LETTER TO THE EDITOR
|Year : 2013 | Volume
| Issue : 3 | Page : 128-129
Corkscrew vessels: Is it a predictor of vanishing testis syndrome?
Sanjay N Oak, Sandesh V Parelkar, Prashant B Joshi, Dinesh Mundada, Shishira Shetty, Satish Kapadnis, Beejal V Sanghvi
Department of Pediatric Surgery, King Edward Memorial Hospital, Parel, Mumbai, Maharashtra, India
|Date of Web Publication||3-Aug-2013|
Prashant B Joshi
Department of Pediatric Surgery, King Edward Memorial Hospital, Parel, Mumbai, Maharashtra
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Oak SN, Parelkar SV, Joshi PB, Mundada D, Shetty S, Kapadnis S, Sanghvi BV. Corkscrew vessels: Is it a predictor of vanishing testis syndrome?. J Indian Assoc Pediatr Surg 2013;18:128-9
|How to cite this URL:|
Oak SN, Parelkar SV, Joshi PB, Mundada D, Shetty S, Kapadnis S, Sanghvi BV. Corkscrew vessels: Is it a predictor of vanishing testis syndrome?. J Indian Assoc Pediatr Surg [serial online] 2013 [cited 2020 Dec 2];18:128-9. Available from: https://www.jiaps.com/text.asp?2013/18/3/128/116053
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. 
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. 
| Diagnosis|| |
The testosterone concentration fails to increase after HCG stimulation test. Level of anti-Mόllerian hormone (low) and inhibin (low) helps in diagnosis. 
Diagnostic laparoscopy finding: Absence of testicular tissue with blind ending vas is traditionally used criteria for diagnosis.  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|
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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.
| References|| |
|1.||Melvin MG, Felix AC. Disorders of sex differentiation. In: Wilson J, Foster D, Kronberg H, Laresn P, editors. Williams Textbook of Endocrinology. 9 th ed. Philadelphia: WB Saunders Company; 1998. p. 1303-26. |
|2.||Vinci G, Anjot MN, Trivin C, Lottmann H, Brauner R, McElreavey K. An analysis of the genetic factors involved in testicular descent in a cohort of 14 male patients with anorchia. J Clin Endocrinol Metab 2004;89:6282-5. |
|3.||Van Savage JG. Avoidance of inguinal incision in laparoscopically confirmed vanishing testis syndrome. J Urol 2001;166:1421-4. |