6.9 222 216

Table 2. Diagnosis and Operation

Operation Case NO. Diagnostic methods Preoperative diagnosis OP. cholangiogram

(CBD diameter, cm) Sonogram, DISIDA scan, MRCP BA

c

^EHBC Typelll BA c EHBC (2) 2 Sonogram, DISIDA scan BA

c

^{EHBC Type} Ii I BA c EHBC (1) 3 Sonogram, DISIDA scan, CT Choledochal cyst Typelil BA c EHBC (1.5)

Kasai operation Kasai operation Kasai operation Kasai operation Kasai operation 4 Sonogram, DISIDA scan, CT BA

c

^EHBC Typelil BA c EHBC (2)

5 Pre & post-natal sonogram, Choledochal cyst Typelil BA c EHBC (4.8) DISIDA scan, CT, MRCP

6 Sonogram, DISIDA scan BA

c

^EHBC

7 Sonogram, DISIDA scan, CT BA

c

^EHBC

BA

c

EHBC : Biliary atresia with extrahepatic biliary cyst

Table 3. Pathology and Outcomes

Case Ohkuma's classification of Hillum status J d·

NO. liver fibrosis . aun Ice

(mlcroductule>150 f.lIJl)

1 Grade 1 +

2 Grade 2 Unknown

3 Grade 1

4 Grade 1 +

5 Grade 2 Unknown

6 Grade 2 +

7 Grade 1 +

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Death cause (follow-up periods)

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hop less discharge Died (13 m) Liver cirrhosis,

refractory cholangitis Alive (9 yr)

Alive (5 yr)

Died (4 m) Liver cirrhosis Alive (21 m)

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