Skip to main content
SpringerLink
Log in

Does Excision of Mullerian Structures Jeopardize Testicular Growth and Function in Persistent Mullerian Duct Syndrome (PMDS): a Single Center Experience

  • Surgery
  • Published:
SN Comprehensive Clinical Medicine Aims and scope Submit manuscript

Abstract

Persistent Mullerian duct syndrome (PMDS) is a rare form of Disorders of Sexual Development (DSD) characterized by persistence of mullerian structures in a phenotypically and genotypically normal male. The most frequent clinical presentation is that of unilateral or bilateral cryptorchidism. Contrasting opinions exist among clinicians regarding the most suitable surgical intervention in this condition and the potential of fertility later. This study revisits PMDS in terms of varied modes of presentation and the optimum surgical approach to such patients. It, thus, attempts to throw light on the pattern of testicular growth and function within a reasonable follow-up time post-removal of mullerian structures and orchidopexy. The study was conducted in a tertiary health care center in North India. It was a retrospective study carried out by retrieving details of ten patients who had undergone treatment over a span of 9 years (January 2010 to January 2019). The clinical features, family history, surgery undertaken, and follow-up are hereby highlighted along with review of literature. Cryptorchidism was the most common presentation. Orchidopexy with excision of mullerian structures could be done in all except one. Testicular position, volume and vascularity, penile growth, and erection were assessed during subsequent visits at 3, 6, 12, and 24 months. All testes were descended with preserved and normal vascularity. Volumes were in normal range and all experienced good erection. Inference regarding fertility was not possible until conclusion of the study due to ongoing follow-up. Cryptorchidism is the most common presenting feature of PMDS. Familial involvement should be kept in mind. Orchidopexy should be the target surgery. Mullerian structures should be removed ensuring testicular viability. This, when performed carefully, ensures normal testicular maturation. However, long follow-ups will be required for conclusive evidence on fertility.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Öçal G. Current concepts in disorders of sexual development. J Clin Res Pediatr Endocrinol. 2011;3:105–14.

    Article  Google Scholar 

  2. Manjunath BG, Shenoy VG, Raj P. Persistent müllerian duct syndrome: how to deal with the müllerian duct remnants-a review. Indian J Surg. 2010;72:16–9.

    Article  CAS  Google Scholar 

  3. Robboy SJ, Bentley RC, Russell P, Anderson P, Fox H, Wells M. Pathology of abnormal sexual development. Haines and Taylor obstetrical and gynecological pathology. 5th ed. United Kingdom: Churchill Livingstone Elsevier; 2003. p. 1209–32.

  4. Nilson O. Hernia uteri inguinalis beim manne. Acta Chir Scand. 1939;83:231.

    Google Scholar 

  5. Berkmen F. Persistent müllerian duct syndrome with or without transverse testicular ectopia and testis tumours. Br J Urol. 1997;79:122–6.

    Article  CAS  Google Scholar 

  6. Renu D, Rao BG, Ranganath K, Namitha. Persistent Mullerian duct syndrome. Indian J Radiol Imaging. 2010;20:72–4.

    Article  Google Scholar 

  7. Sichani MM, Heidapour M, Dadkhah A, Rezavani M. Persistent Mullerian duct syndrome with an irreducible inguinal hernia. Urol J. 2009;6:298–300.

    Google Scholar 

  8. Clarnette TD, Sugita Y, Hutson JM. Genital anomalies in human and animal models reveal the mechanisms and hormones governing testicular descent. Br J Urol. 1997;79:99–112.

    Article  CAS  Google Scholar 

  9. Rajesh A, Farooq M. A rare case of male pseudohermaphroditism-persistent mullerian duct syndrome with transverse testicular ectopia – case report and review of literature. Int J Surg Case Rep. 2017;37:72–5.

    Article  Google Scholar 

  10. Odi TO, Abdur-Rahaman LO, Nasir AA. Persistent mullerian duct syndrome: a case report and review of literature. Afr J Pediatric Surg. 2010;7:191–3.

    Article  Google Scholar 

  11. Prakash N, Khurana A, Narula B. Persistent Mullerian duct syndrome. Indian J Pathol Microbiol. 2009;52:546–8.

    Article  Google Scholar 

  12. Kaore A, Kaore B. Persistent Mullerian duct syndrome. NJIRM. 2012;3:153–4.

    Google Scholar 

  13. Thiel DD, Erhard MJ. Uterine adenosarcoma in a boy with persistent müllerian duct syndrome: first reported case. J Pediatr Surg. 2005;40:e29–31.

    Article  Google Scholar 

  14. Romero FR, Fucs M, Castro MG, Garcia CR, Fernandes Rde C, Perez MD, et al. Adenocarcinoma of persistent müllerian duct remnants: case report and differential diagnosis. Urology. 2005;66:194–5.

    Article  Google Scholar 

  15. Shinmura Y, Yokoi T, Tsutsui Y. A case of clear cell adenocarcinoma of the müllerian duct in persistent müllerian duct syndrome: the first reported case. Am J Surg Pathol. 2002;26:1231–4.

    Article  Google Scholar 

  16. Vandersteen DR, Chaumeton AK, Ireland K, Tank ES. Surgical management of persistent Müllerian duct syndrome. Urology. 1997;49:941–5.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatric Surgery, Vardhman Mahavir Medical College & Safdarjung Hospital, New Delhi, India

    Goutam Chakraborty, Samir Kant Acharya, Nidhi Sugandhi, Deepak Bagga, Saurav Srivastav & Amit Kumar Jadhav

Authors
  1. Goutam Chakraborty
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Samir Kant Acharya
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Nidhi Sugandhi
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Deepak Bagga
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Saurav Srivastav
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Amit Kumar Jadhav
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

GC, SA, and NS were involved in study conception and design. GC did data acquisition. DB performed the operation and did critical revision. SS and AJ did data analysis and interpretation. GC drafted the manuscript. All authors read and approved the final version of this manuscript.

Corresponding author

Correspondence to Goutam Chakraborty.

Ethics declarations

Conflict of Interest

The authors declare that they have no conflict of interest.

Ethical Approval

All procedures were performed in accordance with the ethical standards of the institutional research committee.

Informed Consent

Informed consent for participation was obtained from parents of all children included in the study.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

This article is part of the Topical Collection on Surgery

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Chakraborty, G., Acharya, S.K., Sugandhi, N. et al. Does Excision of Mullerian Structures Jeopardize Testicular Growth and Function in Persistent Mullerian Duct Syndrome (PMDS): a Single Center Experience. SN Compr. Clin. Med. 2, 956–960 (2020). https://doi.org/10.1007/s42399-020-00336-7

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s42399-020-00336-7

Keywords

Search

Navigation