The Surgery of Biliary Atresia

JOHN R. LILLY, M.D., FREDERICK M. KARRER, M.D., ROBERTA J. HALL, M.D.,* GIANNA P. STELLIN, M.D.,tJUAN J. VASQUEZ-ESTEVEZ, M.D.,4 STEPHEN K. GREENHOLZ, M.D.,§ ELIZABETH A. WANEK, M.D.,

and GERHARD P.J. SCHROTER, M.D.

One hundred thirty-one consecutive infants with biliary atresiawere operated on during the 15-year period between 1973 and1988. Six patients did not have biliary reconstruction becauseof advanced cirrhosis or transplant preference. The other 125infants had excision of all nonpatent extrahepatic bile ducts;biliary drainage was provided by a gallbladder-common bile ductconduit in 14 patients and by a Roux-en-Y portoenterostomy in111 infants (including the seven patients with correctable biliaryatresia). The bilioenteric conduit was temporarily exteriorizedand, for the past 2 years, a conduit intussusception valve wasincorporated. Immediate postsurgical bile drainage was achievedin 103 infants (82%). Reoperation during the first 6 postoperativeweeks restored bile flow in 14 of 18 infants who had shut down.Seventy-two patients (57%) had sustained (more than 1 year)relief of biliary obstruction. Postoperative morbidity was sub-stantial. The six children not having corrective surgery diedwithin 19 months. Three patients were lost to follow-up. Sixty-eight patients having Kasai's operation died, 55 from compli-cations of liver disease, 1 from a coexisting malformation, and12 after liver transplantation. Fifty-seven patients are alive, 13by virtue of liver replacement, 9 with mild-to-moderate hepaticsequelae, and 35 (28%) with normal to near-normal liver function.Although none is considered "cured," the 35 children are anic-teric, have normal growth and development, and participate infull school activities (including contact sports). Average follow-up is 85.8 months (range 1 to 15 years).

Presented at the 109th Annual Meeting of the American Surgical As-sociation, Colorado Springs, Colorado, April 10-12, 1989.

Correspondence and reprint requests: John R. Lilly, M.D., PediatricSurgery C 314,

University of Colorado SchooL of Medicine, 4200 East Ninth Avenue,Denver, Colorado 80262.

Current addresses:*University of Pittsburgh, Pittsburgh, Pennsylvania.tUniversity of California, Irvine, Orange, California.tClinica Infantil "La Paz," Madrid, Spain.§Children's Hospital of Michigan, Detroit, Michigan.This work was supported in part by grant RR-69 from the General

Clinical Research Centers Program ofthe Division ofResearch Resources,National Institutes of Health and The Pediatric Liver Center, UniversityHospital, Denver, Colorado.

Accepted for publication: April 13, 1989.

From the Department of Surgery, University of Colorado- School of Medicine, Denver, Colorado

B ETWEEN NOVEMBER 1973 AND April 1988, 148infants were operated on for obstructive jaundice.Seventeen had choledochal cyst or biliary hypo-

plasia syndromes, e.g., arteriohepatic dysplasia. Onehundred thirty-one infants had biliary atresia. Six of the131 infants did not have biliary reconstruction becauseof advanced cirrhosis or preference for liver transplan-tation. The other 125 infants were treated by Kasai's'procedure in which all nonpatent extrahepatic bile ductswere excised and biliary drainage was provided by Roux-en-Y hepatic portoenterostomy (111 patients) or by agallbladder-common bile duct conduit2 (14 patients).

In this communication we review the key surgical de-tails of the operation, describe early maneuvers to main-tain bile flow, outline our long-term results, and identifya conspicuous group of long-term survivors who appearto be restored to normal health.

Materials and Methods

Of the 131 patients, 83 were female and 48 were male,and their ages at operation ranged from two days to 7months (mean, 66 days).

Operative Technique

An operative cholangiogram was possible in only 21patients, 14 of whom had residual patency of the gall-bladder, cystic and common bile ducts, and seven ofwhom had "correctable" (infra vide) biliary atresia. Aftercholangiography or determination ofa fibrotic gallbladder,the common bile duct was divided and the extrahepaticbile ducts were dissected to the liver hilus. Dissection wasthe same for patients with correctable biliary atresia in

289

Ann. Surg. September 1989LILLY AND OTHERS

FIG. 1. Cholangiogram 21days after Kasai operation ina patient with a "gallbladder"reconstruction. The hilaranastomosis has healed; thecystic-common bile ductstructures (white arrows)have dilated; the decompres-sive catheter will be removed.Note the hypoplastic intra-hepatic bile ducts (black ar-rows) and drainage via twoto three major intrahepaticducts into the gallbladder.

whom the blind ending bile cyst was mobilized in con-tinuity with the sclerotic duct structures. Exposure at theliver hilus required full mobilization of the portal veinbifurcation and ligation of its small branches to the resid-ual duct. Fine absorbable sutures were placed at the lateraland posterior margins of the duct-liver interface. Thecommon hepatic duct was transected flush with the liverand immediate histologic examination was obtained. Ifno true bile ducts3 were identified, the posterior suturewas placed on traction, excised, and a second (often re-warding) specimen was obtained.

In the 14 patients in whom the gallbladder, cystic duct,and common bile duct were patent, an incision was madein the gallbladder, which was then anastomosed to thehilar duct transection site. In the last eight patients, acatheter was placed in the gallbladder for postoperativebiliary decompression and was not removed until a chol-angiogram verified healing of the gallbladder-hilar anas-tomosis (Fig. 1), usually between ten and 21 days.

Biliary reconstruction in the other Ill patients, in-cluding those with correctable biliary atresia, was by a 20-cm Roux-en-Y hepatic portoenterostomy. The defunc-tionalized open end of the jejunostomy was anastomosedto the edges ofthe transected duct at the liver hilus (usingthe previously placed lateral duct margin sutures) in acontinuous, single layer. From 1973 to 1977, a Mickuliczenteroenterostomy was incorporated in the bilioenteric

conduit.4 Simple double-barrelled exteriorization of theRoux-en-Y jejunostomy was employed between 1978 and1986. For the past 2 years, a complimentary intussuscep-tion "valve" was placed in the intestinal limb of the ex-teriorized conduit (Fig. 2).

Early postoperative Management

Early diminution or cessation of bile flow was treatedwith intravenous corticosteroids (Fig. 3).5 Cessation ofbileflow recalcitrant to steroid therapy, was treated by reop-eration in which the anterior hilar anastomosis was takendown, the hilum scored, and the anterior anastomosisredone.

In all patients with externalized conduits, bile was col-lected from the "hepatic stoma" daily, its volume mea-sured, and an aliquot analyzed for bilirubin. Bile was refedinto the "intestinal" stoma three times daily. Closure ofthe exteriorized conduit varied from 24 days to 3 years.The duration between primary operation and closuregradually has been shortened and currently is between 6to 12 weeks after operation.

Late Postoperative Management

Cholangitis was treated by systemic antibiotics. Anti-biotic recalcitrant cholangitis early in this series wastreated by reoperation, e.g., stoma revision, catheter de-

290

THE SURGERY OF BILIARY ATRESIA

STEROID EFFECT ON BILIRUBIN EXCRETION

FIG. 2. Artist's drawing of the type of biliary reconstruction currentlyused in Denver. Upper left depicts the initial reconstruction. The "he-patic" limb is as short as possible; and the "intestinal" limb has an in-corporated intussusception valve distally. The conduit is exteriorized.Lower right illustrates the completed reconstruction. The conduit is in-ternalized 6 to 12 weeks after operation. Inset shows details of valveconstruction, i.e., 3 cm of the Roux-en-Y jejunostomy have been intus-suscepted and affixed with seromuscular interrupted silk sutures.

compression,6 scoring of the liver hilus,7 and since 1981,by a change in antibiotic,8 steroid therapy, and only oc-casionally by reoperation. Patients with cholangitis re-calcitrant to these measures were referred for liver trans-plantation. Esophageal variceal hemorrhage was treatedby esophageal endosclerosis,9 and hypersplenism wastreated by splenic embolization.10 Fat soluble vitaminswere given routinely." Standard infant diets were begunafter stomal closure.

Results

Postoperative Bile Drainage

One hundred three of the 125 infants (82%) had early(30 days) bile drainage after operation, i.e., bilirubin con-

centration was higher in bile than in serum. Sustained(more than 1 year) bile flow was achieved in 72 pa-tients (57%).

1.5

BILE 1.0

BILIRUBIN

(mg/day) 0.5 A

A~~~~~~~~~0.0 A A A

10.

STEROID I I I

BOLUS 5

(mg/day) OKasai 5 S 15 2

DAYS POSTOPERATIVE

FIG. 3. The effect of bolus methylprednisolone on falling bile bilirubinconcentration after Kasai's operation. Arrows exemplify dramatic im-provement in bilirubin excretion. Bile flow stabilized 25 days after op-eration in this infant and steroid therapy was stopped.

In approximately one third of infants, the daily bileexcretion markedly decreased in volume and concentra-tion at some point during the first six postoperative weeks.A steroid bolus regimen often reversed the impendingshut-down (Fig. 3). Steroid therapy frequently had to berepeated until bile flow stabilized at a satisfactory level.In 18 infants bile flow diminished and then ceased alto-gether. Prompt reoperation permanently restored bile ex-cretion in 14 patients, one ofwhom is our longest survivor( 15 years).

Cholangitis

Infants who failed to drain bile after operation and,with some exceptions, those who had a gallbladder conduitor a conduit intussusception valve, did not develop post-operative cholangitis. The other 81 infants had one to 27attacks of cholangitis. In most patients, cholangitis re-solved promptly with systemic antibiotic therapy and, ifnot, until 1982, were reoperated on (supra vide). Since1982, 17 patients with antibiotic resistant cholangitis weretreated with adjunctive steroid therapy, with improvementin all but four children who were subsequently referredfor liver transplantation. Susceptibility to cholangitis usu-ally resolved between the first and second postoperativeyear.

Portal Hypertension

Clinical manifestations of portal hypertension, i.e.,esophageal varices, hypersplenism, or ascites, developedin 21 of the 72 patients with sustained bile flow. Bleedingfrom esophageal varices was controlled by one to eightvariceal endosclerosis sessions in all 12 patients who bled.Nine patients with hypersplenism were treated by splenicembolization with excellent long-term results. Mild, tem-

291vol. 210-No. 3

LILLY AND OTHERS

porary ascites were simply observed in three patients; onerequired diuretic therapy. Symptoms of portal hyperten-sion were primarily between the second and third post-operative year, although two patients had an initial var-iceal bleed at ages 5 and 10 years, respectively.

Mortality Rates

The six patients who did not have a Kasai operationdied in 2 to 19 months (average, 8.6 months; Fig. 4).There was no Kasai operative deaths. Three patients werelost to follow-up. Sixty-eight of the 125 children who hadthe Kasai operation died from 3 to 95 (average, 25) monthsafter operation from complications of liver disease; 12 ofthese patients were waiting for transplantation 1 to 16(average, 5.7) months before death. Twelve patients diedafter liver transplantation, one of whom had stable liverfunction (bilirubin < 5 mg%) before grafting. An excep-tional patient died from a coexisting congenital disease.

Fifty-seven patients are alive, 13 by virtue of livertransplantation. Two of the 13 surviving transplant re-cipients were electively transplanted with good liver func-tion (serum bilirubin levels of 1.5 and 2.7 mg%, respec-tively). Nine patients who are alive with their native livershave mild to moderate hepatic disease, and transplanta-tion has been recommended. The remaining 35 childrenare well, anicteric, in normal growth and developmentpercentiles for age, and lead ordinary lives. Follow-up is1 to 16 years (average, 85.8 months).

Survival rate was partially a function ofage at operation(median, 66 days). Long-term survival was 46% in those

BILIARY ATRESIA-DENVER100

-J

n

80

60

40

20

0

0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15

YEARS

FIG. 4. Survival curves of 131 infants with biliary atresia. None of thesix patients without reconstructive surgery lived 3 years. Three-year sur-vival was 6.7% in those who failed to drain bile after operation. Three-year survival of infants achieving bile drainage after operation was 62.3%;current survival is 45.3%.

operated on when younger than the median age and about24% when older than the median. Survival rate improvedover the 15-year study period; the 3-year survival rate(twice the average length of survival for untreated biliaryatresia) was 48.6% for the 5-year period 1973 to 1977,59.6% between 1978 and 1982, and 65% between 1983and 1988.

Discussion

The fundamental aim ofall operations for biliary atresiais the establishment of a permanent biliary fistula. Thiswas the objective of most of the surgical procedures de-vised in the past in which the liver was subjected to im-palement,'2 valvulotomy,'3 partial amputation,'4 amongothers, in surgical attempts to open intrahepatic bile ducts.Simultaneous or subsequent apposition of the intestineoccasionally led to a bilioenteric fistula. Therefore theconcept of Kasai's operation in which the extrahepaticbile ducts were totally excised and the bowel anastomosedto the transected duct stump was not revolutionary, butit did provide for a less abusive, more sensible surgicalapproach.The discovery that biliary atresia was not a static, con-

genital malformation but a progressive, panductular,obliterative disease process was indeed revolutionary. Aspostnatal obliteration ofthe extrahepatic ducts progressed,there was loss of its intrahepatic ductal communication.Ifduct excision was done before loss was complete (about4 months), bile drainage into the coapted intestinal lumen(or gallbladder) could be anticipated.Our concept of the sequence of postsurgical events is

that after hilar duct transection (1) small amounts of biledrain from the open ends of the microscopic communi-cating ducts; (2) this anastomosing plexus of communi-cating ducts"' regresses until the major intrahepatic ductsare breached; and (3) an autoanastomosis occurs betweenmajor intrahepatic duct epithelium and the coapted in-testinal mucosa, a process that takes about 6 weeks. Sup-port for this contention is found in the routine postop-erative observation ofa hilar divot at the duct transectionsite, late postsurgical transhepatic or retrograde cholan-giographic demonstration of only two or three major in-trahepatic ducts draining into the conduit (Fig. 1), andexperimental evidence of a gradual spontaneous biliary-intestinal union.'6"7

This is not simply of hypothetical interest, but ratherclarifies the danger infants are in during the early post-operative period because of the precarious nature of biledrainage. The transected microscopic ducts are prone toinflammatory closure. If the bilioenteric conduit is exter-iorized, this closure is manifest by a precipitous declinein bile output (both in quantity and quality), an event

292 Ann. Surg. * September 1989

THE SURGERY OF BILIARY ATRESIA

occurring in about one third of our patients. Immediateinstitution of steroid therapy often reverses biliary shut-down (Fig. 3) presumably by its anti-inflammatory action.The liability remains until an epithelial-mucosal anas-

tomosis takes place. If diminution of bile flow proceedsto complete cessation, the chances for re-establishmentof biliary drainage by prompt reoperation are good (14of 18 patients in this series) because ducts immediatelyproximal to those occluded are surgically opened. Thus,one of the major advantages of conduit exteriorization isthe immediate recognition ofimpending operative failureand its abortion by summary medical and surgical ma-

neuvers. However, the penalty for exteriorization is even-tual stomal variceal hemorrhage and, consequently, theconduit should be internalized when bile flow reaches a

steady state, 6 to 12 weeks after operation.The ongoing inflammatory process of biliary atresia is

also grossly manifest at the time of initial operation. Theextrahepatic bile ducts are intensely inflamed, whichmakes portal dissection difficult and hazardous. In olderinfants, after obliteration is complete, inflammation isabsent, the operation is easier but the failure rate is high.In fact, the difficulty of the operation is directly propor-

tional to its chance of success.

The hilar component of the operation is identical forall three types of extrahepatic biliary atresia: (1) correct-able, (2) extant gallbladder-common bile duct, or (3) to-tally sclerotic. In the former, anastomosis of intestine tothe blind-ending bile cyst is almost always doomed to failbecause the cyst is scar tissue unlined by epithelium. Inthe second, the gallbladder conduit, the hypoplastic cysticduct-common bile duct may not be able immediately totransmit the full volume of biliary drainage. Decompres-sion by a temporary catheter cholecystostomy (Fig. 1)permits anastomotic healing and gradual dilation of thedistal ducts.2The severity of the intrahepatic bile duct involvement

differs considerably, even in infants of the same age,

thereby accounting for the variability ofthe clinical course.

To some extent, however, the intrahepatic bile ducts are

hypoplastic and stenotic in all infants with biliary atresia.Bile stasis is a consequence and persists for months afteroperation. In conjunction with conduit intestinal flora,both prerequisites for cholangitis exist. Cholangitis afterKasai's operation compounds pre-existing liver damage.Most preventative efforts are directed at reducing bac-

terial contamination. Use of the sterile gallbladder-com-mon bile duct conduit, possible in 10% of cases, virtuallyabolishes the risk of cholangitis. The interposition of a

conduit valve'8"9 also lessens the susceptibility to chol-angitis presumably by decreasing the numbers ofconduitbacteria by the same flow mechanism as the normal il-eocecal valve.

Early Clinical Course

In addition to cholangitis, about 25% of our childrendeveloped clinical manifestations of portal hypertension.Esophageal endosclerosis for variceal hemorrhage9 andsplenic embolization for hypersplenism'0 satisfactorilycontrolled symptoms in our patients. Portal hypertensionmanifestations spontaneously abate after 3 to 4 years be-cause of reduction of portal pressure due to the devel-opment of natural portosystemic shunts or to improvedliver histology.20 Except in a general way, long-term prog-

nosis has not correlated with clinical portal hypertension.Fat-soluble vitamin deficiencies also resolve 1 to 3 years

after operation.

Late Clinical Course

Three years after operation about one third of childrenwith sustained bile drainage continue to have symptomssecondary to residual liver disease. Our patients in thiscategory are either already transplanted or transplantationhas been recommended. The other two thirds of patientssurviving more than 3 years no longer have complicationsof biliary atresia. They are well, anicteric, have flat ab-dominal contours without hepatosplenomegaly, achievenormal growth and development, satisfactorily attendschool (one lettered in football), and endure life adjust-ments as variable as normal for children of their age.

However all patients have some minor aberrations in liverserology (usually liver enzymes), and intrahepatic ducthypoplasia persists on transhepatic hepatic cholangio-grams.

Liver Transplantation

For some time the surgery of biliary atresia has beenone of the most controversial areas in pediatric surgery.The doyen of clinical pediatricians, Dr. Sidney Gellis,2'wrote recently that treatment will be confined to livertransplantation; Dr. Thomas Starzl22 publicly stated thatthe Kasai operation was of only "historic" significance.Meanwhile, Sendai reports steadily improving results;currently, 5-year jaundice-free survival is 53%.23

In our experience the majority of children having por-

toenterostomy procedures ultimately fail the operationand require liver transplantation. Nevertheless, more than25% of all patients are surviving with their native liverswith minimal, if any, stigmata of the disease. It would beclinical larceny to remove the livers of patients in thiscategory and replace them with a homograft. At the mo-ment we know of no method that will accurately predictthis group before operation (although a rough discrimi-nation appears possible as early as 1 month afterwards).24

293Vol. 210 *-No. 3

294 LILLY AND OTHERS Ann. Surg. September 1989

Consequently we firmly believe that whenever feasiblethe Kasai procedure should be the initial operation inpatients with biliary atresia. This policy will protect thosepatients destined to have a near-perfect liver and will fur-nish biliary drainage in most others, thereby sustaininglife, allowing for growth, and improving their chances fora better outcome with transplantation.

References

1. Kasai M, Kimura S, Asakura Y, et al. Surgical treatment of biliaryatresia. J Pediatr Surg 1968; 3:665-675.

2. Lilly JR, Hall RJ, Vasquez J, Shikes RH. Hepatic portocholecys-tostomy: the Denver experience. In Ohi R, ed. Biliary Atresia.Tokyo, Japan: Professional Postgraduate Services, 1987; 173-176.

3. Ohi R, Stellin GP, Shikes RH, Lilly JR. In biliary atresia duct his-tology correlates with bile flow. J Pediatr Surg 1984; 19:467-470.

4. Lilly JR, Altman RP. Hepatic portoenterostomy (the Kasai opera-tion) for biliary atresia. Surgery 1975; 78:76-86.

5. Karrer FM, Lilly JR. Corticosteroid therapy in biliary atresia. J Pe-diatr Surg 1985; 20:593-695.

6. Stellin GP, Uceda JE, Lilly JR. Conduit decompression in biliaryatresia. J Pediatr Surg 1983; 18:782-783.

7. Altman RP, Anderson KD. Surgical management of intractablecholangitis following successful Kasai procedure. J Pediatr Surg1982; 17:894-900.

8. Rothenberg SS, Schroter GJP, Lilly JR. Cholangitis following theKasai operation for biliary atresia. J Pediatr Surg (in press).

9. Lilly JR, Stellin GP. Variceal hemorrhage in biliary atresia. J PediatrSurg 1984; 19:476-479.

10. Brandt CT, Kumpe D, Rothbarth LJ, et al. Splenic embolization inchildren: long-term efficacy. J Pediatr Surg (in press).

11. Sokol RJ. Medical management of an infant or child with chronicliver disease. Sem Liv Dis 1987; 7:155-167.

12. Sterling JA. Artificial bile ducts in the management of congenitalbiliary atresia. J Int Coll Surg 1961; 36:293-296.

13. Potts WJ. The Surgeon and The Child. Philadelphia: WB Saunders,1959.

14. Longmire WP, Sanford MC. Intrahepatic cholangiojejunostomy withpartial hepatectomy for biliary obstruction. Surgery 1948; 24:264-276.

15. Yamamoto K, Fisher MM, Phillips MJ. Hilar biliary plexus in humanliver: a comparative study of the intrahepatic bile ducts in manand animals. Lab Invest 1985; 52:103-106.

16. Touloukian RJ, Barick KW, Vasseur BG, et al. Experimental he-patoportoenterostomy: duct mucosal healing with hybid biliaryintestinal epithelium forming an autoanastomosis. Ped Surg Int(in press).

17. Takemoto H, Tanaka K, Inomata Y, et al. Granulation at the portahepatis following hepatic portoenterostomy for biliary atresia:the healing of experimental hepatoenterostomy. J Pediatr Surg1989; 24:271-275.

18. Okamoto E. Operative techniques for congenital biliary atresia andits long-term results. Jpn J Pediatr Surg 1978; 10:696-706.

19. Freund H, Berlatzky Y, Shiller M. The ileocecal segment: an anti-reflux conduit for hepatic portoenterostomy. J Pediatr Surg 1979;14:169-171.

20. Kasai M, Okamoto A, Ohi R, et al. Changes of portal vein pressureand intrahepatic blood vessels after surgery for biliary atresia. JPediatr Surg 1981; 16:152-159.

21. Gellis S. Pediatric notes. The Weekly Pediatric Commentary 1984;8:43.

22. Starzl TE. Guest Presentation, 19th Annual Meeting, American Pe-diatric Surgical Association, Tucson, Arizona, 1988.

23. Ohi R, Hanamatsu M, Mochizuki I, et al. Progress in the treatmentof biliary atresia. World J Surg 1985; 2:285-293.

24. Vazquez-Estevez JJ, Stewart BA, Shikes RH, et al. Biliary atresia:early determination ofprognosis. J Pediatr Surg 1989; 24:48-51.

DISCUSSION

DR. THOMAS C. MOORE, M.D., PH.D. (Torrance, California): Dr.Lilly has long been a major American Pioneer in the clinical applicationof the Kasai-type approach to the management of biliary atresia in thenewborn, as this impressive report clearly indicates. Many years ago, inthe February 1953 issue of Surgery, Gynecology and Obstetrics, I reportedfrom Indiana University an experience with 31 proved cases of extra-hepatic biliary atresia and suggested the use of a Roux-Y hepatojeju-nostomy for the management of this condition. I now regret in retrospectnot taking my own good advice. A major observation in this 1953 reportwas the rapid advance of biliary cirrhosis in these infants and the absolutenecessity ofearly relief ofbiliary obstruction ifthere was to be any chanceof a successful outcome. Histologic studies of sections of the liver wereobtained in 19 ofthese 31 infants with biliary atresia and showed advancedcirrhosis to be well established by 2 months of age and well underwaywith portal fibrosis and bile duct proliferation in three cases at 4, 4, and6 weeks of age.

In a recent 1987 report to the Pacific Coast Surgical Association, Dr.Timothy Canty of San Diego reported an impressive experience withthe management of biliary atresia by his modification of the Sawaguchiprocedure (a Kasai modification). Seventeen of 18 infants operated onin the first 10 weeks of life were alive and jaundice free 2 and more yearsafter operation (94%). All but two of these patients were 8 weeks of ageor younger at operation and five were in their first month of life. Ofseven patients operated on after 10 weeks of age, a definitive success wasobtained in only one infant (14%). Dr. Lilly has observed the influenceof patient age at operation on the outcome. At what age would yourecommend operation for optimal results?

My preference is for the Suruga II operation, also a Sawaguchi mod-ification. Its principal advantages are no communication whatsoever ofthe biliary conduit with the rest of the intestinal tract and a prolongedhiatus ( 18 months) before restoring intestinal tract communication withthe isolated jejunal biliary conduit to the portahepatis. In a limited ex-perience, our success rate approaches 70%. In two cases there were es-sentially no portahepatis bile ducts visible or on frozen section histologicexamination. Rather than attempt a conduit, only a penrose soft rubberdrain was left at the portahepatis after excision ofthe extrahepatic biliarytract. The drain was exteriorized through a stab wound. In both cases,a substantial drainage of bile at the drain exit site was first observed 6and 7 days after operation. In one ofthese infants, a ring offibro-inflam-matory tissue was found at the portahepatic end of the drain. This firmring greatly facilitated the conduit placement at the portahepatis. Thischild remains jaundice free and healthy 4 years after operation. Has theauthor ever resorted to this temporizing approach?

DR. JAY L. GROSFELD (Indianapiolis, Indiana): Dr. Lilly has been theAmerican leader in the management of these cases and has been a greatproponent of Professor Kasai's procedure.We have recently reviewed our cases, and many of our findings are

similar to Dr. Lilly's. We established a system of postoperative stagingthat could predict outcome. More recently using these criteria we canpredict outcome within 60 days of the Kasai procedure. If you took allcomers in our series of 66 cases, we have exactly the same success rateas Dr. Lilly-about 25%. However ifyou just looked at those infants whowere less than 3 months ofage at operation, more than 30% are successful.