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Persistent Mullerian Duct Syndrome in Adult Men Diagnosed Using Laparoscopy
Images in Clinical Urology Persistent Mullerian Duct Syndrome in Adult Men Diagnosed Using Laparoscopy Omer Demir, Onur Kizer, Volkan Sen, and Adil Ahmet Esen Persistent mullerian duct syndrome is a rare disease that occurs in men with a completely normal phenotype and is characterized by the presence of mullerian duct structures. Diagnostic laparoscopy can reveal an appearance consistent with a uterus, fallopian tubes, or ovotestes. UROLOGY 78: 566, 2011. © 2011 Elsevier Inc.
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ersistent mullerian duct syndrome is commonly diagnosed during the prepubertal or pubertal age.1 A 37-year-old man was admitted to our clinic with complaints of a loss of libido and erectile dysfunction. He had a history of bilateral cryptorchidism and infertility. During his physical examination, the testes could not be palpated in the scrotum or inguinal duct. His secondary sex characteristics were normal. The hormonal evaluation of the patient revealed hypergonadotropic hypogonadism. The magnetic resonance imaging results showed a 12-mm structure that was consistent with undescended testes and located at the level of the left internal iliac vein. Diagnostic laparoscopy revealed an appearance consistent with a uterus, fallopian tubes, or ovotestes (Fig. 1). Subsequently, cystoscopy showed that the verumontanum and prostate lobes were present and the ejaculatory ducts were opening into the verumontanum. The patient’s karyotype analysis was 46XY. Accordingly, we performed laparoscopic gonadectomy to decrease the risk of cancer progression.2 Pathologic examination revealed that the patient’s testes were atrophic. The basal membranes of the seminiferous tubules were thickened, and From the Department of Urology, Dokuz Eylul University School of Medicine, Izmir, Turkey Reprint requests: Omer Demir, M.D., Department of Urology, Dokuz Eylul University School of Medicine, Inciralti, Izmir 35340 Turkey. E-mail: [email protected] Submitted: October 8, 2010; accepted (with revisions): November 15, 2010
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© 2011 Elsevier Inc. All Rights Reserved
Figure 1. Laparoscopic view of uterus and testes.
only Sertoli cells were present in their lumens. Additionally, tubular structures covered with fallopian tube epithelium were found. References 1. Gökce MI, Burgu B, Aydogdu O, et al. Transverse testicular ectopia associated with persistent müllerian duct syndrome: another entity in which magnetic resonance imaging is unreliable. Urology. 2010; 76:1475-1477. 2. Vandersteen DR, Chaumeton AK, Ireland K, et al. Surgical management of persistent mullerian duct syndrome. Urology. 1997;49: 941-945.
0090-4295/11/$36.00 doi:10.1016/j.urology.2010.11.015
P
ersistent mullerian duct syndrome is commonly diagnosed during the prepubertal or pubertal age.1 A 37-year-old man was admitted to our clinic with complaints of a loss of libido and erectile dysfunction. He had a history of bilateral cryptorchidism and infertility. During his physical examination, the testes could not be palpated in the scrotum or inguinal duct. His secondary sex characteristics were normal. The hormonal evaluation of the patient revealed hypergonadotropic hypogonadism. The magnetic resonance imaging results showed a 12-mm structure that was consistent with undescended testes and located at the level of the left internal iliac vein. Diagnostic laparoscopy revealed an appearance consistent with a uterus, fallopian tubes, or ovotestes (Fig. 1). Subsequently, cystoscopy showed that the verumontanum and prostate lobes were present and the ejaculatory ducts were opening into the verumontanum. The patient’s karyotype analysis was 46XY. Accordingly, we performed laparoscopic gonadectomy to decrease the risk of cancer progression.2 Pathologic examination revealed that the patient’s testes were atrophic. The basal membranes of the seminiferous tubules were thickened, and From the Department of Urology, Dokuz Eylul University School of Medicine, Izmir, Turkey Reprint requests: Omer Demir, M.D., Department of Urology, Dokuz Eylul University School of Medicine, Inciralti, Izmir 35340 Turkey. E-mail: [email protected] Submitted: October 8, 2010; accepted (with revisions): November 15, 2010
566
© 2011 Elsevier Inc. All Rights Reserved
Figure 1. Laparoscopic view of uterus and testes.
only Sertoli cells were present in their lumens. Additionally, tubular structures covered with fallopian tube epithelium were found. References 1. Gökce MI, Burgu B, Aydogdu O, et al. Transverse testicular ectopia associated with persistent müllerian duct syndrome: another entity in which magnetic resonance imaging is unreliable. Urology. 2010; 76:1475-1477. 2. Vandersteen DR, Chaumeton AK, Ireland K, et al. Surgical management of persistent mullerian duct syndrome. Urology. 1997;49: 941-945.
0090-4295/11/$36.00 doi:10.1016/j.urology.2010.11.015