Portal venous pressure in biliary atresia

Biliary atresia (BA) is characterized by a variable degree of fibrosis within the liver, causing portal hypertension sometimes evident at the time of presentation.AimThe aims of this study are to measure portal venous pressure (PVP) at time of Kasai portoenterostomy (KP) and to investigate the value of surrogate indices.MethodsAt the time of KP and before any liver manipulation, an attempt was made to recanalize the umbilical vein, allowing a catheter to be sited. Preoperative noninvasive variables included maximum splenic diameter (on ultrasound); platelet count, aspartate aminotransferase, and bilirubin; and the aspartate aminotransferase/platelet index ratio (APRi). Clearance of jaundice was defined as achieving a bilirubin of less than 20 μmol/L. Data are quoted as median (range). Nonparametric statistical tests were used, and P < .05 was regarded as significant.ResultsPortal venous pressure measurements were available in 61 infants, who underwent a KP during the period February 2007 to October 2010. Median age at KP was 52 (19-151) days. Median PVP was 9 (3-26) mm Hg and was significantly lower in those with isolated (n = 47) BA vs cytomegalovirus-associated BA (n = 6) (8 vs 17 mm Hg; P = .02). Overall, PVP correlated with spleen size (r_s = 0.38; P < .0001), APRi (r_s = 0.5; P < .0001), bilirubin (r_s = 0.38; P = .001), and age at KP (r_s = 0.33; P = .0004).Thirty-three infants (56%) cleared their jaundice (to <20 μmol/L). There was no significant difference in median PVP (7 vs 9.5 mm Hg; P = .1) between these groups. To date, 6 children have undergone endoscopy, with 2 requiring intervention for significant varices. Neither infant at time of KP had elevated PVP (3 and 9 mm Hg).ConclusionsPortal venous pressure is a key variable in the assessment of the BA liver, and differences can be shown depending on underlying cause (eg, cytomegalovirus status). Furthermore, it has modest correlation with noninvasive indices (eg, spleen size and APRi) but appears of limited use when used as prognostic index of outcome and indeed in the later development of varices.     

Portal venous velocity in the follow-up of patients with biliary atresia after Kasai portoenterostomy

Background/Purpose: Liver transplantation is the only therapeutic option for biliary atresia (BA) patients with failed Kasai portoenterostomy (PE). The indication for and timing of liver transplantation in these patients are important management issues. The aims of this study are to evaluate the relationship between portal venous velocity (PVV) and clinical and biochemical indicators of liver function in BA, and to examine the role of PVV in the postoperative surveillance of these patients. Methods: Twenty-nine children (mean age, 8 years 4 months) who had PE for BA underwent Doppler ultrasonography to evaluate PVV. Using regression analysis, these findings were correlated with biochemical indicators of liver function. The Mann-Whitney test was used to detect any significant differences in PVV between the Child-Pugh A group and combined Child-Pugh B and C group patients. Results: The mean [plusmn] SD PVV for the Child-Pugh A group and the combined Child-Pugh B and C group was 17.1 cm/s [plusmn] 8.9 cm/s and 10.2 cm/s [plusmn] 3.3 cm/s, respectively, and this difference was statistically significant (P = .037). Conclusions: Doppler ultrasonography measurement of PVV provides adjunctive information to the clinical and biochemical assessment of the liver function status in BA. Its use in the postoperative surveillance of these patients is helpful in identifying the need for and in timing of liver transplantation. J Pediatr Surg 37:873-876.     

Portal vein dynamics in biliary atresia

A prospective sonographic investigation of portal vein caliber was done in 40 children with biliary atresia (n = 37) and biliary hypoplasia (n = 3) from 1 to 135 months old. The patients were divided into two groups according to hepatobiliary function (HBF). HBF was determined by serum bilirubin (greater than less than 1.5 mg/dL), serum albumin (greater than less than 3.5 g/dL), and bile bilirubin excretion (greater than less than 6 mg/d). There were 21 patients with "good" and 19 patients with "poor" HBF. The portal vein caliber, measured just proximal to its bifurcation, was 3 to 16 mm (median 7 mm), in the good HBF patients and 2 to 6 mm (median 4 mm) in the poor HBF group, the difference being statistically significant (P less than .01). Sequential studies of portal vein caliber were done in 30 patients. In the 15 patients with good HBF there was a progressive increase in portal vein caliber in nine, no change in six, and no patient had a decrease in caliber. Of the 15 patients with poor HBF, eight patients had a decrease in portal vein caliber, seven had no change, and no patient had an increase in caliber. The results indicate that patients with biliary atresia and poor HBF have an absolute and/or relative decrease in portal vein caliber and that the decrease may be progressive. Since portal vein anastomosis is essential to successful liver transplantation, referral must be done before portal vein shrinkage eliminates this option.     

Portal hypertension after successful hepatic portoenterostomy in biliary atresia

From 1953 through 1984, we have operated on 225 cases of biliary atresia, and 95 patients are presently surviving. Portal hypertension with esophageal varices was endoscopically confirmed in 26 of 66 patients (39%) examined, 14 with and 52 without jaundice. All these patients except two had had frequent episodes of postoperative cholangitis. Eight patients have undergone treatment for portal hypertension. The treatment for variceal bleeding in jaundice-free infants with biliary atresia should be initiated conservatively, including endoscopic sclerotherapy. The results of our experience, however, justifies the employment of shunt procedures for patients older than 6 or 7 years of age.     

Portal vein complications after liver transplantation for biliary atresia

The objective of this report is to review portal complications (PC) after pediatric liver transplantation (LT) for biliary atresia (BA) in the Bicetre surgical series. From January 1, 1988, to February 28, 1995, 96 children with BA underwent 115 LTs Portal anastomosis was done on either the recipient portal vein (n = 85) or superior mesenteric vein (n = 11). No antiaggregative agents were administered postoperatively. Median follow-up was 50 months (range, 12 to 97). Nineteen PC (16.5%) occurred in 17 recipients: 16 portal thrombosis (PT) and 3 portal stenosis (PS). Fifteen instances of early PT occurred between days 0 and 17 (median, day 2). Emergency thrombectomy was performed in 9 cases (successful in 5). Three children underwent a secondary portosystemic shunt (successful in 2). Three PS were cured by either surgery or balloon dilatation. Four children died, 3 are alive with portal hypertension (PHT), and 10 are alive without PHT. Three- year patient actuarial survival is 82.4% in PC cases and 82% in others (NS). Significant risk factors of PC are young age and weight at the time of LT, small portal vein, and emergency LT. Analysis of our own results and review of the literature suggest that prevention of PC depends primarily on appropriate surgical technique. Reduction of postoperative hypercoagulability may also play an important role: a meta-analysis of 1,257 published pediatric LT show an overall risk of PT of 2.2% in teams using aspirin with or without dipyridamole compared with 7.8% when no antiaggregative agents are given (P = .0001). (Liver Transpl Surg 1997 Jul;3(4):351-8)     

Portal decompression by interposition mesocaval shunt in patients with biliary atresia

Surgical correction of portal hypertension in infants and children has often been frustrated by limitations imposed by the diminutive vessel size. During the past 18 mo, five patients from 1 to 7 yr of age and weighing between 9 and 19 kg, have required portal decompression for bleeding from esophageal varices. In two patients, a previous dissection in the area of the porta hepatis imposed a further technical obstacle. Both of these patients had biliary atresia and one had undergone a successful portoenterostomy (Kasai).¹ Success of the interposition mesocaval graft in adult patients led us to adopt this procedure in our five patients. Autogenous jugular vein was utilized for construction of the shunt in the smaller patients. Radiographic confirmation of shunt patency has been obtained 1 yr postoperatively.     

Corticosteroid therapy in biliary atresia

Sixteen patients with biliary atresia had 44 steroid courses for treatment of cholangitis or diminution of bile flow following Kasai hepatic portoenterostomy operations. A "blast" type (high dose/short duration) steroid administration was employed to potentiate the choleretic and anti-inflammatory effect. There was a significant augmentation of bile flow and a reduction in maximum temperature, serum bilirubin, and alkaline phosphatase.     

Maternal microchimerism in biliary atresia

Background

The aim of this study was to determine the existence and extent of maternal microchimerism in the livers of biliary atresia (BA) patients.

Methods

Two series of investigations were performed based on the sex of our subjects. Subjects for series I were men, of which 6 had BA. Livers were analyzed using X and Y chromosome probes and fluorescent in situ hybridization. Subjects for series II were woman. Nine BA cases and their mothers were HLA typed (class I). Daughter livers were also tested for antibodies to maternal and other HLA. Two cases of neonatal hepatitis, 2 cases of Alagille syndrome, and 1 case of Byler syndrome acted as controls.

Results

All male BA livers were found to contain a mixture of cells with 1 and 2 X chromosomes (ie, XY or XX). All livers from male controls had only 1 X chromosome (ie, XY). All female BA subjects had varying intensities of antimaternal HLA class I (HLA-A) antibodies in their bile duct epithelium and hepatocytes (strong, 5; mild, 3; weak, 1). The liver from the female control did not display any antimaternal HLA class I antibodies (HLA-Ab).

Conclusion

Our preliminary data appear to show that maternal microchimerism is present within the livers of patients with progressive postnatal type BA. We suggest that BA could in fact be a graft-vs-host disease masquerading as an autoimmune reaction triggered by maternal microchimerism, and we intend to pursue this hypothesis further to clarify the etiology of BA.     

Multiple intrahepatic biliary cysts in children with biliary atresia

Purpose: The aim of this study was to investigate the incidence, clinical course, and outcome of multiple intrahepatic biliary cysts in patients with biliary atresia. Methods: From 1992 to 2000, 154 patients with biliary atresia were examined for intrahepatic cysts. They were followed and examined routinely using abdominal ultrasonography. Results: Twenty-three patients had single intrahepatic cysts, and 16 patients had multiple cysts. The incidence of intrahepatic cysts in these patients were 25.3% (39 of 154) for all kinds of cysts and 10.4% (16 of 154) for multiple intrahepatic cysts. Of the 16 patients with multiple cysts, 13 (81.3%) had jaundice, and 15 (93.8%) had a history of cholangitis before cysts were detected. Image studies showed multiple discrete ovoid or round intrahepatic biliary cysts with various sizes along the biliary trees. The cysts decreased in size or number in 7 patients after antibiotic treatment and disappeared in only one patient. The mortality rate was higher in patients with multiple cysts than in those with single cysts (P = .037). Conclusions: Multiple intrahepatic biliary cysts are a common complication in patients with biliary atresia and suggests a poor prognosis. Prolonged parenteral antibiotics treatment should be administered when signs of biliary infection appear. J Pediatr Surg 37:1183-1187.     

Portal vein reconstruction in pediatric living donor liver transplantation for patients younger than 1 year with biliary atresia

Background/PurposeInfants with biliary atresia undergoing living donor liver transplantation (LDLT) are at increased risk of portal vein (PV) complications because of their smaller vascular caliber and sclerosis because of previous Kasai portoenterostomy and recurrent cholangitis.MethodOf 154 children who underwent transplantation between November 2005 and January 2011, 34 with biliary atresia received a transplant while younger than 1 year. Six patients underwent PV reconstruction with an interposition vein graft, and the others underwent the branch patch technique. The clinical characteristics of those who underwent the interposition reconstruction or the branch patch technique were compared, and the PV complications were assessed.ResultsPortal vein complications occurred in 5 patients (14.7%) in the branch patch group. There were 4 patient deaths, and all of them had received branch patch reconstruction. The branch patch reconstruction cases with a sclerotic small caliber (<4 mm) determined by using preoperative ultrasonography showed a significantly high mortality rate (44.4%). All patients with interposition vein graft reconstruction are still alive with excellent graft function without anticoagulation therapy.ConclusionThe interposition vein graft appears to be a feasible option with better graft survival and less PV complications when performing LDLT for biliary atresia in infants younger than 1.     

Reoperation in patients with biliary atresia

Twenty-seven reoperations were done on 23 patients among 100 infants with biliary atresia who have been treated at Tohoku University Hospital between 1971 and 1981. Nineteen patients had a single reoperation and 4 patients had 2 reoperations. We present the results and the role of reoperation in biliary atresia patients in our institution. Excellent bile drainage after reoperation was obtained in 13 of 15 patients with good bile flow after the initial operation. On the contrary, good bile drainage was not obtained by reoperation in 8 of 12 cases without active bile flow after the initial operation. Cessation of bile flow after successful initial operation is an absolute indication for reoperation. Aggressive reoperations under proper indications improve the surgical results in biliary atresia patients.     

Advances in treatment of biliary atresia

During 29 years from 1953 through 1981, 224 patients with congenital biliary atresia were treated and 200 of them underwent corrective operation at the Second Department of Surgery, Tohoku University Hospital. With the advance of surgical techniques and early diagnosis of biliary atresia, the rate of jaundice disappearance improved from the initial 15 per cent to the recent 69 per cent. Important factors contributing to the improvement of operative results included (1) an early operation, preferably within 60 days after birth, (2) a precise dissection and adequate transection of the porta hepatis, (3) prevention of post-operative cholangitis and (4) reoperation if indicated. Delayed operation and occurrence of post-operative cholangitis affected not only immediate but also long term operative results. Although, nearly all the patients with biliary atresia had portal hypertension at the time of surgery, the portal vein pressure decreased after successful operation, mainly due to improvement in portal venous circulation of the liver. It is considered from recent operative results that more than 80 per cent of patients with biliary atresia can be cured when an adequate corrective operation is carried out before 60 days of age and post-operative cholangitis is prevented.     

Portal venous system injuries

Traumatic injuries of the portal venous circulation occur infrequently but often pose management problems. During the past 13 years, 94 patients have been treated at the Ben Taub General Hospital for traumatic injury to the portal venous system, including 37 portal, 45 superior mesenteric, seven splenic, and nine inferior mesenteric venous injuries. Injury resulted from penetrating wounds in all but 17 patients. Associated vascular injuries were present in 80 patients. Twenty-six patients died from failure to control hemorrhage. Six deaths resulted from postoperative complications. Operative approach necessitated lateral venorrhaphy in 66 patients, ligation in 23, end-to-end anastomosis in one, saphenous vein mesocaval shunt in two, end-to-side portacaval shunt in one, and clamping and packing in five. In spite of numerous associated vascular and visceral injuries, portal venous injuries can be successfully managed utilizing generally available vascular reconstructive techniques.     

Congenital biliary atresia and congenital biliary dilatation in siblings.

Congenital biliary atresia or congenital biliary dilatation in siblings have been reported, although the genetic influences in these diseases are considered to be obscure. We report a case of congenital biliary atresia and congenital biliary dilatation in sisters.