True hermaphroditism. A case report with observations on its bizarre presentation.

Area of Science:

• Endocrinology
• Genetics
• Reproductive Medicine

Background:

• XX true hermaphroditism is a rare disorder of sex development characterized by the presence of both ovarian and testicular tissue.
• This case presents an adult XX true hermaphrodite with a male gender identity, exhibiting distinct anatomical variations.

Observation:

• The patient presented with separate perineal vaginal and urethral openings.
• Surgical intervention included total abdominal hysterectomy, vaginectomy, and gonadectomy to facilitate urethroplasty for hypospadias and chordee.
• Prostatic tissue was identified and excised adjacent to the urethra during the procedure.

Findings:

• The case highlights unusual anatomical presentations in adult XX true hermaphroditism.
• Surgical management required addressing multiple congenital anomalies, including hypospadias and chordee.
• The presence of prostatic tissue in this context warrants further investigation.

Implications:

• This case underscores the complexity of managing disorders of sex development.
• Establishing a national registry is crucial for collecting data on rare conditions like XX true hermaphroditism.
• Further studies are needed to understand the full spectrum and long-term outcomes of this condition.