Roberto Lobato

Posterior urethral valves: prognostic factors

To determine which variables besides bladder dysfunction can help to predict the outcome of renal function in boys with posterior urethral valves (PUV).

Bladder dysfunction as a prognostic factor in patients with posterior urethral valves

Objective To investigate the extent to which different types of bladder dysfunction can affect long‐term renal function in boys with posterior urethral valves (PUV).

DOES NEONATAL PYELOURETEROSTOMY WORSEN BLADDER FUNCTION IN CHILDREN WITH POSTERIOR URETHRAL VALVES

PURPOSE We determine whether long-term bladder dysfunction is more frequent in children with posterior urethral valves who undergo early supravesical urinary diversion (pyeloureterostomy) than in those who undergo valve ablation. MATERIALS AND METHODS Urodynamic studies were performed in 59 boys with severe posterior urethral valves who were divided into 2 groups based on initial treatment of valve ablation (30) and bilateral pyeloureterostomy (29). RESULTS Of the 59 boys 25 (42%) had a normal bladder, including 11 with an over distended bladder and 34 (58%) had bladder dysfunction, including instability in 22, poor compliance in 9 and myogenic failure in 3. Of the 30 boys initially treated with valve ablation 14 (46.6%) had a normal bladder, including 7 with an over distended bladder and 16 (53.4%) had bladder dysfunction, including instability in 10, poor compliance in 4 and myogenic failure in 2. Of the 29 boys initially treated with temporary bilateral pyeloureterostomy 11 (38%) had normal bladders, 4 with an over distended bladder and 18 (62%) had bladder dysfunction, including instability in 12, poor compliance in 5 and myogenic failure in 1. There were no statistically significant differences in the number or type of bladder dysfunction between the diversion and valve ablation groups. CONCLUSIONS Therefore, temporary pyeloureterostomy did not affect bladder function adversely in the long term.

Does bladder augmentation negatively affect renal transplant outcome in posterior urethral valve patients

OBJECTIVE Although renal transplant (RT) is a safe and effective treatment for end-stage renal disease, the outcome of RT has been mixed for posterior urethral valve (PUV) patients. In addition, some PUV patients need an augmentation cystoplasty (AC), which may negatively affect their RT outcome. The aim of this study is to compare RT outcome between PUV children with and without AC. MATERIALS AND METHODS Between 1985 and 2012 a total of 309 children received 369 RTs at our institution. Among these patients, 36 were had classified as having PUV. Of these, 12 underwent an AC before RT (AC group) and 24 did not (controls). Data, including age at transplant, allograft source, urological complications, urinary tract infection (UTI) incidence, the presence of vesicoureteral reflux (VUR), and patient and graft survival, were compared between groups. RESULTS Mean age at RT and mean follow-up were 7.6 versus 7.9 years and 8.9 versus 7.9 years in the AC group and in the control group, respectively (not significant [NS]). Allografts were from living donors in 50% of the AC group and in 41.6% of the controls (NS). The rate of UTI was 0.02 UTI/patient/year and 0.004 UTI/patient/year in the AC and control group, respectively (p = 0.001). Of the nine patients with UTI in the augmented group, five (55.5%) had VUR, while 5/8 (62.5%) patients in the control group with UTI had VUR. All patients with VUR in either group had UTIs previously. Of the five AC patients with more than three UTIs, two (40%) were non-compliant with clean intermittent catheterization (CIC), and UTI incidence was not associated with either a Mitrofanoff conduit or the urethra being used for CIC. Graft function at the end of study was 87.8 ± 40.5 ml/min/m(2) in the AC group and 88.17 ± 28.20 ml/min/m(2) in the control group (NS). The 10-year graft survival rate was 100% in AC group and 84.8% in controls. Two patients in the AC group lost their grafts (mean follow-up 13.3 ± 0.8 years) and five in the control group (mean follow-up 7.1 ± 4.7 years). CONCLUSIONS Bladder augmentation does not negatively affect renal outcome in PUV patients undergoing transplantation. However, recurrent UTIs are more frequent in transplanted PUV patients with an AC than in those without AC, and they are generally related to non-compliance with CIC or the presence of VUR but, mostly, they will not result in impaired graft function.

Supraceliac aortic clamping during the anhepatic phase of experimental orthotopic liver transplantation

BACKGROUND The pig tolerates simultaneous clamping of the liver pedicle and inferior vena cava poorly, so venovenous bypass has to be used during the anhepatic phase of experimental orthotopic liver transplantation (OLT). The aim of this work is to assess whether clamping of the supracoeliac aorta during the anhepatic phase (AP) of experimental OLT in pigs allows transplantation in stable hemodynamic conditions. METHODS Fourteen pigs (weight, 16 to 18 kg) received whole liver grafts from 14 age-matched donors and were subsequently divided into two groups: group I, OLT without venovenous bypass during the AP, group II, OLT with supracoeliac aortic clamping during the AP. Variables analyzed were cardiac output (CO) and related variables, mean systemic arterial pressure (MAP), mixed venous oxygen saturation (SvO2), hepatic artery and portal vein blood flow, systemic and hepatic O2 supply and uptake (SDO2, SVO2, HDO2, HVO2, respectively), liver enzymes, glucose, creatinine, and electrolytes. RESULTS In group I, CO, MAP, and SvO2, decreased during the AP (anhepatic) in comparison with baseline (preanhepatic) values (CO, 3.60+/-0.74, preanhepatic, v. 1.21+/-0.25 L x min(-1), anhepatic; P<.05. MAP, 97+/-12, preanhepatic, v. 43+/-17 mm Hg, anhepatic; P<.05. SvO2, 91.6+/-5.6, preanhepatic v. 70.0+/-12.5%, anhepatic; P<.05), and SDO2/SVO2 increased by 16% (preanhepatic) to 33% (anhepatic; P<.05). In group II, CO decreased during the anhepatic phase by only 21% (3.82+/-0.81, preanhepatic, v. 3.07+/-0.99 L x min(-1), anhepatic; not significant), the MAP increased significantly (100+/-8, preanhepatic, v. 135+/-4 mm Hg, anhepatic; P<.05), and SVO2, SDO2, SVO2, and SDO2/SVO2 remained unchanged. After revascularization, none of these variables differed significantly between groups, and levels of liver enzymes, glucose, creatinine, urea, and electrolytes were similar in both groups, both before and aftertransplantation. CONCLUSIONS Experimental OLT can be carried out in pigs without venovenous bypass, but it leads to severe hemodynamic disturbances. Clamping of the supraceliac artery during the AP is well tolerated and results in excellent hemodynamic stability, so it may prove to be a useful technique in liver transplantation in animals, such as dogs or pigs, that do not tolerate simultaneous clamping of the liver pedicle and inferior vena cava as well as human beings.

Bladder neck closure in children: long-term results and consequences.

AIM Only few studies have gathered information on the long-term outcomes of children undergoing bladder neck closure (BNC). In the present study, we analyze the long-term results and consequences in this population. PATIENTS AND METHODS The medical records of 20 patients (12 males and 8 females), who underwent BNC after several failed procedures to improve urinary incontinence were revised. Exstrophy complex was the underlying diagnose in all the cases: bladder exstrophy in 15 and cloacal exstrophy in 5. The median age of the patients at the time of surgery was 11.5 years (range 4-19 years). Previous surgeries were bladder neck reconstruction in 14 patients, bladder neck injection in 4 patients, and previous BNC in 1 patient. Overall 17 patients had concomitant bladder augmentation. The catheterizable stoma was made with appendix in 14, bowel in 3, and ureter in 3. The nine male patients aged>18 years at the time of this study received a questionnaire with specific questions on erections, orgasm, and details of ejaculation and were also asked to provide a semen sample. RESULTS The median follow-up was 10 years (range 2-17 years) and median patient age was 21 years (range 13-32 years). Urinary continence was achieved initially in 16 patients (80%); 4 had a bladder neck fistula, 3 of them underwent surgical revision and achieved dryness. Long-term complications were: bladder stones (eight patients); stomal problems (four stenosis and three leaks); bladder perforation (two patients), and orchitis (one patient). All patients above the age of 18 years reported erections and orgasms although two had medium erectile dysfunction. The ejaculate volume was reported as normal in five, scarce in three, and absent in one, with slow ejection in seven. Only four supplied semen samples and only two showed normal values. CONCLUSIONS BNC is an effective approach to incontinence when other procedures have failed. In the long term, the most frequent complications are those related with catheterizable stoma and stones. The high incidence report of a low fertility index and erectile dysfunction meant further study in a larger cohort.

Renal Transplantation in Augmented Bladders

Not many years ago, children with congenital abnormalities of the lower urinary tract or with bladder dysfunction were denied renal transplantation because they were considered very high-risk recipients. However, in the past few decades, we learned that in children with poorly compliant, low-capacity bladders, augmentation cystoplasty (AC) can create a compliant, low-pressure reservoir that helps preserve the kidney graft. Although the incidence of symptomatic urinary tract infection (UTI) may be greater in pediatric transplant recipients with an AC than in those without, UTI is related more to noncompliance with clean intermittent catheterization or vesicoureteral reflux to the native kidney or graft than to the AC itself, and usually does not lead to impairment of graft function. Today, children with a bladder reconstruction may undergo transplantation with the same outcome (graft survival and function) as those with normal bladders, although there is some possibility of malignant transformation in the intestinal segment used to augment the bladder in these patients.

Quality of Life in Female Epispadias.

Introduction and Aim Female epispadias is a rare congenital anomaly associated with significant urinary incontinence. This study aims to evaluate the results of its treatment in terms of continence, sexuality, and impact of incontinence on quality of life. Materials and Methods We retrospectively reviewed nine patients (5–39 years) treated of female epispadias in our hospital during the period 1976–2013. Urinary continence (ICIQ-SF), sexuality, impact of incontinence on quality of life (Potenziani-14-CI-IO-2000-QOL), and overall quality of life (SF-36) were evaluated. Results All nine women were treated by bladder neck reconstruction (Young–Dees–Leadbetter) and genitoplasty; four received 2.5 (R = 1–5) endoscopic bladder neck injections; one required enterocystoplasty, urinary diversion, and bladder neck closure because of persistent incontinence; five performed pelvic floor rehabilitation; and two took anticholinergics. Three achieved continence; five had mild urinary incontinence; and 1 had moderate urinary incontinence and was awaiting an endoscopic injection. Of the seven who were older than 18 years, five answered the questionnaires of quality of life and sexuality. All had a general quality of life (SF-36) that does not differ from the normal population and had a slight impact (7 [0–15] points) of incontinence on quality of life (Potenziani-14-CI-IO-QOL-2000). Four had a normal sex life, and the fifth had lack of self-confidence due to her incontinence. Conclusion Patients with female epispadias have good long-term results regarding quality of life and sexuality, despite having some degree of urinary incontinence.

Renal Outcome after Combined Liver-kidney Transplantation in Children

Background: Combined Liver and Kidney Transplantation (CLKT) is a recognized treatment option for a select group of paediatric patients with severe liver and kidney disease. The aim of this study is to report Indication and outcome of CLKT in our center and to compare the results with the Isolated Kidney Transplantation (IKT). Patients and method: We retrospectively reviewed children who underwent CLKT between 1997 and 2009. Data analyzed include age, sex, cause of kidney/liver disease, pre-transplant dialysis, donor age, cross-match, and immunosuppression regimen. Additionally, cold ischemia time, Acute Rejection (AR) episodes and patient and graft survival were compared with patients who had IKT in the same period of time. Results: 9 children (5 females, 4 males) underwent CLKT and 127 IKT. In CLKT group, mean patient age was 10.5 ± 3.9 years. Indications for CLKT were: primary hyperoxaluria (n=3), hemolytic uremic syndrome (n=1), Alagille Syndrome (n=1), nephronoptisis (n=1) and polycystic hepatorenal disease (n=3). Five patients were on dialysis before transplant. In all patients, both grafts were obtained from the same donor and cross-matches were negative. Immunosuppression was induced with Basiliximab and maintained with triple therapy (Tacrolimus+Mycophenolate mofetil+Prednisone). Mean cold ischemia time was shorter in CLKT (10.9 ± 3.5 vs. 16.8 ± 4.6 hours) and hospital stay was longer (35.5 ± 10 vs. 13.9 ± 5.3 days) compared to IKT. Long-term mean Glomerular Filtration Rate (GFR) and cystatin levels were similar in both groups. Rejection tended to be less frequent in CLKT (11 vs. 25%) than in IKT. Patient survival was lower (P=0.008) in CLKT. One patient with chronic liver and kidney rejection due to noncompliance died during re-transplantation 5 years after the first transplant. There were no deaths in the IKT group Mean follow-up time is 5.27 ± 2.9 years the IKT group Mean Follow-up time is 5.27 ± 2.9 years. Conclusions: Most common indications for CLKT in our children are primary hyperoxaluria and polycystic disease. Long-term results in children receiving CLKT are comparable to those with isolated kidney transplantation.

Strategy for high cavoatrial tumor thrombous in children with nephroblastoma

INTRODUCTION Wilms tumor surgery with intracaval-atrial extension is a challenge. Modern imaging techniques, precise preoperative thrombus location, and multidisciplinary surgical approach is mandatory. We aim to evaluate the outcome of our patients. PATIENTS AND METHODS Between 1992 and 2005, 52 patients with nephroblastomas underwent surgery in our institution. Nine of them had renal-caval (RC) or cavo-atrial(CA) thrombus extension. Four patients presenting short RC intravascular extension were excluded. RESULTS All cases were treated with pre-postoperative chemotherapy SIOP protocols. The level of the extension was retrohepatic in 2 cases, atrial in 3 patients and it even reached the ventricle in 2 of them. A multidisciplinary team was necessary to plan surgery and in all patients thrombus and tumor could be removed under cardiopulmonary by-pass in 3 cases (CPBP). One pulmonary tamponade due to thrombus migration (CAV) occurred and was solved by CPBP. Three cases were stage III, one stage IV and 1 stage II. Conclusions. Tumor size can be significantly reduced by preoperative chemotherapy. In case of CA extension, CPBP and right liver displacement to gain access to retro-hepatic cava are mandatory in order to reduce surgical complications.