Nephron-sparing surgery in children with primary renal tumor: indications and results

Nephron-sparing surgery (NSS) is the treatment of choice for children with bilateral Wilms' tumor (WT), or with WT on a single kidney, or with WT and a disease of the contralateral kidney, or with benign kidney tumor. NSS is a reasonable alternative to nephrectomy in children at risk of metachronous WT, including children with genetic syndromes, children younger than 1 year of age, and children with hyperplastic nephroblastomatosis. The use of NSS in selected children with "low-risk" or stage I "intermediate-risk" WT and a normal contralateral kidney is still controversial. Available data suggest that, in children with WT, NSS does not impair the outcome and has a renal function advantage over nephrectomy.     

B-cell non-Hodgkin's lymphoma presenting as a primary renal tumour in a child

A 6-year-old boy presented with a unilateral renal tumour thought to be a Wilms' tumour. He had started a treatment but review of the histology and immunohistochemical studies proved the tumour to be a primary renal B-cell lymphoma. This case illustrates the importance of an open-minded approach in the histological examination of small round cell tumours in the kidney and immunohistochemical studies in such cases are strongly advocated. © 1995 Wiley-Liss, Inc.     

Pyeloplasty in children: perioperative results and long-term outcomes of robotic-assisted laparoscopic surgery compared to open surgery

Background

A few studies have compared robotic-assisted laparoscopic pyeloplasty (RALP) with open pyeloplasty (OP) in children, but no previous study includes a long-term follow-up of renal function and hydronephrosis in combination with a thorough prospective follow-up of the RALP patients of at least 2 years.

Objective

To analyze perioperative results and long-term outcome of children with obstruction of the ureteropelvic junction, operated on with RALP compared to OP.

Patients and methods

Children ≤15 years operated on with RALP or OP from 2000 through 2013 were reviewed. Patient demographics, perioperative data, postoperative complications, and long-term outcome were evaluated. The outcome was based on pre- and postoperative examination of renal function, hydronephrosis and flank pain.

Results

129 pyeloplasties (84 OP, 39 RALP, 6 reoperations) on 123 patients were included. RALP had significantly longer operative time and shorter postoperative hospital stay, compared to OP. No difference was found in postoperative need of morphine or complication rates. Mean follow-up for RALP with ultrasound was 29 and 25 months with renal scan, compared to 34 and 28 months, respectively, for OP. The success rate for flank pain was 96 and 94 %, for hydronephrosis 93 and 95 %, and renal function 94 and 92 %, for RALP and OP, respectively.

Conclusions

RALP is a safe method, with advantages compared to OP, and with the same success rate. This study supports the use of RALP in children.

     

Acute renal failure complicating pediatric cardiac surgery: A comparison of survivors and nonsurvivors following acute peritoneal dialysis

Summary The outlook for patients with cardiac surgery complicated by acute renal failure (ARF) is poor, with a reported mortality of 50–67%. In addition to assessing the impact of recent advances in pediatric cardiac surgery on the mortality rate and renal outcome of surgery complicated by ARF requiring peritoneal dialysis (PD), this study compares preoperative, operative, and postoperative variables in patients who survived surgery and those who did not survive. From 1982 through 1988, 44 postoperative cardiac patients developed ARF, and 40 (age: 2 days to 15 years) required PD. Seventeen of 40 patients survived (mortality 57.5%) and 16 of these patients recovered normal renal function. Preoperative variables, including operative age and weight, did not appear to directly influence survival. Operative profiles, including length of cardiopulmonary bypass, aortic cross-clamp time, and hypotension immediately off bypass, did not distinguish surviving patients from those that did not survive. Postoperative variables, such as postoperative hypotension treatment, arrhythmias, hematologic status, cardiac arrest with resuscitation, did not differentiate survivors from nonsurvivors. The mean duration of PD was less than 2 weeks, and compications were infrequent. Renal status following PD in survivors was usually normal.We conclude that recent advances in pediatric cardiac surgery have not further increased the high mortality of surgery complicated by ARF. Survival is associated with renal recovery and thus aggressive treatment using PD is warranted.     

Maternal bariatric surgery: adverse outcomes in neonates

Background  

The obesity epidemic in developed countries has led to an increased prevalence of obese women of reproductive age. As maternal obesity has far-reaching consequences for both mother and child, the consensus is that weight loss before pregnancy will reduce obesity-related morbidity and mortality. Therefore, an increasing number of women become pregnant after undergoing obesity surgery.     

Newborn heart surgery: reasonable expectations and outcomes

This article discusses the necessity for standards for reasonable expectations and outcomes for neonatal treatment of congenital heart disease and the databases that allow such standards to be measured and to be compared among treatment centers.     

Robot-assisted laparoscopic ureteral reimplantation: A single surgeon comparison to open surgery

ObjectiveThe aim was to report a single surgeon's experience comparing open and robot-assisted laparoscopic extravesical ureteral reimplantation (RALUR) to treat vesicoureteral reflux (VUR).Subjects and methodsWe retrospectively reviewed the outcomes of RALUR and open extravesical ureteral reimplantations consecutively performed by a single surgeon between January 2008 and December 2010 using the da Vinci^® Surgical System. Both groups of patients were subjected to identical pre- and postoperative care protocols.ResultsDuring the defined study interval, 20 open and 20 RALUR procedures were completed by a single surgeon at our institution. Gender and VUR grade were similar in both cohorts. Operative times were longer in the RALUR group, but postoperative opioid use (morphine equivalents) was significantly lower in the RALUR group (RALUR: 0.14 mg/kg, open: 0.25 mg/kg, p = 0.021). There was no significant difference in estimated blood loss (EBL) or length of hospitalization (LOH). The overall rate of surgical complications was similar; however, the complications in the open group tended to be less severe than those occurring in the RALUR group. On follow-up, after a median of 52 months for open surgery and 39 months for RALUR, two children had developed a febrile urinary tract infection in both groups, of which one in the open group had persistent VUR.ConclusionThis single-surgeon experience of open and initial experience with RALUR performed with the same surgical technique on consecutive cohorts with identical post-surgical care protocol allows a comparative analysis of outcomes for a surgeon transitioning to RALUR. The RALUR reduces postoperative analgesic requirements while yielding similar clinical outcomes as the open technique.     

儿童心脏直视手术围术期血糖管理

自围术期高血糖与不良预后相关报道以来,关于围术期血糖管理问题就争论不休.虽然关于成年人的大量研究均证实危重患者高血糖发生率高,且高血糖会增加各种并发症及死亡的风险,而行胰岛素控制血糖会明显改善患者的预后.然而儿童糖代谢有别于成年人,特别是对于接受心脏直视手术的患儿,术中接受体外循环术,内环境发生改变,高血糖是否同样是危...     

Introduction to the genetics of primary renal tumors in children

Wilms tumor can be explained only partially by the “two hit” model that was originally developed for retinoblastoma. Heterogeneity of two kinds, operates. The first is that four other primary tumors are regularly observed in children, and the second is that Wilms tumor itself appears to represent more than one genetic entity. All five of these primary renal tumors arise from primary or secondary mesenchyme, renal blastema, or renal epithelium. Mesoblastic nephroma, and possibly clear cell sarcoma, may have some genetic affinity with Wilms tumor, but rhabdoid tumor of the kidney and renal carcinoma do not. At least three different genes seem to be important in the origin of Wilms tumor. One, WT1, whose mutations may be associated with aniridia, may follow the “two hit” model in that there are cases in which both copies of the gene are defective or lost, as expected for a tumor suppressor gene. A second gene, which is associated with Beckwith-Wiedemann Syndrome (BWS) and which has not been cloned, appears to be imprinted in females, and may have an oncogene function. It is evidently activated by gain of a paternal allele or by loss of the inactive, but possibly trans-sensing, maternal allele. Activation of the insulin-like growth factor II gene may be a final common pathway for mutation in both WT1 and BWS. A third gene is unlinked to either of the other two, but its location and function are unknown. It shares with WT1 specificity for Wilms tumor, which is not true of the BWS gene. © 1993 Wiley-Liss, Inc.     

Perioperative renal failure in pediatric heart transplant recipients: outcome and risk factors

PRF is encountered in 10-13% of adult heart transplants. Only one study of a single center's experience with PRF has been reported in pediatric patients. This study examines the effect of PRF on pediatric heart transplant outcome using the UNOS database. A total of 3598 patients met inclusion criteria, of whom 254 (7%) had PRF. The PRF group comprised 31 recipients requiring PRE and 223 recipients requiring POST. Compared with No-PRF patients, PRE patients had similar survival rate and POST patients had decreased survival rate at 30 days, one, five, and 10-yr post-transplant (p < 0.001). PRF patients also had significantly lower graft survival at one, five, and 10 yr (p < 0.001). Risk factors for developing PRF included ECMO, ventilator, and inotropic support at listing and CHD as the listing diagnosis. PRF increased the duration of hospital stay and the incidence of chronic severe renal dysfunction. PRF that requires POST (whether or not it began pretransplant) has a significant negative impact on pediatric heart transplant outcome. Specific characteristics identify patients at particular high risk of developing PRF.     

Renal growth in patients nephrectomized for Wilms tumour as compared to renal agenesis

Compensatory growth in 27 undiseased congenital solitary kidneys and in 31 solitary kidneys in patients with Wilms tumour was monitored in long-term follow-up studies by ultrasound volume biometry. In congenital solitary kidneys hypertrophy was not detectable at the time of birth. Parenchymal mass increase achieved 188% of the volume of a healthy kidney within at least 4 years of life and afterwards paralleled the physiological growth documented in healthy kidney pairs. Disease-free kidneys in Wilms tumour patients all developed a similar 180% volume augmentation within 2–4 years after nephrectomy, irrespective of the chosen mode of radiation and single or tripte chemotherapy. The age of the patient at onset of surgical and concomitant conservative therapy determined slight differences in kinetics but not in degree of compensatory growth.Abbreviations ACD actinomycin-D - VCR vincristin - ADM adriamycin     

Thyroid function and ioduria in infants after cardiac surgery: comparison of patients with primary and delayed sternal closure.

OBJECTIVES: Thyroid hormone alterations after cardiac surgery may be aggravated by the use of iodine antiseptics. We evaluated thyroid function and ioduria in infants with delayed sternal closure (DSC) who are exposed to povidone-iodine for sternal wound protection and compared them with findings in infants after primary sternal closure. DESIGN: Prospective clinical study. SETTING: Pediatric cardiac intensive care unit. PATIENTS: Ninety-three infants after cardiac surgery using cardiopulmonary bypass, 60 of them with primary sternal closure and 33 of them with delayed sternal closure. MEASUREMENTS AND MAIN RESULTS: Thyroid hormones were studied in patients with primary sternal closure immediately after surgery, 5 days and 19 days after surgery, in patients with DSC immediately after surgery, immediately after sternal closure, and 2 wks after sternal closure. Ioduria was evaluated on the first, third, and fifth postoperative days after cardiac surgery with primary sternal closure and immediately after DSC. In both groups of patients, low total triiodothyronine, total thyroxine, thyroxine-binding globulin levels, high reverse triiodothyronine levels, and normal free triiodothyronine, free thyroxine, and thyroid-stimulating hormone levels were recorded immediately after surgery. Concentrations of total triiodothyronine and thyroid-stimulating hormone were lower in the patients with DSC. Five days after primary sternal closure and 2 wks after DSC, all thyroid hormone levels were normal for age. Ioduria after DSC was higher than ioduria after primary sternal closure. CONCLUSIONS: Patients with DSC compared with patients with primary sternal closure display more profound thyroid suppression in the immediate postoperative period. The use of povidone-iodine adhesive drapes with single povidone-iodine mediastinal irrigation in patients with DSC is associated with significant iodine absorption but no significant thyroid dysfunction.     

Maternal primary hyperparathyroidism: discordant outcomes in a twin pregnancy

The case reports a neonate (twin 2 of a twin girl pregnancy) presenting with seizures due to hypocalcaemia. The presumptive cause of the hypocalcaemia was maternal hyperparathyroidism with concurrent vitamin D deficiency. The first twin remained free of hypocalcaemia and was vitamin D replete, despite similar exposure in the pregnancy and similar postnatal care.