How is Tanner scoring used to assess pubertal development in an adolescent or young adult female patient with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome?

Tanner Staging in MRKH Syndrome

Direct Answer

Tanner staging in females with MRKH syndrome is performed identically to the general population, assessing breast development (B1-B5) and pubic hair development (P1-P5), as these secondary sexual characteristics develop normally due to functional ovaries and normal hormonal axis. 1, 2

Understanding MRKH and Normal Pubertal Development

MRKH syndrome affects only the Müllerian structures (uterus and upper 2/3 of vagina) while leaving ovarian function completely intact. 3, 4 This means:

• Patients have normal 46,XX karyotype 3, 5
• Secondary sexual characteristics develop normally 2, 3, 5
• Ovaries are functional and produce normal hormones 2, 5
• External genitalia appear normal 2, 4

Tanner Staging Assessment

Breast Development (Tanner B Stages)

• Assess breast development from B1 (prepubertal) through B5 (adult contour) 1
• Breast development is the first sign of true puberty in females, not pubic hair 1
• Normal breast development typically begins between ages 8-14 years 6
• In MRKH patients, breast development proceeds normally as ovarian estrogen production is unaffected 2, 3

Pubic Hair Development (Tanner P Stages)

• Assess pubic hair from P1 (prepubertal) through P5 (adult distribution) 7, 2
• The case report documents a patient with MRKH at Tanner stage P5 & B4, demonstrating normal progression 2
• Pubic hair before age 8 represents premature pubarche and requires separate evaluation 6

Clinical Application in MRKH

When to Perform Tanner Staging

• Every 6 months until Tanner stage 5 is reached 7
• At every clinical visit to monitor growth and development 7
• During initial evaluation for primary amenorrhea, which is typically the presenting complaint 2, 3, 5

Key Diagnostic Distinction

The critical finding in MRKH is:

• Normal Tanner staging progression (indicating functional ovaries and normal HPG axis) 2, 3
• Combined with primary amenorrhea by age 16 years 6, 2
• This combination distinguishes MRKH from ovarian insufficiency or other causes of delayed puberty 6, 3

Pitfalls to Avoid

Do not confuse isolated pubic or axillary hair (adrenarche) with true pubertal development—breast development (thelarche) is the first sign of HPG axis activation. 1

If a patient with suspected MRKH shows delayed or absent breast development, consider alternative diagnoses such as ovarian insufficiency and measure FSH and estradiol levels. 6

Primary amenorrhea with normal Tanner staging should prompt evaluation for anatomical causes like MRKH, while primary amenorrhea with delayed Tanner staging suggests hormonal causes. 6, 2

Monitoring Schedule

• Document Tanner staging every 6 months during pubertal years 7
• Include height, weight, and growth velocity measurements alongside Tanner staging 1
• For prepubertal girls ≥11 years who fail to initiate puberty, obtain FSH and estradiol levels 6
• No breast development by age 13 with elevated FSH warrants endocrinology consultation 6