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Delayed Puberty Topics in Adolescent Gynecology

Delayed Puberty Topics in Adolescent Gynecology. Michael Wolfe, M.D. University of Kansas School of Medicine Department of Obstetrics and Gynecology. Objectives. Review normal pubertal development Discuss common etiologies of delayed puberty

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Delayed Puberty Topics in Adolescent Gynecology

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  1. Delayed PubertyTopics in Adolescent Gynecology Michael Wolfe, M.D. University of Kansas School of Medicine Department of Obstetrics and Gynecology

  2. Objectives • Review normal pubertal development • Discuss common etiologies of delayed puberty • Discuss evaluation and management of primary amenorrhea

  3. Normal Puberty • Thelarche • Adrenarche • Growth Spurt • Menarche

  4. Tanner Staging – Breast Development

  5. Tanner Staging – Pubic Hair

  6. Prepubertal Period • 3 changes in the low endocrine state of childhood occur • Adrenarche • Decreased repression of the “gonadostat” • Gradual amplification of peptide-peptide and peptide-steroid interactions leading to gonadarche

  7. What is delayed puberty? • Defined clinically as the absence or incomplete development of secondary sexual characteristics by an age at which 95% of children of that sex and culture have initiated sexual maturation • In the U.S. the National Center for Health Statistics states that the upper 95% for females is age 12, with breast development being the first sign

  8. Etiologies • Hypergonadotropic Hypogonadism (43%) • Ovarian failure with normal or abnormal karyotype • Hypogonadotropic Hypogonadism (31%) • Reversible vs. irreversible • Eugonadism (26%) • Anatomic abnormalities of the target organ or outflow tract

  9. Hypergonadotropic Hypogonadism • Postmenopausal levels of FSH and LH • Gonadal Dysgenesis • 45X • 46XX • 46XY • Mosaicism

  10. Hypogonadotropic HypogonadismReversible • Physiologic delay / Functional Hypothalamic Amenorrhea • Weight loss/anorexia • Primary hypothyroidism • Prolactinoma • Congenital adrenal hyperplasia • Cushing’s syndrome

  11. Hypogonadotropic HypogonadismIrreversible • GnRH deficiency • Hypopituitarism, pituitary adenomas, malignant pituitary tumors • Craniopharyngioma • Congenital CNS defects

  12. Eugonadism • Mullerian Agenesis • Vaginal Septum • Imperforate Hymen • Androgen Insensitivity Syndrome

  13. Review • Classification by gonadotropin levels • Hypergonadotropic hypogonadism • Hypogonadotropic hypogonadism • Eugonadism • Disorders by compartment • Compartment 1: Uterus, outflow tract • Compartment 2: Ovary • Compartment 3: Anterior Pituitary • Compartment 4: CNS, Hypothalamus

  14. Evaluation • History • Physical Exam • Imaging Studies

  15. Initial Labs • Uterus Absent • Karyotype • Serum testosterone • Uterus Present • TSH, Prolactin, Progestational Challenge • FSH, LH, estradiol • Other labs

  16. Progestational Challenge • Positive withdrawal bleeding • Suggests intact outflow tract • Likely due to anovulation • Functional ovary, pituitary, and CNS is confirmed • Negative withdrawal bleeding • Administer hormonal cycle of estrogen with progestational agent • May skip this step if certain of normal uterus

  17. Evaluation after estrogen/progesterone • Measure gonadotropin levels (FSH and LH) • Elevated Gonadotropins • Normal Gonadotropins • Low Gonadotropins

  18. Gonadotropin Levels • Elevated gonadotropins • Normal gonadotropins • Low gonadotropins

  19. Treatment Strategies • Correct underlying pathology • Prevent complications of disease process • Sex steroids

  20. Summary • Normal Puberty • Delayed Puberty • Hypergonadotropic Hypogonadism (elevated FSH, LH) • Hypogonadotropic Hypogonadism (low FSH, LH) • Eugonadism • History and Physical Exam • Work-up: TSH, Prolactin, Progestational Challenge, Estrogen/Progesterone cycle, gonadotropin assays, imaging studies

  21. Resources • Clinical Gynecologic Endocrinology and Infertility.6th ed. Speroff, Leon, et al. Lippincott Williams and Wilkins, 1999. • UpToDate, 2004

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