Gonadal Function and Reproductive System Anatomy in Postpubertal Prune-Belly Syndrome Patients.

Area of Science:

• Reproductive Medicine
• Urology
• Genetics

Background:

• Prune Belly Syndrome (PBS) is a rare congenital disorder affecting the urinary and reproductive systems.
• Infertility is a common complication in individuals with PBS, but its precise causes are not fully understood.

Purpose of the Study:

• To investigate the anatomical and functional aspects of the reproductive system in postpubertal patients with Prune Belly Syndrome.
• To identify potential causes of infertility in this cohort.

Main Methods:

• Evaluation of reproductive system anatomy via pelvic MRI and assessment of gonadal function through hormonal profiles and semen analysis.
• Data collected from 15 postpubertal PBS patients regarding orchiopexy history, testicular characteristics, and reproductive organ morphology.

Main Results:

• Most patients had normally positioned testes, but a high prevalence of hypoplastic prostates (66.6%) and absent seminal vesicles (66.6%) was observed.
• Motile spermatozoa were found in 50% of semen samples analyzed, and hormonal profiles were generally normal.
• No vasal abnormalities were detected through MRI.

Conclusions:

• Prune Belly Syndrome patients can exhibit normal sexual hormone levels.
• Abnormalities in the prostate and seminal vesicles are prevalent in PBS patients and likely contribute significantly to their infertility.
• Further research is warranted to fully elucidate the reproductive challenges in PBS.