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Variation of sex differentiation
Published in Joseph S. Sanfilippo, Eduardo Lara-Torre, Veronica Gomez-Lobo, Sanfilippo's Textbook of Pediatric and Adolescent GynecologySecond Edition, 2019
Anne-Marie Amies Oelschlager, Margarett Shnorhavorian
In the first trimester, the prototypes for both the internal male (wolffian) and female (Müllerian) ducts are present. The wolffian, or mesonephric, ducts connect the capillaries of the mesonephros to the urogenital sinus. The Müllerian, or paramesonephric, ducts are adjacent and connect distally at the urogenital sinus. The distal portion of the urogenital sinus is influenced by external genital development. The internal urogenital sinus will develop into the bladder, trigone, and posterior urethra4 (Figure 7.1).
Disorders of sexual differentiation
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
Sarah M Lambert, Howard M Snyder III
A teenage girl with a unilateral absent kidney and primary amenorrhoea has Mayer– Rokitansky–Küster–Hauser’s syndrome and presents with primary amenorrhoea due to congenital absence of the vagina. If desired, vaginoplasty can be performed to create a neovagina. A child with a 45,XO/46,XY karyotype is at increased risk for gonadoblastoma. Prophylactic removal of the streak gonad is often recommended. A girl with 21-hydroxylase deficiency requires glucocorticoid and mineralocorticoid replacement therapy to prevent further virilisation. The urogenital sinus can be repaired using a partial urogenital mobilisation technique.
Embryology of the Female Urogenital System and Clinical Applications
Published in Linda Cardozo, Staskin David, Textbook of Female Urology and Urogynecology - Two-Volume Set, 2017
The diAgnosis of A urogenitAl sinus is confirmed rAdiogrAphicAlly with A retrogrAde contrAst injection, i.e., A genitogrAm (Figure 22.11A). If the genitogrAm is inconclusive And does not reveAl the level of confluence, A cystoscopy And retrogrAde contrAst study should be performed. Depending on the length of the common chAnnel And the complexity of relAted mAlformAtions, different surgicAl procedures Are AvAilAble. The objectives of the surgicAl repAir Are (1) the sepArAtion of urogenitAl trAct to Allow for normAl voiding And (2) the creAtion of An AdequAte vAginAl introitus. A urogenitAl sinus with A low confluence cAn be mAnAged with A "cutbAck" operAtion combined with An inverted u-flAp to widen the introitus to prevent stenosis [63]. When the common chAnnel exceeds 3 cm, the modified totAl urogenitAl sinus
Persistent Labial Minora Fusion in Reproductive Age Women: A Retrospective Case Series of Nine Patients and Review of Literature
Published in Organogenesis, 2021
Ze Liang, Juan Chen, Xin Yu, Lan Zhu
We found that the septum between the urethra and vagina was significantly thin in all patients, which was related to congenital malformation. At the fifth week of embryonic development, the mesodermal tissue, located between the cloaca and the posterior intestine, grows downward to form a cloacal septum. The cloaca is divided into two parts: the urogenital sinus and the rectum. As the embryo develops, the connection between the two parts of the cloaca becomes narrow and forms a cloacal tube, which is closed at 7th week. If embryonic development stops before the seventh week, different degrees of cloacal malformation can form, which may present as a current thin septum between the urethra and vagina.17–19 Exposure to androgen can retard the descent of the sinus ridge of the female mouse and is sufficient to cause vaginal attachment to the urethra and prevent vaginal opening in the vulva. In addition, the masculinization of the vulva is related to androgen actions.19 The relative position of the urogenital sinus ridge and the Müllerian ducts shifts along the urethra until reaching the vulva. When female mouse embryo was treated with methyltestosterone, the vagina was more closely related to the urethra. Besides androgen intake, CAH can lead to similar clinical symptoms. CAH is a hereditary disease that can cause excessive androgen production in the fetal adrenal gland during the embryonic period, leading to vulvar masculinization in patients with chromosome 46, XX. Therefore, excessive androgen or abnormal activation of androgen receptors by unknown factors may be the etiology of congenital labia minor fusion, and further genetic testing is needed.
Urologic view in the management of genitourinary syndrome of menopause
Published in Climacteric, 2023
To fully understand how the decrease of systemic sex hormone levels at the time of menopause affects urologic function, one must gain knowledge of the embryologic development of the lower urinary tract. The female genitals and lower urinary tract share a common embryologic origin, both arising from the hormone-receptor-rich urogenital sinus. As a result, the trigone, urethra and vaginal vestibule, as well as the vagina, contain a high concentration of estrogen, progesterone and androgen receptors [2–4]. Sex hormone receptors have not only been found within the urinary tract but also within the surrounding and supporting structures including the urinary sphincter, peri-urethral tissues and pelvic floor musculature [5].
An alternative approach to vaginal dilation in patients with Meyer-Rokitanski-Küster-Hauser syndrome: two case reports
Published in Gynecological Endocrinology, 2020
Kátia Crys Moura Ogliari, Salete da Silva Rios, Ana Carolina Rios Chen, Juliana Rios Chen, Ceres Nunes Resende, Edward Araujo Júnior
Surgical techniques, despite their proven effectiveness, are more expensive. Surgical and anesthetic risks can be severe and can impact patient’s quality of life. Some immediate complications are perforation of the rectum and bladder, whereas late complications include stenoses and fistulas. Few studies have compared the results of interventional and conservative management, but dyspareunia and anorgasmia were more likely to result from surgical neovaginoplasty [5]. Surgery is currently indicated only in cases of urogenital sinus malformation, cases of vaginal stenosis with fibrosis, when there is a poor response to the chosen clinical approach, or when the patient refuses conservative treatment [5].