Technical Aspects of Hepatic Portal Dissection in Biliary Atresia Ken Kimura, Chikara Tsugawa, Masako Kubo, Yoichi Matsumoto, and Hiroshi Itoh Kobe, Japan 9 During the past 8 yr, 37 patients with a noncorrectable type of biliary atresia have undergone hepatic portoenterostomy or portocholecystostomy at the Kobe Children's Hospital. The hepatic portal dissections employed in this series were classified as "supraportal" (9 procedures), "portal" (25 procedures), and "infra-portal" (3 procedures) based on the level at which the fibrous mass at the porta hepatis was transsected as determined by the operative record and the pathologic findings. Successful biliary drainage was achieved in 19 out of 25 patients (76%) with a "portal" type of dissection, while 1 out of 9 with "supra-portal" and none out of 3 with "infra-portal" type dissections were successful in this respect. Of the 19 patients who achieved significant biliary flow, 8 have lived for 2 - 7 yr without jaundice and 3 others are jaundice-free for shorter intervals. INDEX WORDS: dissection.
Biliary
atresia;
hepatic
portal
T R E A T M E N T of noncorrectable I NtypeT HofE biliary atresia, successful bile drainage is the first step toward cure of the disease. Various factors such as hepatic failure, cholangitis, cirrhosis, or portal hypertension will influence the ultimate outcome, but these are problems to be confronted after successful bile drainage is established. In the attempt to assure successful bile drainage, the technique of the hepatic portal dissection is the most important factor. Today, there is no doubt that the "Kasai procedure" provides hope of survival for infants with the noncorrectable types of biliary atresia. However, there are variations in the technique for hepatic portal dissection that are noted in the literature and all have been described as essential features of the "Kasai procedure." Each surgeon seems to have a unique concept of the optimal point to transect the fibrous structures at the porta hepatis. This may be one of the most important reasons for the different results in reported series. MATERIALS AND METHODS During the past 8 yr, 37 patients with noncorrectable type of biliary atresia have undergone hepatic portoenterostomy or portocholecystostomy at the Kobe Children's Hospital.
Journal of Pediatric Surgery, Vol. 14, No. 1 (February), 1979
The techniques of hepatic portal dissection employed in these patients were classified into three types according to the extent of dissection and level of transection of the hepatic hilar tissues. Designation of "supra-portal," "portal," and "infra-portal" types of dissection was based on the combined gross description of the operative record and the gross and microscopic pathologic findings in the excised tissue. Patients were divided into three groups according to the type of hepatic portal dissection and the outcome of each group was compared focusing on postoperative bile drainage.
Operative Technique By cannulating the gall bladder with a fine catheter, a direct cbolangiography was performed. When there was no visualization of the intrahepatic biliary system, a diagnosis of noncorrectable type biliary atresia was established. After detaching the gall bladder from the liver, a penrose drain was placed around the hepatoduodenal ligament to secure hemostasis in the event of portal venous injury. The fibrous cord, including the remnant of the hepatic duct, was dissected upward denuding the portal vein and the hepatic arteries. Just below the portal bifurcation, the fibrous cord connects with a fibrous mass, that is triangular in shape (Fig. 1); the fine structure of the hepatic hilus is schematically illustrated in Fig. 2 and the type of hepatic portal dissection in Fig. 3. In the group with an "infra-portal" type of dissection, the fibrous cord was transected distal to the mass and an anastomosis was made to the jejunal loop. In the group with a "portal" type of dissection, transection was made through the portal fibrous mass by various methods (Figs. 3 a, b, and c). The fine veins draining from the posterior aspect of the portal venous bifurcation to the portal fibrous mass were individually ligated and divided (Fig. 4). Employing this procedure, it was possible to advance dissection along the margin of the portal venous bifurcation. The posterior surface of the portal fibrous mass became visible by downward retraction on the portal venous bifurcation. Then, the lateral corners of the portal fibrous mass were dissected to the point where the mass is fused to the liver tissue. Under moderate tension, the fibrous mass was then transected at the closest point to the liver with a sharp scissors (Fig. 5). Bleeders were controlled by compres-
From the Departments of Surgery and Pathology, Kobe Children's Hospital, Kobe, Japan. Presented before the X X V Annual International Congress of the British Association of Paediatric Surgeons, Edinburgh, Scotland, June 28 30, 1978. Address reprint requests to Ken Kimura, M.D., Kobe Children's Hospital, l-l-l, Takakuradai, Sumaku, Kobe, Japan, #654. ~J 1979 by Grune & Stratton, Inc. 0022-3468/79/1401-0005501.00/0
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KIMURA ET AL.
DISSECTION
CUT SURFACE
SUPRA-PORTAL
fibrous cord portal fibrous mass
~.~ 9
~
r/,,7,.7.,e.
PORTAL
c . ~
hepatic.' ~ artery portal vein
INFRA-PORTAL
drain Fig. 1.
=LIVER
Dissected portal fibrous mass.
=PORTAL
sion with a sponge or repeated irrigation with warm saline. No electrocautery was used. Portoenterostomy or portocholecystostomy was then carried out using continuous or interrupted fine stitches placed about 1 mm from the edge of the transected stump of the portal fibrous mass. At the lateral corners, the sutures were placed carefully in order to avoid including tissue which may contain the bile ducts. In the group with "supra-portal" dissection, complete resection of the portal fibrous mass was carried out, which required incision 1 to 3 cm into the liver tissue. Excessive hemorrhage required large volume transfusion. When employing this technique, the denuded liver parenchyma was directly exposed to the interior of the jejunal loop after portoenterostomy. A liver biopsy specimen was routinely taken.
#
9
/
,,lIER
~(/ LEVELof DISSECTION SUPRA PORTALf
~
-
~
~
~
~
~
I INFRA-PORTAL
~
Extent of dissection.
~
fibrous
~ cord I ~ ~ ~ ~~ . ~ I
fine
~
-
veins
2,~;,,, .~
/ ......
PO!TAL;~ROUSMASS
'
PORTAL
Fig. 2.
w
/
Fig. 3.
FIBROUS MASS
' REMNANTof I I/HEPATICDUCT
Anatomy of porta hepaUs in biliary atresia,
V
hepaticJ artery
y-,,..
Penrose
drain
Fig. 4. Dissection of the inferior aspect of the portal fibrous mass. The fine veins have to be divided by ligation.
BILIARY ATRESIA
~
29
transected portalfibrous
~ ~~retractor hepatic_~ _~\~ ~ " artery p o r t a l ~ ?: ~ ' ~
/l"
~)~.~
Penrose drain Fig. 5.
The portal fibrous mass is transected.
greater than 50 # in any portion of the fibrous cord, which was considered as the remnant of the hepatic duct. In the group with "portal" type dissection, a number of bile ducts measuring 150-500 # were noted at the transected edge. In only one case, however, were bilateral hepatic ducts identified by microscopic examination. In the group with "supra-portal" dissection, the proximal end of the excised tissue was lined with the liver tissue by both gross and microscopic examination. No "bile lake" was found in these specimens. The portal fibrous mass was apparently totally included in the specimen, and it contained multiple bile ducts of 150-500 ~ diameter in all patients except one. In this unique case, fibrosis was general and few bile ducts were observed in any portion of the portal fibrous mass. The thickness of the portal fibrous mass was 2 5 mm. Bile ducts were found most frequently and with the greatest diameter in the portion closest to the liver parenchyma (Fig. 6). In the liver biopsy specimens, various grades of giant cell transformation, "triaditis," portal fibrosis and bile duct proliferation were seen. Bile plugs were seldom observed (Fig. 7).
Pathologic Examination
Bile Drainage
The proximal end of the excised specimen in all three groups was examined by multisecticin technique. In the "infra-portal" dissection specimens there was no bile duct
In this series, successful bile drainage was defined as a constant bile excretion via the enterostomy or the fecal stream for several months with a concurrent decrease of
Fig. 6.
The border of portal fibrous mass and liver ( X 184). Many bile ducts are present in this part of the portal fibrous mass.
30
KIMURA ET AL.
Fig. 7.
Giant cell transformation, triaditis, fibrosis and cholangitis are seen. (X 460l-
serum bilirubin to normal level and subsidence of clinical jaundice.
RESULTS
Of the 37 patients in the study, three underwent "infra-portal," 25 "portal" and 9 "supraportal" types of dissection. Of the 25 patients with "portal" type dissection, 19 (76.0%) demonstrated bile drainage. Eight of the nine infants in which "supra-portal" dissection was performed and all 3 in which "infra-portal" dissection was carried out had an inadequate volume of postoperative biliary drainage and remained icteric. The (A) age at operation and (B) degree of hepatic fibrosis seen in the three groups of patients were similar. Thus, there was an apparent difference in the clinical outcome in the three groups treated by different technique of hepatic portal dissection (Table 1). Seventeen of the 37 patients were operated upon before 1975. A "supra-portal" type dissection was employed as a planned trial in six consecutive patients in this group. During the same period, in three consecutive patients, an
"infra-portal" type dissection was carried out. The other 8 patients underwent "portal" type dissection and 6 were successful in respect to bile drainage. On the basis of these results and those of Kasai, a "portal" type dissection was planned in all of the 20 patients seen after 1975. Despite this intent in three patients, a "supra-portal" type dissection was actually carried out as demonstrated in the specimens removed. This was because the portal fibrous mass was so small that it was not identified and was included in the excised mass of the liver tissue. In none of these three was there successful biliary drainage. A "portal" type dissection was done in the remainTable 1. Noncorreetable Type Biliary Atresia Hepalic Portal Dissection Supra-portal Portal Infra-portal
Cases
Successful Bile Drainage
Survivors: Over 2 yr
9 25 3 37
1 (11.1%) 19 (76,0%) 0 (0%) 20
0 8 0 8"
"Survived for 2 to 7 yr without jaundice. 1 9 7 0 - 1 9 7 8 Kobe Children's Hospital,
BILIARY ATRESIA
31
ing 17 patients and in t3, bile drainage was successful. Of the total of 19 patients with successful biliary drainage, 4 died of cirrhosis or cholangitis and 2 of congenital heart disease, leaving 13 survivors, 8 of whom have survived for more than 2 yr without evidence of disease. Two of them were operated upon more than 7 yr ago and are enjoying school life and are completely free from symptoms. DISCUSSION
In 1959, Kasai ~ introduced hepatic portoenterostomy as a new surgical approach to the noncorrectable type of biliary atresia, suggesting the possibility of cure for this disease. It was approximately a decade thereafter that this technique became the standard procedure for the noncorrectable type of biliary atresia. During the 1960s, various techniques such as bile duct reconstruction using a prosthesis, 2 thoracic duct diversion to the alimentary tract, 3 diversion of hepatic lymph drainage, 4 and modifications of Longmire's procedure were attempted. All of these trials were ultimately unsuccessful. Although hepatic portoenterostomy was recognized as the definitive procedure by the end of the 1960s, 5 there has been a great variation in the technique of hepatic portal dissection based on each surgeon's concept of the ideal site for the transection of the structures at the porta hepatis. Until the early 1970s, it was believed that a "bile lake" locating in the liver parenchyma was the source of bile drainage after hepatic portoenterostomy. On the basis of this concept, dissection of the porta hepatis 2-3 cm into the liver parenchyma with or without currettage of the liver tissue was carried OUt. 6'7 This concept is largely abandoned today. It is agreed by many surgeons that a meticulous technique is required in the dissection of the porta hepatis to avoid damage to the bile duct remnants, s ~ Kasai has
emphasized the importance of the small bile ducts in the portal fibrous mass as the site of bile drainage based on studies demonstrating that these fine bile ducts have a continuity to the intrahepatic biliary system. This was documented by a reconstruction of the intrahepatic and portal biliary system using a multisection technique. ~4'~5 On the other hand, Suruga stressed in a recent report that identification and preservation of the right and left hepatic ducts at the porta hepatis are of importance.~6 According to this concept, bile drainage comes from the major hepatic ducts that are transected at a proper level in the porta hepatis, not from the multiple small bile ducts in the portal fibrous mass. Suruga has employed microsurgical technique for identification of the fine structures of the porta hepatis and has reported excellent results. ~6 Ueda believes that the optimal site of transection of the portal fibrous mass is located at the level of the first branch of the hepatic artery. ~~ He believes both the lateral hepatic ducts and the fine bile ducts in the fibrous mass are the site of bite drainage. Although concepts and techniques are different among these surgeons, the rate of successful bile drainage is as high as 90% in their recent series, t~ In our series, only patients with a transection of the portal fibrous mass d e m o n s t r a t e d adequate bile drainage. We believe the numerous small bile ducts included in the portal fibrous mass are the site of bile drainage, 8'~7 since an exhaustive pathologic examination of the excised specimen failed to identify large hepatic ducts in patients with successful bile drainage. In performing the portal dissection, it should be stressed that a total excision of the portal fibrous mass should be avoided. For transection of the portal fibrous mass at a proper level, frozen section examination of each fragment of excised tissue may be of help as recommended by Altman and Lilly. ~
REFERENCES
1. Kasai M, Suzuki S: A new operation for non-correctable biliary atresia; hepatic porto-enterostomy. Shujutsu 13:733 739, 1959 2. Shimura H, Nakamura Y, Sakai M: A limit &surgical treatment of biliary atresia. Shujutsu 17:872 887, 1963 3. Suruga K, Hirai Y, Naashima K, et al: Treatment of biliary atresia. Rinshogeka 23:861 868, 1969 4. Fonkalsrud EW, Kitagawa S, Longmire WP: Hepatic
lymphatic drainage to the jejunum for congenital biliary atresia. Am J Surg 112:188-194, 1966 5. Kasai M, Kimura S, Asakura Y, et aI: Panel discussion; treatment of biliary atresia. J Jpn Soc Pediatr Surgeons 5:455-461, 1969 6. Suruga K: Operation of biliary atresia. Shujutsu 24:543-549, 1970 7. Imaizumi S, Ishida M, Saito S, et al: Hepatic portoen-
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terostomy for biliary atresia. J Jpn Societ Pediatr Surg 9:497 503, 1973 8. Kasai M: Operation of Biliary Atresia. Geka 37:1432 1441, 1975 9. Suruga K, Kono S, Tsuchiya H, et al: Microsurgery for hepatic portoenterostomy. Shoni-geka-naika 8:1255 1260, 1976 10. Ueda T: Personal communication. May, 1978 11. Kimura S: Biliary atresia. Gekashinryo 17:51-54, 1975 12. Altman RP, Lilly JR: Technical details in the surgical correction of extrahepatic biliary atresia. Surg Gynecol Obstet 140:953 956, 1975
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13. Lilly JR: Surgical jaundice in infancy. Ann Surg 186:549 558, 1977 14. Oi R, Chiba T, Kasai M: Pathological findings of intrahepatic bile ducts in biliary atresia. Shoni-geka-naika 7:249-256, 1975 15. Chiba T, Sasano N: Studies on intrahepatic biliary ducts of porta hepatis in biliary atresia. Shoni-geka-naika 8:1157 1163, 1976 16. Suruga K, Kono S, Miyano T, et al: Treatment of biliary atresia: Microsurgery for hepatic portoenterostomy. Surgery 80:558 562, 1976 17. Kasai M: Treatment of biliary atresia with special reference to hepatic porto-enterostomy and its modifications. Prog Pediatr Surg 6:5-52, 1974