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Biomedical subjects

G P Stellin

Publications and source records attributed to G P Stellin.

10 recordsLinked to original sources

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↗

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.

Age Factors↗

Forme fruste choledochal cyst.

Four patients had the characteristic features of choledochal cyst except for the cystic component. All patients had stenosis of the distal common bile duct, a "long common channel" secondary to a proximal junction of the common bile and pancreatic ducts, cholecystitis and the classic pathological microscopic features of choledochal cyst in the wall of the common bile duct. Three children had coexisting intrahepatic duct cysts and/or stenosis and one had intrahepatic choledocholithiasis. The clinical presentations were cholangitis (2), pancreatitis (1) and biliary obstruction (1). In all cases the common bile duct was resected and biliary reconstruction was carried out by choledochojejunostomy (Roux-en-Y). Morbidity was minor except in one patient with ductal disease extending far into the intrahepatic ducts. This child developed an anastomotic stricture requiring revision of the anastomosis and long-term "U" tube stenting. Forme fruste choledochal cyst appears to be another variation in the spectrum of pancreaticobiliary malformations of choledochal cyst. Treatment is identical, that is, excision of all malformed ductal tissue.

Adolescent↗

Partial splenic embolization in children with hypersplenism.

Partial splenic embolization (PSE) was successfully accomplished in 10 of 11 children, aged 2-9, who had portal hypertension or variceal bleeding. Nine of the 11 children had undergone portoenterostomy (Kasai operation) for biliary atresia, and two had portal vein thrombosis. After embolization these children had a longer period of fever (mean = 23.7 days) and elevated white blood cell (WBC) count (above 10,000, mean = 13.6 days) than adults who have undergone the same procedure. The leukopenia and thrombocytopenia of hypersplenism were corrected by PSE in seven of eight children, and the condition of the eighth child improved. Among ten patients who had experienced episodes of variceal hemorrhage, the frequency of bleeding episodes was reduced from an average of 2.87 per year before PSE to 0.67 per year after PSE. There were no splenic abscesses and no other significant complications of the treatment. Ultrasound (US) evaluation after embolization demonstrated hypoechogenicity of the infarcted areas and tiny, linear echoes scattered throughout the spleen typical of postinfarction intravascular gas. All nine children who underwent follow-up Tc-99m sulfur colloid scanning showed evidence of splenic regeneration, though none has had recurrence of clinical symptoms. Splenic regeneration following PSE may occur more frequently in children than in adults.

Child↗

In biliary atresia duct histology correlates with bile flow.

Three basic types of microscopic biliary structures at the portahepatis were distinguishable in infants with biliary atresia: bile ducts, collecting ductules of biliary glands, and biliary glands. Correlation between the type of biliary structure and the quantity and quality of post-operative bile flow was possible in 23 instances. At 2 weeks after operation, the 11 patients in whom a bile duct was identified had a daily bile flow of 68.0 +/- 11.5 mL. Bilirubin concentration in the bile was 13.6 +/- 3.3 mg/dL and total daily bilirubin excretion was 8.77 +/- 2.74 mg. In contrast, bile flow in 12 patients having only collecting ductules and/or biliary glands in the porta hepatis was 19.1 +/- 3.9 mL and bilirubin concentration in bile was 1.7 +/- 0.3 mg/dL. Thus, total daily bilirubin excretion was 0.34 +/- 0.08 mg (P less than 0.001). Postoperative cholangitis occurred only in patients with ducts. It is concluded that only bile ducts communicate with the intrahepatic biliary system and drain bile after hepatic portoenterostomy.

Bile↗

Conduit decompression in biliary atresia.

Catheter decompression of the bilioenteric conduit was routinely carried out coincident with stomal closure in 15 consecutive patients with biliary atresia after Kasai hepatic portoenterostomy operation. Postoperatively the catheter was systematically, gradually occluded over a 3- to 8-week period and then withdrawn. Complications were relatively minor and early postoperative cholangitis was avoided.

Bile Ducts↗

Safety and efficacy of glycerol and amino acids in combination with lipid emulsion for peripheral parenteral nutrition support.

The safety and efficacy of administering lipid emulsion with ProcalAmine, a glycerol-based parenteral nutrition solution, for peripheral nutrition has not been previously studied. Thirty-four patients recovering from major trauma or surgery were studied while receiving a peripheral parenteral nutrition regimen of either ProcalAmine with 10% lipid emulsion (group 1) or ProcalAmine with 20% lipid emulsion (group 2) for up to 5 days postinjury. Daily dose was 45 mL/kg ProcalAmine, providing 1.35 g of amino acids/kg and 1.35 g of glycero/kg, and 500 mL/day lipid emulsion. The mean daily nitrogen balance was -0.3 g/day in group 1 and -4.1 g/day in group 2. There was no progressive accumulation of circulating glycerol, and urinary glycerol excretion was minimal (less than 0.2 g/day), indicating effective utilization of glycerol as an energy substrate. Our finding that nitrogen balance was better with 10% fat emulsion suggests a limitation in fat utilization in this setting. Both regimens were well tolerated; there were no adverse clinical reactions and no occurrences of phlebitis in either group.

Adolescent↗