Tunica vaginalis flap for urethrocutaneous fistula repair after proximal and mid-shaft hypospadias surgery: A 12-year experience.

INTRODUCTION: Fistulas are a common complication of hypospadias surgery; they are more frequent after mid-shaft and posterior hypospadias repair. Surgical treatment of fistula still remains challenging with a significant failure rate. The basic principle is to add layers between skin and neourethra in order to decrease the incidence of recurrent urethrocutaneous fistula (UCF). We report our experience of UCF repair using a vascularized tunica vaginalis flap (TVF) after posterior and mid-shaft hypospadias surgery. MATERIAL AND METHODS: A retrospective review of all patients operated on using TVF for UCF in our institution between December 2005 and July 2017 was performed. RESULTS: Among 36 cases, TVF was used at a first attempt in 22 patients; 14 children had a prior attempt to close the fistula, and four of them had two surgeries before TVF repair. UCF was respectively penoscrotal (n = 3, 8%), posterior (n = 19, 53%), midshaft (n = 9, 25%) and anterior (n = 5, 14%). The size of the fistula was more than 5 mm in 26 patients. The UCF was treated successfully in every case after one single procedure. In the three children with two fistulas, both fistulas were successfully treated by the same TVF. After an average follow-up time of 45 months there was no recurrence of the initial UCF. In four cases of undescended testis, it was possible to dissect the flap through an inguinal incision and perform an orchydopexy in the same time. One patient presented a testicular atrophy after undescended testis surgery. DISCUSSION: Area review of published series shows excellent results in UCF repair including recurrent fistula (Table). TVF can aspire to some advantages with regard to a dartos flap (DF). First of all, a nearly 2.5-fold lower incidence of fistula after fistula repair with TVF than with DF (5.1% vs. 12.2%) has been shown. Secondly, TVF allows treating multiple fistulas, and can also be brought to the anterior part of the penis until the balano preputial furrow, allowing curing anterior fistula. Furthermore, it doesn't lead to aesthetic complications such as penile rotation or distal skin necrosis, which can occur during DF procedures. CONCLUSION: TVF is a simple and reproductive technique for UCF repair, with a high success rate. The risk of testicular atrophy has to be considered in case of associated undescended testis surgery, and careful attention must be given to the TVF dissection. This technique should be considered as first choice treatment for any UCF.

Recurrent Intragenic Duplication within the NR5A1 Gene and Severe Proximal Hypospadias.

A heterozygous intragenic duplication within the repeated area (CTGCAGCTG)×2 of the NR5A1 gene was found in a 15-year-old 46,XY DSD (disorders/differences of sex development) patient with micropenis and severe proximal hypospadias. This heterozygous duplication has already been described twice in boys with a similar phenotype, whereas a deletion of 3 amino acids at the same position in the protein SF-1 has been described in a 46,XX patient with primary ovarian failure and short stature. These data suggest that this region within the NR5A1 gene has an important role for SF-1 protein function in gonads and is a hotspot for intragenic rearrangements.

Percutaneous retrograde endovascular occlusion for pediatric varicocele.

BACKGROUND/PURPOSE: The aim of this study was to assess whether percutaneous retrograde endovascular occlusion (PREVO) is effective and safe for the treatment of varicocele in pediatric patients. METHODS: We retrospectively studied 71 children who underwent PREVO for left-sided varicocele. The primary outcome was the proportion of varicocele-free patients 6 months after PREVO as assessed by ultrasonography. RESULTS: Seventy-one boys with left-sided grade III varicocele underwent PREVO at a mean age of 13.2 years. PREVO was performed under local anesthesia in all boys but 2, who required general anesthesia. The procedure was technically feasible in 68 (96%) patients. In the remaining 3 patients, the internal spermatic vein could not be catheterized. Minor short-term complications occurred in 6 patients and resolved fully. No major complications or deaths were recorded. The proportion of varicocele-free patients 6 months after PREVO was 93% (66/71) overall and 97% (66/68) in the patients whose PREVO procedure was feasible. No clinical recurrence was observed during the mean follow-up of 17.5 months. CONCLUSIONS: Percutaneous retrograde endovascular occlusion is an effective minimally invasive approach for varicocele treatment in pediatric patients. It can be safely performed on an outpatient basis under local anesthesia.