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Biomedical subjects

E R Howard

Publications and source records attributed to E R Howard.

At least 19 recordsLinked to original sources

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

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

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

Spontaneous perforation of the bile duct in infants.

Spontaneous perforation of the bile duct is a rare but well documented condition of infants. Six infants are described, five presenting within 2 months of birth without apparent antecedent factors and one associated with an 'acquired' type I biliary atresia at 8 months. All infants underwent definitive surgery, which included decompression of the biliary tree with cholecystenterostomy or T tube drainage. There was no morbidity or mortality associated with such surgical intervention.

Anastomosis, Roux-en-Y

Pre-operative radiological and clinical assessment of hepatic tumours--is biopsy necessary?

The pre-operative investigations of 72 consecutive patients who underwent liver resection for hepatic tumours have been reviewed. A combination of clinical, biochemical and radiological findings correctly predicted histology in 93% of cases (the benign or malignant nature in 97%). Biopsy was performed in only 20 patients. Five of the biopsy reports were incorrect (75% accuracy, benign or malignant nature accuracy 90%). Although guided biopsy can be performed during radiological assessment of focal hepatic lesions we argue that it is rarely indicated and can be misleading in patients proceeding to hepatic resection.

Biopsy

Cholestasis caused by biliary botryoid sarcoma.

A 4-year-old boy with a short history of jaundice and hepatomegaly was studied by endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC). A large obstructing choledochal tumour was found at the level of cystic duct insertion and at surgery, a botryoid sarcoma was removed. Subsequent cytotoxic therapy was complicated by persistent dilatation of the extrahepatic bile ducts which necessitated eventual excision. There was no evidence of persistent tumour.

Bile Duct Neoplasms

Congenital structural abnormalities in biliary atresia: evidence for etiopathogenic heterogeneity and therapeutic implications.

The clinical, surgical, laboratory and histological data of 237 children with extrahepatic biliary atresia were reviewed. Forty-seven patients (20%) had associated congenital anomalies, and of these, 28 had cardiovascular, 22 digestive and 19 splenic malformations. Of the 19 patients with splenic malformations, 13 showed the polysplenia syndrome and two had asplenia. Chromosome studies were performed in eight children, six having associated anomalies, and two of them showed karyotype abnormalities (46,XX,del 18 p- and 49,XXXXY). These observations indicated that biliary atresia could be subdivided into four distinct etiopathogenic subgroups, three involving a congenital form that could arise through a malformation, a disruption or a chromosome abnormality, and the remaining to agents active in the perinatal period (the acquired form). The surgical outcome in 171 patients operated on by an experienced surgeon was not influenced by the presence of anomalies but by the timing of surgery. Seventy-one percent of 24 patients operated on by 8 weeks of age were jaundice-free as opposed to only 34% of those who had later surgery (p less than 0.01).

Abnormalities, Multiple

Intraluminal biliary obstruction.

Jaundice caused by intraluminal bile duct obstruction in infancy is rare but may occur in association with biliary sludge, inspissated bile plugs, or gall stones. Nine boys (aged 2 weeks-6 months) with obstruction caused by inspissated bile (n = 7) or gall stones (n = 2) are presented. Haemolysis was not a factor in the patients' histories but an abnormal entry of the common bile duct into the third part of the duodenum was demonstrated in two and one had an asymptomatic haemangioma. Ultrasonography was the most useful investigation. Surgical removal of the bile duct obstruction was necessary in eight cases and included biliary tract drainage in six and cholecystectomy for changes of cholecystitis in four. Obstruction resolved spontaneously in one infant after percutaneous cholangiography. There were no postoperative complications.

Bile

Focal nodular hyperplasia of the liver: a link with sickle cell disease?

Focal nodular hyperplasia is a benign liver tumour that is rare in children. We report the second case of a child with sickle cell disease presenting with symptomatic focal nodular hyperplasia. The possible pathogenesis of focal nodular hyperplasia and the association with sickle cell disease are discussed.

Child

Focal nodular hyperplasia of the liver: results of treatment and options in management.

Twenty two patients (19 females) with focal nodular hyperplasia were seen between 1973 and 1989. Five were children, and all the adults were aged under 42 years (median 33 years). Fourteen patients (64%) were symptomatic on presentation. Twelve of the 14 adult women had taken the oral contraceptive pill. Twelve patients, nine of whom were symptomatic, underwent hepatic resection shortly after presentation. There were no deaths or major complications, and all remain well on follow up. Four patients underwent either hepatic artery embolisation or ligation. After an interval of six to 10 years they were asymptomatic and only one has histological evidence of residual focal nodular hyperplasia. Of five patients initially treated conservatively, two were asymptomatic and have remained so for three and 13 years. One of the three symptomatic patients became symptom free after stopping the contraceptive pill. The management of focal nodular hyperplasia requires a flexible approach. Lesions which are asymptomatic can be observed with regular ultrasound and treated if they enlarge or become symptomatic. Symptomatic patients who present while taking the contraceptive pill can also have a trial of conservative treatment. Other symptomatic patients, including those who previously took the pill, are best treated by surgical resection, and, where this is not possible, by embolisation.

Adolescent