Silvia Ceccanti

Renal function adaptation up to the fifth decade after treatment of children with unilateral renal tumor: a cross-sectional and longitudinal study.

Mild‐to‐moderate renal function loss may be an independent risk factor for cardiovascular disease and overall mortality. As in adults with renal carcinoma nephrectomy is associated with an high risk for moderate renal function loss, we aimed to assess the renal function adaptation over a long period of time in children with unilateral renal tumor (URT).

Infantile Abdominoscrotal Hydrocele: A Not So Benign Condition

PURPOSE Infantile abdominoscrotal hydrocele is a rarely described condition. We report the outcome of the largest single institution experience managing these lesions. MATERIALS AND METHODS We retrospectively reviewed descriptive case series of all infants treated between January 1998 and December 2007. Postoperative followup ranged from 3 to 107 months (median 48). RESULTS A total of 18 consecutive patients underwent abdominoscrotal hydrocele repair at age 3 to 21 months (median 8). The first 13 patients underwent inguinal hydrocelectomy, which consisted of high ligation of the processus vaginalis and complete excision of the abdominal component of the lesion. The remaining 5 patients underwent plication of the tunica vaginalis accomplished through the scrotum. Included in this group were 2 patients initially treated expectantly because the lesion was without undue tension at diagnosis. Both cases demonstrated spontaneous resolution of the abdominal component of the lesion but ultimately required surgery for scrotal hydrocele. Overall 23 testes (5 bilateral lesions) were operated on, of which 18 had some degree of testicular dysmorphism detected by ultrasonography or during surgery and 15 fully recovered postoperatively. A total of 11 patients experienced 1 or more postoperative complications, including persistent scrotal swelling (7), hypoplastic testis (3), scrotal hematoma (2), inguinal hernia (1) and cryptorchidism (1). Morbidity related to scrotal hydrocelectomy was negligible. There were no recurrences of abdominoscrotal hydrocele in the series. CONCLUSIONS Tense infantile abdominoscrotal hydroceles are associated with a high rate of testicular dysmorphism, which is often reversed by early intervention. Simple transscrotal plication of the tunica vaginalis represents an effective procedure with decreased postoperative complications.

Chronic Kidney Disease in Children With Unilateral Renal Tumor

PURPOSE In patients who have undergone nephrectomy lower stage chronic kidney disease may develop, which is an independent risk factor for cardiovascular disease and overall mortality. We investigated whether the prevalence of lower stage chronic kidney disease is related to the amount of renal parenchyma excised in children with unilateral renal tumor. MATERIALS AND METHODS A total of 15 patients treated with nephrectomy and 10 treated with nephron sparing surgery were enrolled at a single academic center. The Kidney Disease Outcomes Quality Initiative guidelines were used to classify patients by chronic kidney disease stage based on estimated glomerular filtration rate values. The Modification of Diet in Renal Disease study equation and Schwartz equation were used in patients older and younger than 17 years, respectively. RESULTS At a mean followup of more than 12 years 8 patients who had undergone nephrectomy and 1 treated with bilateral nephron sparing surgery presented with stage II chronic kidney disease (estimated glomerular filtration rate 60 to 89 ml/min/1.73 m(2)). Sequential measurements from diagnosis to 12 to 17 years postoperatively showed that stage II chronic kidney disease in patients who had undergone nephrectomy manifested as a negligible postoperative increase in mean ± SD estimated glomerular filtration rate (75.7 ± 25.5 vs 79.4 ± 3.9 ml/min/1.73 m(2), p = 0.6). Five of the 8 patients presented with stage II chronic kidney disease even before nephrectomy. The other 7 patients who had undergone nephrectomy and those treated with nephron sparing surgery presented with a significant postoperative increase in mean ± SD estimated glomerular filtration rate (81.1 ± 24 vs 102.3 ± 3 ml/min/1.73 m(2), p = 0.02, and 88.7 ± 2 vs 107.4 ± 14 ml/min/1.73 m(2), p = 0.005, respectively). CONCLUSIONS A subset of children with unilateral renal tumor presents before and/or after nephrectomy, and not after nephron sparing surgery, with stage II chronic kidney disease, probably due to a reduced renal reserve capacity. Whether patients with preoperative renal dysfunction may benefit from nephron sparing surgery should be studied in a cooperative clinical trial setting.

Posterior urethral valves: relationship between vesicoureteral reflux and renal function.

OBJECTIVE To investigate the relationship between renal function and vesicoureteral reflux before and after valve ablation in patients with posterior urethral valves. In these patients, back pressure may not be the only cause of renal damage. MATERIAL AND METHODS We conducted a retrospective review of 37 patients with valves consecutively treated between 1970 and 2002. Data were available for 31 patients, 19 of whom presented reflux at presentation. Grade of reflux was ascertained by voiding cystourethrography. Overall renal function was measured by serum creatinine, and split renal function was estimated by dimercaptosuccinic acid scan available for all patients but two. RESULTS Before relief of obstruction, there was no correlation between split renal function and grade of reflux into 25 kidneys of the 17 patients (r = -.13; 95% CI, -.50 to .27; P = .51). High-grade reflux (grade IV-V) affected 6 of the 11 renal units, with split renal function >40% vs 11 of the 14 units with split renal function <40% (P = .38). After successful valve ablation, reflux resolved in all the 11 units with split renal function >40% vs 4 of the 14 units with split renal function <40% (P = .0005). CONCLUSION The good renal function of more than half of the renal units with high-grade reflux at presentation, and the persistence of reflux mainly in nonfunctioning or poorly functioning kidneys after valve ablation, support the concept that in some patients with valves, reflux and renal damage are associated anomalies.

Re: Safety in glomerular numbers

Dear Editor, The main issue raised in the interesting educational review “Safety in glomerular numbers” by Dr. Schreuder [1] published recently in Pediatric Nephrology refers to the impact of a low nephron endowment on the outcome of renal function. The conclusion was that children with a low nephron endowment are at greater risk to develop a renal injury characterized by hypertension, glomerular damage, proteinuria, and need for renal transplant. In the review, there is no mention of recent data that suggests that not only renal failure but also minor renal function impairments may be associated with an increased risk of cardiovascular diseases and overall mortality. A meta-analysis of published results suggests that an estimated glomerular filtration rate (eGFR) 30 % lower than that of individuals with two healthy kidneys is associated with a 20–30 % increased risk for major cardiovascular events and death from any cause [2]. Many children with unilateral renal tumor present with renal dysfunction before surgery [3]. In addition, childrenwith unilateral kidney tumor undergoing nephrectomy have a significantly greater risk for new onset of renal dysfunctions than those undergoing nephron-sparing surgery [3]. In our experience, some children with renal tumor may have pre-existing chronic kidney disease (CKD), resulting in reduced renal reserve [3]. We found that about half of the children with unilateral renal tumor presented up to 12–17 years after nephrectomy (and not after nephron-sparing surgery) with mild renal dysfunction. Patients with a postnephrectomy renal function within the range of that of individuals with two healthy kidneys and those with a postnephrectomy renal dysfunction received similar adjuvant therapy [3]. In amore recent, extended follow-up study [4], we found that children nephrectomized for unilateral renal tumor presented after the third decade of life with progressive decline of renal function associated with an increasingly high prevalence of mild to moderate dysfunction. Our conclusion was that many children nephrectomized for unilateral renal tumor will present an ominous renal dysfunction during late adulthood. The time course of eGFR in many children nephrectomized for unilateral renal tumor was not much different from that in children with a congenital solitary functioning kidney who presented after the age of 30 years with a progressive decrease in GFR (Fig. 2 in [1]). The decline in physiological renal functionmight have an impact on patients who are not able to compensate for reduced postnephrectomy renal function. These data suggest that a nephron-sparing surgical approach, if feasible, is desirable not only in adults with unilateral renal-cell carcinoma but also in children with unilateral renal tumor. In addition, we suggest that not only patients with a congenital solitary kidney need “structured follow-up to find and treat risk factors for future health” (the conclusion in [1]), but also patients with unilateral renal tumor.

Laparoscopic pyloroplasty for idiopathic non-hypertrophic pyloric stenosis in a child

Acquired gastric outlet obstruction (GOO) during infancy and childhood represents an uncommon event. We describe a 6-year-old boy with GOO who did not respond to medical treatment or to endoscopic pyloric balloon dilatations. The stenosis was successfully treated with laparoscopic pyloroplasty which relieved the obstruction.

Renal function up to the 5th decade of life after nephrectomy in childhood: A literature review: Nephrectomy in childhood

The aim of the present study was to find out if in children ablation of 50% of renal mass may be associated with a progressive renal damage (Brenner’s hypothesis). We collected 1035 adult or adolescent survivors who underwent nephrectomy for unilateral oncological or non‐oncological causes during childhood. Stratification of all survivors for age revealed that the number of subjects with blood hypertension and/or renal dysfunction (glomerular filtration rate < 90 mL/min per 1.73 m2) to be significantly higher in survivors ≥30 years old in comparison with younger patients. Available data on long‐term renal function after nephrectomy during childhood support the Brenner’s hypothesis.

Renal Function Recovery after Nephrectomy or Nephron-Sparing Surgery in Children with Unilateral Renal Tumor

Introduction Children with unilateral renal tumor (URT) and preoperative renal dysfunction (PRD) may benefit from nephron‐sparing surgery (NSS). To test this hypothesis, we studied the outcome of baseline renal function after nephrectomy or NSS among children with URT. Materials and Methods Retrospective records review of children with URT who underwent nephrectomy (25 children) or NSS (11 children) at our institution. We analyzed the estimated glomerular filtration rate (eGFR) changes over time among patients, stratified by both preoperative renal function (with or without PRD) and surgical extent (NSS vs. nephrectomy). The primary end point was evaluation of compensatory recovery of preoperative eGFR after surgery. Only children older than 2 years at surgery were included in the study. Renal dysfunction was defined as an eGFR < 90 mL/min/1.73 m2. Results After nephrectomy or NSS, patients with PRD presented, on average during adolescence, a significant increase in eGFR, whereas patients without PRD presented, on average during adolescence, a stable eGFR. However, after nephrectomy, 5 of 17 (29%) and 7 of 8 (87%) adolescent patients with baseline eGFR ≤ or > 100 mL/min/1.73 m2, respectively, achieved or maintained two‐kidney eGFR values (T‐KEV) (p = 0.01). After NSS, four adolescent patients with PRD and seven without PRD achieved or maintained T‐KEV. Conclusion The majority of children with URT and low baseline eGFR present with an impaired renal function recovery after nephrectomy and may benefit from NSS. Collaborative studies are needed to support present findings.

Intrahepatic Paratesticular Cyst: Unique Presentation of Vestigial Remnants of Wolffian Duct

To report on a case of a progressively enlarging intrahepatic cyst in a 1-year-old boy who also presented with bilateral undescended testes. At surgery, the lesion emerged from the anterior surface of the liver but was unexpectedly found to arise from the epididymis of the right testis, which was located intra-abdominally. The histologic findings were consistent with a paratesticular cyst of vestigial remnants derived from the Wolffian duct. Such an unusual event has not been previously reported among the possible location of a paratesticular cyst, nor has it been described in the differential diagnosis of cystic lesions of the liver.

Low cervical skin crease approach for superior sternal cleft repair

Superior sternal cleft repair aims to restore bony protection to mediastinal structures and to eliminate visible deformity. We describe a 4-day-old neonate with an isolated defect who successfully underwent primary repair using a low cervical skin crease incision. Such a novel approach combined optimal surgical exposure and excellent cosmesis.