[Growing up with a bladder augmentation]. / Creciendo con una ampliación vesical.

INTRODUCTION: This study assesses long-term outcome of patients with neuropatic bladders who underwent a bladder augmentation before puberty. PATIENTS AND METHODS: A total of 21 patients with low compliant neuropathic bladders who did not respond satisfactory to clean intermittent catheterization and/or anticholinergic therapy underwent bladder augmentation (mean age 8.3 yr, range; 2.5-12). Eighteen patients (86%) had VUR and/or ureterohydronephrosis and 17 (81%) had DMSA renal scars. Renal function was normal in all cases except one. All patients were followed at regular intervals with serum electrolyte and creatinine determination, urinary tract imaging, urodynamic evaluation and 24-hour urine collection. In the 18 cases augmented with intestine, urine cytology, cystoscopy and biopsy were also performed. RESULTS: Mean follow-up was 11 yr (8-14.5) and mean age at the end of follow-up was 19 yr (13.3-26.8). Urodynamic studies showed a significant improvement in bladder compliance. Upper urinary tract dilatation disappeared in all patients, VUR in 13/15p (86%) and no new renal scarring occurred. Renal function was normal at the end of follow-up in 20. Only 1 patient had UTI and another had a bladder stone. All patients are dry and 2 of them do not need clean intermittent catheterization. Urine cytology and cystoscopy were normal and no malignat lessions have been found in the biopsy specimens. CONCLUSIONS: Bladder augmentation done pre-puberty preserves renal function and resolves VUR and/or hydronephrosis in most cases without reimplanting the ureters. Close lifelong follow-up, including a cystoscopy, improves the results and prevents complications.

[Renal angiomyolipoma in childhood: a clinical case]. / Angiomiolipoma renal en la infancia: caso clínico.

The renal angiomyolipomas are unusual benign tumors which more than 50 per cent, are associated with tuberous sclerosis or intestinal linphangiomatosis. They can be discovered in a radiologic study by chance or to be associated with flank and abdominal pain and hematuria. The ultrasonographic imagen is characteristic and its management is conservative.

[Obstruction caused by anterior urethral valves in childhood]. / Obstrucción por válvulas de uretra anterior en la infancia.

Presentation of 3 patients (aged 11 years, 9 months and 9 years old) with diaphragm-like valves in the anterior urethra. Haematuria, urinary tract infection and thin voiding stream were the most common symptoms. Transurethral endoscopic resection with cold blade solved the problem appropriately in two cases, though the youngest patient required resection of stenotic area and end-terminal by-pass of the urethra.

[Cystic cystitis in childhood]. / Cistitis quística en la infancia.

Cystic cystitis is a benign entity highly uncommon throughout childhood and which, from a clinical and radiological point of view, can simulate an intravesical malignant neoplasia (rhabdomyosarcoma); thus an accurate differential diagnosis is required, a biopsy of the lesions being necessary to confirm their nature. The paper presents 2 patients with cystic cystitis discussing the condition's etiology, pathogenesis, signs and symptoms as well as its evolution.

[Comparative study of the degree of patient satisfaction in intermittent catheterization with Lofric and polyvinyl chloride catheters]. / Estudio comparativo del grado de satisfacción del paciente en cateterismo intermitente con el uso de las sondas Lofric y las de cloruro de polivinilo.

PURPOSE: To assess the grade of satisfaction in children on intermittent catheterization with the use of LoFric and PVC conventional catheters. MATERIAL AND METHODS: A total of 40 p with experience in CIC were included in this study. An anonymous questionnaire was sent to all patients after 2-months using the LoFric catheter. Patients were divided in 3 groups (bladder augmentation, artificial sphincter, Mitrofanoff) because of major differences in CIC discomfort between these groups. RESULTS: The questionnaire was completed by 87.5% of the patients (35 p). In 86% (30 p) LoFric catheter training was easy or very easy but in 14% (5 p) it was difficult. Four patients had some difficulty during conventional catheter insertion, in 3 (75%) the difficulty disappeared with the use of LoFric catheter. Of the 51% (18 p) who reported some discomfort during the insertion of conventional catheter, 72% said it was eliminated when the LoFric catheter was used. Of 6 p with some discomfort when removing the conventional catheter, 5 (83%) said it disappeared with the new catheter. Th LoFric catheter was favored by 70% of patients because it reduced the discomfort caused by conventional catheters, bladder insertion was easier and smoother, and gel lubrication was not needed. The 17% of patients reported some difficulty dealing with this slippery catheter. CONCLUSIONS: The use of the LoFric catheter could be justified in patients who report with conventional catheters have some discomfort. It can also be recommended in patients with artificial sphincter, bladder augmentation and Mitrofanoff procedure, in whom any complication related to CIC would have serious consequences.

[The use of a continent urinary stoma in complex reconstructions of the lower urinary tract in children]. / La utilización en el niño de un estoma urinario continente en las complejas reconstrucciones del tracto urinario inferior.

Since its description in 1980, the Mitrofanoff principle has become a widely utilized and successful technique for the management of patients with a variety of urological disorders. We report our experience with this procedure in 14 patients (10 M, 4 F). The age range was 3.5 years to 17 years (average 12 y) and follow-up was from 6 months to 3 years (average 1.7 y). Patients were classified in 2 groups: I) When this procedure was done because of the patient was unable to perform urethral catheterization (8p). II) Concomitant bladder neck transection and Mitrofanoff diversion (6p). The appendice was used in 9p, ileum in 1 and ureter in 4. Bladder augmentation was performed with ureter in 2p and colon sigmoid in 4. In 1p, ileo-cecal segment and in other colon+ileum, were used to replace the bladder. All patients catheterize the Mitrofanoff channel easily, there were no case of stomal stenosis and the conduit was continent in all. We consider that Mitrofanoff principle is a very successful technique and it can be used as the primary continence mechanism or as an adjunct of major urinary tract reconstruction, to ensure complete bladder emptying, in patients unable to perform urethral catheterization.

[Problems associated with nephrectomy of transplanted kidneys]. / Problemas asociados con la nefrectomía del riñón trasplantado.

Graft survival is not indefinite and a proportion of the failures must be removed. This study pretend to know the fate of failed renal transplant (RT) in the child, indication for transplant nephrectomy (TN) and morbidity involved. 96 RT were performed between 1985 and september 1994, during this time 31 grafts were lost: 71% immunologic causes, 13% relapse of original disease and 16% other reasons. Mean patient age and weight were 9 years and 27.3 kg, respectively. TN was performed in 28 (29%) and the most common indication was symptomatic rejection after withdrawal of immunosuppression (35.7%). Postoperative complications were diverse, including wound hematoma (13.6%), major hemorrhage (7%), fluid and electrolyte disorders (45.5) and 1 patient died (4.5%). Re-surgical approach was necessary in the 2 patients with major hemorrhage and 50% of the patients were in need of dialysis in the early postoperative. In this paper we appreciated that child with failed renal allograft could need TX in 90% of the cases, mainly because of symptomatic rejection. Furthermore, this procedure is more risky than simple native nephrectomy.

[Bladder augmentation in reconstruction of the urinary tract (1985-1997)]. / La ampliación vesical en la reconstrucción del tracto urinario (1985-1997).

Bladder augmentation with intestinal or urinary segments has virtually replaced other treatments in the management of both neuropathic and no neuropathic bladder dysfunction that has not responded to pharmacotherapy nor other intervention. We present herein our experience in 55 patients who underwent augmentation cystoplasty. Their mean age were 12.2 years (range 2.5-22.8) and the mean follow-up time was 4 years (1-13.1). They were divided in three groups according to the diagnosis: vesical or cloacal exstrophy (14 patients), neuropathic bladder (36) and posterior urethral valves (5 patients). Indications were: 1) to get a low pressure, high volume reservoir and avoid upper urinary tract damage in low-compliance bladders (41 patients); 2) as an undiversion (8 patients), and 3) prior to renal transplantation. Cystoplasty was performed with bowel segments in 47 cases and ureter in 8, adding some other urological procedures in 22 patients. Mean bladder capacity after 1 year was 400 ml versus 112 as previous value. 52 out of the 55 patients are continent after augmentation. There was no impairment of the renal function in the 5 patients with prior renal failure who underwent cystoplasty. Vesicoureteral reflux disappeared in 78.6% of the patients after cystoplasty. The complications were urinary stones in 5 cases, upper urinary tract infections in 3, and spontaneous bladder perforation in 1 patient. Augmentation cystoplasty is the best choice to achieve a low pressure reservoir, to assure contingency and to avoid progressive damage of the upper urinary tract in neuropathic or no neuropathic pediatric bladder dysfunctions.

[Priapism associated with Fabry's disease]. / Priapismo asociado a enfermedad de Fabry.

We present a rare case of priapism in a child, ten years old, in association with Fabry's disease. The child had a history of disseminated nodular enlargement, crises of fever, intermittent pain in the extremities and ten hours persistent painful erection of the penis. We don't obtain pain or erection relief with sedation, epidural block and irrigation of the corporal bodies. A saphenous-cavernous shunt, in the Grayhack fashion made, being results satisfactory. In the follow-up, the child had sporadic pain in the extremities and no erection of the penis. The cavernosography showed the shunt open. Fabry's disease was confirmed by nodular biopsy and the demonstration of deficient alpha-galactosidase.

[Results of kidney transplantation in children weighing less than 11 kilograms]. / Resultados del trasplante renal en niños con un peso inferior a 11 kilogramos.

Weigh and age are risk factors of graft failure. The aim of the study is to review the characteristics and the outcome of cadaver renal transplant in children weighing less than 11 Kg. From 1985 to 1999 10 cadaver renal transplant were performed in 10 children (7 boys and 3 girls). Primary renal disease were renal dysplasia(3), posterior urethral valves(5) and congenital nephrotic syndrome(2). All except two suffered end stage disease from birth. The cadaver donor age ranged from 4 to 45 years (mean 12.3). Cold ischaemia time was 14 to 30 hours (median 22.8 h). Grafts were placed extraperitoneally in the iliac fossa in all patients and special care was taken in aggressive intravascular volume expansion. In the first 5 children initial immunosuppression consisted of CyA, Pd and Aza. After 1991, the other five received sequential induction therapy with polyclonal antibodies and triple therapy (CyA, Pd, Aza). Renal function was evaluated as GFR yearly by Swartz formula and the actuarial and graft survival rates were obtained by Kaplan-Meier analysis. Patient survival was 90% at 1 and 10 years. Graft survival was 80% at 1 and 5 years; it decreased to 64% at 7 years. Seven continue with their first graft and the mean follow-up time is 6.6 years. Their renal function measured by the mean of GFR yearly decreased lightly from 102 ml/min/1.73 m2 at 1 year to 87.6 ml/min/1.73 m2 at 5 years. A successful patient and graft survival can be achieved in young receiving kidneys and small reciepients can improve their physical and mental development after transplantation.

[Pediatric renal transplantation from related living donor]. / Trasplante renal pediátrico de donante vivo relacionado.

Living related donor (LRD) provides significant advantages when compared with cadaveric donor (CAD) in term of improved patient and graft survival and shorten waiting time. From 1985, 176 kidney transplants were performed at our Center. Of these, 156 (89%) were from CAD and 20 (11%) were from LRD, first degree. The purpose of this paper is to show our experience at 5 years with use of LRD. All donors underwent standardized metabolic workup, angiography assessed and renal function test. Twelve children received their first transplant and 8 were retransplant (6-second, 1-third and 1-fourth). Immunosuppressive therapy consisted of globulin antithymocyte, azathioprine, cyclosporine and prednisolone, using FK506 and mycophenolate mofetil in some of them. Four kidneys with multiple renal arteries were reconstructed ex vivo with microsurgical technique before transplantation. The most significant morbidity was due to FK506-associated thrombotic microangiopathy (TMA) with graft lost. All patients (donor and recipient) survived. Five years graft survival rate is 95% and mean glomerular filtration rate is 81.33 ml/min/1.73 m2.

Are urodynamic studies really needed during bladder augmentation follow-up?

OBJECTIVE: We assessed clinical and urodynamic outcomes, over a minimum 10-year follow-up period, of neuropathic bladder patients treated with a bladder augmentation (BA) to determine if periodic urodynamic studies are needed. MATERIAL AND METHODS: Thirty-two patients with poorly compliant bladders underwent BA at a mean age of 11 years (2.5-18). Mean follow-up was 12 years (10-14.5) and mean patient age at the end of the study was 22 years (12.2-33). During follow-up all patients were controlled at regular intervals with urinary tract imaging, serum electrolyte and creatinine levels, cystoscopy and urodynamic studies. Preoperative, 1-year post-BA and latest urodynamic studies results were compared. RESULTS: Urodynamic studies at 1-year post-BA showed a significant increase in bladder capacity and a decrease in end-filling detrusor pressure compared with preoperative values (396 vs 106 ml; 10 vs 50 cm H(2)O, P<0.0001). The increase in bladder capacity was more significant at the end of the study than after 1 year (507.8 vs 396 ml, P<0.002). Thirteen patients had phasic contractions after 1 year and 11 at the end (not significant, NS), and these contractions were more frequent with colon than with ileum (NS). At the end of follow-up, phasic contraction pressure had decreased while trigger volume had increased (35 vs 28 cm H(2)O; 247 vs 353 ml, NS). All patients are dry and have normal renal function, except one who had mild renal insufficiency before BA. CONCLUSION: BA improves bladder capacity and pressure, and these changes are maintained over time (although phasic contractions do not disappear). Repeated urodynamic studies are only necessary when upper urinary tract dilatation or incontinence does not improve.

Enterocystoplasty in children with neuropathic bladders: long-term follow-up.

OBJECTIVE: This study assesses clinical outcome, after at least 8 years, of augmentation done before or at puberty in neuropathic bladders. PATIENTS AND METHODS: A total of 29 children with neuropathic bladders who did not respond satisfactorily to clean intermittent catheterisation and anti-cholinergic therapy underwent enterocystoplasty at a mean age of 11.8 years (range 3-18). Twenty-one children (72.4%) had vesicoureteral reflux (VUR) and/or ureterohydronephrosis and 22 (75.8%) had dimercapto-succinic acid scars, but all had normal renal function. All patients were followed at regular intervals with urinary tract imaging, serum electrolytes, creatinine, urodynamic evaluation and 24-h urine collection. Urine cytology, cystoscopy and biopsy were performed at the end of follow-up. RESULTS: Mean follow-up was 11 years (range 8-14.5) and mean age at the end of follow-up was 22.2 years (range 13.2-31). Urodynamic studies showed a significant improvement in bladder compliance in all patients. Upper urinary tract dilatation disappeared in all, VUR in 13/17 (76.4%), and no new renal scarring occurred in any patient. At the end of follow-up, renal function was normal in all according to serum creatinine, but cystatin C levels were normal in 27 and elevated in two. Significant proteinuria and low concentrations of renin and aldosterone were present in 80% and 82%, respectively. Only one patient had urinary tract infection, three had bladder stones, and in another a catheterisable channel was made. All patients were dry with normal urine cytology and cystoscopy, and no malignant lesions have been found in the biopsy specimens. CONCLUSION: Enterocystoplasty has preserved renal function and resolved VUR and/or hydronephrosis in most patients. The future implications of proteinuria and the low serum levels of renin and aldosterone, as well as the best indicator for measuring renal function, have yet to be determined. Close, life-long follow-up, including cystoscopy, is necessary to prevent complications.

Bilateral laparoscopic nephrectomy with simultaneous peritoneal dialysis catheter implantation.

Bilateral nephrectomy prior to transplantation is indicated in some patients with end-stage renal disease. The indications for bilateral nephrectomy include persistent heavy proteinuria, refractory hypertension, and urinary tract infections. We report an eight-month-old baby with male pseudohermaphroditism and renal failure secondary to diffuse mesangial sclerosis. While awaiting renal transplantation, dialysis became necessary and the child presented standard drug therapy-resistant hypertension. A bilateral nephrectomy was performed simultaneously to peritoneal dialysis catheter implantation by using laparoscopy. At the present time, the patient is doing well with ambulatory dialysis and all antihypertensive medication has been discontinued. We recommend this technique in children who require bilateral nephrectomy and peritoneal dialyisis. Not only is it somewhat less aggressive than traditional open surgery, but it also reduces post-operative pain, allows earlier initiation of peritoneal dialysis, and improves cosmetic appearance.

[Cystosonography with echoenhancer. A new imaging technique for the diagnosis of vesicoureteral reflux]. / Ecocistografía con contraste: una nueva modalidad de imagen para diagnosticar elreflujo vesicoureteral.

AIM: To analyze the utility of contrast enhanced ultrasonography of the bladder and kidneys (cystosonography) for the diagnosis of vesicoureteral reflux (VUR) by comparing the results of this new imaging modality with those of micturating cystourethrography (MCU). PATIENTS AND METHODS: A total of 169 patients (293 kidney units) aged between 3 days and 18 years were sonographically evaluated for the presence of VUR after filling the bladder with saline and a galactose and palmitic acid suspension (Levograf) as an echoenhancing agent. Ultrasonographic images were obtained during bladder filling and micturation. This procedure was always followed by MCU during the same diagnostic session. RESULTS: In 50 kidney units both cystosonography and MUR detected VUR during bladder filling. In 22 units, only cystosonography detected passive reflux during bladder filling and in 3 only MCU did so. In 56 units, both methods detected active VUR during micturation. In 17 units, only cystosonography detected active reflux during micturation, the results of MCU being normal, and in 5, only MCU detected active reflux. Overall, of the 293 kidney units, VUR was not detected by either of the imaging modalities in 204(69.6%) and was detected by both methods, irrespective of whether it was active or passive, in 63(21.5%). In 19 units, VUR (active or passive) was observed only by cystosonography and in 7 only by MCU. When MCU was used as the reference method, cystosonography had a sensitivity of 90.5% and a specificity of 91.4%. CONCLUSIONS: Contrastenhanced cystosonography is a reliable modality, with sufficient sensitivity and specificity in the diagnosis of VUR and does not expose patients to ionizing radiation.

[Experimental suture of the rat ureter]. / Sutura experimental del uréter en la rata.

The urological complications of renal transplantation in the rat when the bladder dome is used prompted us to study the viability of uretero-ureteral anastomosis with microsurgical techniques. The purpose of the study was to improve certain aspects of the technique employed in renal transplantation in the rat. For the study we utilized 27 Wistar rats. The left ureter was transected and anastomosed by free extramucosal sutures using the 180 degrees biangulation technique. Two groups of 13 rats were studied. For group A, a 6-0 nylon guide was used for the anterior aspect, whereas for group B anastomosis was performed without a guide. A urographic study was performed prior to sacrifice 21 days post-operatively. The pathological analysis included determination of foreign body reaction at the anastomotic site and the status of the renal parenchyma. The findings demonstrated better results were achieved in comparison with the technique previously employed using the bladder dome. Although slightly longer, using a guide seems to afford more reliable results, and is a useful technique in renal transplantation.

[Malformations associated with esophageal atresia]. / Malformaciones asociadas a la atresia de esófago: su influencia sobre la mortalidad. Estudio de 277 casos operados (1965-1981).

Associated malformations present in 277 newborn patients operated during the last sixteen years for esophageal atresia and tracheoesophageal fistula are described. Incidence on concomitant anomalies of each organ system is compared to that published by various authors during this period of time. Mortality, as related to each organ system and to the patient classification according to Waterston risk-groups, is studied during two different periods (1965-1975 and 1976-1981) considering in the second one a significant improvement of pre and postoperative care.

[Diuresis renography in the postoperative course of pyeloureteral stenosis]. / El renograma diurético en la evolución postoperatoria de la estenosis pieloureteral.

Ureteropelvic junction (UPJ) obstruction constitutes the most common form of upper urinary tract obstruction in children. We study the role of diuretic renography and its correlation with other diagnostic methods for postoperative evaluation of this malformation. We reviewed 13 patients, 10 males and 3 females aged 1 months to 15 years, and with right UPJ obstruction in six and left obstruction in seven. In all of them pre and postoperative IVP, abdominal ultrasound, diuretic renography, and pressure flow studies. Were performed our results demonstrated a non-correlation between the findings of excretory urogram, abdominal ultrasound and diuretic renography and a close relation between pressure flow studies and diuretic nephrography. This two last methods are good tools for assessment of UPJ obstruction.