Surgical management and outcomes of 12 cases of Wilms tumour with intracardiac extension from a single centre.

PURPOSE: To review demographics, effect of preoperative chemotherapy on tumour thrombus, imaging, operative strategy, and outcomes of 12 patients presenting with intracardiac extension of Wilms tumour thrombus. METHODS: A retrospective audit was undertaken on patients with intracardiac extension of Wilms tumour. Patients were identified from the oncology database and information obtained on clinical presentation, stage, preoperative treatment, surgical procedures and complications, histology, and survival status. Ethics approval was obtained from the University of Cape Town Human Research Ethics Committee. RESULTS: From 1984 to 2016, 337 children with Wilms tumour were treated. Twelve (3.6%) had intracardiac extension of tumour thrombus, nine into the right atrium, and three into the right ventricle. Ultrasound, computerized tomography, magnetic resonance imaging, and echocardiograms were used to assess thrombus level. Patients were staged as stage III(8) and IV(4). All patients received preoperative chemotherapy. Thrombus retracted from the heart in two cases. One patient died preoperatively. Eleven underwent laparotomy, median sternotomy, and cardiopulmonary bypass (CPB). Four underwent cavectomy. Five required cavoatrial patches. Thrombus extending into the hepatic veins was extracted in five patients. There was one intraoperative complication and one perioperative death. Thrombus histology showed viable tumour in 9 of 11 patients. Three patients died of progressive disease. Seven patients are currently disease free. CONCLUSION: A combination of imaging is required to determine thrombus extent, and this facilitates surgical planning. Preoperative chemotherapy may cause thrombus regression, thus avoiding CPB. CPB offers appropriate conditions for safe tumour thrombus excision. Full management in centres with appropriately experienced staff and facilities for CPB is recommended.

Local anesthetic wound infusion versus standard analgesia in paediatric post-operative pain control.

INTRODUCTION: Local anesthetic wound infusion has shown promising results in adults. Its use in children is limited to some centers and there are only a few prospective trials in this group of patients. METHODS: Sub-fascial continuous local anaesthetic wound infusion (CLAWI) (0.2% Bupivacaine) plus intravenous paracetamol and rescue intravenous morphine was compared to: (a) Epidural bupivacaine (EPI) plus paracetamol and rescue intravenous morphine for patients undergoing laparotomy. (b) Intravenous morphine and paracetamol (standard post-operative analgesia-SAPA) in children undergoing Lanz incision laparotomy for complicated appendicitis. 'InfiltralLong', PANJUNK(®) catheters were placed sub-fascially after peritoneal closure for post-operative bupivacaine infusion. Pain scores were recorded regularly by the same blinded pain specialist. The primary outcomes were pain control and total morphine. The secondary outcomes were time to full feeds, mobilization requirement for urinary catheter and complications. RESULTS: Sixty patients (18 laparotomy-CLAWI, 17 laparotomy-EPI and 12 appendectomy-CLAWI, and 13 appendectomy-SAPA) were analyzed. The average pain score was 2.5 (1-4) in the CLAWI groups, 3.0 (1-5) in the EPI group and 3.5 (2-5) in the SAPA group. Morphine requirements were markedly less for CLAWI. SAPA and EPI groups required urinary catheters for longer and took longer to mobilize (average 4 days compared to 2 days for CLAWI). There were no wound or bupivacaine complications in the CLAWI group. CONCLUSION: Continuous subfascial bupivacaine infusion is reliable, safe and effective in paediatric post-operative pain control with considerably reduced opiate requirements.

Intussusception as a presenting feature of Burkitt lymphoma: implications for management and outcome.

PURPOSE: Intussusception is a recognised but unusual presenting feature of Burkitt lymphoma. We sought to identify the clinical features associated with intussusception in this setting, and assess the outcome following protocol directed chemotherapy. METHODS: A retrospective case note review was performed on patients treated for Burkitt lymphoma at our institution between 1976 and 2010. Cases presenting with intussusception were identified from hospital records and oncology database. RESULTS: Fourteen of the 210 children seen with a diagnosis of Burkitt lymphoma during the study period (6.7%) developed intussusception. Median age was 6.1 years (range 2.5-10.9). Twelve patients presented with recurrent abdominal pain, and two patients with a jaw mass associated with endemic Burkitt lymphoma. Nine patients underwent a right hemicolectomy with ileo-colic anastomosis, and five had segmental small-bowel resections. Three patients had bone marrow involvement at diagnosis, two of whom died. All patients received chemotherapy. Median follow-up was 6.07 years (range 0.1-28.8). CONCLUSIONS: Small bowel lymphoma should be considered in children presenting with intussusception above the normal infantile peak age range. The presentation is often insidious, and complete obstruction may not be apparent. However, when surgically resected, the majority can achieve a good outcome with additional chemotherapy.

Pregnancy following liver transplantation during childhood and adolescence.

More than 80% of pediatric transplant recipients will survive to reach adulthood, and many will consider having children. We report on outcomes and management of five pregnancies in four women undergoing orthotopic liver transplantation during childhood or adolescence and followed up at our Transplant Center. A retrospective clinical folder audit was performed. Mean age at transplantation was 13.3 ± 3.4 yr (range, 10-18 yr). Mean interval between transplantation and pregnancy was 15.4 ± 4.9 yr (range, 10-22 yr). Mean maternal age at conception was 28 ± 3.5 yr (range, 23-32 yr). Mean gestational age was 36.6 ± 1.7 wk. Mean birth weight was 2672 ± 249 g. Immunosuppression was cyclosporin based in three women and tacrolimus based in one woman. Pregnancy complications necessitating the induction of labor included fetal distress and rising maternal liver enzymes in two women, cholestasis of pregnancy and impaired renal graft function in one woman, fetal distress and preeclampsia in one woman. Modes of delivery were normal vaginal delivery in three women and cesarean section in one woman. No maternal or fetal deaths and no congenital malformations occurred. No episodes of rejection occurred during pregnancy. Two women experienced acute cellular rejection requiring an increase in baseline immunosuppression in the first year, following delivery. No graft losses occurred during a mean follow-up of 44 ± 17.9 months post-delivery. With careful management, pregnancy post-liver transplantation can have a successful outcome.

Hepatic venous outflow obstruction in paediatric liver transplantation.

INTRODUCTION: Vascular complications after orthotopic split liver transplantation in children result in significant post-operative complications. MATERIALS AND METHODS: A review of children undergoing liver transplantation from 2004 to 2006 was undertaken. The data was obtained based on a proforma-based analysis. RESULTS: Seven of 106 transplants were identified to have hepatic venous outflow obstruction (HVOO) of whom five were males and two were females. Median age at transplant was 8 years (range 3 years 4 months-15 years). The median donor-to-recipient weight ratio was 2.78 (range 0.97-6.15). ANASTOMOTIC TECHNIQUE: Hepatic vein-IVC in four, Hepatic vein-hepatic vein (HV-IVC) confluence in two and cavo hepatic in one. Ascites was the commonest post op manifestation of HVOO. Although Doppler USG was useful in identifying the venous outflow obstruction, venography confirmed the exact site of obstruction aiding in therapeutic dilatation. Three of seven cases had early onset (<1 month) while 4/7 had late onset (>1 month). 5/7 underwent venography and dilatation, of whom three are well and one is awaiting a repeat venography and dilatation. 2/7 died without intervention and 1/7 died in the waiting list for retransplantation. CONCLUSION: The diagnosis of HVOO requires a high index of suspicion, prompting early venography to manage HVOO successfully. Technical steps to avoid HVOO are to keep the hepatic vein-caval anastomosis short and wide with triangulation and to avoid graft rotation at the hepato caval junction.

Isolated liver transplant in infants with short bowel syndrome: insights into outcomes and prognostic factors.

OBJECTIVE: Selected infants with short bowel syndrome (SBS) and progressive intestinal failure associated liver disease (IFALD) may benefit from isolated liver transplantation (iLTx). The aim of the study is to identify risk factors for unfavourable outcome in iLTx. PATIENTS AND METHODS: A retrospective review of medical records from 1998 to 2005 was undertaken. Risk factors were assessed by comparing long-term survivors with those who died after iLTx. RESULTS: Fifteen iLTx were performed in 14 infants with IFALD. All were parenteral nutrition (PN) dependent, but had tolerated enterally 54% (38-100) of energy intake before iLTx. Median residual bowel was 60 cm (30-200). Eight out of 14 had intact ileocaecal valve (ICV). Median bilirubin was 298 micromol/L (87-715) and all had portal hypertension. Eight out of 9 survivors were weaned from PN after median 15 months. In 4 out of 9 children, nontransplant surgery after iLTx facilitated intestinal adaptation. Growth velocity had improved at 3 years after iLTx (P=0.001). Five children who died had poor enteral tolerance following iLTx (P<0.002), which correlated with pretransplant dysmotility seen in 4 out of 5 children shown by contrast studies (P=0.02)and increased frequency of line infections before (>6/year P<0.04) and after (P<0.001) iLTx. CONCLUSIONS: Isolated liver transplantation is a lifesaving option for selected children with SBS and IFALD. Revised criteria are proposed: progressive IFALD; 50 cm functional bowel in absence of ICV or 30 cm with ICV; 50% daily energy intake tolerated enterally for 4 weeks with satisfactory growth; and children with dysmotile bowel should be assessed for combined liver/bowel transplant unless the dysmotility is resolved and associated with minimal line infections.

Ischaemic preconditioning of the liver before transplantation.

PURPOSE: Assessment of the effect of a short ischaemic time prior to liver transplantation on the liver graft. METHODS: White X Landrace pigs (N=10) were subjected to liver transplantation. Before being removed from the donor animal, the livers were randomised into two groups: group 1--pre-procurement ischaemia (15 minutes' temporary arrest of portal venous and hepatic arterial inflow to the liver, followed by reperfusion of these vessels for a period of 15 minutes); group 2--no prior inflow occlusion (control group). In group 1 a spleno-jugular bypass was established to prevent venous congestion, portal venous hypertension, intestinal oedema and bacterial translocation. The livers were perfused with Eurocollins solution (4 degrees C), after which they were stored on ice for a period of 3 hours' cold ischaemic time. Hepatocellular injury was assessed according to liver cell function tests (aspartate aminotransferase, AST), biochemical indicators of reperfusion injury (malondialdehyde) and histopathology. RESULTS: There was a significant rise of AST in both groups 1 hour after transplantation (from 51 +/- 27 IU/l to 357 +/- 152 IU/l in group 1 and from 29 +/- 10 IU/l to 359 +/- 198 IU/l in group 2). AST levels were marginally lower in group 1 at 2 and 4 hours after transplantation. There was also a rise in malondialdehyde levels in both groups at 5, 20, 40 and 60 minutes after transplantation. Levels of malondialdehyde were lower in the primed group at 5, 20 and 40 minutes, while the levels at 60 minutes after transplantation were comparable. Histological changes, as measured by vacuolisation, neutrophil infiltration and hepatic cell necrosis, were less in livers transplanted after ischaemic preconditioning, although the difference was not significant. CONCLUSIONS: Ischaemic preconditioning of the donor liver seems to decrease hepatocellular damage, reperfusion injury and histological changes in the liver after transplantation. Further studies with larger numbers are indicated.

Is serum gentamicin level a good predictor of graft injury in intestinal transplantation?

INTRODUCTION: Following intestinal transplant (SBT), the early diagnosis and treatment of rejection is a major management aim. The diagnosis of rejection is based on histology of stomal biopsies. Oral gentamycin (2.5 mg/kg) was used for selective decontamination of the digestive system. Our hypothesis was that gentamycin might be absorbed in the presence of graft dysfunction. AIM: Our goal was to assess the correlation between serum gentamycin level and the health of the intestinal graft. SUBJECTS AND METHODS: Among 33 SBT performed from 1993 to 2005, serum gentamycin levels were performed once weekly or more often when there was a suspicion of rejection. All data were analyzed retrospectively. RESULTS: Adequate trough levels were achieved for only 23 patients, six of whom had histologically proven rejection and only one did not have a raised gentamycin content. Five patients with raised levels but no rejection included two with severe intestinal ischemia and three with bowel obstruction/ileus. Four of the five patients required laparotomies. CONCLUSION: We concluded that in our study raised serum gentamycin levels were a good predictor of rejection or significant injury to the graft.

Liver transplantation for children: Red Cross Children's Hospital experience.

BACKGROUND: The liver transplant program for infants and children at the Red Cross Children's Memorial Hospital is the only established pediatric service in sub-Saharan Africa. Since 1985, 250 infants and children have been assessed and 155 accepted for transplantation. METHODS: Since 1987, 76 children (range 6 months to 14 years) have had 79 liver transplants, with biliary atresia being the most frequent diagnosis. The indications for transplantation include biliary atresia (n = 44), metabolic (n = 7), fulminant hepatic failure (n = 10), redo transplants (n = 3), and other (n = 15). Three combined liver/kidney transplants have been performed. Forty-nine were reduced-size transplants with donor: recipient weight ratios ranging from 2:1 to 11:1, and 29 children weighed < 10 kg. RESULTS: Fifty-six (74%) patients survived 3 months to 12 years posttransplant. Cumulative 1- and 5-year patient survival data are 79% and 70%, respectively. However, with the introduction of prophylactic intravenous gancyclovir and the exclusion of hepatitis B virus (HBV) IgG core Ab-positive donors, the projected 5-year pediatric survival has been >80%. Early (<1 month) post-liver-transplant mortality was low, but included: primary malfunction (n = 1); inferior vena cava thrombosis (n = 1); bleeding esophageal ulcer (n = 1); and sepsis (n = 1). Late morbidity and mortality was mainly due to infections: de novo hepatitis B (5 patients, 2 deaths); Epstein-Barr virus (EBV)-related posttransplantation lymphoproliferative disease (12 patients, 7 deaths); and cytomegalovirus disease (10 patients, 5 deaths). Tuberculosis (TB) treatment in three patients was complicated by chronic rejection (n = 1) and TB drug-induced subfulminant liver failure (n = 1). CONCLUSIONS: Despite limited resources, a successful pediatric program has been established with good patient and graft survival figures and excellent quality of life. Shortage of donors due to HBV and human immunodeficiency virus (HIV) leads to significant waiting list mortality and infrequent transplantation.

Nephrotoxic effects of immunosuppressant therapy in pediatric liver transplant recipients.

Drugs used for immunosuppression have been implicated in causing numerous long-term side effects including nephrotoxicity, glucose intolerance, and hyperlipidemia. In this study, we reviewed our pediatric liver transplant recipients in terms of glomerular filtration rate (GFR) as well as fasting glucose and lipid profiles. To date, 79 pediatric liver transplantations have been performed at our center: 24 transplantations of at least 5 months to a maximum of 7.3 years posttransplant are reviewed herein. The mean time posttransplantation was 2.1 years. Nine boys and 15 girls showed a distribution of 19 mixed race, 3 black, and 2 white patients. The mean age at the time of transplantation was 6.6 years (0.8-13.3 years) with 8 cases under the age of 3 years. All recipients started with Cyclosporine Neoral (CSA) as first line, but, at the time of testing, immunosuppression included 5 children on CSA and 19 on Tacrolimus. Radionuclide 51 Cr-EDTA Glomerular Filtration Rates (GFR) showed a range from 21 to 220 mL/min/1.73 m2 (mean 96.1, median 89.8). Seven cases had a GFR less than 75 mL/min/1.73 m2. Twenty-one children were on antihypertensives agents: 15 children on 1 agent and 6 children on 2 agents. On full fasting lipid profiles, the total cholesterol ranged from 2 to 7.9 mmol/L (mean 4.4). Only 1 child is currently on statin therapy. Fasting glucose ranged from 3.2 to 5.9 mmol/L (mean 4.1) No difference was observed in glucose values between CsA and Tacrolimus. Thus, immunosuppressive therapies, such as the calcineurin inhibitors, are known to cause nephrotoxicity, which is of concern in pediatric liver transplant recipients. Almost all our patients currently require antihypertensive therapy. At present, the renal function is adequate in the majority of the group, but this study needs to be extended to other pediatric liver transplant recipients with particular emphasis on those who are more than 5 years posttransplantation.

Malrotation and volvulus in infancy and childhood.

The subject of malrotation and midgut volvulus in infancy and childhood is reviewed from the perspective of experience with 138 patients evaluated in a published series and a further 82 cases seen since. Embryology, historical aspects, clinical presentation, investigation, surgery, and outcome are discussed. The diagnosis of malrotation and volvulus should always be kept in mind when assessing any infant or child with symptoms of vomiting and pain, particularly when the vomiting is bile-stained. The diagnosis cannot be excluded if diarrhea is present, when clinical findings are bland, or even with normal abdominal radiographs. A contrast meal and follow-through should be done and, if correctly interpreted, should be diagnostic. Ultrasound examination may be helpful but is not secure enough to exclude the diagnosis. Laparotomy or laparoscopy is the only way to be sure. Malrotation with its propensity for volvulus is truly a time bomb lying within.

Anal achalasia after pull-through operations for Hirschsprung's disease -- preliminary experience with topical nitric oxide.

INTRODUCTION: Following definitive pull-through for Hirschsprung's disease (HD), a minority of patients develop constipation, incontinence and enterocolitis. The cause for these symptoms in some of the patients is believed to be "internal sphincter achalasia" related to abnormal innervation of the sphincter, with an absent anorectal relaxation reflex. Transanal myectomy, posterior sagittal rectal myectomy, anal dilatation and even intrasphincter injection of botulinum toxin have been used to solve this problem. Nitric oxide (NO) has been identified as the chemical messenger of the intrinsic non-adrenergic, non-cholinergic pathway mediating relaxation of the normal internal anal sphincter when applied topically. BACKGROUND/PURPOSE: To evaluate the effect of topical isosorbide dinitrate (DTN) applied to the anus and its role in the management of patients with HD after pull-through who have ongoing difficulties in stool evacuation. MATERIAL AND METHODS: Four children, aged 2, 5, 7 and 13 years, who all underwent the Soave operation for Hirschsprung's disease, were assessed. Three patients had recurrent episodes of enterocolitis, and all had symptoms of difficulty in rectal/colonic evacuation. Conservative treatment of repeated anal dilatation under anaesthesia had failed to improve symptoms. A rectal myectomy and conversion of Soave to Duhamel procedure had been done in 2 without significant improvement in symptoms. In all patients, ano-rectal manometry was performed before and after application of DTN paste (1 mg/kg/day in two separate doses) which was continued for a minimum period of 3 weeks. Results. Marked symptom improvement was noted in all 4 children. On manometric assessment the median maximum pressure (pre-DTN application) was 165 mm Hg (range 96 - 250), the median sphincter length 2.7 cm (range 2.3 - 3.1) and the high-pressure zone (HPZ) median length 1.6 cm (range 1.2 - 2.1). After application of DTN paste, the maximum pressure dropped by a median of 88 mm Hg (range 46 - 90), total sphincter length shortened to a median of 2.1 cm and the HPZ by a median total length of 1.4 cm (range 0.01 - 0.9). In addition, vector volume was reduced by a median of 59 % (range 40.5 - 77). CONCLUSION: From these results, it is evident that DTN paste is not only an adjunct in the investigation, but can also be used as a temporary form of treatment of obstructive symptoms in Hirschsprung's disease patients.

Congenital solitary intestinal fibromatosis.

Neonatal intestinal obstruction due to a tumour is rare. We report a six-day-old male neonate who presented with abdominal distension and vomiting. Laparotomy revealed colonic obstruction caused by a stenosing fibrotic lesion in the proximal transverse colon. Histopathological examination of the resected specimen confirmed fibromatosis. We believe this represents the third reported case of solitary colonic fibromatosis. The literature on neonatal intestinal fibromatosis is reviewed.

Decreasing blood product requirements after orthotopic liver transplantation.

OBJECTIVES: To document the changing blood product transfusion requirements over time during the first 50 orthotopic liver transplantation (OLT) operations performed at Groote Schuur Hospital. DESIGN: The first 50 OLT operations were divided into three groups chronologically and blood transfusion requirements were compared across the three groups. SETTING: Groote Schuur Hospital, Cape Town. SUBJECTS: Forty-seven patients undergoing 50 OLT operations between October 1988 and September 1999. The majority of patients had end-stage chronic liver disease. Patients were divided chronologically into three groups of 16, 17 and 17 patients. OUTCOME MEASURES: The number of units of blood products transfused in each group was quantified. RESULTS: During the study period there was a significant decrease in the number of units of packed red cells, fresh frozen plasma (FFP) and platelets (but not cryoprecipitate) transfused intraoperatively. The median number of units of packed cells decreased from 13.5 in our first 16 patients (group 1) to 5.0 in our last 17 (group 3). The median number of units of platelets transfused was 9 in group 1 and 0 in groups 2 and 3. No platelets were transfused in our last 22 patients. The average cost of blood products transfused intraoperatively per patient in the three groups decreased from R13 034 in group 1 to R11 389 in group 2 to R7 396 in group 3. CONCLUSION: The number of units of blood products transfused during OLT operations has decreased since the programme started 13 years ago. The reasons include increased experience, the use of aprotinin to block fibrinolysis and the selective use of the thromboelastogram to monitor coagulation intraoperatively. OLT need no longer be regarded as wasteful of blood resources. Use of platelets to treat thrombocytopenia need not be empirical.

Is there a need for postoperative surveillance after day case groin surgery in children?

There is increasing awareness of the value of day case paediatric surgery in fulfilling the needs of the community. Is this cost effective, are there hidden complications and is there a need for routine follow-up? Seven hundred and thirty-one children attending the Red Cross War Memorial Children's Hospital day surgery unit over a 32-month period, and undergoing 912 procedures confined to the inguinoscrotal region, were enrolled in the study. Surgical procedures performed included inguinal herniotomy, orchidopexy and circumcision. Of the 731 children, 159 consecutive children had their inguinal and perineal skin flora analysed preoperatively and in 59 of these children three skin swabs were taken from the groin area: before routine skin preparation for surgery, after the skin preparation and on completion of the operation. Staphylococcus epidermidis predominated as the normal skin flora (70%), followed by Escherichia coli (19%) and Proteus mirabilis (10%), while S. aureus was only found in 2.5% of cases. The efficacy of cleaning with 4% chlorhexidine and 2% povidone-iodine in 70% alcohol is shown by virtual complete eradication of the organisms isolated from the preoperative skin cultures. In only 2 cases (3.4%) were organisms (S. aureus and S. epidermidis respectively) isolated following skin preparation. Cultures taken at the end of operative procedures grew predominantly S. epidermidis (5 cases, 8.5%). Children were discharged from the day unit once they had taken fluids and had passed urine. The wounds were also checked for any evidence of bleeding. Follow-up was done 2 weeks later by means of a visit to the day unit where the same surgeon assessed wound healing according to the Southampton Wound Assessment Scale. Ninety-four per cent of the wounds had healed by primary intention; 4.5% had minor complications and 1.5% had septic complications. Our trial confirms that wound sepsis is an infrequent but significant complication of day case groin surgery. Routine follow-up by surgical day units of minor surgical cases is not warranted and will incur unnecessary cost. Only 1.5% of postoperative cases will require further medical attention and septic lesions in the lymphatic drainage area should be regarded as risk factors.

Liver tumours in children: current surgical management and role of transplantation.

This article reviews the current surgical management of liver tumours in children in the light of improved chemotherapy, surgical techniques and outcomes from transplantation. It is a principle of management that complete removal of a tumour must be achieved for cure. Neoadjuvant chemotherapy may downstage advanced local disease to enable safe curative tumour resection. When this is not achievable, transplant is indicated. Conventional indications for transplant are unresectable stages 3 and 4 tumours confined to the liver. With the realisation that lifelong immunosuppressive therapy has considerable adverse consequences, there has been a recent trend towards extreme and 'acrobatic' liver resection to avoid transplantation, but still obtain a cure. The current literature is reviewed in the light of these trends and our own experience.

The domestication of fire: the relationship between biomass fuel, fossil fuel and burns.

Primitive man's discovery and use of fire had a tremendous impact on modern development. It changed lifestyles, and brought with it new fuel sources and cooking methods. It also introduced devastation, injury, pain, disfigurement, and loss of life, and the need to continuously develop management, training and prevention programs.

Nephron-sparing surgery for bilateral Wilms' tumours: a single-centre experience with 23 cases.

INTRODUCTION: The challenge of management with bilateral Wilms' tumours is the eradication of the neoplasm, while at the same time preserving renal function. Surgical management with a variety of nephron-sparing techniques, combined with chemotherapy and occasionally supplemented by transplantation has evolved over the last 30 years to achieve remarkable success. We document the experience of a single centre in a developing country. MATERIAL AND METHODS: Twenty-three bilateral Wilms' tumours were seen in our service between 1981 and 2007. Treatment was, in most cases, according to National Wilms' Tumour Study Group protocols, with initial bilateral biopsy, neoadjuvant chemotherapy, and tumourectomy. Technique of nephrectomy included full mobilization of the tumour-involved kidney, topical cooling with slush ice, vascular exclusion, tumour resection and reconstruction of the remnant kidney. RESULTS: Twelve patients are alive and free of disease one to 15 years after treatment, all with well-preserved renal function (lowest glomerular filtration rate was 65 ml/min per (1.73 m 2 ). None of the survivors have hypertension. Eleven have died (two of unrelated disease) including six of the seven with spread outside the kidney. All three with unfavourable histology are alive. Four of the five metachronous presentations are alive, as are eight of 12 patients with synchronous bilateral tumours who presented since 2000. CONCLUSIONS: Appropriate chemotherapy and nephron-sparing surgery can achieve good results with preservation of adequate renal function in nearly all cases. Unfavourable histology did not have a reduced survival in our series. Metastatic spread outside the kidney had a poor prognosis.