Hirschsprung's Disease and Its Impact on Male Reproductive Health

A man with a history of surgery for Hirschsprung's disease was diagnosed with normal FSH and low inhibin B at the age of 28. What is the reason? Do FSH and inhibin B interact with each other? What is the probability of having mature sperm in the testicles? What is the probability of obtaining mature sperm through testicular puncture biopsy?

Hirschsprung's disease is a congenital condition that affects the colon and causes problems with passing stool due to missing nerve cells in the muscles of the colon. While it primarily affects the gastrointestinal system, there can be associated conditions or complications that might impact other systems, including the reproductive system.

FSH and Inhibin B Interaction

Follicle-stimulating hormone (FSH) and inhibin B are part of the feedback loop that regulates spermatogenesis (sperm production) in the testes. FSH is produced by the pituitary gland and stimulates the Sertoli cells in the testes to support sperm production. Inhibin B is produced by the Sertoli cells and acts to inhibit the production of FSH, thus providing a feedback mechanism.

• Normal FSH and Low Inhibin B: In a typical scenario, low inhibin B would lead to an increase in FSH due to reduced negative feedback. However, if FSH is normal despite low inhibin B, it could indicate a dysfunction in the feedback mechanism or a primary testicular issue where the Sertoli cells are not functioning properly.

Probability of Having Mature Sperm in the Testicles

The presence of mature sperm in the testicles can be influenced by several factors, including the underlying condition, the extent of any testicular damage, and the overall health of the reproductive system. In the context of a history of Hirschsprung's disease and the given hormonal profile:

• Normal FSH: Suggests that the pituitary function is intact and that there is some level of spermatogenic activity.
• Low Inhibin B: Indicates that Sertoli cell function may be compromised, which could affect spermatogenesis.

Given these factors, the probability of having mature sperm in the testicles may be reduced but not necessarily absent. The exact probability would depend on the extent of testicular function and any other underlying conditions.

Probability of Obtaining Mature Sperm Through Testicular Puncture Biopsy

Testicular sperm extraction (TESE) or testicular biopsy is a procedure used to retrieve sperm directly from the testicles. The success rate of obtaining mature sperm through this method can vary based on the underlying condition and the health of the testicular tissue.

• In cases of normal FSH and low inhibin B: There is a possibility that mature sperm could still be present in isolated pockets within the testicles, even if overall spermatogenesis is impaired.

The probability of success in obtaining mature sperm through testicular puncture biopsy in this scenario would generally be lower than in men with normal inhibin B levels but is not zero. Success rates can vary widely, but some studies suggest that in cases of non-obstructive azoospermia (where sperm production is impaired), the success rate of finding sperm can range from 30% to 50%.

Conclusion

In summary, the interaction between FSH and inhibin B is crucial for regulating spermatogenesis. In a man with a history of Hirschsprung's disease, normal FSH and low inhibin B suggest a potential issue with Sertoli cell function. The probability of having mature sperm in the testicles is reduced but not necessarily absent, and the probability of obtaining mature sperm through testicular puncture biopsy would be lower than average but still possible. Consulting with a reproductive endocrinologist or a urologist specializing in male infertility would provide more personalized insights and potential treatment options.