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== Management ==
== Management ==


* '''Observation is indicated for the first 6 postnatal months to allow spontaneous testicular descent.'''
=== Options ===
** No strong evidence in support of observation as the recommended approach for cases of acquired cryptorchidism.
*'''Observation'''
**'''Indications (1)'''
***'''First 6 post-natal months (corrected for gestational age) to allow spontaneous testicular descent'''
**** No strong evidence in support of observation as the recommended approach for cases of acquired cryptorchidism.
* '''Medical Therapy'''
* '''Medical Therapy'''
** '''Hormone therapy is not currently recommended,''' given the lack of rigorous data supporting its efficacy
** '''Hormone therapy is not recommended'''
* '''If spontaneous testicular descent does not occur, surgical treatment after 6 months of (corrected gestational) age is indicated.'''
***Lack of rigorous data supporting its efficacy
** In boys with a history of prematurity, spontaneous descent may be delayed, and therefore observation is continued for 6 months beyond the expected date of delivery or, especially if testicular position is marginal, until a year of age.
* '''Surgery (orchiopexy/orchidopexy)'''
** '''After spontaneous testicular descent, continued observation is needed because of the risk for recurrent cryptorchidism or testicular re-ascent'''
**'''If spontaneous testicular descent does not occur, surgical treatment after 6 months of (corrected gestational) age is indicated.'''
*** In boys with a history of prematurity, spontaneous descent may be delayed, and therefore observation is continued for 6 months beyond the expected date of delivery or, especially if testicular position is marginal, until a year of age.
*** '''After spontaneous testicular descent, continued observation is needed because of the risk for recurrent cryptorchidism or testicular re-ascent'''
**'''Timing of Surgery'''
***'''Orchidopexy is recommended between 6-18 months of age'''
**** '''Testicular descent is unlikely to occur in full-term babies after 6 months of age'''
**** '''Consider surgical morbidity, comorbidities, life expectancy, and fertility expectations in special situations'''
***** '''UDT is associated with a multitude of syndromes, some of which can lead to limited life expectancy and/or severe developmental delay (e.g., Down’s, Prader-Willi, and Noonan’s syndromes)'''
***** Given the reports of testicular cancer (sometimes at an early age) in these patients, '''we recommend orchidopexy when they are clinically fit for anesthesia for the purpose of surveillance'''
 
=== Orchiopexy/Orchidopexy ===
 
==== Surgical Approach to the Palpable Testis ====
* '''Options: inguinal vs. pre-scrotal/scrotal orchidopexy'''
** '''The traditional approach to surgical treatment of palpable testes is inguinal orchidopexy''' (with repair of an associated hernia if present), although a primary scrotal approach as is an alternative approach.
** '''High (proximal) ligation of the processus vaginalis is an essential surgical step to allow placement of the testis in a sub-dartos pouch within the hemi-scrotum, without tension'''
** '''Inguinal'''
*** '''Maneuvers to provide spermatic cord length include (4):'''
***# '''Transection of lateral fascial bands along the cord'''
***# '''Cranial retroperitoneal dissection'''
***# '''Medial transposition of the testis beneath the epigastric vessels (Prentiss maneuver)'''
***# '''Cranial extension of the incision'''
**** '''Very rarely, the testis cannot be brought to dependent scrotal position after these maneuvers and a two-stage procedure may be considered''' as an alternative to orchiectomy, which is preferentially reserved for visibly abnormal or atrophic testes, postpubertal patients, or cases associated with insufficient vasal length.
*** '''Complications'''
**** '''Uncommon'''
**** '''Serious complications include testicular retraction and atrophy'''
*** '''A minimum of 6 months’ follow-up is recommended to determine postoperative testis position and size.'''
**** Long-term follow-up should be considered for counseling of the patient regarding fertility issues, risk of testicular malignancy, and self-examination.
*** Torsion of a scrotal testis after orchidopexy has been reported but is very rare, and the risk may be minimized by routine extravaginal testicular fixation in a subdartos pouch.
*** If complete intrascrotal testicular atrophy occurs postoperatively, further intervention is not needed, but the option of testicular prosthesis placement should be offered to the patient and family.
**** Implantation of a testicular prosthesis should occur at least 6 months after any scrotal procedure or after puberty and is best performed through an inguinal approach.
**** Fixation of the prosthesis to the dartos and closure of the scrotal fascia above the implant using purse-string nonabsorbable suture are required.
**** Complications including displacement, pain, or infection occur in < 5% of cases.
** '''Scrotal'''
*** Used selectively in many series; '''efficacy and complication rates are similar to those of standard inguinal orchidopexy'''
 
==== Surgical Approach to the Non-palpable Abdominal Testis ====
* '''If the testicle is not palpable preoperatively, as may occur in up to 20% of UDT cases, examination under anesthesia (EUA) can sometimes allow identification of the testicle. Otherwise, diagnostic laparoscopy is the procedure of choice in most centres.'''
** '''In certain non-palpable testicle cases, confident palpation of an ipsilateral scrotal nubbin and identification of contralateral compensatory testicular hypertrophy may preclude diagnostic laparoscopy by means of initially performing a scrotal incision, which allows for testicular nubbin removal and confirmation of the vanishing testicle diagnosis'''. Inguinal exploration and/or laparoscopy can then be reserved for cases in which the initial scrotal approach is non-diagnostic.
*** '''It is critical to highlight the importance of confidently identifying atrophic testicular tissue with associated vas deferens and gonadal vessels if a scrotal or inguinal approach is chosen, as any doubt should trigger further exploration.''' Presence of a looping vas or incorrectly identifying non-gonadal tissue as a nubbin may lead to misdiagnosis, potentially leaving viable testicular tissue in the abdomen'''. In uncertain cases or when tissue analysis is not consistent with atrophic testicular tissue, laparoscopic exploration should be strongly considered'''
* '''Diagnostic laparoscopy'''
** '''Potential findings:'''
**# '''Blind-ending vas and vessels confirms a vanishing intra-abdominal testicle (IAT), and no further exploration is necessary''' (10‒30% of cases).
**#* '''An atretic spermatic cord coursing through a closed inguinal ring is suggestive of a distal vanishing testis, but this finding may be subjective and, conversely, normal appearing vessels may be associated with both viable and vanishing testes'''
**# '''Testicular vessels and vas entering the inguinal canal through the internal inguinal ring.'''
**#* '''Inguinal exploration may find a healthy palpable UDT amenable to standard orchidopexy, or a testicular nubbin either in the inguinal region or, most commonly, in the scrotum.''' Remnant cord structures are usually removed to confirm the diagnosis and because viable residual testicular elements are present in up to 14% of the cases. It should be noted that to date, no cases of intratubular germ cell neoplasia have been reported within these specimens.
**# '''Peeping''' (just inside internal ring) '''or intra-abdominal testis (50%), which will require either an open or a laparoscopic orchidopexy in one or two stages.'''
*** '''If neither vas nor spermatic artery is found at the time of laparoscopy, dissection of the perivesical area and retroperitoneum up to the level of the kidney is required for exclusion of the presence of a testis, because true agenesis is extremely rare'''
**** '''If both testes are non-palpable and not distal to the internal inguinal ring in a genetic male, > 95% are abdominal'''
** '''Therefore, if laparoscopy does not unequivocally localize the testis or blind-ending spermatic artery, additional surgical exploration is needed for definitive diagnosis. This may be performed laparoscopically after the placement of additional working ports.'''
* '''Primary orchidopexy without transection of the spermatic vessels is preferable whenever possible'''
** Pooled success rates for primary, one-stage Fowler-Stephens and two-stage Fowler-Stephens procedures are ≈95%, 80%, and 85%, respectively.
* '''The feasibility of primary vs. Fowler-Stephens orchidopexy depends on the length of the vas and vessels, presence or absence of looping ductal structures, and age of the patient.'''
** Observed testicular position alone may correlate poorly with the ultimate length of the cord after mobilization.
* '''Inguinal approach for the high inguinal canalicular, or intra-abdominal testis'''
** '''Helpful maneuvers to bring a high testicle down to the scrotum while preserving its blood supply:'''
**# '''Divide the lateral fibrous attachments of the cord at the internal inguinal ring'''
**# '''Blunt dissection of the retroperitoneal spermatic vessels''' (which are usually the limiting factor) up to the lower pole of the kidney
**# '''Mobilization of the cord medial to the inferior epigastric vessels (Prentiss maneuver)'''
**# '''Despite these steps, if the testicle still does not reach the scrotum, a Fowler-Stephens (FS) orchidopexy may be performed.'''
* '''Fowler-Stephens orchidopexy'''
** Originally described as a single-stage open inguinal approach for the intra-abdominal testis in which the testicular artery and veins were too short to allow adequate testicular mobilization into the scrotum through standard orchidopexy
** '''The major steps are (4):'''
**# '''Mobilization of any structures extending distal to the internal ring'''
**# '''Transection of the peritoneum lateral to the vessels and distal to the vas'''
**# '''Proximal mobilization of the vessels while maintaining collateral blood supply between the vas and spermatic vessels.  '''
**#* '''The peritoneum should be left intact over the vasal vessels, and the gubernacular vessels should be left intact if possible'''
**# '''Once mobilized, the testis is brought through a new hiatus medial to the epigastrics and lateral to the medial umbilical ligament or through the existing internal inguinal ring.'''
**#* Recall that medial umbilical ligament containst the obliterated umbilical artery
** '''Principles:'''
*** '''Open approach'''
**** '''Ligating and dividing the testicular vessels'''
**** '''Preserving the''' '''distal gubernacular attachments and the collateral vessels on the floor of the inguinal canal, maintaining the cremasteric blood supply'''
*** '''Laparoscopic approach'''
**** '''Ligating and dividing the testicular vessels and cremasteric collaterals'''
**** '''Preserving the gubernaculum''', mimicking one of the surgical steps of the open FS technique, may help decrease the likelihood of testicular atrophy
**** '''Testicle is advanced medial to the inferior epigastric vessels or obliterated umbilical artery'''
**** '''An inguinal hernia or patent processus vaginalis does not require formal repair at the time of laparoscopic orchidopexy.'''
*** '''In general, the preferred approach is avoidance of spermatic vessel transection whenever possible; the available data suggest this is possible in the majority of cases of abdominal orchidopexy.'''
*** '''When the testis is > 2 cm above the internal ring with no vascular redundancy, it is unlikely that the testis will be able to reach the scrotum without dividing the spermatic vessels. In this situation, staged Fowler-Stephens procedure with division of the gonadal vessels at the first stage has a significantly higher success rate than single-stage Fowler-Stephens procedure'''
** '''Open vs. laparoscopic orchidopexy for NPT'''
*** '''The Fowler-Stephens procedure is now typically performed laparoscopically''' with spermatic vessel clipping followed by laparoscopic or open testicular mobilization in the same setting (one-stage, or in a staged approach 6 months later (two-stage).
*** Laparoscopic orchidopexy outcomes are comparable to those of open surgery
*** '''Laparoscopic approach is associated''' with shorter operative time, shorter return to normal activities, '''and reduced risk of atrophy at one year (10% laparoscopic vs. 19% open)'''


* '''Timing of Surgery'''
** '''Orchidopexy is recommended between 6-18 months of age'''
*** '''Testicular descent is unlikely to occur in full-term babies after 6 months of age'''
*** '''Consider surgical morbidity, comorbidities, life expectancy, and fertility expectations in special situations'''
**** '''UDT is associated with a multitude of syndromes, some of which can lead to limited life expectancy and/or severe developmental delay (e.g., Down’s, Prader-Willi, and Noonan’s syndromes)'''
**** Given the reports of testicular cancer (sometimes at an early age) in these patients, '''we recommend orchidopexy when they are clinically fit for anesthesia for the purpose of surveillance'''
* '''Surgical Approach to the Palpable Testis'''
** '''Options: inguinal vs. pre-scrotal/scrotal orchidopexy'''
*** '''The traditional approach to surgical treatment of palpable testes is inguinal orchidopexy''' (with repair of an associated hernia if present), although a primary scrotal approach as is an alternative approach.
*** '''High (proximal) ligation of the processus vaginalis is an essential surgical step to allow placement of the testis in a sub-dartos pouch within the hemi-scrotum, without tension'''
*** '''Inguinal'''
**** '''Maneuvers to provide spermatic cord length include (4):'''
****# '''Transection of lateral fascial bands along the cord'''
****# '''Cranial retroperitoneal dissection'''
****# '''Medial transposition of the testis beneath the epigastric vessels (Prentiss maneuver)'''
****# '''Cranial extension of the incision'''
***** '''Very rarely, the testis cannot be brought to dependent scrotal position after these maneuvers and a two-stage procedure may be considered''' as an alternative to orchiectomy, which is preferentially reserved for visibly abnormal or atrophic testes, postpubertal patients, or cases associated with insufficient vasal length.
**** '''Complications'''
***** '''Uncommon'''
***** '''Serious complications include testicular retraction and atrophy'''
**** '''A minimum of 6 months’ follow-up is recommended to determine postoperative testis position and size.'''
***** Long-term follow-up should be considered for counseling of the patient regarding fertility issues, risk of testicular malignancy, and self-examination.
**** Torsion of a scrotal testis after orchidopexy has been reported but is very rare, and the risk may be minimized by routine extravaginal testicular fixation in a subdartos pouch.
**** If complete intrascrotal testicular atrophy occurs postoperatively, further intervention is not needed, but the option of testicular prosthesis placement should be offered to the patient and family.
***** Implantation of a testicular prosthesis should occur at least 6 months after any scrotal procedure or after puberty and is best performed through an inguinal approach.
***** Fixation of the prosthesis to the dartos and closure of the scrotal fascia above the implant using purse-string nonabsorbable suture are required.
***** Complications including displacement, pain, or infection occur in < 5% of cases.
*** '''Scrotal'''
**** Used selectively in many series; '''efficacy and complication rates are similar to those of standard inguinal orchidopexy'''
* '''Surgical Approach to the Non-palpable Abdominal Testis'''
** '''If the testicle is not palpable preoperatively, as may occur in up to 20% of UDT cases, examination under anesthesia (EUA) can sometimes allow identification of the testicle. Otherwise, diagnostic laparoscopy is the procedure of choice in most centres.'''
*** '''In certain non-palpable testicle cases, confident palpation of an ipsilateral scrotal nubbin and identification of contralateral compensatory testicular hypertrophy may preclude diagnostic laparoscopy by means of initially performing a scrotal incision, which allows for testicular nubbin removal and confirmation of the vanishing testicle diagnosis'''. Inguinal exploration and/or laparoscopy can then be reserved for cases in which the initial scrotal approach is non-diagnostic.
**** '''It is critical to highlight the importance of confidently identifying atrophic testicular tissue with associated vas deferens and gonadal vessels if a scrotal or inguinal approach is chosen, as any doubt should trigger further exploration.''' Presence of a looping vas or incorrectly identifying non-gonadal tissue as a nubbin may lead to misdiagnosis, potentially leaving viable testicular tissue in the abdomen'''. In uncertain cases or when tissue analysis is not consistent with atrophic testicular tissue, laparoscopic exploration should be strongly considered'''
** '''Diagnostic laparoscopy'''
*** '''Potential findings:'''
***# '''Blind-ending vas and vessels confirms a vanishing intra-abdominal testicle (IAT), and no further exploration is necessary''' (10‒30% of cases).
***#* '''An atretic spermatic cord coursing through a closed inguinal ring is suggestive of a distal vanishing testis, but this finding may be subjective and, conversely, normal appearing vessels may be associated with both viable and vanishing testes'''
***# '''Testicular vessels and vas entering the inguinal canal through the internal inguinal ring.'''
***#* '''Inguinal exploration may find a healthy palpable UDT amenable to standard orchidopexy, or a testicular nubbin either in the inguinal region or, most commonly, in the scrotum.''' Remnant cord structures are usually removed to confirm the diagnosis and because viable residual testicular elements are present in up to 14% of the cases. It should be noted that to date, no cases of intratubular germ cell neoplasia have been reported within these specimens.
***# '''Peeping''' (just inside internal ring) '''or intra-abdominal testis (50%), which will require either an open or a laparoscopic orchidopexy in one or two stages.'''
**** '''If neither vas nor spermatic artery is found at the time of laparoscopy, dissection of the perivesical area and retroperitoneum up to the level of the kidney is required for exclusion of the presence of a testis, because true agenesis is extremely rare'''
***** '''If both testes are non-palpable and not distal to the internal inguinal ring in a genetic male, > 95% are abdominal'''
*** '''Therefore, if laparoscopy does not unequivocally localize the testis or blind-ending spermatic artery, additional surgical exploration is needed for definitive diagnosis. This may be performed laparoscopically after the placement of additional working ports.'''
** '''Primary orchidopexy without transection of the spermatic vessels is preferable whenever possible'''
*** Pooled success rates for primary, one-stage Fowler-Stephens and two-stage Fowler-Stephens procedures are ≈95%, 80%, and 85%, respectively.
** '''The feasibility of primary vs. Fowler-Stephens orchidopexy depends on the length of the vas and vessels, presence or absence of looping ductal structures, and age of the patient.'''
*** Observed testicular position alone may correlate poorly with the ultimate length of the cord after mobilization.
** '''Inguinal approach for the high inguinal canalicular, or intra-abdominal testis'''
*** '''Helpful maneuvers to bring a high testicle down to the scrotum while preserving its blood supply:'''
***# '''Divide the lateral fibrous attachments of the cord at the internal inguinal ring'''
***# '''Blunt dissection of the retroperitoneal spermatic vessels''' (which are usually the limiting factor) up to the lower pole of the kidney
***# '''Mobilization of the cord medial to the inferior epigastric vessels (Prentiss maneuver)'''
***# '''Despite these steps, if the testicle still does not reach the scrotum, a Fowler-Stephens (FS) orchidopexy may be performed.'''
** '''Fowler-Stephens orchidopexy'''
*** Originally described as a single-stage open inguinal approach for the intra-abdominal testis in which the testicular artery and veins were too short to allow adequate testicular mobilization into the scrotum through standard orchidopexy
*** '''The major steps are (4):'''
***# '''Mobilization of any structures extending distal to the internal ring'''
***# '''Transection of the peritoneum lateral to the vessels and distal to the vas'''
***# '''Proximal mobilization of the vessels while maintaining collateral blood supply between the vas and spermatic vessels.  '''
***#* '''The peritoneum should be left intact over the vasal vessels, and the gubernacular vessels should be left intact if possible'''
***# '''Once mobilized, the testis is brought through a new hiatus medial to the epigastrics and lateral to the medial umbilical ligament or through the existing internal inguinal ring.'''
***#* Recall that medial umbilical ligament containst the obliterated umbilical artery
*** '''Principles:'''
**** '''Open approach'''
***** '''Ligating and dividing the testicular vessels'''
***** '''Preserving the''' '''distal gubernacular attachments and the collateral vessels on the floor of the inguinal canal, maintaining the cremasteric blood supply'''
**** '''Laparoscopic approach'''
***** '''Ligating and dividing the testicular vessels and cremasteric collaterals'''
***** '''Preserving the gubernaculum''', mimicking one of the surgical steps of the open FS technique, may help decrease the likelihood of testicular atrophy
***** '''Testicle is advanced medial to the inferior epigastric vessels or obliterated umbilical artery'''
***** '''An inguinal hernia or patent processus vaginalis does not require formal repair at the time of laparoscopic orchidopexy.'''
**** '''In general, the preferred approach is avoidance of spermatic vessel transection whenever possible; the available data suggest this is possible in the majority of cases of abdominal orchidopexy.'''
**** '''When the testis is > 2 cm above the internal ring with no vascular redundancy, it is unlikely that the testis will be able to reach the scrotum without dividing the spermatic vessels. In this situation, staged Fowler-Stephens procedure with division of the gonadal vessels at the first stage has a significantly higher success rate than single-stage Fowler-Stephens procedure'''
*** '''Open vs. laparoscopic orchidopexy for NPT'''
**** '''The Fowler-Stephens procedure is now typically performed laparoscopically''' with spermatic vessel clipping followed by laparoscopic or open testicular mobilization in the same setting (one-stage, or in a staged approach 6 months later (two-stage).
**** Laparoscopic orchidopexy outcomes are comparable to those of open surgery
**** '''Laparoscopic approach is associated''' with shorter operative time, shorter return to normal activities, '''and reduced risk of atrophy at one year (10% laparoscopic vs. 19% open)'''
* '''Complications'''
* '''Complications'''
** '''Complications of laparoscopic orchidopexy are rare and potentially include bladder or vascular injury, hypercapnia, delayed small bowel obstruction, testicular ascent''', where the testicle gets pulled to the entrance of the scrotum, '''and vas deferens injury'''.
** '''Complications of laparoscopic orchidopexy are rare and potentially include bladder or vascular injury, hypercapnia, delayed small bowel obstruction, testicular ascent''', where the testicle gets pulled to the entrance of the scrotum, '''and vas deferens injury'''.
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