A rare case of bilateral Vanishing Testicle Syndrome

Vanishing testicle syndrome (VTS) is due to regression of already formed testes. This could occur at any stage during or after testicular descent due any insult resulting in testicular atrophy. VTS is estimated to affect 1:1250 males. We present a rare case of a 12-year-old boy presenting with absent secondary sexual characteristics and short stature found to have bilateral vanishing testicle syndrome. His testes were impalpable at neonatal examination. At the age of 1.5 years an ultrasound scan detected the right testis in the inguinal ring, measuring 1.5 x 1 cm with features suggestive of ischaemia with likely acute or subacute torsion. The left testes was seen in the inguinal canal already atrophied by that time. Subsequently, on surgical exploration the right testis was found atrophied in the inguinal canal, and was replaced in the right sub-dartose pouch, but left testis could not be identified. Surprisingly, 4 months later laparoscopic exploration failed to identify either of the testes except for bilateral atrophied gubernaculum, indicating testicular regression. At the age of 7 years, magnetic resonance imaging confirmed the absence of either of the testes, with no remnants. On examination his height was 146.5cm, which was between the 25th and 50th percentile for that age, and below his mid-parental height range. He had no androgenic hair growth, testes were impalpable and had a prepubertal penis accounting to tanner stage of 1. He had hypergonadotrophic hypogonadism. Pubertal induction was done with testosterone injections which resulted in height gain and development of secondary sexual characteristics.Introduction: The entity Vanishing testicle syndrome (VTS) was coined by Abryaratne et al. in 1969, and is also referred to as "Testicular regression syndrome" [1][2]. The two essential diagnostic criteria are; visualizing the blind-ending spermatic vessels within the retro-peritoneum or spermatic vessels and vas deferens exiting a closed internal inguinal ring, and Testis not palpated during examination under anaesthesia[2][3]. A small fibrotic nodule or nubbin with dystrophic calcification and haemosiderin deposition is usually found at the end of the spermatic cord, with or without identifiable testicular or para-testicular structures [2][3]. Cryptorchidism is reported in 1 -4.6% full term and 1.1 -45.3% pre-mature male infants [4]. This prevalence of cryptorchidism decreases to 1 % by the age of 1 year [5]. Testes are non-palpable in 10 -20% cases of cryptorchidism, and of these VTS accounts for 35 - 60% [6] VTS occurs in < 5% cases of cryptorchidism [7]. VTS is estimated to affect 1:1250 males [8]. We present a rare case of bilateral VTS in a 12 year old boy who presented with short stature and absent secondary sexual characteristics with unpalpable testes.