Use of a tissue expander and a polyglactic acid (Vicryl) mesh to reduce radiation enteritis: case report and literature review.

Management of stage IV rhabdomyosarcoma comprises systemic chemotherapy with local control by conservative surgery and radiotherapy. Abdominal radiotherapy may lead to radiation enteritis causing such serious morbidity as malabsorption, fistulae or stricture formation. The risk increases with the dose of radiation and length of bowel involved. Various methods have been utilised to displace the bowel from the radiation field. Usually these are applied in patients requiring pelvic irradiation. We report a case of metastatic alveolar rhabdomyosarcoma requiring radiotherapy to the right renal bed. Effective displacement of small bowel from the tumour site was achieved by a combined use of a tissue expander and Vicryl mesh. There were no complications from the surgery. This is the first report discussing combined use of a tissue expander and Vicryl mesh to aid radiotherapy to the renal fossa in a paediatric patient.

Sacrococcygeal teratomas: the UK Children's Cancer Study Group's experience. I. Neonatal.

The aim of this study was to review the United Kingdom Children's Cancer Study Group (UKCCSG) experience of sacrococcygeal teratomas (SCT) including histological presentation, response to surgery and chemotherapy, and long term effects of the tumour and treatment. This paper presents the results for those children diagnosed during the neonatal period. Children aged up to 4 weeks with biopsy proven localised or metastatic sacrococcygeal germ cell tumours were eligible. From 1st January 1989 to 31st December 1997 (9 years), 15 UKCCSG centres registered 51 neonates with SCT into GC 8901. Surgery alone was performed in all and the prognosis was good - except for 1 baby who died from massive haemorrhage at the initial operation and 1 who died from the complications of prematurity. Seven of the 51 children (14%) who had teratomas in the neonatal period (5 mature, two immature) had yolk sac tumour (YST) recurrence at: 4, 12, 15, 20, 20, 28 and 32 months of age. These children received chemotherapy in the form of etoposide/bleomycin/carboplatin (JEB) and are alive and well at review. These results emphasise the need for oncological follow-up of SCT and the good response to JEB chemotherapy of malignant teratomas and YST.

A case of fatal tumor embolus following trauma in a patient with undiagnosed Wilms' tumor.

Involvement of the inferior vena cava with tumor thrombus has been reported in 5 to 10% of patients with Wilms' tumor. Preoperative imaging usually alerts the surgeon to the extent of the intravascular extension. We present a case report of trauma to a previously undiagnosed Wilms' tumor that resulted in a fatal intraoperative pulmonary tumor embolus.

Mesh hiatal reinforcement in Nissen fundoplication.

Persistent or recurrent gastro-oesophageal reflux (GOR) following Nissen fundoplication occurs in up to one in five cases, especially if the child is neurologically impaired. We advocate the use of mesh hiatal reinforcement for patients undergoing reoperation for GOR or if the diaphragmatic crura are thought to require reinforcement at the time of the original surgery.

Hirschsprung's disease: present UK practice.

A postal survey was performed to assess the management by consultant members of the British Association of Paediatric Surgeons (BAPS) of a well neonate with Hirschsprung's disease (HD). Replies were received from 63 (84%) of the 75 consultant surgeons who operate on neonates with gastrointestinal problems. Twenty-six surgeons (41%) would aim to perform a primary pull through without colostomy, and 37 (51%) would stage the pull through, with stoma formation in the neonatal period. Timing of definitive surgery varied as did the choice of procedure (Soave 24%, Swenson 8%, Duhamel 62%, other 6%), but there was no evidence that surgical experience influenced the choice of operation. Most surgeons see 4-5 new cases of Hirschsprung's disease per year. This study shows great variation in operative procedures for the same clinical condition, and indicates the need for further audit.

Retrospective study of prognostic value of DNA ploidy and proliferative activity in neuroblastoma.

AIM: To assess the prognostic value of age and stage at diagnosis, site of primary tumour, cell ploidy and N-myc copy number in children with neuroblastoma. METHODS: Flow cytometry was used to determine the cellular DNA content of paraffin wax embedded archival material from 69 cases of neuroblastoma and was successful in 52. RESULTS: The age, stage, and survival distribution of the sampled cases was not significantly different from that in a larger population based series. There were seven diploid ("non-aneuploid") and 45 aneuploid (including two tetraploid and four triploid) tumours. The 10 year survival was significantly better for cases of aneuploid rather than diploid tumours (p < 0.05). An important new finding was that 10 year survival was also significantly better for tumours with a low percentage of cells in S phase (p < 0.03). CONCLUSION: The percentage of cells in S phase, a measure of the proliferative activity of the tumour, correlated with prognosis in neuroblastoma. This should be measured with other biological features of the disease, such as N-myc copy number, when prognostic indicators are being assessed.

MYCN amplification by differential PCR.

A method is described to estimate MYCN (N-myc) oncogene amplification in neuroblastoma by the technique of differential polymerase chain reaction (PCR). The technique is quicker than conventional Southern blotting techniques and does not require radioactive materials. The ability to measure MYCN amplification from smaller amounts of tumor DNA also permits measurement from Tru-cut biopsy samples and opens the possibility of retrospective measurement of MYCN status from single paraffin sections of archival material.

Neuroblastoma: a 32-year population-based study--implications for screening.

This paper describes a retrospective population-based study of neuroblastoma in the West Midlands Health Authority Region--childhood population 1.12 million (OPCS, census 1981)--in which 239 cases were diagnosed between 1st January 1957 and 31st December 1988. The age standardised rate of tumour incidence has remained constant at 7.2 cases per million children per year. The median age at diagnosis was 2 years with 18% of children presenting before the age of 6 months. Fifty children (21%) presented before the age of one year, and for this group of children, the prognosis has improved significantly over the 32-year period (10 year survival increasing from 63% in 1957-67 to 87% in 1978-88), whereas for the 189 (79%) children who presented after one year of age, the prognosis has remained very poor during the study period (10 year survival 1957-67 = 9.5%, 1978-88 = 8.5%). This study supports the need for a prospective study of mass screening at several intervals rather than only at 6 months of age.

Tracheoaortopexy via midline sternotomy in tracheomalacia.

Symptomatic tracheomalacia associated with congenital tracheoesophageal fistula requires active treatment. When symptoms are severe, consideration should be given for surgical correction. Aortopexy via a lateral thoracotomy and tracheopexy via an anterior cervical approach have been described. We present two patients with extensive tracheomalacia who benefited from combined tracheoaortopexy via a midline sternotomy approach.

Oral suction in infants.

This is a description of a simple sucking sump catheter for continuous saliva removal.

The UK Children's Cancer Study Group: testicular malignant germ cell tumours 1979-1988.

The United Kingdom Children's Cancer Study Group (UKCCSG) malignant germ cell tumour (MGCT) studies were undertaken to establish standard protocols of investigation, staging, and treatment. The efficacy of new drug combinations and the value of serial measurements of serum alphafetoprotein (AFP) and human chorionic gonadotrophin (HCG) were evaluated. Following the initial surgery, staging of the tumour was performed using a variety of investigative approaches. In stage 1 testicular tumours, orchidectomy was performed. In more advanced tumours, and in stage 1 tumours that failed to show the expected decline in AFP or recurred, chemotherapy was used after appropriate surgery. Seventy-three boys, under 14 years of age, with testicular MGCTs have been entered into the UKCCSG studies since 1979. Serum AFP was measured preoperatively, or within 2 weeks of operation, in 70 boys. It was unequivocally elevated in 69. Monitoring by serial AFP measurement proved valuable in assessing response and in early detection of recurrence. HCG was measured in 46 boys, and was raised in three. Sixty-seven (91%) of the tumours were yolk sac (Teilum) tumours, four were immature teratoma, and two were mixed MGCTs. The only non-AFP producing tumour was an immature polydermal teratoma in a 1-year-old boy. Serum HCG was raised in three boys with yolk sac tumours, one with a mixed teratoma, and one 14-year-old boy who had a mixed MGCT. The results of treatment were assessed on April 1, 1989 (median time from diagnosis, 3 years 4 months). Seventy-one boys were alive, 48 of whom had been cured by orchidectomy alone. The remaining 25 patients received chemotherapy.(ABSTRACT TRUNCATED AT 250 WORDS)