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At least 19 recordsLinked to original sources

Total urogenital sinus mobilization: a modified perineal approach for feminizing genitoplasty and urogenital sinus repair.

PURPOSE: We report a modification of the total urogenital sinus mobilization technique adapted to the repair of isolated persistent urogenital sinus and masculinized external female genitalia. MATERIALS AND METHODS: The records of 6 girls undergoing total urogenital sinus mobilization were reviewed. Diagnosis was the adrenogenital syndrome in 4 girls, and persistent urogenital sinus and true hermaphroditism in 1 girl each. Mean patient age at the time of surgery was 5.9 years (range 4.5 months to 19.5 years). The surgical technique was modified by using the perineal approach since all patients had normal anorectal anatomy. A posterior perineal skin flap was used to widen the vaginal introitus. The wall of the mobilized urogenital sinus was opened and used to create a mucous lined vestibule. In those cases of the adrenogenital syndrome reduction clitoroplasty was performed at the same time. Postoperative results were assessed subjectively by observation of the vulvar appearance and objectively by determining the location of the urethral meatus and caliber of the vagina. RESULTS: Mean followup was 3.7 months (range 1 to 9). There were no postoperative complications. Most patients were discharged home within 3 days of surgery. All patients have a satisfactory cosmetic appearance. The urethral meatus was situated in the vestibule and easily accessible. There were no changes in voiding habits postoperatively in those patients who were toilet trained before surgery. Vaginal calibration was performed in 4 patients and mean vaginal caliber was 10.5 Hegar (range 6 to 14). CONCLUSIONS: The repair of persistent urogenital sinus less than 3 cm. long can be accomplished with total urogenital sinus mobilization through the perineal approach. The technique can be combined with reduction clitoroplasty for the surgical management of girls with masculinized external genitalia.

Adolescent↗

[New approach in the surgical treatment of the urogenital sinus].

The urogenital sinus is an embriological anomaly which consists on a common channel from the urethra and vagina. The major incidence is produced in the congenital adrenal hyperplasia's context. In certain occasions it can be associated to an imperforate anus, then the malformation is called a cloacal defect. There are multiple surgical techniques to correct this malformation and different therapeutical approaches (without surgery, surgery at one or various times, early or delayed surgery) being the newest one the total urogenital mobilization. The purpose of this work is to reflect our experience with this technique. We present seven girls with urogenital sinus (3 with congenital adrenal hyperplasia, 2 with a cloacal defect, and the other 2 associated to ambiguous genitalia). Five patients were operated in the first year of life. The outcome has been favorable, and the cosmetic and functional results have been very good. The surgical technique consists on posterior sagittal incision, it can be done transanorectal if necessary, the urethrovaginal union is achieved and both structures are mobilized together, connecting them to the perineum, as a single unit. We believe that the total urogenital mobilization is actually the surgical technique to be chosen in every of urogenital sinus, for being easier, allowing early realization (girls under 1 year old), correcting simultaneously other anomalies, reducing the complications (urethrovaginal fistula, vaginal structure, or acquired vaginal atresia); and the result is excellent.

Abnormalities, Multiple↗

Stromal-epithelial interactions: II. Regulation of prostatic growth by embryonic urogenital sinus mesenchyme.

Embryonic urogenital sinus mesenchyme (UGM) has been demonstrated previously to be a potent inductor of prostatic morphogenesis and functional differentiation when associated with either embryonic urogenital sinus epithelium (UGE) or urothelium derived from adult urinary bladder (ABLE) and grown in male hosts. To determine the role of mesenchyme in prostatic acinar growth, homotypic tissue recombinants of 16-day-old embryonic mouse urogenital sinus mesenchyme and epithelium (UGM mouse + UGE mouse) were prepared in which the relative amounts of UGM to UGE were varied from approximately 0.1:1 to 100:1. Recombinants were grown for 1 month in intact male hosts after which the amount of acinar growth was assessed by histological analysis and by determination of wet weight and DNA content. The latter criteria are valid measures of acinar content of histologically normal prostatic tissue because the rodent prostate is composed of greater than 80% ductal-acinar tissue. From this analysis the amount of acinar growth was found to be determined by the amount of mesenchymal tissue and was independent of the amount of epithelium utilized to prepare the tissue recombinants. Similarly, the magnitude of growth in hetero-specific rat-mouse tissue recombinants prepared with urogenital sinus epithelium or adult bladder epithelium (ABLE) and urogenital sinus mesenchyme (UGM mouse + UGE rat, UGM mouse + ABLE rat, UGM rat + UGE mouse, or UGM rat + ABLE mouse) is determined by the source of UGM. Although the initial DNA content per UGM is comparable between mouse and rat, the overall acinar growth induced by UGM rat is about 10-fold greater than that induced by UGM mouse when recombined reciprocally with UGE mouse and UGE rat, respectively. These results suggest that UGM is of fundamental importance as a regulator of prostatic epithelial growth. The relevance of this finding to the development of human benign prostatic hyperplasia is discussed.

Animals↗

Prostate development requires Sonic hedgehog expressed by the urogenital sinus epithelium.

The prostate gland develops from the urogenital sinus by a testosterone-dependent process of ductal morphogenesis. Sonic hedgehog (Shh) is expressed in the urogenital sinus epithelium and the time course of expression coincides with the formation of the main prostatic ducts. Expression is most abundant in the lumen of the urogenital sinus and in the contiguous proximal duct segments. The initial upregulation of Shh expression in the male urogenital sinus depends on the presence of testosterone. The function of Shh was examined in the male urogenital sinus which was transplanted under the renal capsule of an adult male host mouse. Blockade of Shh function by a neutralizing antibody interferes with Shh signaling and abrogates growth and ductal morphogenesis in the transplanted tissue. These observations show that testosterone-dependent Shh expression in the urogenital sinus is necessary for the initiation of prostate development.

Animals↗

Transanorectal approach for the treatment of urogenital sinus: preliminary report.

The treatment of the urogenital sinus with normal rectum still represents a challenge. A perineal approach with or without a skin flap seems to be effective for those patients with a low implantation of the vagina. However, in patients with a high vaginal implantation, this treatment frequently fails to provide a good, functional vagina due to a narrow, strictured vaginal opening. Based on previous experience in the treatment of more than 80 patients with a persistent cloaca, a posterior sagittal transanorectal approach with a protective colostomy was performed in three patients with urogenital sinus and normal rectum. The pelvis was approached through a midsagittal posterior incision; the coccyx was split and the entire anorectal sphincteric mechanism was divided in the midline. The rectum was bivalved in the midline including both posterior and anterior rectal walls. This provided excellent exposure to the urogenital sinus. The vagina was then fully separated from the urogenital sinus (as described in cases of persistent cloacas), and then mobilized and sutured to the perineum. The rectum and sphincteric mechanism were meticulously reconstructed. A midline incision assures the preservation of anorectal innervation, and provides excellent exposure to the pelvis. Anal dilatations are not necessary to maintain a patent and supple anorectal opening because the rectum has two suture lines, one in front of the other. After the colostomy was closed, all patients had appropriate bowel control for their age; two of them are fully continent for urine and the third one still has a suprapubic cystostomy tube waiting for a repair of an additional urethral malformation.

Anal Canal↗

Two urogenital sinus syndromes. Interstitial cystitis and focal vulvitis.

Forty-six young women had unusual and presumably noninfectious disorders of unknown etiology involving tissues derived from the embryonic urogenital sinus (urogenital sinus syndromes). Ten women had interstitial cystitis, and 25 had focal vulvitis. Eleven women had both interstitial cystitis and focal vulvitis. The affected groups were similar in demographic and other characteristics. Most were white and in their mid-20s. Both unusual conditions occurred in the same woman more often than could be expected by chance. This observation suggests that some common, perhaps autoimmune mechanism may be involved in the etiology of these syndromes.

Adolescent↗

Prospective evaluation of feminizing genitoplasty using partial urogenital sinus mobilization for congenital adrenal hyperplasia.

PURPOSE: We present our experience with partial urogenital sinus mobilization in girls with congenital adrenal hyperplasia, with particular attention to vaginal caliber, introitus position, urinary continence and genital appearance. MATERIALS AND METHODS: We present data on a prospective evaluation of 24 girls with congenital adrenal hyperplasia who underwent feminizing genitoplasty using urogenital sinus mobilization with preservation of the pubourethral ligaments during a 4-year period. Urogenital sinus length determined by cystoscopy and degree of external genitalia virilization, defined according to Prader classification, were evaluated before reconstruction. At followup patients were examined while under sedation for evaluation of overall external genitalia cosmesis and calibration of the vagina. Urinary continence status and voiding efficiency were assessed clinically in toilet trained patients by voiding diary, and measurement of bladder capacity and post-void residual by ultrasound. RESULTS: Patient age at operation ranged from 1 to 16 years (median 28.5 months), with a mean followup of 25 months (8 to 47). Degree of virilization was Prader type III in 3 children (12.5%), type IV in 16 (66.7%) and type V in 5 (20.8%). Urogenital sinus length was 2.5 cm or less in 17 children. Cosmetic results were good in 21 patients (87.5%) and satisfactory in 3 (12.5%). The vaginal and urethral openings were separate and identified at the surface of the vestibule in 21 girls. Adequate caliber of the mobilized vagina was achieved in 23 patients (95.8%). None of the 20 toilet trained children had urinary incontinence or recurrent urinary tract infections, with normal bladder emptying in 18 and small post-void residual in 2 (10%). CONCLUSIONS: Urinary continence and excellent cosmetic appearance with adequate exteriorization of the vaginal and urethral openings can be achieved in most children with urogenital sinus anomaly treated with partial urogenital sinus mobilization.

Adolescent↗

A neuroanatomical comparison of humans and spotted hyena, a natural animal model for common urogenital sinus: clinical reflections on feminizing genitoplasty.

PURPOSE: Surgical treatment of the common urogenital sinus phallus has been one of the most challenging areas in pediatric urology. To better understand the neuroanatomy of the common urogenital sinus phallus, we evaluated an animal model naturally having this condition, the spotted hyena, Crocuta crocuta. We compared the neuroanatomy of male and female humans and spotted hyenas using anatomical, immunohistochemical and 3D reconstruction techniques. We also examined the implications of the pattern of clitoral innervation for the unique challenges faced by female spotted hyenas, the only extant species of mammal that mates and gives birth through the clitoris. MATERIALS AND METHODS: Three adult male and 3 female spotted hyenas were studied. With the animals under anesthesia gross anatomical examination was performed before and after artificial erection. Histological analysis was performed on one 95-day fetal male and female spotted hyena specimens, and on 18 human male and female fetal external genitalia specimens using antibodies raised against the neuronal marker S-100. Three-dimensional computer reconstruction using serial sections allowed analysis of the neuroanatomy of the penis, clitoris and common urogenital sinus of the fetal spotted hyena and human. RESULTS: Compared to other mammals, the clitoris and penis of spotted hyenas were remarkably similar in size and configuration in the flaccid and erect states. Male and female hyenas had a single opening on the tip of the glans penis/clitoris. The basic anatomical structures of the corporeal bodies in both sexes of humans and spotted hyenas were similar. As in humans, the dorsal nerve distribution was unique in being devoid of nerves at the 12 o'clock position in the penis and clitoris of the spotted hyena. Dorsal nerves of the penis/clitoris in humans and male spotted hyenas tracked along both sides of the corporeal body to the corpus spongiosum at the 5 and 7 o'clock positions. The dorsal nerves penetrated the corporeal body and distally the glans in the hyena. In female hyenas the dorsal nerves fanned out laterally on the clitoral body. Glans morphology was different in appearance in both sexes, being wide and blunt in the female and tapered in the male. CONCLUSIONS: The neuroanatomy of the male and female external genitalia in the spotted hyena, Crocuta crocuta, although grossly similar, has distinct anatomical and functional characteristics. The clitoris of the spotted hyena is a classic example of a natural animal model of a common urogenital sinus. The neuroanatomical characteristics of the spotted hyena may be a useful model to simulate the anatomy of common urogenital sinus anomaly in humans.

Animals↗

Surgical management of urogenital sinus abnormalities.

This report describes 12 cases of urogenital sinus malformations and their management in female infants, 10 with imperforate anus. Excluded were cases of urogenital sinus malformation secondary to the adrenogenital syndrome or primary sexual ambiguity. Immediate definition of the anatomy by appropritae endoscopy and radiography is essential to plan the correct operative approach. This can be a relatively simple flap vaginoplasty from below, together with perineal anoplasty in some cases. Others may require an extensive combined abdominal-perineal approach with vaginal pullthrough, ureteral reimplantation, creation of a urethra from the urogenital sinus, resection of vaginal septum, and creation of perineal skin flaps to join to the pulled through vagina which may be too short to reach the perineum.

Adolescent↗

Successful bladder management for a pure urogenital sinus anomaly.

We report on a case of a pure urogenital sinus anomaly presented with bladder distention. A seven-day-old girl with an abdominal distension was referred to the Division of Urology, Hakodate Central Hospital, Hakodate, Japan. A common urogenital sinus without abnormalities in the labium, clitoris or anus was found. A computed tomography (CT) scan documented a distended bladder without hydrometrocolpos or hydroureteronephrosis. Cystography performed at 44 days revealed a large flaccid bladder without ureteral reflux. Urinary management by an indwelling urethral catheter was maintained until 3 months, when an endoscopic examination was performed and a stenotic urethral-type urogenital sinus with low confluence was diagnosed. Parents successfully instituted clean intermittent catheterization as a temporary urinary management, and postvoid residual urine gradually decreased. At 2 years of age, flap vaginoplasty was performed. In an urodynamic study performed postoperatively, the detrusor pressure during voiding was 40-50 cmH(2)O. The patient maintained spontaneous voiding without consequences. Appropriate urinary care is essential to prevent urological complications in cases with a pure urogenital sinus anomaly.

Female↗

The impact of prenatal androgens on vaginal and urogenital sinus development in the female mouse.

PURPOSE: In females abnormal urogenital virilization can occur secondary to prenatal exposure to exogenous or endogenous androgens. We studied the effects of different doses of prenatal androgens on urogenital sinus development and the location of the vaginal confluence in a mouse model. MATERIALS AND METHODS: Timed pregnant C57/6 mice were exposed to 2, 5 and 10 mg testosterone propionate on gestational days 14 through 18. On gestational day 19 the genital tubercles and internal genitalia were examined grossly and histologically for the presence of virilization. Three-dimensional computer reconstruction was done and plastic cast injection molds of the urogenital sinus were made in select specimens. RESULTS: Microscopic analysis confirmed the spectrum of virilization, which occurred in 98% of testosterone propionate treated female fetuses. Plastic cast injection showed that affected females had a longer urogenital sinus, more proximal confluence and shorter vagina in a dose dependent manner. Histological sections and 3-dimensional reconstruction revealed that the bladder neck moved proximal under the pubic bone, also in a dose dependent manner. CONCLUSIONS: Prenatal exposure to increasing levels of androgen causes urogenital sinus elongation in a female mouse fetus. In the mouse model the confluence area moves proximally together with the bladder neck in a dose dependent manner.

Animals↗

Urogenital sinus and cloacal malformations.

Urogenital sinus and cloacal malformations comprise a wide spectrum of abnormal anatomy. This article discusses lessons learned from treating these anomalies for 4 decades. Once the outlook was dim for these children. Today there is a good surgical solution for most of their problems.

Child↗

Morphologic and biochemical alterations in rat prostatic tumors induced by fetal urogenital sinus mesenchyme.

Because fetal urogenital sinus mesenchyme (UGM) has been found to be highly inductive when recombined with normal adult prostate tissues or normal and neoplastic bladder epithelium, we investigated whether fetal UGM also interacts with established hormone-responsive and unresponsive rat Dunning and Nb prostate tumors. Our results indicate that: 1) fetal UGM acts directly on selected rat prostatic tumors by inducing histomorphologic changes (e.g., inducing acinar ductal structures and secretory activity) in the tumors toward more differentiated forms resembling that of the adult prostate gland; 2) fetal UGM either increased the growth rate of or maintained the sizes of three of the four interacting rat prostatic tumors; and 3) fetal UGM markedly reduced the lactate dehydrogenase activity of Nb-autonomous tumor toward a level comparable to that of the normal rat prostate gland. Our data suggest that fetal UGM can directly affect the growth and differentiative functions of selected rat prostatic tumors.

Animals↗

Complications at menarche of urogenital sinus with associated anorectal malformations.

Persistent urogenital sinus is frequently associated with a wide spectrum of complex anatomic abnormalities involving the urinary, genital, and gastrointestinal tracts. Failure to accurately define these abnormalities can result in serious complications. One such group of complications occurs relatively late in the clinical course of these children and has received little attention. These are the complications related to menarche. This report reviews the anatomy, complications, and management in five such patients. In this group the onset of menses was associated with hydrosalpinx, pyosalpinx, hematocolpos, hematometrocolpos tubo-ovarian abscess, ruptured ovarian endometrioma, endometriosis, and peritonitis. Anatomic abnormalities included double and septate vagina, vaginal atresia and stenosis, uterus didelphys, and uterus bicornis unicollis, all predisposing to inadequate menstrual flow. Each of these children required surgical intervention. These cases stress the need for an awareness of not only the urinary but the vaginal and uterine abnormalities. Careful early definition of the anatomy and long-term follow-up of children with urogenital sinus malformation is important in order to avoid potential future complications.

Abnormalities, Multiple↗

A new classification for genital ambiguity and urogenital sinus anomalies.

OBJECTIVE: To describe a new system for classifying genital ambiguity and urogenital sinus abnormalities. Prader's classification (which attempts to describe the degree of masculinization) has been used for decades, while the urogenital sinus has generally been described as simply high or low; neither classification fits the spectrum of anomalies, nor do they allow for outcome data research. METHODS: An ideal description of genital ambiguity and urogenital sinus anomalies should include the degree of masculinization, phallic size, external genital appearance, and the true location of the vaginal confluence in relation to the bladder neck and the perineal meatus. We describe a new system to define these variables. RESULTS: In the PVE classification developed, P represents stretched phallic length and width, V the true vaginal location according to the distance from the bladder neck to the vagina and the distance from the vagina to the perineal meatus, and E the external genital appearance or Prader number. CONCLUSIONS: The PVE classification system aids in surgical planning and will be helpful in the analysis of surgical outcomes of genital ambiguity and urogenital anomalies.

Child↗

Urogenital sinus and anorectal malformation: experience with 22 cases.

Urogenital sinus and anorectal malformation is a complex problem. Anatomy must be defined precisely in each to guide surgical reconstruction, details of which can vary greatly. Although primary reconstruction in the newborn has been accomplished, it may be safer in most to start with decompression colostomy and definition of the anatomy. If the vagina is very distended with urine, it should be drained by some means. Separate abdominal perineal pullthrough of the colon without vaginoplasty should never be performed, because it can cause serious obstructive uropathy when the rectal--urogenital sinus fistula is divided. Ureteral reimplantation is often necessary. Many of these children have undergone urinary diversion; most of those are suitable candidates for "undiversion."

Abnormalities, Multiple↗

[Urogenital sinus and cloacal malformations in infancy and childhood: surgical considerations].

The relative rarity of urogenital sinus and cloacal anomalies, the wide range of their anatomical variants furthermore the number of different surgical options makes the successful management of a child with such urogenital abnormality one of the greatest challenges to the paediatric surgeon. Based on their own experience and the literature the authors give a review of embryology, pathology, and diagnosis of urogenital sinus and cloacal abnormality. They detail the new surgical therapy developed by Hendren and Pena which has been adopted in their routine. This therapy has significantly improved the functional outcome and prognosis of these anomalies. Over the past 20 years 25 patients with urogenital sinus and cloacal abnormalities were surgically treated in the author's institute.

Adolescent↗

Responses of NBT-II bladder carcinoma cells to conditioned medium from normal fetal urogenital sinus.

In vitro studies were conducted to determine whether conditioned medium from rat fetal urogenital sinus explants would affect phenotypic characteristics of NBT-II urinary bladder carcinoma cells in culture. NBT-II cells were exposed to medium (30%, v/v) conditioned for 48 h by intact urogenital sinus explants derived from 18-day fetal rats. Upon exposure for 23 h the [3H]thymidine incorporation by NBT-II cells was decreased by 40.3% relative to control cultures. This effect was paralleled by a similar decrease in proliferation. NBT-II cultures decreased in cell number by 32.1 and 45.8% on days 2 and 4, respectively, after exposure to conditioned medium. Although cell proliferation was inhibited, conditioned medium acted to induce an increase in protein secretion. An increase of 18.6% was observed in the incorporation of [35S]methionine into newly synthesized, secreted proteins by NBT-II cells exposed to conditioned medium for 23 h. Morphologically the NBT-II cells exposed to conditioned medium were larger, more spread out, and exhibited a greater array of lamellipodia and filopodia, although [35S]methionine incorporation into cellular proteins was decreased by 11.1%. These results suggest that diffusable factors produced by fetal urogenital sinus explants can induce changes in proliferation, protein synthesis, protein secretion, and phenotypic morphology of NBT-II carcinoma cells in culture.

Animals↗