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The Undescended Testicle: Diagnosis and Management STEVEN G, DOCIMO, MLD, University of Bteburgh School of Meine Pitsburg, Pernsylvania RICHARD L SILVER, M.D, leffersoa Medical Calle of Thomas feteson Universi, Philp, Penns WILLIAM CROMIE, M.D, Univenty of Chieago Pritker School of Medicine, Chica, Tinos Early diagnosis and management of the undescencled testicle are needed to preserve fertil- ty and improve early detection of testicular malignancy. Physicat exemination of the testi- de can be difficult; consultation should be considered if a normal testis cannot be definitely jentified. Observation is not recommended beyond one year of age because it delays treat- ‘ment, lowers the rate of surgical success and probably impairs spermatogenesis. By six ‘months of age, patients with undescended testicles should be evaluated by a pediatric urol- ‘gist or other qualified subspecialist who can assist with diagnosis and treatment. Earlier referral may be warranted for bilateral nonpaipable testes in the newborn or for aay child with both hypospadias and an undescended testis, Therapy for an undescended testiie should begin between six months and two years of age and may consist of hormone or sur- gical treatment. The success of either form of treatment depends on the position of the tes- tice at diagnosis. Recent improvements in surgical technique, including ‘aparoscopic approaches to diagnosis and treatment, hold the promise of improved outcomes. While ‘orchiopexy may not protect patients from developing testicular malignancy, the procedure allows for earlier detection through self-examination of the testicles. (Am Fam Physician © 4 patient infor. mation handout on the undescended testicle, writen by the authors of os atti is provided fon page 2087, | | | thas been well documented that men with 12006;62:2037-44,2047-8) Tisarice was ders ——_ap-ey_ryprorchiism, or undescended pe unde the testicle, is usually diagnosed TESTICULAR CANCER. es ofthe Beda @ ae : Urology Assecation uring the newbom examin hers S tion, Recognition of the eondi- tion, identification of associ- ated syndromes, proper diagnostic evaluation and timely referral for urologic surgical ther= apy are important steps in preventing adverse ‘consequences. Consequences of Cryptorchidism ‘The rationale for treatment of the unde scendad testicle isthe prevention of potential sequelae. The most common problems asso: ciated with undescended testicles are testcu- Jar neoplasm, subfertility, testicular torsion and inguinal hernia, Although the likelihood of testicular cancer is higher in men with an undescended tests, the risk does not warrant radical surgery, such as removal of all intra-abdominal testes. ‘Novraasen 1, 2000 / Vounir 2» NUMMER 9 ve aatp. oat history of undescended testicle haves higher- ‘than-expecte incidence of testicular germ cel cancers. While the likelihood of developing testicular cancer has probably beer overest- ‘ated in the pas, the incidence among men with an undescended testicle is approximately ‘one in 1,000 to one in 2,500. Although signif ‘cant higher than the risk among the general ‘population (:100,000}, this level of risk does not warrant radical therapy, such as removal of all intra-abdeminal testes. About 20 percent of testicular tumors in :nen with unilateral eryporchidism oceur on the side with the normally descended test- cle’; this finding supports the argument against indiscriminate removal of unde- scended testes. Cryptorchidism and testicular ‘eancer may also be manifestations ofa genetic testicular abnormality: therefore, the devel- ‘opment of cancer in an undescended tests ‘ay not be caused by testicular malposition, Although there is no proof that orchiopexy [AMERICAN Fayaty Piscias 2037 Treatment for the undescended tests is recommended as early as six months of age and should be completed before age two. reduces the risk of testicular cancer, itis per- formed to ease detection through testicular self-examinatior suareRUTy ‘As a group, men who have had an unde- scended testicle have lower sperm counts, poorer quality sperm and lower fertility rates than men whose testicles descended nor- ‘mally: The likelihood of subferiity increases with bilateral involvement and increasing age atthe time of orchiopexy. Impaired spermato- genesis may be partially caused by underlying, genetic abnormalities that can increase the sk of germ cell neoplasia, For this reason, impairment may not be completely reversible ‘through surgical intervention, Unlike the risk of testicular cances, how- ever, there seems to be an advantage to early orchiopexy for protection of fertility." Through testicular biopsy at the time of orchiopexy, germ cell density has been shown to decrease over time, beginning as early es ‘one year of age. For this reason, treatment of the undescended testicle is recommended as early as six months of age and should be completed before age two. ‘The Authors STEVEN G. DOCIMO, MO. sdrector of pediatric logy ct the Chidtens Hospital (of Pitsburgh, Pa, and professor of urciegy atthe Unversity of Pitsburg School of Meine RICHARD | SLVER, M.D, curenty has a arate practice atfilated with the Aled duPont Hospital for Chien, Wiington, Oe WILLIAM CROME, MLD, professor and director of pediatric ulogy a he Univer- ‘By of Chicago "rzter School af Medne, Chscago ‘Ackiresscomespendence to Steven G, Docimo, M.D, Decay, Pda Urology Ci ‘reas Hospital of Ptsburgh, 3705 Fh ve, Psburgh, PA 15212. Ropes are ot ‘ralabi from the authors 2038 Avanicay Beaty Puvstcra wneaatp ote ‘TESTICULAR TORSION AND INGUINAL HERNIA Although there is litle solid evidence, the incidence of testicular torsion is thought to be higher in undescended testes than in normal scrotal testes.” Torsion of an undescended tes- ticle often occurs with the development of a testicular tumor," presumably caused by increased weight and distortion ofthe normal «dimensions of the organ. Torsion of an intra- abdominal testicle may present as an acute abdomen. A nonpalpable testicle on physical ‘examination should be a clue to the diagno- sis, but offen the torsion is only diagnosed at the time of abdominal exploration. ‘Most true cases of undescended testicles are associated with a patent processus vag nals" IF an overt hernia is present, expedi tious hernia repair with orchiopexy atthe age of presentation is undertaken. Otherwise, the hernia should be repaired at the time of ‘orchiopexy. A man with an untreated, ande- scended testicle and an occult inguinal hernia ‘may present at any time with symptoms and complications typical of any inguinal hernia, Presentation and Anatomy Most undescended testicles are present at birth, Up to one third of premature male newborns are born with an undescended tes- ticle and 3 to 5 percent of term male infants are affected.” By three months of age, the incidence is reduced to 0.8 percent; between three months of age and adulthood, the inci- dence does not change.:* Watchful waiting is not an option because true undescended tes- ticles rarely descend spontaneously ater three months of age. A proposed algorithm for the management of newborns with erypt- orchidismn, including suggestions on winen to refer patients to a pediatric urologist, is pre~ sented in Figure 1 ‘Occasionally a testicle that is noted in early childhood to be in 2 scrotal position will “ascend” and become truly undescended. While physicians used to believe that this finding represented an error in physicat Vouine 62, Nustsex 9 / Novents 1, 2000 Undescended Testicle Management of Cryptorchidism UUndescended test oF i verolne ruta Pate nortan ‘Surgery Surgery Measure LH, FSH, Cc .. a io oth oo fee 4 aomleeee so) fe eee eee to De as | ie vine ie ‘therapy Nortnat Intersexual | | failure pe l Le soley cum nelane tone malaet suey tnubape may laparoscopy or open ‘therapy, by diagnosis ‘or open agonadal; ocean cera ata me eg oe Remreetemene Ce) Cee FIGURE 1, Management ofthe infant with cryptorchidism, cluding suagested times for referral to a pediatric urologist. Older children should be referred on discovery of an undescended testicle, (LH = luteinizing hormone; FSH » follcle-stim- Ulating hormone; MIS = millerian inhibiting substance; hCG = human chorionic gonadotropin) examination, testicular ascent is now a well- documented phenomenon. It occurs in older children and young infants.“ In older chil dren, testicular ascent probably represents an ectopic testicle with enough gubernscular laxity to reach the scrotum in early child- hood. As the child grows, the ectopie guber- naculuum tethers the testicle, which is pulled Novisser 1, 2000 7 Vows 62, Nene 9 cephalad out of the scrotum." The mecha- nism of testicular ascent in the infantis not well established because this phenomenon is rarely reported. Undescended testicies can be categorized ‘on the basis of physical and operative find- ings: (1) truc undescended testicles (includ- ing intra-abdominal, peeping at the internal ww cato.raiap ‘Avsican Pasty Pistcray 2039 The retractile testicle is the most important type of undescended tests to distinguish on physical examination because no hormone or surgical therapy is required. ting and canalicular testes), which exist along, the normal path of descent and have @ nor- mally inserted gubernaculum (2) ectopic tes ticles, which have an abnormal gubernacular insertion; and (3) retractile testicles, which are not truly undescended. The most impor: sat category to distinguish on physical examination is the retracile testis, because no hormone or surgical therapy is required for this condition “Approximately 20 percent of infants who present with cryptorchidism have at east one FIGURE 2. Examination of the groin for an undescended testicle is often enhanced with the use of lubrication. (4) The examining hand is swept along the inguinal canal, starting at the superiolateral extent of ‘the inguinal canal. f the testicle is present, it wil either “pop” under the examiner's fingers (6,0), or be manipulated into the scrotum, ‘where it will be palpated by the opposite hand (0). 2040 Ananucan Fayuuy Prvstczas wen atp att nonpalpable testicle. Through surgical exam- ination, about one half of nonpalpable testes are found to be intra-abdominal, while the rest represent absent (vanishing) or atrophic testes” The vanishing testicle is thought to be caused by intrauterine testicular torsion. Physical Examination A general physical examination that emphasizes the signs of syndromic features ‘may reveal an underlying reason for crypt- orchidism, such as Prader-Willi, Kall: ‘or Laurence-Moon-Bied! syndromes." The genitalia should be examined for evi hypospadias or ambiguity. Concurrence of hypospadias and undescended testis is com- monly associated with states of intersexuality ly mixed gonadal dysgenesis and teu aphroditism.® Testicular examination of the infant and young child requires a two-handed tech nique. Ore hand should star at the hip and gently sweep along the inguinal canal, aided by surgical lubricant or warm soapy water, if necessary (Figure 2, photo A). A true unde- scencied or ectopic inguinal testicle will be felt to “pop” under the examiners fingers during ver (Figure 2, photos B and C). A Jow ectopic or retractile testicle willbe felt by the opposite hand as it is “milked” into the scrotum (Figure 2, photo D). The ectopic tes- cle will immediately spring out of the scr tum when it s released. The retractile testicle will remain momentarily in the scrotum until further stimulation causes a cremasteric reflex. Differentiation of a retractile testis from a true undescended testis is sometimes diff cult; consuitation with a urologist may be valuable, Position, consistency and size ofthe ‘undescended testicle in relation to the oppo- sit tests are noted. Ifa tests cannot be pal- pated in the inguinal canal or the scrotum, or in ectopic sites such as the femoral region or perineum, evaluation for a nonpalpable testis ‘must be initiated. Sometimes tissue in the scrotum may feel like an atrophic testicle nce of he this Vous 62, Numer 9 / Novena 1, 2000 Occasionally ths tissue represents gubernac- ‘ulum or dissociated epididymis and vas def- erens,* and may coexist with an intra- abdominal testis. Unless the presence of a testicle is clear, examination by a urologist is indicated. Evaluation of the Bilateral Nonpalpable Testis, {A phenotypically male newborn with bilat- cral nonpalpable testicles should be considered to be a genetic female with congenital adrenal hyperplasia until proved otherwise. Congenital adrenal hyperplasia may rarely present with @ ‘normal male phenotype and is if-thresten- ing condition. Ultrasound examination of the pelvie structures, karyotyping and mea- surements of serum electrolytes, testosterone, imallerian-inhibiting hormone, and adrenal hhormones and metabolites (17-hydroxypro- gesterone) should be considered in the initial evaluation. An older child with bilateral non- palpable testicles should be evaluated hormon- ally for testicular absence? Serum studies should include testosterone, luteinizing hormone (LH), follice-stimulat- ing hormone (FSH) and miilerian-inhibiting substance (MIS), Flevations in LH and FSH, as well as the absence of detectable MIS, sug- {est testicular absence. Measurements of thyroid hormone and cortisot levels should bbe considered beceuse hypogonadism may ‘occur with pituitary aplasia, A stimulation test using intramuscular human chorionic gonadotropin (hCG) can be administered to check for evidence of testosterone produc- tion, Definite hormonal evidence of testicular absence may preclude surgical exploration in the rare case of a newborn with bilateral absent testicles, but a minimum of both a negative hCG stimulation test and elevated gonadotropins must be included. Normal gonadotropin levels or detectable MIS levels, warrant surgical exploration, even with aneg- ative hCG stimulation test Except in certain cireumstences, radiologic valuation of the nonpalpable testile is not [Novewnar 1,2000 / Vouiner 62, Nosnen 9 Undescended Testicle In the United States, the only hormone approved for use in the treatment of cryptorchidism is human chorionic gonadotropin. ‘warranted.** None of the commonly applied studies, such as ultrasonography, computed tomography or magnetic resonance imaging, is sensitive enough to detect the majority of {ntra-abdominal testes, and none is specific «enough to exclude an intra-abdominal test ‘Therefore, whether or not a testicle is located by the radiologic study, surgical exploration is required. Ultrasound examination may be advisable in the infant with bilateral nonpal- ppable testes to look for gonads and to exclude the presence ofa uterus, which would suggest «state of intersexuality. Ultrasound examina- tion may also be helpful in the overweight child to detect inguinal testicles that ave difi- cult to palpate. ‘Therapy for the Undescended Testis ‘Treatment for cryptorchidism ean be hor- ‘mona, surgical or a combination of the two. Because the process of testicular descent is hormonally mediated, it can sometimes be induced with hormone administration, Administration of systemic testosterone is minimally effective in achieving testicular descent because the process depends on a paracrine effect—high local levels of testos- terone that cannot be achieved systemically. Using gonadotropins to stimelate the testicle to produce testosterone may help patients to achieve these high local levels, In the United States, the only hormone labeled for the treatment of cryptorchidism is ACG, which is administered intramuscularly. ‘There are many protocols for the use of hCG. One stich protocol is the administration of 1,500 to 2,500 U two times per week for four weeks. Whatever protocol is used, the likeli- hhood of success is greatest in the most distal true undescended testicles, In. theory, an wor aatp orgy Arnica Baty PHYSICIAN 2041 The most significant complication of orchiopexy is testicular atrophy. 2042. Anrccan Fasany Pisa ectopic testis should not respond to hormone ally prevented from descending. A high undescended testis is unlikely to descend completely; iit does twill probably ascend ater the hormone stimula- tion is withdrawn2* Some side effects of hCG administration can be disturbing for parents. These include enlargement of the penis, pubic hair growth, increased testicular size and aggressive behavior during administration ‘Studies suggest that gonadotropin-releas- ing hormone (GnRH) is more effective CG in achieving testicular descent.” Unfor- tunately, the reported results of hormonal therapy in the United States and Europe vary widely, probebly beca: variable numbers of boys with retractile testes, At any rate, GaRH is not currently labeled for use in the treatment of cryp. torchidism in the United States. apy because itis ph of the inclusion of SURGERY FOR THE UNDESCENDED TESTIS ‘The inguinal orchiopexy is a well-estab- lished operation for the palpable unde scended testcle2* Postoperative management of the condition is fairly straightforward. To prevent dislodgment of the testis from the scrotum, the use of toys that must be stra: dled, such as bicycles, should be avoided for two weeks. Sports activities should also limited in the ofder chile Examination in the early postoperative period (one to two weeks after surgery) allows the physician to assess wound healing and remove any fixating sutures, Repeat examination should be performed at least three months after surgery to assess the posi- tion and size of the testicles. ‘The most significant complication of orchiopexy is testicular atrophy. Dissection of the testicular vessels and/or postoperative FIGURE 3. (Top) Laparoscopic appearance of a normal right-sided vas deferens and spermatic vessels exiting the internal inguinal ring. Note ‘that the vas deferens and vessels clearly meet at the ring. (Bottom) Here, the vas deferens and vessels do not meet, suggesting that they are blind ending—the characterstc finding of the “vanishing testis. swelling and inflammation can result in ischemic injury and testicular atrophy. Although this isa rare complication of row tine orchiopexy, a meta-analysis” of the ro: logic literature saggested an 8 percent failure rate of orchiopexy, even in the distal unde- scended testis, and failure of more than percent of orchiopexies for intra-abdominal testes. Other potential complications include ascent of the testis (Which would require 2 second orchiopexy), infection and bleeding. Orchiopexy should be performed by urologists who are well versed in the surgi: ccal procedure and the management of plications, ‘Vounss 62, NowBrk 9 / Novis 1, 2000 FIGURE 4A. A low intra-abdominal testicle iden tified atthe level of the internal ring. A laparo- scopic orchiopexy maintaining the integrity of the spermatic vessels is possible in this case. SURGICAL MANAGEMENT (OF THE NONPALPABLE TESTIS. Surgery for the nonpalpable testicle is diag nostic and potentially therapeutic. Initially, tis important to determine whether a testis exists, If the absence ofa tests i surgically confirmed by identifying blind-ending testicular vessel, the surgery should be terminated (Figure 3) Intra-abdominal blind-ending vessels. are found in 9.8 percent of boys with nonpalpable testes° Sometimes the testicular vessels are ‘raced to an abdominal, inguinal or scrotal tes ticular remnant, which is then removed, In about one half of cases, an intra-abdominal testis is found (Figures 4a through 4c), which is either brought to the scrotum or removed, “The two initial surgical approaches to the nonpalpable testis are the open inguinal and diagnostic laparoscopic techniques. In the ‘open inguinal approach," the groin is explored. If cord structures or testicular ren nants aze found, they are removed, and the procedure is terminated. If the groin exp ration is negative, the incision is extended, and the peritoneum is entered in a search for an intra-abdominal testis, The second surgical approach to the nonpal- pable testis is laparoscopic. Diagnostic laparoscopy, which isa safe procedure in expe- rienced hands, is performed initially" Using Novusanen 1, 2000 / Vorunae 62, Nusa 9 Undescended Testicle FIGURE 48, 4 high testicle iocated well above the Internal inguinal ring, FIGURE 4C, Testicle managed in two stages, With initia! clipping of the spermatic vessels to allow collateral blood supply from the vas def trens to develop more fully a laparoscope placed through the umbilicus, the inguinal rings are examined, and the status of the processus vaginalis (patent or non- ent), wolffian structures and testicular ves~ ified. The presence of blind-ending spermatic vessels confirms an absent tests, allowing termination of the pro- cedure without a groin incision, If vessels and vas deferens exit the internal ring, the groin can be explored. If an intra-abdominal testis is identified, the physician can then choose the best surgical approach, Anica Brat Pe Undescended Testicle REFERENCES 1 2044 Anerucan Fanuuy Presicue eczowsii D, McLaughlin I, Lackgren G, Adami Ho, Pesson 1, Ccurence of restiur cancer in patents operated on for cyplarchidism and Ingoinal hema I Uro\1991;148°4251-4 Marin DC. Matgnancy in the cyptorchid tests Uso Cin orth A 1982:9:371-. ‘Swetcow Al Higgs CD, Pike MC. Rsk of testeus lar cance in conor of boys with exyptorhidism BM) 1997.34 1507-11 [Pubished erstum ap peas in BAW 1997-315:1129), Kogan Si. Ferity in cyptrchidism. An overview in 1987. Eur) edit 1987;146\supl 2)", McAleer, Pocker MG,

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