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Complete testicular epididymal dissociation in the abdominal cryptorchid testis
Journal of Pediatric Urology (2013) 9, 1023e1027
Complete testicular epididymal dissociation in the abdominal cryptorchid testis Shilpa Sharma*, Amita Sen Department of Pediatric Surgery, Post Graduate Institute of Medical Education and Research, Dr RML Hospital, New Delhi, India Received 11 January 2013; accepted 5 February 2013 Available online 2 June 2013
KEYWORDS Epididymis; Cryptorchidism; Non-fusion; Dissociation
Abstract Aim: To document the incidence of complete testicular epididymal dissociation (CTED) in the cryptorchid testis and evaluate its operative outcome. Methods: The presence of CTED was noted prospectively in cases of undescended testis and the operative findings were studied. Results: CTED was encountered in 11 testes in 10 boys out of 29 intra-abdominal testes among the 142 undescended testes (8%) operated. Ages of patients varied from 18 monthse14 years (median 4.5 years). All 11 testes were intra-abdominal. In 5 out of 11 testes, the dissociation was associated with a wide separation of the epididymis and testis. The dissociated epididymis was in the scrotum attached to the gubernaculum while the testis was intra-abdominal. One case had bilateral CTED. Successful subdartos orchidopexy was done for 8 testes, 6 after Prentiss maneuver. Two gonads were fixed just below the pubic tubercle. Orchiectomy was done in one case with a small sized testis with a short gonadal vessel. Conclusion: CTED was encountered in 8% of cases of cryptorchidism. A palpable nubbin-like tissue in the scrotal sac in the presence of CTED may suggest a descended dissociated epididymis with an intra-abdominal testis. Successful subdartos orchidopexy was possible in 73% of testes with CTED. ª 2013 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Despite more than a century of research on cryptorchid testis, many aspects of cryptorchidism are still not well
* Corresponding author. Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India. Tel.: þ91 1126593309, þ91 9810863996; fax: þ91 1126588641. E-mail addresses: [email protected], shilpastemcell@gmail. com (S. Sharma).
defined and remain controversial. Understanding the morphological abnormalities associated with cryptorchidism is critical to the treatment and expected outcome in terms of fertility. Ductal abnormalities, patent processus vaginalis with inguinal hernias and testicular maldevelopment have been associated with the cryptorchid testis, more often with the abdominal testis. There is controversy surrounding the role of the epididymis in testicular descent, as epididymal abnor
1477-5131/$36 ª 2013 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.jpurol.2013.02.017
1024 malities have been reported both in descended and undescended testes, the incidence being higher in the presence of a patent processus [1e3]. Complete dissociation of the epididymis and testis, reported as early as 1961, is rare with only isolated case reports [4e9]. Here we describe an observational series of complete non-fusion of the epididymis and testis in 11 undescended testes. Our aim was to document the incidence of complete testicular epididymal dissociation (CTED) in the cryptorchid testis and to evaluate its operative outcome.
Methods The presence of CTED was noted prospectively in cases of undescended testis operated from October 2008 to May 2011. Necessary institutional approval was obtained, and the subjects/parents gave informed consent for carrying out this work. The operative findings of these cases were evaluated.
Results Over a 2 years 8 months period, 142 undescended testes in 130 boys were operated. CTED was encountered in 11 testes in 10 boys out of the 29 intra-abdominal testes among the 142 undescended testes (8%) [Fig. 1]. The ages of the patients varied from 18 monthse14 years with a median of 4.5 years. All 11 testes with CTED were intraabdominal. The open inguinal approach was used for all cases due to the lack of adequate facilities for a laparoscopic procedure. Two cases had been operated in the past. Both of these had bilateral undescended testes. One case had been subjected to right orchidopexy earlier (as a staged procedure with the testis fixed in the inguinal canal); the left side had remained non-operated. The right testis was found in the inguinal canal in this case, but did not have any dissociation and thus was not included in this series. The left testis was intra-abdominal and had CTED. Bilateral orchidopexy was done. The other case of bilateral undescended testes had been subjected
Figure 1 Complete testicular epididymal dissociation in an undescended testis.
S. Sharma, A. Sen to left herniotomy elsewhere without any mention of finding the testis in the operating notes. Only right inguinal exploration was done for this boy without doing anything for the left side. A big hernial sac along with CTED was found with wide separation of the epididymis present in the scrotum from the intraabdominal testis. Similar widely separated CTED was encountered in four more testes. Thus in five out of the 11 testes, the epididymis was in the scrotum attached to the gubernaculum while the testis was intra-abdominal. The average distance between the epididymis and testis was 9.6 cm (8e11). One of these cases with bilateral undescended testes had similar findings on both sides. Two of these five testes had an associated inguinal hernia. Successful orchidopexy was done for eight testes (73%) with the gonad fixed in the subdartos pouch (one bilateral case), six after Prentiss maneuver [Fig. 2]. The whole processus vaginalis sac was separated from the gubernaculum and lifted off along with the epididymis and the vas. The dissection proceeded to include the testis attached to a surrounding retroperitoneal layer that was dissected from the retroperitoneal structures, and brought out medially to the epigastric vessels through the superficial inguinal ring as a straight tract into the scrotum. Two gonads in which the gonadal vessels were short could only be brought down to reach up to just below the pubic tubercle, and were fixed near the origin of the scrotum [Fig. 3]. Orchiectomy was done in one case of a 14-year-old boy with a small sized testis with a short gonadal vessel.
Discussion Normally, the testis is loosely linked to the epididymis along its posterior border which at its lower pole gives rise to the vas deferens [10]. Turek et al., in 1994 described the characteristics of the epididymis in 112 cases into six types as follows: Type 1- Head and tail attachment with a “looped” body (84%); Type 2- Complete attachment to the testis (12%); Type 3- Head attachment only (3%); Type 4Tail attachment only- Rare (0%); Type 5- Non-fusion (1%); Type 6- Anomalies of ductal patency- Rare (0%) [11]. The classification has been further modified to divide Type 1 into Ia and Ib, the latter being associated with a longer loop [12]. Abnormalities that inhibit sperm transport like complete caput separation, atresia and agenesis have been reported in 8% patients with cryptorchidism [1]. Complete dissociation of the testis and epididymis is rare, occurring in only 0.9% of all healthy boys [11]. This failure of fusion between testis and epididymis may be encountered during operations for undescended testis in children. In this series, the incidence was 8% of the cryptorchid testes and 38% of the intra-abdominal testes. Kropp et al. described nonfusion of testis and epididymis in 5 testes in 4 boys in 325 cases of undescended testis, an incidence of 1% [6]. Kucukaydin et al. reported an incidence of 3% of non-fusion in a series of 222 undescended testes [12]. The incidence is higher in our series as a meticulous search for the intra-abdominal testis was done by tracing the uncoiled epididymis along with vas back to the retroperitoneal and intra-abdominal region to locate the missing
Testicular epididymal dissociation
Figure 2
1025
Flow diagram outlining the surgical management in cases of complete testicular epididymal dissociation.
testis. The spermatic vessels were traced to locate the testis. Also the median age of the patients as 4.5 years was high in this series, attributed to delayed presentation of the patients due to lack of awareness, poverty, socio-cultural taboos and non availability of experienced doctors in the remote villages. This higher age at presentation associated with a larger size of the tissues might have helped to visualize the non-fusion properly with the naked eye. It has been reported that the epididymis is more likely to be abnormal when the processus vaginalis is patent [1,3,13]. Heath et al. reported a high incidence of 75% of epididymal abnormalities being associated with truly undescended testes with a hernial sac compared with an incidence of 20% in undescended testes with no hernial sac and ectopic testes [13]. Similar findings have been reported by Elder et al., who documented the presence of epididymal abnormalities in 71% boys with an undescended testis in the presence of a patent processus, compared to 16% without a patent processus (p < 0.01) [1]. They also studied epididymal anomalies in normally descended testes in boys operated for hernia or hydrocele, and reported that 50% of boys with a hydrocele or hernia had an epididymal abnormality in the presence of a communicating patent processus, compared to 10% without a communicating patent
Figure 3 Flow diagram outlining the age in years at surgery and the operative procedure carried out.
processus (p < 0.01) [1]. These authors thus hypothesized that most epididymal abnormalities probably do not contribute to testicular maldescent [1]. Han et al. also found that the epididymal anomalies were strongly associated with the patency of the processus vaginalis irrespective of testicular descent (p < 0.001) [3]. Epididymal anomalies are more common in association with undescended (61%) than with descended (43%) testes (p Z 0.415) [3]. Incomplete attachment of the caput epididymis was the most common anomaly (35%), followed by detachment of caput and cauda epididymis (31%) in their series [3]. Similarly, the incidence of epididymal abnormalities in undescended testes (41%) has been reported to be higher than in ectopic testes (25.9%) (p < 0.05) [13]. Koff et al. had also reported a significantly higher incidence of complex epididymal abnormalities such as complete disruption between the testis and epididymis in cryptorchidism compared to ectopic testis (p < 0.001) [7]. Non-fusion was present in 0/166 of the ectopic testis and 5/222 (3%) cases of undescended testes in another series of 388 epididymides in 312 boys [12]. In this series, the gubernaculum was found attached to the normally descended epididymis in five cases with widely separated CTED, while the testis was intraabdominal, indicating the likely dragging role of the epididymis in testicular descent and contradicting the traditional teaching that the gubernaculum is attached to the testis. The presence of CTED had probably been the reason why the testis remained in the abdomen, as it failed to descend with the epididymis due to its non-fusion with the epididymis. A similar finding has also been reported by Al-Arfaj in a single case report who also felt that the scrotal position of the epididymis along with the gubernaculum associated with an intra-abdominal testis could reflect an essential role of the epididymis in the process of testicular descent [9]. An abnormal epididymis or epididy maletesticular interaction may play a role in inhibiting the descent and final maturation of the gonad [14]. Among the occasional cases of dissociation reported in the literature, in most cases both the epididymis and the testis have remained undescended [6,8]. The average distance between the epididymis and testis was 9.6 cm (8e11)
1026 in this series. Marshal et al. described a 7.5 cm separation between the detached epididymis and testis in a single case of non-palpable undescended testis [5]. The longer distance in our series may be attributed to the higher age at presentation. Two of the five testes with widely separated CTED in this series were associated with a wide patent processus vaginalis through which there was protrusion of the intestinal contents (hernia). Scrotal orchidopexy was possible in these two cases having an age of 18 months and 4 years. Al-Arfaj et al. also reported a case of bilateral inguinal hernia associated with an undescended abdominal testis completely separated from a normally descended epididymis on the left side [9]. The surgical procedure done for the condition correlated with the age at surgery. Successful scrotal orchidopexy was done for 8 testes; standard orchidopexy in 2 boys at ages 1.5 and 4 years; and in 6 following the Prentiss manoeuvre at ages 3e7. In two boys, 9 and 10 years old, the testis was fixed at just below the pubic tubercle. Orchiectomy was done in one case, in a 14-year-old boy with small sized testis and short gonadal vessel. The clinical significance of dealing with a diagnosis of CTED is that a judgemental error is likely to occur if the blind ending epididymis is mistaken for an atrophic testis and removed or left as such without operation. Thus, a careful search for the missing testis is recommended for all cases with a palpable nubbin in the scrotum [6]. This nubbin could actually be the descended epididymis with a dissociated intra-abdominal testis. The best way to find the testis (when it exists) is to follow the spermatic vessels from the sub renal area. Another surgical implication of CTED, especially when widely separated, is that the isolated epididymis is prone to torsion. Elert et al. reported two cases of isolated torsion of the epididymis in association with complete dissociation of testis and epididymis [15]. The embryological basis for this dissociation anomaly can be explained by the fact that the testis evolves from the sex cord of the gonadal ridge while the epididymis and vas evolve from mesonephric ducts and the Wolffian duct. The testis and epididymis develop in close relationship to unite later by fusion of the efferent ducts with the rete testis, facilitated by the Y-chromosome, androgens, inducers and suppressor substances. The last stage in gonadal development, the testicular descent, is induced by chorionic gonadotropin and androgen hormones supported by the gubernaculum [16]. Epididymal anomalies have been strongly associated with patency of the processus vaginalis [3]. This provides evidence for the hypothesis that a common stimulus, possibly androgens, may be required for epididymal development and obliteration of the processus vaginalis [3]. Another hypothesis may be that the epididymis attached to the gubernaculum, as documented in widely separated CTED in five testes in this series, is responsible for keeping the processus patent and dragging the testis along with it. Hence, due to its abnormality and non-fusion with the testis, the testis was unable to descend and remained intra-abdominal. It has been uniformly noted that cryptorchid testis within an open processus vaginalis is a frequent operative finding. Herzog et al. have reported that almost obligate
S. Sharma, A. Sen additional epididymal dissociation (90%) and a ST-index (spermatogonia per tubulus) of 0e0.2 in over 50% of the patients are an indication of a complex and severe form of cryptorchidism, and recommended postoperative treatment with a LHRH-analogue [17]. Major epididymal abnormalities are probably associated with severe impairment of sperm maturation and hindered transportation [7]. Gill et al. reported that biopsy of the testes with severe anomalies of ductal fusion showed preservation of germ cells in 69 per cent and diminished germ cells in 31 per cent [2]. The higher the arrest of testicular descent, the more grossly abnormal was the associated ductal system [2]. Thus they hypothesized that early successful orchidopexy alone may not ensure subsequent fertility despite the presence of normal germ cells [2]. The limitation of this study is that it was an observational study in a limited number of patients. Further larger studies may provide more concrete evidence and explanations of these findings. However, the important findings in this study may pave the way for explaining reasons for impaired fertility despite successful orchidopexy, especially in bilateral cases. The recent advances with in-vitro fertilization using intra cytoplasmic sperm injection may prove beneficial for these patients. Full documentation of the operative findings and proper counselling is recommended. An important clinical implication of this study is that a differential diagnosis of a palpable epididymis should be kept for all cases of clinically palpable scrotal nubbins, a term usually referring to an atrophic testicular tissue.
Conclusion Failure of fusion between testis and epididymis may be encountered during attempted orchidopexy. Association of a large inguinal hernia with intestinal contents in an undescended testis should give rise to suspicion for this anomaly. A palpable nubbin-like tissue in the scrotal sac may suggest a descended dissociated epididymis with an intra-abdominal testis.
Conflict of interest None.
Funding statement None.
Ethical approval Necessary approval was taken from the Institution and the patients for carrying out this work.
References [1] Elder JS. Epididymal anomalies associated with hydrocele/hernia and cryptorchidism: implications regarding testicular descent. J Urol 1992;148:624e6.
Testicular epididymal dissociation [2] Gill B, Kogan S, Starr S, Reda E, Levitt S. Significance of epididymal and ductal anomalies associated with testicular maldescent. J Urol 1989;142:556e8. [3] Han CH, Kang SH. Epididymal anomalies associated with patent processus vaginalis in hydrocele and cryptorchidism. J Korean Med Sci 2002;17:660e2. [4] Lythgoe J. Failure of fusion of the testis and epididymis. Br J Urol 1961;33:80e5. [5] Marshall FF, Weissman RM, Jeffs RD. Cryptorchidism: the surgical implications of non-union of the epididymis and testis. J Urol 1980;124:560e1. [6] Kropp W, Ringert RH, Hartung R. Dissociation of testis and epididymis in incomplete descent. Urologe A 1985;24:108e9. [7] Koff WJ, Scaletscky R. Malformations of the epididymis in undescended testis. J Urol 1990;143:340e3. [8] Emanuel ER, Kirsch AJ, Thall EH, Hensle TW. Complete separation of the testis and epididymis. J Pediatr Surg 1997;32:754e5. [9] Al-Arfaj AA, Al-Saflan AA. Complete separation of the testis and epididymis presenting as inguinal hernia. Saudi Med J 2002;23:1275e7. [10] de Kretser DM, Temple-Smith PD, Kerr JB. Anatomical and functional aspects of the male reproductive organs. In:
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Bandhauer K, Frick J, editors. Disturbances of male fertility. Berlin: Springer- Verlag; 1982. p. 1e131. Turek PJ, Ewalt DH, Snyder 3rd HM, Duckett JW. Normal epididymal anatomy in boys. J Urol 1994;151:726e7. Kucukaydin M, Ozokutan BH, Turan C, Okur H, Ko ¨se O. Malformation of the epididymis in undescended testis. Pediatr Surg Int 1998;14:189e91. Heath AL, Man DW, Eckstein HB. Epididymal abnormalities associated with maldescent of the testis. J Pediatr Surg 1984; 19:47e9. Mininberg DT, Schlossberg S. The role of the epididymis in testicular descent. J Urol 1983;129:1207e8. Elert A, Hegele A, Olbert P, Heidenreich A, Hofmann R. Isolated epididymal torsion in the dissociation of testis and epididymis. BJU Int 2002;89:971. Hadziselimovic F. Hormonal regulation of testicular descent and maldescent. Prog Clin Biol Res 1985;203:167e76. Herzog B, Rosslein R, Hadziselimovic F. The role of the processus vaginalis in cryptorchidism: does a patent processus vaginalis have a prognostic importance for predicting subsequent fertility? Eur J Pediatr 1993;152(Suppl. 2): S15e6.
Complete testicular epididymal dissociation in the abdominal cryptorchid testis Shilpa Sharma*, Amita Sen Department of Pediatric Surgery, Post Graduate Institute of Medical Education and Research, Dr RML Hospital, New Delhi, India Received 11 January 2013; accepted 5 February 2013 Available online 2 June 2013
KEYWORDS Epididymis; Cryptorchidism; Non-fusion; Dissociation
Abstract Aim: To document the incidence of complete testicular epididymal dissociation (CTED) in the cryptorchid testis and evaluate its operative outcome. Methods: The presence of CTED was noted prospectively in cases of undescended testis and the operative findings were studied. Results: CTED was encountered in 11 testes in 10 boys out of 29 intra-abdominal testes among the 142 undescended testes (8%) operated. Ages of patients varied from 18 monthse14 years (median 4.5 years). All 11 testes were intra-abdominal. In 5 out of 11 testes, the dissociation was associated with a wide separation of the epididymis and testis. The dissociated epididymis was in the scrotum attached to the gubernaculum while the testis was intra-abdominal. One case had bilateral CTED. Successful subdartos orchidopexy was done for 8 testes, 6 after Prentiss maneuver. Two gonads were fixed just below the pubic tubercle. Orchiectomy was done in one case with a small sized testis with a short gonadal vessel. Conclusion: CTED was encountered in 8% of cases of cryptorchidism. A palpable nubbin-like tissue in the scrotal sac in the presence of CTED may suggest a descended dissociated epididymis with an intra-abdominal testis. Successful subdartos orchidopexy was possible in 73% of testes with CTED. ª 2013 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Despite more than a century of research on cryptorchid testis, many aspects of cryptorchidism are still not well
* Corresponding author. Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India. Tel.: þ91 1126593309, þ91 9810863996; fax: þ91 1126588641. E-mail addresses: [email protected], shilpastemcell@gmail. com (S. Sharma).
defined and remain controversial. Understanding the morphological abnormalities associated with cryptorchidism is critical to the treatment and expected outcome in terms of fertility. Ductal abnormalities, patent processus vaginalis with inguinal hernias and testicular maldevelopment have been associated with the cryptorchid testis, more often with the abdominal testis. There is controversy surrounding the role of the epididymis in testicular descent, as epididymal abnor
1477-5131/$36 ª 2013 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.jpurol.2013.02.017
1024 malities have been reported both in descended and undescended testes, the incidence being higher in the presence of a patent processus [1e3]. Complete dissociation of the epididymis and testis, reported as early as 1961, is rare with only isolated case reports [4e9]. Here we describe an observational series of complete non-fusion of the epididymis and testis in 11 undescended testes. Our aim was to document the incidence of complete testicular epididymal dissociation (CTED) in the cryptorchid testis and to evaluate its operative outcome.
Methods The presence of CTED was noted prospectively in cases of undescended testis operated from October 2008 to May 2011. Necessary institutional approval was obtained, and the subjects/parents gave informed consent for carrying out this work. The operative findings of these cases were evaluated.
Results Over a 2 years 8 months period, 142 undescended testes in 130 boys were operated. CTED was encountered in 11 testes in 10 boys out of the 29 intra-abdominal testes among the 142 undescended testes (8%) [Fig. 1]. The ages of the patients varied from 18 monthse14 years with a median of 4.5 years. All 11 testes with CTED were intraabdominal. The open inguinal approach was used for all cases due to the lack of adequate facilities for a laparoscopic procedure. Two cases had been operated in the past. Both of these had bilateral undescended testes. One case had been subjected to right orchidopexy earlier (as a staged procedure with the testis fixed in the inguinal canal); the left side had remained non-operated. The right testis was found in the inguinal canal in this case, but did not have any dissociation and thus was not included in this series. The left testis was intra-abdominal and had CTED. Bilateral orchidopexy was done. The other case of bilateral undescended testes had been subjected
Figure 1 Complete testicular epididymal dissociation in an undescended testis.
S. Sharma, A. Sen to left herniotomy elsewhere without any mention of finding the testis in the operating notes. Only right inguinal exploration was done for this boy without doing anything for the left side. A big hernial sac along with CTED was found with wide separation of the epididymis present in the scrotum from the intraabdominal testis. Similar widely separated CTED was encountered in four more testes. Thus in five out of the 11 testes, the epididymis was in the scrotum attached to the gubernaculum while the testis was intra-abdominal. The average distance between the epididymis and testis was 9.6 cm (8e11). One of these cases with bilateral undescended testes had similar findings on both sides. Two of these five testes had an associated inguinal hernia. Successful orchidopexy was done for eight testes (73%) with the gonad fixed in the subdartos pouch (one bilateral case), six after Prentiss maneuver [Fig. 2]. The whole processus vaginalis sac was separated from the gubernaculum and lifted off along with the epididymis and the vas. The dissection proceeded to include the testis attached to a surrounding retroperitoneal layer that was dissected from the retroperitoneal structures, and brought out medially to the epigastric vessels through the superficial inguinal ring as a straight tract into the scrotum. Two gonads in which the gonadal vessels were short could only be brought down to reach up to just below the pubic tubercle, and were fixed near the origin of the scrotum [Fig. 3]. Orchiectomy was done in one case of a 14-year-old boy with a small sized testis with a short gonadal vessel.
Discussion Normally, the testis is loosely linked to the epididymis along its posterior border which at its lower pole gives rise to the vas deferens [10]. Turek et al., in 1994 described the characteristics of the epididymis in 112 cases into six types as follows: Type 1- Head and tail attachment with a “looped” body (84%); Type 2- Complete attachment to the testis (12%); Type 3- Head attachment only (3%); Type 4Tail attachment only- Rare (0%); Type 5- Non-fusion (1%); Type 6- Anomalies of ductal patency- Rare (0%) [11]. The classification has been further modified to divide Type 1 into Ia and Ib, the latter being associated with a longer loop [12]. Abnormalities that inhibit sperm transport like complete caput separation, atresia and agenesis have been reported in 8% patients with cryptorchidism [1]. Complete dissociation of the testis and epididymis is rare, occurring in only 0.9% of all healthy boys [11]. This failure of fusion between testis and epididymis may be encountered during operations for undescended testis in children. In this series, the incidence was 8% of the cryptorchid testes and 38% of the intra-abdominal testes. Kropp et al. described nonfusion of testis and epididymis in 5 testes in 4 boys in 325 cases of undescended testis, an incidence of 1% [6]. Kucukaydin et al. reported an incidence of 3% of non-fusion in a series of 222 undescended testes [12]. The incidence is higher in our series as a meticulous search for the intra-abdominal testis was done by tracing the uncoiled epididymis along with vas back to the retroperitoneal and intra-abdominal region to locate the missing
Testicular epididymal dissociation
Figure 2
1025
Flow diagram outlining the surgical management in cases of complete testicular epididymal dissociation.
testis. The spermatic vessels were traced to locate the testis. Also the median age of the patients as 4.5 years was high in this series, attributed to delayed presentation of the patients due to lack of awareness, poverty, socio-cultural taboos and non availability of experienced doctors in the remote villages. This higher age at presentation associated with a larger size of the tissues might have helped to visualize the non-fusion properly with the naked eye. It has been reported that the epididymis is more likely to be abnormal when the processus vaginalis is patent [1,3,13]. Heath et al. reported a high incidence of 75% of epididymal abnormalities being associated with truly undescended testes with a hernial sac compared with an incidence of 20% in undescended testes with no hernial sac and ectopic testes [13]. Similar findings have been reported by Elder et al., who documented the presence of epididymal abnormalities in 71% boys with an undescended testis in the presence of a patent processus, compared to 16% without a patent processus (p < 0.01) [1]. They also studied epididymal anomalies in normally descended testes in boys operated for hernia or hydrocele, and reported that 50% of boys with a hydrocele or hernia had an epididymal abnormality in the presence of a communicating patent processus, compared to 10% without a communicating patent
Figure 3 Flow diagram outlining the age in years at surgery and the operative procedure carried out.
processus (p < 0.01) [1]. These authors thus hypothesized that most epididymal abnormalities probably do not contribute to testicular maldescent [1]. Han et al. also found that the epididymal anomalies were strongly associated with the patency of the processus vaginalis irrespective of testicular descent (p < 0.001) [3]. Epididymal anomalies are more common in association with undescended (61%) than with descended (43%) testes (p Z 0.415) [3]. Incomplete attachment of the caput epididymis was the most common anomaly (35%), followed by detachment of caput and cauda epididymis (31%) in their series [3]. Similarly, the incidence of epididymal abnormalities in undescended testes (41%) has been reported to be higher than in ectopic testes (25.9%) (p < 0.05) [13]. Koff et al. had also reported a significantly higher incidence of complex epididymal abnormalities such as complete disruption between the testis and epididymis in cryptorchidism compared to ectopic testis (p < 0.001) [7]. Non-fusion was present in 0/166 of the ectopic testis and 5/222 (3%) cases of undescended testes in another series of 388 epididymides in 312 boys [12]. In this series, the gubernaculum was found attached to the normally descended epididymis in five cases with widely separated CTED, while the testis was intraabdominal, indicating the likely dragging role of the epididymis in testicular descent and contradicting the traditional teaching that the gubernaculum is attached to the testis. The presence of CTED had probably been the reason why the testis remained in the abdomen, as it failed to descend with the epididymis due to its non-fusion with the epididymis. A similar finding has also been reported by Al-Arfaj in a single case report who also felt that the scrotal position of the epididymis along with the gubernaculum associated with an intra-abdominal testis could reflect an essential role of the epididymis in the process of testicular descent [9]. An abnormal epididymis or epididy maletesticular interaction may play a role in inhibiting the descent and final maturation of the gonad [14]. Among the occasional cases of dissociation reported in the literature, in most cases both the epididymis and the testis have remained undescended [6,8]. The average distance between the epididymis and testis was 9.6 cm (8e11)
1026 in this series. Marshal et al. described a 7.5 cm separation between the detached epididymis and testis in a single case of non-palpable undescended testis [5]. The longer distance in our series may be attributed to the higher age at presentation. Two of the five testes with widely separated CTED in this series were associated with a wide patent processus vaginalis through which there was protrusion of the intestinal contents (hernia). Scrotal orchidopexy was possible in these two cases having an age of 18 months and 4 years. Al-Arfaj et al. also reported a case of bilateral inguinal hernia associated with an undescended abdominal testis completely separated from a normally descended epididymis on the left side [9]. The surgical procedure done for the condition correlated with the age at surgery. Successful scrotal orchidopexy was done for 8 testes; standard orchidopexy in 2 boys at ages 1.5 and 4 years; and in 6 following the Prentiss manoeuvre at ages 3e7. In two boys, 9 and 10 years old, the testis was fixed at just below the pubic tubercle. Orchiectomy was done in one case, in a 14-year-old boy with small sized testis and short gonadal vessel. The clinical significance of dealing with a diagnosis of CTED is that a judgemental error is likely to occur if the blind ending epididymis is mistaken for an atrophic testis and removed or left as such without operation. Thus, a careful search for the missing testis is recommended for all cases with a palpable nubbin in the scrotum [6]. This nubbin could actually be the descended epididymis with a dissociated intra-abdominal testis. The best way to find the testis (when it exists) is to follow the spermatic vessels from the sub renal area. Another surgical implication of CTED, especially when widely separated, is that the isolated epididymis is prone to torsion. Elert et al. reported two cases of isolated torsion of the epididymis in association with complete dissociation of testis and epididymis [15]. The embryological basis for this dissociation anomaly can be explained by the fact that the testis evolves from the sex cord of the gonadal ridge while the epididymis and vas evolve from mesonephric ducts and the Wolffian duct. The testis and epididymis develop in close relationship to unite later by fusion of the efferent ducts with the rete testis, facilitated by the Y-chromosome, androgens, inducers and suppressor substances. The last stage in gonadal development, the testicular descent, is induced by chorionic gonadotropin and androgen hormones supported by the gubernaculum [16]. Epididymal anomalies have been strongly associated with patency of the processus vaginalis [3]. This provides evidence for the hypothesis that a common stimulus, possibly androgens, may be required for epididymal development and obliteration of the processus vaginalis [3]. Another hypothesis may be that the epididymis attached to the gubernaculum, as documented in widely separated CTED in five testes in this series, is responsible for keeping the processus patent and dragging the testis along with it. Hence, due to its abnormality and non-fusion with the testis, the testis was unable to descend and remained intra-abdominal. It has been uniformly noted that cryptorchid testis within an open processus vaginalis is a frequent operative finding. Herzog et al. have reported that almost obligate
S. Sharma, A. Sen additional epididymal dissociation (90%) and a ST-index (spermatogonia per tubulus) of 0e0.2 in over 50% of the patients are an indication of a complex and severe form of cryptorchidism, and recommended postoperative treatment with a LHRH-analogue [17]. Major epididymal abnormalities are probably associated with severe impairment of sperm maturation and hindered transportation [7]. Gill et al. reported that biopsy of the testes with severe anomalies of ductal fusion showed preservation of germ cells in 69 per cent and diminished germ cells in 31 per cent [2]. The higher the arrest of testicular descent, the more grossly abnormal was the associated ductal system [2]. Thus they hypothesized that early successful orchidopexy alone may not ensure subsequent fertility despite the presence of normal germ cells [2]. The limitation of this study is that it was an observational study in a limited number of patients. Further larger studies may provide more concrete evidence and explanations of these findings. However, the important findings in this study may pave the way for explaining reasons for impaired fertility despite successful orchidopexy, especially in bilateral cases. The recent advances with in-vitro fertilization using intra cytoplasmic sperm injection may prove beneficial for these patients. Full documentation of the operative findings and proper counselling is recommended. An important clinical implication of this study is that a differential diagnosis of a palpable epididymis should be kept for all cases of clinically palpable scrotal nubbins, a term usually referring to an atrophic testicular tissue.
Conclusion Failure of fusion between testis and epididymis may be encountered during attempted orchidopexy. Association of a large inguinal hernia with intestinal contents in an undescended testis should give rise to suspicion for this anomaly. A palpable nubbin-like tissue in the scrotal sac may suggest a descended dissociated epididymis with an intra-abdominal testis.
Conflict of interest None.
Funding statement None.
Ethical approval Necessary approval was taken from the Institution and the patients for carrying out this work.
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