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Biomedical subjects

C Dicks-Mireaux

Publications and source records attributed to C Dicks-Mireaux.

At least 37 records · Page 2Linked to original sources
PubMed1994

Conservative surgery in multimodal therapy for pelvic rhabdomyosarcoma in children.

Twenty-six previously untreated children, median age 3.4 years, with pelvic rhabdomyosarcoma (RMS) were seen between 1983 and 1988. Fourteen were girls. The planned strategy was to conserve pelvic organs, especially the bladder, by using primary chemotherapy, conservative surgery and, in most cases, radiotherapy. With a median follow-up of 71 months (range 34-103 months) overall survival was 73%, with no treatment-related death. The bladder salvage rate of 88% in survivors with bladder base/prostate primaries was much higher than that reported by the United States Intergroup Rhabdomyosarcoma Studies (IRS), though many of the preserved bladders did not function normally. We identified problems with both radiological and histological off-treatment monitoring. The overall accuracy of computerised tomographic (CT) scanning for prediction of tumour recurrence was only 81%, and endoscopic biopsies proved misleading in four of the ten bladder base/prostate patients monitored by serial cystoscopy. We conclude that a higher cure rate can be achieved by using intensive chemotherapy/radiotherapy and conservative surgery to treat children with pelvic RMS. Factors that might contribute to our favourable bladder salvage results, compared with those of the IRS, include (a) the fact that one of two specialist surgeons monitored and operated on all these patients and (b) our increasing awareness, during the study, that post-chemotherapy/radiotherapy histopathology and pelvic CT scan appearances may be misleading. Referral to paediatric centres with special experience of pelvic RMS may help raise the rate of bladder salvage in these children.

Adolescent↗
PubMed1994

Hepatic ultrasound findings in the glycogen storage diseases.

Hepatic ultrasonography was performed on 70 patients with the hepatic glycogen storage diseases (GSDs) to assess parenchymal echogenicity. 27 patients had GSD-I, 24 had GSD-III and 19 had GSDs-VI/IX; ages varied from 0.6 to 35.7 years (median 11.7). 31 (44%) had normal or mild parenchymal changes, and 41% (11/27) of those with GSD-I, 25% (6/24) with GSD-III and 11% (2/19) with GSDs-VI/IX had marked changes. No relationships were found between the ultrasonographic appearances and other indices of metabolic control, including plasma triglycerides, total cholesterol or height standard deviation score. Seven adult patients (21-29 years) were found to have hepatic tumours: six with GSD-I and one with GSD-III. Those with GSD-I and tumours tended to have the more severe hepatic parenchymal changes. We conclude that ultrasonography may be useful in identifying patients with GSD-I at risk of hepatic tumour formation.

Adenoma↗
PubMed1993

Phrenic nerve damage via a right thoracotomy in older children with secundum ASD.

Phrenic nerve damage (PND) in children after cardiac operations is now recognized as being more frequent than previously thought. In a prospective study on 400 children, we previously demonstrated electrophysiologic evidence of postoperative PND in approximately 16% of patients, with one third of cases occurring in children under 18 months. In the past 18 months, 30 children have had atrial septal defect (ASD) repairs as their only operative procedure. Fourteen children had ASD repairs via a midline incision, and 16 ASD repairs were via a right thoracotomy. No PND (assessed by phrenic nerve latency) was found after a midline approach. In the right thoracotomy group, 5 children had evidence of PND (31%; p = 0.05). Four of these 5 patients were female and more than 14 years of age. The incidence of damage in this pubescent group was 80% (p < 0.05). In the older age group the duration of ventilation was not prolonged, but affected patients had symptoms of fatigue and breathlessness postoperatively. These data suggest a strong association between right thoracotomies for ASD repairs and PND, especially in the female pubescent group when a low submammary skin incision (seventh to eighth space) is used with a fifth to sixth space entry into the thoracic cavity. In conclusion, the right thoracotomy approach for ASD repair appears to be a significant risk factor for PND in older children.

Adolescent↗
PubMed1992

The magnetic resonance appearances of the normal thymus in children.

The normal thymus in children is highly variable in size and shape. In some cases this has led to the misdiagnosis of mediastinal pathology and an unnecessary thoracotomy. Twenty-five children without suspicion of mediastinal pathology and five children with suspected mediastinal pathology were imaged using magnetic resonance imaging (MRI). MRI is recommended to distinguish between the normal and abnormal thymus in difficult cases where other imaging techniques are inconclusive.

Child↗
PubMed1991

Risks and benefits of percutaneous biopsy and primary chemotherapy in advanced Wilms' tumour.

Between 1982 and 1988, 36 children with advanced Wilms' tumour underwent percutaneous trucut needle biopsy followed by chemotherapy before definitive surgery. Nephrectomy was performed after a median of 14 weeks of chemotherapy. Substantial reduction in tumour bulk was achieved in 94% of patients. Biopsy morbidity was low and complete concordance between the histological assessment of the tumour in the biopsy specimen and at subsequent nephrectomy was confirmed in 26 of 28 (93%) patients. The overall clinical value of trucut biopsy was 83% (30/36 patients). Survival rates in this high-risk group were comparable to those of children with less advanced disease. Chemotherapy may be the primary treatment of choice for patients with Wilms' tumour. Percutaneous biopsy allows definition of histology in most patients without increasing morbidity.

Adolescent↗
PubMed1991

Wilms' tumour: pre- and post-chemotherapy CT appearances.

Pre-operative chemotherapy is used in our institution for patients with Wilms' Tumours (WT) when surgical 'operability' is in doubt. To date, the computed tomographic (CT) appearances of chemotherapy-induced changes in WT have not been described. We have analysed CT examinations of 18 children undergoing pre-operative chemotherapy to assess the effects of treatment on size, extent and qualitative changes of the tumour. Clinical response to chemotherapy was associated with a reduction in tumour size of at least 50%. Cystic changes were commonly seen within tumours following chemotherapy. CT did not reliably differentiate lymph nodes involved by tumour from those showing only reactive change. Pre-chemotherapy CT scans were incorrect in predicting liver invasion in 4/18 (22%) cases: of these, two were right-sided tumours, and two were bilateral.

Child↗
PubMed1990

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↗
PubMed1990

Neonatal hypothyroidism: comparison of radioisotope and ultrasound imaging in 54 cases.

Fifty-four neonates with congenital hypothyroidism identified by the North East and North West Thames Regional hypothyroid screening programme between January 1985 and December 1987 were investigated with radioisotope (Tc99m) and ultrasound scans of the thyroid before treatment with 1-thyroxine was commenced. Compared with the radioisotope scans, ultrasound identified normally sited thyroid tissue in only 7 out of 10 cases, and ectopic thyroid tissue in only 5 out of 26 cases. Three out of 18 cases with no isotope uptake in the neck appeared to have normally sited tissue on ultrasound scan. We conclude that in our hands ultrasound of the neck is of only limited value in the assessment of young infants with congenital hypothyroidism.

Congenital Hypothyroidism↗
PubMed1990

Hepatic imaging with computed tomography of chronic tyrosinaemia type 1.

Tyrosinaemia type 1 (fumaryl acetoacetase deficiency, hepato-renal tyrosinaemia) is a rare inborn error of metabolism which, in its chronic form, leads to cirrhosis in early childhood and subsequent development of hepatocellular carcinoma in a high proportion of cases. Imaging with computed tomography has an important role in assessing the progress of the liver disease and may be helpful in timing liver transplantation. The radiological features of seven cases are described and the implications discussed.

Amino Acid Metabolism, Inborn Errors↗
PubMed1990

Malignant mesenchymoma associated with a congenital lung cyst in a child: case report and review of the literature.

Primary lung tumors are uncommon in children, and malignant mesenchymal tumors form only a small proportion of these. Leiomyosarcomas occur more commonly than rhabdomyosarcomas, whereas malignant mesenchymomas are exceedingly rare. Of the total number of primary pulmonary rhabdomyosarcomas and malignant mesenchymomas of lung reported in children, 50% have occurred in association with congenital lung cysts. The relationship between abnormal morphogenesis and neoplasia is well documented in the kidney. A similar relationship may exist in the lung between cystic parenchymal maldevelopment and embryonal mesenchymal tumors. We report a 4-year-old boy with a malignant mesenchymoma of lung arising within a congenital lung cyst; one similar case has previously been reported to our knowledge.

Child, Preschool↗
PubMed1990

Skeletal assessment in neuroblastoma--the pitfalls of iodine-123-MIBG scans.

This study was carried out to compare iodine-123 metaiodobenzylguanidine ([I123I]MIBG) and technetium-99m-methylene diphosphonate bone scans (99mTc-MDP) in the detection of skeletal involvement by neuroblastoma. Forty-four children with neuroblastoma underwent both [123I] MIBG and 99mTc-MDP scans within a 4-wk period; bone marrow examination also was performed; all these investigations were done both at diagnosis and at follow-up. At diagnosis, four children with Stage 4 disease had normal [123I]MIBG scans but abnormal 99mTc-MDP scans, while at follow-up there were four children with negative [123I]MIBG studies who later died from disseminated neuroblastoma. All eight scans are considered false-negative. In 24 children, the [123I]MIBG revealed more extensive disease with 161 positive sites while the 99mTc-MDP scan showed only 100 positive sites; 34 of these sites were common to both studies. This study shows that underassessment of skeletal involvement by neuroblastoma occurred using [123I]MIBG scans and that one cannot therefore substitute [123I]MIBG for 99mTc-MDP bone scans in the staging of neuroblastoma.

3-Iodobenzylguanidine↗
PubMed1989

Osteosarcoma--presumed lymph node metastases in two cases.

Two patients with osteosarcoma of a lower limb and presumed lymph node metastases are described. The metastases presented in both children as palpable masses, one in the inguinal region and the other in the pelvis. One was visible on plain radiography; both were demonstrated on computed tomography and bone scintigraphy. The inguinal mass was present at the first examination and was resected with the primary lesion. The pelvic mass presented 12 months after amputation and was unresectable; its subsequent rapid growth was demonstrated by computed tomography. In one patient the osteosarcoma was of the osteoblastic type.

Adolescent↗
PubMed1989

Difficulties in diagnosis of congenital H-type tracheo-oesophageal fistulae.

The case notes of 12 children with congenital H-type tracheo-oesophageal fistulae diagnosed at the Hospital for Sick Children, Great Ormond Street, who presented between 1980 and 1986 were reviewed. All patients presented early in the neonatal period with recurrent chest infections; abnormal chest radiographs were found in eight. Ten of a total of 19 contrast studies were negative. Tube oesophagograms were more likely to demonstrate a fistula than conventional contrast studies. Any delay in surgery was due to delay in diagnosis rather than to delay in presentation. The results suggest that tube oesophagograms should be performed early where there is clinical suspicion of an H-type fistula, and that other investigations (for example bronchoscopy) should be considered if the tube oesophagogram does not demonstrate a fistula.

Abnormalities, Multiple↗