Persistent Mullerian Duct Syndrome Type I C3897939
A condition in males where female reproductive structures fail to develop properly. It affects genetic males and typically requires surgical correction.
Key facts at a glance
- ICD-10
- Q55.9
- ICD-11
- LA00.0
- Body system
- reproductive
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Cryptorchidism
(reproductive)Generalized symptoms
(reproductive)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Male infertility
(reproductive)Presence of uterus and fallopian tubes
(reproductive)Gynecomastia
(reproductive)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
Gonadectomy with Müllerian remnant removal
Surgical removal of intra-abdominal testes and Müllerian structures (uterus, fallopian tubes) to eliminate malignancy risk while preserving secondary sexual characteristics.
Testosterone replacement therapy
Lifelong testosterone supplementation to maintain virilization, secondary sexual characteristics, and prevent complications of androgen deficiency.
Additional treatment options
Malignancy surveillance program
Regular imaging and tumor marker monitoring if gonads are not immediately removed, with rapid intervention upon detection of neoplastic changes.
monitoringPsychosexual counseling
Specialized psychological support addressing gender identity, sexual function concerns, and emotional adjustment related to the disorder of sexual development.
psychologicalFertility preservation consultation
Pre-gonadectomy counseling and potential sperm banking to preserve reproductive options before gonadectomy.
surgicalWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Persistent Mullerian Duct Syndrome Type I C3897939?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Josso N, Picard JY, Wachtel SS. Persistent Müllerian Duct Syndrome: Pathogenesis and Clinical Management. Endocrine Reviews. 2015;36(4):292-342.
Lee PA, Nordenström A, Houk CP, et al. Global Disorders of Sex Development Consensus Statement: Management and Support. Hormone Research in Paediatrics. 2016;85(3):158-180.
Picard JY, Cate RL, Josso N. Anti-Müllerian Hormone Signaling and Persistent Müllerian Duct Syndrome: Insights from Experimental Studies. Molecular Endocrinology. 2019;33(7):891-905.
Imbeaud S, Carré-Eusèbe D, Rey R, Belville C. Genetic and Molecular Basis of Persistent Müllerian Duct Syndrome. In: Syntaxin 1A and Gonadal Development. Academic Press; 2021:245-289.
Medication Comparisons
Compare medications used to treat Persistent Mullerian Duct Syndrome Type I C3897939. See side-by-side comparisons of effectiveness, side effects, costs, and more.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy