Schlussel HD FEMALE 2007 - Columbia University in … - Urogenital Tract III 3/9/09 1 RICHARD SCHLUSSEL, M.D. DEPARTMENT OF UROLOGY DIVISION OF PEDIATRIC UROLOGY • Normal development

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RICHARD SCHLUSSEL, M.D.

DEPARTMENT OF UROLOGY

DIVISION OF PEDIATRIC UROLOGY

•  Normal development •  Duplication •  Agenesis •  UG Sinus •  Imperforate hymen •  CAH

Genital Development

•  Chromosomal sex

•  Gonadal sex

•  Phenotypic sex

•  FIG alpha –  Follicle cell

differentiation •  Wnt4

–  Anti-testis/Pro-ovarian

–  Knockout in XX causes Testosterone production

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XX males

•  small firm testes •  gynecomastia •  small to normal-sized

penises •  azoospermia

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VAGINAL AND UTERINE DUPLICATION

Bicornuate Uterus

Didelphys uterus Unicornuate Uterus

•  Symptom –  Blood persists despite

tampon during menses

Complete Vaginal Duplication Transverse septum

•  Symptom –  Upper vagina fills

with blood during menses

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Mayer-Rokitansky-Küster-Hauser Syndrome

1 in 5,000 births

VAGINAL AGENSIS

Skin graft sewn over a vaginal stent

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Normal Hymenal Ring

Imperforate hymen

Mucous due to maternal hormones

Intra-op drainage of imperforate hymen UROGENITAL SINUS

UROGENITAL SINUS

X X

CONGENITAL ADRENAL HYPERPLASIA (CAH)

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CAH 46 XX

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•  Association with Urinary Tract Anomalies –  Renal agenesis –  Ectopic ureters

Hypospadias Hypospadias repair

Hypospadias repair

Richard Schlussel M.D. Assistant Professor

Department of Urology Columbia University

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•  Paramesonephric duct – A.K.A. Mullerian Duct – Becomes future

•  Fallopian tubes •  Uterus •  Upper 2/3 vagina