Extracorporeal Testicular Ectopia through Femoral Region: A Rare Variant of Scrotoschisis

ABSTRACT

A 2-year-old male child presented to us with absent left testis in scrotum since birth. Parents noted a pinkish white globular mass in medial aspect of left thigh. At the time of presentation (2 years old) he had a 2 x 2 cm, firm, subcutaneous swelling located on the medial aspect of the left thigh. Ultrasonography was suggestive of left ectopic testis of size 1 x 1.2cm in the femoral region. Orchidopexy was done. In our case the term scrotoschisis will not be appropriate as testicular extrusion has occurred through femoral region and not the scrotum. This can be considered as extracorporeal testicular ectopia or bubonoschisis, respective to the location of the defect. We are reporting a case of extra corporeal testicular ectopia through the femoral region for the first time.

INTRODUCTION

Scrotoschisis is an extremely rare congenital defect in which the testis eviscerates through an opening high on the anterior wall of the scrotum close to the median raphe. Its mechanism remains unclear.[1] We are reporting a case of extracorporeal testicular ectopia through the femoral region for the first time.

CASE REPORT

A 2-year-old male child presented to us with absent left testis in the scrotum since birth. He was a full-term male, born through normal vaginal delivery with uneventful antenatal history. Parents noted a pinkish–white globular mass in medial aspect of the left thigh at birth [Figure 1]. Initially, this patient was managed conservatively, but later on sent to us for surgical management.

Figure 1.

The pinkish–white globular mass (exposed gonad) after birth marked with red arrow

At the time of presentation (2 years old), he had a 2 cm × 2 cm, firm swelling located on the medial aspect of the left thigh in a subcutaneous plane with restricted mobility. The skin at the site was puckered and the swelling was not visible on inspection, was palpable at the site of puckered skin in the left femoral region [Figure 2a]. The scrotum was poorly developed on the left side. The right testis was normal in shape and size and located in the right hemiscrotum. Ultrasonography was suggestive of left ectopic testis of size 1 cm × 1.2 cm in the femoral region. On inguinal exploration, the testis was traced using the spermatic cord structures. It was dissected off from its attachment to subcutaneous tissue, mobilized and orchidopexy was done [Figure 2b]. The postoperative period was uneventful. The child was followed up till 6 months postoperatively. Testis was palpable in the scrotum.

Figure 2.

(a) The site of puckered skin at the left femoral region is marked with yellow arrow. (b) Intraoperative image after mobilization of left ectopic testis

DISCUSSION

Ectopic testis is malposition of the testis outside of the line of its normal descent. It is an uncommon congenital anomaly with reported incidence of 1%. The testis descends through the inguinal canal, but then occupies a final abnormal position. The most common ectopic site is the superficial inguinal pouch, but the other rarer sites include perineum, femoral canal, and contralateral inguinoscrotal region.[1]

In scrotoschisis, the testicular descent is along its normal pathway, but due to a defect in the scrotal skin wall, extrusion of testis occurs. However, in our case, the terminology scrotoschisis will not be appropriate as testicular extrusion has occurred through the femoral region and not the scrotum. This can be considered “Extracorporeal testicular ectopia” or bubonoschisis, respective to the location of the defect.[2]

Around 17 cases of scrotoschisis through the scrotal defect have been reported in literature.[3] Few cases were associated with meconium periorchitis. Most of the cases that have been reported were either bilateral or left sided.[4,5] One case report stated testicular extrusion through inguinal canal. After going through English literature on PubMed and Google Scholar, we could not find any case of congenital testicular extrusion through the femoral region. Extracorporeal testicular extrusion seems to affect otherwise healthy newborn males. Many theories have been proposed for the development of scrotoschisis, but none of them are proven. Some suggest scrotal wall defect is due to failure of differentiation of the mesenchymal layer which results in rupture or avascular necrosis of overlying epithelium. Other theories include meconium periorchitis and external mechanical compression effect. The occurrence of meconium periorchitis is the best theory available till date.[6] There is still no theory to support the mechanism of development of extracorporeal ectopia due to its rarity and limited availability of literature.

Presentation of this condition is usually immediately after birth. This condition is not considered an emergency as long as torsion is avoided by wrapping the extrusion in sterile wet gauze. Testicular torsion has been reported in a case of bilateral scrotoschisis.[4]

All cases reported in literature were neonates within 15 days of life. In our case, the attenders presented to us when the child was 2 years old as an ectopic testis. The parents were advised by local practitioner for a surgical consultation at birth, but due to financial crises, they could not follow-up. The attenders showed an image [Figure 1] of the neonate with testicular extrusion through the left femoral region. We are assuming that the surrounding skin has epithelized over the exposed gonad in due course of time. Probably this case gives us an option of waiting for nature to take its course. In this case, extracorporeal testicular ectopia at birth eventually became ectopic testis. There is no such case that documents or comments on the course of disease as every case reported till date has been surgically managed at the earliest to avoid the risk of torsion.

A routine workup was done for ectopic testis and left open orchidopexy was done. The patient was followed up for 6 months and testis was palpable in the left hemiscrotum.

CONCLUSION

We are presenting this case for its rarity. This is the first case reporting extracorporeal testicular ectopia through the femoral region. Extracorporeal testicular ectopia is a rare congenital anomaly affecting the normal healthy male. The condition is managed with orchidopexy and has good prognosis. The long-term prognosis needs to be studied.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.