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Biomedical subjects

E R Howard

Publications and source records attributed to E R Howard.

At least 73 records · Page 4Linked to original sources

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↗

Extrahepatic biliary atresia and twinning.

1. Four pairs of discordant twins were observed in a series of 237 extrahepatic biliary atresia patients ascertained in London. 2. The twinning prevalence (1.7%) was as expected considering the ethnic composition of the sample. 3. Out of a total of 17 other twin pairs reported in the literature, only one was concordant for the disease. Since only 17 instances of familial cases have been described, the conclusion is that any influence of genetic factors in this condition is likely to be manifested indirectly, possibly in the form of increased susceptibility of the biliary epithelium to infectious or toxic agents.

Biliary Atresia↗

Selective arterial embolisation for hepatic trauma.

The technique of selective arterial embolisation for patients presenting with major hepatic trauma is described. We have used this technique successfully in four patients, three of whom had continued bleeding after emergency laparotomy. It can abolish localised intrahepatic arterial haemorrhage and arteriovenous shunting. Selective embolisation may prove to be a useful technique in reducing the mortality of major hepatic trauma.

Adolescent↗

Primary malignant liver tumors in childhood: assessment of resectability with high-field MR and comparison with CT.

Nine children (mean age 20 months), with proven primary malignant hepatic tumors have been examined prospectively by high-field magnetic resonance (MR) imaging to assess tumor resectability. All patients had comparative ultrasonography (US), 8 patients had X-Ray computed tomography (CT), and surgical correlation was available in 8 patients. The hepatic and portal veins and the inferior vena cava were visualized in all patients on MR and in 4 of 8 patients on CT. MR accurately defined liver parenchymal involvement in all 8 patients who had surgical exploration. CT underestimated disease in 2 cases, and defined tumour margins less clearly than MR. MR identified abnormal extrahepatic tissue when present, but was unable to distinguish viable tumor from necrotic tumor or reactive nodes. High quality short TR/short TE spin echo images were obtained by combining cardiac triggering and signal averaging. Short TI inversion recovery images demonstrated tumor and lymphadenopathy most clearly. We conclude that MR is the imaging method of choice for the assessment of liver tumor resectability in children.

Child, Preschool↗

Chronic intussusception.

Chronic intussusception is a rare but completely correctable cause of failure to thrive in infants and children. The presenting features differ from acute intussusception. We present the case of a 16 month old boy presenting with a three week history of anorexia, diarrhoea, and weight loss with subsequent delayed diagnosis.

Chronic Disease↗