Eur J Pediatr Surg 2010; 20(1): 53-54
DOI: 10.1055/s-0029-1202853
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Transverse Testicular Ectopia and Persistent Mullerian Duct Syndrome: Video-assisted Treatment

M. Dòmini 1 , T. Gargano 1 , G. Ruggeri 1 , A. Gentili 2 , R. Manuele 1 , M. Lima 1
  • 1Department of Pediatric Surgery, University of Bologna, Bologna, Italy
  • 2S.Orsola-Malpighi, PICU, Bologna, Italy
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Publikationsverlauf

Publikationsdatum:
09. April 2009 (online)

Introduction

Persistent Mullerian duct syndrome (PMDS) is a rare autosomal recessive disorder in which, due to a deficiency of the Mullerian inhibiting substance (MIS), the complete development of the Mullerian duct structures occurs in the presence of a male phenotype and a 46 XY karyotype. In these patients, a uterus, tubes and vagina are often present which open into a normal male prostatic urethra. These female structures are usually found during surgical correction of an inguinal hernia or during a laparoscopic exploration for non palpable testis. Transverse testicular ectopia (TTE) is frequently present.

Here we present the case of a 6-month-old boy with this rare association and its treatment by video-assisted excision of the Mullerian remnant, video-assisted orchidopexy and laparoscopic closure of the inguinal defects.

References

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Correspondence

Prof. M. Lima

Department of Pediatric Surgery

University of Bologna

Bologna

Italy

Telefon: 39/51/636 36 51

Fax: 39/51/636 36 57

eMail: mario.lima@unibo.it