Undescended testes: a clinical and surgical review.
Key Words: Undescended testis, cryptorchidism, orchidopexy, laparoscopy.
1. Explain the anatomy and physiology of cryptorchidism (undescended tests).
2. Discuss the evaluation process for determining undescended testis in a patient.
3. List treatment options of cryptorchidism/undescended testis before surgical procedures are considered.
4. Describe the impact cryptorchidism may have on the future health of male infants presenting with this disorder.
Undescended testis (UDT), also known as cryptorchidism, is the most common congenital disorder of the genitourinary system affecting male infants (Dayanc et al., 2007). The incidence of UDT is 2% to 4%, but as many as one third of pre-mature newborn males are affected (Ferguson & Agoulnik, 2013). The true pathophysiology of UDT is not fully understood, although several theories exist. Clinical research conducted as early as the 1940s demonstrated that uncorrected UDT has profound implications, particularly on fertility potential and malignant transformation of some testicular cell lines (Ferguson & Agoulnik, 2013). A contemporary perspective on the pathophysiology of the undescended testis, the impact on testicular function, and risk of testicular cancer, as well as current guidelines regarding optimal timing for surgery and types of surgical procedures, are presented.
Anatomy and Pathophysiology
The word "cryptorchidism" implies a hidden testis (Kraefer, 2004). The testis may prove to be absent, truly undescended, in an ectopic location, or ascended (Kolon, et al., 2014). The term serves as a broad clinical category and includes a variety of conditions. Thus, the use of precise terminology to characterize specific physical findings on examination is critical for accurate diagnosis and treatment planning. UDT can be divided into primary or secondary types. In primary UDT, the testis failed to complete its migration from its para-renal embryological origin to the scrotum. Instead, it may be located in a non-palpable intra-abdominal position or may be palpable in an intra-cannicular position, suprascrotal position, or an ectopic position (outside of the normal path of descent). In secondary UDT, a testis that had previously been in a scrotal position can be pulled into a supra-scrotal position as a result of scarring, often after surgical repair of an inguinal hernia (Steinbrecher, 2014). Retractile testis is a separate diagnosis, defined as a testis that retracts secondary to a brisk cremasteric reflex. It has been cited as a confounding diagnosis for UDT because clinically, the two entities may be difficult to accurately distinguish on examination (Kollin, Granholm, Nordenskjold & Ritzen, 2013). Additionally, the term "ascended testis" describes a testis, often a previously retractile testis, once documented in a scrotal position that has subsequently ascended to a supra-scrotal position spontaneously (Steinbrecher, 2014).
A familiarity with normal testicular descent facilitates a better understanding of UDT. Testicular descent to the scrotum occurs over three phases (Bay, Main, Toppari, & Skakkebaek, 2011). The first phase (transabdominal phase) begins between gestational weeks 10 to 15 and is usually completed by gestational weeks 22 to 25 (Bay et al., 2011). Under the influence of insulin-like hormone 3 (INSL3), the testes are pulled caudally by the gubernaculum, finally coming to rest in the lower abdomen at the internal ring, the developing internal entrance to the inguinal canal (Hutson et al., 2013). The second phase of descent (trans-inguinal phase), occurs between gestational weeks 25 to 30 and is a rapid process wherein the testes migrate through the short inguinal canal to the external inguinal ring (Bay et al., 2011). The third phase of descent (scrotal phase) occurs as the pregnancy approaches term during gestational weeks 30 to 35 (Bay et al., 2011). Governed in part by androgen production, the testes travel from the external inguinal ring to the final position in the scrotum (Bay et al., 2011). During descent, the testes and the cord structures are enveloped in layers of the abdominal wall, and thus, the coverings of the testicles and cord structures correlate with the anatomy of the abdominal wall (Tanagho & McAninch, 2000). The innermost layer, the tunica vaginalis, represents the evagination of the peritoneum (Tanagho & McAninch, 2000). The internal spermatic fascia is derived from the transversalis fascia (Tanagho & McAninch, 2000). The cremasteric muscle and its fascia arise from the internal oblique muscle and finally the external spermatic fascia represents the external oblique muscle (Tanagho & McAninch, 2000). The staged nature of testicular descent demonstrates why the prevalence of UDT is so much higher among premature neonates because preterm delivery interrupts the natural progression of the testes.
In anatomical studies, UDT is often associated with other anomalies of development. It is most frequently associated with inguinal hernias and epididymal anomalies (Favorito, Costa, & Sampaio, 2006). Inguinal hernias develop secondary to a patent processes vaginalis. This tract obliterates during the course of normal testicular descent, but in the setting of an UDT, a patent tract may persist and pose a risk for development of an inguinal hernia. The incidence of epididymal anomalies range between 36% to 79% and was noted to be more frequent in the setting of a patent processus vaginalis (Favorito et al., 2006). Epididymal anomalies are classified based on variation in the testicular-epididymal anatomical relationship (Marshall, 1982). Traditional definitions identify normal anatomical relationships as a connection between the testis and epididymal head and tail (Type 1) or along the entirety of the epididymis (Type 2) (Marshall & Shermeta, 1979). Abnormal anatomical relations include a connection between the epididymal head only (Type 3) or tail only (Type 4), total disjunction (Type 5), and epididymal atresia (Type 6) (Marshall & Shermeta, 1979). These abnormal anatomical variants may impede the transit of sperm and may have implications for future fertility (Mollaeian, Mehrabi, & Elahi, 1994).
Evaluation of UDT
Clinically, the presence of unilateral UDT is more common than that of bilateral UDT. Approximately 80% of cases are unilateral, while the remaining 20% represent bilateral UDT (Singh, Hamada, Bukavina & Agarwal, 2012). Identified risk factors for UDT include premature birth, low birth weight, family history of UDT, and maternal alcohol or tobacco use (Mayo Foundation for Medical Education and Research, 2014). The strongest of these is believed to be prematurity and the incidence of UDT among premature neonates, which has been reported to be greater than 30% (Kollin et al., 2013). Among both term and preterm neonates, many cases of UDT spontaneously descend postnatally. One study found that of cases with spontaneous descent, 82% descended by two months of age, an additional 10% by six months of age, and another 8% by 12 months of age (Kollin et al., 2013). This trend has been validated by other studies, suggesting that most postnatal UDT spontaneously descend by three months (corrected for gestational age) (Kollin et al., 2013). Recent American Urological Association (AUA) guidelines highlight that a UDT is very unlikely to spontaneously descend after six months (corrected for gestational age) and recommend referral to a specialist if the testis is not completely descended by six months of age (Kolon et al., 2014).
A careful physical examination is key to establishing the most accurate diagnosis as well as appropriate therapy. A practical approach to the examination begins with determination of whether the UDT is palpable or non-palpable (Tekgiil et al., 2012). To examine for a UDT, the examiner positions the non-dominant hand at the anterior superior iliac spine and sweeps in a lateral to medial direction towards the groin. To assist with this maneuver, a small amount of lubricant jelly or wet soap may be applied to the abdomen and the procedure repeated ("the soap test"). The dominant hand should be positioned interiorly to catch the testis, and if possible, anchor it in the scrotum for one minute to exhaust the cremasteric reflex. If upon release the testis remains in a scrotal position, it should be declared a retractile testis. Retractile testes result from a brisk cremasteric reflex and usually resolve by puberty, after which the reflex is dampened (Kollin et al., 2013). Ascended and possibly retractile testes may also be at risk for germ cell maldevelopment and diminished fertility because both demonstrate similar histopathological patterns compared to UDT (Rusnack et ah, 2002). While they do not require surgical correction, retractile testes should be monitored at least annually for resolution because they are at higher risk for subsequent ascent (Kollin et ah, 2013). Studies demonstrate between 2% to 45% of retractile testes may ascend permanently, ultimately requiring surgical intervention (Mayr et ah, 1995). If a palpable testis cannot be coaxed to the scrotum or immediately ascends upon release of traction, it is considered to be a UDT and warrants surgical intervention.
A non-palpable UDT requires additional evaluation. A thorough regional examination of the femoral, penile, and perineal areas is required to rule out the possibility of an ectopic testis (Tekgiil et ah, 2012). In the setting of unilateral UDT, the contralateral testicle should be examined for signs of enlargement. This could represent compensatory hypertrophy and suggests complete absence of the other testicle. The compensatory reaction of the normal testis serves to support normal growth and development despite the lack of a second testis (Laron & Zilka, 1969). The finding of con tralateral testicular size greater than 1.8 cm in a pre-pubertal boy with a nonpalpable UDT has been demonstrated to be highly indicative of prenatal testicular loss and may impact a clinician's choice of surgical approach (Keays, Lorenzo, Bush, Leonard, & Snodgrass, 2013).
The presence of bilateral non-palpable UDTs is an indication for evaluation for a disorder of sexual development (DSD). The patient should also be inspected for other genitourinary abnormalities, such as hypospadias or signs of feminization, which may also suggest DSD. If there is concern for DSD, immediate referral to both a pediatric urologist and pediatric endocrinologist is recommended (Kolon et al., 2014). Congenital adrenal hyperplasia needs to be ruled out in the phenotypic male with bilateral non-palpable UDTs. In addition, one should assess for the presence of any functioning testicular tissue. Electrolytes, mullerian inhibiting substance (MIS) level, testosterone, and a karyotype should be obtained in such infants (Kolon et al., 2014). While approximately 85% of UTDs are isolated clinical conditions, 15% of patients may present with UDT as part of a larger syndrome (Singh et al, 2012). Some of these syndromes involve disorders of the central hypothalamic-pituitary axis (Kallman syndrome, Prader-Willi syndrome), while others involve congenital weakness of the abdominal musculature (Eagle-Barrett syndrome) or an abdominal wall defect (gastroschisis) (Singh et al., 2012).
The evaluation of UDT is clinical, with no initial role for imaging modalities. Diagnostic laparoscopy is the current gold standard to either locate an intra-abdominal testis or confirm the presence of a blind-ending spermatic cord (absent testis), which is associated with testicular regression syndrome (Pirgon & Dundar, 2012). Ultrasound, CT scan and MRI imagining studies have all been investigated to determine if pre-surgical imaging could assist with identifying and localizing non-palpable testes (Tasian, Copp, & Baskin, 2011). Having a reliable and accurate form of imaging could prevent unnecessary surgery in the event of an absent testis or at least reduce the surgical burden by providing additional information to guide providers' choice of surgical approach. However, both original research by Elder (2002) and a recent review by Tasian et al. (2011) examined the utility, indications, and effectiveness of diagnostic imaging, and found no evidence to suggest a role for imaging in routine evaluation of UDT.
Ultrasound is a relatively inexpensive, noninvasive, and accessible form of imaging, and therefore, it is commonly used in evaluation of UDT (Elder, 2002). The review by Tasian and colleagues (2011) found that 67% of U.S. general pediatricians reported ordering imaging during the evaluation of boys with UDT, and of those, 94% utilized ultrasound (Tasian et al., 2011). Despite its common use, ultrasound does not reliably localize non-palpable testes or provide additional information to direct surgical approach (Elder, 2002), and thus, its use is discouraged. Even though the physical examination of an obese patient may be more difficult, Tasian et al., still found similar accuracy of exam under anesthesia as compared to non-obese patients. Thus, no additional evidence to warrant imaging was recommended even in that population (Tasian et al., 2011). The most recent AUA guidelines reiterate the same opinion and discourage the practice of imaging in the routine evaluation of UDT because it does not provide additional information or help guide treatment decisions (Kolon et al, 2014). An exception to these recommendations is in the evaluation of a patient with bilateral UDT and signs of ambiguous genitalia. In that setting, ultrasound can be utilized to assess for Mullerian structures (cervix and uterus) (Ogilvy-Stuart, & Brain, 2004).
Management of UDT
The only definitive management for an undescended testis is surgical intervention. Hormonal treatments have been used to encourage descent of the UDT. Both human chorionic gonadotrophin (hCG) and gonadotrophin-releasing hormone (GnRH) have been used, with proposed mechanisms of utilizing androgen influence to encourage descent. These medical therapies appear more appropriate for patients with low-level UDT and retractile testes, but success rates are still poor (Tekgiil et al., 2012). One recent systematic review demonstrated that hormonal therapies performed only marginally better than placebo, with maximal success rates of approximately 20% (Penson, Krishnaswami, Jules, & McPheeters, 2013). The role of using hormones as a neoadjuvent therapy in conjunction with surgery to improve fertility outcomes has become a recent topic of interest. A randomized controlled trial assessed the fertility indices of pre-pubertal males with UDT who were pre-operatively treated with four weeks of GnRH compared to a control group. Results found that testicular biopsies taken at the time of orchiopexy demonstrated a higher fertility index in the pre-treated group (Schwentner et al., 2005). There are, however, no long-term studies to date demonstrating whether this has implications for fertility in adulthood. Given the significant consequences of UDT on fertility and potential malignancy, definitive and corrective surgical therapy has become the mainstay of treatment for UDT. Therefore, hormonal therapies are no longer recommended by the most recent AUA guidelines (Kolon et al., 2014).
Once the decision is made to proceed with surgery, the office nurse is critical in the pre-operative and post-operative preparation. Parents are often fearful of anesthesia, particularly with infants, and of the long-term effects of an undescended testis. The urology nurse can help provide information to the parents to alleviate their anxieties, identify those who may benefit from having child life services present the day of surgery, and discuss the typical perioperative experience. The office nurse will often be the first clinician responding to parent questions. Consequently, it is important that the urology nurse understand the pediatric urologist's post-operative care and the impact that cryptorchidism may have on fertility and testicular cancer.
While surgery is the only definitive treatment for true UDT, the patient should always undergo an examination under anesthesia (EUA) at the start of the case. With the benefit of a general anesthetic to relax the patient, examination may reveal a palpable UDT or a retractile testicle (Zakaria et al., 2013). The subsequent change in diagnosis may obviate the need for surgery or alter the surgical approach, and thus, is recommended (Kolon et al., 2014).
The surgical approach to the palpable UDT may be through an inguinal incision (standard approach) or a high scrotal incision. The location of the testis and the surgeon's preference determine the approach used. The high scrotal approach as described by Eltayeb (2014) involves a single high scrotal incision, dissection of the sub-dartos pouch, and then extension of that dissection cranially to the entrance of the external ring. After obtaining access to the testicle and cord structures, the remainder of the procedure resembles a traditional orchidopexy (Eltayeb, 2014). One randomized controlled study demonstrated a significantly shorter operative time using the high scrotal approach but no difference regarding post-operative complications (Eltayeb, 2014). A systematic review by Novaes, Carneiro Neto, Macedo, & Barroso Junior, (2013) investigated efficacy and safety of the high scrotal approach found similar results, with efficacy ranging between 88%-100% depending on location of the UDT Additionally, proponents of the high scrotal approach acknowledge that it may be better suited for more distally located UDT as cases where the UDT is proximal to the external ring required conversion to traditional inguinal approach (Eltayeb, 2014). The same study demonstrated overall success rates of 91.4% but only a 66.6% success rate for proximally located UDT (Eltayeb, 2014).
Our practice traditionally uses an inguinal incision because it provides excellent exposure and ability to easily ligate the patent processus vaginalis, if present, at the level of internal inguinal ring. Further, it is our experience that the inguinal incision, when made in a normal skin crease, results in a cosmetically appealing scar and with a small incision minimizes postoperative discomfort. During the mobilization of the cord structures, dissection of the cremasteric muscle fibers and the lateral spermatic fascia is performed to gain cord length. The Prentiss maneuver can also be performed to obtain additional cord length. This maneuver involves either ligating the inferior epigastric vessels or routing the UDT beneath the vessels, resulting in a more medial course that may allow the testicle to be brought down to the desired anatomical location (Sfoungaris, Mouravas, Petropoulos, & Filipppoulos, 2012). The success rates of such open procedures are greater than 96% (Kolon et al., 2014). Post-operative testicular atrophy occurs in less than 2% of cases and varies based on the preoperative location of the testis, with ectopic testes being at greater risk than testes in the inguinal canal (Ein, Nasr, Wales, & Ein, 2014).
A non-palpable UDT is typically managed laparoscopically, either via a single-stage laparoscopic orchiopexy or two-stage (Fowler-Stephens) laparoscopic orchiopexy (Mehendale, Shenoy, Shah, Chaudhari, & Mehendale, 2013). The decision to proceed with a one-stage or two-stage orchiopexy depends on the location of the UDT and the length of the cord. UDT can be classified as low-level, high-level, or peeping. If located closer to the internal ring of the inguinal canal, the UDT is considered low-level because the testis has already achieved some descent. In this situation, a single-stage laparoscopic orchiopexy has been shown to achieve good results (Mehendale et al., 2013). A peeping testis is a UDT that is inconsistently palpable. The testes intermittently "peep" through the inguinal canal, at times palpable, and at other times becoming nonpalpable (Elderwy et al., 2014). Management can utilize either an open or laparoscopic approach with fairly comparable results although laparoscopy has been shown to be slightly more effective (Elderwy et al., 2014).
If the UDT is located closer to the iliac vessels, it is classified as high-level. If traction on the scrotum and gubernaculum does not pull the testis into the inguinal canal, the first stage of a two-stage Fowler-Stephens is performed. By clipping the testicular vessels and allowing collateral vasculature to develop based on the vasal vessels (stage one), the testis can be mobilized with greater freedom and surgically positioned in the correct anatomical position four to six months later (stage two) (Mehendale et al, 2013).
Studies comparing the laparoscopic one- and two-stage Fowler-Stephens procedures demonstrate success rates of 78.7% and 86%, respectively (Kolon et al., 2014). While outcomes are similar between open and laparoscopic approaches, one recent meta-analysis demonstrated significantly decreased morbidity associated with the laparoscopic approach (Guo et al., 2011). In management of high-level UDT, one-and two-stage Fowler-Stephens orchiopexy demonstrated success rates of 80% and 85%, respectively, favoring a two-stage approach with respect to postoperative testicular viability (Elyas et al., 2010).
Nursing staff play a critical role in the operative treatment for the child with non-palpable UDT. While maintaining and stocking necessary surgical equipment are standard operating room protocol, the nurse, in our practice, also functions as the point person to update and notify parents of intraoperative findings in children undergoing laproscopy.
In the event that the EUA confirms the existence of a nonpalpable testis, the nurse notifies the family that laparoscopy will be performed. A caudal nerve block is placed and the patient is placed in a supine position. A feeding tube or small French indwelling catheter is placed per urethra to drain the bladder and the anesthesiologist decompresses the stomach with an orogastric tube. The laparoscopic camera port is placed through an umbilical incision. At this time, this is the only port that is opened and utilized. The surgeon examines both internal rings (see Figures 1 and 2), identifies the location of the testis, if present, and determines whether a single or two-stage approach is needed. The circulating nurse notifies the parents of the findings and surgical approach. Familiarity with the length and steps of the procedure allows the OR nurse to respond appropriately to anxious parent's questions. Two additional laparoscopic trocars are placed, and the disposable clip applier are opened (see Figure 3). A laparoscopic babcock is used to grasp and retract the testicular vessels up toward the abdominal wall (see Figure 4) and two laparoscopic clips are applied (see Figure 5). Once the clips have been applied, the abdomen is inspected for bleeding and injury to intra-abdominal structures, the ports removed and the incisions closed and infiltrated with 0.25% bupivacaine hydrochloride injection (Marcaine[R]) if a caudal block was not placed at the start of the procedure.
Although not commonly performed, the AUA guidelines indicate that orchiectomy may be considered in select cases. In the setting of a normal contralateral testis, the provider may consider orchiectomy in the event of very short testicular vessels and vas deferens or a dysmoprhic or very hypoplastic testis (Kolon et al., 2014). In the event of dysmorphic or hypoplastc testis, an intra-operative testicular biopsy may be taken to assess for testicular viability and malignancy to determine whether salvage therapy or orchiectomy is more appropriate. Appropriate therapy is also somewhat dependent on age of the patient because it is recommended that pre-pubertal boys undergo orchiopexy, if possible (Kolon et al, 2014). For post-pubertal boys, the European Society for Pediatric Urology recommends orchiectomy instead of orchiopexy (Tekgiil et al., 2012). Histological examinations of surgical specimens from post-pubertal males with UDTs have confirmed the lack of normal spermatogenesis as well as the presence of carcinoma-in-situ (Rogers et al., 1998). Certain studies have demonstrated that the majority of those testes will not contribute to fertility, are at significantly increased risk for malignancy, and may be susceptible to torsions. Therefore, some experts advocate for orchiectomy (Kolon et al., 2014). Every effort should be made to bring the testis to the correct anatomical position because there is a paucity of data regarding the psychosocial effects for boys with only one descended testicle, and the UDT may still contribute to testosterone production. Our practice performs a testis biopsy on all post-pubertal UDTs at the time of orchidopexy. For a patient 32 years of age or older, a unilateral UDT does not require surgical intervention provided it is easy to examine (Rogers et al., 1998). Instead, it may be closely observed via annual examinations because the relative risk of developing a malignancy decreases after that age (Pettersson, Richiardi, Nordenskjold, Kaijser, & Akre, 2007).
The impact and adoption of early surgical intervention is under review. Studies have already started to investigate changes in clinical practice but have yet to demonstrate a significant trend towards earlier age of orchiopexy (Aggarwal, Rehfuss, & Hollowed, 2014; Bradshaw, Corbet-Burcher, & Hitchcock, 2014). However, those studies highlight potential areas for quality improvement, including continuing to emphasize early referral and educational sessions for primary care providers who are likely to encounter newborns with UDT (Aggarwal et al., 2014). Urologic nurses, pediatric urologists and pediatric urologic surgeons can help educate colleagues regarding these new guidelines to promote earlier surgical intervention in children with UDTs.
Impact of UDT
The studies in the post-pubertal population with UDT have helped support the case for earlier surgical correction to minimize the possible sequelae of infertility and testicular cancer (Banks et al., 2013; Hutson et al., 2013; Kollin et al., 2013). The rationale for earlier surgical correction is supported by the presence of permanent changes within the UDT, noted to develop as early as 15 months to 2 years of age (Cortes, Thorup, & Visfeldt, 2001). While there are competing hypotheses that strive to explain the histological changes noted in a UDT, the true mechanism responsible for subsequent infertility and malignancy remains an area of active research. One hypothesis, the common cause hypothesis (testicular dysgenesis syndrome), suggests that an unidentified etiology may be a common risk factor for both the development of UDT and abnormal cell development (Ferguson & Agoulnik, 2013). An alternative hypothesis, the position hypothesis, theorizes that the malposition of the testis in a supra-scrotal position exposes the testis to environmental factors that promote dysregulation of the cell-lines and increase the risk of infertility and malignancy (Cortes, Petersen, & Thorup, 2008).
Although the causative mechanism is not truly known, studies have demonstrated specific histological changes in UDT in both human and animal models. During normal development, neonatal gonocytes transform into adult dark spermatogonium at approximately six months of age (Hutson et al., 2013). Subsequently, some adult dark spermatogonium will further differentiate into primary spermatocytes, while others will remain as a pool of stem cells to support continual spermatogenesis. Others will undergo apoptosis as a function of normal cell-line regulation (Thorup, Kvist, ClasenLinde, Petersen, & Cortes, 2013). In UDT, the initial transformation from neonatal gonocyte to adult dark spermatogonium, as well as the controlled apoptosis of extraneous adult dark spermatogonium, are both inhibited; the former predisposes an individual to infertility, and the later to malignancy (Hutson et al., 2013).
Impact on Fertility
Studies have investigated infertility in patients with a history of UDT by examining histological samples, testicular volumes, and long-term follow up of fertility parameters (Huston et al., 2013; Kollin et al., 2013; Thorup et al., 2013; van Brakel et al, 2013). Biopsies taken at the time of orchiopexy demonstrated fewer germ cells per tubular cross-section in the UDT, and that finding was subsequently associated with increased risk of infertility (Cortes et al., 2008). Researchers have also been able to correlate the degree of histo-pathological changes with the location of the UDT, noting that an intra-abdominal testis demonstrates the most severe changes (Wilkerson, Bartone, Fox, & Hadziselimovic, 2001). In addition to histological findings, patients' testicular volume has also been assessed and has been shown to be an acceptable predictor of spermatogenesis (Kollin et al., 2013). Several studies have shown that patients with a history of UDT have a significantly lower testicular volume compared to both a control group, and if applicable, the individual's normally descended testis (Kollin et al., 2013; van Brakel et al., 2013). Additionally, one recent study demonstrated a clear and direct relationship between the duration of suprascrotal position and severity of testicular growth impairment (Kollin et al., 2013). In that study, patients who underwent early surgical intervention at the age of nine months had significantly larger testicular volumes compared to the patients who underwent late surgical intervention at the age of three years (Kollin et al., 2013). Fertility follow up using semen analysis has also demonstrated that men with a history of UDT have significantly lower semen concentration and motility as compared to controls (van Brakel et al., 2013). In combination, these findings suggest that early correction to optimize normal development of the testicular germline cells is crucial to maximizing future fertility potential. However, paternity and time to conception are the gold standard assessments of fertility, but those metrics are challenging to assess, requiring long-term cohort follow up (Wilkerson et al.. 2001).
Impact on Risk for Testicular Germ Cell Tumor
In addition to concerns about fertility, UDT has long been established as a risk factor for development of testicular germ cell tumors (TGCT), most commonly, seminoma (Banks et al., 2013). A recent meta-analysis has estimated that the relative risk for developing TGCT in men with a history of UDT is approximately 4%, considerably lower than historic reports that previously estimated relative risk as high as 18% (Banks et al., 2013). The same meta-analysis found that early orchidopexy resulted in a lower associated risk of developing TGCT (Banks et al, 2013). In addition, orchidopexy facilitates the ability for a physician to examine the testis for the development of malignancy; studies have shown that relative risk remains high even after surgical correction (Banks et al., 2013). The effect of earlier surgical intervention on the risk of malignant transformation remains to be seen as contemporary patient cohorts continue in studies with long-term follow up.
UDT and Risk of Testicular Torsion
Patients with UDT are also at risk for torsion (Mano, Livne, Nevo, Sivan & Ben-Meir, 2013). Torsion of a UDT is a urological emergency that is difficult to diagnose and requires prompt surgical treatment to salvage a viable testicle (Moore, Marr, & Huang, 2011). This potential complication has also been used to support the case for earlier surgical treatment of UDT (Mano et al., 2013).
UDT is a common congenital disorder with potential sequelae that include infertility and malignancy. Many cases of UDT spontaneously resolve within the first three months of life. Radiological studies have no role in the diagnosis of UDT or planning of surgical approach (Elder, 2002). For cases that do not spontaneously resolve, the current guidelines recommend referral to a specialist by six months of age to allow for earlier surgical correction of UDT (Kolon et al., 2014). This window allows for spontaneous descent because it may occur before six months of age, but also encourages surgical intervention early enough to optimize fertility potential and decrease risk of malignant transformation (Chan, Wayne, & Nasr, 2014; Kolon et al., 2014).
Surgical management is the gold standard treatment for UDT. The surgical approach varies with location of the UDT and surgeon preference. Use of an inguinal incision for palpable UDT and laparoscopic Fowler-Stephens two-stage approach for intra-abdominal UDT are preferred approaches in our practice. The goal of earlier intervention is to prevent further damage to the testes. In addition to optimizing fertility potential, earlier surgical intervention serves to both decrease the risk of cancer as well provide a palpable testis that can be readily examined in the future. The use of accessible and well-validated clinical recommendations will promote standardized care for patients with UDT, ideally reducing the risks of future complications for these patients as they age.
Urologic nurses and associates are key components of the surgical team, ensuring that the necessary equipment is readily available during the procedure, while also paying close attention to cost-containment. Prioritizing patient safety and facilitating communication with the family throughout the surgery is a crucial aspect of the nurse's role. The office nurse is critical to parent/patient education both preoperative and post-operative. A clear understanding of the procedure to be performed, the pediatric urologist's post-operative recommendations, normal postoperative healing course, and risks associated with an undescended testis (infertility, testicular cancer) are key for the office nurse to be able to effectively counsel and advocate for parents and patients.
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Jennifer Fantasia, BS, is a Senior Medical Student, the University of Massachusetts Medical School, Worcester, MA.
Jeremy Aidlen, MD, is an Assistant Professor of Surgery/Pediatrics, UMass-Memorial Medical Center, University of Massachusetts Medical School, Worcester, MA.
Wendy Lathrop, RN, is a Surgical RN and a member of the Pediatric Urology Surgical Team, UMassMemorial Medical Center, University of Massachusetts Medical School, Worcester, MA.
Pamela Ellsworth, MD, is a Professor of Urology; Vice-Chair, Department of Urology; Chief, Division of Pediatric Urology, UMassMemorial Medical Center, University of Massachusetts Medical School, Worcester, MA.
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|Title Annotation:||CNE SERIES|
|Author:||Fantasia, Jennifer; Aidlen, Jeremy; Lathrop, Wendy; Ellsworth, Pamela|
|Date:||May 1, 2015|
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