Liver transplantation in children.
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Child↗
Biomedical subjects
J R Lilly
Publications and source records attributed to J R Lilly.
Surgical jaundice in infants: other than biliary atresia.
After biliary atresia, the lesions responsible for surgical jaundice in the infant are perforation of the common bile duct, choledochal cyst, bile plug syndrome, and miscellaneous congenital lesions in descending order of frequency. Perforation of the common bile duct commonly presents with an insidious onset of bilious ascites and is best treated by simple peritoneal drainage. Choledochal cyst usually presents later in childhood but presents in infancy if obstruction of the biliary tree is complete or near complete. Excision is the treatment of choice. Any condition leading to alteration in bile composition may cause bile plug syndrome. Spontaneous resolution is the rule: occasionally, intraoperative irrigation is necessary. Most miscellaneous lesions lend themselves to operative correction.
Choledochal Cyst↗
Biliary atresia and the polysplenia syndrome.
There is a widely held but unsubstantiated belief that in infants with biliary atresia and coexisting polysplenia syndrome, the Kasai operation fails. An equally poor prognosis has been forecast for patients with this complex treated by liver transplantation. From 1975 to 1989, 16 of 131 infants with biliary atresia (12%) had one or more components of the polysplenia syndrome: polysplenia (8), intestinal malrotation (12), preduodenal portal vein (6), absent inferior vena cava (6), aberrant hepatic artery (4), or abdominal heterotaxia (4). Although technically more demanding, 12 of the 15 polysplenia patients achieved biliary drainage after operation. (One patient had exploration only, because of parental preference). Four children are alive; two are anicteric and well at ages 5 and 8 following Kasai's operation, and two by virtue of liver transplantation done at ages 4 and 7. Three of the five patients who had liver transplantation died. Acturial survival was 44% at 5 years, not significantly different from that of the 115 nonpolysplenia patients (48%). When associated with the constellation of anomalies known as the polysplenia syndrome, biliary atresia is most likely caused by an early (at approximately the fifth week) embryonic insult. The anomalies do not preclude successful biliary reconstruction using the Kasai procedure or successful liver transplantation.
Abnormalities, Multiple↗
Surgery for biliary atresia.
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Anastomosis, Roux-en-Y↗
The blind loop syndrome in children.
Anatomical abnormalities of the small bowel that cause intestinal stagnation result in bacterial overgrowth and a blind loop syndrome (BLS). Bacterial breakdown of bile salts and deamination of protein lead to malabsorption, steatorrhea, and fat-soluble vitamin deficiencies. Four children developed BLS as a complication of necrotizing enterocolitis, jejunal atresia, gastroschisis, and biliary atresia. BLS was suggested by abdominal pain, feculent vomiting, steatorrhea, and hypoalbuminemia. Dilated, stagnant bowel loops were demonstrated in each instance by upper gastrointestinal contrast study. Positive intestinal bacterial aspirates were confirmatory. Antibiotic treatment in two patients improved symptomatology but all children ultimately required surgery. Surgical procedures consisted of blind loop resection, intestinal plication, and catheterization of the bilioenteric conduit. All patients are now asymptomatic but one child suffers from parenteral nutrition-related cirrhosis and another requires chronic antibiotic therapy.
Bacterial Infections↗
Biliary atresia registry, 1976 to 1989.
The Registry provides information about 904 children with biliary atresia from more than 100 institutions. There was a 1.4 to 1 female predominance; racial distribution was 62% caucasian, 20% black, 11% Hispanic, 4.2% asian, and 1.5% American Indian. Eight hundred sixteen (90%) underwent corrective surgery (median age at operation, 69 days). Intraoperatively, 70% had totally obliterated extrahepatic bile ducts, 22% had patency of the gallbladder and distal common duct, whereas only 8% had "correctable" biliary atresia (proximal duct patency). A variety of reconstructions were used, but the majority of patients had a Roux-en-Y portoenterostomy with or without exteriorization. Follow-up was available for 670 children (74%) with average length of follow-up of 5 years (range, 1 to 16 years). Five-year actuarial survival was 48% following Kasai's operation, but was less than 10% (at 3 years) if no operative correction was done. Survival was unaffected by sex, type of reconstruction, or cholangitis. Predictors of a bad outcome were (1) caucasian race; (2) operative age greater than 60 days; (3) presence of cirrhosis at initial biopsy; (4) totally nonpatent extrahepatic ducts; (5) absent ducts at the level of transection in the liver hilus; and (6) subsequent development of varices or ascites. Identification of factors predictive of the ultimate outcome provide a basis for either continued efforts with management of Kasai's operation or for early referral for liver transplantation.
Age Factors↗
Agenesis of the extrahepatic bile ducts: report of five cases.
Five patients were encountered in whom agenesis either of the proximal extrahepatic biliary ducts (four patients) or total absence of the extrahepatic bile ducts and gall bladder (one patient) were evaluated. Jaundice was diagnosed from at birth to 3 weeks of age (average, 1.2 weeks of age) in these five patients. The patients' ages ranged from 2 to 8 weeks at the time of surgical exploration. Findings at surgery showed either absence of the entire extrahepatic biliary ducts or proximal bile duct remnants, no evidence of an inflammatory process, and no fibrous mass present at the portahepatis. Liver biopsy specimens showed histological evidence of cholestasis, minimal bile duct proliferation and fibrosis, and nearly complete absence of inflammation. In three patients in whom a portocholecystostomy was performed, no bile flow was obtained. Two patients underwent surgical exploration and liver biopsy only. One patient died as a result of severe congenital heart disease at 3 months of age. Four patients have undergone successful hepatic transplantation. These patients are now 10 months to 6 years of age. In our review of the literature, we were unable to find any reports of bile duct agenesis despite the fact that it appears to be a known phenomenon. We conclude that patients with biliary agenesis have early onset of jaundice when compared with patients with biliary atresia, absence of inflammation at the portahepatis at the time of surgical exploration, as well as on biopsy of the liver. Portoenterostomy or portocholecystostomy are likely to fail. We believe that liver transplantation is the treatment of choice for this rare entity.
Biliary Atresia↗
Portal venous velocity in biliary atresia.
From December 1986 to April 1989, 38 patients with biliary atresia (eight newly diagnosed) were evaluated with doppler ultrasound of the portal venous system. Peak and mean velocities were computer derived from the spectral waveform. Good velocity was greater than 15 cm/s, intermediate velocity was 8 to 14 cm/s, abnormal velocity was less than 7 cm/s or hepatofugal. Patients were grouped according to clinical status: group 1 (n = 14), normal liver function; group 2 (n = 15), recurrent cholangitis; group 3 (n = 2), established bile drainage but complicated cirrhosis; group 4 (n = 7), failed portoenterostomy. All patients with normal liver function (group 1) had good or intermediate velocities. Thirteen of 15 patients with recurrent cholangitis (group 2) had good or intermediate velocities. Both patients in this group with abnormal velocities required transplantation. In group 3 the patient with abnormal velocity is on the transplant waiting list. In group 4, abnormal velocities preceded or coincided with deterioration of liver function in five of seven patients. Doppler ultrasound provides useful anatomic information, determines direction of flow, quantitates velocity of flow, and, when performed serially, provides adjunctive information on liver status in children with biliary atresia. These preliminary results suggest that patients with abnormal or significantly decreasing velocity are destined for transplantation. Patients with good portal venous velocity warrant ongoing, aggressive surgical management.
Adolescent↗
Congenital biliary tract disease.
Jaundice in the pediatric patient requires prompt and directed evaluation. This dictum is highlighted in infants with biliary atresia, in whom the progressive sclerosing process results in complete obliteration of patent but microscopic hilar biliary structures by 4 months of age. Kasai's operation, if done before that time, will re-establish bile drainage in 90% of infants. One fourth to one third of patients achieve long-term jaundice-free survival. Complications of cholangitis, portal hypertension, and fat malabsorption are experienced by many patients. In children with early or late operative failure, liver replacement now offers legitimate hope for extended survival. Choledochal cyst is a conglomerate of pancreaticobiliary anomalies consisting of a choledochal cyst, a common-channel-type pancreaticobiliary junction, intrahepatic cystic disease, and partial obstruction of the distal common bile duct. Many patients have one or more of these malformations. It is now widely accepted that the preferred treatment of choledochal cyst is total excision of the diseased biliary duct with reconstruction by Roux-en-Y choledochojejunostomy. "Internal" excision avoids injury to other structures in the hepatoduodenal ligament, particularly if pericystic inflammation is present. Congenital perforation of the common bile duct responds in most cases to simple peritoneal drainage of the perforation. Retention of the tube cholecystostomy is useful for subsequent cholangiographic follow-up. Tube cholecystostomy may also be useful for irrigation of the biliary tract in infants with inspissated bile syndrome.
Biliary Atresia↗
Biliary atresia: early determination of prognosis.
Analyses of bile bilirubin during the first month after Kasai operations, and of the liver biopsies obtained at the time of initial surgery, were done in 67 patients with biliary atresia. Bilirubin excretion (milligrams per day) was determined as the product of the bile volume and its concentration. Operative liver biopsies were evaluated for fibrosis, bile duct proliferation, bile stasis, giant cell transformation, and parenchymal degeneration; the severity of each abnormality was graded on a scale of 0 to 4. A forward stepwise regression procedure using the Cox proportional hazards model identified the relationship between survival and covariants. Thirty-nine of 67 patients died. Of these, 38 excreted less than 6 mg of bilirubin per day during the first postoperative month. The other patient died of a coexisting anomaly. Nine other patients who excreted less than 6 mg of bilirubin per day are alive but are either jaundiced or awaiting transplantation. Nineteen patients who excreted greater than or equal to 6 mg of bilirubin per day are alive (mean follow-up, 61 months) with normal or near-normal liver function. The severity of liver fibrosis, bile duct proliferation, and bile stasis did not correlate with survival, whereas giant cell transformation and parenchymal degeneration were highly significant (P less than or equal to .000 and P less than or equal to .0003, respectively). Twenty-six infants with grade 1-4 giant cell transformation or grade 2-4 parenchymal degeneration had a mean survival of 11 months.(ABSTRACT TRUNCATED AT 250 WORDS)
Bile↗
Cholangitis after the Kasai operation for biliary atresia.
One hundred seventy-nine episodes of cholangitis in 28 consecutive patients having a Kasai operation for biliary atresia during the past 3 1/2 years were analyzed. The diagnosis was made primarily on the basis of unexplained fever (greater than 38.0 degrees C). An increase in serum bilirubin or a decrease in bile volume and in bile bilirubin concentration were often confirmatory, but other laboratory data including serum hepatic enzymes and blood and bile culture data were of little or inconsistent value. All patients were treated with systemic antibiotics. The best results were obtained with third-generation cephalosporins or imipenemcilastatin with the addition of aminoglycosides in recalcitrant cases. Antibiotic therapy was modified if defervescence did not occur within the first 24 hours. Cholangitis refractory to antibiotics was aggressively treated with pulse steroid therapy, and in some cases, operative intervention, both with good clinical success (60% and 73%, respectively).
Anti-Bacterial Agents↗
Intralesional steroid injection in the management of esophageal stricture.
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Child, Preschool↗
Splenic embolization in children: long-term efficacy.
Eighteen partial splenic embolization procedures (PSEs) were performed in 17 children for hypersplenism (13) and/or esophageal variceal hemorrhage (12). The underlying disease was biliary atresia (BA) in nine children, portal vein thrombosis (PVT) in four, and biliary cirrhosis (BC) in four. From 20% to 90% of the spleen was embolized. Immediate morbidity was high, albeit minor, and the initial hospitalization was protracted for an average of 16 days. The children were followed from 4 to 81 months (average, 34.2). Four patients with BA patients subsequently had liver transplantation at an average of 20 months after PSE. In ten of 13 patients with hypersplenism, hematologic indexes returned to and remained normal throughout follow-up. The three exceptional patients (who had only 20%, 60% and 60% splenic embolization) developed recurrent mild hypersplenism, one of whom was reembolized and is free from hypersplenism 22 months later. Variceal hemorrhage was ameliorated in all 12 patients (average, 2.4 episodes of hemorrhage per year before PSE, 0.5 per year afterwards). Overwhelming postsplenectomy sepsis did not occur in an aggregate follow-up of 48.5 years. PSE is a legitimate treatment alternative for hypersplenism and for esophageal varices in children.
Adolescent↗
The surgery of biliary atresia.
One hundred thirty-one consecutive infants with biliary atresia were operated on during the 15-year period between 1973 and 1988. Six patients did not have biliary reconstruction because of advanced cirrhosis or transplant preference. The other 125 infants had excision of all nonpatent extrahepatic bile ducts; biliary drainage was provided by a gallbladder-common bile duct conduit in 14 patients and by a Roux-en-Y portoenterostomy in 111 infants (including the seven patients with correctable biliary atresia). The bilioenteric conduit was temporarily exteriorized and, for the past 2 years, a conduit intussusception valve was incorporated. Immediate postsurgical bile drainage was achieved in 103 infants (82%). Reoperation during the first 6 postoperative weeks restored bile flow in 14 of 18 infants who had shut down. Seventy-two patients (57%) had sustained (more than 1 year) relief of biliary obstruction. Postoperative morbidity was substantial. The six children not having corrective surgery died within 19 months. Three patients were lost to follow-up. Sixty-eight patients having Kasai's operation died, 55 from complications of liver disease, 1 from a coexisting malformation, and 12 after liver transplantation. Fifty-seven patients are alive, 13 by virtue of liver replacement, 9 with mild-to-moderate hepatic sequelae, and 35 (28%) with normal to near-normal liver function. Although none is considered "cured," the 35 children are anicteric, have normal growth and development, and participate in full school activities (including contact sports). Average follow-up is 85.8 months (range 1 to 15 years).
Anastomosis, Roux-en-Y↗
The Boix-Ochoa antireflux operation in infants with small stomachs.
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Esophagus↗
Reoperation for congenital choledochal cyst.
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Common Bile Duct Diseases↗