R. Peter Altman

Ductal remnants in extrahepatic biliary atresia: A histopathologic study with clinical correlation.

Atretic ducts were studied in 34 infants undergoing the portoenterostomy procedure for biliary atresia. The specimens were grouped into three categories on the basis of the size of the lumen at the proximal margin: Type I with a lumen 150 mu or greater, Type II with single to multiple ductal structures measuring less than 150 mu, and Type III with no identifiable epithelium-lined structures in fibrous connective tissue. A significant correlation existed between postoperative bile drainage and the duct type; bile drainage occurred in all five patients with Type I ducts, 18 of 21 patients with Type II ducts, and one of eight patients with Type III ducts. Furthermore, the duct type was the only feature that correlated with the ultimate outcome. Ductal inflammation and hepatic histology did not correlate with postoperative bile drainage or clinical course.

Surgical management of intractable cholangitis following successful Kasai procedure

Anastomotic revision/resection procedures were carried out in five patients in whom refractory cholangitis jeopardized an apparently favorable surgical outcome after portoenterostomy for biliary atresia. Mobilization and displacement of the liver improves exposure of the porta hepatis. All five patients remain jaundice-free seven months to 4 yr after reoperation.

Update on the analysis of the need for pediatric surgeons in the United States.

An updated version of the 1975 analysis of pediatric surgical manpower needs in the United States by regional questionnaire analysis indicates that pediatric surgeons and capable hospital facilities are unusually well-distributed and that the estimated need is close to the present number practicing. Pediatric surgery grew by a factor of over 30% relative to population over the last five years, but this growth rate would be excessive if it continued. The growth included not only graduates from approved training programs but also from nonapproved training programs and from immigration. Limitations should probably be placed on the latter two sources of entry into the practice system. A computer analysis was made to project numbers of pediatric surgeons per unit population until the year 2010 with differing levels of trainee output. The study indicates that 15-17 individuals entering the system from all sources are sufficient to allow for modest continued growth of the specialty if current population trends continue.

Results of re-operations for correction of extrahepatic biliary atresia***

This communication reviews the surgical experience with re-operations performed for correction of extrahepatic biliary atresia upon 22 patients at the Childrens Hospital National Medical Center between 1972 and 1978.

Timing of liver transplantation in biliary atresia-results in 71 children managed by a multidisciplinary team

BACKGROUND Kasai portoenterostomy (KP) remains the initial surgical therapy for biliary atresia (BA). Liver transplantation (LTx) is offered after a failed KP or if KP is not feasible. The timing of LTx in these children is not well established. We attempted to define factors that may help choose the optimal timing for LTx in children with BA managed by a multidisciplinary team including a pediatric surgeon, hepatologist, and liver transplant surgeon. METHODS Records of children who underwent LTx for BA at our institution between January 1998 and December 2006 were reviewed. Clinical data such as pre-LTx pediatric end-stage liver disease (PELD) score, location of KP, and outcome were evaluated. RESULTS Seventy one children underwent 77 liver transplants for BA at an average age of 25 months (range, 3-216 months). Sixty-one had a previous KP, 30 at our institution. Ten had LTx without KP. The overall patient survival was 94.4% and overall graft survival was 87% at median follow-up of 58 months (range, 6-111 months). Four patients died, 1 because of vascular thrombosis despite repeat LTx, 1 because of fungal infection after LTx, and 2 because of causes unrelated to LTx. Six children required retransplantation. Living donor liver transplantation was performed in 32 of these children with 91% patient and graft survival. Fifty-three children had a PELD score of 10 or higher with patient and graft survivals of 92% and 86%, respectively. Eighteen children had a PELD score of less than 10 with patient and graft survivals of 100%. For the 30 children who underwent KP at our institution, the median age at LTx was 9 months (range, 3-168 months), and patient and graft survival were both 93%. CONCLUSIONS Outcome of LTx for BA is excellent. Children with higher PELD scores (>/=10) at LTx may have worse outcome. Children with a PELD score of less than 10 survived with their original grafts. In children with BA, the PELD score should be monitored and may help stratify patients for eventual LTx. When a child with BA is deemed a candidate for LTx, the PELD score should be determined. A PELD score that approaches 10 should trigger discussion of LTx and living donor liver transplantation with the family.

Potential errors in the diagnosis and surgical management of neonatal jaundice

Despite advances and refinements in diagnostic techniques, distinguishing between cholestatic syndromes and extrahepatic biliary atresia is not always possible. Because there are inherent errors in all diagnostic studies including nuclear scanning, ultrasonography, and liver biopsy some infants presumed to have biliary atresia will come to surgery when, in fact, the bile ducts are partially or completely patent. The decision to proceed with a portoenterostomy or to terminate the procedure depends upon the appearance of the liver and histology of the biopsy.

Right pneumonectomy syndrome: A lethal complication of lung resection in a newborn with cystic adenomatoid malformation

A newborn girl underwent a right pneumonectomy for cystic adenomatoid malformation. She subsequently developed compression of the trachea and left mainstem bronchus by a combination of aortic arch, pulmonary artery, and ductus arteriosus. She died while planning aortic suspension, and the anatomic relationships were confirmed by autopsy. This problem is potentially treatable by a variety of means.

Alpha1-fetoprotein in neonatal hepatobiliary disease+

It has been suggested that the quantitative estimation of serum alpha-1-fetoprotein may help in distinguishing the neonatal hepatitis syndrome from biliary atresia. We measured the serum AFP concentration in 52 neonates and infants with various hepatobiliary disorders, including neonatal hepatitis syndrome (group I), biliary atresia (group II), and other hepatopathies such as choledochal cyst (group III). The mean serum AFP concentration in patients with neonatal hepatitis was significantly greater than the mean concentration in the other two groups. There was no significant difference between the mean serum AFP concentrations in patients with biliary atresia and in group III patients. Patient age was noted to be an important factor: Serum AFP levels greater than 35 microgram/ml in infants one to four months of age suggpst the diagnosis of neonatal hepatitis syndrome. Serum AFP levels below 10 microgram/ml in infants less than four months of age suggest the diagnosis of biliary atresia or hepatopathies other than neonatal hepatitis. However, the variable and significant overlapping of serum AFP values between 10 and 35 microgram/ml limit the diagnostic value of this test.

Pediatric Hepatobiliary Disease

The surgical aspects of pediatric hepatobiliary disease concern a unique set of diagnoses, disease processes, diagnostic issues, and treatment problems. In this context, this article discusses features of biliary atresia, choledochal cyst, gallbladder disease, and liver abscess in the infant, child, and adolescent.

Portal hypertension: American Academy of Pediatrics Surgical Section Survey—1981

Hepatic (cirrhosis) accounts for a higher percentage of portal hypertension than extrahepatic portal vein obstruction. Some patients with extrahepatic portal hypertension (portal vein obstruction) have a more favorable clinical status. Nonoperative management (balloon tamponade, vasopressin) will usually succeed in controlling bleeding (both groups). Most patients can be stabilized before surgical procedure. Results of devascularization procedures are encouraging. Central splenorenal shunt was most commonly employed (both groups). The Warren shunt is gaining popularity. There are no clear advantages of any particular method of portosystemic shunting in either group. The results of shunt therapy in cirrhotic patients are comparable to those observed in patients with extrahepatic portal hypertension.