Presentation
History of a small penis and hypospadias with recurrent attacks of urinary retention.
Patient Data
Normal CT appearance of the liver, spleen, adrenals, kidneys, and pancreas.
Normal CT appearance of the urinary bladder.
Absent prostate and seminal vesicles.
Small (micro) penis with hypospadias.
Case Discussion
In this case, there's an absence (agenesis) of the prostate and both seminal vesicles. There's a small (micro) penis with small testicles size for the patient's age. Clinically, there's a delayed appearance of the secondary sex characters.
The patient had 46 XY karyotyping results with normal levels of FSH and LH, yet with a high testosterone/DHT (dihydrotestosterone) ratio of 66.2 (Normal range in males: 8-16).
Hence the diagnosis of 5-alpha reductase deficiency was made.
Congenital anomalies of the prostate and seminal vesicles are rare and include agenesis, hypoplasia, ectopia and congenital cysts. Prostatic agenesis can be seen in patients with 5 α-reductase deficiency and testicular feminization. This is usually associated with urogenital organ anomalies, like ambiguous genitalia, micropenis, epispadias, hypospadias, or bladder exstrophy. This could be also associated with prune belly syndrome and posterior urethral obstruction 1.
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