Raimondo M. Cervellione

Effect of failed initial closure on bladder growth in children with bladder exstrophy.

PURPOSEnFailed initial bladder exstrophy closure may hinder the natural course of bladder growth compared to successful primary reconstruction. By measuring successive bladder capacities within the first 5 years of life, we compared the rate of bladder growth in children with failed vs successful initial closure.nnnMATERIALS AND METHODSnWe used an approved bladder exstrophy database to identify and review retrospectively patients with classic bladder exstrophy who underwent repeat cystograms between ages 1 and 6 years. Two groups of patients were identified--those with successful neonatal closure (group 1) and those with successful reclosure after an initial failed procedure (group 2). A generalized linear mixed model was fit to evaluate the impact of treatment group and age on bladder growth.nnnRESULTSnWe identified 48 patients in group 1 (75% male) and 62 in group 2 (71% male). Initial pelvic osteotomy was done in 60% of group 1 and 34% of group 2. Patients in group 1 had significantly larger cystographic capacity at 2, 4, 5 and 6 years after successful bladder closure compared to those in group 2 (p <0.05). The bladder tended to grow at a significantly slower rate in group 2 (9.38 cc yearly) compared to group 1 (14.76 cc yearly, p = 0.005).nnnCONCLUSIONSnPatients with initial failed bladder exstrophy closure showed significantly smaller cystographic capacities and slower bladder growth compared to those who underwent successful neonatal bladder closure. These data clearly underscore the importance of a secure, successful primary closure.

Penile ischemic injury in the exstrophy/epispadias spectrum: New insights and possible mechanisms

OBJECTIVEnPartial or complete penile loss following bladder exstrophy and/or epispadias repair has been reported in the literature progressively more frequently.nnnPATIENTS AND METHODSnThe authors report new cases of penile injury following bladder exstrophy and/or epispadias repair referred to their centers and not previously published. They review the literature on this subject and offer an explanation as to the likely mechanism for the penile injury and recommendations to avoid this complication.nnnRESULTSnSeven new cases of partial or complete penile loss following bladder exstrophy or epispadias repair have been recently referred to the authors institutions. Twenty-one patients have previously been reported in the literature. Altogether, 24 cases occurred after bladder exstrophy closure: 23 after complete primary repair of exstrophy (Mitchell repair) and one after first-stage radical soft-tissue mobilization (Kelly repair). Nineteen of 24 patients did not have a pelvic osteotomy at the time of primary closure. Four cases occurred after epispadias repair: two following the second-stage radical soft-tissue mobilization (Kelly repair) and two following penile disassembly epispadias repair (Mitchell repair).nnnCONCLUSIONnExstrophy closure combined with epispadias repair can be followed by ischemic penile injury, particularly when osteotomy is not performed. Compression of the pudendal vessels after pubic apposition and/or direct injury to the pudendal vessels play an important role in the pathogenesis of this complication.

The application of pelvic osteotomy in adult female patients with exstrophy: applications and outcomes

Study Type – Therapy (case series)

Vaginoplasty in the Female Exstrophy Population: Outcomes and Complications

OBJECTIVEnVaginal stenosis is a common sequela in adolescents who have undergone reconstruction for classic bladder exstrophy in infancy. We sought to determine the incidence of vaginal stenosis in our patient population and the outcome of treatment in the first three decades of life.nnnPATIENTS AND METHODSnAn institutional review board approved bladder exstrophy database was used to identify and retrospectively review classic female bladder exstrophy patients aged 12-30 years treated at the authors institution. Patients who underwent vaginoplasty were identified and the following outcomes were measured: age at surgery, method used for the reconstruction, complications and incidence of re-stenosis.nnnRESULTSnNinety-one female classic bladder exstrophy patients were identified. Twenty-nine patients (31.8%) underwent vaginoplasty because of vaginal stenosis at a mean (SD) age of 15 (3) years. Twenty-four patients underwent perineal flap vaginoplasty, three posterior cut-back vaginoplasty and two YV vaginoplasty. One patient developed wound infection and dehiscence which required reoperation (3.4%). No patient experienced vaginal re-stenosis.nnnCONCLUSIONSnvaginal stenosis is common after reconstruction of female classic bladder exstrophy. Vaginoplasty is highly successful in the exstrophy population when performed in the second or third decade of life with a low risk of complications.

Urinary Diversion in Early Childhood: Indications and Outcomes in the Exstrophy Patients

OBJECTIVEnTo evaluate indications and applicability of continent and incontinent urinary diversion (CUD and IUD, respectively) in early childhood in patients with classic bladder exstrophy (CBE).nnnMETHODSnUsing an institutionally approved exstrophy database, patients with CBE born after 1980 who underwent CUD or IUD by 2 surgeons within the first 5 years of life were identified. All aspects of their care and clinical outcomes were studied.nnnRESULTSnIn the CUD group (n = 14), only 21% had successful primary closure. Indications were desire to be dry (7), persistent hydronephrosis (4), urinary tract infections (UTIs) (1), repeat CUD (1), and inaccessible proper follow-up (1). Three patients had neobladder creation, 10 had bladder augmentation with continent stomas, and 2 underwent ureterosigmoidostomy. Currently, all patients are dry with clean intermittent catheterization (CIC). In the IUD group (n = 5), only 1 had successful primary closure. In addition to small, noncontractile bladders, the indications for IUD were severe hydronephrosis (2), recurrent UTIs (2), and noncompliance with catheterization (1). Four patients were re-diverted to CUD after a mean of 9.4 years and 1 has colon conduit. All are socially dry via catheterization. There was no case of renal function loss or malignant transformation.nnnCONCLUSIONnThe need for early diversion in CBE is primarily driven by upper tract changes after secondary closure and social factors. Urinary diversion can be safe in younger children with a favorable continence outcome.

Construction of a Natural Looking Inverted Umbilicus for Bladder Exstrophy

PURPOSEnMost attempts to create an umbilicus in the exstrophy abdomen eventually leave the end result of a flat scar rather than an inverted structure. We describe a technique that allows the creation of a better looking inverted umbilicus.nnnMATERIALS AND METHODSnA total of 19 patients between 5 days and 7 years old underwent umbilicoplasty. At the apex of the vertical midline wound the skin and superficial fascia are elevated off the anterior rectus sheath well beyond the top of the skin incision. The skin edge at the most superior apex of the wound is sutured to the linea alba, thereby inverting the skin and fixing it to the sheath. Two skin flaps based superiorly are cut from the margin of the inverted skin. The flaps are then rotated medial and sutured to the linea alba to form the base of the new umbilicus. The superficial fascia inferior to the umbilicus is opposed and the skin edges are approximated.nnnRESULTSnA total of 18 patients had bladder exstrophy and 1 had cloacal exstrophy. Of the patients 11 underwent bladder/cloacal exstrophy closure, 3 underwent epispadias repair, 3 underwent bladder neck repair, and 2 underwent bladder augmentation and continent cutaneous diversion. Mean +/- SD followup was 6 +/- 4 months. The technique allowed the creation of an inverted umbilicus in all patients. None experienced infection or dehiscence. The cosmetic result obtained was excellent and durable.nnnCONCLUSIONSnWe recommend the creation of an inverted umbilicus in the exstrophy population at bladder closure or later in life if umbilicoplasty is required.

Sexual function and fertility issues in cases of exstrophy epispadias complex

In patients with EEC, the issues such as sexuality, sexual function and fertility gain more importance once theses patients advance from puberty to adulthood. The aim of this review is to critically examine the available evidence on these issues. A systemic literature search was performed in Medline over the last 25 years using the key words: Exstrophy, sexual function and pregnancy. Search results were limited to studies of patients with exstrophy published in English literature. A total of 1500 publications were found and subsequently screened by title and when appropriate by abstracts. Of these, 40 publications pertinent to the subject were included for the analysis. The publications were supplemented by an additional 15 publications obtained from their bibliographies. The studies were rated according to the guidelines published by the US department of health and human services. Heterosexuality is usually expressed in both the sexes and most of them have adequate sexual function. Urinary diversion in some series seems to result in better ejaculatory hence fertility outcome in male patients. Recent series have shown equally good results with primary reconstruction. Most of the female patients have normal fertility while male patients have significantly low fertility. Most of the male and female patients with EEC have adequate sexual function. Most of the female patients have normal fertility while most of the male patients have significantly low fertility.

Re: Female epispadias management: perineal urethrocervicoplasty versus classical Young-Dees procedure. A. Cheikhelard, Y. Aigrain, H. Lottmann and S. Lortat-Jacob. J Urol, suppl, 2009; 182: 1807-1812.

To the Editor: We read with interest this article that confirms the value of the initial perineal approach to female epispadias. The authors describe their technique in detail and credit its creation to Manzoni and Ransley, who presented this approach in a video at the annual meeting of the European Society for Paediatric Urology in 2007. The procedure described in this well written article is quite similar to what we reported in 1993, and what we subsequently wrote about and illustrated in Campbell’s Urology. We agree with the authors that the perineal approach should be the primary choice for the treatment of female epispadias. However, our experience at The Johns Hopkins Hospital with 44 females with epispadias seems to suggest that long-term urinary continence cannot always be achieved with only the initial perineal approach. In our view there seems to be a paradox in the article between what is shown in the illustrations and the printed narrative regarding what is actually done or not done to the bladder neck. In our experience the perineal approach does not allow bladder neck modeling as demonstrated in figure 1 in the article, mainly because of the pubic bones, which in epispadias are not particularly separated. Since the bladder neck is either nonexistent or quite patulous, a bladder outlet procedure is still necessary in most patients, and for this reason the treatment for female epispadias often requires more than 1 reconstructive operation.

Outcome study of lower pole heminephrectomy in children

Objective. This study assessed the role and long-term outcome of lower pole heminephrectomy in the treatments of non-functioning lower renal moieties in children with duplex kidneys. Material and methods. In a period of 10 years 31 lower pole heminephrectomies were performed in 30 patients with duplex systems. Eight patients were diagnosed prenatally, 24 patients (80%) presented with urinary tract infection and three (10%) with vomiting and failure to thrive, and five patients also had other symptoms. The indication for lower pole heminephroureterectomy was reflux nephropathy in non-functioning lower pole in 28 patients (93%), pelviureteric junction obstruction in one patient (3%) and cystic dysplasia in one patient (3%). The surgical technique used was a combination of anterolateral loin incision plus right or left inguinal incision in 28 patients (90%). Results. The operative course was uneventful, no blood transfusion were required, and no damage to the upper pole moiety or upper pole ureter was observed. Four patients (13%) had immediate postoperative complications and three (10%) had late postoperative complications. Long-term follow-up revealed no complications in 27 patients (90%) and three patients (10%) with increased urinary frequency. Conclusions. The results indicate that lower pole heminephrectomy is the treatment of choice in cases of non-functioning dilated lower segments of duplicated kidneys. The use of two incisions in this procedure is effective in preventing the need for further surgical treatment secondary to complications of the stump.

Outcome study of upper pole heminephroureterectomy in children

ObjectiveWe assessed the role and long-term outcome of upper pole heminephroureterectomy in the treatments of non-functioning upper renal moieties in children with duplex kidneys.MethodsIn a period of 10xa0years, forty-three patients (male: female ratio 6:37) underwent upper pole heminephroureterectomy; a total of 25 patients were diagnosed prenatally. Imaging modalities included renal and bladder ultrasound in all 43 patients, static 99xa0m technetium dimercaptosuccinic acid (DMSA) in 21 patients, micturating cystourethrogram in 28 patients, MAG-3 in 13, cystoscopy in 30, IVU in 31 and DTPA, retrograde pyelography, antegrade pyelography in 1 patient.The main presentation was with urinary tract infections. Operation was performed through a combination of anterolateral loin incision plus right or left inguinal incision in 34 patients and high flank incision in 9 patients, for various abnormalities. One patient underwent also initial puncture of a ureterocele.ResultsFive patients (12%) had immediate post-operative complications. Thirteen patients (30%) had late post-operative complications. Long-term follow-up revealed no complication in 35 (81%) patients and one (2%) patient had one episode of UTI and intermittent abdominal pain. Three patients (7%) had long-term incontinence. Four patients (9%) had a second procedure performed.ConclusionBased on our experience, it seems that upper pole heminephroureterectomy is the treatment of choice in cases of obstructed upper segments of duplicated kidneys, when the affected segment contributes to less than 10% of the overall renal function.