The Development of a Intrahepatic Biliary Cyst after Kasai Operation for Biliary Atresia

(1)

=Abstract=

The Development of a Intrahepatic Biliary Cyst after Kasai Operation for Biliary Atresia

Woo-Hyun Park, M.D., Soon-Ok Choi, M.D.

Division

0/

Pediatric SUrgery, Departments

0/

Surgery Keimyung University, Dongsan Medical Center

Taegu, Korea

A 6 and a half year-old girl developed recurrent cholangitis following hepatic portoenterostomy for biliary atresia. Computed tomogram showed an ovoid cyst (4.5 x 4.0 cm in size) in the left hepatic lobe and another tubular dilatation (2.0 x 0.8 cm in size) in the right hepatic lobe. Percutaneous transhepatic cholangio- drainage(PTCD) and cystogram showed an ovoid

cyst

in the left hepatic lobe (Tsuchida type A), measuring 6.6 x 5.0 cm in size. Following drainage and admini- stration of parenteral antibiotics she became afebrile and anicteric. However she continued to drain 45-150 cc of bile per day via the tube over the next 2 weeks.

The patient successfully underwent intrahepatic cystojejunostomy with intraoper- ative ultrasonographic guidance. This case illustrates relapsing cholangitis caused by Tsuchida type A intrahepatic cyst, successfully managed with PTCD followed by an internal drainage procedure.

Index Words:

Biliary atresia, Cholan!{itis, Intrahepatic cyst

19811;1 Hashimoto~ Yura¹7]- 'ifs:.~]:::11~O!]}.i

'T-

~ {i~

7clIi\7cH!

'ifs:.5:.<>J~(percutaneous transhepatic cholangiography, 0] ii]-

PTC)

~ A] i;~ ii]-o:J ;Q

41

'ifs:.~ ~

4'-

~-E <lJ-~ ~ .!i.Jl~ ~ 0] PTe

Correspondence: Woo-Hyun Park, M.D., Division of Pe- diatric Surgery, Departments of Surgery, Keimyung Univ- ersity, Dongsan Medical Center, 194 Dongsan-dong, Choon-Ru, TaeRu 700-712, Korea

~

c-l*Oi

3:

g

l1j-~ }.j-(Ultrasonography,

0]

ii]- US),

~{!li±~~oJ(O]ii]- CT), EC::: A]-7]~~o,P'J(0]ii]

MRJ)o] Kasal^l 4'-~ ~ ~7]-0i] o]%~~}.i

Zlt.l1

'if s:.'cl-~(intrahepatic

biliary

cyst, o]ii]-

rnC)

0] E.%;<.]

\"'ill] .!i.Jl ~Jl~q2.12 . .:::Lc.1Jl 0] rnc~ ~ ~ 'if

:s:.~.9.l %-.B.~ Jt!~.9.l ii]-Y.~ '€f51;<.]~}.i

rnCOi] r11

~ ~}.g71~ ')l ~lliO!l rJl~

.!Lil7]-

~o] ~q2.12.

- 126-

(2)

Fig. 1. Abdominal CT. A; an ovoid cyst(C) (45 x40 mm in size) in the left hepatic lobe, B; another tubular dilatation (20 x 8 mm in size) in the right hepatic lobe(arrow head).

A~ A}%.g- .:i1. '?i1 i!J- % '@"% ?.::i::£

'';H!

~ Kasai ~ + 6^A

· ll

~ a:j o}o1]Al '6-£ ~.9.] ~ ~ 0] :u-,*~'fl 01]

t@-"cl~ IBC£ ~~ ?;iE..£ -'8:cg~ ~aj]~ 78"'Bt"}

a:j -l-!:-~.:i1.~i!J- cl~o1 .!i!..:i1.t"}c l:l}o] ct.

~I

~o}c 6\1 67R-%J. ~ a:jo}£ 4"iJ:u- A]4,=-.£]C :u-

~~ .:i1.'?;1Jl}

9J-'€." g

?::t:.£ "id~t"}~,ct. ~ ~~.g- 1VJo]9Jct. i!J-?1~

g,

.!i!.1i'1 .~ + 58 "iJ 01] ~S.J!j]Sll

~(type III-b-v)~£ 7V}0]Y~(:U--l-!:-~ -=§-;<J ~~

~) ~ ;<J%-{l t@ ~C?4"* ll.!l.!:!. ~~] ~

g

^~~^o1jJ'l

't~}ct. ~ + 50"iJJl} 110 "iJ01] if"'J 'it.5:.~E..£

!f-

7o} ill] "id ~ ~1.fi. ~;j-q.

!f-

~ -¥l1 '6-£ ~ E..£ "id

~A] A]ii~~ USo1]Al ,*~'fl01] 15X1Omm 3.7]Qj 'J3':.-"J ~~7} .!i!.~ct. ~ + 130~01] %'@"o] ~

~o] .::i::llS1~q. ~

4-

3\1 107R-%J.01] ~£~PJ1"*

Fig. 2. Percutaneous transhepatic cholangiogram, sho- wing a non-communicating cyst (Tsuchida type A), measuring 66 x 50 mm in size.

~~.£ ~il:lt"}a:j 1]1A]78% o]%~ ~~"* ~~~

% ~;j-E..Dj 0]

uJl

A]ii~~ USo1jJ'l ~01] .!i!.0]1i:l.

:u-1]1 '6-.5:. 'J3':.-"J ~~7} 36 X 33 mm £ :I:'6- cl

7 1 Al

:V. ~ ct.

"id~A] ~{}~E..£ 9J-'@"0] :v.~.:i1., :U-;<J o}~.g

+~ ::;i'"o} 2 ii~7.] ,€ilo]£ ~7.] S1~E..Dj l:lVJl:l]

rJ17} :V.~E..L.}

*Yc

ict~q. ~.7.a :U-7]-'6'-

7a

^A^}-'cl'

Total bilirubin 7.2 mgjdL, Direct bilirubin 4.4 mg/

dL, ALP 483 U/L, AST 54 UjL, ALT 112 UjL 0]

9J c}. US ~ CToJ] Ai TI'*'fl Qj ~.:rC?4.9]

'8-

^3':.),a

~~7} 45 X40 mm£ :I:'6- cl

7'i Al

:V.5j,E..Dj(.=I.'8 lA), +~'flo1]£ 20x8mm Qj ~t@Qj '6-~ ~;<J 01 .!i!.~ct(.=I.'l:l IB). ~~ qg

\t

PTCD~ CT .5:.

%~ ~o} A]ii~o};jq. PTCD-"] llA]~ '6-~ s:°cl

~011Al 'J~-"cl ~~.g- 66X50mm 3.7]£ .!i!.~E..

oj ;<J~..2..£ llH~S1c .::i::7,J~ ict~q(Tsuchida type A)(.=I.'E] 2). PTCD~ 7oH- 11 A] ~~

uJl

^~~

~ '6-iI- 0] 40 mL ~£ L.}~E..Dj ifIlH°J: 7,:lAH~.J2}

E. Coli7} IlH°J:.s:]~q· "iJ ?"iJ +01] qA] {)'~.£]

01 :v.c IlH«l1~% ~i5R 'J~:I:°cl~%- A]ii~t"}a:j .!i!.

Y

'J~-"cl ~~o] 45 X35 mm£ 3.7]7} ~.::i::S1o.]

:V. ~

g..

PTCD.2}

78

^~^~"5J),~ ;(~] .!f-O:j £ '?i1 0] ~ 01

~E..Dj ~7o}~E..'£ %'@"£ ::t:.11S1~E..L.} IlH«l1~%

~i5R '6-~0] o}if-01] 45-160 mL(lla if 64 mL)7} 2y :u- ;ti14,=- IlH~.s:]~ct. A]:U-⁰] 7.]1...}.5:. llH«l1~~ ~i5H 7.]4,=-~E...ii'. '6-~0] IlH~S101Al 0J~ 15 ~"iJ01] 7R

*

t"}a:j ~ %- US .5:.% t"H] ,*~:u-'fl 1]1 'J%-=§-;<J

T~~ ~ .!:!.c.}-'£- ~~~(Intrahepatic cystojejunos-

tomy with Braun anastomosis)%- +~ ~ +~~.

(retrogastric and retrocolic) ~ 70]£ A] ii~o};j ct. ~ + 6"iJ011 99I1lTc_DISIDA TI'it£ ?A}~ A]ii~t"}O:j

- 127-

(3)

~ ~ Jlj-, q] If-~ 91 %~ st! 5:7 t Kasai y~ If-~ 5'- llR ~ ~ Sd..2.. Y. ~

If-;:

'-8-"5'.:g-;;<J--R-~ If-~ 5'- llH ~ ~

~ 3:1 0] ~~~Sd.cj-. ~ ~ 7 ~oJ] ~ -R-A1]ido]

sqst!"6t~.2..t9 sqst!A] ~-"a Zl7]~ 7,:j AtoJH ~-"a Zl7]

'0

7,:j}V-J- ~ ~c.]~Jtl7.] 2.4 mgJdL, ~.::<J ~ c.]~Jtl7.] 1.3. mg/dL, ALP 587 U/L, AST 103 U/L, ALT 72 U/L 0] Sd. cj-. ~ st! ~ 1 7R-%I oj] US oj] Ai

4

~Zl'{lt.J1 '-8-"5'~ 8 X8 mm37] 5'- fJ"0] ~Di~.2..t9,

~~'{l ~~£ lOX6mm 5'- 37]7t ~.!2..cj- 5:ij-

~Di ~Sd.cj-. ifJ= ~ Zlt.J1 '-8-~"'el ~~ ~ 9-7]-3cj.2..5'- 7-l]4i- ~-3cj ~~"6tjl7-t ~cj-.

7'tAto] y~ ~ 7t'::<J- ~"6tjl oj]~oJ] 'Ftll~

°d

ifJ= ~ 77]~];: ~~ ~-g. 'tt£ ~ 0] t9 y~ ¥}7-t 40- 60 % oJP·i ~"'~~cj-12 . .::z.2.iy. 0] 'tt£~91 st!~ ~

~~"6] it~~Di ~7-] ?J-cj-.

-2-211

^{oj] ;:}0] 'tt£~Jlj

~'i1iiHAi Zlt.J1 '6-£91 ~:&oJ] ~~ .!2..jl7t fJ"0]

y.~jl ~cj-. Kimura ,;2~ 'tt£~o] ~~ ¥}otoJ]

Ai PTC~ o]%"6te:j Zlt.J1 'tt~ ~.::<J-o] 0] 'tt£~

91 st! ~ ~ 3:1 ~ .!2..jl "6t~ cj-. .::z. ~ e:j 2.i 7-i 7-t ~ 0]

~ ~ i'-7]-3cj~ US ~~ MRI~ A]i;~~ ~Jlj-, Betz ,;1O~ 44oj]'F 8oj](18 %), Nakama ,;8-g. 24 oj]

'F 6oj](25 %), , Takahashi '; 11 ~ ~ ifJ=-3cj.2..5'- MRI 7,:lAt ~ A] i;~ ~ 16oj] 'F 4oj](25%)oJ]"'i IBot y.F.}

'it

~ .!2..jl "6t~ q .

Tsuchida ,;9 ~ IBC91 ~'E11oJ] u:J-4 37t7-]5'- ~

*"6t~;:t:~] Type A~ tl]'?:l.~"'el '-8-"5'(a non-communicating cyst), Type B;: '?:l. ~"'el '-8-"5'(a biliary cyst with a tiny communication with the intestinal loop), Type C;: q~"'el '?:l.~"'el '-8-"5' (multiple cystic dilatation of the intrahepatic biliary duct).

IBC ~ ~ uJl;: q] If-~ '6-£ ~ 0] % lfr"6t;: 3:1.2..£

.!i!.jl~ jl ~ q4.6.11. lfrq]£ Betz '; ¹⁰~ AH ~"'el '6-

£~o] IBC~ 0]:7]"6t;: st!~o] ~ y ~qjl i'-;;<J-

"6t~q. ~ 'tt£~o] ~,,~W~ 0]5'- ~iiR Zlt.J1 '6-

~.9.j 1ll]~7t ~"~"6te:j IBOt ,,~~ y ~cj-jl 1l.~

"6t~q. R'2f"6t~ IBC9.j- 'tt£~ ~ ~1l~ ~7-l]7t

~jl '6-£~0] ~"~"6t~ Zl7]~0] c-l* Y.UBt7-];:

t:~] Tsuchida ,;9 ~ Type C7t 7t;;<J- oj]~7t ~7-] ?J-

;: 3:1.2..5'- .!i!.jl"6t~ q.

IBC~Ael91 7]~ ~ '~A77t7-] ~~"6] ~.fi97-]7-]~

?J-~.2.. Y. e:j 2.i R ~ ~ 0] ~e:j "6t~ 3:1.2..5'- .!2..jl ~ jl ~ q. e:j 2.i '?:l. ~A% ~ ~ ~oJ] tl] ~ '6-~ ~tl]

- 128-

7t "'el.:g--3cj.2..5'- i9, q"6tc-l4 £ Zl-91 ~ ~"el '3<l {l % :& ~:&7t {)i;~~~ .!2..jl"6t~;:I:~]3.13-15 Werlin,;5

'3<l Tsuchida %9£ ~ ~oJ]£ {)i;~~;: Zl-91 ~~

"'el '3<l {l%:& ~:&5'- ~i;H Zl-t.J1 'tt~o] lll]~.£]Di

IBOt ~,,~W 3:1 o]c.]-jl ~~"6t~q. Takahashi

,; 11

-g.

Zl--R-lf-

{l %5:~ Jlj- Zl-,,~ 7,:j 5:~ 91 intralobular space91 {l%"'el '3<l ~~"'el ~:&9.j-91 ~7-l]~ '?:l.-=r- iiR ~ ~Jlj-, IBOt y.F.}'d- ¥}7-t7t IBOt y.F.}y.7-]

?J-~ ¥}7-t.!2..q .::z. ~£7t {l~ 3:1.2..5'- .!2..jl"6t~}.i IBC~ "'el-g. 'tt £ 1ll] ~ ~ Jlj- ~ ~ st! ~ oj] 91 iiR ~,,~

"6t~ 3:1 0]4 jl i'-;;<J-"6t~ q.

IBC91 ~]li~ 371]

.!f-

7t7-]5'- q]~~;: t:~]

:3i

~;: PTCDO]jl2.6.9 %~;: y~ R~.2..5'- 9111H'1ll

~( external drainage) '3<l t.J111H '1ll ~(internal drainage) 0] .!2..jl~Sd.q4.5.9. 0] ~ill];: l~t PTCD~ "6tjl

'i;J-"~A1] -i'-e:j5'- ~.AJ--g. ~ot~7-]~ IBOt 7-l]4i-

'it

ot ~Sd.jl 1l-'1l i9, ~ ~ ~iiR 7-l]4i--3cj ~ '6-~ %~

0] ~Di}.i Zlt.J1 '-8-"5'.:g-7-J--R-~~~ A]ii~"6t~~t:~]

oj]"'J-.!2..q£ IBC ~7.] ~~o] 1j7-] ?J-o}J.i ~ 'F

~

g

3Ij-£ %.2..5'- y~o] 7t

'0

"6t~ q. Type A

7d

~ Saito %4 '3<l Werlin ,;5 ~ 'T-~-3cj 7.i7.] ~ i'-7-J-'9!J.7-]

~ Nakama '; 8 ~ PTCD ~ ~iiR

7d

:&A1] ~ ~~ ~ i'-'1l i;te:j 7.]li ~ oj]~ .!2..jli;t~ q. Type B oJ]Ai;:

PTCD9.j- ifJ-"~~]~ -i'-e:ji;t~ ~~o] ~~~~ IBC 7t idDi~

Y

~qjl i;t~q5.8. 7.]li llJ-~~ IBC 91 %~, ~~], 37] oj] u:J-4 3i~~ ~~o] ~Ri;t c.]4 "~4i9,q. ~ ~ill]oJ]Ai ~~ Zl-'{l91 ~~ '3<l 4~ Zl-'{l91 ~ ~ ~~.9.j ~:&;: ifJ=~ 7-l]4i- ~~

~ ~R7t ~.2..c.] c.]- ,,~Zj-i9, q.

~ ~ IBC~ 'tt£~91 'FR~ .!(i~'F91 i;ty.o]

£5'- 'tt£1ll]~ ~ 'T-~ ~ ~ ~ ~oJ];: 67H-%I t.J17-]

1l;'!°tcj- ~

Jti-61

i'-7]-3cj.2..5'- US, CT ~;: MRI~

A]i;~i;te:j IBC ~,,~ e:jlf-~ 7,:l}.t~ ~R7t ~qjl

"~Zj-~t9, IBOt C+F.}C+;: ¥}ot;: ifJ= ~ '6-£~ ~

~.2..~ Jl ~~~oJ] 4i-i;t7] uJl-l¥:-oJ] % c-l 7-ti'- ",]

~i;] ~-3cji;te:j -3cj~~ 5:7.] ~ ~i;te:jo]: ~qjl ,,~

Zj-i9,q.

1. Hashimoto T, Yura J: Percutaneous transhepatic cholangiography (PTC) in biliary atresia with special reference to the structure of the intrahepatic bile ducts. J Pediatr Surg 16:22- 25, 1981

(4)

2. Kimura K, Hashimoto S, Nishijima E, Muraji T: Percutaneous transhepatic cholangiodrainage after hepatic portoenterostomy for biliary atresia. J Pediatr Surg 15:811-816, 1980

3. Ito T, Horisawa M, Ando H: Intrahepatic bile ducts in biliary atresia-a possible factor deter- mining the prognosis. J Pediatr Surg 18: 124- 130, 1983

4. Saito S, Nishima T, Tsuichida Y: Intrahepatic cysts in biliary atresia after successful hepato- portoenterostomy. Arch Dis Child 59:274-275, 1984

5. Werlin SL, Sty JR, Starshak RJ, Glicklich M, Nathan R: Intrahepatic biliary tract abnormalities in children with corrected extrahepatic biliary atresia. J Pediatr Gastroenterol Nutr 4:537-541, 1985

6. Gleghorn EE, Rosenthal P, Vachon L, Dia- ment M: Long-term external catheter biliary drainage for recurrent cholangitis after hepato- portoenterostomy. J Peidatr Gastroenterol Nutr 5:485-488, 1986

7. Ishii K, Matsuo S, Hirayama Y, Taguchi T, Yakabe S, Ikeda K, Hirata T, Kawanami T:

Intrahepatic biliary cysts after hepatic portoenterostomy in four children with biliary atresia.

Pediatr Radiol 19:471-473, 1989

8. Nakama T, Kitamura T, Matsui A, Makiko S, Senyuz OFS, Kanazawa K: Ultrasonographic findings and management of intrahepatic biliary tract abnormalities after portoenterostomy.

J Pediatr Surg 26:32-36, 1991

- 129-

9. Tsuchida Y, Honna T, Kawarasaki H: Cystic dilatation of the intrahepatic biliary system in biliary atresia after hepatic portoenterostomy. J Pedialr Surg 29:630-634, 1994

10. Betz BW, Bisset III GS, Johnson ND, Bali- streri WF: MR imaging of biliary cysts in children with biliary atresia: Clinical associ- ations and pathologic correlation. Am J Radiol 162: 167 -1 71, 1994

11. Takahashi A, Tsuchida Y, Suzuki N, Kuroiwa M, Ikeda H, Hirato J, Hatakeyama S: Incid- ence of intrahepatic biliary cysts in biliary atresia afterhepatic portoenterosotmy and asso- ciated histopathologic findings in the liver and porta hepatis at diagnosis. J Pediatr Surg 34:1364-1368, 1999

12. Ohi R, Ibrahim M: Biliary atresia. Semin Pediatr Surg 1:115-124, 1992

13. Altman RP, Chandra R, Lilly JR: Ongoing cirrhosis after successful porticoenterostomy in infants with biliary atresia. J Pediatr Surg 10:

686-684, 1975

14. Gautier MG, Valayer J, Odievre M, Alagille D: Histological liver evaluation 5 years after surgery for extrahepatic biliary atresia: A stu- dy of 20 cases. J Pediatr Surg 19:263-268,

1984

15. Luenzmann K, Schweizer P: The influence of cholangitis on the prognOSis of extrahepatic biliary atresia. Eur J Pediatr Surg 9:19-23, 1999