Corkscrew vessels: Is it a predictor of vanishing testis syndrome?

Sir,

Vanishing testes syndrome is a rare birth defect in which a baby is born without either of the testicle. Although the specific cause of the condition is unknown, it may be genetic in some cases. Sex organs develop during early fetal life at around 8-9 weeks of gestation. Vanishing testes syndrome or anorchia occurs between 12 and 14 weeks when the testes are lost during the phase of male differentiation. The penis and scrotum forms and continues to develop, but both the testicles are absent.[1]

Although the boy will develop normal external genitalia before the onset of puberty, the symptoms will appear at the time of puberty. Puberty is later than expected and secondary sex characteristics are not present. The penis does not grow as expected, body and pubic hair are not present, and the voice does not deepen as it should during puberty.[1]

DIAGNOSIS

The testosterone concentration fails to increase after HCG stimulation test. Level of anti-Mόllerian hormone (low) and inhibin (low) helps in diagnosis.[2]

Diagnostic laparoscopy finding: Absence of testicular tissue with blind ending vas is traditionally used criteria for diagnosis.[3] However, the presence of corkscrew vessels entering into deep inguinal ring with no testes palpable externally neither visible internally is also observed in vanishing testis syndrome. Thus, according to our institute’s experience of 32 cases of vanishing testis syndrome diagnosed on laparoscopy over a period of 5 years, presence of corkscrew vessels was found in all cases of vanishing testis syndrome [Figure 1].

Figure 1

Laparoscopic view showing corkscrew vessels in vanishing testicular syndrome

We have seen this condition on one side only in some cases, while the other one has been normal. This suggests that there could be an event of intrauterine vascular accident on one side or there could be torsion testis. This event may have lead to desiccation of the vessels, accentuating the corkscrew nature of the vessel.

Presence of corkscrew hypoplastic nature of vessel with vas entering to closed, deep inguinal ring with lymph node at confluence of great vessels are signs to alert the nature of shrivelled gonad like a nubbin of tissue. This should enable the surgeon to take a decision regarding the following:

Proceeding with inguinal exploration or waiting till puberty so that appropriate size of testicular implant can be used

Excision or retention of gonad and fixation of nubbin into the scrotum

Placement of artificial gonad in a planned phase or keeping it ready on the shelf in the theatre

TREATMENT

Hormone supplements, prosthetic testicle implants, and counselling.