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.

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.

Radioprotective reverse orchidopexy.

BACKGROUND/PURPOSE: With the improvement in long-term outcome from childhood malignancy, the expected quality of life is important. This study was to assess the value of reverse orchidopexy in patients requiring radiotherapy to the groin or pelvic area. METHODS: The details of 6 boys (age 2 to 13 years) who had malignancy necessitating groin or pelvic radiotherapy were reviewed. Each had reverse orchidopexy of the contralateral testis for the course of the radiotherapy treatment. Brief case summaries are presented. The testis was replaced in the scrotum 4 to 12 months after the reverse orchidopexy. RESULTS: The protected testis continued to grow and was considered normal at 1 to 7 years later. One individual had fathered a child. CONCLUSION: Reverse orchidopexy is a promising method of preserving testicular function in boys who require radiotherapy to the groin or pelvis area.

The primary management of testicular maldescent in gastroschisis.

Gastroschisis (GS) is the commonest abdominal-wall defect in the Western world. The conventional practice has been reduction of the viscera and closure of the abdominal wall as an emergency procedure. The testis is often a part of the prolapsed viscera along with the bowel loops, stomach, fallopian tube, etc. The primary management of prolapsed (PT) (3) and intra-abdominal (5) testes (IAT) in this condition was studied in 16 consecutive male babies with GS, each was managed by simple reposition of the testes and closure of the abdominal wall. The babies were followed up for spontaneous descent of the testes. At 18-month follow-up, all five IAT had descended into the scrotum spontaneously and were palpably normal. Of the three extra-abdominal PT, two had descended into the scrotum and were normal in size and on palpation. One was palpable in the superficial inguinal pouch. Simple reposition of the testes into the abdomen and closure of the abdominal defect is the correct approach for primary management of PT or IAT in a newborn with GS.

Central-venous access through the peripheral route in surgical neonates: an audit of 125 consecutive lines from a regional neonatal centre.

A long Percutaneous silastic IV line is frequently used in surgical neonates for infusion of hyperosmolar parenteral nutrition fluid into a central vein for several days without the need for operative insertion of a Broviac catheter or risks of direct puncture of a central vein. Our study was aimed at auditing the performance of 125 consecutive lines over a 2-year period. During this period, insertion was attempted in 125 babies; in 13 cases the line could not be inserted because of technical problems. The gestational ages varied between 25 and 41 weeks and weights between 630 g and 4.2 kg. Success did not appear to be related to the age or weight of the baby. The mean duration of complication-free performance was 22.4 days. There was a significant difference between the complication rate of lines inserted in the operating theatre versus those on the ward (P < 0.05). There was no significant increase in complications in lines used for over 4 weeks. The technique adopted by us for inserting these lines is likely to succeed in the majority of cases, including premature and small-for-dates babies. In our experience, lines inserted in the controlled theatre environment either before or after abdominal surgery performed better. The manufacturer's recommendation to electively change the line every 4 weeks needs further prospective evaluation.

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.

The treatment of Wilms' tumour: results of the United Kingdom Children's cancer study group (UKCCSG) second Wilms' tumour study.

The aims of the UKW2 study were: (1) to further refine treatment for stage I and II favourable histology (FH) patients; (2) to consolidate the UKW1 results for stage III FH patients; (3) to improve the outlook for patients with inoperable primary tumours and those patients with stage IV and unfavourable histology disease. Treatment consisted of primary nephrectomy, wherever possible, followed by chemotherapy and radiotherapy, as dictated by stage and histology. Treatment was refined successfully for stage I and II FH patients. The 4-year event-free survival for these two groups was 94% and 91%, respectively. Stage III FH patients had a 4-year event free survival of 84%. The outlook for patients with clear cell sarcoma of the kidney is as good as for patients with favourable histology, whilst that for patients with anaplastic or rhabdoid variants remains poor. The outlook for the majority of children with Wilms' tumour is now excellent.

Pediatric oncology and hematology in Birmingham, England.

The hematology and oncology service at Birmingham Children's Hospital was established in the late 1960s and now is one of the largest in the United Kingdom. It provides comprehensive care for the entire range of childhood malignancies, coagulation disorders, and hemoglobinopathies and other hematological disorders, and undertakes bone marrow transplant and megatherapy/peripheral blood stem cell procedures. Research includes clinical trials of treatments of childhood cancers; molecular biology studies on leukemia, Hodgkin's disease, neuroblastoma, and sarconas; childhood cancer epidemiology, and geographical and racial incidence; and treatment of hemophilia and molecular investigation of coagulation disorders. These activities involve collaboration with local, national, and international research groups.

UKCCSG's germ cell tumour (GCT) studies: improving outcome for children with malignant extracranial non-gonadal tumours--carboplatin, etoposide, and bleomycin are effective and less toxic than previous regimens. United Kingdom Children's Cancer Study Group.

BACKGROUND: We report the efficacy and late effects of carboplatin, etoposide, and bleomycin (JEB) for extracranial non-gonadal tumours (GCII, 1989-95) compared with the 5 previous regimens (GCI, 1979-1988) consisting of 3 vincristine, actinomycin, and cyclophosphamide (VAC) and 2 platinum-based protocols. METHODS AND RESULTS: Median follow-up for 52 patients in the GCI study and 46 in GCII was 105 and 48 months, respectively. For GCI, 5- and 10-year actuarial survival was 63% (95% Confidence interval 50 to 75%) or 72% (57 to 83%) if 6 cases given low-dose VAC were excluded. For GCII, 5-year survival was significantly greater at 95% (83 to 99%), p = 0.01. Event-free survival was 46% at 5 years for GCI (33 to 59%) or 52% excluding the low-dose VAC cases (38 to 66%), while for GCII it was 87% (74 to 94%), p = 0.002. Five-year event-free survival of 21 children given cisplatin, etoposide, and bleomycin (BEP) in GCI was 57% (37 to 76%) compared with 87% (74 to 94%) for 46 given JEB in GCII, P = 0.02. Late effects in 30 evaluable survivors of GCI and 43 GCII included renal impairment in 6 in GCI and 0 in GCII and deafness in 11 and 4, respectively. Among 17 survivors of sacrococcygeal tumours treated in GCI, 4 have neuropathic bladder/bowel and another shortening of a leg. In GCII, 4 of 26 have neuropathic bladder/bowel with lower limb weakness in one. CONCLUSIONS: We found JEB to be more effective and less toxic than our previous regimens. Some survivors of sacrococcygeal tumours have neurological late effects.

Temporary balloon occlusion of the inferior vena cava as an alternative to cardiopulmonary bypass in resection of Wilms' tumour with vena cava extension.

Up to 10% of patients undergoing nephrectomy for Wilms' tumour have tumour thrombus involving the vena cava. A new radiological technique is described utilising a temporary occlusion balloon inserted via an open venotomy of the left internal jugular vein into the retro-hepatic cava to create a bloodless field to facilitate surgery.

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.

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).

Xanthogranulomatous pyelonephritis in childhood: pre-operative diagnosis is possible.

OBJECTIVE: To diagnose xanthogranulomatous pyelonephritis (XGP) in childhood pre-operatively. PATIENTS AND METHODS: Eleven cases of XGP presenting to Birmingham Children's Hospital over a 10-year-period are reported. Nine (82%) were diagnosed preoperatively on the clinical and radiological findings. RESULTS: The characteristic clinical findings were urinary tract infection (9 cases), palpable renal mass (8 cases) and anaemia (8 cases). The most common radiological findings were enlarged kidney (9 cases), renal calculi (7 cases) and non-functioning kidney (6 cases). Renal ultrasound typically demonstrated a central echoic area (6 cases) and multiple hypoechoic areas in the parenchyma (7 cases). A computed tomography (CT) scan was performed in three cases. This showed characteristic multiple, low attenuation, unenhanced areas within the parenchyma with extension of the inflammatory process into peri-nephric fat (two cases). Ten cases (91%) were of the diffuse type. There was extension into the peri-nephric fat in eight cases (73%). Three cases (27%) were associated with congenital urological abnormalities. Nephrectomy was performed in 10 cases and a partial nephrectomy in one. CONCLUSION: XGP is uncommon in childhood but should always be considered in the differential diagnosis of renal masses, especially in the presence of anaemia. Nephrectomy usually results in a permanent cure.

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.