Новый вариант техники перемещающей уретропластики с нерасчленяющей спонгиопластикой при дистальных формах гипоспадии у детей

Introduction. Recently, there have been publications devoted to laparoscopic removal of a bladder ear. The article sums up the multi-center experience in laparoscopic diverticulectomy.Materials and methods. Case histories of 30 children who underwent surgeries from 2012 to 2019 in 7 outpatient clinics were analyzed retrospectively. 22 boys (73.3%) and 8 girls (26.7%) were operated. At the time of the surgery, the mean age of the patients was 53.5 months. 15 (50.0%) children had right-sided diverticulum, 13 (43.3%) children suffered from left-sided one, and 2 had bilateral diverticulum (6.7%). The disease onset was presented by acute urinary tract infection in 25 (83.3%) children and urination dysfunction in 5 (16.7%). The laparoscopic access technique was used in all children. An isolated diverticulum was excised and the wall of the urinary bladder was restored. When the diverticulum was combined with a vesicoureteral reflux or ureterovesical anastomosis, ureteral reimplantation was added. Results. No conversions were found during the laparoscopy. Following the surgery, 3 (10.0%) children developed stent-associated pyelonephritis. No patient had complications during the observation that lasted from 6 months to 6 years. Conclusion. Laparoscopic technique is an effective and safe approach of treating a bladder ear in children.

Rationale: Pediatric patients with WTl-associated syndromes (including Wilms’ tumor-aniridia syndrome and Denys-Drash syndrome), Perlman syndrome, mosaic aneuploidy, and Fanconi anemia with a biallelic breast cancer type 2 susceptibility protein mutation have the highest risk of developing Wilms’ tumor. Patient concerns and diagnosis: We describe a patient with bilateral metachronous Wilms’ tumor, ambiguous genitalia characterized by 46, XY disorder of sexual development (DSD) with scrotal hypospadias and bilateral abdominal cryptorchidism, but without nephropathy. At the age of 7 months, the child underwent left nephrectomy with left orchiopexy. At follow-up after 8 months, a second tumor with a diameter of 10 mm was detected in abdominal CT scans at the lower pole of the right kidney. Intervention: Intra-operative macroscopic inspection of the right kidney revealed a tight attachment of the right proximal ureter to the tumor. Thus, retroperitoneoscopic resection of the lower pole of the right kidney had to be changed to an open surgical procedure with partial resection of the proximal ureter and high uretero-ureterostomy. We subsequently performed orchiopexy and two-stage correction of hypospadias using a free skin graft. Outcomes: At the last follow-up at the age of 8 years, no pathology requiring treatment was noted. A pair-end-reading (2 × 125) DNA analysis with an average coverage of at least 70 to 100 × revealed a previously unknown heterozygous mutation in exon 7 of the Wilms’ tumor suppressor gene 1 (WT1) gene (chr11:32417947G>A), leading to the appearance of a site of premature translation termination in codon 369 (p.Arg369Ter, NM_024426.4). This mutation had not been registered previously in the control samples “1000 genomes,” Exome Sequencing Project 6500, and the Exome Aggregation Consortium. Thus, to the best of our knowledge this represents a newly identified mutation causing incomplete Denys-Drash syndrome.

ПЕРВЫЙ ОПЫТ УРЕТЕРОУРЕТЕРОАНАСТОМОЗА (УРЕТЕРОПИЕЛОАНАСТОМОЗА) ПРИ УДВОЕННОЙ ПОЧКЕ У ДЕТЕЙ

Use of polyacrylate-polyalcohol copolymer (PPC) after endoscopic correction (EC) of vesico-ureteral reflux (VUR) is highly effective but is associated with a higher risk of obstructive complications (OC) compared with other implants. We undertook a STROBE compliant retrospective investigation and studied the OC risk factors to increase the practical safety of PPC. Overall, 798 patients (464 [58.1%] girls and 334 [41.9%]) boys) from 5 hospitals in whom PPC was routinely used were evaluated retrospectively. The patients were subdivided into 2 groups. Group I consisted of 754 (94.5%) children (449 [59.5%] girls and 305 [40.5%] boys) without OC. Median age was 41 months [Q1: 18.0; Q3: 81.0]. Group II comprised 44 (5.5%) patients (29 [65.9%] boys and 15 [34.1%] girls) experiencing OC, and their median age was 21.5 months [Q1: 12.0; Q3: 43.0]. Clinical and renal ultrasound examinations were carried out 1 day and 1 month after EC, and then every 6 months after EC. At the follow-up examination approximately 6 months after EC, voiding cysto-urethrography (VCUG) was performed. All patients with OC underwent diuretic renography. OC occurred in 44 (5.5%) of 798 children, in some cases as late as 60 months after endoscopic injection of the bulking agent PPC for correction of VUR. Univariate analysis revealed that younger age (P < .001), higher grade of VUR (P < .001), male gender (P < .001), second injection (P = .003), and EC injection using hydrodistension implantation technique (HIT; P < .001) represented significant risk factors. At multivariate analysis, only male gender (P = .0078), younger age (P = .0044), HIT technique (P < .0001), and second injection (P = .04) represented significant risk factors for the occurrence of OC. We identified young age, male gender, high reflux grade, HIT technique, and second endoscopic injections as factors associated with the risk of OC after EC of VUR using PPC as a bulking agent. Thus, patients who have undergone EC with PPC must be monitored sonographically for occurrence of OC for at least 60 months after the intervention.

Difficulties in the treatment of hypospadias in boys persist to this day. After surgical correction of hypospadias, fistulas, strictures, urethral diverticula, retraction of the meatus, glans dehiscence and other complications occur. At the same time, it is quite difficult to understand the whole variety of proposed methods for correcting hypospadias, which creates confusion for specialists and negatively affects the results of treatment. The literature describes more than 300 different methods of correcting hypospadias, but none of the methods is perfect, and there are no generally accepted treatment standards. Despite this, it is an established fact that the choice of the surgery procedure depends on the type of hypospadias. A successful technique of hypospadias repair should be completed with a good cosmetic and functional result. The article presents an overview of the distension techniques of urethroplasty. For the first time, the experience of urethral advancement was presented at the end of the XIX century, but the technique was unpopular and did not have significant success. However, by the end of the XX century, more effective distension techniques of urethroplasty began to be developed, which were widely used around the world (Koff S.A., Ti-Seng Chang, Belman A.B., MAGPI, LUM, etc.). According to scientific literature, urethral advancement is a safe and reliable way to correct distal hypospadias, and it is considered as an alternative to creating a neourethra. This technique has many competitive advantages, such as the short operation time, the absence of urethral tubularization, excellent functional and cosmetic results, and a small number of complications. It, therefore, follows that distension techniques of urethroplasty are considered a good option for correcting distal hypospadias, which should be in the arsenal of every pediatric surgeon and urologist.