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Biomedical subjects

E R Howard

Publications and source records attributed to E R Howard.

At least 37 records · Page 2Linked to original sources
PubMed1998

Antenatal diagnosis of congenital anomalies of the biliary tract.

BACKGROUND: The accuracy of the technique of antenatal ultrasonography in the diagnosis of congenital bile duct lesions is unknown. METHODS: Thirteen patients with proven biliary disease who had abnormal antenatal scans were reviewed. Two infants had type I cystic biliary atresia and one had a noncommunicating segmental dilatation of the bile duct in a type 3 biliary atresia. The remainder had choledochal cysts and included two patients with intrahepatic cysts. The correct diagnosis was made antenatally in only two (15%) cases. Of the remaining patients, seven received a diagnosis of intraabdominal cysts of unknown etiology, three of duodenal atresia, and one ovarian cyst. The median gestational age at the antenatal diagnosis was 20 weeks. RESULTS: Jaundice developed in 11 infants, and dilatation of intrahepatic biliary radicals was noted in four of the choledochal cysts. Obstructive jaundice and increasing cyst size were indications for early surgery, and twelve infants underwent a laparotomy at a median age of 4 weeks. During the median follow-up period of 2 years, 12 of the 13 patients have lost their jaundice or remained anicteric. Antenatal diagnosis offers the possibility of early definitive surgery for uncomplicated choledochal dilatation and the chance of improved outcome for surgically treated biliary atresia. An algorithm is suggested for the management of antenatally detected cystic biliary lesions.

Biliary Atresia↗
PubMed1997

Developmental intrahepatic shunts of childhood: radiological features and management.

The purpose of this study was to evaluate the role of radiological techniques in the diagnosis and management of developmental intrahepatic shunts. Hepatic vascular fistulae are recognised sequelae of liver trauma and intrahepatic tumours. However, there are rare developmental malformations which may present in childhood or later life and which may carry life-threatening complications. Retrospective analysis of clinical and radiological data was carried out in 24 patients. Anomalies evaluated were: (a) direct communication between hepatic artery and hepatic veins; (b) congenital hepatoportal arteriovenous malformations; and (c) congenital portocaval anastomosis with persistent flow through the ductus venosus. Although rare, the prompt recognition of these vascular anomalies allows early surgical or radiological intervention and reversal of the haemodynamic complications.

Arteriovenous Fistula↗
PubMed1997

Biliary atresia: the King's College Hospital experience (1974-1995)

UNLABELLED: The survival experience of 338 infants born with biliary atresia between January 1973 and December 1995 was analyzed. All the infants had their initial surgery at a single UK centre. These infants were divided into three groups based on year of birth; group 1 (1970s, n = 38); group 2 (1980s, n = 182), and group 3 (1990s, n = 118). The data from group 1 were incomplete and selected, and comparisons with the remaining groups were therefore restricted. However, all infants who had been treated since 1980 underwent portoenterostomy or hepaticojejunostomy and were included. RESULTS: In the whole cohort there were 89 deaths (26%), 79 children (23%) who underwent liver transplantation and 170 children (50%) who were alive at last follow-up. The 5- and 10-year actuarial survival for group 2 was 50% and 41%, respectively and the 5-year actuarial survival for group 3 was 60%. Overall, 57 children have survived to 10 years after surgery for biliary atresia. There has been a progressive fall in the age at surgery from a median of 77 days in group 1, through 69 days in group 2 to 56 days in group 3 (P < .0001). However, there was no significant difference in outcome to 5 years between the age cohorts (< 40 days, 41 to 60 days, 61 to 99 days, and > or = 100 days; P > .1) for the infants treated since 1980 (n = 200). CONCLUSIONS: Portoenterostomy is an effective long-term procedure for biliary atresia in about 40% to 50% of infants. The remaining 50% to 60% will require transplantation mostly within 2 years of age, although there is also a continuing need beyond 5 and 10 years. The age at surgery has limited usefulness as a predictor of survival after portoenterostomy and certainly should not be used to dictate primary treatment.

Actuarial Analysis↗
PubMed1997

Congenital extrahepatic portocaval shunts--the Abernethy malformation.

The first account of an absent portal vein and a congenital mesenterico-caval shunt was given by John Abernethy in 1793. Five new cases of congenital extrahepatic portocaval shunts are described in this report. One end-to-side shunt with congenital absence of the portal vein (type 1 shunt) is the 13th case in the literature. Four are side-to-side (type 2) shunts of which there have been only two previous reports. Type 1 shunts are associated with other congenital abnormalities and have only been recognized in girls. Five of these cases developed liver tumours. Type 2 shunts are rarer, and four of five have been boys. They are not usually associated with other anomalies. Encephalopathy may be associated with these shunts in adults, and surgical closure of the side-to-side shunts is therefore recommended. End-to-side shunts are not correctable but the abnormal anatomy may create a problem for hepatic transplantation, should this be necessary for associated disorders such as biliary atresia. "The Abernethy Malformation" would seem to be a suitable eponym for congenital extrahepatic portocaval shunts.

Adolescent↗
PubMed1997

The treatment of biliary atresia in Europe 1969-1995.

European studies of biliary atresia have suggested that the aetiology is heterogeneous. Histological studies of the liver and biliary remnants excised at portoenterostomy have failed to identify any prognostic features except for the size of bile ductules in the porta hepatis. Most of the major series have confirmed that there is a relationship between age at portoenterostomy and clearance of jaundice which has been achieved in more than 67% of infants under 10 weeks of age. Cholangitis reduced survival and bleeding from esophageal varices has occurred in more than 19% of long-term survivors. The 5-year jaundice-free survival rate after portoenterostomy is 37% and the 10 year rate is 18%. It is predicted that these survival rates will improve. Orthotopic transplantation now results in long-term survival in 70% of patients who fail the portoenterostomy operation and long-term survival is now achieved in a majority of children born with biliary atresia.

Biliary Atresia↗
PubMed1997

Complications following the Kasai operation for biliary atresia: European experience.

Biliary atresia is a rare but important disease of infancy having an incidence of about 1 in 16,000 births. In most infants the disease process involves both the intra- and extra hepatic parts of the biliary system (Fig. 1) and histologically it has been characterized as a panductular cholangiopathy. Destruction and disappearance of biliary structures is progressive.

Journal Article↗
PubMed1996

Angiosarcoma of the liver in children.

The clinico-pathological features of four cases of pediatric hepatic angiosarcomas are described. One case was initially diagnosed in infancy and underwent resection of a left-sided benign hemangioendothelioma of the liver. Further resection of a lesion in the right liver was followed by malignant transformation. Primary hepatic resection of the tumor was not possible in three other cases, and all received courses of chemotherapy without significant tumor shrinkage. One child underwent liver transplantation but died 4 months later of immunosuppression complications. The remaining three children died of disseminated malignancy. Histological verification of malignancy was difficult in three cases in which there was discrepancy between the rapid growth of the liver tumor and the microscopic features of benign hemangioendothelioma.

Child↗
PubMed1996

Macroscopic appearance at portoenterostomy--a prognostic variable in biliary atresia.

UNLABELLED: A number of features (eg, age at time of surgery, size of microscopic biliary ductules) have been suggested to be useful predictors of the efficacy of portoenterostomy in the treatment of biliary atresia. However, no previous study has attempted to quantify the macroscopic appearance of the degree of hepatobiliary damage found during initial surgery. METHODS: A macroscopic appearance at portoenterostomy (MAP) score was calculated for 30 infants treated consecutively at King's College Hospital, London. The MAP score was summated from four features (liver consistency, size of portal remnants, degree of portal hypertension, and associated extrahepatic anomalies), with the possible range being from 0 to 6. The infants had follow-up prospectively for a median of 32 months. RESULTS: Twenty infants were anicteric at the time of analysis; their median time to clear jaundice was 48 days (Success group; n = 20). Of the remaining infants, eight had undergone transplantation, one had died awaiting transplantation, and one was still jaundiced (Failure group; n = 10). There was a significant correlation between the MAP score and the age at time of surgery (rB = .57, P = .0005), but only the MAP score was significantly different between the Success and Failure groups (P = .02). Component analysis showed that the portal-remnant subscore contributed most to discrimination (P = .007). The time until clearance of jaundice for the Success group was related to liver consistency (P = .01) and portal remnants (P = .02). CONCLUSION: Simple observation by an experienced surgeon (quantified as the MAP score) was a useful indicator of the success of this procedure and may be as valuable as the more sophisticated histological or biochemical tests.

Bile Ducts↗
PubMed1996

Paediatric surgical manpower in the 1990s.

An accurate audit of consultant manpower in paediatric surgery in the UK was performed based on two surveys carried out in 1992 and 1994. There were 70 paediatric surgeons in 1992 and 84.5 in 1994 (a 21 per cent increase). The ratio of paediatric surgeon per population was approximately 1 in 800,000 for the UK although marked regional differences were noted. The expressed aim of the British Association of Paediatric Surgeons is to achieve a ratio of 1 per 500,000 population which implies a further increase to about 110 consultant posts in the UK. It will be necessary to plan for a commensurate increase in the number of training posts in this specialty.

Child↗
PubMed1995

Experience of orthotopic liver transplantation and hepatic resection for hepatocellular carcinoma of less than 8 cm in patients with cirrhosis.

Fifteen patients with cirrhosis underwent orthotopic liver transplantation for small hepatocellular carcinoma (HCC) and 12 patients with cirrhosis underwent hepatic resection for similar HCC. All tumours were of the non-fibrolamellar variant. The majority of the patients in the transplant group had Child's grade B or C cirrhosis. Median follow-up was 37 months with a minimum of 18 months. Eleven of 12 patients in the resection group had Child's grade A cirrhosis. Median follow-up was 29 months with a minimum of 16 months. Actuarial survival rates at 1 and 3 years for the transplanted patients were 80 and 63 per cent and all were tumour free. Tumour recurrence rate was 15 per cent. The overall 1- and 3-year tumour-free survival rates for patients in the resection group were 61 and 33 per cent. Tumour recurrence rate was 45 per cent. The results show orthotopic liver transplantation to be an important surgical option in cirrhotic patients with small HCC, particularly in those with moderate to severe hepatic decompensation.

Adult↗
PubMed1995

Improved outcome for children with hepatoblastoma.

Between 1981 and 1993, 41 children were treated for hepatoblastoma. Clinical, radiological and pathological data were reviewed retrospectively, focusing on surgical aspects of treatment and outcome. Fourteen children underwent primary resection of the hepatic tumour. One infant with severe congenital anomalies received only palliative treatment. Of 26 with irresectable disease, pulsed cytotoxic chemotherapy (cisplatin and doxorubicin) enabled subsequent surgical excision in 22 and one child with persistent extensive intrahepatic disease was successfully treated by liver transplantation. Thus, with a policy of selective preoperative chemotherapy, 90 per cent of hepatoblastomas were resectable. There were no perioperative deaths from haemorrhage but one child died from an intraoperative tumour embolus. A total of 28 survivors, 27 of whom are disease-free, were followed for a median of 5 years. The cumulative probability of survival in patients treated with intent to cure was 67 per cent. Analysis of survival data suggested a favourable outcome for those with a pure fetal histological tumour subtype. These results demonstrate significant progress in the treatment of hepatoblastoma.

Child↗
PubMed1995

Biliary amylase and congenital choledochal dilatation.

The relationship between levels of biliary amylase measured at operation and clinical features was studied in a series of 55 children with congenital biliary dilatation (choledochal cyst) who presented between 1976 and 1993. There were 36 cystic and 19 fusiforms dilatations in the series. The most common modes of presentation were painless jaundice (n = 23) and pancreatitis (n = 22). Five infants presented with abnormal antenatal ultrasound examinations. Children with pancreatitis were older than those with painless jaundice (4.2 versus 1.5 years; P = .005), and a higher proportion had raised levels of biliary amylase (100% versus 44%; P < .0001). There was no difference in the age at presentation (P = .32), clinical mode of presentation (P = .3), or the level of biliary amylase (P = .25) between cystic and fusiform dilatations. A correlation was found between age at surgery and biliary amylase in the cystic (rs = 0.55; P = .001) but not in the fusiform group (P = .22). All infants with antenatal diagnoses were cystic dilatations. Choledochal cystic dilatations that were diagnosed antenatally did not have significant amylase reflux, suggesting that the aetiology of this subgroup is truly congenital. Children who present at a later age with pancreatitis invariably have high levels of biliary amylase, which is presumed to occur because of a common channel and reflux of biliary and pancreatic secretions.

Adolescent↗
PubMed1995

Prognostic indicators in congenital diaphragmatic hernia.

Congenital diaphragmatic hernia is associated with significant mortality and morbidity. The aim of this study was to compare a series of tests with respect to prediction of outcome. Tidal volume and compliance of the respiratory system (CRS) were measured preoperatively and on the first and second postoperative days. The maximum and modified ventilation indexes and the maximum Paco2 were noted for the first 6 hours of life and the first 6 hours postoperatively. In addition, it was recorded whether the stomach was within the ipsilateral hemithorax preoperatively. Twenty infants were studied (median gestational age, 38 weeks; range, 31 to 40), six of whom had a poor outcome, ie, they died or remained oxygen-dependent after 28 days. A CRS of less than 0.18 mL/cm H2O/kg was the most accurate predictor of poor outcome, with 66% sensitivity and 100% specificity. The authors conclude that lung function measurement are useful in the assessment of infants with congenital diaphragmatic hernia.

Carbon Dioxide↗
PubMed1995

Hemangioendothelioma of the liver in infants.

Hemangioendotheliomas are the most common type of hepatic vascular tumors that present in infancy. Eleven infants (nine boys, two girls) were referred for definitive management from 1970 through 1990. Ten were symptomatic, and the majority required intensive medical therapy because of cardiac failure. All were treated surgically. Three underwent partial hepatectomy for unilobar disease, and eight had ligation of the hepatic artery because of bilobar disease. There were two deaths (18%) in the early part of the series. Ligation of the hepatic artery was completely successful in controlling cardiac failure in six infants and was partially successful in one. There are two surgical options for treating symptomatic hepatic hemangioendotheliomata in infancy. Bilobar multifocal disease can be treated successfully by ligation of the hepatic artery; if localized, hemangioendothelioma can be resected, with rapid control of symptoms.

Blood Loss, Surgical↗
PubMed1995

Congenital hepatoportal arteriovenous fistula.

BACKGROUND: Two children with congenital hepatoportal arteriovenous fistulas have been investigated and treated surgically. These cases have been reviewed with five cases previously reported. METHODS: Two children, 5 months and 14 months of age, presenting with failure to thrive, hepatosplenomegaly, ascites, and recurrent gastrointestinal bleeding with evidence of portal hypertension, were found to have congenital hepatoportal arteriovenous fistulas. RESULTS: Doppler ultrasonographic examination was important in identifying abnormal portal venous flow. Angiogram identified the fistulas, confirming the diagnosis. Both patients had significant portal hypertension (pressure more than 30 mm Hg). Surgical resection in one child was unsuccessful, but surgical ligation of the hepatic artery controlled the symptoms in both patients. CONCLUSIONS: Rapid collateralization of the hepatic arterial blood supply made embolization a short-term therapeutic measure, and surgical ligation of the hepatic artery is the treatment of choice for congenital hepatoportal arteriovenous fistulas.

Arteriovenous Fistula↗
PubMed1995

Choledochal cysts: lessons from a 20 year experience.

Cystic dilatation of the biliary tree is a rare congenital anomaly. To determine mode of presentation, diagnostic pitfalls, and long term outcome after surgery, 78 children (57 girls, 21 boys) with choledochal cyst treated between 1974 and 1994 were reviewed. Anatomical types were: Ic (n = 44), If (n = 28), IVa (n = 4), and V (n = 2); a common pancreaticobiliary channel was identified in 76% patients. Age at presentation ranged from 0-16 (median 2.2) years, six patients being diagnosed by prenatal ultrasonography. Of the 72 patients diagnosed postnatally, 50 (69%) presented with jaundice, associated with abdominal pain in 25 or a palpable mass in three, 13 (18%) presented with pain alone, and two (3%) with a palpable mass. The classic triad of jaundice, pain, and a right hypochondrial mass was present in only four (6%). Four children presented acutely after spontaneous perforation of a choledochal cyst, two presented with ascites and one cyst was discovered incidentally. Plasma and/or biliary amylase values were raised in 30 of 31 patients investigated for abdominal pain; seven had evidence of pancreatitis at operation. In 35 of 67 (52%) patients referred without previous surgery, symptoms had been present for more than one month, and in 14 of them for more than one year, before diagnosis. Delayed referral was due to misdiagnosis as hepatitis (n = 12), incomplete investigation of abdominal pain (n = 6), and failure to note the significance of ultrasonographic findings (n = 10). Two patients referred late died from liver failure. Of the 76 patients with type I or IV cysts, 59 underwent radical cyst excision and hepaticojejunostomy as a primary procedure and 10 as a secondary operation after previously unsuccessful surgery. Sixteen patients have been lost to follow up but most of the remainder are well after a mean period of 4.1 (0.1-13) years. Choledochal cysts are often misdiagnosed, but prognosis is excellent if radical excision is performed.

Adolescent↗
PubMed1994

Patterns of portal vein occlusion and their aetiological significance.

Clinical and angiographic findings in 53 patients with portal vein occlusion were reviewed to determine the relationship between the pattern of venous occlusion and its aetiology. Five major patterns were identified. Group 1 comprised three children with idiopathic intrahepatic portal vein occlusion alone (hepatoportal sclerosis). Those in group 2 (25 patients), with occlusion of the main portal vein, accounted for almost half the total number and for most of those with a probable congenital aetiology. The ten patients in group 3 had angiographic occlusion and collateralization of the main portal and superior mesenteric veins. Intra-abdominal sepsis, other than that from pancreatic disease, was associated with this pattern of venous occlusion in which the splenic vein is spared. No patients with isolated portal and splenic vein occlusion (group 4) were recorded. Widespread thrombosis throughout the portal venous system (group 5; 15 patients) had a multifactorial aetiology but was typically observed in hypercoagulable states that were also associated with hepatic vein occlusion or other deep vein thromboses.

Adolescent↗