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Biomedical subjects

J R Lilly

Publications and source records attributed to J R Lilly.

At least 73 records · Page 4Linked to original sources
PubMed1985

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↗
PubMed1985

Contemporary surgery of biliary atresia.

The etiology of biliary atresia is not due to a congenital malformation but rather to a continuing process beginning in utero that affects not only the extrahepatic biliary ducts but also the intrahepatic parenchyma. Over the last decade, the outlook for patients who were previously felt to be uncorrectable has been significantly improved by Kasai's operation. Successful biliary reconstruction depends on early diagnosis and treatment (before three months of age). The essentials of hepatic portoenterostomy consist of excision of the entire extrahepatic duct structure with anastomosis of an intestinal conduit to the area of the transected duct at the liver hilus. After operation, many patients experience complications, including cholangitis, portal hypotension, and vitamin deficiencies. Despite these difficulties, growth and development continue on a relatively normal course, and long-term survival has been accomplished in many children. For those in whom biliary drainage is not achieved or with significant parenchymal damage, liver transplantation should be considered as part of ongoing care.

Bile Ducts↗
PubMed1985

Esophageal endosclerosis in children.

During the past 6 years, 25 consecutive patients with esophageal variceal hemorrhage were treated by esophageal endosclerosis (direct injection of varices with a sclerosing agent). The primary disease in the 25 children was portal vein thrombosis (11 patients), biliary atresia (nine patients), and hepatic cirrhosis from cystic fibrosis (three patients), alpha 1-antitrypsin deficiency (one patient), and neonatal hepatitis (one patient). Thirteen patients were treated during acute, major variceal hemorrhage. Esophageal endosclerosis was repeated at regular intervals until all esophageal varices were obliterated. Twenty-one patients completed therapy. Four patients died: one of a complication of therapy and three of the primary disease. Other than the one death, complications were minor. Recurrent esophageal variceal hemorrhage has not been encountered in follow-up from 9 months to 6 years after completion of therapy.

Bile Ducts↗
PubMed1984

The multiple coagulopathies of biliary atresia.

Detailed coagulation studies were done prospectively on 43 patients with biliary atresia who had undergone Kasai operation (hepatic portoenterostomy). Patients were divided into three groups based on levels of factor V, factor II, and Echis II and/or response to vitamin K: no coagulopathy (46.5% of patients); coagulopathy of liver disease (30.2% of patients); and coagulopathy of vitamin K deficiency (23.3% of patients). Patients with the coagulopathy of liver disease had significantly lower levels of factors XII, V, and antithrombin III as well as longer thrombin times than patients with no coagulopathy or vitamin K deficiency. Factor V levels were decreased only in patients with more advanced liver disease; normal levels of factor V were not usually helpful in differentiating liver disease and vitamin K deficiency. The prothrombin time, factor VII-X levels, and factor II levels were significantly different for all three groups; the most abnormal values occurred in the vitamin K-deficient group. Comparison of the Echis II level to factor II coagulant activity was helpful in deciding whether a coagulopathy was due to liver disease, vitamin K deficiency, or both. Factor VIII levels were elevated in all groups. Factor VIII coagulant activity was significantly higher by the two-stage (TGT) method than by the one-stage (PTT) method. Hypersplenism causing neutropenia and thrombocytopenia was commonly seen after the age of 5 years. Vitamin E deficiency was more common than vitamin K deficiency; however, all vitamin K-deficient patients were vitamin E deficient. Coagulation status correlated well with hepatobiliary scan data, but not serum bilirubin levels. Recommendations for treatment of patients with vitamin K deficiency and/or liver disease are discussed.

Bile Duct Diseases↗
PubMed1984

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↗
PubMed1984

Variceal hemorrhage in biliary atresia.

Eleven of 47 patients (23%) with sustained bile drainage after Kasai's operation for biliary atresia had major variceal hemorrhage. Hemorrhage was esophageal in origin in five children and from the exteriorized conduit in eight. Two patients bled from both sites. The first major hemorrhage occurred at an average patient age of 40.4 months. Clinical manifestations of portal hypertension were poorly correlated with liver functions, however, markedly decreased calibre of the intrahepatic venous radicles was noted by ultrasonography in all patients examined. Primary treatment was by esophageal endosclerosis or by disconnection of portosystemic shunts coincident with stomal closure. Adjunctive splenic embolization was employed in six patients. Recurrent hemorrhage has not been encountered in limited follow-up.

Abdominal Muscles↗
PubMed1983

Primary tracheomalacia.

Tracheomalacia is a rare congenital malformation of the tracheobronchial cartilages in which the supporting cartilaginous rings permit expiratory collapse of the airway. The condition is usually mild and self-limited. There is a severe variant, however, that is life-threatening and warrants separate categorization. Four children with severe primary tracheomalacia were treated recently. The clinical symptoms, diagnostic findings, and eventual treatment of these patients were highly distinctive and almost identical in all 4, permitting us to make the following observations: (1) primary severe tracheomalacia must be suspected in infants with unexplained respiratory distress manifested by stridor and cyanosis; (2) symptoms are not present at birth but appear insidiously after the first weeks of life, are markedly aggravated by respiratory tract infections, and are made worse by agitation; (3) bronchoscopy is essential for definitive diagnosis and should be employed early in the diagnostic process; (4) tracheostomy is probably essential in most instances; and (5) resolution, although spontaneous, does not occur until after 2 years of age.

Cartilage Diseases↗
PubMed1983

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↗
PubMed1983

The surgery of pulmonary artery "sling".

Two consecutive infants with pulmonary artery sling underwent successful surgical repair utilizing a median sternotomy for surgical exposure and adjunctive aortopexy. Bronchoscopy demonstrated severe tracheomalacia in both patients which was corrected under direct bronchoscopic control. Median sternotomy (rather than recommended left thoracotomy) provided perfect exposure of the pulmonary artery at its bifurcation thus permitting easy reconstruction of pulmonary artery. Aortopexy eliminated postoperative respiratory symptoms. Both patients are well.

Bronchoscopy↗
PubMed1983

Diagnosis of biliary atresia with radionuclide hepatobiliary imaging.

Sixteen patients with biliary atresia and 11 patients with neonatal hepatitis were studied preoperatively with either Tc-99m-diethyl-IDA or TC-99m-diisopropyl-IDA. Two parameters were evaluated: hepatocyte clearance and time to appearance of radioactivity in the intestine. Two observers, using a visual grading system of 1 to 4, gave the 16 patients with biliary atresia a hepatocyte clearance grade of 1.7 +/- 0.6 (mean +/- SD); intestinal radioactivity was not seen through 24 hours. The hepatocyte clearance grade of the 11 patients with neonatal hepatitis was 2.1 +/- 0.9 (mean +/- SD) (p greater than 0.05); intestinal radioactivity was seen in nine of 11 patients (p less than 0.001). Using both parameters, 91% of the patients were classified correctly, 4% were misclassified, and 6% were classified as indeterminate; sensitivity and specificity for biliary atresia were 97% and 82%, respectively. Radionuclide imaging with the newer technetium-99m-labeled hepatobiliary radiopharmaceuticals appears promising for the noninvasive diagnosis of biliary atresia.

Bile Ducts↗
PubMed1982

The clinical spectrum of right Bochdalek's hernia.

Congenital right posterolateral (Bochdalek's) diaphragmatic hernia usually has clinical manifestations different from those of left Bochdalek's hernia; it often masquerades as a pleural effusion, an asymptomatic intrathoracic mass, or an intestinal obstruction. The primary reason for the difference in symptoms is the presence of the liver on the right, which occludes the diaphragmatic defect and permits normal development of the ipsilateral lung. We recommend a transabdominal approach for surgical repair.

Diagnosis, Differential↗