Case Report

A Case Report of Transverse Testicular Ectopia with Testicular Microlithiasis Qiqi Lam, BSc (Hons) MSc,1 Gemma Miles, MBBS BSc (Hons),1 Emma Marsdin, MBBS, BSc, MRCS,2 Paul McInerney, MB, ChB, MD (Urol),2 Simon Freeman, MBBS, MRCP1 1 2

Radiology Department, Derriford Hospital, Derriford Road, Plymouth, Devon, PL68DH, United Kingdom Urology Department, Derriford Hospital, Derriford Road, Plymouth, Devon, PL68DH, United Kingdom

Received 13 February 2014; accepted 25 September 2014

ABSTRACT: Transverse testicular ectopia is a rare congenital anomaly in which both testes migrate along the same inguinal canal toward the scrotum. This report describes the case of the rarest form of transverse testicular ectopia in a 23-year-old man with coexisting testicular microlithiasis in one of the two testes as diagnosed on sonography and confirmed on MRI. We are unaware of any previous published reports of this association. Although the etiology of both conditions is debatable, each is believed to be associated with an increased risk of testicular malignancy, and this poses a dilemma for C 2014 Wiley the future management of this patient. V Periodicals, Inc. J Clin Ultrasound 43:520–523, 2015; Published online in Wiley Online Library (wileyonline library.com). DOI: 10.1002/jcu.22254 Keywords: microlithiasis; scrotum; testicular ectopia; ultrasonography

INTRODUCTION

T

ransverse testicular ectopia (TTE) is a rare congenital anomaly with approximately 150 cases reported worldwide to date.1 TTE occurs when there is migration of both testes along the same inguinal canal toward the scrotum and is considered to be an exceptional variant of undescended testis. The most common presentation of TTE is in young children with cryptorchidism and contralateral inguinal hernia. There is no known preference in any ethnic group or for either side. Its detection is mostly incidental during surgical Correspondence to: Q. Lam C 2014 Wiley Periodicals, Inc. V

520

exploration or laparoscopy for a symptomatic hernia, although increasingly, a preoperative diagnosis can be made on sonography (US), CT, MRI, or magnetic resonance venography.1

CASE REPORT

A 23-year-old man was referred by his general practitioner for a scrotal US examination, with the clinical indication of an impalpable right testis in the scrotum or inguinal canal; the patient himself was uncertain whether he had ever had two testes present in the scrotum. The US examination was performed with a Toshiba AplioXG diagnostic US system (model SSA-790A) equipped with a broadband lineararray transducer (PLT-1204BT) with a center frequency of 12 MHz (Toshiba Medical Systems Corporation, Tochigi-ken, Japan). The US examination demonstrated two abutting masses within the left hemiscrotum (Figure 1). An area of acoustic shadowing was demonstrated between the two masses, interpreted as representing an edge (refraction) artifact owing to the rounded shape of the abutting upper and lower poles of the two masses, suggesting that they are separate. The appearance of both masses suggested testicular tissue. The superomedial mass, which measured 1.7 3 1.3 3 1.2 cm, had the appearance of normal testicular parenchyma. The inferolateral mass measured 2.2 3 2.0 3 1.7 cm and was shown to contain multiple echogenic nonshadowing foci consistent with the presence of microliths (Figure 2). Only a single epididymis could be identified. The right testis was absent from the right JOURNAL OF CLINICAL ULTRASOUND

TRANSVERSE TESTICULAR ECTOPIA

FIGURE 1. Sonogram of the left hemiscrotum in sagittal section demonstrates two abutting masses within the same hemiscrotum. Note the edge (refraction) artifact between the masses. The apparently lower echogenicity in the inferior (caudal) mass is the result of anterior soft-tissue attenuation and was not seen on other views (eg, Figure 2).

FIGURE 2. Sonogram of the left hemiscrotum in sagittal section demonstrates numerous small nonshadowing echogenic foci in the inferolateral testis consistent with classic testicular microlithiasis.

hemiscrotum. An extension of the US examination to the inguinal regions, abdomen, and pelvis did not demonstrate any abnormality or identify an ectopic right testis. Color Doppler examination demonstrated perfusion of both the left hemiscrotal masses but did not clearly indicate whether they had a common or separate blood supply. These unusual US appearances suggested the possibility of TTE with microlithiasis present in one of the testes. The other diagnoses considered were an extratesticular or epididymal mass in the left hemiscrotum and an ectopic right testis. In view of the US findings, an MRI scan of the abdominal, pelvic, inguinal, and scrotal VOL. 43, NO. 8, OCTOBER 2015

FIGURE 3. Sagittal T2-weighted MRI demonstrates two abutting testes (arrows) within the same hemiscrotum.

regions was undertaken with a Siemens Magnetom Avanto 1.5-T MRI scanner (Siemens Healthcare, Erlangen, Germany). Imaging of the abdomen and pelvis was performed with a phased-array torso coil in 8-mm axial and 4-mm coronal T2-weighted fast-spin (HASTE) echo sequences and 4-mm coronal T1 fast-spin echo sequences. Imaging of the scrotum was performed with high-resolution small field-of-view parameters with 3-mm-slice thickness and T2weighted fast-spin echo sequences in multiple planes and T1 fast-spin echo sequences in the coronal plane. MRI verified the absence of the right testis within the hemiscrotum and the abdominal, pelvic, and inguinal regions (Figure 3). The right gonadal vessels and spermatic cord were not demonstrated. The two abutting masses within the left hemiscrotum were separated by a tunical margin, appearing as two distinct areas of testicular tissue. The microlithiasis identified on the US examination was not visible on the MRI examination. Both kidneys and seminal vesicles were normal morphologically. There was no evidence of any inguinal canal hernia or m€ ullerian-duct structures. The patient declined surgical exploration and, in view of the presence of microlithiasis within one of the two testes, has been taught regular scrotal self-examination and offered annual surveillance scrotal US.

DISCUSSION

TTE is also known as crossed testicular ectopia, transverse aberrant testicular maldescent, 521

LAM ET AL

unilateral double testis, and testicular pseudoduplication. It is a rare condition, occurring in only 1 in 4 million male patients.1 Several investigators have attempted to explain the etiology of TTE. Lockwood2 suggested the multiple-insertion theory, in which TTE results from an anomaly of the gubernaculum, forming multiple attachments between testicular and pelvic anatomy, which abnormally positions the ectopic testis. Hutson3 believes that testicular descent is biphasic, with transabdominal descent to the internal inguinal ring dependent on m€ ullerian-inhibiting substance and inguinoscrotal descent into the scrotal sac dependent on androgens. Clarnette et al4 proposed that testicular descent is dependent on the gubernaculum, cranial suspensory ligament, and hormones, according to Hutson’s biphasic model. Other theories suggest that the unilateral testicular descent is caused by intraabdominal pressure5 or early fusion of the Wolffian ducts.6 The testes may have developed from the same genital ridge if the ductus deferens is fused.7 Separate ductus deferens implies later crossing of the testis.8 More recently, Morris et al9 suggested a link between retroperitoneal lymphatic malformation and TTE. Three types of TTE have been described1: Type I, associated with inguinal hernia alone (50%), Type II, associated with persistent m€ ullerianduct structures (30%), and Type III, associated with abnormalities other than persistent m€ ullerian-duct structures, such as inguinal canal hernia, scrotal abnormalities, genitourinary abnormalities, hypospadias, and pseudohermaphroditism (20%). Our case of TTE is most unusual for several reasons. First, the late presentation in a 23year-old is uncommon. Second, there is no radiologic evidence of an inguinal hernia or persistent m€ ullerian-duct structures to classify this case as type III, the rarest form of TTE. Third, both testes in this case are situated within the same hemiscrotum, meaning there is complete inguinoscrotal descent of the ectopic testis. Finally, the majority of TTE cases reviewed describe the two testes to be similar morphologically. We are unaware of any previously published reports in which testicular microlithiasis (TM) has been associated with TTE.10 TTE is a recognized independent risk factor for testicular malignancy and infertility.11,12 The association between TM and testicular germ-cell tumors (TGCT) is well known to most US practitioners; however, the presence of a 522

causal link between these two conditions is more controversial.13 The presence of TM in patients with features of the testicular dysgenesis syndrome, however, does appear to confer a significantly higher risk for development of TGCT, and many authorities recommend surveillance US for patients with TM and a second risk factor for TGCT.13,14 Surgical management of TTE, depending on the location of the ectopic testis and its vasculature, includes transseptal orchiopexy, extraperitoneal transposition of the testis, herniotomy, and orchiectomy.1 Our patient had the unusual combination of TTE with TM. We are unaware of any previous reports of these two conditions in the same patient. The main limitation of this report is the lack of surgical correlation, because the patient declined surgical exploration, although the results of two different imaging modalities and a clinical examination by an experienced consultant urologist all concur that both testes are situated in the same hemiscrotum. This patient poses some dilemmas for further management, in view of the independent risk of TTE for TGCT and recommendations for US surveillance in patients with TM plus another risk factor for TGCT. We have therefore offered annual scrotal US examinations to this patient and intend to continue this to the age of 55 years, in line with emerging recommendations for managing TM.

REFERENCES 1. Menon A, Uddesh S, Deshkar AM, et al. Transverse testicular ectopia: a case report. J Evol Med Dent Sci 2013;2:7601. 2. Lockwood CB. Development and transition of the testis, normal and abnormal. J Anat Physiol 1888; 22(Pt 4):505. 3. Hutson JM. A biphasic model for the hormonal control of testicular descent. Lancet 1985;24:419. 4. 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. 5. Frey HL, Rajfer J. Role of the gubernaculum and intraabdominal pressure in the process of testicular descent. J Urol 1984;131:574. 6. Gupta RL, Das P. Ectopia testis transversa. J Indian Med Assoc 1960;35:547. 7. Kimura T. Transverse ectopy of the testis with masculine uterus. Ann Surg 1918;68:420. 8. Gray SW, Skandalakis JE. Anomalies of testicular descent. In: Gray SW, Skandalakis JE, editors. Embryology for Surgeons: The Embryological JOURNAL OF CLINICAL ULTRASOUND

TRANSVERSE TESTICULAR ECTOPIA Basis for the Treatment of Congenital Defects. Philadelphia: Saunders;1972, p 588. 9. Morris MW, Cauthen W, Bofill JA, et al. Retroperitoneal lymphatic malformation and transverse testicular ectopia: a unique clinical presentation. J Pediatr Surg 2013;48:e17. 10. Tirman P, Werle D, Tchelepi H. Sonographic and computed tomographic fusion: imaging features of nonseminomatous testicular carcinoma in transverse testicular ectopia. J Ultrasound Med 2012; 31:654. 11. Manassero F, Cuttano MG, Morelli G, et al. Mixed germ cell tumor after bilateral orchidopexy in persistent m€ ullerian duct syndrome with

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transverse testicular ectopia. Urol Int 2004; 73:81. 12. Lip SZL, Murchison LED, Cullis OS, et al. A metaanalysis of the risk of boys with isolated cryptorchidism developing testicular cancer in later life. Arch Dis Child 2012;98:20. 13. Richenberg J, Brejt N. Testicular microlithiasis: is there a need for surveillance in the absence of other risk factors? Eur Radiol 2012;22:2540. 14. Tan IB, Ang KK, Ching BC, et al. Testicular microlithiasis predicts concurrent testicular germ cell tumors and intratubular germ cell neoplasia of unclassified type in adults: a meta-analysis and systematic review. Cancer 2010;116:4520.

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A case report of transverse testicular ectopia with testicular microlithiasis.

Transverse testicular ectopia is a rare congenital anomaly in which both testes migrate along the same inguinal canal toward the scrotum. This report ...
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