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Basics

Outline


BASICS

Overview!!navigator!!

  • The clitoris appears larger than normal and may to protrude through the vulvar lips at the ventral commissure
  • It develops from the embryonic genital tubercle in the absence of testicular testosterone production, or the conversion of testosterone by 5α-reductase to the active form, dihydrotestosterone
  • The clitoris is the female homolog of the penis
  • Anatomy
    • Corpus cavernosum clitoris—erectile tissue
    • Corpus clitoris (body of the clitoris)—5 cm in length
    • Crura—attached to the ischial arch
    • Glans clitoris—2.5 cm in diameter; situated in the fossa at the ventral commissure of the vulva; well-developed median sinus and lateral sinuses may be present
  • Systems affected—reproductive
  • May be associated with sex chromosome abnormalities

Signalment!!navigator!!

  • Females
  • Congenital
  • Concurrent ovarian neoplasia—GTCT
  • Iatrogenic drug administration—anabolic steroid, progestin
  • Intersex condition

Signs!!navigator!!

  • Historical—known drug administration; female offspring of treated mare
  • Enlargement of the glans clitoris beyond the expected norm
  • May be visible externally as swelling of the ventral vulvar commissure
  • May protrude from the clitoral fossa between labia
  • May be associated with abnormal cyclicity
  • May be associated with other structural genital anomalies, internal and/or external
  • May be associated with stallion-like behavior and conformation

Causes and Risk Factors!!navigator!!

  • Administration of anabolic steroids
  • Progestin (altrenogest) usage for estrus control, behavior modification, and pregnancy maintenance; female progeny have associated altered gonadotropin secretion and increased clitoral size to 21 months of age; no effect on reproductive function
  • Aberrant endogenous sex-steroid production—granulosa cell tumor in dam during gestation alters fetal development
  • Hormonally active ovarian neoplasm in a postpubertal female—GTCT
  • Mixoploidy or sex chromosome aberration—presence of testis-determining factor

Diagnosis

Outline


DIAGNOSIS

Differential Diagnosis!!navigator!!

  • Intersex conditions
  • Pseudohermaphrodite—may be associated with clitoral enlargement
  • Hypospadias penis
  • Hypoplastic penis with incomplete closure of embryonic urethral folds
  • Associated with prominent perineal median raphe, ventrally displaced vulva, and caudad direction of penis
  • Most common presentation—64,XX male

CBC/Biochemistry/Urinalysis!!navigator!!

N/A

Other Laboratory Tests!!navigator!!

Hormonal Assay

  • Testosterone/hCG challenge—baseline blood sample; administer 3000 IU hCG IV, with additional blood samples collected at 3 and 24 h; increased testosterone indicates testicular tissue is present, i.e. Leydig cell production
  • Estrone sulfate—produced by Sertoli cells in the testicle; couple with hCG challenge to improve diagnostic accuracy
  • Investigate (rule out presence of) a GTCT by measurement of inhibin (sensitivity 80%), anti-Müllerian hormone (98%), and testosterone (48%)

Immunology

  • Test for presence of 5α-reductase or cytosolic receptor
  • Use labial skin only, as the receptors are site specific

Imaging!!navigator!!

  • Ultrasonography coupled with transrectal palpation of internal genitalia for ovarian pathology or internal genital anomaly
  • Note there is no pathognomonic appearance of a GTCT

Other Diagnostic Procedures!!navigator!!

N/A

Pathologic Findings!!navigator!!

N/A

Treatment

TREATMENT

N/A

Medications

Outline


MEDICATIONS

Drug(s) of Choice!!navigator!!

N/A

Contraindications/Possible Interactions!!navigator!!

N/A

Follow-up

Outline


FOLLOW-UP

Patient Monitoring!!navigator!!

N/A

Prevention/Avoidance!!navigator!!

  • Rational causative drug use
  • If genetic, analysis of pedigree
  • If heritable, elimination of parent stock from breeding pool

Possible Complications!!navigator!!

N/A

Expected Course and Prognosis!!navigator!!

N/A

Miscellaneous

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MISCELLANEOUS

Associated Conditions!!navigator!!

Intersex conditions.

Age-Related Factors!!navigator!!

Congenital

Zoonotic Potential!!navigator!!

N/A

Pregnancy/Fertility/Breeding!!navigator!!

Associated abnormalities may preclude fertility.

Abbreviations!!navigator!!

  • GTCT = granulosa–theca cell tumor
  • hCG = human chorionic gonadotropin

Suggested Reading

Ball BA, Almeida J, Conley AJ. Determination of serum anti-Müllerian hormone concentrations for the diagnosis of granulosa-cell tumours in mares.Equine Vet J 2013;45:199203.

Christensen BW, Meyer-Wallen VN. Sex determination and differentiation. In: McKinnon AO, Squires EL, Vaala WE, Varner DD, eds. Equine Reproduction, 2e. Ames, IA: Wiley Blackwell, 2011:22112221.

Lear TL, Villagomez AF. Cytogenetic evaluation of mares and foals. In: McKinnon AO, Squires EL, Vaala WE, Varner DD, eds. Equine Reproduction, 2e. Ames, IA: Wiley Blackwell, 2011:19511962.

Author(s)

Author: Peter R. Morresey

Consulting Editor: Carla L. Carleton