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[3 new cases of internal male pseudohermaphroditism]
1Service Chirurgie Pédiatrique, Hôpital Sainte-Marthe, Avignon.
Abstract:
The authors report 3 new cases of Male Internal Pseudohermaphrodism. It is a scarce entity. The discovery is fortuous during the cure of hernia or cryptorchidism. The family history has nothing particular. The main problem of this syndrome is its treatment. Our attitude consisted in conserving the mullerian derivatives and treating the hernia or the cryptorchidism. Hysterectomy is not justified for the following reasons; no case of cancerisation of the mullerian derivatives has not yet been reported. The vasa deferens and uterus are in intimate relationship; the dissection is therefore dangerous. We did not perform systematic castration in order to protect fertility. The removal of the testis is performed only when the gland is intraabdominal and undescendable considering the major risk of cancerisation.
Insights
Male Internal Pseudohermaphrodism is a rare condition often discovered incidentally. Treatment focuses on managing associated conditions like hernias and cryptorchidism while preserving Müllerian structures to protect fertility.
Area of Science:
- Endocrinology
- Pediatric Surgery
- Genetics
Background:
- Male Internal Pseudohermaphrodism (MIPH) is a rare disorder of sexual development.
- It is characterized by the presence of testes and male external genitalia with female internal reproductive organs (Müllerian derivatives).
- Diagnosis is often incidental during surgical repair of hernias or cryptorchidism.
Observation:
- The study presents three new cases of MIPH.
- Discovery occurred during routine surgical procedures for hernia or undescended testes.
- No significant family history was noted in these cases.
Findings:
- The primary challenge in MIPH is management and treatment.
- The authors advocate for conservative management, including preserving Müllerian derivatives.
- Surgical interventions focused on hernia repair and cryptorchidism, avoiding hysterectomy due to low cancer risk and surgical complexity.
Implications:
- Conservative management of MIPH, including preserving Müllerian structures, may safeguard fertility.
- Hysterectomy is not routinely indicated in MIPH cases.
- Testicular removal is reserved for intra-abdominal, undescendable testes due to cancer risk.