The United Kingdom Children's Cancer Study Group's second germ cell tumor study: carboplatin, etoposide, and bleomycin are effective treatment for children with malignant extracranial germ cell tumors, with acceptable toxicity.

PURPOSE: To evaluate carboplatin, etoposide, and bleomycin (JEB) in children with malignant extracranial germ cell tumors (GCTs). PATIENTS AND METHODS: Malignant GCTs in children aged 0 to 16 years were excised without major morbidity or otherwise biopsied. Stage I testicular and some ovarian GCTs were resected and monitored with alpha-fetoprotein (AFP) ("watch-and-wait" approach). Patients with recurrent stage I disease and all other patients received JEB (etoposide 120 mg/m(2) on days 1 through 3, carboplatin 600 mg/m(2) on day 2, and bleomycin 15 mg/m(2) on day 3). Courses were administered every 3 to 4 weeks until remission, and then two more courses were given. Chemotherapy toxicities were assessed using World Health Organization or Brock grading. RESULTS: Between January 1989 and December 1997, 192 patients were registered. Eight were excluded because either there was no histologic diagnosis (n = 3) or chemotherapy was given off-study (n = 5). The remaining 184 patients had germinoma (n = 20), malignant teratoma (n = 55), embryonal carcinoma (n = 1), yolk sac tumor (n = 107), or choriocarcinoma (n = 1). Forty-seven patients were treated with surgery alone, and 137 patients received JEB. The 5-year survival rate in March 1999 for all 184 patients was 93.2% (95% confidence interval [CI], 87.9% to 96.3%); for the 137 JEB-treated patients, it was 90.9% (95% CI, 83.9% to 95.0%), with an event-free survival rate of 87.8% (95% CI, 81.1% to 92.4%). The median follow-up after JEB treatment was 53 months (range, 0 to 109 months); the median number of courses was five (range, three to eight). Site, stage, and AFP level had prognostic significance. Nonfatal hematologic toxicity was common, but deafness and pulmonary and renal toxicities were rare. One child died of a thoracic tumor and bronchopulmonary dysplasia, and another died of acute myeloid leukemia. CONCLUSION: Conservative surgery, a watch-and-wait approach after complete excision, and JEB for those requiring chemotherapy produced high cure rates and few serious complications.

Paediatric surgical oncology. 7--germ cell tumours.

Malignant germ cell tumours occur in children in widely differing sites in the trunk and cranium. The role of surgery in the treatment regimens has been made more definitive by advances in chemotherapy. For both the surgeon and the medical oncologist, serum tumour markers are reliable guides to the progress of disease and success of treatment. While correct surgical management alone is sufficient for a high cure rate of testicular tumours, good results for tumours in other sites are being obtained by careful planning of biopsy, chemotherapy and surgical excision.

Cisapride reduces neonatal postoperative ileus: randomised placebo controlled trial.

AIM: To assess the efficacy of cisapride in reducing ileus persisting to the tenth postoperative day after neonatal abdominal surgery. METHODS: A prospective, randomised, double blind trial comparing rectal cisapride (1.4-2.3 mg/kg/day) with placebo over seven days was undertaken in 33 neonates. RESULTS: Seven of 12 (58%) patients receiving placebo and eight of 11 (73%) receiving cisapride achieved a first sustained feed during treatment. Of those receiving cisapride, the first sustained feed occurred at 2.3 days (SEM 0.6) compared with 4.7 days (SEM 0.8) with placebo. By the seventh day the mean daily net enteral balance was 69 (SEM 18) ml/kg in the cisapride subgroup and 17 (SEM 8) ml/kg for those receiving placebo. Stool was passed on 6.3 (SEM 0.4) treatment days in the cisapride subgroup compared with 4.1 (SEM 1.0) treatment days in the placebo subgroup. CONCLUSION: Cisapride is effective in neonates with a prolonged ileus after abdominal surgery.

Management of intestinal atresia complicating gastroschisis.

In a personal series of 22 patients referred with gastroschisis, five (23%) had intestinal atresia. The records of these patients seen over the past 12 years have been studied in order to elucidate the correct management of their complex problems. The indications for primary anastomosis or stoma formation probably depend upon the degree of damage and dilatation of the preatretic intestine at its initial presentation. In patients with a high jejunal or proximal ileal atresia, primary anastomosis should be attempted, but if the dilatation is severe, intestinal decompression may be required. In patients with distal ileal or colonic atresias, the establishment of a stoma and secondary closure is the treatment of choice.

Oral suction in infants.

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

Recent experience in the treatment of rhabdomyosarcoma.

Twenty-two patients with rhabdomyosarcoma have presented in the years 1970--1977. Of 8 patients who had inadequate postoperative chemotherapy and radiotherapy, or none at all, only 2 patients with small completely resectable tumors are alive and well at 6 and 4 yr after surgery. Fourteen patients had intensive combination chemotherapy in addition to appropriate surgery and radiotherapy. Five have died and the remaining nine are well and free of disease at follow up ranging from 9 mo to over 5 yr. A continued place in the treatment regime is advocated for early surgery aimed at total excision of the primary tumor where this is feasible.

Intestinal malrotation: the role of small intestinal dysmotility in the cause of persistent symptoms.

To investigate the possibility that small intestinal dysmotility is a cause of long-standing and persistent symptoms in patients with malrotation, we retrospectively reviewed 94 patients operated on for intestinal malrotation. In 50 patients operated on during the neonatal period, associated abnormalities were common (24%) and all presented with obstructive symptoms; only three (6%) had continuing mild symptoms the postneonatal period (aged 1 month to 1 year), five of whom (22%) had other abnormalities. Recurrent vomiting was the most common presentation (11/23); only 5 patients (21%) had acute obstruction. Response to operation was good in 18 (78%) and two had persistant symptoms; both died. Twenty-one patients presented beyond infancy (aged greater than 1 year), only 19 of whom had symptoms of less than 2 months' duration. Eight (47%) of those with long-standing symptoms had no relief from operation. Small bowel motility was recorded manometrically in 4 patients with long-standing symptoms. The findings suggest that small intestinal dysmotility may be common in patients with malrotation and persistant symptoms.

Pseudomembranous colitis: a lethal complication of Hirschsprung's disease unrelated to antibiotic usage.

Seventy-two new cases of Hirschsprung's disease were seen between 1980 and 1985. Twenty-six patients (36%) developed the clinical features of enterocolitis and, of nine patients who died, colitis was the immediate cause of death in six. Histologic material was available from 20 patients with colitis; this showed nonspecific inflammation typical of Hirschsprung's colitis in 13 cases but seven had pseudomembranous colitis (PMC). Five of the patients with PMC had not recently been exposed to antibiotics. PMC was responsible for three of the six deaths from colitis. All patients with Hirschsprung's disease who develop signs of colitis should have stool testing for Clostridium difficile toxin and should be treated with an antibiotic active against Clostridium difficile.

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)

Parenteral nutrition-related cholestasis in postsurgical neonates: multivariate analysis of risk factors.

The medical records of 74 neonates dependent on parenteral nutrition for at least 21 days after emergency abdominal surgery (performed between 1988 and 1992) were reviewed respectively. The role of enteral starvation, prematurity, composition and duration of parenteral nutrition, and sepsis in the evolution of parenteral nutrition-related cholestasis was evaluated by multiple regression analysis. The most important factors for cholestasis were low gestational age (median, 34 weeks), early exposure to parenteral nutrition, and sepsis. Episodes of sepsis were associated with a 30% increase in the bilirubin level. Enteral starvation and composition and the duration of parenteral nutrition solutions did not correlate significantly with the development of cholestasis. Prevention of sepsis should be the priority in minimising cholestasis in postsurgical neonates who are dependent on parenteral nutrition.

Duodenal atresia: a comparison of three modes of treatment.

To determine the most successful mode of treatment, 33 consecutive cases of duodenal atresia treated by duodenoduodenostomy and not associated with other gastro-intestinal anomalies were analysed retrospectively. These patients have been placed in a nonrandomised fashion into one of three groups: Group A: Duodenostomy (side to side) with gastrostomy and transanastomotic feeding tube (n = 12); Group B: Duodenoduodenostomy (diamond shape) with jejunostomy feeding tube (n = 12); Group C: Duodenoduodenostomy (diamond shape) only (n = 9). A nasogastric tube was used in all cases. There was no difference between the groups for gestational age, birthweight, and age at operation. The outcome measures used to compare these groups were the time taken to achieve full preanastomotic feeds and the duration of hospital stay. There was no difference in time taken to achieve full pre-anastomotic feeds between Group A and Group B. Patients in Group C took significantly less time to achieve full pre-anastomotic feeds than either of the other two groups (p < 0.05, Mann-Whitney U). The duration of hospital stay was also significantly shorter for patients in Group C (median = 12 days) than for patients in either Group A or B (median = 24, 20 days respectively) (p < 0.05, Mann-Whitney U).

Oesophageal substitution with free and pedicled jejunum: short- and long-term outcomes.

In children, the indications for oesophageal substitution are principally, long gap oesophageal atresia (OA), severe anastomotic disruption following primary repair of OA and severe caustic or peptic strictures. We present an outcome review of eight cases who underwent oesophageal substitution with jejunum at our institution between 1986 and 2001. The purpose of this study was to evaluate our experience with free/pedicled jejunal grafts and its long-term outcome as an oesophageal substitute. Operative and postoperative outcome with free and pedicled jejunal grafts in four cases of pure OA, two cases of OA and distal tracheo-oesophageal fistula (TOF), one patient with a high retrolaryngeal oesophageal web and one case of severe caustic oesophageal stricture. Six patients had an oesophagostomy and a gastrostomy fashioned previously. Eleven free jejunal grafts were performed in six patients (three intraoperative redo interpositions for immediate graft loss, three separate grafts in one patient and two free grafts in two patients). One patient's pedicled jejunal graft proximally required microvascular anastomosis while the other had a pedicled graft without microvascular anastomosis. Early postoperative complications included four upper anastomotic leaks (three free grafts, one pedicled with microvascular support), pneumothorax requiring prolonged ventilation and Horner's syndrome. Recurrent laryngeal nerve injury occurred in the patient who had a high retrolaryngeal oesophageal web. During follow up (5-18 years) late complications of upper anastomotic stricture in four patients and graft redundancy with subsequent kinking of the lower anastomosis were observed in one patient. Three patients established a complete oral diet; a further three patients relied on supplemental gastrostomy feeds and one patient is entirely gastrostomy fed. There were two late deaths, one from aspiration and the other from a severe asthmatic attack (5 and 7 months postoperatively, respectively). Our results indicate that there are significant complications related to the use of free jejunal grafts. Early recognition and treatment are of paramount importance in the ultimate achievement of a successful technical outcome.

Does gastroschisis reduction require general anesthesia? A comparative analysis.

BACKGROUND: Outcome after gastroschisis repair without general anesthesia is controversial, and published conclusions are variable with no comparative studies. AIM: The aim of this study was to present a comparative study evaluating outcome after gastroschisis repair with and without general anesthesia. METHODS: An ambispective nonrandomized study of a cohort of 51 neonates born with gastroschisis between July 1998 and December 2003 was performed. Twenty-four neonates (group 1) had conventional reduction under general anesthesia, and 27 (group 2) cotside minimal intervention reductions were without general anesthesia. RESULTS: Groups were comparable regarding gestational age, birth weight, and quality of eviscerated bowel. Statistical significance (P < .05) was seen between groups 1 and 2 with regard to age at reduction of gastroschisis (5.6 +/- 2.5 vs 3 +/- 1 hours) and time taken for completion of gastroschisis reduction (58.1 +/- 15 vs 49 +/- 14 minutes). No statistical significance (P > .05) was seen with respect to start of feeds (10.4 +/- 3.6 vs 10.9 +/- 4.1 days), duration of total parenteral nutrition (21.5 +/- 7.3 vs 22.4 +/- 6.8 days), and total hospital (stay 29 +/- 10 vs 30 +/- 13 days). Admission to the intensive care unit was required in 92% in group 1 for 1 to 6 days vs 7% in group 2 for 3 to 6 days. There was 1 death in group 1 (4%). Total hospital cost in group 1 was 12,283 pounds sterling +/- 2438 pounds sterling vs 6208 pounds sterling +/- 2120 pounds sterling in group 2 (P = .013). CONCLUSIONS: Neonates with gastroschisis, whose bowel was reduced without general anesthesia, have similar outcomes to those whose bowel was reduced under general anesthesia. Both approaches appear to be safe and effective, but reduction without general anesthesia was cost-effective.

Persistent rectal prolapse in children: sclerotherapy and surgical management.

Persistent rectal prolapse is an uncommon but distressing condition in children. Significant controversy exists regarding its surgical management. The aim of this study was to identify a successful management strategy for persistent rectal prolapse in the paediatric population. Records of all children with rectal prolapse treated surgically at Birmingham Children's Hospital between 1995 and 2003 were retrospectively reviewed. Demographic data, clinical presentation, investigations, treatment modality, complications, and outcome were recorded. Inclusion criteria for the study were failure of conservative management leading to operative treatment. An exclusion criterion was cystic fibrosis. A total of 24 patients with persistent rectal prolapse were identified. Two children with cystic fibrosis were excluded from the analysis. Children below the age of 5 years, group I (n=17), were successfully managed by submucous hypertonic saline injections. Eighty-three percent (14/17) were cured by injection sclerotherapy in this group, 12/14 (71%) requiring one injection and 2/14 requiring a second injection. In the three (17.6%) children in group I in whom sclerotherapy failed, cow's milk protein (CMP) allergy was identified as the causative factor. Children older than 5, group II (n=5), either had behavioural problems (n=3) or were autistic (n=2). This group of children with adult-type, full-thickness rectal prolapse were found to be refractory to initial attempts of injection sclerotherapy. All five children were successfully managed with surgical correction. We conclude that rectal submucous hypertonic saline injections are highly effective for managing early-onset idiopathic childhood rectal prolapse. CMP allergy should be considered in young children with recurrent rectal prolapse. We recommend early definitive corrective surgery in older children with persistent rectal prolapse, as they do not respond to conservative measures or injection sclerotherapy.

Stereotaxic neurosurgery in the management of cerebral palsy.

Ten children with cerebral palsy are presented on whom stereotaxic operations on the central nervous system were performed with the aim of ameliorating athetosis and spasticity. Tere were seven alert and co-operative children with spastic hemiplegia or diplegia, of whom six received benefit from thalamotomy or dentatotomy. The seventh, a child with diplegia, had improvement of his left lower limb, but the right became worse. One child with spastic diplegia, in whom a thoracic meningocoele had been closed at birth, was not improved by bilateral dentatotomy. Two severely quadriplegic children each had bilateral dentatotomy; one was a child with dystonic and spastic quadriplegia. In both cases the resulting reduction in tone and extensor spasm rendered the nursing of these patients much easier. The place of stereotaxic surgery in the central nervous system in the management of children with cerebral palsy is discussed. We suggest that in selected cases the stereotaxic operation should be performed early in order to gain the greatest benefit. Stereotaxic surgery should be regarded as an integral part of the management which involves close co-operation of paediatrician, physiotherapist, neurosurgeon and orthopaedic surgeon.

The place of laparoscopy in the management of the impalpable testis.

In a four-year period 46 boys have been laparoscoped in the search for 55 impalpable testes. Thirty-three per cent of the testes were located intrabdominally. Another 53 per cent were absent. This could be diagnosed by laparoscopy alone in 35 per cent. Laparotomy was needed in only 3 boys to locate the testes.

Is specialist centre delivery of gastroschisis beneficial?

This study aims to establish the usefulness of delivering neonates with gastroschisis in a regional obstetric and neonatal centre without the facility of on site surgery. A retrospective analysis was performed on the notes of 43 consecutive neonates with gastroschisis referred to Birmingham Children's Hospital over a 10 year period. Two groups were compared: those delivered at the regional obstetric centre (n = 9) and those delivered peripherally (n = 34). Both groups underwent postnatal transfer. There were no significant differences with regard to gestational age, birth weight, caesarean section rate, time to operation, and mortality. Primary closure rates were 89% for the regional centre group and 94% for the peripheral hospital group. Mean time to full enteral feeding was 24 days for the regional centre group and 23 days for those delivered peripherally. These data show that good results can be achieved with postnatal transfer. If on site surgery is not available, neonatal services are adequate peripherally, and the transfer distance is not too great, then delivery in a regional obstetric centre with subsequent postnatal transfer offers no advantage.

The long-term sequelae of ileocolic anastomosis in childhood: a retrospective survey.

The long-term effects of ileocolic anastomosis in children are not known. We therefore carried out a survey of all such patients treated at our hospital between 1971 and 1985. Thirty-seven patients were identified and invited to attend for follow-up. Twenty-seven (73 per cent) were examined and included in the survey. Mean age at survey was 7.8 years (range 1.6-17.6 years) and mean duration since operation was 6.3 years. Necrotizing enterocolitis and intussusception were the commonest indications for operation. As judged by height, weight and skin fold thickness, all patients were well nourished. Seven (26 per cent) had loose stools, five had chronic folic acid deficiency and one was vitamin B12 deficient. We conclude that resection of the ileocaecal region is well tolerated in childhood and does not affect growth. Loose stools are directly related to the length of ileal resection.

Impalpable testis: testicular vessel division in treatment.

Between 1984 and 1989, 110 boys with 130 impalpable testes were investigated and treated. One hundred and six boys underwent laparoscopy. Twenty had bilateral impalpable testes and 13 had a palpable, but maldescended, testis on the other side. Forty-nine testes were absent and one atrophic testis was found in the scrotum. Twelve orchidectomies were performed. One child had a microvascular transfer procedure. The remaining 67 testes were treated by orchidopexy: 28 single-stage and 39 two-stage procedures. One of the single-stage and 29 of the two-stage orchidopexies included division of the testicular vessels. Twenty-three testes examined 12 or more months after staged testicular vessel division revealed a good result in 15.