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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
Penile agenesis occurs in 1 in 30 million births. It appears to be the result of a development failure of the genital tubercle during the fourth week of embryogenesis. The scrotum appears normal and contains normal testicles (Figure 80.22). Patients can present with an imperforate anus and a rectourethral fistula, with a normal anus and a rectourethral fistula, or with the urethra located in the perineum inside a skin tag resembling a foreskin. Patients are otherwise normal 46,XY males.
Male genital anomalies
Published in Prem Puri, Newborn Surgery, 2017
Rare anomalies of the penis may present at birth, including urethral duplication (Figure 110.3) and megalourethra, which may be associated with prune belly syndrome.20 Partial duplication of the caudal embryo may lead to duplication of the penis, while penile “agenesis” is usually a form of posterior ectopia, with the erectile tissue and urethra buried in the perineal body and the meatus on the anterior lip of the anal canal.29–31 The latter anatomy is similar to the normal situation in marsupials, where the scrotum is inguinal in position and the phallus is in the perineum. Minor variants of penoscrotal transposition are common in DSD patients.32
Penile Agenesis with Urethrorectal Fistula and Vesicoureteral Reflux
Published in Fetal and Pediatric Pathology, 2022
Oumnia Bencharef, Karima Fouraiji, El Ouafi El Aouni Kamili, Mohamed Oulad Saiad
Penile agenesis occurs in the fourth week of embryonic development secondarily to a failure of the development of genital tubercle into penis [1]. Skoog proposed an anatomical classification of penile agenesis depending on the position of the ectopic urethra in relation to the anal sphincter: Post-sphincteric (60%), Pre-sphincteric (28%), like our patient, and urethral atresia (12%) [2].