Penile repair in patients with epispadias-exstrophy complex-can we prevent resultant hypospadias?

PURPOSE: Male genitalia reconstruction in patients with exstrophy-epispadias complex often leads to secondary hypospadias, thus requiring additional surgery and resulting in possible complications. We describe a technique of male epispadias repair to prevent resultant hypospadias. MATERIALS AND METHODS: The complete penile disassembly technique is modified by multiple Z-plasties, which enable creation of a funnel-like bladder neck and urethral plate lengthening. A total of 29 patients 2 months to 12 years old presenting with pure epispadias and bladder exstrophy were treated with this technique between 2004 and 2011. These patients were compared to 19 patients 1 day to 11 years old with exstrophy-epispadias treated with the standard penile disassembly technique between 2000 and 2004. RESULTS: Outcome measures were urethral orifice position, penile length and complications. There was no statistically significant difference between the 2 groups regarding penile length or complications. In the study group the urethra reached the tip of the glans in all patients, whereas the creation of intentional hypospadias was necessary in 6 patients (31.5%) in the control group (p <0.05). CONCLUSIONS: The modified technique was effective in obtaining appropriate meatal location without decreasing the penile length. The complication rate was not changed. Creation of resultant hypospadias at genitalia reconstruction in male epispadias should strongly be prevented.

Bladder augmentation using acellular collagen biomatrix: a pilot experience in exstrophic patients.

PURPOSE: A preliminary experience on in vivo bladder wall regeneration in a subset of patients born with exstrophy-epispadias complex is reported. The objective was to improve bladder capacity and compliance without bowel augmentation. METHODS: Five patients (3 males, 2 females), mean age 10.4 years, presenting poor bladder capacity and compliance after complete exstrophy repair, underwent bladder augmentation using small intestinal submucosa (SIS) scaffold. Ultrasonography, cystoscopy with cystogram, assessment of bladder volume and compliance and bladder biopsy were performed before surgery (T0), at 6 (T1) and 18 months (T2) follow-up. Histology was compared with normal bladder specimens. Wilcoxon test was adopted for statistics. RESULTS: Bladder capacity and compliance resulted increased (+30%) at T1 (p < 0.05) and remained stable at T2, despite dry intervals did not changed significantly. Bladder biopsy at T1 showed no evidence of SIS, but normal transitional mucosa and sero-muscular layer containing smooth muscle fascicles, small nerve trunks and vessels within abundant type-3 collagen. Muscle/collagen ratio was decreased compared with controls at T1 and T2 (p < 0.05). No kidney damage, bladder diverticula, or stones were observed at 3 years follow-up. CONCLUSIONS: Bladder regeneration was feasible in these patients, but bladder capacity and compliance was poorly increased to obtain significant clinical benefit. Histology showed poor muscle components. The acellular matrix grafting failed to provide long-term effective results in terms of continence achievement.

Posterior urethral valves: long-term outcome.

Posterior urethral valves represent the most common cause of bladder outlet obstruction in infancy that impairs renal and bladder function. Long-term outcome of patients with previous PUV is evaluated. Patients over 18 years of age, treated from 1982 to 1995 before the age of 3 years were considered. Previous surgery, renal function, bladder activity, urinary incontinence, and fertility/sexual activity were evaluated. Clinical interview, creatinine clearance, uroflowmetry with ultrasound post-void urine residue, and self-administered questionnaire were recorded. Out of 45 identified records, 24 patients (53.3%) accepted to be enrolled (age 18-34 years, mean 23 years). The mean follow-up was 19.5 years (16-30 years). Out of the 21 excluded patients, 20 did not reply to the clinical interview and 1 died at age of 6 years. All the 24 patients had early endoscopic section of PUV; nine also received transient ureterocutaneostomy or vesicostomy. Ureteroneocystostomy was performed in five patients and ureterocystoplasty with unilateral nephrectomy in two. At follow-up chronic renal failure was detected in 13 patients (54.1%) and 9 (37.5%) had arterial hypertension. End-stage renal disease developed in five patients (20.8%): three had successful renal transplantation and two were in dialysis. Lower urinary tract symptoms were present in seven patients (29.1%). No significant fertility deficit and sexual dysfunction were observed in 23 patients, while 1 patient was azoospermic. No paternity was reported so far. Long-term outcome of patients with previously treated PUV is mandatory. Kidney, bladder, and sexual functions should be monitored till adulthood to verify any modified behaviour.

Urodynamics investigation on children with vesicoureteral reflux identifies overactive bladder and poor compliance in those with voiding dysfunction.

BACKGROUND: Up to 50% of children with vesicoureteral reflux (VUR) may have associated voiding dysfunction. It is thought to be an important determinate of the severity and resolution of VUR; however, to date there has been no objective measurement defining the impact of voiding dysfunction in children with VUR. The purpose of this study is to compare the urodynamic parameters of children with VUR who have and do not have symptomatic voiding dysfunction. METHODS: We performed a retrospective study of 138 children with a diagnosis of primary VUR who underwent urodynamic investigations. Information regarding patient demographics, grade and laterality of VUR and clinical history of bladder dysfunction were assessed. Urodynamic parameters recorded included detrusor overactivity, early and late compliance, voiding pressure, post-void residual volume and functional bladder capacity. Statistical analysis was performed using t Student analysis, Pearson's χ(2) test or Fischer's exact test, with a p < 0.05 as being significant. RESULTS: The mean age of the patients at the time of urodynamic evaluation was 5.8 years (SD 4.4). 30% had symptomatic voiding dysfunction based on the clinical history. Children without a history of voiding dysfunction had higher grades of VUR as compared to those with it (p = 0.002). Bladder hypertone, detrusor overactivity, detrusor hypereflexia and poor late bladder compliance presented a statistically significant higher incidence the incidence of bladder overactivity and poor late bladder compliance was higher in children with bladder dysfunction than those without it. CONCLUSION: Our findings suggest that voiding dysfunction does have objective and quantifiable effects on bladder dynamics. Urodynamic evaluation may play a role in the management of children with VUR by identifying those with bladder dysfunction secondary to abnormal voiding habits.

One-port retroperitoneoscopic assisted pyeloplasty versus open dismembered pyeloplasty in young children: preliminary experience.

PURPOSE: We propose 1-port retroperitoneoscopic assisted pyeloplasty as a minimally invasive approach and compare the results to open dismembered pyeloplasty. MATERIALS AND METHODS: All patients 6 months to 5 years old presenting with ureteropelvic junction obstruction between January 2008 and June 2009 were offered 1-port retroperitoneoscopic assisted pyeloplasty. Age matched patients who underwent open dismembered pyeloplasty during 2007 served as controls. The ureteropelvic junction was isolated retroperitoneoscopically and exteriorized through a single operative trocar. Pyeloplasty was performed in an open fashion with Double-J® stenting. Operative time, postoperative pain, surgical complications, hospital stay, ultrasound and mercaptoacetyltriglycine nuclear scan results at 6-month followup were evaluated and compared. Chi-square test and Student's t test were adopted for statistical analysis, with p <0.05 considered statistically significant. RESULTS: A total of 28 children (17 males) with a mean age of 18 months were treated with 1-port retroperitoneoscopic assisted pyeloplasty (18 left side). The control group consisted of 25 patients (11 males) with a mean age of 19 months who underwent open dismembered pyeloplasty (10 left side). Median operative time was 95 minutes (range 70 to 130) in 1-port retroperitoneoscopic assisted pyeloplasty and 72 minutes (58 to 102) in open dismembered pyeloplasty (p <0.05). Median postoperative hospital stay was 2.4 days with the 1-port approach and 6.1 days with the open procedure (p <0.05). Postoperative pain was significantly less in the 1-port group. Skin scar length was 1.4 to 2.9 cm (median 1.7) with 1-port retroperitoneoscopic assisted pyeloplasty and 3.5 to 6.0 cm (4.3) in the open group (p <0.05). CONCLUSIONS: The 1-port retroperitoneoscopic assisted pyeloplasty represents a safe and effective minimally invasive technique to treat hydronephrosis and could be the treatment of choice in young children. The procedure does not require laparoscopic suturing skills, and combines the advantages of open and laparoscopic pyeloplasty.

Laparoscopic Dismembered Repair in Two Patients with Retrocaval Ureter.

Retrocaval ureter (RCU) or circumcaval ureter is a rare cause of congenital hydronephrosis. The surgical correction of RCU should be performed in all patients with obstruction and hydronephrosis symptoms, lumbar pain, urinary tract infections, hematuria, or urolithiasis. Traditionally, an open surgical approach was used for the treatment of RCU. Nowadays, surgical correction of these anomalies is performed using minimally invasive techniques. We report on two cases treated with our standardized laparoscopic technique using only three 5-mm trocars. The proposed approach could be considered as the first-line treatment for RCU.

Life-saving vascular access after combined liver and kidney transplantation: A challenging access to the right atrium.

Exhaustion of vascular accesses is a major complication in patients undergoing hemodialysis, especially in pediatric setting. We report the case of a boy treated for loss of hemodialysis access after a combined liver-kidney transplantation and transient renal dysfunction. An interventional dilatation of calcific superior vena cava allowed to insert a stable central venous line for dialysis until full graft recovery. Careful management of central lines allows to spare the main vessels and reduces the need for unusual accesses.

Minimally Invasive Treatment of Pediatric Extrinsic Ureteropelvic Junction Obstruction by Crossing Polar Vessels: Is Vascular Hitching a Definitive Solution? Report of a Multicenter Survey.

BACKGROUND: This article aimed to evaluate the outcome of laparoscopic vascular hitch (VH) as an effective alternative to dismembered pyeloplasty in the treatment of children with extrinsic ureteropelvic junction obstruction (UPJO) by crossing vessels (CVs), by reporting the experience of three Italian centers of Pediatric Surgery. METHODS: From 2006 to 2014, 54 children (18 girls and 36 boys, average age 10.7 years) affected by suspected extrinsic UPJO by CV were treated in three different institutions: 51 patients underwent laparoscopic VH, while 3 patients, in which a coexisting intrinsic etiology of UPJO was doubtful, underwent laparoscopic dismembered pyeloplasty. Preoperative diagnostic workup included: ultrasound (US)/Doppler scan, mercaptoacetyltriglycine (MAG3)-renal scan, and functional-magnetic-resonance-urography (fMRU). Symptoms at presentation were recurrent abdominal/flank pain and hematuria. All patients presented intermittent hydronephrosis (range 18-100 mm) on US and an obstructive pattern on MAG3 renogram. RESULTS: Average operative time was 108 minutes, and average hospital stay was 1.8 days. As for complications, we reported a small abdominal wall hematoma and a high UPJ distortion without obstruction in 2 patients, not requiring reintervention (grade I Clavien-Dindo). At follow-up (range 12-96 months), all patients reported resolution of symptoms, decrease in the hydronephrosis grade, and improved drainage on MAG3 renogram. CONCLUSIONS: Our results demonstrated the safety and efficacy of laparoscopic VH for treatment of extrinsic UPJO by CV, registering excellent outcomes in a very selected patient population. The careful selection of patients through intraoperative assessment of anatomical and functional aspects is a crucial step to confirm indications for VH and maintain a high success rate with the procedure. We believe that any concerns regarding the coexistence of intrinsic stenosis should certainly lead to opening the collecting system and to performing a classic dismembered pyeloplasty.

Long-Term Follow-Up of Testicular Microlithiasis in Children and Adolescents: Multicenter Prospective Cohort Study of the Italian Society of Pediatric Urology.

Introduction Testicular microlithiasis (TM), characterized by the presence of intratubular calcifications in a single or both the gonads, is an uncommon entity with unknown etiology and outcome in pediatric and adolescent age. In this study, the results of a multicenter long-term survey are presented. Materials and Methods From 11 units of pediatric urology/surgery, patients with TM were identified and yearly, followed up in a 7-year period, adopting a specific database. The recorded items were: age at diagnosis, presenting symptoms/associated abnormalities, ultrasonographic finding, surgery and histology at biopsy, if performed. Results Out of 85 patients, 81 were evaluated yearly (4 patients lost to follow-up). TM was bilateral in 66.6% of the patients. Associate genital abnormalities were present in 90%, more frequently undescended/retractile testis (23.4%) and varicocele (22.2%). TM remained unchanged at 4.7 years follow-up in 77 patients (93.8%) and was reduced in 4 patients after 1 to 5 years of inguinoscrotal surgery. Orchiectomy was performed in three patients (3.7%), one for severe testicular hypoplasia and two for seminoma (2.5%), respectively, concurrent and metachronous to diagnosis of TM. Tumorectomy with parenchymal sparing surgery was performed in a teratoma associated with TM. Conclusion TM is a controversial entity, often associated with several inguinogenital features, which rarely can recover. Testicular malignancy, although present in TM, has not proven definitively associated to microliths. Proper counseling, yearly ultrasound, and self-examination are long-term recommended.

Microperc for pediatric nephrolithiasis: technique in valdivia-modified position.

INTRODUCTION: Micropercutaneous nephrolithotomy (microperc) has been recently proposed in adult patients. We present our initial experience in children using a modified technical approach. METHODS: After the retrograde positioning of a 5F catheter, a 4.85F metallic needle (1.9 mm) was used for direct percutaneous calyceal access under ultrasonographic and fluoroscopic guidance with the patient in supine Valdivia-modified position. Once a three-way connector was inserted to the needle, stone fragmentation was performed under direct vision using a 200 µm holmium:YAG laser fiber and a 0.9-mm high-resolution (10,000 pixels) optic flexible wire connected with the telescope, with intermittent saline infusion. The transureteral catheter was removed within 18 to 24 hours. RESULTS: A total of five patients aged 18 months to 11 years (mean, 5.8 years) received microperc lithotripsy in a 6-month period. The mean stone size was 15.0 mm (± 2.5 mm). Conversion to retrograde intrarenal surgery was necessary in one case, due to stone migration and poor visibility. Moderate hydronephrosis was present in one kidney. The mean operative time was 56 minutes (± 23 minutes). The blood loss was insignificant (< 0.6 Hbg/dL) and postoperative discomfort was minimal. The mean hospital stay was 2.4 ± 0.6 days for four patients after the removal of ureteral catheter. The JJ stent was removed after 15 days in the last patient. The overall stone-free rate at 1 month was 100% and no ancillary procedures were required. Conclusions Microperc is a new cost-effective and time-saving technique that we demonstrated as safe and effective in minimally invasive procedure for lower pole and pelvic renal stones of small size. Indications and limits should be defined by multicenter randomized studies, comparing the former procedures.

Treatment of pediatric renal stones in a Western country: a changing pattern.

BACKGROUND: Over the last 10years the miniaturization of endoscopic instruments made minimally invasive procedures for kidney stones feasible even in children. The evolution in management of kidney stones in a tertiary care center in Europe is reported. METHODS: Patients treated in our hospital for kidney stones from 2002 to 2011 were reviewed and group A (2002 to 2006) was compared with group B (2007 to 2011). The therapeutic options offered were Extracorporeal Shock Waves Lithotripsy (ESWL), Retrograde Intrarenal Surgery (RIRS), Percutaneous Lithotripsy (PCNL) and open surgery. Outcome measures were: first treatment chosen, stone free rate after a single procedure, and retreatment. Results were compared by chi-square test, with p <0.05 considered statistically significant. RESULTS: 333 patients, mean age 9.7years, were treated, 161 in group A and 172 in group B. ESWL was the first option in both groups, but decreased by 34% in group B vs A. In contrast, RIRS and PCNL increased by 17% and 16%, respectively, in group B vs group A. Open surgery was never required in primary lithiasis cases without associated malformations. CONCLUSION: The advent of PCNL and RIRS has significantly changed the pattern of renal stone treatment in the pediatric age group. A progressive increase of endourologic minimally invasive procedures was recorded. Open surgery should be a very rare option.

Selective arterial embolization of giant renal tuberous sclerosis.

Different minimally invasive methods have been proposed to treat renal manifestation of tuberous sclerosis complex (TSC), in order to preserve kidney parenchyma and avoid nephrectomy. The case of a boy with bleeding giant angiomyolipoma treated by selective arterial embolization is discussed. The extensive renal lesions resulted significantly decreased, with resolution of arterial hypertension and abdominal symptoms. Differential renal function increased from 26.6% to 32.6%. Renal involvement in TSC requires careful parenchymal-sparing procedures. Selective embolization of the main renal lesions should be considered as first line therapy.