Note: This guideline has been updated. The National Guideline Clearinghouse (NGC) is working to update this summary. The recommendations that follow are based on the previous version of the guideline.
Levels of evidence (1a-4) and grades of recommendation (A-C) are defined at the end of the "Major Recommendations" field.
Background
In the case of bilateral non-palpable testes and any suggestion of sexual differentiation problems (e.g., hypospadias), urgent endocrinological and genetic evaluation is mandatory (Level of evidence: 3; Grade of recommendation B).
Diagnosis
A physical examination is the only method of differentiating between palpable or non-palpable testes. There is no additional benefit in performing ultrasound, computed tomography (CT), magnetic resonance imaging (MRI) or angiography.
Clinical examination includes a visual description of the scrotum and an examination of the child while supine and in crossed-leg position. The examiner should inhibit the cremasteric reflex with his non-dominant hand right above the symphysis in the groin region before touching, or reaching for, the scrotum. The groin region may be 'milked' towards the scrotum in an attempt to move the testis into the scrotum. This manoeuvre also makes it possible to differentiate between an inguinal testis and enlarged lymph nodes, which can give the impression of an undescended testis.
A retractile testis can generally be brought into the scrotum, where it will remain until a cremasteric reflex (touching the inner thigh skin) will retract it again into the groin.
A unilateral, non-palpable, testis and an enlarged, contralateral, testis may suggest testicular absence or atrophy, but this finding is not specific and does not preclude surgical exploration. In the case of an inguinal, non-palpable testis, a careful visual inspection should be made of the femoral, penile and perineal region to exclude an ectopic testis.
There is no reliable examination to confirm or rule out an intra-abdominal, inguinal and absent/vanishing testis (non-palpable testis), except for diagnostic laparoscopy (Level of evidence: 1b; Grade of recommendation A). Before carrying out a laparoscopic assessment, an examination under general anaesthesia is recommended because some, originally non-palpable, testes are palpable under anaesthetic conditions.
Treatment
If a testis has not descended by the age of 1 year, there is no benefit in waiting for a spontaneous descent. To prevent histological deterioration, treatment should be carried out and finished before 12-18 months of age.
Medical Therapy
Medical therapy using human chorionic gonadotrophin (hCG) or gonadotrophin-releasing hormone (GnRH) is based on the hormonal dependence of testicular descent, with maximum success rates of 20%. The success rate for hormonal therapy for testicular descent becomes less as the higher the undescended testis is located. HCG is given in a total dosage of 6.000 to 9.000 units, split up into four doses within 2-3 weeks, depending on weight and age. GnRH is given in a dosage of 1.2 mg/day, divided into three doses per day, for 4 weeks using a nasal spray.
Medical treatment can have a beneficial effect on increasing the patient's fertility index when given before (dosage as described above) or after (low intermittent dosages) surgical orchidolysis and orchidopexy. The fertility index can be a predictor of fertility later in life (Level of evidence: 1b; Grade of recommendation A). On the other hand, long-term follow-up data on hormonal treatment are not available yet.
Surgery
Palpable Testis
Surgery for the palpable testis includes orchidofuniculolysis and orchidopexy, via an inguinal approach, with success rates of up to 92%. It is important to remove and dissect all cremasteric fibres to prevent secondary retraction. Associated problems, such as an open processus vaginalis, must be carefully dissected and closed. It is recommended that the testis is placed in a subdartos pouch. With regard to sutures, there should either be no fixation sutures or they should be made between the tunica vaginalis and the dartos musculature.
The lymph drainage of a testis that has undergone surgery for orchidopexy has been changed from iliac drainage to iliac and inguinal drainage (which is important in the event of later malignancy).
Non-palpable Testis
In the situation of a non-palpable testis, inguinal surgical exploration with the possibility of performing laparoscopy should be attempted. There is a good possibility of finding the testis via an inguinal incision, but in rare cases, it is necessary to search into the abdomen if there are no vessels or vas deferens in the groin. Laparoscopy is the most appropriate way of examining the abdomen for a testis. In addition, either removal or orchidolysis and orchiopexy can be performed via laparoscopic access.
Before starting diagnostic laparoscopy it is recommended that the child should be examined again under general anaesthesia because a previously non-palpable testes might now be palpable under anaesthesia.
An intra-abdominal testis in a 10-year-old boy or older with a normal contralateral testis should be removed. In bilateral intra-abdominal testes, or in a boy younger than 10 years, a one-stage or two-stage Fowler-Stephens procedure can be performed. In the event of a two-stage procedure, the spermatic vessels are either laparoscopically clipped or coagulated proximal to the testis to allow development of collateral vasculature. The second-stage procedure, in which the testis is brought directly over the symphysis and next to the bladder into the scrotum, can also be performed by laparoscopy 6 months later. The testicular survival rate in a one-stage procedure varies between 50% and 60%, with success rates rising up to 90% in a two-stage procedure. Microvascular autotransplantation can also be performed with 90% testicular survival rate. However, the procedure requires very skilful and experienced surgical techniques.
Prognosis
Boys with one undescended testis have a lower fertility rate, but the same paternity rate as boys with bilateral descended testes. Boys with bilateral undescended testes have both lower fertility and paternity rates.
Boys with an undescended testis have a 20-fold higher chance of developing testicular malignancy, a risk that is uninfluenced by any kind of treatment. Screening both during and after puberty is therefore recommended for these boys. Recently, a Swedish study with a cohort of almost 17,000 men who were treated surgically for undescended testis and followed for a total of almost 210,000 person-years revealed that treatment for undescended testis before puberty decreased the risk of testicular cancer. The relative risk of testicular cancer among those who underwent orchiopexy before reaching 13 years of age was 2.23 compared to the Swedish general population; for those treated at 13 years of age or older, the relative risk was 5.40. Furthermore, a systematic literature review and meta-analysis by an American group has also concluded that prepubertal orchiopexy may decrease the risk of testicular cancer and that early surgical intervention is indicated in children with cryptorchidism.
Boys with retractile testes do not need medical or surgical treatment but require close follow-up until puberty.
Due to the lack of spontaneous testicular descent after the age of 1 year, and because of the potential loss of testicular quality, it is recommended that surgical orchidolysis and orchidopexy are performed at the latest by 12-18 months of age. The evidence to date suggests that hormonal treatment, either pre- or post-operatively, may have a beneficial effect on fertility later in life.
Definitions:
Levels of Evidence
1a Evidence obtained from meta-analysis of randomized trials
1b Evidence obtained from at least one randomized trial
2a Evidence obtained from at least one well-designed controlled study without randomization
2b Evidence obtained from at least one other type of well-designed quasi-experimental study
3 Evidence obtained from well-designed non-experimental studies, such as comparative studies, correlation studies and case reports
4 Evidence obtained from expert committee reports or opinions or clinical experience of respected authorities
Grades of Recommendation
- Based on clinical studies of good quality and consistency addressing the specific recommendations and including at least one randomized trial
- Based on well-conducted clinical studies, but without randomized clinical studies
- Made despite the absence of directly applicable clinical studies of good quality
