INTRODUCTION
Biliary atresia still remains one of the most intract- able gastrointestinal surgical diseases in infancy. The introduction of liver transplantation has revolution- ized the protocols for the treatment of this condition, but hepatic portoenterostomy, e.g., the Kasai proce- dure, is still the first-choice surgical treatment.
The main clinical manifestations of biliary atresia are persistent jaundice, clay-coloured faeces and hep- atomegaly. Although symptoms do not show a great difference, anatomical findings in the biliary tract vary from case to case. Only about 10 to 20% of the patients with biliary atresia have an extrahepatic bile duct large enough to perform a mucosa-to-mucosa anastomosis to the intestine. These correctable-type cases can undergo hepatico-enterostomy. The re- maining patients, about 85 to 90%, do not have any bile duct amenable to a conventional anastomosis.
For these non-correctable types of patient, hepatic portoenterostomy should be performed.
Hepatic porto-enterostomy was first devised in 1957 as a corrective operation for patients with a non- correctable type of biliary atresia. The basis of this procedure is that the intrahepatic bile ducts are pat- ent in early infancy and minute intrahepatic bile ducts are present in the cone-shaped fibrous tissue, replacing extrahepatic biliary radicles. In hepatic portojejunostomy, the extrahepatic bile ducts, in- cluding fibrous remnants at the porta hepatis, are to- tally removed and bile drainage established by anas- tomosis of an intestinal conduit to the transected surface at the porta hepatis. Microscopic biliary structures at the liver hilus drain bile into the intesti- nal conduit and, in time, an autoapproximation
between the intestinal and ductal epithelial elements occurs.
Successful hepatic portoenterostomy depends on early diagnosis and operation (preferably before 60 days of age), adequate operative technique, preven- tion of post-operative cholangitis, and precise post- operative management.
Post-operative cholangitis is the most frequent and serious post-operative complication, resulting in fatal septicemia or re-obliteration of hepatic por- toenterostomy. Although various modified recon- struction procedures for prevention of cholangitis exist, the authors now perform a double-valved (spur valve and intussuscepted valve) hepatic portojeju- nostomy. The incidence of cholangitis, however, re- mains almost the same as the original Kasai proce- dure using a long Roux-en-Y anastomosis with a length of the limb from 50 to 70 cm. As late complica- tions, liver cirrhosis and portal hypertension and/or hypersplenism have been documented. It has been shown that both a late operation and complication of cholangitis aggravate hepatic fibrosis and induce portal hypertension.
As to pre-operative management, in addition to the routine pre-operative care for abdominal surgery, vitamin K, 1~2 mg/kg per day, is usually given for sev- eral days before surgery. The bowel is prepared with tobramycin sulfate and metronidazole orally, at a dose of 10 mg/kg per day, each starting 36 h before operation. Early discontinuation of oral feeding and enema are properly enforced. Blood is cross- matched, and pre-operative broad-spectrum antibio- tics are administered.
Ryoji Ohi, Masaki Nio
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The operation is undertaken under general anaesthe- sia with tracheal intubation. The patient is placed in the supine position. A pillow is put under the back of the patient for better exposure. A laparotomy is per- formed through an right subcostal incision from the
costal margin to the medial border of the left rectus muscle. Some pediatric surgeons recommend mobi- lizing and completely exposing the liver to allow ade- quate visualization of the porta hepatis.
Figure 33.1
Figure 33.2
A liver biopsy is taken. A catheter is inserted into the small gallbladder to take a cholangiogram. After the occlusion of the extrahepatic bile duct is confirmed by cholangiography, the gallbladder is freed from the liver and dissection is advanced along with the cystic duct toward the common hepatic duct. The dissec- tion is helped by the use of binocular loupes.
A superficial peritoneum on the hepatoduodenal ligament is opened and the anatomy of the involved bile duct and the hepatic arteries is assessed. Dilated lymphatic channels around the hepatoduodenal liga- ment should be carefully ligated and divided to avoid excessive lymphatic fluid loss. The common bile duct remnant is carefully dissected, since it is often adher- ent to the surrounding tissues.
Figure 33.1
Figure 33.2
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Figure 33.3, 33.4
The common bile duct is clamped and divided adja- cent to the duodenum. After the common bile duct is severed, it is pulled up and the hepatic duct remnant is freed from underlying hepatic arteries and the portal vein. The hepatic duct usually transforms into a cone-shaped fibrous tissue, which is situated crani- al to the bifurcation of the portal vein. It continues to the portal tracts in the liver. Even if a cyst-like struc- ture is present, it should be removed and should not be used for anastomosis to the intestine.
The separation of the fibrous remnants from the right and left portal veins is carefully advanced pos- teriorly. The bifurcation of the right and left portal veins must be retracted to obtain proper exposure of the porta hepatis. Several small branches bridging from the portal vein to the fibrous remnants are identified and divided between ligatures, facilitating downward displacement of the portal vein. The pos- terior aspect of the fibrous remnants is exposed deep and wide enough behind the bifurcation of the portal vein.
Common bile duct
Cystic duct
Portal vein Stump of common bile duct
Figure 33.3 Figure 33.4
Figure 33.5
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The dissection between the anterior aspect of the fi- brous remnants and the quadrate lobe of the liver is also sharply and sufficiently advanced. The dissec- tion of the fibrous remnants must be accomplished
as widely as possible, i.e., to 5 to 6 mm proximal of the anterior branch of the right hepatic artery at the right side and to the umbilical point of the left portal vein at the left side.
Figure 33.6
The transection of the fibrous remnants is carefully carried out by the use of small-sized round-shaped scissors or a sharp knife at the level of the posterior surface of the portal vein. Although some surgeons confirm the presence of microscopically patent ducts at the level of anastomosis by using frozen sections during surgery, the authors do not use frozen-section guidance because they always transect the portal bile
duct remnants at the same level. Haemorrhage from the cut surface of the porta hepatis is occasionally considerable. Irrigation with warm saline stops the bleeding, usually within 10 min. Ligation or cautery should not be applied because of the possibility of accidental obliteration of small bile ducts that may be opening on the transected surface.
Figure 33.5
Figure 33.6
Figure 33.7
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The next step is the construction of a Roux-en-Y loop of the jejunum. A Roux-en-Y anastomosis with the ascending limb, approximately 50 cm in length, is made. The end of the gastric limb of the intestine is anastomosed not to the back, but to the lateral side of the hepatic limb of the intestine. Most surgeons cur-
rently do not use a cutaneous stoma because of the frequent bleeding that occurs when the patient devel- ops portal hypertension, and the technical difficul- ties that arise when subsequent liver transplantation may become necessary. Further, there was no reduc- tion in incidence of cholangitis.
Figure 33.8, 33.9
A spur valve (A) and an intussuscepted valve (B) are made, which may prevent cholangitis. Three centi- metres of the antimesenteric half of the seromuscu- lar coat is removed from the biliary limb proximal to the anastomosis. The gastric and biliary limbs are then coapted over the denuded mucosa with sutures along the edges of the incised seromuscular layer.
Subsequently, the intussuscepted valve is created in
the biliary limb proximal to the spur valve. The vasa recti of the conduit are divided by the width equal to the intestinal diameter. The seromuscular layer of the devascularized intestine is then removed. The de- nuded segment and an equivalent length of its proxi- mal portion of the intestine is intussuscepted into the distal segment. This valve with fixed with eight to ten interrupted sutures.
Figure 33.7
Figure 33.8 Figure 33.9
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Figure 33.10
After completion of valves, the hepatic limb is brought up retrocolically. The end of the intestine is anastomosed around the transected end of the fi- brous remnants at the porta hepatis by full-thickness interrupted sutures using 5/0 absorbable sutures.
Stitches must not be placed in the transected surface of the fibrous remnants in which minute bile ducts are present. After all interrupted sutures of the poste- rior row are placed in position, they are tied.
Figure 33.10
Figure 33.11, 33.12
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The anterior row of suture is made as same as the posterior row of the anastomosis. Again, it should be emphasized that stitches must not be placed in the transected surface of the fibrous remnants, but around the transected area.
Interrupted seromuscular sutures are made using 5/0 silk between the anterior wall of the jejunum and the quadrate lobe of the liver to fix the intestine at the porta hepatis. Several stitches are placed between the posterior wall of the jejunum and the hepatoduoden- al ligament. Irrigation of the abdominal cavity with saline should be done sufficiently. Intestine, especial- ly the jejunum including the biliary limb is carefully placed in order in the abdominal cavity to prevent il- eus. A Penrose drain is placed in the foramen of Win- slow. After the intestine in the upper abdomen, in- cluding hepatic limb, is covered by Seprafilm, the ab- dominal cavity is closed in layers.
Patients are placed in an oxygen tent and intrave- nous fluid is given.
Decompression of the gastrointestinal tract should be performed by nasogastric aspiration and enema.
Oral feeding is usually started on the fifth or sixth post-operative day when bowel activity resumes. Pre- vention of post-operative cholangitis is a main goal of the post-operative management. For this purpose, we routinely use antibiotics, choleretics and steroids as post-operative medication. For antibiotics, amika- cin sulfate in a dose of 8 mg/kg per day, q8h, is ad- ministered for 7 days after the operation and then cephalosporin in a dose of 50–80 mg/kg per day is given intravenously for a few months until the serum bilirubin level falls below 2 mg/dl. As choleretics, intravenous injection of 3 ml 10% dehydrocholic acid diluted with 7 ml 5% glucose is started on the day of operation and is given every 12 h. Ursodeoxycholic acid at a dose of 0.5 g/day is also given orally. Pred- nisolone, 10 mg twice a day intravenously, is started after the seventh post-operative day. Steroid therapy is continued intravenously for 4 days and it is switched to oral administration of 20 mg bid pred- nisolone every other day for several months.
Figure 33.11
Figure 33.12
CONCLUSION
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Between 1953 and 2001, a total of 321 patients with bil- iary atresia underwent surgery in our hospital.
Among them, 43 patients underwent double-valved hepatic portoenterostomy (the valved procedure).
Bile drainage after surgery was obtained in 95% of the cases. The incidence of post-operative cholangitis was 56% in the valved procedure. Of the cases treated by this procedure, 49% are still alive without jaundice after hepatic portoenterostomy alone, and 30% are alive after subsequent liver transplantation.
The combination of hepatic portoenterostomy with subsequent liver transplantation is the treat- ment of choice for patients with biliary atresia. It is important, however, to attempt to keep the patient’s own organ by continuing efforts to achieve the best possible results with hepatic portoenterostomy.
The authors’ current strategy for surgical treat- ment for patients with this disease include (1) early diagnosis, including prenatal diagnosis, (2) hepatic portoenterostomy without stoma formation, (3) close postoperative care, especially for prevention of post- operative cholangitis, (4) revision of hepatic por- toenterosotmy only in selected cases who showed good bile drainage after the first procedure, (5) early liver transplantation in patients with absolutely failed hepatic portoenterostomy, (6) avoidance of laparotomy for the treatment of oesophageal varices and hypersplenism (injection sclerotherpy and splenic artery embolization), and (7) consideration of exploratory laparotomy or primary liver trans- plantation for patients with advanced liver disease at the time of referral.
SELECTED BIBLIOGRAPHY
Kasai M (1974) Treatment of biliary atresia with special refer- ence to hepatic porto-enterostomy and its modifications.
Prog Pediatr Surg 6 : 5–52
Ohi R (1988) A history of the Kasai operation: hepatic portoen- terostomy for biliary atresia. World J Surg 12 : 871–874 Ohi R (1991) Biliary atresia: modification to the original por-
toenterostomy operation. In: Howard ER (ed) Surgery of liver diseases in children. Butterworth, Oxford, pp 72–77
Ohi R (2000) Biliary atresia; a surgical perspective. Clin Liver Dis 4 : 779–804
Ohi R, Nio M (1998) The jaundiced infant: biliary atresia and other obstructions. In: O’Neill JA, Rowe MI, Grosfeld JL, Fonkalsrud EW, Coran AG (eds) Pediatric Surgery, vol II.
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