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Biomedical subjects

E R Howard

Publications and source records attributed to E R Howard.

At least 55 records · Page 3Linked to original sources
PubMed1994

Does the morphology of the extrahepatic biliary remnants in biliary atresia influence survival? A review of 205 cases.

Resected extrahepatic remnants taken at the time of portoenterostomy were examined in a single-center review of 205 cases of biliary atresia. The morphological features of the size and number of residual ducts at the porta hepatis and the degree of inflammation at the porta hepatis were analyzed using a semiquantitative scoring system. The morphology of the common hepatic and common bile duct was classified into seven types. These features were then related to age at time of initial surgery and to survival. This showed that few or absent ductal remnants at the porta hepatis and absence of portal inflammation were predictors of poor prognosis. These histological features may represent the "burnt out" end result of the disease process. There was no correlation between age at time of portoenterostomy and either portal duct patency or portal inflammation. The common hepatic and common bile duct were variably involved in the sclerosing process, but the patterns of obliteration were not indicative of prognosis. The severity of intrahepatic biliary cholangiopathy and the extent of liver damage may ultimately be more important to survival in the long term.

Bile Ducts, Extrahepatic↗
PubMed1994

Extrahepatic biliary atresia: a first-trimester event? Clues from light microscopy and immunohistochemistry.

Biliary atresia is an obliterative disorder of the bile ducts, causing obstructive jaundice in neonates. In this study, the developing biliary system of normal human embryos and fetuses was examined and compared with the resected extrahepatic biliary remnants from 205 cases of biliary atresia. At the porta hepatis level, it was found that the primary biliary ductal plate undergoes a specific sequence of remodelling, resulting in the formation of large tubular bile ducts surrounded by thick mesenchyme, between 11 and 13 weeks postfertilisation. These developing ducts are in luminal continuity with the extrahepatic biliary tree throughout gestation. Contrary to long-held belief, no "solid phase" was observed in the development of the extrahepatic bile duct. Examination of the biliary remnants in biliary atresia showed that the porta hepatis is encased in fibrous tissue, and a variable pattern of obliteration of the common hepatic and common bile ducts was observed. Anticytokeratin immunostaining showed similarities between the abnormal ductules within the porta hepatis in biliary atresia, and the developing bile ducts in the first trimester. Biliary atresia may be caused by failure of the remodelling process at the hepatic hilum, with persistence of fetal bile ducts poorly supported by mesenchyme. As bile flow increases perinatally, bile leakage from these abnormal ducts may trigger an intense inflammatory reaction, with subsequent obliteration of the biliary tree.

Bile Ducts, Extrahepatic↗
PubMed1994

Longterm outcome after injection sclerotherapy for oesophageal varices in children with extrahepatic portal hypertension.

A consecutive series of 36 children with bleeding from oesophageal varices secondary to extrahepatic portal hypertension was successfully treated by endoscopic injection sclerotherapy and followed up over a mean period of 8.7 years after variceal obliteration. There were no deaths from portal hypertension or its treatment and morbidity related to oesophageal sclerotherapy was minimal. Endoscopic injection sclerotherapy alone proved safe and effective in controlling variceal bleeding from portal hypertension in over 80% of the children. Recurrent variceal bleeding developed in 10 (31%) patients but half of these were effectively treated by further sclerotherapy. Gastric variceal bleeding unresponsive to sclerotherapy necessitated successful portosystemic shunt surgery in four (13%) patients. Two children required splenectomy for painful splenomegaly. In most children injection sclerotherapy is the best treatment for the primary management of bleeding oesophageal varices, reserving portosystemic shunting or other surgical procedures for those with bleeding from gastrointestinal varices.

Adolescent↗
PubMed1993

Incidence of haemorrhoids and anorectal varices in children with portal hypertension.

A prospective study of 60 children with portal hypertension showed a significant incidence of haemorrhoids (33 per cent), anorectal varices (35 per cent) and external anal varices (15 per cent). Four children (7 per cent) complained of anorectal symptoms. A comparison of extrahepatic with intrahepatic disease showed that the former was associated with a higher incidence of both haemorrhoids and anorectal varices (57 versus 26 per cent and 64 versus 26 per cent respectively). The occurrence and severity of haemorrhoids was related to the number of previous oesophageal sclerotherapy sessions (P = 0.001). Problems relating to anorectal pathology were unusual, but treatment with haemorrhoidal sclerotherapy or banding was satisfactory for the symptomatic patients.

Adolescent↗
PubMed1993

Hepatic histology and the development of esophageal varices in biliary atresia.

Histological features in liver biopsy specimens taken from 71 infants at the time of surgery for biliary atresia (portoenterostomy) were analyzed using a scoring system and compared with an endoscopic grading of esophageal varices performed at a mean age of 3.4 years. The analysis showed no correlation between a "global" score, which represented the severity of all histological changes in the original biopsy specimen, and the severity of esophageal varices. Further analysis also showed no correlation with any individual histological feature (eg, fibrosis). These findings failed to confirm a previous study, which suggested a relationship between liver changes at surgery and the later development of esophageal varices in children with biliary atresia.

Biliary Atresia↗
PubMed1993

Chemotherapy effects on hepatoblastoma. A histological study.

The histopathological features of hepatoblastoma in 17 patients treated with preoperative chemotherapy were compared with those in 11 patients not subjected to chemotherapy during the same 11-year period. Tumor necrosis was more extensive in patients receiving preoperative chemotherapy. Two tumors, however, were apparently unaffected by chemotherapy. There was no obvious correlation between the extent of necrosis and the number of courses of chemotherapy. There also seems to be no evidence of preferential ablation of a particular morphological type of tumor. The most notable feature in cases treated with chemotherapy was the extensive presence of osteoid. Osteoid was present in 36% of untreated cases, occupying < 5% of the surface area, compared with 82% in the treated group. In seven cases, osteoid occupied > 40% of the surface area. This finding raises speculation about the role of chemotherapy in the maturation of tumors that have an inherent ability to differentiate. A long-term study is needed to clarify the prognostic significance of mature heterologous elements in hepatoblastoma.

Adolescent↗
PubMed1993

Association between HLA and extrahepatic biliary atresia.

The etiopathogenesis of extrahepatic biliary atresia (EHBA) remains undefined. There are clinical and pathological suggestions supporting the idea that EHBA could consist of at least two forms: the congenital (embryonic or fetal) and the acquired (perinatal) types. To test the hypothesis that susceptibility to this disease would be influenced by host genetic factors, we studied the human leukocyte antigen (HLA) system in 55 patients with and without major extrahepatic congenital anomalies. We found, especially in those without associated malformations, a significantly higher frequency of HLA-B12, of haplotypes A9-B5 and A28-B35, and of their disequilibrium values, as compared with the 8th International Histocompatibility Workshop controls. This study suggests that immunogenetic factors may play a role in determining susceptibility to EHBA, and the different HLA frequencies in those with and without anomalies lend support to the hypothesis that biliary atresia may be an etiologically heterogeneous disorder.

Biliary Atresia↗
PubMed1993

Complications and limitations of injection sclerotherapy in portal hypertension.

Injection sclerotherapy is now the accepted first line treatment for bleeding oesophageal varices, although it is associated with an impressive list of rare complications. The main problem concerns the strategy for uncontrollable or recurrent bleeding. Patients with uncontrolled bleeding may be referred for surgery after considerable blood loss and are then extremely difficult to assess. The effects of blood loss on liver function can lead to an unduly pessimistic assessment of liver status. An effective choice of emergency surgical procedure may require considerable surgical expertise. Oesophageal transection and devascularisation are satisfactory for many patients with oesophageal varices secondary to cirrhosis and should nearly always control bleeding. Difficulties arise in patients who are grossly obese and in those who have undergone extensive surgery in the upper abdomen. Problems may also be encountered in those treated by repeated sclerotherapy, which may have caused severe inflammatory change and thickening around the lower oesophagus and upper stomach. We believe that an emergency mesocaval shunt using either a vein graft or a synthetic material such as polytetrafluoroethylene is the procedure of choice for this difficult group of very sick patients. The surgical exposure is satisfactory and not unduly prolonged in even the largest patients and the technique does not interfere with any subsequent transplant operation. There is a greater choice in the management of the patient with less urgent bleeding from recurrent varices after sclerotherapy. Repeat sclerotherapy may be effective for small oesophageal varices while liver transplantation may be indicated in the patient with deteriorating liver function. A selective distal splenorenal shunt should be considered for patients with intact splenic and left renal veins and a mesocaval vein graft for the remainder. We would therefore suggest that surgery should still be considered for the management of portal hypertension, particularly in the following circumstances: (1) Uncontrollable bleeding during the initial course of sclerotherapy; (2) Life threatening haemorrhage from recurrent varices; (3) Bleeding from ectopic varices not accessible to sclerotherapy; (4) Uncontrollable bleeding from oesophageal ulceration secondary to injection sclerotherapy; (5) Severe, symptomatic hypersplenism; (6) For patients who live in communities remote from blood transfusion facilities and adequate medical care. The management of the complications of portal hypertension continues to pose problems. We believe that the best results should come from a combined management approach using injection sclerotherapy as primary treatment and surgery for complications and for haemorrhage from unusual anatomical sites.

Esophageal and Gastric Varices↗
PubMed1993

Non-communicating multiseptate gall bladder and choledochal cyst: a case report and review of publications.

A 14 year old girl with multiseptate gall bladder and cystic dilatation of the biliary tree is presented. This is the 20th published case report of patients with multiseptate gall bladder and only the second to be associated with a choledochal cyst. The cystic spaces of the gall bladder did not communicate with the neck of the gall bladder or the rest of the biliary tree, and this unusual feature has not been previously described. A multiseptate gall bladder with a normal biliary tree commonly causes symptoms suggestive of cholecystitis, although gall stones are seldom present. Diagnosis is confirmed by an oral cholecystogram or ultrasound scan that may show the fine intraluminal septae, and these features should be looked for in patients with biliary symptoms without biliary calculi. Cholecystectomy is curative for the isolated gall bladder anomaly but hepaticojejunostomy may be necessary for an associated choledochal cyst.

Adolescent↗
PubMed1993

Biliary atresia splenic malformation syndrome: an etiologic and prognostic subgroup.

BACKGROUND: The polysplenia syndrome is the most common extrahepatic anomaly found in association with extrahepatic biliary atresia. This subgroup may have a different cause and a worse prognosis than do infants with biliary atresia alone, and this hypothesis has been tested by analyzing the King's College Hospital series. METHODS: The case records of 308 infants treated between 1975 and 1991 for biliary atresia were examined for extrahepatic anomalies. RESULTS: Twenty-three (7.5%) infants had polysplenia and biliary atresia. There were also four infants with other types of splenic malformation: two with double spleen and two with asplenia. The presence of other anomalies such as situs inversus and portal vein anomalies in all the categories of splenic malformation suggests that they formed part of a larger association for which we now propose the term biliary atresia splenic malformation (BASM) syndrome. There was no difference in age at presentation and in biochemical test results of liver function before operation between infants with BASM and those with biliary atresia alone. Four (15%) infants with BASM were born to mothers with diabetes (three insulin dependent and one with gestational diabetes treated by diet alone). There were no other cases of maternal diabetes in the series as a whole. Actuarial "survival" (death or transplant) of infants with BASM after initial corrective operation was worse than that in a control group without anatomic anomalies (p < 0.05). CONCLUSIONS: BASM syndrome appears to be a distinct subgroup in infants with biliary atresia. This subgroup may have a different cause and tends to have a worse prognosis than do control subjects. Whether this is caused by the presence of the other anomalies (e.g., cardiovascular anomalies), which are in themselves detrimental, is unclear.

Abnormalities, Multiple↗
PubMed1992

Symptomatic hemorrhoids and anorectal varices in children with portal hypertension.

There have been few reports of the anorectal problems encountered in children with portal hypertension. We report the incidence of anorectal symptoms in a retrospective study of 189 children treated by injection sclerotherapy for esophageal varices secondary to portal hypertension. Anorectal symptoms, proctoscopic findings and treatment have been recorded in this group. The incidence of symptomatic hemorrhoids and rectal varices was 4.2%. Hemorrhoids are rare in children and the increased incidence can be assumed to be secondary to portal hypertension. Treatment is advised only for symptomatic patients and injection sclerotherapy is satisfactory for the majority.

Child↗
PubMed1992

The late presentation of postero-lateral congenital diaphragmatic hernias.

Four patients presenting after the neonatal period with a combination of respiratory and gastro-intestinal symptoms and signs which point to the diagnosis of congenital postero-lateral diaphragmatic hernias are discussed. Late presentation is more common than previously acknowledged. Early correct diagnosis and treatment are associated with an excellent clinical outcome.

Adult↗
PubMed1992

The relative importance of familial, reproductive and environmental factors in biliary atresia: etiological implications and effect on patient survival.

1. The clinical records of 237 patients with extrahepatic biliary atresia (EHBA) attending King's College Hospital, London, between March 1973 and October 1985 were analyzed in terms of familial and reproductive factors, as well as of their possible effect on patient survival. 2. The male:female ratio was 0.93, and the ages of the patients' mothers and fathers were significantly higher than would have been expected from sibship data. Similarly, the number of first-born children having EHBA was less than expected. 3. In a subsample of 189 mothers, the obstetrical histories of women who had children with associated EHBA (20% of the total) showed: 1) a higher frequency of illness before and during pregnancy; 2) a higher level of pharmaceutical drug consumption during pregnancy, and 3) more fetal losses, especially in the gestation immediately preceding the patient's birth. 4. Age at death (average 15.1 +/- 13.2 months) and survival rates depend critically on surgical intervention and were not related to the presence or absence of extrahepatic malformations or to the type of atresia. 5. The present observations, taken together with those of others, indicate that problems in the reproductive process or exposure to noxious environmental agents may be etiological factors in associated EHBA.

Adolescent↗