Hypospadias preputial flap onlay technique - Step by step.

PURPOSE: To present one way of performing onlay preputial flap for hypospadias. PATIENTS AND METHODS: This procedure was performed following the methodology used in one hypospadias expert center to correct hypospadias in boys who are not elective for Koff procedure and in whom Koyanagi procedure is not needed. Operative details were described, and post-operative management were given as example. RESULTS: Long-term results of this technique showed a 10% complication rate (dehiscence, strictures or urethral fistulas) 2 years after surgery. CONCLUSIONS: This video is a step by step description of the onlay preputial flap technique giving the general methodology and also the details resulting from years of practice in one hypospadias expert center.

Surgery of Anomalies of Gonadal and Genital Development in the "Post-Truth Era".

This article aims to examine the current issues of debate concerning the management of atypical gonadal and genital development (AGD). Understanding this complex subject begins with defining the distinct AGD conditions, the aims and nature of surgical treatments, and the perceptions of affected individuals and their families. The evolving societal and medical contexts in this field are confronting facts and opinions, leading to a significant change in attitudes and approaches.

Spontaneous bladder rupture in non-augmented bladder exstrophy.

OBJECTIVE: Bladder perforation is not commonly described in bladder exstrophy patients without bladder augmentation. The goal of this study was to identify the risk factors of spontaneous perforation in non-augmented exstrophy bladders. METHODS: The study was a retrospective multi-institutional review of bladder perforation in seven male and two female patients with classic bladder exstrophy-epispadias (E-E). RESULTS: Correction of E-E was performed using Kelly repair in two and staged repair in seven (Table). Bladder neck repair was performed in eight patients at a mean age of 6 years. Three patients had additional urethral surgery. Before rupture, six patients were voiding only per urethra. Two patients were voiding urethrally but were also performing occasional CIC via a Mitrofanoff. One patient was performing CIC 3 hourly per urethra. Six were dry during the day. Six of the patients had lower urinary tract symptoms: five had frequency and four were straining to void. Two had suffered episodes of urinary retention. Pre-rupture ultrasound showed that the upper urinary tract was dilated in four patients. Micturating cystourethrogram was performed in six showing vesico-ureteral reflux in five. Two had urethral stenosis. Nuclear medicine was done in three patients with two abnormal differential function. Urodynamics was performed in two patients with low capacity (100 mL) and hypocompliant (<10) bladders. Both had high leak point pressures: 60 cmH2O at 100 mL. The mean age at rupture was 11 years, with a range of 5-20 years. Patients presented with abdominal pain, associated with signs of intestinal obstruction in seven and fever in two. Eight patients underwent laparotomy and one prolonged drainage via SPC. Simple closure was performed in seven and bladder neck closure in one, because of extension of the rupture inferiorly. All patients recovered well. Following rupture, five underwent augmentation and Mitrofanoff. One of these suffered a recurrent rupture. Two other patients refused augmentation and Mitrofanoff and one of these has since had a subsequent rupture. CONCLUSIONS: The limitations of this series include the small number of patients and its retrospective nature, without knowledge of the incidence. Bladder rupture is a risk even in non-augmented bladder exstrophy. It is potentially life-threatening and most often requires laparotomy. Rupture occurs because of poor bladder emptying and/or high pressure. Urodynamics may identify those at risk. CIC with or without augmentation should not be delayed once poor bladder emptying and/or high pressure are identified.

What is the outcome of paediatric gastrocystoplasty when the patients reach adulthood?

OBJECTIVE: To document the long-term outcomes of paediatric augmentation gastrocystoplasty (AGC) in terms of preservation of renal function and maintenance of dryness, and to analyse the rate of complications. PATIENTS AND METHODS: The medical records of children who had undergone AGC between 1992 and 2000 (minimum time interval of 15 years) were reviewed retrospectively. The following data were collected: age at surgery, the cause of bladder dysfunction, functioning of the AGC, any complications, and the long-term outcome of the patients. All of the patients were re-contacted by telephone. RESULTS: A total of 11 AGCs were carried out between 1992 and 2000, at a median (range) age of 11 (6.5-14) years. The diagnosis of patients undergoing AGC included myelomeningocele (four), bladder exstrophy (four), posterior urethral valves (one), irradiated bladder (one), and Prune Belly syndrome (one). The median [interquartile range (IQR)] follow-up was 17 (15-19.5) years. Renal function was preserved or improved in seven of the 11 patients and nine patients were dry after AGC. Seven of the 11 patients reported symptoms linked to haematuria-dysuria syndrome, which was resistant to treatment in one case and requiring excision of the gastric patch. Three of the 11 patients developed a tumour on the gastric graft after a median (range) delay of 20 (11-22) years after the initial procedure. All had gastric adenocarcinoma of which two were metastatic at the time of diagnosis requiring pelvectomy with pelvic lymph node dissection and adjuvant chemotherapy. Seven of the 11 patients underwent excision of the gastric patch after a median (IQR) time of 11 (8.5-20.5) years. CONCLUSIONS: Our long-term data confirmed that most patients undergoing AGC had preservation of their renal function and were continent. However, long-term, AGC was associated with a significant risk of malignant transformation and a high rate of surgical re-intervention involving removal of the gastric patch. These results question the use of this technique for bladder augmentation, irrespective of the indication. We highlight the importance of strict endoscopic follow-up of all patients already having undergone an AGC and the need to inform and educated patients about tumour-related symptoms.

Surgery in disorders of sex development (DSD) with a gender issue: If (why), when, and how?

Ten years after the consensus meeting on disorders of sex development (DSD), genital surgery continues to raise questions and criticisms concerning its indications, its technical aspects, timing and evaluation. This standpoint details each distinct situation and its possible management in 5 main groups of DSD patients with atypical genitalia: the 46,XX DSD group (congenital adrenal hyperplasia); the heterogeneous 46,XY DSD group (gonadal dysgenesis, disorders of steroidogenesis, target tissues impairments …); gonosomic mosaicisms (45,X/46,XY patients); ovo-testicular DSD; and "non-hormonal/non chromosomal" DSD. Questions are summarized for each DSD group with the support of literature and the feed-back of several world experts. Given the complexity and heterogeneity of presentation there is no consensus regarding the indications, the timing, the procedure nor the evaluation of outcome of DSD surgery. There are, however, some issues on which most experts would agree: 1) The need for identifying centres of expertise with a multidisciplinary approach; 2) A conservative management of the gonads in complete androgen insensitivity syndrome at least until puberty although some studies expressed concerns about the heightened tumour risk in this group; 3) To avoid vaginal dilatation in children after surgical reconstruction; 4) To keep asymptomatic mullerian remnants during childhood; 5) To remove confirmed streak gonads when Y material is present; 6) It is likely that 46,XY cloacal exstrophy, aphallia and severe micropenis would do best raised as male although this is based on limited outcome data. There is general acknowledgement among experts that timing, the choice of the individual and irreversibility of surgical procedures are sources of concerns. There is, however, little evidence provided regarding the impact of non-treated DSD during childhood for the individual development, the parents, society and the risk of stigmatization. The low level of evidence should lead to design collaborative prospective studies involving all parties and using consensual protocols of evaluation.

Long-term outcome of hypospadias surgery: current dilemmas.

PURPOSE OF REVIEW: To list the main questions and dilemmas raised by hypospadias management and its long-term evaluation. RECENT FINDINGS: Criteria of evaluation of hypospadias have changed in terms of anatomical description and biological screening. A better understanding of the causative and epidemiological aspects of the development anomalies of the genital tubercle has led to a more adjusted biological and surgical approach. The place of preoperative biological screening, preoperative hormonal stimulation and the choice of urethroplasty are the three main fields of discussion between hypospadiologists. Evaluation of outcome is the critical point as there are no current consensual protocols, and long-term results are often lacking as well as psychological evaluation. SUMMARY: Efforts are made to coordinate the management and evaluation of disorders of sex development (DSD) patients and more specifically hypospadias patients. National, European (EuroDSD) and international (consensus conferences) aim at finding common tracks to improve the quality of treatment and follow-up.

Undescended testes in children: the paediatric urologist's point of view.

Undescended testes (UDT) are found in 1% boys at the age of 1-year old, 3% of full-term male infants and 33% in premature babies at birth. Spontaneous descent is possible until 6 months of age. The two main phases of descent of the testis during gestation are described, as well as the possible aetiologies, the consequences of an abnormal migration, and the main locations of UDTs. Clinical examination is essential and should be done in optimal conditions. Laparoscopic exploration is the only valuable complementary investigation in the case of non-palpable UDT.