Tri-orchidism: A rare case report

Abstract

Tri-orchidism which means 3 testes in the scrotal cavity, is the most common type of the polyorchidism. It is the rarest anomaly of the urogenital tract, and occurs more commonly on the left side. Superanumerary testes shows scrotal and extra-scrotal presentation and is associated with several clinical features and complications. We report a case of triorchidism in a 29-year-old man who presented with left hemiscrotal pain, later on diagnosed on clinical and sonographic examinations. The management depends on different factors and include conservative treatment with follow up, orchidopexy and orchidectomy. Orchidectomy is advised in suspected case of malignancy.

Introduction

The term polyorchidism means more than 2 testes in either hemiscrotum. It is an extremely rare congenital anomaly of urogenital tract. The most common type is tri-orchidism where 3 testes are present. In literature, less than 200 cases are reported [1]. The first case of triorchidism was reported by Blasius in 1670 at routine autopsy [2]. The exact cause is not known, but some suggest that during the early embryogenesis, the longitudinal or transverse division of the urogenital ridge by peritoneal bands development is the possible mechanism. This entity is incidentally diagnosed during surgical exploration [3].

Tri-orchidism occurs more on the left side, because the left testis is more prone to subdivision due to its larger size and different vascular anatomy as compared to the right testis [3,4]. The extra testis is commonly present in the Scrotum (66%), inguinal canal (23%) or retroperitoneum (9%) [5].

Polyorchidism is associated with several complications including maldescent (40%), inguinal hernia (30%), malignancy (6%), hydrocele (9%), infertility, testicular torsion and varicocele [4].

Management of patients with triorchidism is controversial and depends on multiple factors including the location of the testis, the reproductive potential, size of the testis and age of the patient [6].

Here we report a case of tri-orchidism in a 29-year-old man with imaging features on ultrasound and MRI, who incidentally diagnosed during scrotal ultrasound scan for pain.

Case report

A 29-year-old male presented to urology department of our hospital, who has been complaining of nontraumatic dull pain in left hemiscrotum. On physical examination the left hemiscrotum revealed a tender round lesion superior to the left testis which was firm and mobile, while right testis was normal. There was no evidence of lymphadenopathy or inguinal hernia. He was referred to radiology department for ultrasound. On sonography, the left hemiscrotum demonstrated 2 small sized testicles with homogeneous echo-texture and normal blood flow, each having equal volume of 6 cc and 5.8 cc respectively. They showed shared epididymis and vas deference (Fig. 1). There was no evidence of hydrocele, varicocele or any other pathology. The right testis was normal and larger than both testes in left hemiscrotum. The complementray MRI revealed that the left hemiscrotum containing 2 round to oval shaped structures with signal characteristics identical to normal testicular tissue (Fig. 2). The man was diagnosed with type II polyorchidism and referred back to the urologist, who decided to manage the patient conservatively considering the patient's age and need to maintain the fertility, and advised the patient for routine follow up with instructions to report any changes in size, shape, or pain.

Fig. 1.

Tri-orchidism. Right hemi-scrotal cavity shows a single testis. Left hemi-scrotal cavity shows 2 relatively small sized testes.

Fig. 2.

MRI scrotum, coronal plane demonstrates right hemiscrotal single testis and left hemiscrotal 2 relatively small sized testes.

Discussion

Tri-orchidism is classified as a rare congential anomaly of urogenital tract with less than 200 cases published in the medical literature. It is termed as 3 testes, with supernumerary testis is commonly located in the left hemiscrotum with prevalence of 65% compared to the right hemiscrotum [[7], [8], [9]].

This anomaly is common in men aged 20-40 years and often found in in the scrotum (66%), inguinal canal (23%), and retroperitoneum (9%) [[7], [8], [9]]. In our case the supernumerary testis was located in the left hemiscrotum.

The supernumerary testis is more frequently located in the left hemiscrotum because of size and vascular anatomy differences as compared to the right side. Hassan et al. [10], have reported an exceptional case of triorchidism in the right testis, which can be explained by difference in testicular size.

It is accidently diagnosed during surgical exploration. The patient often presents with nontender lesion [[11], [12], [13], [14]]. It is best diagnosed on ultrasound and MRI, however histological examination remained the gold standard.

The main etiology of this entity is mal development of the primitive genital ridges and the mesonephric duct between the fourth and sixth week of embryological development, with resultant duplication of the horizontal or longitudinal plan and subsequently, polyorchidism [7,8,11,15].

Polyorchidism was first classified by Leung in 1988, who documented the first case in 1985 [15]. It was then modified by Thum into 4 types (Fig. 3) on the basis of anatomical orientation of testis and its outflow ducts. They are as follow,

• •Type I – Supernumerary testis lacking an epididymis or vas, with no attachment to the ipsilateral testicle.
• •Type II – The supernumerary testis shares a common epididymis and vas deferens with the primary testicle.
• •Type III – The supernumerary testis has its own epididymis but shares a common vas deferens with the ipsilateral testicle.
• •Type IV – Complete duplication of testes, epididymis, and vas.

Fig. 3.

Classification of polyorchidism by Leung, by Bril_Laurel-Follah_F.

The most common types which constitute about 90% of the cases are 2 and 3, while type 4 is the rarest.

In our case the patient is classified as type II polyorchidism.

Polyorchidism is associated with several complication including maldescent (40%), inguinal hernia (30%), malignancy (6%), hydrocele (9%), infertility, testicular torsion and varicocele [4]. In our case, no associated pathology was appreciated.

Management of patients with triorchidism is controversial and depends on multiple factors including the location of the testis, the reproductive potential, size of the testis and age of the patient [4,6]. A conservative approach with regular self examinations and imaging is preferred when reproductive function maintenance is crucial. For polyorchidism with an extra scrotal testis, particularly in young patients of reproductive age, orchidopexy followed by observation is recommended. Orchidectomy is advised in cases suspected of malignancy [7,8,11,14].

Conclusion

Tri-orchidism which a rare urogenital anomaly, is diagnosed accidentally during physical examination, surgical exploration or ultrasound scan. The primary, readily available and cost-effective modality for its diagnosis is ultrasound followed by MRI for better evaluation, while histological exam remained the gold standard. This entity should be considered in the differential diagnosis of solid testicular masses. It is associated with multiple complications which should be taken into consideration during management. The management of this anomaly depends on multiple factors such as location, size and anatomy of the testis as well as age of the patient, which should be addressed considering the risk and benefit.

Patient consent

I report a case titled “Tri-orchidism: A rare case report”, for which written informed consent has been taken from the patient.