Urogenital Sinus Research Articles

Urinary continence after high urogenital sinus repair conducted with posterior prone approach: electromyography-uroflowmetric assessment.

We aimed to present the results of urinary continence assessment objectively with electromyography (EMG)-uroflowmetry after high urogenital sinus (HUGS) repair with posterior prone approach without division of rectum. The records of patients who underwent HUGS repair via posterior prone approach between January 2005 and July 2018 were reviewed retrospectively. Incontinence, dysuria, hesitation, and straining during urination were evaluated during the clinical follow-up. Dysfunctional voiding scoring system was used as a questionnaire. Patients were re-evaluated with EMG-uroflowmetry in terms of voiding volume and pattern, voiding time, maximum flow rate, average flow rate, maximum flow time, and post-voiding residual volume. Seven patients with HUGS were treated with a posterior prone approach. The median age of the patients was 18months (8-21months). The median UGS length was 4.4cm (3.6-5.5cm), urethral length was 1.1cm (1.0-1.5cm), and vaginal length was 4.9cm (4.1-5.1cm). No urination or defecation problems were described by the patients or their parents. When the results of the dysfunctional voiding scoring systems questionnaire were analyzed, results scored 7 (range 5-8). EMG-uroflowmetric test graphics of the patients showed normal flow curves without plateau, intermittency or irregularity. Pelvic EMG assessment was normal in all patients. EMG-uroflowmetry has shown objectively that urinary continence and normal voiding pattern are preserved after HUGS repair with posterior prone approach without division of rectum.

Long-term outcomes of the surgical treatment of girls with a classical form of congenital adrenal hyperplasia

Introduction. Currently, to study long-term outcomes of feminizing genitoplasty, which is performed to girls with congenital adrenal hyperplasia, is an important step due to the limited data in literature on this topic. The purpose was to assess long-term outcomes of feminizing genitoplasty in congenital adrenal hyperplasia in girls depending on their gender identity, sexual orientation and the anatomy of external genitalia and vagina.Material and methods. A retrospective analysis of 8 case–histories of girls who were operated on at the Arkhangelsk Regional Children’s Hospital in 2001-2004 was made. In 7 of them, who had low urogenital sinus, feminizing genitoplasty included vaginoplasty with a perineal flap; in one patient, who had a high urogenital sinus, the shortened vagina was advanced down to create a missing part with posterior skin (Fortunoff) and anterior mucous (Passerini) flaps. The Prader scores ranged from III to V. Mean age at the time of surgery was 5.6 y.o. (0.5-14). Mean follow-up period was 17.6 (16-18.5) years.Results. All patients had female gender identity. All four sexually active women reported heterosexual orientation. Cosmetic results were good as stated by physicians, parents and patients themselves. Vaginal stenosis was noted only in one patient.Conclusion. The results obtained have shown that the one-stage reconstruction (clitoroplasty and vaginoplasty) for congenital adrenal hyperplasia can be successfully performed in childhood; thus, there is no any reason to refuse of the accepted tactics in treating this group of patients.

Robot-assisted excision of urachal cyst: case report in a child

BackgroundThe urachus is an embryological structure of the urogenital sinus and allantoid that connects the allantois to the early bladder in fetal life and then remains as the median umbilical ligament connecting the umbilicus to the dome of the bladder. An early laparoscopic procedure could trigger a quiescent urachal remnant to become symptomatic, causing a lesion or infection either during carbon oxide contamination or insufflation or a periumbilical or suprapubic port placement.Case presentationA 15-year-old girl complaining of supra-pubic abdominal pain. About 2 months previously, she had undergone laparoscopic appendectomy for acute appendicitis, and early postoperative period was uneventful. She underwent a robotic-assisted excision of a urachal cyst.ConclusionsIt has been suggested that early laparoscopic procedures could trigger previously asymptomatic urachal remnants to become symptomatic. Robot-assisted excision of a urachal cyst is a safe, effective alternative to open surgery in children.

Pitfalls of Prenatal Diagnosis guiding Postnatal Management in Congenital Adrenal Hyperplasia(CAH)

Background: 21-hydroxylase deficiency is the most common form of CAH and is associated with a variety of clinical phenotypes (salt wasting SW, simple virilizing SV and non-classic NCCAH). Commonly, there is a strong genotype-phenotype correlation for SW and NCCAH, but this is less predictable with the SV forms. We present a case with prenatal diagnosis of classic CAH which demonstrated genotype-phenotype discordance. Clinical Case: Ex 39 weeker female born to non-consanguineous parents was prenatally diagnosed with CAH based on routine genetic screen. Mother was noted to be a carrier for Intron 2G and father was a carrier of p.I172N both known to be pathogenic variants on CYP21A2. At 23 weeks gestational age, DNA analysis revealed fetus was compound heterozygous for both mutations which is most commonly associated with either SV or SW phenotype. At birth, infant had mild edema of the labia majora which resolved; the clitoris was not enlarged. There was no genital virilization or urogenital sinus. Newborn screen sent at 15 hours of life:17 OHP 132 ng/ml, repeat on DOL 3:77 ng/ml and DOL 13:59.9 ng/ml. High dose cosyntropin stim test on DOL 3 at 0 min: Cortisol not done, 17 OHP 1279 ng/dl; 60 min: Cortisol 12.3mcg/dl, 17OHP 3394 ng/dl. DOL 5 at 0 min: Cortisol 2.7 mcg/dl, ACTH 164.5 pg/ml, 17OHP 803.7 ng/dl; 60 minute: Cortisol 7.6mcg/dl, 17OHP 5920 ng/dl. Using available 17OHP normograms, the infant’s stimulated 17 OHP levels were not consistent with classic CAH. Unstimulated testosterone on DOL 3: 25ng/dl, DOL 5: 14 ng/dl. Ultrasound showed adrenal gland thickness 4mm bilaterally (upper limit of normal). Hydrocortisone (HC) was started on DOL 5 at 35 mg/m2/day after the stim test. On DOL 7, HC was increased to 100 mg/m2/day; fludrocortisone 0.1 mg twice daily and NaCl 0.5 g/day were started (Na 131, K 6.5, plasma renin activity 80.2). Upon discharge (DOL 13), infant was on HC 35 mg/m2/day, Fludrocortisone 0.1mg twice daily and NaCl 2g/day. Doses were adjusted accordingly during outpatient follow up. Currently infant is 4 months of age, thriving and remains on HC 11.3 mg /m2/day, Fludrocortisone 0.05 mg twice daily and NaCl 750 mg daily. Postnatal genetic analysis confirmed prenatal genotype. Conclusion: In general, genotype-phenotype correlation has 80–90% concordance. However clinicians should be aware of genotype/phenotype discrepancies that exist in order to carefully guide postnatal management based on prenatal genetic analysis. Our patient’s 17-ohp was done by LC/MS, which is standard now for most specialized endocrine laboratories. However, the 17-ohp nomograms for CAH, which are frequently used for subtype categorization, are based on RIA levels. Further studies would be helpful in creating an updated normogram using LCMS specifically for the neonatal period, as confirmatory screening of CAH will become more common with the rise in parental prenatal carrier screening.

Unique minimally invasive management of cervicovaginal agenesis

A 10-year-old premenarchal girl presented with worsening abdominal pain for 3 months. She had undergone laparotomy twice at 1 and 9 years of age for urogenital sinus and subacute intestinal...

Study of Expression of P53 in Prostatic Lesions

Prostate gland is an accessory gland of the male reproductive system.Prostate begins development from the mesenchymal tissue, surrounding the urogenital sinus in the 3 rd month of gestation. Epithelial buds invaginate from the posterior sinus on either side of verumontanum. Concurrently, Wolffian ducts develop into seminal vesicles, epididymis, Vasdeferens and ejaculatory ducts that are stimulated by fetal testosterone. Histologically all malignant lesions encountered were Conventional Adenocarcinoma of Prostate. The commonest pattern seen was acinar followed bycribriform and fused glandularpattern. Conventional Adenocarcinoma forms the most commontype of prostatic carcinoma and they are moderately differentiated byGleason’s microscopicgrading. Serum Prostate Specific Antigen (PSA) levels increases proportionately with advancing clinical stage. Studies have shownthat serial increase in serum PSA is associated with incidence of occult carcinoma.In the present study, the TURP specimen received was ranged in the volume from 8 to 20cc. Every tissue was carefully examined for the presence of yellow and firm or gray hard areas and necrotic areas. After fixation and processing, 4 -5µ sections were cut and Hematoxylin and Eosin staining was done. In the present study all the benign lesions stained negative except for cases were the PSA value is more than >50ng/ml with p53 IHC and in prostate carcinoma positive indicating epithelial marker positive for prostate gland. In coclusio, the IHC expression of p53 is significantly up- regulated in malignant lesions and have importance in prognostic factors and disease survival.

46. Management of Hematocolpos in Lower Vaginal Atresia by Image-Guided Drainage with Interventional Radiology: A Case Series

<h3>Background</h3> Obstructive uterovaginal anomalies such as lower vaginal atresia (LVA), can lead to hematocolpos (HC) and subsequent pain at presentation. Definitive management of long LVA requires vaginoplasty with diligent postoperative dilation to prevent vaginal stenosis. However, many adolescents are not emotionally mature enough to adhere to this. Menstrual suppression and delayed vaginoplasty is recommended, however pain symptoms may require drainage of HC. We present a novel approach to management of symptomatic HC via image-guided percutaneous drainage by Interventional Radiology (IR). <h3>Case</h3> Case 1: 14 yo with small bladder and stress urinary incontinence presented with pelvic pain and was found to have a solitary uterus, 6 cm LVA, and a large HC on MRI. She declined vaginoplasty and opted for IR drainage. Ultrasound (US)-guided transabdominal percutaneous uterine access was obtained and tissue plasminogen activator (tPA) was infused through a drain to thin the HC fluid to allow complete aspiration. Norethindrone acetate (NAT) was initiated for menstrual suppression. Cases 2 and 3: 10 yo presented with abdominal pain and found to have a solitary uterus, 5.3 cm LVA, and a large HC on MRI. Menstrual suppression with NAT failed to control her pain; after counseling, she declined vaginoplasty and opted for IR drainage. US-guided percutaneous access was obtained, tPA infused to thin the HC, and pigtail drain placed to assist with further decompression. Cone beam CT verified the patient's anatomy and confirmed drain position. Drain was removed post-operative day 1. Symptomatic re-accumulation of HC occurred after 15 months of menstrual suppression and a repeat US-guided drainage was performed with complete decompression; GnRH agonist arm implant was placed for long-term menstrual suppression. Case 4: 15 yo with complex history including urogenital sinus, renal transplant, and urinary diversion was admitted to the hospital with pelvic pain. MRI suggested a dilated bicornuate uterus with HC and 6 cm LVA. She was counseled on management options, and elected IR drainage. US-guided, percutaneous drainage of the uterine cavity was performed with complete decompression of HC. Cone beam CT confirmed uterine anatomy after drainage. NAT was initiated for long-term menstrual suppression. Table 1 provides a detailed summary of each case. <h3>Comments</h3> We present a novel approach to management of symptomatic HC from LVA via image-guided percutaneous drainage of HC by IR. This simple outpatient procedure can successfully relieve symptoms and allow for menstrual suppression to serve as a temporizing measure until patients are mature enough to undergo definitive surgery for various obstructive uterovaginal anomalies.

133 The Vulvar Exam: An Essential Tool for the Urologist

Vestibulodynia has beens shown to occur in up to 16% of women over the age of 18 and commonly presents in conjunction with interstitial cystitis (IC). The embryologic origin of the vestibule is the urogenital sinus which explains why these two pathologies are so closely linked. Interestingly, vestibulodynia has been shown to appear concurrently in up to 88% of cases of IC. However, AUA guidelines do not specifically recommend a vulvar exam when evaluating IC. We present the case of a 30 year old woman with severe urinary urgency for years, treated by multiple providers who had significant findings on vulvar exam. She had been diagnosed with IC in the past and had been taking multiple medications and undergone numerous procedures (including urodynamics and bladder botox injection). A thorough vaginal and vestibular exam was conducted in clinic and demonstrated re-creation of her urgency symptoms during palpation of the vestibule.

Topographic Relationships of the Peritoneal Canal of Testudines, Crocodylia, and Aves: Evolutionary Implications

Cloacae and peritoneal canals of species of Testudines, Crocodylia, and Aves were analyzed with the purpose of identifying and describing their morphology and investigating their possible relationship with other cloacal structures. Studies were conducted from dissections and routine histological preparations. The cloaca is located in the pelvic cavity and differs between Testudines and Crocodylia. In the former, the cloaca is divided into three compartments—the coprodeum, urodeum, and proctodeum—without folds separating them. In contrast, in Crocodylia the coprodeum, urodeum, and proctodeum are separated by discrete folds, the coprourodeal and uroproctodeal folds. The visceral layer of the peritoneum forms the peritoneal canal and also differs in Testudines and Crocodylia. In the former, the cranial opening of the peritoneal canal is lateral to the urogenital sinus and the canal is caudally projected into the phallus, laterally followed by the ejaculatory groove until the caudal end of the organ. In Crocodylia, the cranial opening of the peritoneal canal is lateral to the coprodeum and the canal extends caudally until reaching a papilla in the body of the phallus, where it terminates. Histologically, the mesothelium of the peritoneal canal has a simple pavement appearance. In Testudines, regions with a simple cubic epithelium were found, indicating intense cell activity. The lamina propria is characterized by a thickening of the connective tissue, moderately dense with a variable thickness and with a layer of bundles of smooth muscle arranged in different directions. In Caiman yacare, bundles of striated skeletal muscles were found on the loose connective tissue that involves the peritoneal canal. The cloacal and peritoneal fluids have a strong protein similarity. The peritoneal canal reflects functional characteristics related to reproduction. No peritoneal canals were found in birds.

Posterior cloaca: A urogenital rather than anorectal anomaly

'Persistent Cloaca' refers to one of the major groups of anorectal anomalies in the female when a single perineal orifice is located at the normal site of the urethra draining both urogenital and alimentary tracts. However, 'posterior cloaca' is a new term developed by Pena to describe a unique defect in females in which a common urogenital sinus is deviated posteriorly to open into normally located anorectum (type A) or in the perineum slightly anterior to the anus (type B). The study included seven girls diagnosed as posterior cloaca. Their age at presentation ranged from 1 to 108 months (median 12 months). The main presentation was abnormal external genitalia, while two cases underwent colostomy (±vaginostomy) at birth. Surgical reconstruction varied according to the degree of deviation from normal anatomy. Partial urogenital sinus mobilization (PUM) was used to correct minor forms; while at the other end of the spectrum (absent urinary bladder), continent urinary diversion was performed. Due to the high incidence of renal and urological anomalies, regular follow up at paediatric nephrology/urology clinics was advised for affected cases. All seven cases had a common urogenital confluence characteristically deviated posteriorly. The degree of backward deviation of the common urogenital orifice was variable ranging from mild to severe posterior displacement. In six cases (85%), the common urogenital orifice was shifted backwards in the perineum approaching the anal verge (type B). In one case, the common urogenital orifice opened internally into the anorectum (type A). Major urinary tract anomalies were quite common (5 of 7 cases; 71%): absent urinary bladder (2 cases); single kidney (2 cases); crossed ectopic kidney (1 case); hydroureteronephrosis (2 cases). Posterior cloaca is a rare anomaly in the female essentially affecting the lower urogenital tract with a high incidence of associated renal anomalies. Management should be tailored according to the degree of developmental defect.

Delayed presentation of anorectal malformations in a tertiary care hospital in India.

To study delayed presentation of ARMs, management and its effect on surgical and functional complications. It is a retrospective study from March 2015 to March 2020. All the patients satisfying the criteria of delayed ARMs, i.e., presenting 7days after birth were included. Information regarding type of ARM, mode of presentation, time of presentation, associated anomalies, management strategy, postoperative complications and functional outcome was noted. Minimum follow-up period was 6months. Out of 102 patients with ARM, 44 patients presented late. Among the 44 patients, 9 were males and 35 were females. Associated comorbidities observed are low birth weight (n = 9) and preterm (n = 13). Associated anomalies observed were cardiac (n = 18), renal (n = 8), other gastrointestinal (n = 5) and skeletal (n = 1). (1) Male: rectourethral fistula-2 (staged repair), anal stenosis-3 (anoplasty) and anocutaneous fistula-4 (anoplasty). (2) Female: vestibular fistula: 15 (6 primary definitive surgery + 9 staged repair), ectopic anus: 3 (staged repair), anal stenosis: 2 (anoplasty), urogenital sinus: 4 (staged repair), H-type ARM: 8 (staged repair) and persistent cloaca: 3 (staged repair). Primary repair was done in 15 patients (34%), and staged repair was done in 29 patients (65.9%). Anoplasty was done in 9 patients, ASARP (modified tsuchida's procedure) in 8 patients and PSARP in 27 patients. Postoperative complications observed were constipation (n = 21, 47.7%), fecal incontinence (n = 12, 27.27%) with perianal excoriation in 2 patients, anal stenosis (n = 3, 6.8%) and rectal mucosal prolapse (n = 2, 4.5%) CONCLUSION: Delayed presentation of ARMs is not uncommon and is more common in females. Management is almost similar to those who present early. Those who present with chronic constipation and megarectum require staged repair. Complications were more frequent with delayed presentation. Hence, every newborn should have careful examination of perineum and screened for ARM to avoid possible morbidity and mortality.

Errors and сomplications in the treatment of children with anorectal malformations

Purpose. This manuscript aims to introduce errors and complications of diagnosis and treatment in children with anorectal malformations (ARM).&#x0D; Methods. A retrospective analysis of 63 children with ARM treated at a single tertiary Speransky childrens Hospital.&#x0D; Results. The patients ages ranged from 2 mo to 17 y. o. (median, 6 y. o.). The types of ARM included: rectourethral fistula 27%, rectoperineal fistula 17.5%, rectovestibular fistula 15.9%, rectobladderneck fistula 6.3%, no fistula 7.9%, cloaca 11.1%, cloaca with urogenital sinus and disorder of sex development 1.6%, pouch colon 1.6%, rectal stenosis 4.8%, anal duplication 3.2%, and rectovaginal fistula 3.2%. Of these patients, 76% underwent surgery earlier at another hospital (surgical treatment completed), 14% had stomas, and 10% did not have any prior procedures. The historical analysis showed diagnostic errors in 48% of children (untimely diagnosis, incorrect interpretation of the ARM variant, prolonged delay in anorectoplasty). Errors led to emergency procedures or changes in subsequent surgical treatment (further ostomy, excess bowel resection) in 22% of cases. After anorectoplasty (stenosis, mislocated anus/rectum, rectal prolapse), complications were detected in 56% of cases, whereas ostomy complications were observed in 5% of cases. Long-term problems after the surgical treatment (constipation, incontinence, and pseudoincontinence) were evident in 98% of children. Different surgical reconstructive techniques of the sphincter formation had been performed previously in 13% of patients. Moreover, they most often had spinal pathology as the cause of functional disorders. Only half of the childrens parents had information about bowel management, 38% did not follow the recommendations and usually had fecal impaction and pseudoincontinence. 45% of children/parents performed non-effective or irregular enemas and required corrective treatment.&#x0D; Conclusion. It is recommended that Russian pediatric surgeons treat children with ARM, according to Russian pediatric surgeons guidelines consistent with international protocols to avoid errors and complications.

10 Segmental cervical aplasia in a mare with mosaic X-chromosome aneuploidy

Segmental cervical aplasia is a congenital Müllerian abnormality characterised by the complete or partial failure of cervical development resulting from abnormal fusion of the Müllerian ducts to the urogenital sinus. In the present case report, we describe a congenital segmental cervical aplasia in a Colombian creole mare. The mare was presented to the Animal Reproduction Clinic of Universidad Nacional de Colombia for diagnosis because the external orifice of her cervix was not detected when a uterine lavage was attempted as a therapy for detectable uterine fluid accumulation. The mare had a history of 20-day oestrous cycles confirmed by receptivity to a mature stallion and had no history of natural service or artificial insemination. A complete breeding soundness evaluation of the mare including transrectal palpation, ultrasonography, vaginoscopy, endoscopy, transvaginal aspiration of the uterine fluid and cytogenetic analysis, and an oestrous cycle follow-up were performed. Clinical and ultrasonographic evaluation of the genital tract revealed normal-size ovaries with structures suggestive of regular ovarian activity. Ovulation was confirmed by the formation of a corpus haemorrhagicum followed by a mature corpus luteum in diestrus. In addition, granular free-floating fluid material distending the uterus was detected. Upon vaginal examination, the organ ended in a blind bag with a small papilla with no evident external os cervix. Cytology of the uterine fluid obtained by transvaginal aspiration showed predominant neutrophils with diplococcus bacteria and inflammatory cells compatible with inflammatory content. Cytogenetic analysis of 134 metaphase lymphocytes showed that the mare had an abnormal karyotype [64,XX]/[63,XO]/[65,XXX] with a ratio of 45%, 45%, and 10%, respectively. G- and C-banded analysis was conducted for the X chromosome. Mosaicism of the X chromosome was diagnosed, and the observed congenital segmental cervical aplasia was proposed as the clinical consequence of the mosaicism detected. To our knowledge, this is the first case of this reproductive pathology in a Colombian mare with regular ovarian activity and X chromosome aneuploidy in mosaic form. The cause of the persistently contaminated uterine content in this mare was not clear; it is possible that via the systemic or transcervical route, bacterial contamination could have colonized the uterus, resulting in chronic inflammation and fluid accumulation. This case report demonstrates the importance of performing an adequate routine gynaecological examination in mares to determine their reproductive health. In most cases, the diagnosis of congenital pathologies of the cervix is an incidental finding when performing breeding programs or therapeutic strategies for managing uterine inflammatory conditions. Furthermore, cytogenetic analysis is an important complementary tool for clinical reproductive examination, to accurately identify causes of congenital malformations, and to determine additional causes of reproductive failure in mares.

Sonographic presentation acute hematometra and massive hematocolpos in a pubertal girl: A case report

Background: Imperforate hymen is an uncommon obstructive congenital anomaly of the female external reproductive channel due to lack of canalization (lower) vaginal plate between the vaginal and urogenital sinus; with a prevalence rate of less &lt; 0.1%. Hematocolpos / imperforate hymen with heamatometra often presents as a pelvic mass that compresses the urinary bladder causing acute urine retention and pain.&#x0D; Case presentation: A 14-year-old presented with lower abdominal pain for 5 months. Patient physical examination revealed normal secondary sexual characteristics with a thickened than normal hymen. She presented with delayed menarche; a physician earlier prescribed analgesic and anthelmintic medication for pain management. After conservative hymenotomy about 360ml of dark-tan haem (blood) was drained in few minutes. Postoperative recovery was uneventful; normal menstruation occurred after 4 weeks. &#x0D; Conclusion: Diagnosis through physical examination and ultrasound requires vigilance and a high degree of suspicion; virginity sparing is a viable treatment option due to social reasons and religious beliefs.

Feminizing genitoplasty in childhood: aiming for achievable outcomes

BackgroundEarly genital reconstruction may be recommended in cases of congenital adrenal hyperplasia to avoid the stigma that can affect these individuals with variable degrees through different cultures. However, the separation and mobilization of a high vagina has remained challenging with less satisfactory surgical outcomes. Therefore, the value of early vaginal reconstruction has been questioned in favour of delaying the whole repair after puberty. In this report, the author has adopted the third option in-between, which comprises early reconstruction of the external genitalia and delaying the more challenging vaginal reconstruction (if needed) to be performed after puberty.ResultsThe study included ten consecutive cases of CAH who underwent feminizing genitoplasty during the period 2016 through 2019. Their age at operation ranged from 8 to 84 months (mean 31; median 15).In five cases (50%), the technique of limited urogenital sinus mobilization adopted in this report succeeded in bringing the vaginal introitus down to the perineum. Those cases had originally a low vagina. In the rest of cases, labial retraction showed a common but wide urogenital introitus perfectly lined by urogenital mucosa. The outcomes have been considered satisfactory to a great extent for both parents and doctors regarding cosmesis and lack of functional complications (voiding problems). Longer follow up is still needed to assess the sexual function when these girls become sexually active.ConclusionIn cases of congenital adrenal hyperplasia, reduction clitoroplasty combined with partial urogenital sinus mobilization can achieve predictable and satisfactory outcomes. In about 50% of cases, this approach is sufficient to bring the vagina down to the perineum. In the other half of cases with higher vagina, the possibility of satisfactory sinus intercourse can be studied when these girls grow and become sexually active; otherwise, a delayed vaginal reconstruction may turn to be necessary.

Laparoscopic robotic-assisted “keel” bladder neck construction

Robotic technology has gained popularity allowing performance of several complex and difficult reconstructive procedures. This video demonstrates the advantages of the robotic approach for a "keel" bladder neck construction in an obese patient. A 13 year-old-girl (BMI=28) already treated elsewhere for a uro-genital sinus (Total Urogenital Mobilization) has been evaluated for disturbing persistent total urinary incontinence. Cisto-colposcopy revealed an extremely short and tortuous urethra. Bladder neck was wide open and incompetent. Video-Urodynamic evaluation showed a bladder with a capacity of 280mL with low voiding pressures (14-16cmH20) and continuous urinary leakage. MAG3 scan confirmed normal renal function. Thus, the girl underwent a laparoscopic robotic-assisted "keel" bladder neck construction. The patient was discharged on 5th post-operative day without complications. After 4 years she is voiding spontaneously (300cc) without post-void residuals. She has presented a persistent mild stress incontinence successfully treated with subsequent endoscopic bulking agent injection. Robotic access to the bladder neck region is an excellent option with ideal anatomical exposure compared to conventional open surgery, especially in obese patients. The "keel" procedure allows to reconfigure and tubularize a wide and incompetent bladder neck creating a continent funnel without the absolute need of concomitant ureteric reimplantation.

Нарушения половой дифференцировки: состояние проблемы через 15 лет после чикагского консенсуса

It is well known that the nomenclature and classification were changed in 2005 at the international consensus conference on intersex disorders, held in Chicago, where, among others, the following recommendations were proposed: (1) all children should be assigned a gender identity, and this should be done as quickly as possible, taking into account the time required for the examination. (2) all infants with congenital adrenal hyperplasia and 46,XX karyotype, including those with pronounced masculinization, must be raised as women. (3) Surgical treatment should be performed early and in cases of feminizing genitoplasty, clitoral reduction should be performed simultaneously with reconstruction of the urogenital sinus (separation of the vagina and urethra). An analysis of contemporary literature shows that all these theories, proposed 15 years ago at the Chicago meeting, failed to stand the test of time. New nomenclature and classification are constantly being revised. Currently, many groups of patients want to abolish the term «sexual maturity disorders.» Recommendations regarding gender reassignment and appropriate early surgical treatment have been completely ignored in some countries. All this was largely facilitated by the confrontational activities of a number of support groups.

Disorders of sex differentiation: state of the problem 15 years after the Chicago consensus

It is well known that the nomenclature and classification were changed in 2005 at the international consensus conference on intersex disorders, held in Chicago, where, among others, the following recommendations were proposed: (1) all children should be assigned a gender identity, and this should be done as quickly as possible, taking into account the time required for the examination. (2) all infants with congenital adrenal hyperplasia and 46,XX karyotype, including those with pronounced masculinization, must be raised as women. (3) Surgical treatment should be performed early and in cases of feminizing genitoplasty, clitoral reduction should be performed simultaneously with reconstruction of the urogenital sinus (separation of the vagina and urethra). An analysis of contemporary literature shows that all these theories, proposed 15 years ago at the Chicago meeting, failed to stand the test of time. New nomenclature and classification are constantly being revised. Currently, many groups of patients want to abolish the term &laquo;sexual maturity disorders.&raquo; Recommendations regarding gender reassignment and appropriate early surgical treatment have been completely ignored in some countries. All this was largely facilitated by the confrontational activities of a number of support groups.

Primary vaginal calculus in a woman with urogenital sinus anomaly: a case report

BackgroundPrimary vaginal calculus is rare and often misdiagnosed due to its low incidence. The formation of primary vaginal calculus is mainly due to the pooling and stasis of urine within the vagina, and associated with urogenital tract abnormalities.Case presentationWe present a case of a 23-year-old woman with urogenital sinus anomaly who presented with a vaginal calculus. The patient was not suspected of a calculus in the vagina until the patient suffered amenorrhea and dyspareunia. Pelvic computed tomography (CT) and magnetic resonance imaging (MRI) confirmed the patient had urogenital sinus anomaly with vaginal calculus. For the reason, the calculus was removed by surgery, and the reconstruction of vagina and urethra was performed. The postoperative recovery and follow-up were uneventful.ConclusionsAlthough vaginal calculus and urogenital sinus anomaly are extremely rare in literature, the radiologist should be familiar with the imaging appearance of urogenital sinus anomaly, and be aware of the possibility of vaginal calculus.

FLAP METHOD WHEN OPERATING ATRESIAE ANI VE-&#x0D; STIBULARIS

As you know, anomalies in the development of the anal orifice are divided: into complete atresia - when it is completely absent, and into incomplete, when the aperture, being placed in a normal place, is too narrow to pass fecal masses (stenosis), or the intestine opens on some in another place, either in the back wall of the vagina along its length behind the hymen (abouchement retro-hymenal), or in front of it; ex. въ fossa navicularis (abouch. ant-hymenal). In embryonic life), the rectum and urogenital parts open in the urogenital sinus or cloaca, formed from the connection of the gradually deepening genital sulcus with allantois, the opening of the Mllerian canal and rectum, and the intestine is separated from the Mllerian canal by a plate and the fetus crotch tissue.