Gianantonio Manzoni

Tubularized incised plate hypospadias repair for proximal hypospadias

PURPOSE We report surgical technique and outcomes in consecutive patients with primary distal hypospadias. MATERIALS AND METHODS A prospectively maintained database of all patients operated by WS in 2000-2008 was reviewed for pertinent data in consecutive patients. RESULTS A total of 551 consecutive patients of mean age 17 months underwent distal tubularized incised plate hypospadias repair by urethral plate tubularization with (459) or without (92) midline incision. Follow up occurred for 426 (77%) at a mean of 8.2 months. Calibration and/or uroflowmetry were obtained in 279 (65%). Complications developed in 19 (4%), including nine fistulas, nine glans dehiscences and one delayed meatal stenosis from balanitis xerotica obliterans. These complications could not be attributed to meatal location, urethral plate configuration or incision, suture materials or methods for urethroplasty and glansplasty, or to use or not of a dartos flap barrier layer. CONCLUSIONS No contraindication to urethral plate tubularization with or without incision was found in 551 consecutive patients operated for distal hypospadias. Reliability of the procedure was confirmed by the low complication rate and success using varied suture materials and methods.

Tubularized Incised Plate Hypospadias Repair: Results of a Multicenter Experience

PURPOSE We report a multicenter experience using tubularized incised plate urethroplasty to correct distal hypospadias. MATERIALS AND METHODS A total of 148 patients underwent repair by 6 pediatric urologists at different institutions in the United States and Europe. RESULTS Tubularized incised plate repair created a functional neourethra with a vertically oriented meatus. Complications, including meatal stenoses and fistulas, occurred in 10 patients (7%). CONCLUSIONS Tubularized incised plate urethroplasty can be performed in most cases of distal hypospadias. Cosmetic results are superior to those of other popular techniques.

Hypospadias surgery: when, what and by whom?

Hypospadias is repaired by paediatric surgeons, paediatric urologists, adult reconstructive urologists and plastic surgeons. This review is unique in representing all four specialities, to provide a unified policy on the management of hypospadias. The surgeon of whichever speciality should have a dedicated interest in this challenging work, ideally having an annual volume of at least 40–50 cases. The ideal time for primary repair is at 6–12 months old, although when this is not practicable there is another opportunity at 3–4 years old. A surgical protocol is presented which emphasises both functional and cosmetic refinement. Using a logical progression of a very few related procedures allows the reliable correction of almost any hypospadias deformity. A one‐stage repair is used when the urethral plate does not require transection and its axial integrity can be maintained. Occasionally, when the plate is of adequate width and depth, it can be tubularized directly using the second stage of the two‐stage repair. When (usually) the urethral plate is not adequately developed and requires augmentation before it can be tubularized, then that second‐stage procedure is modified by adding a dorsal releasing incision ± a graft (alias Snodgrass and ‘Snodgraft’ procedures). The two‐stage repair offers the most reliable and refined solution for those patients who require transection of the urethral plate and a full circumferential substitution urethroplasty. From available evidence this protocol combines excellent function and cosmesis with optimum reliability. Nevertheless, it would be complacent to assume that these gratifying results will be maintained into adult life. We therefore recommend that there is still a need for active follow‐up through to genital maturity.

Vesicoureteral Reflux: Current Trends in Diagnosis, Screening, and Treatment

CONTEXT Vesicoureteral reflux (VUR) is present in approximately 1% of children in North America and Europe and is associated with an increased risk of pyelonephritis and renal scarring. Despite its prevalence and potential morbidity, however, many aspects of VUR management are controversial. OBJECTIVE Review the evidence surrounding current controversies in VUR diagnosis, screening, and treatment. EVIDENCE ACQUISITION A systematic review was performed of Medline, Embase, Prospero, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, clinicaltrials.gov, and the most recent guidelines of relevant medical specialty organizations. EVIDENCE SYNTHESIS We objectively assessed and summarized the published data, focusing on recent areas of controversy relating to VUR screening, diagnosis, and treatment. CONCLUSIONS The evidence base for many current management patterns in VUR is limited. Areas that could significantly benefit from additional future research include improved identification of children who are at risk for VUR-related renal morbidity, improved stratification tools for determining which children would benefit most from which VUR treatment option, and improved reporting of long-term outcomes of VUR treatments.

Failed hypospadias in paediatric patients

Failed hypospadias refers to any hypospadias repair that leads to complications or causes patient dissatisfaction. The complication rate after hypospadias repairs ranges from 5–70%, but the actual incidence of failed hypospadias is unknown as complications can become apparent many years after surgery and series with lifelong follow-up data do not exist. Moreover, little is known about uncomplicated repairs that fail in terms of patient satisfaction. Risk factors for complications include factors related to the hypospadias (severity of the condition and characteristics of the urethral plate), the patient (age at surgery, endocrine environment, and wound healing impairment), the surgeon (technique selection and surgeon expertise), and the procedure (technical details and postoperative management). The most important factors for preventing complications are surgeon expertise (number of cases treated per year), interposition of a barrier layer between the urethroplasty and the skin, and postoperative urinary drainage. Major complications associated with failed hypospadias include residual curvature, healing complications (preputial dehiscence, glans dehiscence, fistula formation, and urethral breakdown), urethral obstruction (meatal stenosis, urethral stricture, and functional obstruction), urethral diverticula, hairy urethra, and penile skin deficiency.

Bladder urothelial neoplasms in pediatric age: Experience at three tertiary centers

INTRODUCTION Urothelial bladder neoplasms (UBN) typically occur in patients in their sixth or seventh decade of life while they are infrequent in children and young adults. They occur in 0.1-0.4% of the population in the first two decades of life. Their management is controversial and paediatric guidelines are currently unavailable. OBJECTIVE To further expound the available data on the outcome of patients younger than 18 year old diagnosed with UBN. STUDY DESIGN We retrospectively reviewed the files of all the consecutive paediatric patients with UBN treated in three tertiary paediatric urology units from January 1999 to July 2013. Lesions were classified according to the 2004 WHO/ISUP criteria as urothelial papillomas (UP), papillary urothelial neoplasm of low malignant potential (PUNLMP), low-grade urothelial carcinoma (LGUC), and high-grade urothelial carcinoma (HGUC). RESULTS The table shows the results. Management after TURB varied among centres. One centre recommended only follow-up US at increasing intervals whereas another follow-up US plus urine cytologies and endoscopies, every three months in the first year, and at increasing intervals thereafter. After a median follow-up of 5 years (range 9 months-14.5 years), none of the patients showed disease recurrence or progression. DISCUSSION UBN is an uncommon condition in children and adolescents and, unlike in adults, its incidence, follow-up and outcome still controversial. Paediatric guidelines are currently lacking and management varies among centres. Gross painless haematuria is the most common presenting symptom. Therefore, this symptom should never be underestimated. US is generally the first investigation and additional imaging seems unnecessary. TURB often allows for complete resection. Lesions are generally solitary, non-muscle invasive, and low-grade (mainly UP and PUNLMP). Ideal follow-up protocol is the most controversial point. Reportedly, recurrence or progression during follow-up is uncommon in patients under 20 years, recurrence rate 7% and a single case of progression reported so far. Accordingly, a follow-up mainly based on serial US might be considered in this age group compared to adults where also serial endoscopies and urine cytologies are recommended. In the selection of the follow-up investigations, it should also be taken into consideration that urine cytology has a low sensibility in the detection of low-grade lesions while cystoscopy in young patients requires a general anaesthesia and hospitalization, and carries an increased risk of urethral manipulation. CONCLUSION UBN is a rare condition in children. Ultrasound is generally accurate in order to visualize the lesion, and TURB can treat the condition. Lesions are generally low-grade and non-muscle invasive, but high-grade lesions can also be detected. In present series, after TURB, follow-up US monitoring at increasing intervals was used at all centres, follow-up cystoscopies were added in two centres, but with different schedules. Urine cytologies were considered only at one centre. After a median follow-up of 5 years (range 9 months-14.5 years), none of the patients showed recurrence or progression of the disease.

Observational retrospective study on acquired megalourethra after primary proximal hypospadias repair and its recurrence after tapering.

INTRODUCTION Acquired megalourethra (AMU) after repair of proximal hypospadias can be a serious complication. An observational retrospective study of its incidence among different types of repair was performed. MATERIALS AND METHODS Clinical charts of patients operated on for proximal hypospadias were reviewed. INCLUSION CRITERIA all primary hypospadias operated in 1991-2004, with the meatus positioned in proximal penile, scrotal or perineal position. RESULTS Of 770 hypospadias cases treated, 130 (16%) were proximal. Seventy-two patients (55%) were treated using preputial flaps: 36 with a tubularized preputial island flap (TIF) and 36 an onlay island flap (OIF). Fifty-eight patients (45%) underwent staged repairs: Belt-Fuquà (BF) in 18 and Bracka procedure in 40 cases. After a mean follow up of 16 years (range 6-19) the overall incidence of complications for each technique was: TIF 36%; OIF 33%; BF 25%; two-stage Bracka 7.5%. The most common complication encountered was neo-urethral fistula. AMU occurred in only 5 cases, none with associated distal urethral stenosis, all in the TIF and OIF groups, and all successfully treated by reduction re-do urethroplasty. CONCLUSION A very small number of the patients operated using preputial island flaps techniques developed AMU. None of the staged repairs developed AMU, and this is the preferred choice in proximal hypospadias when the urethral plate requires division and/or substitution. All cases of AMU resolved after urethral tapering.

Different imaging strategies in febrile urinary tract infection in childhood. What, when, why?

Urinary tract infections (UTIs) are a common problem in childhood. During the last decade, published papers led to a deep revision in the use and usefulness of imaging tools in children with febrile UTIs. This new body of knowledge was partially endorsed by the guidelines published in 2007 by the UK’s National Institute for Clinical Excellence and in 2011 by the American Academy of Pediatrics. Nevertheless, new data continuously arise and the scientific debate always revives. Recommendations of published guidelines and strengths and weaknesses of the available biochemical and imaging tools are here critically analysed for giving to the reader a complete, up-to-date and flexible overview on this hot topic.

Adenine Phosphoribosyltransferase Deficiency: An Underdiagnosed Cause of Lithiasis and Renal Failure

We describe an infant affected by adenine phosphoribosyltransferase (APRT) deficiency diagnosed at 18 months of age with a de novo mutation that has not been previously reported. APRT deficiency is a rare defect of uric acid catabolism that leads to the accumulation of 2,8 dihydroxyadenine (2,8-DHA), a highly insoluble substance excreted by the kidneys that may precipitate in urine and form stones. The child suffered from renal colic due to a stone found in the peno-scrotal junction of the bulbar urethra. Stone spectrophotometric analysis allowed us to diagnose the disease and start kidney-saving therapy in order to avoid irreversible chronic kidney damage. APRT deficiency should always be considered in the differential diagnosis of pediatric urolithiasis.

Standardization of pediatric uroradiological terms : a multidisciplinary European glossary

To promote the standardization of nephro-uroradiological terms used in children, the European Society of Pediatric Radiology uroradiology taskforce wrote a detailed glossary. This work has been subsequently submitted to European experts in pediatric urology and nephrology for discussion and acceptance to improve the quality of radiological reports and communication among different clinicians involved in pediatric urology and nephrology.