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Primary AmenorrheaAka: Delayed Menarche

See Also
Definition
1. No menstrual period by:
  1. Sixteen years old or
  2. One year beyond Family History
2. No secondary sexual characteristics by 14 years old
Causes
1. See Hypogonadism
2. Axis 1: Hypothalamus or Central
  1. Anovulation (10%)
  2. Post-hormonal contraceptive Amenorrhea
  3. Constitutional (6%): Family History
  4. Kallmann's Syndrome (Rare): Associated with Anosmia
3. Axis 2: Pituitary
  1. Pituitary Tumor (8%)
    1. Pituitary Adenoma (hormone producing)
    2. Pituitary Null Cell Tumor (No hormone produced)
    3. Empty Sella Syndrome
    4. Pituitary Tuberculosis
    5. Pituitary Schistosomiasis
  2. Hyperprolactinemia (2%)
4. Axis 3: Ovarian
  1. Polycystic Ovary Syndrome (10%)
    1. Findings: Obesity, Hirsutism, and Acne Vulgaris
  2. Turners Syndrome and Mosaics (30%)
    1. Findings: Short Stature, Web neck, and Shield Chest
  3. Genetic Male (10%)
5. Axis 4: Uterus
  1. Mullerian Agenesis (20%)
    1. Mayer-Rokitansky-Kuster-Hauser Syndrome
Primary Amenorrhea Algorithm
1. See Female Tanner Stage for Sexual Development
2. Abnormal Breast development and Female Tanner Stage
  1. Normal Height with low gonadotopins (FSH, LH <5 IU)
    1. See Hypogonadotropic Hypogonadism
    2. Obtain Karyotype (previously Buccal Smear)
      1. Buccal Smear normal: GnRH Deficient
      2. Buccal Smear abnormal: Pure Gonadal dysgenesis
  2. Short Height and excess gonadotropins (FSH>20, LH>40)
    1. See Hypergonadotropic Hypogonadism
    2. Obtain Karyotype (previously Buccal Smear)
      1. Karyotype 46XX: Pituitary Defect
      2. Karyotype 45XO: Turner's Syndrome
3. Normal Breast development and Female Tanner Stage
  1. Uterus Absent on ultrasound
    1. Karyotype (previously Buccal Smear)
    2. Karyotype 46XX: Mullerian Dysgenesis
    3. Karyotype 46XY: Male Pseudo-Hermaphrodite
  2. Uterus Present on ultrasound
    1. Consider obstruction to flow
      1. Imperforate hymen
      2. Transverse vaginal septum
    2. Consider Hyperandrogenism (e.g. Hirsutism)
      1. Polycystic Ovary Syndrome
      2. Adrenal tumor
      3. Ovarian tumor
    3. Consider other causes
      1. See Secondary Amenorrhea
      2. Delayed Puberty
      3. New pituitary Tumor
References

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This page was written by Scott Moses, MD, and was last revised 1/26/2008. Please send comments and questions to the editor. Contact information may be found on the page About the Family Practice Notebook. These pages are best viewed with Microsoft Internet Explorer 5.0.