Distal ureteral atresia – an embryological dilemma

Introduction: Distal ureteral atresia is a rare urinary tract anomaly generally associated with ipsilateral renal dysplasia and abnormalities such as multicystic dysplastic kidney, hydronephrosis and megaureter in the contralateral kidney. Despite burgeoning investigation modalities, definitive preoperative diagnosis of this condition is rarely feasible, also the embryological disarray of events that result in the development of this anomaly and the associated malformations is not clearly understood. Case presentation: We hereby report two cases of distal ureteral atresia and discuss the diversity in their presentations, diagnosis, atypical associations and management and review the possible embryological mal-development. Conclusion: Distal ureteral atresia with urogenital sinus as in Case 1 has not been documented so far and a plausible embryological explanation is deduced regarding its occurrence. The course of the affected kidney following timely and adequate relief of obstruction in Case 2 is depicted, highlighting the eventual management. Level of evidence: Not applicable

18F-FDG PET/CT in a Case of Urothelial Carcinoma in the Urachus Presenting as Colon Cancer

Urachal cancer arises from an embryologic remnant of the urogenital sinus and allantois and accounts for approximately 1% of bladder malignancies. The most encountered histologic subtype is adenocarcinoma. We present a 76-year-old man suspected to have an advanced sigmoid cancer infiltrating nearby organs. A supplemental 18F-FDG PET/CT showed high tracer uptake in a tumorous process coherent with the dome of the bladder wall involving the sigmoid colon. Cystoscopy revealed a normal bladder wall, except for a small edematous area in the anterior bladder. Biopsies from the sigmoid colon and transurethral resection from the bladder confirmed a urothelial carcinoma originating from the urachus.

Peculiarities of feminizing surgical correction in children with disorders of sexual development

Purpose – to analyze own results of surgical treatment of serious forms of disorders of sexual development (DSD) in children after feminizing surgeries. To define practical protocol guides on the choice of the type of surgical correction. Materials and methods. Over the past 5 years surgeons of Lviv regional paediatric clinical hospital «OHMATDYT» have examined 12 children with DSD, signs of hermaphroditism (intersex). After complex examination the following states were diagnosed: androgenital syndrome in 5 girls (referred for further treatment to Kyiv paediatric medical centres); true hermaphroditism – 1 child; mixed gonadal dysgenesis (MCG) – 3 children; female pseudohermaphroditism (without determining genesis) – 1 child; partial testicular feminization syndrome, Morris syndrome (male pseudohermaphroditism) – 2 children. In connection with marked masculinization of external genitalia two children with MCG and the girl with pseudohermaphroditism underwent feminizing surgery, namely genital zone reconstruction, mobilization and excision of urogenital sinus walls, excision of genital cavernous bodies (in children with MCG) with clitoroplasty, vulvoplasty with labioplasty and vaginoplasty. Children with Morris syndrome are being prepared to feminizing surgery. Results. Each child had a personal examination plan. Verification of a child’s state lasted from several weeks to 2–3 months. Children who underwent feminizing surgery did not have male structures and had relatively well-developed female structures. More «natural», less traumatizing correction was preformed for each specific case. In all children who underwent surgery vagina opened into urogenital sinus lower than external urethral sphincter, so there was no need to form front vagina wall (back and side walls were formed). Out of all children treated in our hospital gender was legally changed to the opposite. Conclusions. The birth of a child with DSD is a most challenging problem for parents, doctors, psychologists and social workers. Such states require complex examination. Children with severe hypospadias must undergo genetic examination. Statistic data and our own experience show higher frequency of feminizing surgeries in such cases. The most difficult part of such surgeries proves to be the excision of genital cavernous bodies with clitoroplasty and clitoris translocation; the most responsible part in functional sense is vaginoplasty. Surgical treatment is not the final stage of treatment for such patients. Children with DSD require constant further dynamic checkups by gynaecologists, urologists, oncologists, endocrinologists, psychologists with relevant therapy correction. The research was carried out in accordance with the principles of the Helsinki declaration. The study protocol was approved by the Local ethics committee of all participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: disorders of sexual development (DSD), hermaphroditism, feminizing correction.

Complete duplication of the urethra in a boy with epispadias

Duplication of the urethra is a rare abnormality that can occur with other urogenital tract congenital pathologies. Ischemia at embryogenesis, urogenital sinus defect, Muller`s duct anomaly and incomplete mesodermal fusion are the pathophysiological mechanisms of the pathology onset. Duplication of the urethra diagnostics is based on clinical examination, disease anamnesis, urethrography and magnetic resonance imaging. It is nessessary to consider Effmann and Leibowitz classification, urethras and prostate location and urethral orifice distance on operation of additional urethra removing. Clinical case. Presenting a clinical case of a boy with grade I epispadias and complete duplication of the urethra. Urination was maintained and was performed from the dorsal-epispadic urethra, the ventral urethra was obliterated in the distal region. According to the data of magnetic resonance imaging: double urethra is traced, dorsal – comes out from the anterior parts of the bladder wall, continues along the dorsal surface under the dorsal neurovascular bundle, above the cavernous bodies. The urethra opening is traced at the level of the upper surface of the head of the penis. The distal / ventral urethra comes out from the posterior parts of the lower bladder wall, typically continues down through the prostate embryo, rotated forward between of the corporas cavernosas stipes, and had a typical location with except for the level of the penis head, where it bends and merges with the dorsal urethra, which opens with a single opening. The surgery of excision of an additional urethra on child was performed. During the examination of the child of 6 months after surgery: urination with one stream from the ventral urethra at the apex of the head of the penis, stream freely passes the Nelaton catheter No. 10, child holds urine, there was no complaints. Performing of the next stage of the operation (penis plastic surgery) is planned after 1 year of the previous stage. Conclusions. In cases of epispadias is possible a congenital defect – a complete douplication of the urethra with the ventral urethra obliteration at the top of the head, which may not be determined in epispadias surgery preparation. Doubling of the urethra in boys requires magnetic resonance imaging for a better orientation in the defect anatomy and to determine the tactics of further surgical treatment. The research was carried out in accordance with the principles of the Helsinki declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: complete duplication of the urethra, surgical treatment, children.

RECONSTRUCTION OF THE FEMALE UROGENITAL COMPLEX IN THE PRENATAL PERIOD OF ONTOGENESIS

In 9-weeks prefetuses, urogenital complexes are located lower than in prefetuses of previous age groups. In the vertical sections of the urogenital cords, the paramesonephric ducts diff erentiate into the uterine tubes. Above the entrance to the pelvis, the urogenital cords pass obliquely in the vertical direction, at the level of which the paramesonephric ducts are connected, on both sides of which the mesonephric ducts are located. The primary kidneys are completely reduced in their upper two-thirds, the reduction of mesonephric cells in the lateral areas of the lower third of the kidneys begins. The caudal divisions of the paramesonephric ducts touch dorsally to the wall of the urogenital sinus, while the lower ends of the mesonephric ducts are in the thickness of the wall. The urogenital cords (mesonephric and paramesonephric ducts) protrude above the upper ends of the primary kidneys and below the permanent kidneys. The mesonephric and paramesonephric ducts are clearly demarcated by a layer of loosely spaced mesenchymal cells.In 10-weeks prefetuses, due to the connection of the lower vertical parts of the paramesonephric ducts, a common uterine- vaginal canal is formed, lined with pseudolayered epithelium. Mesonephric ducts retain their lumen in fragments.In 11-weeks prefetuses, the upper vertical sections of the urogenital cords, in connection with the reduction of the mesonephric ducts, contain mainly paramesonephric ducts, which are separated from each other by a loose layer of the mesenchyme. From these sections of paramesonephric ducts uterine tubes are formed. Oblique (middle) sections of the paramesonephric ducts with further development are transformed into intrauterine parts of the uterine tubes. The lower caudal sections of the paramesonephric ducts are transformed into the uterus and the upper two-thirds of the vagina.In 12-weeks prefetuses, the primary kidneys are almost completely reduced. Only single mesonephric tubules within their caudal area are determined. In parallel, there are intense processes of formation of internal female reproductive organs. The cranial ends of the uterine tubes expand, acquiring a funnel- shaped shape. The outer border of the infundibulum of the tube reminds a zigzag line, which is evidence of the beginning of the formation of the fi mbria of the uterine tube. The lumens of the mesonephric ducts are reduced to 6±0.1 μm, and in some parts of their walls are in contact with each other. Transformation of paramesonephric ducts into their derivatives (uterine tubes, uterus, upper part of the vagina) is accompanied by corresponding histological changes of their walls.

Imaging of urinary tract in children in different clinical scenarios: a guide for general radiologists

Background Children are frequently referred to the pediatric radiology department due to complaints related to the urinary tract. Main body We aimed to describe common clinical scenarios in paediatric urology practice and their recommended imaging diagnostic algorithms. Practical imaging approach to following common clinical scenarios and the common radiological findings are discussed: (A) perinatal urinary tract dilatation and other congenital anomalies; (B) recurrent urinary tract infection; (C) enuresis and daytime urinary incontinence; (D) abdominal masses; (E) flank pain; (F) hematuria; (G) trauma; (H) nonpalpable testis, ambiguous genitalia and common urogenital sinus anomalies; and I) renovascular hypertension Conclusions Imaging investigations should be tailored according to the clinical presentation in a stepwise approach aiming for optimum patients’ care.

5-HT3 Signaling Alters Development of Sacral Neural Crest Derivatives That Innervate the Lower Urinary Tract

The autonomic nervous system derives from the neural crest (NC) and supplies motor innervation to the smooth muscle of visceral organs, including the lower urinary tract (LUT). During fetal development, sacral NC cells colonize the urogenital sinus to form pelvic ganglia (PG) flanking the bladder neck. The coordinated activity of PG neurons is required for normal urination; however, little is known about the development of PG neuronal diversity. To discover candidate genes involved in PG neurogenesis, the transcriptome profiling of sacral NC and developing PG was performed, and we identified the enrichment of the type 3 serotonin receptor (5-HT3, encoded by Htr3a and Htr3b). We determined that Htr3a is one of the first serotonin receptor genes that is up-regulated in sacral NC progenitors and is maintained in differentiating PG neurons. In vitro cultures showed that the disruption of 5-HT3 signaling alters the differentiation outcomes of sacral NC cells, while the stimulation of 5-HT3 in explanted fetal pelvic ganglia severely diminished neurite arbor outgrowth. Overall, this study provides a valuable resource for the analysis of signaling pathways in PG development, identifies 5-HT3 as a novel regulator of NC lineage diversification and neuronal maturation in the peripheral nervous system, and indicates that the perturbation of 5-HT3 signaling in gestation has the potential to alter bladder function later in life.

A rare case of persistent urogenital sinus in an adult woman associated with unilateral rudimentary horn with ipsilateral renal agenesis and contralateral dermoid cyst

Persistent urogenital sinus (PUGS) is an uncommon developmental cloacal anomaly, with Incidence of 0.6 in 10000 female births. Herein we depict the case of a 22-year of age lady who presented with Infertility for 16 months with dyspareunia and was found to have Persistent urogenital sinus associated with other urogenital anomalies as unilateral rudimentary horn with ipsilateral renal agenesis and contralateral dermoid cyst. The patient was successfully treated with the excision of the sinus, the rudimentary horn and the dermoid cyst.