ANNALS OF SURGERY

Vol. 186

November

1977

No.

5

Surgical Progress

Surgical Jaundice in Infancy JOHN R. LILLY, M.D.

The surgery of congenital malformations of the biliary system has undergone a revolutionary change during the past decade. Although Kasai's hepatic portoenterostomy operation for biliary atresia is the most dramatic example, radical departures from conventional surgical treatment also have been advocated for choledochal cyst, Caroli's disease, congenital perforation of the bile ducts and biliary hypoplasia. This communication describes the lesions responsible for obstructive jaundice in the early months of life, briefly reviews standard treatment and consolidates the available information about the new operative procedures currently proposed for congenital anomalies of the biliary tract.

T HE HOST OF CONDITIONS which may be responsible for jaundice in the first weeks of life can be

simply categorized into hematologic, enzymatic/metabolic, infectious and obstructive. Diagnostic tests are definitive, and operation has nothing to offer, for those conditions in the first three categories. On the other hand, there are no reliable studies which will differentiate mechanical obstruction of the extrahepatic ductal system from the hepatocellular cholestasis of neonatal hepatitis. Even liver biopsy is liable to significant misinterpretation. Consequently, if hematologic, metabolic and infectious investigations are norReprint requests: John R. Lilly, M.D., Department of Surgery, University of Colorado School of Medicine, 4200 East Ninth Avenue, Denver, Colorado, 80262. Supported in part by MRIS 8118-01 and 7227-01 from the Veterans Administration; by Grant #s AM17260 and AM-07772 from the National Institutes of Health; and by Grant #s RR-00051 and RR-00069 from the General Clinical Research Centers Program of the Division of Research Resources, National Institutes of Health. Submitted for publication June 21, 1977.

549

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

mal, operative cholangiography is the next logical step in the diagnostic work-up. Postponement of cholangiography at this stage, as was previously advocated, jeopardizes the chances for a successful operative outcome in patients with mechanical obstruction in addition to increasing the likelihood of irreversible hepatic damage. Moreover, current information indicated morbidity is minimal from operative cholangiography in patients with neonatal hepatitis.26 Operative cholangiography is performed through a minilaparotomy incision. Dilute contrast material is injected through a cholecystostomy catheter to fill both the intra and extrahepatic ductal system. If the cholangiogram fails to demonstrate reflux into the intrahepatic bile ducts, the distal common duct should be temporarily occluded with a non-crushing clamp and the study repeated. In patients with biliary atresia, the gallbladder is generally nonpatent. Under these circumstances, the incision is extended and a careful exploration of the porta hepatis undertaken. The specific lesions found at operation are: biliary atresia (correctable and noncorrectable); biliary hypoplasia; choledochal cyst; congenital dilatation of the intrahepatic bile ducts (Carolis' disease); inspissated bile plug; and congenital perforation of the common bile duct (Table 1). Biliary Atresia Biliary atresia has been traditionally classified into "correctable" and "noncorrectable" types. In the cor-

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TABLE 1. Surgical Jaundice in Infancy Disease Entities

I. 2. 3. 4. 5. 6.

Biliary atresia " correctable" " noncorrectable' " Biliary hypoplasia Inspissated bile plug Choledochal cyst Caroli's disease Perforation of the extrahepatic bile ducts

rectable type, the proximal bile ducts are patent but end blindly before the duodenum (Fig. 1). Theoretically, this kind of anatomy is susceptible to total surgical correction. In noncorrectable biliary atresia, the lumen of the proximal extrahepatic bile duct is obliterated and in approximately 90Wo of cases the obliteration extends throughout the entire extrahepatic ductal system. In the remaining 10%, the gallbladder, cystic duct and distal common duct are still patent though often diminutive in caliber. The ratio of correctable to noncorrectable cases is about 1:10.

Correctable Biliary Atresia Ladd25 in 1928 reported operative success in two infants with correctable biliary atresia. He achieved internal biliary drainage by anastomosis of the blindending common hepatic duct to the duodenum in one patient and by cholecystgastrostomy in another. Anastomosis of the patent part of the bile duct or of the gallbladder to the duodenum was also advocated by Gross13 with the admonition that the anastomosis be stented over a catheter. Currently, most surgeons prefer a Roux-en-Y jejunal anastomosis to the common hepatic duct. It is believed that the defunctionalized jejunum will lessen the incidence of ascending cholangitis. This belief has not been fully substantiated. Whether from recurrent cholangitis, the sequellae of preoperative biliary obstruction or coexisting intrahepatic ductular disease (akin to that present in noncorrectable cases), many patients with correctable biliary atresia have permanent liver damage despite restoration of bile flow. A recent, comprehensive survey reported long term survival in only half of the afflicted patients.17 Noncorrectable Biliary Atresia The former surgical philosophy for this type of biliary atresia is revealed by its prefix. The operative finding of nonpatent extrahepatic bile ducts was considered hopeless and the patient was consigned to an early death. A number of imaginative surgical procedures were proposed over the years but their success was inconsistent and usually limited to the author. The radical new approach by Kasai consisted

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of resection of the entire ductal system and anastomosis of the proximal transected common hepatic duct to the intestine. Kasai's20 initial English communication in 1968 in which bile drainage was reported in a significant percentage of cases was met with surprise, disbelief, and occasionally hostility. The latter reaction was particularly incomprehensible since aside from liver transplantation, no alternative therapy was available. Subsequently the success of Kasai's hepatic portoenterostomy operation has been confirmed by surgeons throughout the world.5'9'30'36 The basis for biliary drainage with Kasai's procedure is the presence of miniscule biliary ductules in the scarred "nonpatent" extrahepatic bile duct (Fig. 2). These ductules communicate with the intrahepatic biliary system and when surgically transected, drain bile from the liver into the interposed bowel. Of prime importance is the timing of the operation as the presence of these communicating ductules is definitely a function of age. Thus, Kasai (personal communication, 1977) reported biliary drainage in 88% of patients having operation under 60 days of age; 20%o from 90-120 days of age and total failure in patients over four months old. The technical details of hepatic portoenterostomy

FIG. 1. Operative findings in "correctable" biliary atresia. The dilated, bulbous end of the common hepatic bile duct (arrow) should be opened and anastomosed to a Roux-en-Y jejunojejunostomy (choledochojejunostomy). In this case, the gallbladder (+) does not communicate with the patent part of the biliary ductal system. (Courtesy of Dr. J. G. Randolph)

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have been previously described.'19 The essential components of the operation are: 1) a high transection of the common hepatic duct at the liver hilus, 2) anastomosis of the stump of the amputated duct to the defunctionalized end of a Roux-en-Y jejunojejunostomy (Fig. 3), 3) exteriorization of the biliary-jejunal conduit (Fig. 4). Postoperative cholangitis has been a major complication in all reported series and undoubtedly contributes to permanent liver damage. Venting of the bilio-enteric conduit appears to lessen the severity of the complication by preventing reflux of intestinal contents to the anastomotic site at the liver. The susceptibility to cholangitis continues as long as partial obstruction of the biliary system exists. Therefore, take down of the exteriorized conduit with reestablishment of direct biliary-intestinal continuity should not be done for at least two to three years after operation.

FIG.

2.

Photomicrograph of the

common

hepatic bile duct

at the

site of transection at the liver hilus in an infant with "noncorrectable" biliarv atresia. The biliary ductules communicate with the intrahepatic biliarv tree and are presumed to act as conduits

for bile after the Kasai procedure. (H and E. original magnification x

120).

551

vein

FIG. 3. Drawing of the bilio-enteric anastomosis in the hepatic portoenterostomy operation. Interrupted fine silk sutures are used to join the open end of the jejunum to the transected bile duct at the liver hilus.

Postoperative cholangitis can be avoided in those 10% of patients whose distal extrahepatic biliary system remains patent. Anastomosis of the stump of the transected common hepatic duct directly to the gallbladder permits bile to flow from the liver into the gallbladder thence through the cystic and common ducts to the duodenum (Fig. 5).19,23,2716 This type of reconstruction should be done preferentially whenever natural biliary structures are available for anastomosis. The results of hepatic portoenterostomy in the Denver series are shown in Table 2. Sustained bile drainage was obtained in 15 of 28 patients (54%). Exclusion of patients over four months old at operation (in whom failure invariably occurred) raises the success rate to 60%. Achievement of bile drainage, however, did not equate with cure nor even necessarily to longevity. Despite relief of extrahepatic biliary obstruction as evidenced by loss of jaundice, progressive excretion of biliary pigments and lipids in bile with concomitant decrease in elevated serum levels,3' five of 15 patients developed manifestations of progressive intrahepatic disease. This subgroup had increasing hepatosplenomegaly and ascites and after three to five months bile output began to decrease in quantity and quality. Four of these children died in hepatic failure within the first postoperative year. In the majority of patients with sustained bile drainage, however, the intrahepatic component of the disease appeared to stabilize in about one year. Furthermore, three of ten children in our series had marked improvement in liver histology in biopsies done one and one half to two years after operation.

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1977

transected bile duct

-gall bladder

common

iL

FIG. 4. Drawing of the completed hepatic portoenterostomy operation. The defunctionalized limb of a Roux-en-Y jejunojejunostomy is exteriorized as a Mikulicz anastomosis in its mid portion. A crushing clamp is applied to the Mikulicz spur six months after operation which converts the double barraled enterostomy to a single barreled stoma. Subsequently bile may flow directly into the gastro-intestional tract.

hepatic duct

common bile duct

FIG. 5. Drawing of hepatic portocholecystostomy. The gallbladder is opened and anastomosed to the transected bile duct at the liver hilus. Bile flows from the liver into the gallbladder, thence through the cystic and common bile ducts to the duodenum. The artist has drawn the transected bile duct much too long. The transection of the duct should be at the liver hilus as shown in Figure 3.

back to 1963 was 28%.41 The longest surviving patient having liver replacement for biliary atresia is now seven and one half years since transplantation. Liver transplantation and the Kasai procedure are not competitive operations, since, to be effective, hepatic portoenterostomy must be done in the first months of life when liver replacement is rarely possible. Furthermore, the previously constructed Rouxen-Y bilio-enteric conduit may be utilized for biliary drainage of the transplanted liver. cases

Parenthetically, one of these children is in his fifth postoperative year and appears to be cured. In addition, Alagille (personal communication, 1977) reported spontaneous disappearance of esophageal varices in five chronically surviving patients and Kasai22 found continuing improvement in liver histopathology in patients five to ten years after operation. Nevertheless, most patients with "successful" hepatic portoenterostomy operations are left with varying degrees of permanent liver damage. The extent to which these children will be able to carry on a normal life is as yet unknown. Liver replacement is a feasible procedure for children with biliary atresia in whom hepatic portoenterostomy fails or there is evidence of progressive disease despite relief of biliary obstruction. The results of liver transplantation reported by Starzl et al.4A have steadily improved since the inception of the program in 1963. More than half of the last 25 recipients have survived for at least one year. Although a high incidence of associated perihepatic anomalies31 complicates the transplant operation in children with biliary atresia, the overall one year survival including all

Biliary Hypoplasia (Fig. 6) Biliary hypoplasia is not a discrete clinical entity. It is a description of an operative or radiographic TABLE 2. Clinical Results of Hepatic Portoenterostomy

Total Experience

28 Patients

Absent or temporary bile drainage Sustained bile drainage Progressive Disease

13* 15 (54%) 5 (4 dead in 1 year) 10

Stable (7) or Improved (3) *

3 patients 4 months at operation.

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FIG. 6. Operative cholangiogram in a patient with biliary hypoplasia. There is complete patency of the extrahepatic biliary tract but the calibre is diminutive throughout. Continuity is demonstrated between the intrahepatic biliary system and the duodenum. The final diagnosis in this case was neonatal hepatitis but almost identical cholangiographic findings have been seen in patients with alpha,-antitrypsin deficiency, intrahepatic biliary atresia, choledochal cyst and in patients who subsequently developed classic biliary atresia.

finding which has been found in a variety of hepatobiliary disorders (Table 3). The concept of a multidisease origin was not appreciated in the past and until recently biliary hypoplasia was felt to represent a mild form of biliary atresia. Clinical reviews reported early death in some patients, prolonged survival in many, and amelioration of jaundice in a few.'1324'34 The reason for the unpredictable and variable clinical course was the multiplicity of diseases being reported. For example, a personal experience was recently reported in which biliary hypoplasia was found in chance association in patients with neonatal hepatitis, alpha,-antitrypsin deficiency, choledochal cyst and biliary atresia.28 The prognosis in these patients was related to their primary disease. Operative flushing, stenting and bypass of the hypoplastic ductal system has been attempted with mediocre results. The surgery of biliary hypoplasia obviously depends on the fundamental disease process. At the moment, operation has no place in the treatment of patients with intrinsic liver disease (alpha, antitrypsin deficiency, paucity of the intrahepatic bile ducts, neonatal hepatitis). Indeed, circumstantial evidence suggests that the hypoplastic extrahepatic ductal system is a secondary phenomenon and not directly responsible for jaundice in these infants. In one of my patients with alpha,-antitrypsin deficiency for in-

stance, the extrahepatic biliary system was markedly diminutive even though jaundice had completely resolved several months earlier. If the underlying disease in patients with biliary hypoplasia is obstructive (biliary atresia, choledochal cyst) operation may eventually prove efficacious. Since most evidence indicates biliary atresia is a progressive disease, operative cholangiography done early in its course may demonstrate patent, albeit small, extrahepatic bile ducts. Subsequently, total ductular obliteration occurs. This precise sequence of events has been reported in several patients ultimately proven to' have biliary atresia and in an infant with a choledochal cyst.'4"16'28 Consequently, reexploration in four to six weeks should be considered in patients with biliary hypoplasia in whom liver histology is nondefinitive and jaundice progressive. If total ductular obliteration has occurred, hepatic portoenterostomy should be done. TABLE 3. Primary Disease in Biliary Hypoplasia 1. 2. 3. 4. 5.

Extrahepatic biliary atresia Choledochal cyst Neonatal hepatitis Alpha1-antitrypsin deficiency Intrahepatic biliary atresia

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COMMON HEPATIC DUCT1,,-' e Oft/ ~~~I A '

00#0

,,

I

If-

de/ t

,oIr-

I

CHOLEDOCHAL CYST

I

It

I

I I

COMMON B ILE DUCT

FIG. 7. Total operative excision of the choleodchal cyst (and the attached gallbladder) is the surgical procedure of choice. The distal common bile duct is suture-ligated. The transected normal common hepatic duct is anastomosed to the defunctionalized limb of a jejunojejunostomy (Roux-en-Y).

Choledochal Cyst (Fig. 7) Choledochal cyst is a congenital malformation of the extrahepatic bile ducts characterized by 1) gross dilatation of the common bile duct, 2) partial obstruction of the distal common bile duct, and 3) a normal proximal common hepatic duct and intrahepatic biliary tree. Pain, mass, and jaundice are the typical triad of symptoms found in older children. In infants, jaundice is often the only sign and thus the condition may be indistinguishable from other causes of obstructive jaundice in the neonatal period. Liver biopsy is not diagnostic.6 Ultrasound echography, however, will often detect the cyst even in this young age group. In the United States, conventional treatment of choledochal cysts has been by internal biliary drainage of the cyst into the duodenum or jejunum. The surgical procedures are plagued postoperatively by ascending cholangitis and by anastomotic stricture. Most choledochal cysts have no mucosal lining and conse-

Ann. Surg. * November 1977

quently a mucosa-to-mucosa anastomosis with intestine is impossible. Under these circumstances, progressive stricture of the anastomosis may be anticipated. Furthermore, the wall of the choledochal cyst is pathologic consisting for the most part of fibrotic scar tissue. The cyst usually persists, therefore, in spite of intestinal decompression thereby serving as a stagnant reservoir for bacterial overgrowth and predisposing to recurrent cholangitis. Biliary lithiasis40 and malignancy12 are additional complications of standard cyst-draining types of operation. For these reasons, total excision of choledochal cyst is the surgical procedure of choice. Exceptions to primary resection are patients with acute, poorly controlled cholangitis or severe, potentially reversible liver damage. In these patients preliminary intestinal drainage of the cyst should be done and excision of the choledochal cyst performed when the unfavorable circumstances are reversed. Most patients with choledochal cyst, however, are candidates for primary excision. The previously reported high operative mortality rate for total cyst excision caused many American surgeons to shy away from the procedure. In a 1975 collective review,12 however, the mortality rates for cyst excision and cyst drainage (Roux-en-Y cholecystjejunostomy) operations were not significantly different, seven and 17% respectively. Resection of the choledochal cyst and end-to-end anastomosis of the common hepatic and common bile ducts (choledochocholedochostomy) has been reported,7'39 but because of distance disparity, is rarely possible. Moreover, partial obstruction of the distal common bile duct is common in patients with choledochal cyst which would mililate against the success of this kind of direct biliary reconstruction. Consequently, after excision, biliary-enteric drainage by anastomosis of the common hepatic duct to a Roux-en-Y jejunojejunostomy is usually advocated. The technical aspects of excision and Roux-en-Y choledochojejunostomy have been described by Kasai and colleagues,21 Jones and coworkers,18 and Saito and Ishida.38 These workers performed external dissection, mobilization and resection of the choledochal cyst but point out the potential danger to the adjacent vascular structures in the portal triad during operation. The portal vein and hepatic artery are often encased in inflamatory tissue secondary to chronic biliary obstruction and recurrent cholangitis, difficult to identify and displaced by the cyst from their normal anatomic location. The technical problem is compounded if a previous cyst drainage procedure has been performed. To minimize the danger of inadvertent vascular injury, excision of the last four personal cases of

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choledochal cyst was carried out from the inside of the cyst.29 In this procedure the cyst is opened transversely across its entire anterior, lateral and medial aspects leaving the posterior wall (adjacent to the portal vein and hepatic artery) intact. An arbitrary plane ofdissection is selected in the remaining posterior wall which separates it into two layers. Incision of the separated inner layer permits the cyst to be stripped from the residual posterior wall by blunt dissection. The choledochal cyst is then totally resected except for the thin shell of the posterior wall still adherent to the vessels. In infants with uncomplicated choledochal cysts, minor degrees of hepatic injury from preexisting biliary obstruction may be expected to return to normal. However, after some point in time, liver damage becomes irreversible and in patients with delayed diagnosis, an adverse long term prognosis has been reported.1 15,35'44 Prognosis is also guarded in 1) patients with choledochal cyst and complete biliary obstruction and 2) in patients with coexisting cystic involvement of the intrahepatic biliary system. The former situation may well represent a form of biliary atresia and be subject, therefore, to the progressive hepatic changes of the parent disease. Kasai and colleagues21 reported early death in five of eight patients with choledochal cyst and complete biliary obstruction. In the latter patients with coexisting cystic involvement of the intrahepatic biliary system, biliary stasis may persist in spite of remedial surgery of extrahepatic disease. If the intrahepatic lesion is extensive, the condition is generally termed Caroli's disease. Caroli's Disease In 1958 Caroli8 described a congenital anomaly of the biliary tree characterized by multiple cystic malformations of the intrahepatic bile ducts (congenital dilatation of the intrahepatic bile ducts). Although the lesion reported by Caroli specifically involved only major segmental bile ducts, the term "Caroli's disease" has been popularly applied to many types of congenital dilatation of the intrahepatic bile ducts. True Caroli's disease is rare, often associated with congenital hepatic fibrosis and cystic disease of the kidney and usually presents as cholangitis in older children and adults. In infants, almost all cases of congenital dilatation of the intrahepatic bile ducts appear to be part of the disease spectrum of choledochal cyst. That

is, cystic dilatation of the intrahepatic bile ducts is seen in conjunction with varying degrees of dilatation of the common bile duct (Fig. 8). The intrahepatic component of the disease may be as cylindrical, saccular or fusiform cysts, and is frequently

FIG. 8. Transhepatic cholangiogram in a patient with congenital dilatation of the intrahepatic bile ducts. One of the intrahepatic biliary cysts (arrow) has been filled with contrast material by the needle injection. Subsequent drainage into the dilated extrahepatic biliary system is shown. (Courtesy of Dr. D. C. Hitch).

asymmetrical, involving predominantly one major intrahepatic branch. The latter finding has therapeutic as well as prognostic implications The incidence of coexisting intrahepatic disease in patients with choledochal cyst is probably considerably higher than was previously appreciated. In 16 patients with choleodchal cysts reported by Tsuchida and Ishida43 operative cholangiography uncovered conspicuous cystic dilatation of the intrahepatic bile ducts in nine instances. Infants with this type of Caroli's disease (cystic involvement of both the

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Ann. Surg. * November 1977

If the intrahepatic cystic disease is limited to a single intralobar bile duct, hepatic lobectomy or segmentectomy is probably the procedure of choice. In a recent collective review (in which most of the patients were adults), Watts and others45 reported that major hepatic resection was performed on six occasions for unilobar disease. Both patients having lobectomy as the primary procedure recovered well. There was one death in the four patients subjected to lobectomy after various other operations had proven unsuccessful; the other three patients apparently did well.

....~~~~~~~~~~~~~~~~~~~~... . . . . . . . FIG. 9. Operative cholangiogram in a patient with congenital perforation of the common bile duct. The biliary pseudocyst (arrow) should not be confused with a choledochal cyst nor should failure of contrast media to enter the duodenum necessarily be interpreted as distal common bile duct obstruction (Reprinted with permission of Surgery, 75:664, 1974).

intra- and extrahepatic bile ducts) may present with jaundice in the newborn period and have a clinical picture indistinguishable from that found in patients with a simple choledochal cyst. Definitive surgery for most patients with congenital dilatation of the intrahepatic bile ducts is impossible since adequate drainage of intrahepatic cysts is difficult to achieve by intestinal decompression of the extrahepatic biliary system. At the moment, total resection of the extrahepatic bile ducts with Roux-enY jejunal anastomosis to the common hepatic duct at the liver hilus appears to provide the best chance for unimpeded bile flow. All internal drainage procedures, however, leave intrahepatic disease behind and recurrent cholangitis, biliary lithiasis, and hepatic abscess have been frequent postoperative complications.45 Malignant degeneration has also been reported. 12,45

Inspissated Bile Plug In infants with inspissated bile plug, there is actual mechanical obstruction of the extrahepatic bile ducts by inspissated bile. The condition should not be confused with the "inspissated bile syndrome" which was a misnomer in the 1940s for a common sequella of erythroblastosis fetalis. Jaundice is infants with the inspissated bile syndrome is usually not obstructive but hepatocellular in origin1' and, in most instances, resolves spontaneously. In exceptional cases of massive hemolysis from Rh or ABO blood group incompatibility, however, resolution of hemolytic jaundice may be followed by biliary obstruction. Obstruction is a secondary phenomenon as a consequence of the earlier excessive bile pigment excretory load. The bile becomes inspissated resulting in mechanical bile duct obstruction. Inspissation may proceed to the production of bile pigment stones.3 The background of earlier hemolysis is the major clue to diagnosis of inspissated bile plug although a few cases have been reported in which an hemolytic antecedent was absent.4 Liver biopsy, as has been so often noted in this monograph, is nondefinitive and operative cholangiography is required for diagnosis. In most instances, simple irrigation of the extrahepatic bile ducts is curative. In patients with authentic choledocholithiasis, manual extraction of biliary stones may be necessary. Duodenotomy and sphincterotomy for extraction of the calculus was required in a personal case. Inspissated bile plug is an infrequent cause of obstructive jaundice, at present, due to early diagnosis of blood group incompatibility and prompt exchange transfusion.

Congenital Perforation of the Common Bile Duct

Congenital perforation of the common bile duct, though uncommon, occurs with sufficient frequency to warrant consideration in the differential diagnosis of jaundice in the early months of life. The almost

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uniform location of the perforation at the junction of the common bile and cystic ducts is strong circumstantial evidence of a congenital origin. The perforation is most often pinhole in size, consequently, bile extravasation is gradual permitting the formation of a biliary pseudocyst. The usual clinical manifestation of the disease is jaundice which becomes apparent during the first to third month of life. Systemic signs may be minimal and thus the infant easily blends into the diagnostic mix of surgical jaundice in infancy. Operative cholangiography demonstrates the perforation in the common bile duct and often the biliary pseudocyst as well (Fig. 9). Misinterpretation of the latter as a choledochal cyst may be disastrous if an intestinal anastomosis is done to the pseudocyst. In addition, since contrast material readily spills out through the ductal perforation, the cholangiogram frequently fails to fill the distal common duct and duodenum, thereby suggesting a mechanical obstruction. Common duct dilatation, sphincterotomy, T-tube drainage and choledochoenterostomy37 have been performed in what were probably needless surgical measures to overcome a phantom distal obstruction. In two recent collective reviews,33'37 it was apparent that simple peritoneal drainage of the area of the ductal perforation was sufficient operative treatment in the majority of cases. Peritoneal drainage was followed by spontaneous closure of the perforation, resolution of the biliary pseudocyst and cure of the "mechanical obstruction" (probably bile sludge) of the distal common duct. Retention of the cholecystostomy catheter is a helpful surgical adjunct since it may be utilized for postoperative cholangiography. 10.33 Abdominal drains should not be removed until sealing of the ductal perforation is radiographically demonstrated. Since the perforation of the common duct appears to be the only fault in biliary embryogenesis, the long term prognosis is excellent.

per cent of patients in whom the gallbladder and the distal biliary tree are still patent, hepatic portocholecystostomy. 3) Billary hypoplasia-Hepatic portoenterostomy for patients in whom hypoplasia progresses to biliary atresia. 4) Choledochal cyst-Total excision and choledochojejunostomy (Roux-en-Y). 5) Congenital dilatation of the intrahepatic bile ducts (Caroli's disease)-Resection of the dilated portion of the extra-hepatic bile duct and Roux-en-Y choledochojejunostomy. Hepatic resection for patients in whom the intrahepatic cystic disease is limited to a single segment or lobe. 6) Inspissated bile plug-Irrigation of the extrahepatic bile ducts and in cases of choledocholithiasis, manual extraction of biliary calculi. 7) Congenital perforation of the common bile duct-Simple peritoneal drainage of the site of ductal perforation and cholecystostomy. The majority of these operations have not yet become standard surgical practice. Enough experience has been gained with each, however, to justify their legitimate consideration in the surgeon's selection of operation for patients with congenital malformations of the biliary tract.

Conclusions Physicians are now aware of the pernicious consequences of unresolved biliary obstruction and thus are referring infants with inexplicable jaundice for surgical exploration at an earlier age than at any time in the past. The surgeon undertaking the care of these patients has an unparalleled opportunity to effect a lifelong cure and should be knowledgable of the new developments taking place in this field. The specific operations currently being advocated for congenital malformations of the biliary tract are: 1) "Correctable" biliary atresia-Roux-en-Y choledochojejunostomy. 2) "Noncorrectable" biliary atresiaKasai's hepatic portoenterostomy operation. In the ten

8.

References 1. Alonso-Lej, F., Rever, W. B. and Pessagno, D. J.: Congenital Choledochal Cyst, with a report of 2, and an Analysis of 94, Cases. Int. Abstr. Surg., 108:1, 1959. 2. Altman, R. P. and Lilly, J. R.: Technical Details in the

3. 4. 5. 6. 7.

9.

Surgical Correction of Extrahepatic Biliary Atresia. Surg. Gynecol. Obstet., 140:952, 1975. Benson, C. D., Lofti, M. W. and Hertzler, J. H.: Surgical Aspects of Biliary Tract Disease in the Infant and Child. Tr. West. Surg. A., 1966. Bernstein, J., Braylan, R. and Brough, A. J.: Bile-plug Syndrome: A Correctable Cause of Obstructive Jaundice in Infants. Pediatrics, 43:273, 1969. Bill, A. H., Brennom, W. S., and Huseby, T. L.: Biliary Atresia. New Concepts of Pathology, Diagnosis, and Management. Arch. Surg., 109:367, 1974. Brough, A. J. and Bernstein, J.: Liver Biopsy in the Diagnosis of Infantile Obstructive Jaundice. Pediatrics, 43: 519, 1969. Cahlin, E., Lundholm, K., Sahlin, O., et al.: Choledochal Cyst. A Case Operated with Excision and Anatomical Reconstruction. Acta. Chir. Scand., 140:161, 1974. Caroli, J., Soupalt, R., Kossakowski, J., et al.: La Dilatation Polykystique Congenitale des Voies Biliaires Intraheaptiques. Essai de classification. Sem. Hop. Paris, 34:488, 1958. Danks, D. M., Campbell, P. E., Clarke, A. M., et al.: Extrahepatic Biliary Atresia. Am. J. Dis. Child., 128:684,

1974. 10. Dinner, M.: Biliary Peritonitis Due to Idiopathic Perforation of the Common Bile Duct. S. Afr. J. Surg., 13:207, 1975. 11. Dunn, P.: Obstructive Jaundice and Haemolytic Disease of the Newborn. Arch. Dis. Child., 38:596, 1963. 12. Flanigan, D. P.: Biliary Cysts. Ann. Surg., 182:635, 1975. 13. Gross, R. E.: The Surgery of Infancy and Childhood. Philadelphia, W. B. Saunders Company, 1953. 14. Hays, D. M., Woolley, M. M., Snyder, W. H.: Diagnosis of Biliary Atresia: Relative Accuracy of Percutaneous Liver Biopsy, Open Liver Biopsy, and Operative Cholangiography. J. Pediatr., 71:598, 1967. 15. Hays, D. M., Goodman, G. N., Synder, W. H., et al.:

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17. 18. 19. 20.

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25. 26.

27. 28. 29. 30.

31. 32. 33.

34.

35. 36. 37.

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Congenital Cystic Dilatation of the Common Bile Duct. Arch. Surg., 98:457, 1969. Holder, T. M.: Atresia of the Extrahepatic Bile Duct. Am. J. Surg., 107:458, 1964 Izant, R. J., Jr., Akers, D. R., Hays, D. M., et al.: Biliary Atresia Survey. Surgery Section, Am. Acad. Pediatr., 1965. Jones, P. G., Smith, D. E., Clarke, A. M. and Kent, M.: Choledochal Cysts: Experience with Radical Excision. J. Pediatr. Surg., 6:112, 1971. Kasai, M.: Treatment of Biliary Atresia with Special Reference to Hepatic Porto-enterostomy and its Modifications. Prog. Pediatr. Surg., 6:5, 1974. Kasai, M., Kimura, S., Asakura, Y., et al.: Surgical Treatment of Biliary Atresia. J. Pediatr. Surg., 3:665, 1968. Kasai, M., Asakura, Y. and Taira, Y.: Surgical Treatment of Choledochal Cyst. Ann. Surg., 182:844, 1970. Kasai, M., Watanabe, I. and Ohi, R.: Follow-up Studies of Long Term Survivors after Hepatic Portoenterostomy for "Noncorrectable" Biliary Atresia. J. Pediatr. Surg., 10:173, 1975. Kimura, S., Kumagai, Y., and Uchiyama, T.: Hope for Repair of Biliary Atresia. Z. Kinderchir., 14:190, 1974. Krant, S. M. and Swenson, O.: Biliary Duct Hypoplasia. J. Pediatr. Surg., 8:301, 1973. Ladd, W. E.: Congenital Atresia and Stenosis of the Bile Ducts. JAMA, 91:1082, 1928. Lawson, E. E. and Boggs, J. D.: Long-term Follow-up of Neonatal Hepatitis. Pediatrics, 53:650, 1974. Lilly, J. R.: The Japanese Operation for Biliary Atresia: Remedy or Mischief? Pediatrics, 55:12, 1975. Lilly, J. R.: The Surgery of Biliary Hypoplasia. J. Pediatr. Surg., 11:815, 1976. Lilly, J. R.: Total Excision of Choledochal Cyst. Surg. Gynecol. Obstet, in press. Lilly, J. R. and Altman, R. P.: Hepatic Portoenterostomy (the Kasai Operation) for Biliary Atresia. Surgery, 78:76, 1975. Lilly, J. R. and Chandra, R. S.: Surgical Hazards of Coexisting Anomalies in Biliary Atresia. Surg. Gynecol. Obstet., 139:49, 1974. Lilly, J. R. and Javitt, N. B.: Biliary Lipid Excretion after Hepatic Portoenterostomy. Ann. Surg., 184:369, 1976. Lilly, J. R., Weintraub, W. H. and Altman, R. P.: Spontaneous Perforation of the Extrahepatic Bile Ducts and Bile Peritonitis in Infancy. Surgery, 75:664, 1974. Longmire, W. P.: Congenital Biliary Hypoplasia. Ann. Surg., 159:335, 1964. Longmire, W. P., Mandiola, S. A. and Gordon, H. E.: Congenital Cystic Disease of the Liver and Biliary System. Ann. Surg., 174:711, 1971. Odievre, M., Valayer, J., Razemon-Pinta, M., et al.: Hepatic Portoenterostomy or Cholecystostomy in the Treatment of Extrahepatic Biliary Atresia. J. Pediatr., 88:774, 1976. Prevot, J. and Babut, J. M.: Spontaneous Perforations of the Biliary Tract in Infancy. Prog. Pediatr. Surg., 3:187, 1971. Saito, S. and Ishida, M.: Congenital Choledochal Cyst (Cystic

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1977

Dilatation of the Common Bile Duct). Prog. Pediatr. Surg., 3:604, 1968. Scharli, A. and Bettex, M.: Congenital Cyst: Reconstruction of the Normal Anatomy. J. Pediatr. Surg., 3:604, 1968. Shields, A. B.: Congenital Cystic Dilatation of the Common Bile Duct. Am. J. Surg., 108:142, 1964. Starzl, T. E., Porter, K. A., Putnam, C. W., et al.: Liver Replacement in children. In Berenberg, S. R., (ed.): Liver Diseases in Infancy and Childhood. The Hague, Netherlands, Martinus Nijhoff Medical Division, 1976. Starzl, T. E., Porter, K. A., Putnam, C. W., et al.: Orthotopic Liver Transplantation in Ninety-three Patients. Surg. Gynecol. Obstet., 142:487, 1976. Tsuchida, Y. and Ishida, M.: Dilatation of the Intrahepatic Bile Ducts in Congenital Cystic Dilatation of the Common Bile Duct. Surgery, 69:776, 1971. Valayer, J. and Alagille, D.: Experience with Choledochal Cyst. J. Pediatr. Surg., 10:65, 1975. Watts, D. R., Lorenzo, G. A. and Beal, J. M.: Congenital Dilatation of the Intrahepatic Biliary Ducts. Arch. Surg., 108:592, 1974.

Selected Annotated Bibliography 1. Kasai, M.: Treatment of Biliary Atresia with Special Reference to Hepatic Porto-enterostomy and its Modifications. Prog. Pediatr. Surg. 6:6, 1974. This is an excellent comprehensive

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5.

review of the author's experience with his operation for biliary atresia. The morphological basis for the operation, technical details, postoperative management and long term prognosis are presented. Lilly, J. R. and Javitt, N. B.: Biliary Lipid Excretion after Hepatic Portoenterostomy. Ann. Surg., 184:369, 1976. For a while, relief of jaundice following Kasai's operation was thought due to surgical drainage of perihepatic lymphatics. In this report, analysis of the constituents of postoperative bile drainage document that a functional bilio-enteric anastomosis is created. Flanigan, D. P.: Biliary Cysts. Ann. Surg., 182:635, 1975. In this collective review of choledochal cyst, conclusive evidence is presented supporting total excision of choledochal cyst in preference to internal drainage procedures. Starzl, T. E., Porter, K. A., Putnam, C. W., et al.: Orthotopic Liver Transplantation in Ninety-three Patients. Surg. Gynecol. Obstet., 142:487, 1976. The overall experience with liver transplantation in the Denver series is presented. The best results have been in pediatric recipients. Refinement in operative technique and methods of post transplant management have resulted in improved survival. Watts, D. R., Lorenzo, G. A. and Beal, J. M.: Congenital Dilatation of the Intrahepatic Biliary Ducts. Arch Surg., 108: 592, 1974. This collective review focuses on the short and long term complications associated with different types of operations for congenital dilatation of the intrahepatic bile ducts. The authors conclude that when feasible because of localized disease, hepatic resection is the surgical procedure of choice.