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Biomedical subjects

R P Altman

Publications and source records attributed to R P Altman.

At least 19 recordsLinked to original sources
PubMed1992

Acquired coarctation of the aorta.

Coarctation of the aorta is usually caused by a congenital narrowing of the aorta. This report describes two children who developed hypertension secondary to an acquired coarctation of the aorta. In one patient the coarctation was temporally related to umbilical artery catheterization and was associated with thrombosis and aneurysmal dilatation of the aorta. In the second patient, the coarctation occurred after surgical aortotomy during the removal of an intrathoracic neuroblastoma. Patients who have interventional damage to the aorta should be periodically examined for the appearance of a coarctation. Although an acquired coarctation of the aorta is an infrequent complication of invasive or surgical procedures, it should be identified since it represents a remediable cause of hypertension in children.

Aortic Coarctation↗
PubMed1992

Three pediatric patients with extension of prostatic embryonal rhabdomyosarcoma anterior to the bladder into the space of Retzius.

Invasive embryonal rhabdomyosarcoma of the prostate often extends superiorly into the bladder base requiring partial cystectomy as part of excision of the primary tumor. Three patients were studied with ultrasonography CT and MRI and showed the extension of the tumor superiorly and anterior to the bladder in the prevesical space of Retzius. Complete excision with bladder sparing was successful in two of the patients. The imaging of pelvic tumors in children (although well studied with ultrasonography, CT) is helped by MRI in the sagittal plane and/or lateral films of intravenous pyelograms and cystograms to establish the relation of the tumor to the bladder.

Child↗
PubMed1992

Choledochal cyst.

The most commonly encountered variant of choledochal cyst (type I) is characterized by fusiform dilatation of the bile duct. Patients usually come to medical attention within the first decade of life. The diagnosis is readily established by ultrasound or computed tomography. Choledochal cyst has been diagnosed in the fetus by maternal ultrasound. Treatment is by surgical excision.

Child↗
PubMed1990

Obstruction of the Roux limb after portoenterostomy for biliary atresia: a delayed complication.

We report the case of a 5-year-old girl who underwent a Kasai portoenterostomy for extrahepatic biliary atresia. The conduit was exteriorized until 11 months of age. She was doing well, with stable portal hypertension until she suddenly developed jaundice, acholic stools, and bacteremia not responsive to a course of steroids and intravenous antibiotics. Suspecting obstruction at the site of the previously exteriorized anastomosis, a percutaneous cannulation of the conduit was performed. Catheterization of the conduit obstruction unkinked it and reestablished bile flow. She has remained anicteric with stable liver function.

Anastomosis, Roux-en-Y↗
PubMed1978

Portal decompression in infants and children with the interposition mesocaval shunt.

The mesocaval graft for portal decompression is applicable in infants and children with portal hypertension secondary to extrahepatic or intrahepatic causes. It is recommended in patients in whom extensive previous surgery in the portahepatis (Kasai procedure) would make dissection of the portal vein difficult and endanger the integrity of the functioning biliary conduit. It can be performed in patients who have had previous splenectomy or in whom portacaval or splenorenal shunts have failed. Autogenous jugular vein is favored for creation of the shunt.

Adult↗
PubMed1978

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.

Bile Ducts↗
PubMed1978

Liver resection in children with hepatic neoplasms.

In the past ten years, 28 patients with primary tumors of the liver have been treated. There were 11 benign tumors, including four hamartomas, three patients with focal nodular hyperplasia, and two each with congenital cysts and hemangioma. Hamartomas and masses of focal nodular hyperplasia should be excised when possible, but both are benign lesions; therefore life threatening excisions at the porta hepatis should be avoided. Cysts are often resectable, but when occupying all lobes of the liver, they can be successfully managed by marsupialization into the free peritoneal cavity. If resectable, hemangiomas should be removed; when occupying most of the liver as they often do, patients may be subject to platelet trapping or to cardiac failure. In some instances these lesions have been controlled by steroids, radiation therapy or hepatic artery ligation. Of 17 malignant tumors seen, 12 proved to be hepatoblastomas. Nine of the 12 patients underwent liver resection, of whom four are cured, (33%). There were three children with hepatocellular carcinomas and two with embryonal rhabdomyosarcoma. One child from each of these groups is cured by surgical excision. At present the only known cures in children with primary malignant liver neoplasms have been achieved by operative removal.

Carcinoma, Hepatocellular↗
PubMed1978

The portoenterostomy procedure for biliary atresia: a five year experience.

The portoenterostomy (Kasai) procedure in infants with biliary atresia has dramatically altered the outlook for this heretofore fatal disease. When performed on infants under three months of age, bile drainage can be achieved in a majority of the patients. Since 1972, 37 infants have been treated with this operation at our institution. Diagnostic operative cholangiography and liver biopsy are recommended if the cause of conjugated hyperbilirubinemia is presumed to be obstructive. When biliary atresia is encountered, identification of the atretic ducts with transection high in the porta hepatis is carried out. Thirty-two infants have had the portojejunostomy, while five, in whom the proximal hepatic ducts were atretic but the gall bladder and distal ducts were patent, underwent portocholecystostomy. Examination of the resected fibrous duct tissue revealed a statistically significant correlation between ductal histology and postoperative outcome. Extended bile drainage has been achieved in 26 of 37 patients. Seventeen exhibit near normal growth and development four months to five years postoperatively. Seven have died with progressive liver disease despite bile drainage. Two additional patients died, jaundice free, from unrelated causes. Despite bile drainage, progressive hepatic fibrosis has been confirmed by serial biopsies in 14 patients. This finding indicates that biliary obstruction is not the sole component in the development of biliary cirrhosis. These data suggest that extrahepatic biliary atresia is a dynamic obliterative process, which can be favorably modified in approximately 50% of the infants by early surgical treatment.

Bile Ducts↗
PubMed1977

Experience with in-service examinations of pediatric surgical trainees.

A formal examination is an effective device for assessing certain aspects of a resident's competence. Participation by outside examiners is advisable. Generalizations on the basis of 2 yr of experience may be premature, however, the oral examination, strengthened by outside examiners and complemented by a written clinical problem-solving test, has provided insight into the candidates' abilities and into their progress from the first to the second year. In addition, this process has identified strengths and weaknesses in the training program, which has led to modifications of the surgical curriculum. It is concluded that comprehensive oral and written examination of pediatric surgical trainees at the completion of their first year and again at the end of their second year yields information that is of importance to the trainee and to the faculty.

Clinical Competence↗
PubMed1977

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.

Biliary Tract↗
PubMed1977

Evolution of surgical treatment of bilateral Wilms' tumor.

Bilateral Wilms' tumor (stage V) accounts for 10 to 15% of patients with nephroblastoma. Heretofore, surgical resection in many of these children presented insoluble technical problems. Ex vivo renal perfusion with "bench" surgical excision and revascularization has been applied in the management of a child with bilateral Wilms' tumor unresectable by conventional techniques. Complete extirpation of tumor was accomplished. Furthermore, the autotransplanted kidney was removed from the field of radiation therapy given after operation. By selective application of this technique, the child with bilateral Wilms's tumor can be approached with a greater expectation of cure.

Child, Preschool↗
PubMed1977

Recent developments in hepatobiliary surgery.

The surgical management of infants and children with hepatobiliary disorders has undergone some important advances in recent years. Careful assessment over long periods will be necessary to determine if the new procedures discussed will endow these children with a normal or near-normal life span, free of disease.

Biliary Tract↗