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Embryology, Anatomy, and Physiology of the Male Reproductive System
Published in Karl H. Pang, Nadir I. Osman, James W.F. Catto, Christopher R. Chapple, Basic Urological Sciences, 2021
Develops from the inferio-medial migration and midline fusion of the genital folds which forms the midline raphe.Failure of fusion leads to the separation of the labioscrotal folds without a median raphe - Appears as a bifid scrotum, often seen with proximal hypospadias.
Surgical treatment of disorders of sexual development
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
Rafael V. Pieretti, Patricia K. Donahoe
Male pseudohermaphroditism occurs in 46,XY genetic males with deficient masculinization of the external genitalia due to insufficient testosterone production, conversion, or inadequate target organ response. Many patients with male pseudohermaphroditism have been raised as males. However, if the female gender is chosen, gonadectomy should be done at the time of perineal reconstruction. The patient with an absent or rudimentary vagina usually requires only a clitoroplasty and labioscrotal reduction. The labioscrotal folds should be partially reduced during the first procedure and dilatation or a substitute vaginoplasty planned for the late adolescent or early adult years. Patients with testicular feminization in whom an introitus is often present may have this dilated with bougies at a later age to form a functional vagina.
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
External female genital development: Without high levels of DHT, the protophallus remains small as the clitoris and the labioscrotal folds remain separated and develop into the labia majora. The urethra remains in the perineal location, and the distal vagina and hymen separate from the urogenital sinus. The labia minora remain separated below the dorsal hood of the clitoris. Distally, the distal urogenital sinus separates into the urethra and distal one-third of the vagina.
Genetic Analysis Reveals Complete Androgen Insensitivity Syndrome in Female Children Surgically Treated for Inguinal Hernia
Published in Journal of Investigative Surgery, 2021
Nurin A. Listyasari, Gorjana Robevska, Ardy Santosa, Aurore Bouty, AZ Juniarto, Jocelyn van den Bergen, Katie L. Ayers, Andrew H. Sinclair, Sultana MH Faradz
Clinical data including medical history, age of initial diagnosis, gender assigned, family history, and consanguinity were obtained. All patients had a physical examination in order to rule out other syndromes associated with genital anomalies. Clinical evaluation by expert clinicians included a detail description of labioscrotal folds, genital tubercle, number and location of the perineal openings and gonads. A blood sample was obtained for karyotyping, hormonal analysis, and DNA extraction. G-banding cytogenetic analysis of peripheral blood lymphocytes was performed for all patients in our laboratory. Serum concentrations of follicle stimulating hormone (FSH), luteinizing hormone (LH), and total testosterone (T) were measured by commercially available immunoassays. Genomic DNA was extracted from peripheral EDTA-blood samples using the salting out method [6].