Congenital Adrenal Hyperplasia: Preliminary Observations of the Urethra in 9 Cases

Abstract

Our results suggest that the vagina enters the urogenital sinus at a fixed distance from the bladder neck. With increasing virilization, the majority of urethral lengthening occurs primarily distal to the vaginal opening, that is urogenital sinus lengthening.

Disorders of Sexual Development: Surgical Management

“…We are in agreement with Ganesan that the distance from the bladder neck to the vagina is relatively constant in CAH [ 9 ]. Overall, three elements contribute to an appearance of a higher confl uence: (1) a longer distance from the vagina to the UGS meatus; (2) the virilization of the UGS gives an appearance of a male-like external sphincter, verumontanum, and prostatic-like tissue [ 10 ]; and (3) a more compact, android pelvis.…”

Review of Current Surgical Techniques and Medical Management Considerations in the Treatment of Pediatric Patients with Disorders of Sex Development

“…1c, d). It appears as if the distance from the bladder neck to the vagina is relatively constant [13], while the distance from the vagina to the meatus of the UG sinus changes and lengthens giving the more severely virilized a high confluence appearance [14]. In most patients, the labia majora are anteriorly displaced and the labia minora are absent.…”

A Case of Persistent Urogenital Sinus: Pitfalls and challenges in diagnosis

“…2 While PUGS can present as an isolated anomaly, it has also been associated with other diseases, including congenital adrenal hyperplasia or McKusick-Kaufman syndrome. [3][4][5] The presence of PUGS along with digit anomalies, retinitis pigmentosa, obesity, learning disabilities and male hypogenitalism are phenotypical characteristics of Bardet-Biedl syndrome. 6 In addition, PUGS may also be seen in handfoot-genital syndrome, in which affected males have hypospadias and females have both Müllerian and limb abnormalities.…”