The term ascending testis is applied to two clinical conditions:

Agarwal and colleagues have presented results from the first longitudinal study of retractile testes in the USA; they showed that the retractile testis has a significant risk (32 %) of becoming an ascending testis; however, other studies report much less incidence, 2 % [2].

The incidence of secondary ascending testis (postoperative testicular ascent) is 0.8–2.8 % in boys who undergo surgery for inguinal hernia [3].

It has proposed criteria to differentiate an incompletely descended testis from a retractile testis: (1) Incompletely descended testes are smaller than the contralateral gonad. (2) The testis rapidly retracts out of the scrotum when the testis is released. (3) Pain is elicited when the testis is manipulated into the scrotum.

To diagnose an ascending testis, several criteria must be satisfied:

Ectopic testicle was also reported at other abnormal rare positions like the undersurface or the dorsum of the penis, the anterior abdominal wall below the umbilicus, and near the kidney or at the suprarenal glands [10].

One case of intrascrotal supernumerary testicle along with hypospadias was reported by Zur Kenntnis [11].

Perineal ectopic testis is a rare congenital anomaly in which the testis is abnormally situated between the penoscrotal raphe and the genitofemoral fold; it represents approximately 1 % of all undescended testicles [12] (Fig. 5.3).

Theories based on embryological development attempt to explain the occurrence of ectopic testes. During intraembryonic life, an intra-abdominal gubernaculum develops and terminates in the inguinal region. It guides the testis in transabdominal descent to this area. Later, an extra-abdominal gubernaculum part forms and grows from the inguinal region to the scrotal fundus, guiding the inguinoscrotal descent. Ectopic testes may arise out of failure of proper positioning of the extra-abdominal gubernaculums or its failure to form. If the testis deviates from its course through obstruction (obstructive theory), an ectopic also arises [13, 18].

Testicular ectopia is usually unilateral, but few cases of bilateral ectopia were reported [13] (Fig. 5.4).

Most authorities agree that testicular cancer is more common in a misplaced testis than in a normally descended organ. Currently, however, there are no satisfactory reports on whether the risk of testicular cancer in ectopic testis is higher than that of cryptorchidism.

Diagnosis is easily based on physical examination that reveals an empty, hypoplastic scrotum and a small mass lateral to it (Fig. 5.5).

Ultrasound is diagnostic in almost all cases; even antenatal study reported to pick up few cases; sometimes MRI is indicated, and laparoscopy proved to be useful to diagnose rare cases of ectopy in babies with impalpable testicle [14].

Perineal ectopic testis is frequently associated with an inguinal hernia and can sometimes be associated with other disorders such as hypospadias and scrotal anomalies. A case of bilateral perineal ectopic testis was reported.

Echography can also study traumatic lesions and testicular vascularization. Perineal testis can be discovered, while a complication like torsion or hernia strangulation is the same as undescended testis [15].

Treatment is surgical and there is no indication for hormonal therapy; most authorities agree that testicular cancer is more common in a misplaced testis than in a normally descended organ. Currently, however, there are no satisfactory reports on whether the risk of testicular cancer in ectopic testis is higher than that of cryptorchidism.

Thus, education about testicular self-examination and long-term follow-up of these patients are just as important as operative procedures, and it’s indicated as soon as the diagnosis is affected. There is no need to delay surgery in perineal ectopic testis and there is no need to wait for probable descent. Surgery is indicated even if there is no hernia present because of nonnegligible traumatisms and complications. The testis is relocated and fixed into the scrotal dartos pouch through an inguinal incision. Orchidopexy is usually easy to perform so the length of the testicular vessels and vas deferens is adequate [16].

The gubernaculum was usually found to lie fixed to the perineum, and the ectopic testis and empty scrotum are often well developed. Perineal incision is not necessary and retroperitoneal dissection is not required while perineal adherences are frequent. The long-term prognosis seems to be excellent for some authors; because of the histopathological features involved, prognosis is better than that of cryptorchidism. In fact the natural history of the ectopic testis has been described as normal or less abnormal than that of a truly cryptorchid testis. While Hutcheson, in a comparative study, has found similar pathological findings in ectopic and undescended testis and suggests that ectopic and undescended testis are variant of the same congenital anomaly. What’s sure is that an early detection of the ectopy and its surgical repair on time favored the functionality of the testis [17].

It is an uncommon anatomical abnormality in which both the gonads migrate toward the same hemiscrotum; only about a hundred cases have been reported in literature. The ectopic testis may lie in the opposite hemiscrotum, in the inguinal canal, or at the deep inguinal ring. An inguinal hernia is invariably present on the side to which the ectopic testis is migrated.

Variations in the anatomical position of the vasa deferentia and abnormalities of insertion of the vas into the testis can occur. Fusion of vasa deferentia has been described previously also based on the presence of various associated anomalies,

The largest incidence is found in Europe and Japan.

The most frequent anomalies associated with CTE are inguinal hernia, persistent Müllerian remnants, and incomplete descent of the nonectopic testis.

Persistent Müllerian duct remnants—tubes, rudimentary uterus, and hemiuterus—occurred in 38 % of TTE cases.

TTE has been classified into three types:

The mean age at presentation is 4 years. In most of the cases, the correct diagnosis was not made preoperatively and the condition was revealed during herniotomy. Recently, MRI and MR venography have been suggested for preoperative location of impalpable testis. Laparoscopy is useful for both diagnosis and management of TTE and the associated anomalies [22, 23]. Treatment includes transscrotal orchidopexy or extraperitoneal transposition of the testis, search for Müllerian remnants and other anomalies, and long-term postoperative follow-up. Like all dysgenetic testes, infertility and progression to malignancy are relatively frequent with TTE [24].

Associated Müllerian duct structures, if any, should be ruled out by laparoscopy or minilaparotomy in the same sitting.

Persistent Müllerian duct remnants—rudimentary uterus and hemiuterus—occurred in 38 % of CTE cases. Persistence of Müllerian remnant in phenotypically normal males represents a recessive trait with male sex restriction, in chromosome 19, resulting in abnormal Müllerian-inhibiting substance (MIS) receptors, or inactive forms of MIS or even inadequate synthesis of MIS, by the fetal testis (Fig. 5.7).

Crossed testicular ectopia is a rare congenital anomaly in which both testes are located in one hemiscrotum as a result of deviation from normal testicular descent. In most cases the right testis is found in the left hemiscrotum. The ectopic testis has its own spermatic cord (vas and blood vessels), which originates from the appropriate abdominal site but descends to the opposite side after passing anterior to the urinary bladder [27].

Monorchism is a normal phenomenon in nonhuman animals, notably in beetles.

Although some patients with anorchia present with ambiguous external genitalia or microphallus, most have a normal phenotype.

But many cases are associated with other urinary or anorectal and limb anomalies Fig. 5.8.

The basal plasma concentrations of anti-Müllerian hormone (AMH), inhibin B, and testosterone were very low or undetectable with high level of FSH hormone and, together with 46, XY complement, are sufficient for diagnosis of anorchia, and the hCG test is not necessary [26].

There are three theories to explain congenital absence of the testis: (a) absence of testicular development during fetal period, (b) discontinuation of vascular supply to the testes during fetal period, and (c) atrophy caused by intrauterine testicular torsion [27].

Discontinuation of testicular vascularization during fetal period would occur by extravaginal torsion (spermatic cord torsion) that would be the most frequent mechanism involved in testicular agenesis.

Absent testicles (acquired anorchia) could be due to testicular regression syndrome where dystrophic calcification and hemosiderin deposits are found at the site of the testis, with no evidence of viable testicular tissue, but with relatively normal spermatic cord elements.

The genitalia of these patients varied from a female appearance, ambiguous external genitalia, to normal male aspect and anorchia.

Anti-Müllerian hormone is used to facilitate the evaluation of intersex disorders and as a marker of ovarian reserve assessment in the infertility cases. When the testes are non-palpable, a single measurement of serum AMH level can distinguish between cryptorchidism and anorchia.

Diagnostically, the lack of testosterone response to hCG stimulation is the hormonal hallmark of bilateral congenital anorchia.

Ultrasound is unreliable in distinguishing between anorchia and intra-abdominal testis, giving both false-positive and false-negative findings, but it is sometimes helpful in visualizing a testis within the inguinal canal. Magnetic resonance imaging (MRI) is also unhelpful alone, although more reliable when combined with angiography, but this requires a general anesthesia.

Consequently, laparoscopy remains the investigation of first choice in cases of impalpable testis, being both highly reliable and providing positive guidance for further management. In this regard, there are five possible laparoscopic findings.

Excision of any testicular and vas remnants is recommended in cases of anorchia, and implantation of testicular processus at the adult age could be considered especially in bilateral cases, but the indication in unilateral case (monorchia) is controversial.

It is a genetic disorder found in males, characterized by abnormally small testes. The condition is associated with (and often secondary to) a number of other genetic disorders, including Klinefelter’s syndrome and Prader–Willi syndrome, as well as other multiple malformation disorders. The degree of abnormality of the testes can be determined by the use of an orchidometer. In addition, microorchidism may also occur as a result of shrinkage or atrophy of the testis due to infections like mumps and post trauma, in survivors from testicular torsion, and many cases after orchidopexy may have a small testicle.