Nicklaus Children’s Hospital, Miami, FL, USA
KeywordsLaparoscopyTesticular diseaseCryptorchidismVaricocelePediatric surgeryPediatric urologyGeneral surgeryOrchiopexyUndescended testicles
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Cryptorchidism or undescended testes (UDT) is the failure of one or both testes to descend from the pararenal embryological origin to the base of the scrotum. It is one of the most common congenital abnormalities found in newborn males and affects mainly preterm males . It may be located in a non-palpable, intra-abdominal position or may be palpable in an intracanalicular, suprascrotal, or ectopic position. If uncorrected, boys with UDT are at a higher risk of infertility and malignanc y [2–5]. It is therefore important to identify these patients early and ensure follow-up so that orchiopexy can be performed in a timely manner if spontaneous descent does not occur.
The incidence of cryptorchidism is 4–5 % for full-term boys and as high as 30 % in premature boys [1, 6], of which 10 % are bilateral . At 1 year of age, the incidence reduces to as low as 0.8 % . Children with cryptorchidism have a higher risk for malignancy (seminoma) in the ipsilateral testis with a 10–30 fold increased risk , as well as increased risk of malignancy in the contralateral testis . This increase in malignancy may be decreased if children undergo an orchiopexy before they reach puberty .
It is unclear if cryptorchidism is secondary to an innate testicular defect or if there is direct impairment of the descent mechanism. Genetic and environmental factors have been reported for both congenital and acquired nonsyndromic cryptorchidism. Barthold et al.  reported that reduced breastfeeding and increase in soy formula feeding are potential risk factors for acquired cryptorchidism, as hormonally active components of breast milk and soy formula could influence the establishment of normal testis position in the first months of life, leading to apparent ascent of testes in childhood.
Male Sexual Development
The development and descent of testes are dependent on the interaction of multiple chemical and mechanical factors. In humans, gonadal differentiation begins during 6th week of gestation when bipotential gonadal tissue starts undergoing feminization or masculinization. In males, the SRY gene on the Y chromosome produces testes differentiating factor (TDF) that induces the bipotential gonadal tissue to become testes. Sertoli cells in the testes secrete anti-Mullerian hormone that leads to involution of the paramesonephric (Mullerian) duct, which would otherwise result in female internal reproductive structures. Leydig cells in the testes produce testosterone that stimulates the mesonephric (Wolffian) duct to become male internal genitalia. Testosterone is converted to dihydrotestosterone (DHT) that leads to development of external male genitalia.
Testicular descent is required to lower the temperature of the testes by 2–3 °C below body temperature in order for spermatogenesis to occur. Testicular descent is subdivided into the transabdominal migration of testes and the transinguinal descent into scrotum.
Definitions and Classificatio n
A testis is defined as undescended if the distance between the top of the pubic crest and the middle of the testes is less than 4 cm in mature boys or less than 2.5 cm in premature boys . They may remain in the abdominal cavity or they may be palpable in the inguinal canal (intracanalicular) or just outside the external ring (suprascrotal) . Testes which in early childhood are located in a scrotal position but then ascend are called acquired undescended testes [12–14]. A retractile testis represents a normal variant of testicular position ; it is not found in the lower part of the scrotum, as the testes is pulled cephalad by the cremasteric muscle, but can be manipulated without pain into the scrotum and remains in place after release . An absent testis may be due to agenesis or atrophy secondary to intrauterine vascular compromise, also known as the “vanishing testis syndrome” or testicular regression syndrom e .
The diagnosis is mainly by physical exam. In the case of an impalpable testis, it is important to examine the perineum , the base of the penis, and the high thigh, all rare but described positions of ectopic gonads. Imaging, such as ultrasound (US), can aid in the localization, though imaging studies have yield low sensitivity and specificity.
Medical Managemen t
Spontaneous testicular descent usually is completed by 6 months of age , and by 1 year of age, the incidence decreases to 0.8 %, possibly secondary to decreasing testosterone levels starting 3–4 months postnatally . Therefore, it is acceptable to observe patients, particularly preterm patients, for up to 6 months of age.
The main challenge of surgical repair is tension-free placement of the testes in the scrotum while avoiding damage to the testicular vessels and atrophy of the testicle. In 1959, Fowler and Stephens described a technique for high UDTs; this technique divides the foreshortened testicular artery, allowing the mobilization of the testis to the scrotum . This was later modified in 1984 by Ransley et al. who described a two-stage technique. First, a preliminary vessel ligation is performed, allowing development of collateral vessels; 6–8 weeks later, the testis is placed in the scrotum .
Diagnostic laparoscopy for UDT was first introduced by Cortesi in 1976 and is currently the gold standard for the diagnosis of a non-palpable testis . The laparoscopic approach for cryptorchidism was first described in 1994  and has replaced the combined inguinal/retroperitoneal open approach.
A UDT may be situated along its normal route of descent or in an ectopic position . Therefore, the role of the physician is to differentiate between palpable or non-palpable and uni- or bilaterally UDT, which will help determine the treatment algorithm . Assessment of UDT may be difficult in an awake and uncooperative child or in an obese child with a large suprapubic fat pad. Also, distinguishing between a testis and thickened gubernaculum can be challenging, perhaps requiring additional examination in a follow-up visit. The baby is positioned supine in a relaxed environment, and each side is assessed with the index finger sliding from above the inguinal area to block off the external ring. If the testis is identified, it should be grasped by the opposite hand and pulled gently down to the scrotum. Once in the scrotum, it is released. If the gonad stays in the scrotum, the diagnosis of retractile testicle is made, and no surgical procedure is warranted. Should there be any question regarding retractile versus cryptorchid testis , a reevaluation can be scheduled. A non-palpable testis could represent testicular agenesis/dysgenesis, an intra-abdominal UDT, or atrophy from antecedent torsion, the distinctions between them made at laparoscopy. A helpful clinical finding is that in the presence of testicular atrophy , the contralateral testis is often hypertrophied .
Laboratory tests are not routinely performed. In older boys, semen analysis can be performed to establish a baseline and subsequently during follow-up to assess sperm quality and motility after surgery. In cases where there is bilateral non-palpable UDT, a human chorionic gonadotropin (HCG) simulation test is helpful to determine if there is any functioning testicular tissue . On occasion, full descent will be noted after HCG, allowing the clinician to arrive at the diagnosis of retractile testes. While the testicles will recede as the hormonal stimulation dissipates, the condition will correct itself at puberty.
Imaging modalities such as US, computed tomography, and magnetic resonance have been used, although these have low sensitivity and specificity in localizing UDT [2, 3]. Even if no intra-abdominal testes are identified with imaging, their presence is not excluded in an intra-abdominal location [2, 3]. Laparoscopic evaluation is still advised, so many clinicians choose to skip imaging altogether in the case of impalpable testicles.
Surgical Indication s
Testicles above the scrotum at 6 months of age are unlikely to descend spontaneously, and surgery should be considered [4, 5]. The appropriate age of orchiopexy has decreased with time; initially orchiopexy was delayed until 5 years of age , though others recommended it before 2 years of age . Current recommendations deem that orchiopexy should be performed by age 1 year, as dysplastic changes in the gonad begin to show up at that time, although no improvement in testicular function has been reported.
The indications for surgery are non-palpable abdominal testis , high-canalicular testes, bilateral cryptorchidism, abdominal wall defects, polyorchidism, splenogonadal fusion, and transverse testicular ectopia [7, 24]. A laparoscopic approach may be contraindicated in the presence of extensive prior retroperitoneal or abdominal surgery, prior peritonitis, abdominal wall infection, large hernia, cardiopulmonary disease, or coagulopathy .
The testis is a paired organ surrounded by the tunica albuginea and subdivided into numerous lobules by fibrous septa. The seminiferous lobules open into the rete testis, where they join the efferent duct and epididymis, which drains to the vas deferens. The vas courses through the inguinal canal; the seminal vesicles join to form the ejaculatory duct, which opens before the prosthetic urethra. Arterial supply is via the spermatic, vas deferential, and external cremasteric arteries. Venous drainage is through pampiniform plexus, which drains into the internal spermatic vein; the right then joins the IVC, and the left drains directly into the renal vein. Testicular lymphatics drain into the retroperitoneal lymph nodes, the right into the interaortocaval area, and the left into the para-aortic area.
The operative management of a high intra-abdominal testis can be challenging due to the presence of a short spermatic cord that limits testis mobility and precludes a tension-free orchiopexy . The surgeon must be familiar with various techniques to help determine which option is best for each patient.
The patient is catheterized to empty the urinary bladder and then prepped from xiphoid to mid thighs.
The patient is positioned supine, and the surgeon will stand on the side opposite the patient’s undescended testicle, e.g., on the right side of the table for left cryptorchidism. After port placement, the table should be rolled so the side of cryptorchidism is elevated, allowing easier visualization of the retroperitoneum.
5-mm 30-degree laparoscope
Three 5-mm ports
Maryland and nontraumatic graspers
5-mm clip applier
After abdominal access is obtained through the umbilicus, a 5-mm port is placed, and pneumoperitoneum established.
Inspect the abdomen and abort if the vas and vessels are seen leaving the abdomen at the internal ring. A conventional groin exploration is indicated in this eventuality.
Look for the vas deferens at its origin and follow it toward the testicle.
Add two 5-mm working ports on the side of the abdomen contralateral to the affected gonad, lateral to the umbilicus on the side of the table where the surgeon is standing.
Assess, based on the position of the testicle near to or far away from the internal ring, the probability of getting the testicle well placed and tension-free in the scrotum with a single-stage procedure.
If a two-stage Fowler-Stephens orchiopexy is advisable, perform high endoclipping of the gonadal vessels and terminate the procedure.
If a one-stage procedure is chosen, carefully place cephalad traction on the testis to confirm that the epididymis and vas deferens do not loop into the inguinal canal, so that these are not injured during the following step.
Identify and transect the gubernaculum testis to allow complete mobilization.
Place traction on the peritoneum and divide it medially to the gonadal vessels, over the median umbilical ligament, and then over the bladder.
Similarly, the peritoneum lateral to the vessels is dissected as high as possible lateral to the psoas edge.
This should free up the peritoneum over the gonadal vessels; continue dissection to the root of the small bowel mesentery.
Using a Maryland grasper, push over the pubic bone while inverting the scrotum.
Identify the external inguinal ring, which is the thinnest area.
Push the Maryland grasper gently through the anterior abdominal wall.
Make an incision in the scrotum to create a sub-dartos pouch.
On the outside of the body, insert a Maryland grasper all the way through a 5-mm trocar, so that the trocar is pushed up against the handle of the instrument.
Push the Maryland grasper through the scrotal incision into the abdomen through the hole you have made from above and advance the 5-mm trocar through the scrotum into the abdomen.
Remove the Maryland dissector.
Introduce a laparoscopic nontraumatic grasper and grasp the testicle, paying attention not to grasp the epididymis or vas deferens and verifying that the vessels are not twisted.
Pull the testis through the neo-canal into the trocar and withdraw it with the trocar into the scrotum, being careful to gauge the tension on the vascular pedicle.
Grab the testis using a DeBakey forceps and place anchoring stitches between its capsule and the dartos pouch using 3-0 Prolene.
Deflate the abdomen and assess the testicle’s position.
If the length is insufficient to pull the testes into the scrotum, consider a Fowler-Stephens orchiopexy ; using an endoclip applier, clip and transect the testicular vessels as high as possible, which should give more length, while the gonad draws its blood supply from collaterals derived from the vas.
Reinsufflate the abdomen and assess for hemostasis.
Deflate the abdomen, remove trocars, and close port sites and scrotal skin overlying the testicle.
The peritoneum around the internal ring of the inguinal canal will allow the development of collateral blood supply to the testis . During the second-stage dissection of the medial aspect of the peritoneum at the level of the internal ring, it is important to preserve the cremasteric artery, as this will contribute to the development of testicular blood supply . If ligation of the vessels in the spermatic cord is necessary to gain length, high ligation preserves the collateral vessels between the testicular and deferential arteries .
If the testicle is so high that a Fowler-Stephens procedure seems inevitable, high division of the testicular pedicle before any peritoneal dissection with return to do the orchiopexy in a few months often allows the collaterals the best chance to develop via the artery of the vas and the cremasters.
If the vas deferens ends blindly, with no testicle at its end, the gonad must still be sought by identifying the distal end of the testicular artery, usually very high in the abdomen. This gonad can be simply resected to remove the risk of malignant degeneration in a testicle which will have no spermatogenic function.
Testicular atrophy , if it occurs, is usually evident 6 months after the procedure, although it can be delayed until a year postoperatively.
Recurrent cryptorchidism may be secondary to insufficient mobilization or inadequate testicular fixation [23, 27]; it can be corrected with a second-stage orchiopexy, performed at least 6 months after the initial procedure .
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