- Email: [email protected]
Prenatal Testicular Torsion: Not Always in the Late Third Trimester
Pediatric Case Reports Prenatal Testicular Torsion: Not Always in the Late Third Trimester Fanny Sauvestre, Gwenaëlle André, Marie-Hélène Harran, Marie Hemard, Dominique Carles, and Fanny Pelluard Prenatal testicular torsion is a very rare morbid entity, described in the literature to occur when the testicle is intrascrotal, around the 34th week of gestation. Here we report a case of early testicular necrosis. This male fetus was the product of a medical abortion at 27 weeks. During evisceration, a left testicular nubbin free in the peritoneal cavity was found. Histologically, it was extensively necrotic. Because of the location, the size, and the histological features of this necrotic testicle, we conclude that it was the result of torsion of the pedicle that occurred around the 20th week of pregnancy. UROLOGY 89: 132–133, 2016. © 2016 Elsevier Inc.
W
e report a case of a very early testicular necrosis. Because of the location, the size, and the histological features of this necrotic testicle, we hypothesize that it was the result of torsion of the pedicle that occurred around the 20th week of pregnancy. This was a very unusual and a fortuitous finding at the time of a fetopathological examination of a 27-week-old fetus.
into smaller and spindle-shaped juvenile Leydig cells (starting from the 20th week of pregnancy), we hypothesize that torsion occurred around this time. Complete autopsy did not show other visceral anomaly. The spina bifida was associated with an Arnold-Chiari malformation and enlarged cerebral ventricles.
COMMENT CASE REPORT This male fetus was the product of a medical abortion at 27 weeks related to a lumbosacral neural tube defect. The placenta weighed 268 g (expected value: 240 g) and did not showed any gross or histological anomaly. The fetus was not macerated and he weighed 952 g (expected value: 1050 g) for a size of 36.5 cm (expected value: 36 cm). External examination showed a large neural tube defect (35 mm by 25 mm) located in the lumbosacral area. During evisceration, we found the right testicle present in the right hemiscrotum, as expected, but the left one was free in the peritoneal cavity. This testicle and the epididymis were reduced in size related to the term (5 mm in long axis) and uniformly brown colored (Fig. 1). The right testicle was normally colored and seemed to be greater than the expected value (10 mm in long axis). Histologically, the left testicle was extensively necrotic and all structures were present but ghostly, according to a coagulative necrosis. Calcifications were present. There was not any other sign of disturbed testicular development. Because of the presence of ghost fetal Leydig cells that constituted a huge portion of the testis, and their state not yet dedifferentiated Financial Disclosure: The authors declare that they have no relevant financial interests. From the Department of Fœtopathology, CHU de Bordeaux, Bordeaux, France; the Department of Gynecology, Clinique Lafourcade, Bayonne, France; and the Department of Gynecology, CHU de Bordeaux, Bordeaux, France Address correspondence to: Fanny Sauvestre, M.D., Unité de Pathologie Fœtoplacentaire, CHU de Bordeaux, 33076 Bordeaux Cedex, France. E-mail: fanny.sauvestre@ u-bordeaux.fr Submitted: September 9, 2015, accepted (with revisions): November 18, 2015
132
© 2016 Elsevier Inc. All rights reserved.
Intrauterine testicular torsion is a rare event, occurring in 1/17,0001 to 1/80,000 births.2 In our medical center, it is the first case that we have observed on a series of 5590 consecutive fetal and pediatric autopsies of male subjects. All previously reported cases occurred after the 34th week of pregnancy and were diagnosed either by echography or in the neonatal period by the presence of a contralateral hydrocele.1-4 Bilateral forms were described3 but unilaterality is the rule, thus preserving the testicular function. In previous reported cases, the contralateral healthy testis was found normal in size,1,2 or increased.5-7 In our case, we do not have a clear explanation to the increased size of the right testicle, whereas there is neither inflammation nor notable hyperplasia of the Leydig cells. Some authors indicate that this contralateral increase in size could be a possible sign of testicular torsion. This hydrocele may represent a significant compensatory enlargement,6 or it may be the consequence of secondary inflammatory reaction after contralateral torsion or congenital abnormality due to incomplete closure of the processus vaginalis.7 On contrary, Burge thinks that there is no significance for contralateral hydrocele and it may reflect the general incidence in neonatal population.5 Occurring at a late stage of the pregnancy, the twisted testicle is usually intrascrotal, but the intra-abdominal testicular nubbins was present five times in a series of 44 cases reported by Emir et al.8 Burge suggested that torsion could happen before testis migration until the scrotum, and could explain these testicular nubbins.5 Some authors distinguished vanishing testicle that are blind-ended vas deferens or vessels without testicular remnants and testicular nubbins when there are http://dx.doi.org/10.1016/j.urology.2015.11.024 0090-4295
Figure 1. Macroscopic and microscopic features of testicles. (A) Macroscopic photograph of the testicles showing the small necrotic left testicle compared with the large right one. Scale bar 5 mm. (B and C) Microscopic coagulative necrosis with calcifications (HE stain, original magnification ×40 (B) and ×100 (C)). (Color version available online.)
degenerative testicular tissues with seminiferous tubules and hemosiderin deposits.9 This concept leads to two nonexclusive etiologic assumptions: (1) testicular maldevelopment related to an endocrinopathy has been mentioned as an etiology of testicular regression, (2) but evidences seem to be in favor of the antenatal torsion theory, although a twisted spermatic cord is not always found.9 Echographists, pediatricians, as well as fetal and neonatal pathologists have considered that during the examination of internal genitalia, absent testicle in one or both hemiscrotum can be due to testicular agenesis, intraabdominal testicular nubbins, intra-abdominal or intracanalicular testicle (cryptorchidism), or vanishing testicle.9 Necrosis and functional testicular loss are the consequences of a prenatal torsion2 and the management remains controversial. Because the possibility of persistent viable cells and the following risk of malignant degeneration, excision is usually recommended, associated with orchiopexy of the contralateral testis.3,10,11
CONCLUSION An incidental finding of a very early prenatal testicular torsion in the second trimester was made. This affection is a rare event and it is the first case described that has occurred in the second trimester of pregnancy. This observation allows discussion of the fact that prenatal testicular torsion does not always happen in the late third trimester, as usually described.
UROLOGY 89, 2016
Acknowledgment. The authors thank Mike Philcox for his reading and corrections of the manuscript. References 1. Ganni P, Vachhani N, Udayasankar U. Intrauterine testicular torsion. J Urol. 2014;191:217-218. 2. Melcer Y, Mendlovic S, Klin B, et al. Fetal diagnosis of testicular torsion: what shall we tell the parents? Prenat Diagn. 2015;35:167173. 3. Arena F, Nicòtina PA, Scalfari G, et al. A case of bilateral prenatal testicular torsion: ultrasonographic features, histopathological findings and management. J Pediatr Urol. 2005;1:369-372. 4. Devesa R, Muñoz A, Torrents M, et al. Prenatal diagnosis of testicular torsion. Ultrasound Obstet Gynecol. 1998;11:286-288. 5. Burge DM. Neonatal testicular torsion and infarction: aetiology and management. Br J Urol. 1987;59:70-73. 6. Koff SA. Does compensatory testicular enlargement predict monarchism? J Urol. 1991;146:632-633. 7. van der Sluijs JW, den Hollander JC, Lequin MH, et al. Prenatal testicular torsion: diagnosis and natural course. An ultrasonographic study. Eur Radiol. 2004;14:250-255. 8. Emir H, Ayik B, Eliçevik M, et al. Histological evaluation of the testicular nubbins in patients with nonpalpable testicle: assessment of etiology and surgical approach. Pediatr Surg Int. 2007;23:41-44. 9. Mizuno K, Kojima Y, Kamisawa H, et al. Feasible etiology of vanishing testicle regarding disturbance of testicular development: histopathological and immunohistochemical evaluation of testicular nubbins. Int J Urol. 2012;19:450-456. 10. Rozanski TA, Wojno KJ, Bloom DA. The remnant orchiectomy. J Urol. 1996;155:712-714. 11. Storm D, Redden T, Agular M, et al. Histologic evaluation of the testicular remnant associated with the vanishing testes syndrome: is surgical management necessary? Urology. 2007;70:1204-1206.
133
W
e report a case of a very early testicular necrosis. Because of the location, the size, and the histological features of this necrotic testicle, we hypothesize that it was the result of torsion of the pedicle that occurred around the 20th week of pregnancy. This was a very unusual and a fortuitous finding at the time of a fetopathological examination of a 27-week-old fetus.
into smaller and spindle-shaped juvenile Leydig cells (starting from the 20th week of pregnancy), we hypothesize that torsion occurred around this time. Complete autopsy did not show other visceral anomaly. The spina bifida was associated with an Arnold-Chiari malformation and enlarged cerebral ventricles.
COMMENT CASE REPORT This male fetus was the product of a medical abortion at 27 weeks related to a lumbosacral neural tube defect. The placenta weighed 268 g (expected value: 240 g) and did not showed any gross or histological anomaly. The fetus was not macerated and he weighed 952 g (expected value: 1050 g) for a size of 36.5 cm (expected value: 36 cm). External examination showed a large neural tube defect (35 mm by 25 mm) located in the lumbosacral area. During evisceration, we found the right testicle present in the right hemiscrotum, as expected, but the left one was free in the peritoneal cavity. This testicle and the epididymis were reduced in size related to the term (5 mm in long axis) and uniformly brown colored (Fig. 1). The right testicle was normally colored and seemed to be greater than the expected value (10 mm in long axis). Histologically, the left testicle was extensively necrotic and all structures were present but ghostly, according to a coagulative necrosis. Calcifications were present. There was not any other sign of disturbed testicular development. Because of the presence of ghost fetal Leydig cells that constituted a huge portion of the testis, and their state not yet dedifferentiated Financial Disclosure: The authors declare that they have no relevant financial interests. From the Department of Fœtopathology, CHU de Bordeaux, Bordeaux, France; the Department of Gynecology, Clinique Lafourcade, Bayonne, France; and the Department of Gynecology, CHU de Bordeaux, Bordeaux, France Address correspondence to: Fanny Sauvestre, M.D., Unité de Pathologie Fœtoplacentaire, CHU de Bordeaux, 33076 Bordeaux Cedex, France. E-mail: fanny.sauvestre@ u-bordeaux.fr Submitted: September 9, 2015, accepted (with revisions): November 18, 2015
132
© 2016 Elsevier Inc. All rights reserved.
Intrauterine testicular torsion is a rare event, occurring in 1/17,0001 to 1/80,000 births.2 In our medical center, it is the first case that we have observed on a series of 5590 consecutive fetal and pediatric autopsies of male subjects. All previously reported cases occurred after the 34th week of pregnancy and were diagnosed either by echography or in the neonatal period by the presence of a contralateral hydrocele.1-4 Bilateral forms were described3 but unilaterality is the rule, thus preserving the testicular function. In previous reported cases, the contralateral healthy testis was found normal in size,1,2 or increased.5-7 In our case, we do not have a clear explanation to the increased size of the right testicle, whereas there is neither inflammation nor notable hyperplasia of the Leydig cells. Some authors indicate that this contralateral increase in size could be a possible sign of testicular torsion. This hydrocele may represent a significant compensatory enlargement,6 or it may be the consequence of secondary inflammatory reaction after contralateral torsion or congenital abnormality due to incomplete closure of the processus vaginalis.7 On contrary, Burge thinks that there is no significance for contralateral hydrocele and it may reflect the general incidence in neonatal population.5 Occurring at a late stage of the pregnancy, the twisted testicle is usually intrascrotal, but the intra-abdominal testicular nubbins was present five times in a series of 44 cases reported by Emir et al.8 Burge suggested that torsion could happen before testis migration until the scrotum, and could explain these testicular nubbins.5 Some authors distinguished vanishing testicle that are blind-ended vas deferens or vessels without testicular remnants and testicular nubbins when there are http://dx.doi.org/10.1016/j.urology.2015.11.024 0090-4295
Figure 1. Macroscopic and microscopic features of testicles. (A) Macroscopic photograph of the testicles showing the small necrotic left testicle compared with the large right one. Scale bar 5 mm. (B and C) Microscopic coagulative necrosis with calcifications (HE stain, original magnification ×40 (B) and ×100 (C)). (Color version available online.)
degenerative testicular tissues with seminiferous tubules and hemosiderin deposits.9 This concept leads to two nonexclusive etiologic assumptions: (1) testicular maldevelopment related to an endocrinopathy has been mentioned as an etiology of testicular regression, (2) but evidences seem to be in favor of the antenatal torsion theory, although a twisted spermatic cord is not always found.9 Echographists, pediatricians, as well as fetal and neonatal pathologists have considered that during the examination of internal genitalia, absent testicle in one or both hemiscrotum can be due to testicular agenesis, intraabdominal testicular nubbins, intra-abdominal or intracanalicular testicle (cryptorchidism), or vanishing testicle.9 Necrosis and functional testicular loss are the consequences of a prenatal torsion2 and the management remains controversial. Because the possibility of persistent viable cells and the following risk of malignant degeneration, excision is usually recommended, associated with orchiopexy of the contralateral testis.3,10,11
CONCLUSION An incidental finding of a very early prenatal testicular torsion in the second trimester was made. This affection is a rare event and it is the first case described that has occurred in the second trimester of pregnancy. This observation allows discussion of the fact that prenatal testicular torsion does not always happen in the late third trimester, as usually described.
UROLOGY 89, 2016
Acknowledgment. The authors thank Mike Philcox for his reading and corrections of the manuscript. References 1. Ganni P, Vachhani N, Udayasankar U. Intrauterine testicular torsion. J Urol. 2014;191:217-218. 2. Melcer Y, Mendlovic S, Klin B, et al. Fetal diagnosis of testicular torsion: what shall we tell the parents? Prenat Diagn. 2015;35:167173. 3. Arena F, Nicòtina PA, Scalfari G, et al. A case of bilateral prenatal testicular torsion: ultrasonographic features, histopathological findings and management. J Pediatr Urol. 2005;1:369-372. 4. Devesa R, Muñoz A, Torrents M, et al. Prenatal diagnosis of testicular torsion. Ultrasound Obstet Gynecol. 1998;11:286-288. 5. Burge DM. Neonatal testicular torsion and infarction: aetiology and management. Br J Urol. 1987;59:70-73. 6. Koff SA. Does compensatory testicular enlargement predict monarchism? J Urol. 1991;146:632-633. 7. van der Sluijs JW, den Hollander JC, Lequin MH, et al. Prenatal testicular torsion: diagnosis and natural course. An ultrasonographic study. Eur Radiol. 2004;14:250-255. 8. Emir H, Ayik B, Eliçevik M, et al. Histological evaluation of the testicular nubbins in patients with nonpalpable testicle: assessment of etiology and surgical approach. Pediatr Surg Int. 2007;23:41-44. 9. Mizuno K, Kojima Y, Kamisawa H, et al. Feasible etiology of vanishing testicle regarding disturbance of testicular development: histopathological and immunohistochemical evaluation of testicular nubbins. Int J Urol. 2012;19:450-456. 10. Rozanski TA, Wojno KJ, Bloom DA. The remnant orchiectomy. J Urol. 1996;155:712-714. 11. Storm D, Redden T, Agular M, et al. Histologic evaluation of the testicular remnant associated with the vanishing testes syndrome: is surgical management necessary? Urology. 2007;70:1204-1206.
133