Gynecologic Issues for Girls with Fanconi’s Anemia Jill Huppert, MD MPH Pediatric and Adolescent Gynecology Cincinnati Children’s Hospital Medical Center August 2012 Objectives Anatomy and Puberty  Normal GYN care  Children (≤ 12 years )  Adolescents (13-21 years)  Women (>22 years)  Fanconi effects  Fanconi effects Chronic illness  Treatments  GYN cancer  Fertility / Premature Menopause  What is Pediatric and Adolescent Gynecology? Specializing in the  Gynecologic and reproductive health care of girls from birth to age 22* * Older if chronic illness http://www.cincinnatichildrens.org/svc/alpha/g/gynecology/ Reproductive Health? Preserving the future!  Prevention of:  Ovarian problems  Sexually transmitted diseases  Cervical and vulvar cancer  Timing of pregnancy  Problems with Anatomy Discovered at birth or puberty  Common:  Hymen variations  Labial assymetry  Rare:  Absent vagina  Absent or malformed uterus  www.youngwomenshealth.org  Hymen variants Affects: 1 in every 500 -1000 girls Uterine abnormalities Normal Didelphus Septate Unicornuate Affects: Bi-cornuate 1 in every 1000-5000 girls FA effects on anatomy Largely unknown:  Few reported cases of anomalies-  unicornuate uterus ? 25% with gonadal problems  Normal Puberty: average Breast buds: avg age 10 (range 8-13)  Vaginal discharge  Labial changes  Armpit and pubic hair: age 8-13  Menses:  Average 2 years after breast buds  Age 12.1 years in US  Height peaks before menses 