World J. Surg., 2, 571-580, 1978
WbddJournal
Sdrgery
Technique and Results of Operative Management of Biliary Atresia Morio Kasai, M.D., Hiroshi Suzuki, M.D., Eisuke Ohashi, M.D., Ryoji Ohi, M.D., Tsuneo Chiba, M.D., and Atsutake Okamoto, M.D. SecondDepartmentof Surgery,TohokuUniversitySchoolof Medicine,Sendai,Japan
Intrahepatic bile ducts are patent in the liver of patients with biliary atresia in early infancy. Small bile ducts at the porta hepatis continuing to the intrahepatic ducts usually terminate in fibrous tissue replacing the hepatic radicles, which does not extend to the common hepatic duct. Therefore, the level of transection of the fibrous tissue at the porta hepatis is of prime importance for successful hepatic portoenterostomy. Transection should be done at the level of the posterior surface of the portal vein. The overall cure rate of surgery for biliary atresia was 30%. In our recent series, however, the results have markedly improved and the cure rate has been increased to 55%. There are 47 patients living without jaundice, 22 of them for more than 5 years. The longest survivors are 23 years old. All the longterm survivors are leading a normal life for their age, although 6 of them have some physical or mental handicap. Early operation, adequate surgical technique, and prevention of postoperative cholangitis are requisites for successful hepatic portoenterostomy.
sufficiency is not uncommon even in patients who were relieved of jaundice by operation [4-6]. There have been many documented methods [712] for making an intestinal conduit for hepatic portoenterostomy to prevent postoperative ascending cholangitis. It is our present policy to use a doubleY hepatic portojejunostomy for patients having a fibrous common bile duct, and hepatic portocholecystostomy for patients having a patent gallbladder and common bile duct. For success in surgery of biliary atresia, early operation, adequate operative technique, and prevention of postoperative cholangitis are essential. The purpose of this communication is to present the technical details of hepatic portoenterostomy, as well as the normal and pathological anatomy of the porta hepatis, the knowledge of which is essential for a successful operation. Early and long-term results are presented. Finally, the postoperative management is described.
Biliary atresia of the noncorrectable type has become a curable disease by the introduction of hepatic portoenterostomy. Recent reports [1-3] on follow-up studies of survivors after hepatic portoenterostomy indicate that many of the long-term survivors showed normal growth and development. Nevertheless, the results of operation for biliary atresia are still unsatisfactory, since sustained drainage of bile has been restored in only 30-45% of patients, and liver cirrhosis resulting in rupture of esophageal varices, hypersplenism, or hepatic in-
Anatomy
In biliary atresia, extrahepatic bile ducts are usually replaced by fibrous tissue. The tissue from the porta hepatis contains many small bile ducts communicating with the intrahepatic bile ducts. Figure 1 shows a 3-dimensional reconstruction of bile ducts in the fibrous tissue of the porta hepatis taken from a patient with noncorrectable biliary atresia, in whom
Reprint requests: Professor Morio Kasai, The Second Department of Surgery, Tohoku University School of Medicine, Seiryochi, 1-1, Sendai, Japan.
0364-2313/78/0002-0571 $02.00 9 1978 Soci6t6 Internationale de Chirurgie 571
572
World J. Surg. Vol. 2, No. 5, September, 1978
Fig. 1. Three-dimensional reconstruction of bile ducts found in the encircled portion of the extrahepatic bile duct removed from a patient with biliary atresia of type III.
active excretion of bile was achieved by operation. Small bile ducts in continuity with intrahepatic ducts were obliterated within 1,500/z from the proximal end of the resected specimen and did not connect with the common hepatic duct. If the fibrous tissue had been transected 1 or 2 mm on the duodenal side in this patient, few or no ducts draining bile would have been encountered at the cut end. The level of transection of the fibrous tissue at the porta hepatis is of prime importance for successful hepatic portoenterostomy. Operative findings, as well as histologic studies of extrahepatic bile ducts, suggest that the cause of biliary atresia is not a developmental anomaly but an inflammatory process during the fetal period [9]. If so, the location of fibrous remnants of the bile ducts in biliary atresia should be the same as that of normal bile ducts. Therefore, accurate knowledge of the normal anatomy of the porta hepatis is indispensable to hepatic portoenterostomy. Figure 2 shows the anatomical relationship between bile ducts and blood vessels around the porta hepatis in an adult who died from disease unrelated to the biliary tract. Bile ducts and the hepatic artery are situated above the portal vein. The right hepatic artery runs behind the bile ducts. Figure 3 demonstrates sagittal cut surfaces of the porta hepatis. The anatomical location of the bile ducts relative to the artery and the portal vein should be noted. Although the common hepatic duct is located anterior to the blood vessels, its right and left branches run cranial and posterior to the main branches of the portal vein. Figure 4 shows a schema of the sagittal cut surface of the porta hepatis of a patient with biliary atresia. The common hepatic duct replaced by fibrous tissue is anterior to the artery and portal vein, and the fibrous tissue in the porta hepatis is situated
Fig. 2. Normal anatomical relationship between bile ducts and blood vessels in the porta hepatis.
cranial and posterior to the bifurcation of the portal vein. Figure 5 is a low magnification photomicrograph of the sagittal cut surface of the porta hepatis of a patient who died of ascending cholangitis 2 months after hepatic portojejunostomy. Dilated bile ducts are seen near the anastomosed jejunum, but they did not reach the level of transection. In this patient, transection should have been done at the level of the posterior surface of the portal vein.
Surgical Technique Before operation, daily 5 mg doses of vitamin K are given for several days. The abdomen is opened through an upper transverse incision that extends from the right costal margin to the lateral border of
M. Kasai et al.: Operative Management of Biliary Atresia
Fig. 3. Sagittal cut surfaces of the porta hepatis. Right and left branches of the hepatic duct run cranial and posterior to the main branches of the portal vein.
Fig. 4. A schema of the sagittal cut surface of the porta hepatis of a patient with biliary atresia of the noncorrectable type.
the left rectus muscle. When occlusion of the common bile duct is confirmed by cholangiography, the gallbladder is freed from its liver bed and the dissection is advanced along the cystic duct toward the common bile duct (Fig. 6A). The junction of the common hepatic duct and cystic duct is occasionally thickened and forms a fibrous nodule. The common bile duct is frequently adherent to the surrounding tissues. A heavy silk thread is passed around the common bile duct. By upward traction on the silk thread, the posterior surface of the duct is separated from the underlying structures toward the porta hepatis. The right branch of the hepatic artery, and the bifurcation of the portal vein are identified. The common bile duct is then severed at its distal portion after ligation. The common bile duct with the gallbladder is pulled up and dissection is advanced between the portal vein and the fibrous
573
mass replacing the hepatic duct radicles (Fig. 6B). Usually 2 or 3 small branches of the portal vein enter the fibrous mass. These small veins must be carefully isolated and divided between ligatures (Fig. 6C). The portal vein is then pushed downwards and its posterior surface is freed from the fibrous mass. Dissection must be extended bilaterally to the entrance of the portal vein into the hepatic parenchyma. The transection of the fibrous mass is then carried out with scissors at the level of the posterior surface of the portal vein (Fig. 6D). Ligation or cauterization should not be applied to stop bleeding from the cut surface of the fibrous mass, because small bile ducts may be accidentally ligated with blood vessels or destroyed by cauterization. Irrigation with warm saline, about 50~ C, is effective in stopping hemorrhage, usually within 10 min. In cases in which the gallbladder is absent or which have been explored elsewhere, an incision is made in the hepatoduodenal ligament alongside the duodenum after a small amount of saline has been infiltrated. The hepatic artery and the portal vein are exposed. The peritoneum and fibrous tissue covering the blood vessels are brought up with a clamp, and dissection is advanced between the fibrous tissue and the underlying structures toward the porta hepatis (Fig. 7). The next step in the operation is the formation of a double-Y hepatic portojejunostomy (Fig. 8). This starts with creation of a Roux-en-Y loop. The jejunum is divided 10--15 cm distal to the ligament of Treitz. The distal cut end is closed by 2 layers of sutures and the proximal cut end is anastomosed to the side of the jejunum 45 cm distal to the closed end. The closed end of the Roux-en-Y limb is brought up to the porta hepatis through the transverse mesocolon. A small incision, 5-8 mm in length, is made on the antimesenteric border of the ascending limb adjacent to its closed end to create a stoma. The stoma of the jejunum is then anastomosed to the cut surface of the fibrous mass at the porta hepatis. First, a continuous over-and-over suture is placed using 4-0 Dexon | or chromic catgut between the margin of the jejunal stoma and the side of the fibrous mass around its cut end (Fig. 9). Sutures must not be placed on the transected surface of the fibrous mass to avoid obliteration of small bile ducts. After completion of the continuous suture, interrupted sutures using 4-0 silk are placed between the seromuscular layer of the jejunum and the fibrous tissues of the hepatoduodenal ligament posteriorly, and the quadrate lobe anteriorly. The last step of the operation is the formation of the second Y anastomosis and an external fistula. The ascending Roux-en-Y limb is divided 15 cm distal to the anastomosis at the porta hepatis, pre-
574
World J. Surg. Vol. 2, No. 5, September, 1978
Fig. 5. Low magnification photomicrograph of the sagittal cut surface of the porta hepatis of a patient who died of ascending cholangitis 2 months after hepatic portoenterostomy.
serving the vascular arch, and the proximal cut end is anastomosed to the side of the distal limb 7 cm from its cut end. The open end of the distal limb is brought out through a small stab wound in the left upper abdomen and fixed to the skin with several interrupted sutures, forming an external fistula. The abdomen is closed after a Penrose drain is inserted into the foramen of Winslow. Figure 10 schematically illustrates the sagittal profile of hepatic portoenterostomy. When the patency of the bile duct from the gallbladder to the duodenum is confirmed by cholangiography, the gallbladder is anastomosed to the porta hepatis to form a hepatic portocholecystostomy (Fig. 11). The cystic artery should be carefully preserved when the gallbladder is mobilized from the liver. Minimal dissection of the common bile duct is required for the preservation of a good blood supply. A small amount of saline is infiltrated into the perivascular tissue of the hepatoduodenal ligament. The right hepatic artery and the portal vein are first exposed. The fibrous tissue above the blood vessels is grasped by a clamp and is separated from the artery and portal vein. Dissection is then advanced between the fibrous tissue and the portal vein as previously described, and transection of the fibrous hepatic radicles is carried out at the posterior level of the portal vein. After transection of the fibrous tissue at the porta hepatis, an incision is made in the gallbladder. In cases with a large gallbladder, the fundus of the gallbladder is partially excised. The gallbladder is then anastomosed around the cut end of the fibrous tissue in a fashion similar to the anastomosis of the jejunum in hepatic portojejunostomy. The abdomen is closed in layers after a Penrose drain is inserted into the foramen of Winslow.
Postoperative M a n a g e m e n t
The patients are usually placed in an oxygen tent and a nasogastric tube is inserted for gastrointestinal decompression for several days after operation. Vitamin K in a dose of 1-2 mg/kg bodyweight is given parenterally every day. The fluid loss from the Penrose drain and the jejunal stoma is measured and replaced. Oral feeding is usually started on the 4th or 5th postoperative day. Medium chain tryglyceride formula is recommended for oral feeding in these patients because fat absorption is usually impaired by poor bile excretion during the early postoperative period. For prevention of ascending cholangitis, cephalosporin and aminoglycoside are administered in doses of 50-80 mg/kg per day of cephalexin or cephalozine intravenously for 10-14 days and 1 mg/kg per day of gentamicin intramuscularly for 7 days after operation. In cases without signs of cholangitis, administration of cephalosporin and aminoglycoside is stopped and aminobenzyl penicillin in a dose of 200-400 rag/day is given orally for several months. Following this, trimethoprim (40 rag) and sulfamethoxazole (200 rag), which may be obtained in proprietary tablet form, are prescribed daily and continued for several months more. As a choleretic, intravenous injection of 3 ml of 10% dehydrocholic acid diluted with 7 ml of 5% glucose is started on the evening of the day of operation and is given every 12 hours for 4 weeks or more. Oral administration of 0.3-0.4 g each of dehydrocholic acid and ursodesoxycholic acid (if available) is also added and is continued for several years. In patients with poor excretion of bile, 10 mg of prednisolone is given twice a day intravenously or
M. Kasai et al.: Operative Management of Biliary Atresia
A
575
~)'177,',/~,'r
Fig. 6. Technique of hepatic portoenterostomy. A. Dissection of gallblader and common bile duct is the first step in operation for biliary atresia. B. Dissection is advanced between the portal vein and the fibrous mass replacing the hepatic duct radicles. C. Two or three small branches of the portal vein entering into the fibrous mass are divided between ligatures. D. Transection of the fibrous mass is carried out at the level of the posterior surface of the portal vein.
intramuscularly, after the 6th postoperative day. The dosage of steroid is decreased e v e r y 3 days and changed to the oral administration of a daily dose o f 0.5 mg betamethasone, which is continued for 2 or 3 weeks. Routine administration of steroids may be useful after operation for " n o n c o r r e c t a b l e " biliary atresia. At any time when signs of ascending cholangitis appear, intravenous administration of antibiotics and choleretics should be resumed immediately. Ascending cholangitis usually appears as a sudden increase in temperature, without immediate jaundice. Steroids, in the same dosage as mentioned above, should be given in patients in whom treatment by antibiotics and choleretics is not effective. It is advisable to continue administration of oral
choleretics for 2 years or more. Vitamin D should be given when the amount of discharge from the jejunal stoma is large. The jejunal stoma is usually closed 21/2-3 years after operation.
Results
Of 174 patients with p r o v e n biliary atresia, 172 underwent a curative operation at the T o h o k u University Hospital during the period from 1953 through 1977. Jaundice disappeared in 52 of them (30%) after operation. The cure rate was 52% in type I atresia involving the c o m m o n bile duct, 17% in type II atresia involving the c o m m o n hepatic duct, and 29% in type III atresia involving the porta hepatis
576
World J. Surg. Vol. 2, No. 5, September, 1978
Fig. 8. Schema of double-Y hepatic portoenterostomy. Fig. 7. In cases in which the gallbladder is absent or which have been explored elsewhere, the peritoneum and fibrous tissue covering the vessels are brought up with a clamp and dissection is advanced toward the porta hepatis. (Table 1). The operative results have improved markedly since 1972 when hepatic portoenterostomy was modified in order to prevent postoperative cholangitis. The cure rate among 65 patients from 1971 through 1977 was 55.4%. Table 2 shows a correlation between age of the patients at the time of operation and postoperative bile excretion after hepatic portoenterostomy in type III biliary atresia in our recent series. Active bile excretion was restored in 91% of patients younger than 60 days of age. The older the patient, the p o o r e r are the operative results. A 10-day delay of operation may result in a decrease of the success rate by one-half. Our results show that 60 days of age is the critical time for surgical treatment of biliary atresia. By the end of April, 1978, a total of 47 patients were living without jaundice, 22 of them having survived more than 5 years and 9 more than 10 years. The longest survivors are now 23 years of age. All 22 long-term survivors are leading normal lives for their age, but 6 of them have some handicap. One 23-year-old girl has Turner's syndrome with physical retardation, another 23-year-old girl has intestinal malabsorption subsequent to several episodes of adhesive intestinal obstruction, and a 7year-old boy has mental retardation due to craniostenosis. T w o other patients have manifestations of portal hypertension; one of these, a 14year-old girl, has been doing well since a splenorenal shunt was carried out at 9 years of age. Finally,
Fig. 9. Hepatic portoenterostomy. A continuous overand-over suture is placed between the margin of the jejunal stoma and the cut margin of the fibrous mass.
one patient has giant splenomegaly but does not show signs of portal hypertension or hypersplenism. The remaining 16 patients have no symptoms or severe objective abnormalities.
Discussion
Until recently, biliary atresia was the most miserable disease in the pediatric surgical field. Only 520% of patients were previously considered to be amenable to operation, and the operative results were poor even in patients with biliary atresia of the correctable type. F o r so-called " n o n c o r r e c t a b l e " biliary atresia, several surgical procedures [13-16] had been proposed, but none had been fruitful.
M. Kasai et al.: Operative Management of Biliary Atresia
577
r
Fig. 11. Schema of hepatic portocholecystostomy. Fig. 10. Sagittal profile of completed hepatic portoenterostomy.
Table 1. Results of surgical treatment of biliary atresia in
the Second Department of Surgery, Tohoku University, 1953-1977.
Type of atresia
Total no. patients
27 II 30 III 117 Unknown 8 Total 182
No. patients operated upon 27 30
I
115
0 172
Disappearance of jaundice No. patients
%
14 5 33 -52
52 17 29 -30
Table 2. Operative results of surgical treatment of type III
biliary atresia, 1971-1977. Bile excretion Age at operation (days) No. patients Good Poor None < 60 61-70 71-90 > 91 Total
11 18 14 6 49
10(91%) 10 (56%) 6 (43%) 1 (17%) 27 (55%)
1 7 7 2 17
0 1 1 3 5
Hepatic p o r t o e n t e r o s t o m y was first reported as a radical operation for " n o n c o r r e c t a b l e " biliary atresia in the Japanese literature in 1959 [17], in the German literature in 1963 [18], and in the English literature in 1968 [19]. H o w e v e r , it has taken a long time for this approach to be generally accepted in the world. One of the reasons why p o r t o e n t e r o s t o m y failed to achieve general acceptance was the com-
mon misconception that extrahepatic biliary atresia was usually accompanied by intrahepatic atresia. The second reason was that the operation lacked mucosal approximation between bile ducts and intestine, which was generally considered essential to successful biliodigestive anastomosis. The third reason was the poor operative results reported by surgeons who performed this procedure in the early period. It was suggested by Danks [20] and proven by us [21-23] that the intrahepatic bile ducts were patent from the interlobular ductules to the porta hepatis in almost all patients with biliary atresia in early infancy. While it was reasonable to believe that mucosal anastomosis is important for the success of biliodigestive anastomoses, in biliary atresia of the noncorrectable type, mucosa-to-mucosa suturing is impossible because of the minute size of the bile ducts at the porta hepatis. Hepatic p o r t o e n t e r o s t o m y is not an ideal operation for joining the biliary and intestinal tracts, but it is the best that can be done. After hepatic p o r t o e n t e r o s t o m y , the flow of bile may be a very important factor in protecting the minute openings of the bile ducts at the porta heparis against stricture formation. For the successful surgery of biliary atresia, early operation, appropriate operative technique, and prevention of postoperative cholangitis are indispensable. Poor results of hepatic p o r t o e n t e r o s t o m y reported by some authors [24] seem to be due to a failure to meet these essential requirements. We now believe from the operative results in our r e c e n t series of patients that 80 or 90% of patients with biliary atresia can be cured when all 3 requisites for successful operation are satisfied. Cirrhosis, frequently associated with portal hypertension, has been reported by many authors in long-term survivors of operations for biliary atresia [2-5, 25]. In our series, 2 patients showed signs of
578
portal hypertension and another had giant splenomegaly. We have found in an unpublished study that the pressure in the portal vein, measured at the time of the initial operation, was high in all 19 patients examined, ranging from 170 to 400 mm water. In 5 patients, the portal pressure was again determined 3 to 28 months after the first operation. In 3, there was a decrease of pressure ranging from 44 to 135 mm water, and in the remaining 2, a pressure increase of 71 and 150 mm, respectively. The latter 2 patients had frequent attacks of cholangitis after operation. These results indicate that portal hypertension has developed in most patients with biliary atresia prior to surgery, but that it can improve after a successful operation. Delayed operation and postoperative cholangitis seem to contribute to late complications, as well as to early failure of bile flow.
World J. Surg. Vol. 2, No. 5, September, 1978
4. 5.
6. 7.
8. 9.
R~sum~
Chez les nouveaux-n6s atteints d'atr6sie biliaire, les canaux biliaires intra-h6patiques sont perm6ables. Dans le hile h6patique, ces canaux intrah6patiques se terminent par de fins canalicules enserr6s dans du tissu fibreux, qui ne se poursuivent pas jusqu'au canal h6patique commun. Le niveau de section du tissu fibreux dans le hile h6patique est donc tr6s important pour le succ6s de l'h6paticoent6rostomie. Cette section dolt 6tre faite au niveau de la face post6rieure de la veine porte. L a chirurgie pour atr6sie biliaire donnait 30% de gu6risons. Dans notre r6cente s6rie, les r6sultats ont 6t6 am61ior6s jusqu'A 55% de gu6risons. Nous avons 47 malades qui vivent sans ict6re, dont 24 depuis plus de 5 ans. Les plus longues survies atteignent 23 ans. Tous ont une vie normale pour leur fige, malgr6 le fait que 7 d'entre eux pr6sentaient certains handicaps physiques ou mentaux. Le succ~s de l'h6paticoent6rostomie d6pend de la pr6cocit6 de l'intervention, de la qualit6 de la technique op6ratoire et de la pr6vention de l'angiocholite postop6ratoire.
10.
11. 12. 13. 14. 15. 16. 17. 18.
19. References
20. 1. Kasai, M., Watanabe, I., Ohi, R.: Follow-up studies of long-term survivors after hepatic portoenterostomy for "noncorrectable" biliary atresia. J. Pediatr. Surg. 10:173, 1975 2. Miyata, M., Satani, M., Ueda, T., Okamoto, E.: Long-term results of hepatic portoenterostomy for biliary atresia: special reference to postoperative portal hypertension. Surgery 76:234, 1974 3. Kobayashi, A., Utsunomiya, T., Kawai, S., Ohbe,
21. 22. 23.
Y.: Congenital biliary atresia: analysis of 97 cases with reference to prognosis after hepatic portoenterostomy. Am. J. Dis. Child. 130:830, 1976 Altman, R.P., Chandra, R., Lilly, J.R.: Ongoing cirrhosis after successful porticoenterostomy in infants with biliary atresia. J. Pediatr. Surg. 10:685, 1975 Odi~vre, M., Valayer, J., Razemon-Pinta, M., Habib, E., Alagille, D.: Hepatic portoenterostomy or cholecystostomy in the treatment of extrahepatic biliary atresia. J. Pediatr. 88:774, 1976 Carcassonne, M., Bensoussan, A.: Long-term results in treatment of biliary atresia. Prog. Pediatr. Surg. 10:151, 1977 Sawaguchi, S., Akiyama, Y., Saeki, M., Ohta, Y.: The treatment of congenital biliary atresia, with special reference to hepatic portoentero-anastomosis. Fifth Annual Meeting of the Pacific Association of Pediatric Surgeons, 1972, Tokyo, Japan Kasai, M.: Hepatic portoenterostomy and its modifications for "noncorrectable" biliary atresia. Paediatrician 3:204, 1974 Kasai, M.: Treatment of biliary atresia with special reference to hepatic portoenterostomy and its modifications. Prog. Pediatr. Surg. 6:5, 1974 Suruga, K., Nagashima, K., Kohno, S., Miyano, T., Kitahara, T., inui, M.: A clinical and pathological study of congenital biliary atresia. J. Pediatr. Surg. 7:655, 1972 Lilly, J.R., Altman, R.P.: Hepatic portoenterostomy (the Kasai operation) for biliary atresia. Surgery 78:76, 1975 Ikeda, K., Suita, S.: Hepatic portogastrostomy using a gastric tube for the treatment of congenital biliary atresia. Z. Kinderchir. 17:360, 1975 Sterling, J.A.: Artificial bile ducts in the management of congenital biliary atresia: a clinical report. J. Int. Coll. Surg. 36:293, 1961 Williams, L.F., Jr., Dolling, J.A.: Thoracic ductesophagus anastomosis for relief of congenital biliary atresia. Surg. Forum 14:189, 1963 Suruga, K., Yamazaki, Z., Nagashima, K., Iwai, S., Mori, W.: [A new operation for biliary atresia]. Shuzutsu 18:910, 1964 Fonkalsrud, E.W., Kitagawa, S., Longmire, W.P., Jr.: Hepatic lymphatic drainage to the jejunum for congenital biliary atresia. Am. J. Surg. 112:188, 1966 Kasai, M., Suzuki, S.: [A new operation for "noncorrectable" biliary atresia--hepatic portoenterostomy]. Shuzutsu 13:733, 1959 Kasai, M., Kimura, S., Wagatsuma, M., Suzuki, H.: Die chirurgischen Behandlungen der angeborenen Missbildungen des Gallengangs. Therapiewoche 12:710, 1963 Kasai, M., Kimura S., Asakura, Y., Suzuki, H., Taira, Y., Ohashi, E.: Surgical treatment of biliary atresia. J. Pediatr. Surg. 3:665, 1968 Danks, D.M., Campbell, P.E.: Frequencies of certain liver diseases and abnormalities in Victoria, Australia birth defects. Orig. Article Series 8:119, 1972 Ohi, R., Kasai, M., Takahashi, T.: Intrahepatic biliary obstruction in congenital bile duct atresia. Tohoku J. Exp. Med. 99:129, 1976 Chiba, T., Kasai, M., Sasano, N.: Reconstruction of intrahepatic bile ducts in congenital biliary atresia. Tohoku J. Exp. Med. 115:99, 1975 Chiba, T., Kasai, M., Sasano, N.: Histopathological
M. Kasai et al.: Operative Management of Biliary Atresia
studies on intrahepatic bile ducts in the vicinity of the porta hepatis in biliary atresia. Tohoku J. Exp. Med. 118:199, 1976 24. Campbell, D.P., Poley, J.R., Bhatia, M., Smith, E.I.: Hepatic portoenterostomy--Is it indicated in the
579
treatment of biliary atresia? J. Pediatr. Surg. 9:329, 1974 25. Altman, R.P.: Portal decompression by interposition mesocaval shunt in patients with biliary atresia. J. Pediatr. Surg. 11:809, 1976
Invited Commentary R. Peter Altman, M.D. The Children's Hospital, Washington, D.C., U.S.A.
Professor Kasai's efforts on behalf of infants with biliary atresia have revolutionized the approach to these infants. Prior to the introduction of his portojejunostomy procedure, a variety of surgical approaches had been attempted, but all resulted in failure and frustration. Kasai's great contribution is not only a lucid description of the operative approach to this lesion but a tenable explanation as to why his procedure succeeds where others have failed. His demonstration that the fibrous tissue at the porta hepatis contains minute biliary ductules communicating with the intrahepatic bile ducts is the basic concept on which the operation is founded. Thus, bile ducts are not absent in patients with biliary atresia but rather are present as fibrous cords. These ductal remnants have been identified and confirmed histologically in every case explored at our institution. Kasai correctly stresses that a favorable surgical result depends upon early operation. Presumably, the microscopic ductular patency is lost at about 3 months of age, thus accounting for the discrepancy in surgical results in patients operated on after this time. Early surgical intervention implies expeditious diagnostic evaluation. In the majority of infants presenting with mixed jaundice, most hematologic, infectious and metabolic causes can be diagnosed or excluded within a few days. The association of alphal-antitrypsin deficiency with infantile cholestasis is now generally appreciated [1]. Therefore, we routinely screen jaundiced infants for this inherited metabolic disorder and have identified 5 cases over the past 4 years. Surgical exploration is not performed in the infant with alphal-antitrypsin deficiency. No single diagnostic laboratory test, x-ray, or isotope scan is absolutely reliable, and surgical exploration is advised for any infant in whom the cause of conjugated hyperbilirubinemia remains unexplained.
Kasai stresses the essential technical point in the portal dissection, namely, that the level of transection of the fibrous tissue at the porta hepatis is crucial. The regional arterial anatomy is variable and, therefore, the most reliable landmark for the division of the fibrous tissue has been the cephalad aspect of the bifurcating portal vein. Microscopic ductular patency is routinely demonstrated at this level whereas, the more distal hepatic duct is invariably obliterated. The biliary intestinal anastomosis must be constructed so as to incorporate all ductal elements within the intestinal lumen. To accomplish this, the posterior row of sutures is placed behind the portal vein by retracting its bifurcation caudally. We have found that placement of these sutures is facilitated by employing an interrupted technique using fine double-arm sutures. Approximately 5 or 6 are needed to establish the posterior row. The anterior row is performed as described by Kasai. Many modifications of the jejunal limb have been devised, all designed to reduce or eliminate postoperative cholangitis [2]. Kasai's double Roux-en-Y technique shows promise in this regard. We currently favor venting the conduit by a double-barrel Mikulicz technique. Routine intraoperative cholangiography is not performed. However, an operative cholangiogram is obtained if the gallbladder is obviously patent. The finding of a gallbladder, cystic, and common bile duct patent to the duodenum gives the surgeon the option of constructing a portocholecystostomy. Anatomically, this has obvious advantages. The entry of bile into the duodenum across the normal sphincter eliminates postoperative reflux with attendant cholangitis. However, my own experience with this technique has been disappointing. Bile flow was achieved in 6 of 8 patients having portocholecystostomy; however, 5 of the 6 required re-
WbddJournal
Sdrgery
Technique and Results of Operative Management of Biliary Atresia Morio Kasai, M.D., Hiroshi Suzuki, M.D., Eisuke Ohashi, M.D., Ryoji Ohi, M.D., Tsuneo Chiba, M.D., and Atsutake Okamoto, M.D. SecondDepartmentof Surgery,TohokuUniversitySchoolof Medicine,Sendai,Japan
Intrahepatic bile ducts are patent in the liver of patients with biliary atresia in early infancy. Small bile ducts at the porta hepatis continuing to the intrahepatic ducts usually terminate in fibrous tissue replacing the hepatic radicles, which does not extend to the common hepatic duct. Therefore, the level of transection of the fibrous tissue at the porta hepatis is of prime importance for successful hepatic portoenterostomy. Transection should be done at the level of the posterior surface of the portal vein. The overall cure rate of surgery for biliary atresia was 30%. In our recent series, however, the results have markedly improved and the cure rate has been increased to 55%. There are 47 patients living without jaundice, 22 of them for more than 5 years. The longest survivors are 23 years old. All the longterm survivors are leading a normal life for their age, although 6 of them have some physical or mental handicap. Early operation, adequate surgical technique, and prevention of postoperative cholangitis are requisites for successful hepatic portoenterostomy.
sufficiency is not uncommon even in patients who were relieved of jaundice by operation [4-6]. There have been many documented methods [712] for making an intestinal conduit for hepatic portoenterostomy to prevent postoperative ascending cholangitis. It is our present policy to use a doubleY hepatic portojejunostomy for patients having a fibrous common bile duct, and hepatic portocholecystostomy for patients having a patent gallbladder and common bile duct. For success in surgery of biliary atresia, early operation, adequate operative technique, and prevention of postoperative cholangitis are essential. The purpose of this communication is to present the technical details of hepatic portoenterostomy, as well as the normal and pathological anatomy of the porta hepatis, the knowledge of which is essential for a successful operation. Early and long-term results are presented. Finally, the postoperative management is described.
Biliary atresia of the noncorrectable type has become a curable disease by the introduction of hepatic portoenterostomy. Recent reports [1-3] on follow-up studies of survivors after hepatic portoenterostomy indicate that many of the long-term survivors showed normal growth and development. Nevertheless, the results of operation for biliary atresia are still unsatisfactory, since sustained drainage of bile has been restored in only 30-45% of patients, and liver cirrhosis resulting in rupture of esophageal varices, hypersplenism, or hepatic in-
Anatomy
In biliary atresia, extrahepatic bile ducts are usually replaced by fibrous tissue. The tissue from the porta hepatis contains many small bile ducts communicating with the intrahepatic bile ducts. Figure 1 shows a 3-dimensional reconstruction of bile ducts in the fibrous tissue of the porta hepatis taken from a patient with noncorrectable biliary atresia, in whom
Reprint requests: Professor Morio Kasai, The Second Department of Surgery, Tohoku University School of Medicine, Seiryochi, 1-1, Sendai, Japan.
0364-2313/78/0002-0571 $02.00 9 1978 Soci6t6 Internationale de Chirurgie 571
572
World J. Surg. Vol. 2, No. 5, September, 1978
Fig. 1. Three-dimensional reconstruction of bile ducts found in the encircled portion of the extrahepatic bile duct removed from a patient with biliary atresia of type III.
active excretion of bile was achieved by operation. Small bile ducts in continuity with intrahepatic ducts were obliterated within 1,500/z from the proximal end of the resected specimen and did not connect with the common hepatic duct. If the fibrous tissue had been transected 1 or 2 mm on the duodenal side in this patient, few or no ducts draining bile would have been encountered at the cut end. The level of transection of the fibrous tissue at the porta hepatis is of prime importance for successful hepatic portoenterostomy. Operative findings, as well as histologic studies of extrahepatic bile ducts, suggest that the cause of biliary atresia is not a developmental anomaly but an inflammatory process during the fetal period [9]. If so, the location of fibrous remnants of the bile ducts in biliary atresia should be the same as that of normal bile ducts. Therefore, accurate knowledge of the normal anatomy of the porta hepatis is indispensable to hepatic portoenterostomy. Figure 2 shows the anatomical relationship between bile ducts and blood vessels around the porta hepatis in an adult who died from disease unrelated to the biliary tract. Bile ducts and the hepatic artery are situated above the portal vein. The right hepatic artery runs behind the bile ducts. Figure 3 demonstrates sagittal cut surfaces of the porta hepatis. The anatomical location of the bile ducts relative to the artery and the portal vein should be noted. Although the common hepatic duct is located anterior to the blood vessels, its right and left branches run cranial and posterior to the main branches of the portal vein. Figure 4 shows a schema of the sagittal cut surface of the porta hepatis of a patient with biliary atresia. The common hepatic duct replaced by fibrous tissue is anterior to the artery and portal vein, and the fibrous tissue in the porta hepatis is situated
Fig. 2. Normal anatomical relationship between bile ducts and blood vessels in the porta hepatis.
cranial and posterior to the bifurcation of the portal vein. Figure 5 is a low magnification photomicrograph of the sagittal cut surface of the porta hepatis of a patient who died of ascending cholangitis 2 months after hepatic portojejunostomy. Dilated bile ducts are seen near the anastomosed jejunum, but they did not reach the level of transection. In this patient, transection should have been done at the level of the posterior surface of the portal vein.
Surgical Technique Before operation, daily 5 mg doses of vitamin K are given for several days. The abdomen is opened through an upper transverse incision that extends from the right costal margin to the lateral border of
M. Kasai et al.: Operative Management of Biliary Atresia
Fig. 3. Sagittal cut surfaces of the porta hepatis. Right and left branches of the hepatic duct run cranial and posterior to the main branches of the portal vein.
Fig. 4. A schema of the sagittal cut surface of the porta hepatis of a patient with biliary atresia of the noncorrectable type.
the left rectus muscle. When occlusion of the common bile duct is confirmed by cholangiography, the gallbladder is freed from its liver bed and the dissection is advanced along the cystic duct toward the common bile duct (Fig. 6A). The junction of the common hepatic duct and cystic duct is occasionally thickened and forms a fibrous nodule. The common bile duct is frequently adherent to the surrounding tissues. A heavy silk thread is passed around the common bile duct. By upward traction on the silk thread, the posterior surface of the duct is separated from the underlying structures toward the porta hepatis. The right branch of the hepatic artery, and the bifurcation of the portal vein are identified. The common bile duct is then severed at its distal portion after ligation. The common bile duct with the gallbladder is pulled up and dissection is advanced between the portal vein and the fibrous
573
mass replacing the hepatic duct radicles (Fig. 6B). Usually 2 or 3 small branches of the portal vein enter the fibrous mass. These small veins must be carefully isolated and divided between ligatures (Fig. 6C). The portal vein is then pushed downwards and its posterior surface is freed from the fibrous mass. Dissection must be extended bilaterally to the entrance of the portal vein into the hepatic parenchyma. The transection of the fibrous mass is then carried out with scissors at the level of the posterior surface of the portal vein (Fig. 6D). Ligation or cauterization should not be applied to stop bleeding from the cut surface of the fibrous mass, because small bile ducts may be accidentally ligated with blood vessels or destroyed by cauterization. Irrigation with warm saline, about 50~ C, is effective in stopping hemorrhage, usually within 10 min. In cases in which the gallbladder is absent or which have been explored elsewhere, an incision is made in the hepatoduodenal ligament alongside the duodenum after a small amount of saline has been infiltrated. The hepatic artery and the portal vein are exposed. The peritoneum and fibrous tissue covering the blood vessels are brought up with a clamp, and dissection is advanced between the fibrous tissue and the underlying structures toward the porta hepatis (Fig. 7). The next step in the operation is the formation of a double-Y hepatic portojejunostomy (Fig. 8). This starts with creation of a Roux-en-Y loop. The jejunum is divided 10--15 cm distal to the ligament of Treitz. The distal cut end is closed by 2 layers of sutures and the proximal cut end is anastomosed to the side of the jejunum 45 cm distal to the closed end. The closed end of the Roux-en-Y limb is brought up to the porta hepatis through the transverse mesocolon. A small incision, 5-8 mm in length, is made on the antimesenteric border of the ascending limb adjacent to its closed end to create a stoma. The stoma of the jejunum is then anastomosed to the cut surface of the fibrous mass at the porta hepatis. First, a continuous over-and-over suture is placed using 4-0 Dexon | or chromic catgut between the margin of the jejunal stoma and the side of the fibrous mass around its cut end (Fig. 9). Sutures must not be placed on the transected surface of the fibrous mass to avoid obliteration of small bile ducts. After completion of the continuous suture, interrupted sutures using 4-0 silk are placed between the seromuscular layer of the jejunum and the fibrous tissues of the hepatoduodenal ligament posteriorly, and the quadrate lobe anteriorly. The last step of the operation is the formation of the second Y anastomosis and an external fistula. The ascending Roux-en-Y limb is divided 15 cm distal to the anastomosis at the porta hepatis, pre-
574
World J. Surg. Vol. 2, No. 5, September, 1978
Fig. 5. Low magnification photomicrograph of the sagittal cut surface of the porta hepatis of a patient who died of ascending cholangitis 2 months after hepatic portoenterostomy.
serving the vascular arch, and the proximal cut end is anastomosed to the side of the distal limb 7 cm from its cut end. The open end of the distal limb is brought out through a small stab wound in the left upper abdomen and fixed to the skin with several interrupted sutures, forming an external fistula. The abdomen is closed after a Penrose drain is inserted into the foramen of Winslow. Figure 10 schematically illustrates the sagittal profile of hepatic portoenterostomy. When the patency of the bile duct from the gallbladder to the duodenum is confirmed by cholangiography, the gallbladder is anastomosed to the porta hepatis to form a hepatic portocholecystostomy (Fig. 11). The cystic artery should be carefully preserved when the gallbladder is mobilized from the liver. Minimal dissection of the common bile duct is required for the preservation of a good blood supply. A small amount of saline is infiltrated into the perivascular tissue of the hepatoduodenal ligament. The right hepatic artery and the portal vein are first exposed. The fibrous tissue above the blood vessels is grasped by a clamp and is separated from the artery and portal vein. Dissection is then advanced between the fibrous tissue and the portal vein as previously described, and transection of the fibrous hepatic radicles is carried out at the posterior level of the portal vein. After transection of the fibrous tissue at the porta hepatis, an incision is made in the gallbladder. In cases with a large gallbladder, the fundus of the gallbladder is partially excised. The gallbladder is then anastomosed around the cut end of the fibrous tissue in a fashion similar to the anastomosis of the jejunum in hepatic portojejunostomy. The abdomen is closed in layers after a Penrose drain is inserted into the foramen of Winslow.
Postoperative M a n a g e m e n t
The patients are usually placed in an oxygen tent and a nasogastric tube is inserted for gastrointestinal decompression for several days after operation. Vitamin K in a dose of 1-2 mg/kg bodyweight is given parenterally every day. The fluid loss from the Penrose drain and the jejunal stoma is measured and replaced. Oral feeding is usually started on the 4th or 5th postoperative day. Medium chain tryglyceride formula is recommended for oral feeding in these patients because fat absorption is usually impaired by poor bile excretion during the early postoperative period. For prevention of ascending cholangitis, cephalosporin and aminoglycoside are administered in doses of 50-80 mg/kg per day of cephalexin or cephalozine intravenously for 10-14 days and 1 mg/kg per day of gentamicin intramuscularly for 7 days after operation. In cases without signs of cholangitis, administration of cephalosporin and aminoglycoside is stopped and aminobenzyl penicillin in a dose of 200-400 rag/day is given orally for several months. Following this, trimethoprim (40 rag) and sulfamethoxazole (200 rag), which may be obtained in proprietary tablet form, are prescribed daily and continued for several months more. As a choleretic, intravenous injection of 3 ml of 10% dehydrocholic acid diluted with 7 ml of 5% glucose is started on the evening of the day of operation and is given every 12 hours for 4 weeks or more. Oral administration of 0.3-0.4 g each of dehydrocholic acid and ursodesoxycholic acid (if available) is also added and is continued for several years. In patients with poor excretion of bile, 10 mg of prednisolone is given twice a day intravenously or
M. Kasai et al.: Operative Management of Biliary Atresia
A
575
~)'177,',/~,'r
Fig. 6. Technique of hepatic portoenterostomy. A. Dissection of gallblader and common bile duct is the first step in operation for biliary atresia. B. Dissection is advanced between the portal vein and the fibrous mass replacing the hepatic duct radicles. C. Two or three small branches of the portal vein entering into the fibrous mass are divided between ligatures. D. Transection of the fibrous mass is carried out at the level of the posterior surface of the portal vein.
intramuscularly, after the 6th postoperative day. The dosage of steroid is decreased e v e r y 3 days and changed to the oral administration of a daily dose o f 0.5 mg betamethasone, which is continued for 2 or 3 weeks. Routine administration of steroids may be useful after operation for " n o n c o r r e c t a b l e " biliary atresia. At any time when signs of ascending cholangitis appear, intravenous administration of antibiotics and choleretics should be resumed immediately. Ascending cholangitis usually appears as a sudden increase in temperature, without immediate jaundice. Steroids, in the same dosage as mentioned above, should be given in patients in whom treatment by antibiotics and choleretics is not effective. It is advisable to continue administration of oral
choleretics for 2 years or more. Vitamin D should be given when the amount of discharge from the jejunal stoma is large. The jejunal stoma is usually closed 21/2-3 years after operation.
Results
Of 174 patients with p r o v e n biliary atresia, 172 underwent a curative operation at the T o h o k u University Hospital during the period from 1953 through 1977. Jaundice disappeared in 52 of them (30%) after operation. The cure rate was 52% in type I atresia involving the c o m m o n bile duct, 17% in type II atresia involving the c o m m o n hepatic duct, and 29% in type III atresia involving the porta hepatis
576
World J. Surg. Vol. 2, No. 5, September, 1978
Fig. 8. Schema of double-Y hepatic portoenterostomy. Fig. 7. In cases in which the gallbladder is absent or which have been explored elsewhere, the peritoneum and fibrous tissue covering the vessels are brought up with a clamp and dissection is advanced toward the porta hepatis. (Table 1). The operative results have improved markedly since 1972 when hepatic portoenterostomy was modified in order to prevent postoperative cholangitis. The cure rate among 65 patients from 1971 through 1977 was 55.4%. Table 2 shows a correlation between age of the patients at the time of operation and postoperative bile excretion after hepatic portoenterostomy in type III biliary atresia in our recent series. Active bile excretion was restored in 91% of patients younger than 60 days of age. The older the patient, the p o o r e r are the operative results. A 10-day delay of operation may result in a decrease of the success rate by one-half. Our results show that 60 days of age is the critical time for surgical treatment of biliary atresia. By the end of April, 1978, a total of 47 patients were living without jaundice, 22 of them having survived more than 5 years and 9 more than 10 years. The longest survivors are now 23 years of age. All 22 long-term survivors are leading normal lives for their age, but 6 of them have some handicap. One 23-year-old girl has Turner's syndrome with physical retardation, another 23-year-old girl has intestinal malabsorption subsequent to several episodes of adhesive intestinal obstruction, and a 7year-old boy has mental retardation due to craniostenosis. T w o other patients have manifestations of portal hypertension; one of these, a 14year-old girl, has been doing well since a splenorenal shunt was carried out at 9 years of age. Finally,
Fig. 9. Hepatic portoenterostomy. A continuous overand-over suture is placed between the margin of the jejunal stoma and the cut margin of the fibrous mass.
one patient has giant splenomegaly but does not show signs of portal hypertension or hypersplenism. The remaining 16 patients have no symptoms or severe objective abnormalities.
Discussion
Until recently, biliary atresia was the most miserable disease in the pediatric surgical field. Only 520% of patients were previously considered to be amenable to operation, and the operative results were poor even in patients with biliary atresia of the correctable type. F o r so-called " n o n c o r r e c t a b l e " biliary atresia, several surgical procedures [13-16] had been proposed, but none had been fruitful.
M. Kasai et al.: Operative Management of Biliary Atresia
577
r
Fig. 11. Schema of hepatic portocholecystostomy. Fig. 10. Sagittal profile of completed hepatic portoenterostomy.
Table 1. Results of surgical treatment of biliary atresia in
the Second Department of Surgery, Tohoku University, 1953-1977.
Type of atresia
Total no. patients
27 II 30 III 117 Unknown 8 Total 182
No. patients operated upon 27 30
I
115
0 172
Disappearance of jaundice No. patients
%
14 5 33 -52
52 17 29 -30
Table 2. Operative results of surgical treatment of type III
biliary atresia, 1971-1977. Bile excretion Age at operation (days) No. patients Good Poor None < 60 61-70 71-90 > 91 Total
11 18 14 6 49
10(91%) 10 (56%) 6 (43%) 1 (17%) 27 (55%)
1 7 7 2 17
0 1 1 3 5
Hepatic p o r t o e n t e r o s t o m y was first reported as a radical operation for " n o n c o r r e c t a b l e " biliary atresia in the Japanese literature in 1959 [17], in the German literature in 1963 [18], and in the English literature in 1968 [19]. H o w e v e r , it has taken a long time for this approach to be generally accepted in the world. One of the reasons why p o r t o e n t e r o s t o m y failed to achieve general acceptance was the com-
mon misconception that extrahepatic biliary atresia was usually accompanied by intrahepatic atresia. The second reason was that the operation lacked mucosal approximation between bile ducts and intestine, which was generally considered essential to successful biliodigestive anastomosis. The third reason was the poor operative results reported by surgeons who performed this procedure in the early period. It was suggested by Danks [20] and proven by us [21-23] that the intrahepatic bile ducts were patent from the interlobular ductules to the porta hepatis in almost all patients with biliary atresia in early infancy. While it was reasonable to believe that mucosal anastomosis is important for the success of biliodigestive anastomoses, in biliary atresia of the noncorrectable type, mucosa-to-mucosa suturing is impossible because of the minute size of the bile ducts at the porta hepatis. Hepatic p o r t o e n t e r o s t o m y is not an ideal operation for joining the biliary and intestinal tracts, but it is the best that can be done. After hepatic p o r t o e n t e r o s t o m y , the flow of bile may be a very important factor in protecting the minute openings of the bile ducts at the porta heparis against stricture formation. For the successful surgery of biliary atresia, early operation, appropriate operative technique, and prevention of postoperative cholangitis are indispensable. Poor results of hepatic p o r t o e n t e r o s t o m y reported by some authors [24] seem to be due to a failure to meet these essential requirements. We now believe from the operative results in our r e c e n t series of patients that 80 or 90% of patients with biliary atresia can be cured when all 3 requisites for successful operation are satisfied. Cirrhosis, frequently associated with portal hypertension, has been reported by many authors in long-term survivors of operations for biliary atresia [2-5, 25]. In our series, 2 patients showed signs of
578
portal hypertension and another had giant splenomegaly. We have found in an unpublished study that the pressure in the portal vein, measured at the time of the initial operation, was high in all 19 patients examined, ranging from 170 to 400 mm water. In 5 patients, the portal pressure was again determined 3 to 28 months after the first operation. In 3, there was a decrease of pressure ranging from 44 to 135 mm water, and in the remaining 2, a pressure increase of 71 and 150 mm, respectively. The latter 2 patients had frequent attacks of cholangitis after operation. These results indicate that portal hypertension has developed in most patients with biliary atresia prior to surgery, but that it can improve after a successful operation. Delayed operation and postoperative cholangitis seem to contribute to late complications, as well as to early failure of bile flow.
World J. Surg. Vol. 2, No. 5, September, 1978
4. 5.
6. 7.
8. 9.
R~sum~
Chez les nouveaux-n6s atteints d'atr6sie biliaire, les canaux biliaires intra-h6patiques sont perm6ables. Dans le hile h6patique, ces canaux intrah6patiques se terminent par de fins canalicules enserr6s dans du tissu fibreux, qui ne se poursuivent pas jusqu'au canal h6patique commun. Le niveau de section du tissu fibreux dans le hile h6patique est donc tr6s important pour le succ6s de l'h6paticoent6rostomie. Cette section dolt 6tre faite au niveau de la face post6rieure de la veine porte. L a chirurgie pour atr6sie biliaire donnait 30% de gu6risons. Dans notre r6cente s6rie, les r6sultats ont 6t6 am61ior6s jusqu'A 55% de gu6risons. Nous avons 47 malades qui vivent sans ict6re, dont 24 depuis plus de 5 ans. Les plus longues survies atteignent 23 ans. Tous ont une vie normale pour leur fige, malgr6 le fait que 7 d'entre eux pr6sentaient certains handicaps physiques ou mentaux. Le succ~s de l'h6paticoent6rostomie d6pend de la pr6cocit6 de l'intervention, de la qualit6 de la technique op6ratoire et de la pr6vention de l'angiocholite postop6ratoire.
10.
11. 12. 13. 14. 15. 16. 17. 18.
19. References
20. 1. Kasai, M., Watanabe, I., Ohi, R.: Follow-up studies of long-term survivors after hepatic portoenterostomy for "noncorrectable" biliary atresia. J. Pediatr. Surg. 10:173, 1975 2. Miyata, M., Satani, M., Ueda, T., Okamoto, E.: Long-term results of hepatic portoenterostomy for biliary atresia: special reference to postoperative portal hypertension. Surgery 76:234, 1974 3. Kobayashi, A., Utsunomiya, T., Kawai, S., Ohbe,
21. 22. 23.
Y.: Congenital biliary atresia: analysis of 97 cases with reference to prognosis after hepatic portoenterostomy. Am. J. Dis. Child. 130:830, 1976 Altman, R.P., Chandra, R., Lilly, J.R.: Ongoing cirrhosis after successful porticoenterostomy in infants with biliary atresia. J. Pediatr. Surg. 10:685, 1975 Odi~vre, M., Valayer, J., Razemon-Pinta, M., Habib, E., Alagille, D.: Hepatic portoenterostomy or cholecystostomy in the treatment of extrahepatic biliary atresia. J. Pediatr. 88:774, 1976 Carcassonne, M., Bensoussan, A.: Long-term results in treatment of biliary atresia. Prog. Pediatr. Surg. 10:151, 1977 Sawaguchi, S., Akiyama, Y., Saeki, M., Ohta, Y.: The treatment of congenital biliary atresia, with special reference to hepatic portoentero-anastomosis. Fifth Annual Meeting of the Pacific Association of Pediatric Surgeons, 1972, Tokyo, Japan Kasai, M.: Hepatic portoenterostomy and its modifications for "noncorrectable" biliary atresia. Paediatrician 3:204, 1974 Kasai, M.: Treatment of biliary atresia with special reference to hepatic portoenterostomy and its modifications. Prog. Pediatr. Surg. 6:5, 1974 Suruga, K., Nagashima, K., Kohno, S., Miyano, T., Kitahara, T., inui, M.: A clinical and pathological study of congenital biliary atresia. J. Pediatr. Surg. 7:655, 1972 Lilly, J.R., Altman, R.P.: Hepatic portoenterostomy (the Kasai operation) for biliary atresia. Surgery 78:76, 1975 Ikeda, K., Suita, S.: Hepatic portogastrostomy using a gastric tube for the treatment of congenital biliary atresia. Z. Kinderchir. 17:360, 1975 Sterling, J.A.: Artificial bile ducts in the management of congenital biliary atresia: a clinical report. J. Int. Coll. Surg. 36:293, 1961 Williams, L.F., Jr., Dolling, J.A.: Thoracic ductesophagus anastomosis for relief of congenital biliary atresia. Surg. Forum 14:189, 1963 Suruga, K., Yamazaki, Z., Nagashima, K., Iwai, S., Mori, W.: [A new operation for biliary atresia]. Shuzutsu 18:910, 1964 Fonkalsrud, E.W., Kitagawa, S., Longmire, W.P., Jr.: Hepatic lymphatic drainage to the jejunum for congenital biliary atresia. Am. J. Surg. 112:188, 1966 Kasai, M., Suzuki, S.: [A new operation for "noncorrectable" biliary atresia--hepatic portoenterostomy]. Shuzutsu 13:733, 1959 Kasai, M., Kimura, S., Wagatsuma, M., Suzuki, H.: Die chirurgischen Behandlungen der angeborenen Missbildungen des Gallengangs. Therapiewoche 12:710, 1963 Kasai, M., Kimura S., Asakura, Y., Suzuki, H., Taira, Y., Ohashi, E.: Surgical treatment of biliary atresia. J. Pediatr. Surg. 3:665, 1968 Danks, D.M., Campbell, P.E.: Frequencies of certain liver diseases and abnormalities in Victoria, Australia birth defects. Orig. Article Series 8:119, 1972 Ohi, R., Kasai, M., Takahashi, T.: Intrahepatic biliary obstruction in congenital bile duct atresia. Tohoku J. Exp. Med. 99:129, 1976 Chiba, T., Kasai, M., Sasano, N.: Reconstruction of intrahepatic bile ducts in congenital biliary atresia. Tohoku J. Exp. Med. 115:99, 1975 Chiba, T., Kasai, M., Sasano, N.: Histopathological
M. Kasai et al.: Operative Management of Biliary Atresia
studies on intrahepatic bile ducts in the vicinity of the porta hepatis in biliary atresia. Tohoku J. Exp. Med. 118:199, 1976 24. Campbell, D.P., Poley, J.R., Bhatia, M., Smith, E.I.: Hepatic portoenterostomy--Is it indicated in the
579
treatment of biliary atresia? J. Pediatr. Surg. 9:329, 1974 25. Altman, R.P.: Portal decompression by interposition mesocaval shunt in patients with biliary atresia. J. Pediatr. Surg. 11:809, 1976
Invited Commentary R. Peter Altman, M.D. The Children's Hospital, Washington, D.C., U.S.A.
Professor Kasai's efforts on behalf of infants with biliary atresia have revolutionized the approach to these infants. Prior to the introduction of his portojejunostomy procedure, a variety of surgical approaches had been attempted, but all resulted in failure and frustration. Kasai's great contribution is not only a lucid description of the operative approach to this lesion but a tenable explanation as to why his procedure succeeds where others have failed. His demonstration that the fibrous tissue at the porta hepatis contains minute biliary ductules communicating with the intrahepatic bile ducts is the basic concept on which the operation is founded. Thus, bile ducts are not absent in patients with biliary atresia but rather are present as fibrous cords. These ductal remnants have been identified and confirmed histologically in every case explored at our institution. Kasai correctly stresses that a favorable surgical result depends upon early operation. Presumably, the microscopic ductular patency is lost at about 3 months of age, thus accounting for the discrepancy in surgical results in patients operated on after this time. Early surgical intervention implies expeditious diagnostic evaluation. In the majority of infants presenting with mixed jaundice, most hematologic, infectious and metabolic causes can be diagnosed or excluded within a few days. The association of alphal-antitrypsin deficiency with infantile cholestasis is now generally appreciated [1]. Therefore, we routinely screen jaundiced infants for this inherited metabolic disorder and have identified 5 cases over the past 4 years. Surgical exploration is not performed in the infant with alphal-antitrypsin deficiency. No single diagnostic laboratory test, x-ray, or isotope scan is absolutely reliable, and surgical exploration is advised for any infant in whom the cause of conjugated hyperbilirubinemia remains unexplained.
Kasai stresses the essential technical point in the portal dissection, namely, that the level of transection of the fibrous tissue at the porta hepatis is crucial. The regional arterial anatomy is variable and, therefore, the most reliable landmark for the division of the fibrous tissue has been the cephalad aspect of the bifurcating portal vein. Microscopic ductular patency is routinely demonstrated at this level whereas, the more distal hepatic duct is invariably obliterated. The biliary intestinal anastomosis must be constructed so as to incorporate all ductal elements within the intestinal lumen. To accomplish this, the posterior row of sutures is placed behind the portal vein by retracting its bifurcation caudally. We have found that placement of these sutures is facilitated by employing an interrupted technique using fine double-arm sutures. Approximately 5 or 6 are needed to establish the posterior row. The anterior row is performed as described by Kasai. Many modifications of the jejunal limb have been devised, all designed to reduce or eliminate postoperative cholangitis [2]. Kasai's double Roux-en-Y technique shows promise in this regard. We currently favor venting the conduit by a double-barrel Mikulicz technique. Routine intraoperative cholangiography is not performed. However, an operative cholangiogram is obtained if the gallbladder is obviously patent. The finding of a gallbladder, cystic, and common bile duct patent to the duodenum gives the surgeon the option of constructing a portocholecystostomy. Anatomically, this has obvious advantages. The entry of bile into the duodenum across the normal sphincter eliminates postoperative reflux with attendant cholangitis. However, my own experience with this technique has been disappointing. Bile flow was achieved in 6 of 8 patients having portocholecystostomy; however, 5 of the 6 required re-
