Search research articles

ABOUT JoVE

Overview Leadership Blog JoVE Help Center

AUTHORS

Publishing Process Editorial Board Scope & Policies Peer Review FAQ Submit

LIBRARIANS

Testimonials Subscriptions Access Resources Library Advisory Board FAQ

RESEARCH

JoVE Journal Methods Collections JoVE Encyclopedia of Experiments Archive

EDUCATION

JoVE Core JoVE Business JoVE Science Education JoVE Lab Manual Faculty Resource Center Faculty Site

Terms & Conditions of Use

Search research articles

Related Experiment Video

Updated: Jul 9, 2026

Robotic Duodenum-preserving Total Pancreatic Head Resection for Intraductal Papillary Mucinous Neoplasms

Robotic Duodenum-preserving Total Pancreatic Head Resection for Intraductal Papillary Mucinous Neoplasms

Published on: April 17, 2026

A visual pitfall: persistent Müllerian duct syndrome (PMDS).

K A Marcus¹, F J J Halbertsma, J Y Picard

¹Department of Paediatrics, Màxima Medical Centre Veldhoven, The Netherlands. k.marcus@mmc.nl

Acta Paediatrica (Oslo, Norway : 1992)

|December 7, 2007

Summary

Persistent Müllerian Duct Syndrome (PMDS) is a rare condition where individuals have both male and female internal reproductive structures. Early diagnosis through understanding gonadal embryology is crucial for appropriate management and monitoring potential complications.

Related Concept Videos

Mitral Valve Prolapse I: Introduction

Mitral Valve Prolapse I: Introduction

IntroductionThe mitral valve, one of the heart's four valves, regulates blood flow. These valves have flaps that open and close to direct blood properly through the heart and body. During each heartbeat, the flaps open for blood to pass through and seal shut to prevent backflow. Specifically, the mitral valve opens to allow blood flow from the heart's upper left chamber to the lower left chamber. It then closes securely as the lower left chamber contracts to pump blood to the body, preventing...

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by

Same author

Persistent Müllerian duct syndrome due to anti-Müllerian hormone receptor 2 microdeletions: a diagnostic challenge.

Human reproduction (Oxford, England)·2020

Same author

Brain imaging can predict neurodevelopmental outcome of Group B streptococcal meningitis in neonates.

Acta paediatrica (Oslo, Norway : 1992)·2018

Same author

A delayed diagnosis of salt-wasting congenital adrenal hyperplasia.

Clinical endocrinology·2016

Same author

Long-term effects of oxandrolone treatment in childhood on neurocognition, quality of life and social-emotional functioning in young adults with Turner syndrome.

Hormones and behavior·2015

Same author

Safety and efficacy of oxandrolone in growth hormone-treated girls with Turner syndrome: evidence from recent studies and recommendations for use.

Hormone research in paediatrics·2014

Same author

Splice site mutations in GH1 detected in previously (Genetically) undiagnosed families with congenital isolated growth hormone deficiency type II.

Hormone research in paediatrics·2013

Area of Science:

Endocrinology
Reproductive Biology
Medical Genetics

Background:

Persistent Müllerian Duct Syndrome (PMDS) is a rare intersex condition characterized by the presence of Müllerian duct remnants in phenotypically normal males.
It arises from defects in anti-Müllerian hormone (AMH) synthesis or its receptor, crucial for Müllerian duct regression during male sexual development.

Observation:

PMDS can present diagnostic challenges, particularly during surgeries for conditions like cryptorchidism or inguinal hernia, due to the unusual combination of male external genitalia and female internal structures.
Diagnosis relies on identifying Müllerian structures in virilized individuals lacking signs of hypocortisolism or fetal androgen exposure.

Findings:

Karyotyping and gonadal biopsy are essential for confirming the diagnosis of PMDS.

Related Experiment Videos

Last Updated: Jul 9, 2026

Robotic Duodenum-preserving Total Pancreatic Head Resection for Intraductal Papillary Mucinous Neoplasms

Robotic Duodenum-preserving Total Pancreatic Head Resection for Intraductal Papillary Mucinous Neoplasms

Published on: April 17, 2026

The risk of malignant transformation in Müllerian remnants is not well-defined, necessitating careful management.

Implications:

Orchidopexy with lifelong follow-up is recommended for cryptorchidism in PMDS patients due to unclear malignant transformation risks.
Surgical removal of Müllerian remnants may be considered for urologic symptoms, requiring meticulous attention to the ductus deferens.
Fertility prognosis in PMDS patients remains uncertain despite optimal treatment strategies.