Complete epididymal separation presenting as polyorchidism

Case Report

Complete Epididymal Separation Presenting as Polyorchidism Antonio Alberto Zuppa, MD,1 Lorenzo Nanni, MD,2 Flavio Di Gregorio, MD,3 Federica Visintini, MD,1 Valentina Buonuomo, MD,2 Claudio Pintus, MD,2 1 Division of Neonatology, Department of Pediatrics, Universita` Cattolica del S. Cuore, Policlinico ‘‘A. Gemelli,’’ Rome, Italy 2 Division of Pediatric Surgery, Department of Pediatrics, Universita` Cattolica del S. Cuore, Policlinico ‘‘A. Gemelli,’’ Rome, Italy 3 Department of Radiology, Universita` Cattolica del S. Cuore, Policlinico ‘‘A. Gemelli,’’ Rome, Italy

Received 25 April 2005; accepted 10 October 2005

ABSTRACT: We describe an unusual case of a 21month-old boy with complete separation of the testis and epididymis. Two homogeneous structures were observed in the scrotum of a newborn boy in addition to a third structure. Polyorchidism was suspected, but follow-up sonographic studies showed a decrease in the echogenicity of the left scrotal structure. Surgical exploration revealed the testis and epididymis to be completely separated, with no dupliC 2006 cated testis. Orchidopexy was then performed. V Wiley Periodicals, Inc. J Clin Ultrasound 34:258–260, 2006; Published online in Wiley InterScience (www. interscience.wiley.com). DOI: 10.1002/jcu.20190 Keywords: epididymal separation; polyorchidism; cryptorchidism; ultrasonography

F

usion

abnormalities

are

commonly

en-

countered during orchidopexy for undescended testes. According to Mollaeian et al,1 such anomalies are found in 36% of all cryptorchid testes. Complete separation of the testis and epididymis is an exceedingly rare phenomenon that accounts for 1.2–5% of all fusion abnormalities.1–2 This anomaly is generally visualized during inguinal exploration,3 and no imaging description has yet been reported. To our knowledge, our case represents the first sonographic demonstration of complete epididymal separation.

Correspondence to: L. Nanni ' 2006 Wiley Periodicals, Inc.

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CASE REPORT

Physical examination of an otherwise normal newborn revealed bilateral scrotal testes and a left inguinal swelling that did not transilluminate. Sonographic examination was performed with a multifrequency 7.5–13-MHz linear-array transducer connected to an Aplio scanner (Toshiba, Tokyo, Japan). Two oval, mildly echogenic structures were demonstrated in the scrotum, the longitudinal diameters of which were 17 and 15 mm, respectively, on the right and left side. Both structures were homogeneous, and the left one was surrounded by a small amount of fluid (Figure 1). An additional oval structure with the same echotexture was visualized in the left inguinal region; its longest diameter was 11 mm (Figure 2). No vascular supply was detectable on color Doppler examination in any of the 3 structures. Renal sonography showed a mild distension of the renal collecting system on the left side measuring 6 mm transversely and 5 mm anteroposteriorly. The findings of the scrotal sonographic examination suggested polyorchidism. Follow-up sonographic studies performed at 2 and 8 months of age showed a slight decrease in echogenicity of the left scrotal structure compared with the previous examination. Doppler findings were similar to those obtained at birth. At 21 months of age, surgical exploration was performed. The left testis was found within the external inguinal ring. At this level, the vas diverged from the spermatic cord and reached the epididymis, which was located in the scroJOURNAL OF CLINICAL ULTRASOUND—DOI 10.1002/jcu

EPIDIDYMAL SEPARATION

FIGURE 1. Sonogram of the scrotum obtained at 10 days of life shows an oval mass in each half of the scrotum. L, left hemiscrotum; R, right hemiscrotum.

FIGURE 3. Intraoperative photograph of the left inguinal region shows the testis and the epididymis completely separated. C, spermatic cord; E, epididymis; T, testis; V, vas deferens.

FIGURE 2. Songram of the left inguinal region (I) and left hemiscrotum (S) at 10 days of life shows 2 distinct masses.

tum; the testis and the epididymis were completely separated (Figure 3). No duplicated cord or testis were found. Formal orchidopexy was then performed. DISCUSSION

Polyorchidism is an extremely rare anomaly. Reports in the literature are few, and most of these concern case reports.4–7 The anomaly has been identified preoperatively in adult patients, VOL. 34, NO. 5, JUNE 2006—DOI 10.1002/jcu

and its sonographic and MRI features have been described.8 To the best of our knowledge, the sonographic appearance of complete epididymal separation has not been described. Some authors have stated that the diagnosis of polyorchidism can be made with sonography alone and that surgical exploration is unnecessary to confirm the presence of an accessory testis.5,8 In the present case, sonography proved inadequate to distinguish the scrotal epididymis from the normal contralateral testis. 259

ZUPPA ET AL

Though sonography remains a cornerstone in identifying the testis and disclosing its parenchymal characteristics and dimensions at any age, color Doppler imaging cannot be considered a reliable tool in assessing testicular vascularity in the newborn because of the minimal amount of blood flow in a normal prepubertal testis. Greater anatomic detail may be expected from MRI, but this cannot be considered a routine examination in pediatrics (particularly if a benign lesion is suspected) because of the absolute need of deep sedation or general anesthesia. Because of the barely detectable blood flow of the neonatal testis, the finding of a supernumerary structure in the scrotum or in the groin of a newborn cannot be interpreted as being a testis with any certainty. In conclusion, when polyorchidism is suspected because of the presence of a supernumerary scrotal structure, the possibility of complete epididymal separation should be considered in the differential diagnosis.

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REFERENCES 1. Mollaeian M, Mehrabi V, Elahi B. Significance of epididymal and ductal anomalies associated with undescended testis: study in 652 cases. Urology 1994;43:857. 2. Dean AL, Major JW, Ottenheimer EJ. Failure of fusion of the testis and epididymis. J Urol 1952;68: 754. 3. Emanuel ER, Kirsch AJ, Thall EH, et al. Complete separation of the testis and epididymis. J Pediatr Surg 1997;32:754. 4. Khetan N, Torkington J, Jamison MH. Polyorchidism presenting as retractile testes. BJU Int 1999; 83:524. 5. Mastroeni F, D’Amico A, Barbi E, et al. Polyorchidism: 2 case reports. Arch Ital Urol Androl 1997; 69:319. 6. Spranger R, Gunst M, Ku¨hn M. Polyorchidism: a strange anomaly with unsuspected properties. J Urol 2002;168:198. 7. Thum G. Polyorchidism: case report and review of literature. J Urol 1991;145:370. 8. Chung TJ, Yao WJ. Sonographic features of polyorchidism. J Clin Ultrasound 2002;30:106.

JOURNAL OF CLINICAL ULTRASOUND—DOI 10.1002/jcu