Robert H. Shikes

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)

Ultrastructure of fibrolamellar oncocytic hepatoma.

The fibrolamellar variant of hepatocellular carcinoma is described and the ultrastructural findings in three cases are reported. The findings confirm the oncoytic nature of the tumor and the presence of distinctive bands of collagen. This tumor may represent a neoplasm of hepatic oncocytes. It appears to have distinct epidemiologic differences from other hepatocellular carcinomas, which typically arise in cirrhotic livers, and it may have a better prognosis. As such, it should be recognized as a distinct clinicopathologic subset in the broad group of hepatocellular carcinomas.

Biliary atresia in the newborn

A prenatal sonographic diagnosis of extrahepatic biliary atresia was made and, 76 hours after birth, operatively confirmed. A standard Kasai operation was performed, with the exception of the use of an ancillary appendiceal conduit to provide biliary drainage of an independent bile duct draining the right anterior hepatic segment. Hepatic and ductal histology were identical to those usually found in biliary atresia in a 6- to 8-week-old infant. The child is well at 16 months.

Surgical implications of bronchopulmonary dysplasia

Twenty of 30 patients with bronchopulmonary dysplasia (BPD) had major tracheobronchial abnormalities, which in 18 could be incriminated as contributing to their symptoms. There were 15 examples of tracheobronchial stenosis, 13 of tracheomalacia, nine of bronchomalacia, and one of tracheal web. Sixteen patients had operations. Tracheostomy was successful as a temporizing measure in ten patients although there was significant morbidity. Balloon dilatation of tracheobronchial stenosis produced temporary (2) or long-term (2) improvement in four patients. Electroresection of tracheobronchial stenosis was successful in the three instances it was employed. Lobectomy for lobar emphysema was curative in both patients. Aortopexy for tracheomalacia improved all three patients. We conclude that (1) symptomatic major airway lesions are not uncommon manifestations of BPD, (2) many of the lesions are amenable to surgical therapy, and (3) bronchoscopic evaluation should be considered early in the course of infants with BPD.

Pancreatic islet cell necrosis: Association with hemolytic-uremic syndrome

A 3-year-old girt was admitted to a community hospital with 5% dehydration following two days of vomiting, abdominal cramps, and bloody diarrhea. Initial physical examination revealed a slightly dehydrated, irritable child. Serum analysis on admission included Na 138 mEq/L, K 4.2 mEq/L, C1 102, mEq/L, HCO3 18 mEq/L, BUN 15 mg/dl, glucose 179 mg/dl, Hgb 14.9 gm/dl, and Hct 43%. Fluids were administered intravenously. On the second hospital day the child became lethargic and disoriented. The hematocrit was 34% and the platelet count 108,000/ram 3. A review of the blood smear showed fragmented red cells, and a diagnosis of hemolytic uremic syndrome was made. The patient became anuric on the second hospital day and was transferred to University of Colorado Health Sciences Center. On admission, physical examination revealed an alert but irritable child with supine blood pressure of 120/62 mm Hg. The abdomen was distended and diffusely tender, with hypoactive bowel sounds and guarding but no rebound tenderness. The remainder of the examination was normal. Serum analysis revealed Na 127 mEq/L, K 5.0 mEq/L, CI 100 rnEq/L, HCO~ 13.7 mEq/L, BUN 66 mg/dl, creatinine 4.9 mg/dl, glucose 168 mg/dl, WBC 30,500/ram 3 with 52% neutrophils, 16% bands, and 25% Iymphs, Hgb 9.8 gm/dl, Hct 29%, and platelets 55,000/ram 3. Peritoneal dialysis was initiated using a 1,5% dextrose dialysate. The dialysate was changed to 4.25% dextrose as needed to maintain a normal intravascular volume. The patient received intravenous alimentation with 5% dextrose, 1% Freeamine (McGaw Laboratories, Inc., Glendale, Calif.), and Intralipid (Cutter Laboratories, lnc, Berkeley, Calif.) 1 gm/kg/day. Serum glucose concentration remained between 150 to 350 mg/dh

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.

Segmental Lung Resection in the First Year of Life

Segmental lung resection was performed in 5 infants with congenital lung disease. The lesions were cystic adenomatoid malformation in 2, congenital cystic disease in 2, and congenital lobar emphysema in 1. A higher than expected incidence of major segmental bronchovascular anomalies was encountered (3 of 5 patients). Other than prolonged air leak in 1 patient, complications were negligible. All patients were well from one to six and one-half years after operation. Segmentectomy is a sensible operation for congenital pulmonary disease and ultimately may have as its greatest virtue the preservation of normal lung tissue.