[Acquired diaphragmatic hernia in a child with a related liver transplant]. / Priobretennaya diafragmal'naya gryzha u rebenka s rodstvennoi transplantatsiei pecheni.

Acquired diaphragmatic hernia in children is rare. Even less often, this disease occurs after liver transplantation for biliary atresia. In our case, diaphragmatic hernia was acquired, since the patient underwent repeated chest X-ray examinations including CT prior to liver transplantation. There were no signs of hernia. Clinical manifestations of diaphragmatic hernia were absent throughout 9 months after liver transplantation and manifested acutely with combination of symptoms of respiratory failure and intestinal obstruction. Surgical treatment was performed after emergency consultation with attending doctor.

[Ladd's syndrome in older children]. / Sindrom Ledda u detei starshego vozrasta.

Most often, Ladd's syndrome clinically manifests within the first month after birth. There are few reports devoted to diagnosis of this syndrome in patients aged 6-10 years. We present 2 children with Ladd's syndrome. The disease manifested at the age of 14 years in both patients. One child was diagnosed before complications that made it possible to carry out successful surgical treatment with subsequent recovery. The second child had necrosis of rotated small bowel. This child died in long-term postoperative period due to short bowel syndrome despite adequate treatment. In some cases, Ladd's syndrome manifests in puberty. Contrast-enhanced X-ray examination of gastrointestinal tract is indicated in children for periodic abdominal pain associated or not associated with vomiting.

[Renal abscess in children].

INTRODUCTION: Renal abscesses are relatively rare in children, but they can lead to prolonged hospital stay and life-threatening complications. Scrutiny of the literature over the past two decades indicates the absence of a unified tactic for the treatment of purulent-destructive forms of pyelonephritis in children, while more and more articles are appearing in terms of a low -key approach to the treatment of renal abscess in children. MATERIALS AND METHODS: From 2005 to 2019, we treated 59 children with the renal abscess. Among the patients were 22 (37.3%) boys and 37 (62.3%) girls. The location of the abscess on the right was determined in 30 (50.8%) children, on the left, in 29 (49.2%). The average age of the patients was 109 months. The median size of kidney abscess among all patients was 29 [21; 42] mm (range from 12 to 69 mm). RESULTS: The results of treatment were evaluated in the period from 3 months to 5 years. In 27 (45.8%) patients, conservative treatment gave a positive effect, while in 32 (54.2%) abscess puncture was performed under ultrasound guidance. The median hospitalization in patients after an abscess puncture was 15 [14; 18] days, against 13 [9; 17] days for children receiving only antibiotic therapy. The duration of hospital stay was significantly longer in the group of patients who underwent puncture (p=0.019). The effectiveness of conservative therapy was a lot lower in patients with a kidney abscess of more than 3 cm, 60% versus 31% (p=0.026). All 59 patients recovered completely, and none of them required an open surgery to drain a suppurative focus of a kidney or nephrectomy. CONCLUSION: Conclusion. Our experience confirms the literature data, indicating the need to use a conservative approach to the treatment of patients with the renal abscess as a first-line therapy. Identification of an abscess with a diameter of more than 3 cm in patients considerably increases the likelihood of using an abscess puncture with the absence of the efficacy of a conservative approach.

[New coronavirus infection (COVID-19) associated with kidney infarction in a seventeen-year-old child].

In December 2019, a new SARS-CoV-2 virus, called COVID-19, was identified in the Wuhan province of China, the spread of which determined the development of a pandemic. The lack of publications with evidence-based clinical studies dictate the necessity to cover various, including rare, pathological effects of the virus on the human body and the subsequent effect on it. Nowadays, there is already evidence that the SARS-CoV-2 virus causes specific damage to the vessels (endothelium), myocardium, and kidneys. The pathophysiologic mechanisms leading to acute kidney injury during COVID-19 infection are unclear, but may be due to direct exposure of the kidney tubules and endothelial cells to the virus. We present a clinical case of a kidney infarction in a 17-year-old girl with a severe course of a new coronavirus infection. At the time of the description of this clinical case, there are no publications on COVID-19 associated with kidney infarction in children in the Russian and foreign literature which is available to us.

[The results of treatment of newborns with postrenal anuria due to upper urinary tract obstruction, caused by fungal bezoar].

AIM: To evaluate the results of different methods of urine derivation in patients with postrenal anuria due to upper urinary tract (UUT) obstruction, caused by fungal bezoar. MATERIALS AND METHODS: The results of treatment of postrenal anuria in 8 patients without congenital obstructive anomalies of UUT in 5 clinics from 2004 to 2018 were analyzed. All patients from the birth received continuously two or more antibiotics for diseases not related to kidneys and the urinary tract. Median of gestational age was 32 weeks [31.5; 38.5]. There were 4 boys (50%), and 4 girls (50%). The duration of anuria at the time of hospitalization was 2 days [1.5; 5]. Creatinine level at admission was 218 mol/l [164.5; 392.5] and urea was 17.9 mmol/l [13.2; 24.95]. In all cases, candida albicans was revealed in urine. Postoperative complications were graded according to the Clavien-Dindo classification. RESULTS: All patients received systemic antifungal therapy; UUT drainage was performed in 7 (87.5%) cases. Ureteral catheter was placed bilaterally in 2 (28.6%) patients (4 renal units). There were 5 complications (Clavien-Dindo grade IIIb). Bilateral percutaneous nephrostomy was performed in 3 (42.8%) patients (6 renal units), and there were 2 complications (Clavien-Dindo grade IIIb and V). Open pyelostomy was performed in 2 (28.6%) patients (one bilateral, one on the right side; a total of 3 renal units). There was only one complication of Clavien-Dindo grade V. The average number of surgical procedures required to alleviate UUT obstruction per one renal unit was as following: 2.25 for ureteral catheterization; 1.17 for percutaneous nephrostomy; 1 for open pyelostomy. Regardless of the drainage method, diuresis tended to be normalized and azotemia decreased during the first postoperative day. In one case, the obstruction was eliminated by antifungal therapy without drainage of UUT. Mortality rate was 25% (n = 2). CONCLUSION: Ureteral catheterization is a minimally invasive, safe method for decompression of UUT obstruction, caused by fungal bezoar, which is non-inferior to nephrostomy tube or open pyelostomy. Ureteral catheterization can be used as a primary method of urine derivation in children with UUT obstruction caused by candida bezoar.

[Histological study of urethral plate: a search of the best material for surgical treatment of hypospadias in children].

AIM: to study the microstructure of the urethral plate in patients with a proximal form of hypospadias to justify the use of tissues from the ventral penile surface when creating an artificial urethra. MATERIALS AND METHODS: A histological study of surgical specimens from 10 patients with proximal hypospadias was carried out. The urethral plate, a strip of tissue from the ectopic external urethral orifice located in the penoscrotal angle, to the coronary sulcus, was completely excised and processed by various techniques. A staining with hematoxylin and eosin, picrofuchsin according to Van Gieson, as well as immunohistochemical typing of CD 34, collagen (type I, III, and IV), fibronectin, and smooth myocytes using alpha-smooth muscle actin was performed. RESULTS: All elements which are present in a healthy urethra, such as epithelial cells, blood vessels, normal collagen fibers, smooth muscle cells and complexes of glands, were found in the microstructure of the urethral plate. CONCLUSION: The cytoarchitectonic features of the ventral penile tissues are similar to the microstructure of the healthy urethra, which provides a rationale to consider the urethral plate as a material with optimal bioresistant properties for urethroplasty.

[Modification of two-stage technique of bracka with preservation and tubularization of the urethral plate for repair of proximal hypospadias].

INTRODUCTION: Surgical treatment of proximal hypospadias is associated with a number of difficulties and unsuccessful repeated interventions. The changing approaches to the treatment of hypospadias with the revision of the attitude to urethral plate provide new opportunities in resolving this problem. AIM: to improve the results of surgical correction of hypospadias by creating "artificial" urethra that is very close to a native one in its functional qualities. MATERIAL AND METHODS: From 2011 to 2017 . two-stage urethroplasty using Bracka technique was used in 41 patients with a proximal hypospadias aged 1-16 years. From those, 32 patients were undergone to primary intervention (78%), and 9 boys previously had unsuccessful urethroplasty performed by different techniques (22%). Oral mucosa graft was used in 9 patients (22%), and in other 32 cases inner preputial skin was harvested (78%). The classical and modified Bracka technique were performed in 19 and 22 cases, respectively. The urethral plate was preserved in 22 cases and "artificial" urethra was created by its tubularization, which contributes to the reduction of the required length of the harvested prepucial graft. RESULTS: The complication rate in patients after classical Bracka technique was 31.6% (n=6) in comparison with 4.5% in group of the modified urethroplasty (n=1). Thus, the efficiency of modified technique was significantly better than that of the classical intervention (<0,05). CONCLUSION: The preservation and use of urethral plate for urethroplasty during the two-stage surgical correction of the proximal hypospadias allows to decrease the total length of "artificial" urethra and to improve the properties of the newly formed urethra.

[Disorders of sexual differentiation in children: a critical look at open questions (part I)].

Management of children with disorders of sexual differentiation (DSD) continues to cause questions and receive criticism. This review discusses some contradictions concerning the newly proposed terminology, nomenclature, and classification. Congenital adrenal hyperplasia (CAH) is the most common cause of DSD, and its management also remains controversial. Therefore, in this part of the article, we critically analyze available literature on gender identity, the need and timing of surgery in children with DSD and 46, XX karyotype.

[Disorders of sexual differentiation in children: a critical look at open questions (part II)].

Surgical management of children with disorders of sexual differentiation (DSD) is one of the most difficult problems in pediatric urology. In the second part of the article we continue to discuss the controversies concerning the surgical management of patients with the classical form of congenital adrenal hyperplasia and 46, XX karyotype - feminizing genitoplasty and its different types.

[The volume of the contralateral testis in unilateral non-palpable cryptorchidism a diagnostic criterion to optimize surgical strategy].

AIM: Given the need to reduce the number of unnecessary laparoscopies, this study aimed to calculate the threshold values of contralateral testicular volume in unilateral non-palpable cryptorchidism to optimize diagnosis and surgical strategy. MATERIALS AND METHODS: From 2010 to 2016, contralateral testicular volume was prospectively evaluated in 78 patients aged under nine years with unilateral non-palpable cryptorchidism. The volume of healthy testes was calculated by multiplying length * width * height * 0.71. The patients were divided into two groups. Group 1 comprised 41 (52.6%) children with inguinal or intra-abdominal testes, who underwent orchiopexy for undescended testis. The second group consisted of 37 (47.4%) patients with severely hypoplastic testes that needed to be removed, or with testicular aplasia. In both groups, we matched the age (in months) at the time of surgery and the volume of the contralateral testis. Logistic regression was used to develop the diagnostic model. For the model, the ROC-curve (Receiver Operating Characteristic) was constructed, and the threshold of the contralateral testicular volume was selected. RESULTS: All patients with contralateral testicular volume greater than 2.25 ml had testicular aplasia, or hypoplasia (sensitivity 100.0%, specificity 73.0%). All patients with a contralateral testicular volume less than or equal to 1.04 ml had inguinal or intra-abdominal testes (specificity of 100%, the sensitivity of 34%). The area under the ROC curve calculated to assess the quality of the model was 92.6%, suggesting the high predictive power of the model. CONCLUSION: Revision scrototomy is indicated in patients with testicular volume exceeding 2.26 ml, while diagnostic laparoscopy is the optimal primary intervention in patients with the contralateral testicular volume of less than or equal to 1.04 ml.

[Uretero-ureteral anastomosis (ureteropyeloanasto-mosis) for the duplicated upper urinary tract in children].

INTRODUCTION: The duplex kidney is one of the most common congenital abnormalities of the urinary tract, and various surgical procedures exist for the management of this condition. Depending on the surgeon preferences, patients with duplex kidney may undergo heminephrectomy with ureterectomy using open or laparoscopic approach, uretero-cysto-anastomosis of the ectopic ureter or two ureters in a single block. Recently, there have been reports of inter-ureter anastomoses. Here we report our multicenter experience in open and laparoscopic uretero-ureteral anastomosis (UUA). The study aimed to improve the treatment results in children with urodynamic dysfunction due to the duplicated upper urinary tract. MATERIALS AND METHODS: We retrospectively analyzed medical records of 64 children treated from 2007 to 2017. There were 22 (32.8%) boys and 43 (67.2%) girls with mean age 40.2 months at the time of surgery. All of them had duplex kidneys, including 27 (42.2%) right-sided and 37 (57.8%) left-sided. Of them, 15 (23.4%) children underwent distal UUA (DUAA), and 49 (76.6%) had proximal UUA (PUUA). RESULTS: After surgery, acute pyelonephritis occurred in three patients. In the early postoperative period, a prolonged urinary leakage from surgical site drainage was observed in three (6.1%) patients after laparoscopic PUUA. One (4.2%) child developed a stumpitis. Thus, complications occurred in 7 (10.9%) children; one of them (1.6%) needed additional treatment. CONCLUSION: Distal and proximal UUA is a safe and effective surgical treatment for urinary outflow disorders in patients with the duplicated upper urinary tract, minimizing the risk of the duplex kidney dysfunction.

[Laparoscopic heminephrureterectomy for duplex kidney in children].

INTRODUCTION: Duplication of the upper urinary tract is one of the most common congenital urological anomalies. In patients with critically decreased or lost function of one of the renal segments, heminephrureterectomy is usually the treatment of choice. Until recently, this was an open surgery; in cases of complete removal of the ureter, an additional incision in the iliac region was required. Currently, heminephrureterectomy is increasingly performed laparoscopically. We report the experience in laparoscopic heminephrureterectomy (LHNUE) in 10 clinics in Russia and Belarus. Some of them have already used this technique for 10 years. AIM: The study aimed to to improve the treatment results in children with urodynamic dysfunction due to duplicated upper urinary tract. MATERIALS AND METHODS: We retrospectively analyzed medical records of 111 children treated from 2007 to 2016. There were 26 (23.4%) boys and 85 (76.6%) girls with mean age 44.6 months (from 2 to 170) at the time of surgery. All children included in the study had complete duplex kidneys, including 51 (45.9%) right-sided and 60 (54.1%) left-sided. All the children underwent LHNUE for a critical decrease or absence of function of the upper or lower segment of the duplex kidney caused by the following pathology: obstruction of the ureterovesical junction with the development of the megaureter of the upper ureteral segment in 57 (51.4%) patients; ureterocele in 28 (25.2%); extra-vesical ectopic ureter with urinary incontinence in 10 (9.0%) girls; high-grade UVR in 16 (14.4%) patients. RESULTS: There were no conversions in this series of patients. The mean operative time was 135 minutes (60-240 min.). All children included in the study were followed for 1 to 9 years after surgery. Complications occurred in 17 (15.3%) patients, of whom 12 (10.8%) required repeat surgery. In one patient with the loss of lower pole function, the treatment result was considered unsatisfactory. CONCLUSION: LHNUE for duplex kidney is performed by a few clinics and is still at the stage of development and accumulation of experience. Nevertheless, LHNUE, though an effective treatment modality, carries the risk of reducing or losing the function of the retained segment.

[Comparison of the results of correction of medium and proximal hypospadias using onlay island flap and tubularized incised plate methods in children].

Results of treatment of 35 children with primary medium and proximal hypospadias, including 15 boys undergoing Onlay Island Flap (OIF) and 20 boys undergoing Tubularized Incised Plate (TIP) were analyzed. At the time of OIF surgery the average age of children was 50.7 (14-130) months, TIP--26 (9-74) months. Long-term outcomes (at least one year after surgery) were registered in all children. After OIF, complications were observed in 9 (60%) children; after TIP--in 5 (25%). Statistically significant differences in the number of postoperative complications were not identified, but re-operation was statistically significantly less required in children undergoing TIP (p = 0.016).

[Features of drainage of pyelocaliceal system in children after laparoscopic pyeloplasty].

The study was aimed to improvement of results of treatment of children with congenital hydronephrosis due to reduction ofinvasiveness of operative approach and optimization of method of urine derivation in the postoperative period. From February 2008 to February 2012, 30 laparoscopic pyeloplasty were performed (16 boys, 14 girls, age from 2 months to 14 years, body weight 5500 g and more). Transabdominal pyeloplasty was performed by the Hynes-Anderson method. In 13 cases the ureteral stent deployment was performed, in 8 - pyelostoma was performed, nephrostomy performed earlier was maintained in 2 children, and in 7 children nephrostomy was performed by the proposed method. The mean duration of surgery was 110 (80-240) min. Results were followed for 3 to 53 months. 1 child on the 2nd day had falling pielostomy with the development of pyelonephritis, and 1 child had formation of urinoma. Both children had consistent anastomosis not required re-pyeloplasty. One child had persistent anastomotic stricture, which required re-pyeloplasty. Follow-up examinations included the ultrasound and intravenous urography. At the present moment, there are no complaints at all children, exacerbations of infections of the urinary system are not registered. Thus, laparoscopic pyeloplasty is an effective technique regardless of the age of the children, but it is necessary to further improve the drainage of the pelvicalyceal system in the postoperative period.

[Preputial reconstruction in surgical correction of distal hypospadia in children].

Treatment for distal hypospadia was conducted in 148 children aged from 11 months to 14 years admitted to the urological department of the Syktyvkar Central Children's Hospital in 1998-2010. Operation MAGPI was made in 12 (8.8%) children, TIP--in 87 (61.8%), Mathieu--in 15 (11.0%), displacing urethroplasty--in 25 (18.4%). Preputial reconstruction was performed in 29 (21%) children: in 21 in displacing urethroplasty and in 8 in conduction of TIP operation. The number of complications in displacing urethroplasty and TIP is less than in MAGPI and Mathieu operations. Significantly less number of complications was seen only in comparison of TIP with MAGPI. Operations with preputial reconstruction had no such complications as urethral fistula, meatostenosis and separation of the glans penis. Two cases of partial preputial separation after the operation were observed. Thus, preputial reconstruction can be conducted in most children with distal hypospadia, the risk of postoperative complications being the same.