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Undescended testicle
1) Cryptorchidism, or undescended testes, occurs when the testes fail to descend into the scrotum. This can be due to abnormalities in testicular descent during embryonic development between 5-9 months of gestation. 2) The incidence of cryptorchidism is about 3-5% in full term infants but higher in premature infants. By age 1, about 1-1.5% of boys will have undescended testes. 3) Cryptorchidism increases the risk of testicular cancer. About 10% of all testicular cancers occur in undescended testes. The risk is higher for intra-abdominal versus inguinal undesc
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Undescended testicle
- 1. Cryptorchidism Stephen Confer, MD BenO. Donovan, MD Brad Kropp, MD Dominic Frimberger, MD University of Oklahoma Department of Urology Section of Pediatric Urology
- 2. Embryology of Gonads •5th week - primordial germ cells migrate from yolk sac along dorsal mesentary to the posterior body wall
- 3. Migration of primordial germcells migrate from the posterior extra- embryonic mesoderm through the mesenteries and into the gonadal ridge Embryology of Gonads
- 4. Embryology of Gonads •5 weeks- Sexually indifferent – identical structures in male and female • Gonadal ridges develop on dorsal abdominal wall week 5 – future ovaries or testes • Wolffian ducts – future male ducts • Müllerian ducts – future female ducts
- 5. Embryology of Gonads •Mesenchymal proliferation occurs and genital ridges are formed • Paramesonephric (Mullerian) ducts begin to form lateral to the mesonephric ducts • After the 6th week genital ridges pursue fates as male or female. • “Y” does this happen?
- 6. SRY acts onthe indifferent gonad to start the process of male sexual development Molecular Determination of Sex
- 7. Male Genital Development •Sex cord cells differentiate into Sertoli cells only if SRY protein is present • 7th week – Sertoli cells organize to form testis cords – Will eventually form seminiferous tubules – Testis cord distally forms rete testis – Medially rete testis connect to mesonephric ducts via 5-12 tubules (efferent ductules) • Vas deferens develops from mesonephric duct
- 8. Male Genital Development •As Sertoli cells differentiate in response to SRY, secrete a glycoprotein hormone Mullerian-inhibiting substance (MIS) – MIS causes paramesonephric (mullerian) ducts to regress between 8th -10th week – Remnants may be found • Appendix testis
- 9. Male Genital Development•9th -10th weeks – Leydig cells differentiate – Early testosterone regulated by placental chorionic gonadotrophin – eventually regulated by pituitary gonadotrophins • 8th -12th weeks – testosterone secretion by Leydig cells stimulates transformation of mesonephric duct to the vas deferens – Cranial portion – VD; ED – Caudal portion -SV; Ejac. Duct – Communication with rete testis is not established until 3rd month
- 10. Gonadal Descent • Initialdescent in both sexes relies on the gubernaculum – Forms in the peritoneal folds during 7th week – Superior end is attached to gonad, inferiorly it is attached to fascia between the external and internal obliques • Processus vaginalis – Evagination of peritoneum
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- 12. Testicular Descent • Layersencountered by processus vaginalis – Transversalis fascia – Muscle fibers of internal oblique (cremasteric) – Thin layer of external oblique muscle (external spermatic fascia) • Descent of testicle – At level of internal ring by 3rd month – Complete descent to scrotum between 7th -9th month
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- 14. Normal Anatomy Duct System Bloodsupply Blood Testis Barrier
- 15. Spermatic cord supplies •Blood Supply • Lymphatics – Upper lumbar LN • Nerve supply – Sympathetic- T10-11 – Unable to differentiate – Suggested to increase or decrease dilation
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- 20. Cryptorchidism • Means hiddentestis • Testis develops intraabdominally, disorders of descent are common • No geographic or ethnic distributions • Testes descend to cooler scrotum to facilitate spermatogenesis in humans
- 21. Embryology of TestisDescent • Germ cells migrate from yolk sac to genital ridges at 6 weeks • Testis differentiation begins to occur at 7 weeks as SRY protein is produced • Testosterone and MIF are secreted by testis during week 8 • Testosterone is controlled by maternal hCG, causes wolffian differentiation into epididymis and vas • MIF is produced by Sertoli cells and causes mullerian regression=>appendix testis
- 22. Embryology of TestisDescent (con’t) • Growth of the gubernaculum is believed to be controlled by testis paracrine function • Testis is never more than 1.3 mm from internal ring at any point in its development • Testis remain in position but processus vaginalis extends into scrotum from 12th week to 7th month
- 23. Embryology of TestisDescent (con’t) • At 7 months, the vas and testicular vessels increase in size, the gubernaculum swells, and the processus extends into scrotum completely • As the gubernaculum swells, the inguinal canal and scrotum stretch=>testis rapidly descends • The epididymis precedes the testis into the scrotum • Canal portion of processus obliterates, testicular portion persists as tunica vaginalis • Gubernaculum atrophies
- 24. Incidence of TesticularDescent • Of 1500 full term infants, 3.4% had true UDT • Prematurity may predispose to UDT because birth occurs before descent • Of 142 premature infants, 30.3% had UDT • Low birth weight also predisposes to UDT, as weight is gained, testes descend • At 1 y.o., 0.8-1.5% have UDT, 75% of full-term and 95% of premature UDT descend during first year • Most descend during first 3 months after birth probably due to elevated serum androgens during first 3 months
- 25. Incidence of TesticularDescent (con’t) • Cremasteric reflex is most active during 2-7 years old=>retractile testes are misdiagnosed as UDT – Estimates of UDT at 5 y.o. are about 10% • Testes do not ascend postnatally • Incidence of UDT in adulthood is < 1% • Absence of one or both testes on exploration is 3-5% • 10% of patients have bilateral UDT • 14% of patients with UDT have a family member with UDT
- 26. Theories Regarding the Mechanismsof Testicular Descent • Traction of the testis by gubernaculum and cremasters – Severing the gubernaculum does not stop descent in animals – Weak attachments between gubernaculum and scrotum • Differential growth of the body wall in relation to immobile gubernaculum – Proximity to internal ring is maintained as body grows – Gubernaculum swells and grows faster than the body • Intraabdominal pressure pushes the testis through the inguinal canal – Increased pressure promotes descent supported by theoretical and experimental data (Schechter 1960, Bergin 1970, Gier 1970)
- 27. Theories Regarding the Mechanismsof Testicular Descent (con’t) • Development and maturation of the epididymis contributing to descent – Probably not as important as initially thought • Effects of the genitofemoral nerve – Severing the nerve prevents descent in rodents
- 28. Endocrine Aspects ofUDT • Exact mechanism of androgen induction of descent is unknown • No consistently discernable abnormalities have been identified in patients w/ UDT – Conflicting data on baseline LH, FSH, testosterone levels and response to GnRH stimulation • Endocrine factors probably play a major role in descent – Testosterone induces testis descent in humans – Androgens affect the nuclei of the genitofemoral nerve to release modulating factors for gubernacular development – Boys with spinal cord defects at or below nuclei of the nerve have a higher incidence of UDT – DHT binds to rat gubernaculum
- 29. H-P-T Axis Disordersand UDT Hypothalamus LHRH Kallman’s Syndrome Pituitary Gland LH (FSH) Anencephaly Testis Testosterone Spermatic cord Gubernaculum Processus Vaginalis Organ Hormone DisorderOrgan Hormone Disorder DHT Pseudovaginal perineoscrotal hypospadias Steroid-Receptor Complex Testicular Feminization Reifenstein’s Syndrome 20,22-desmolase 3β-HSD, 17-hydroxylase 17,20-desmolase 17β-HSD
- 30. Classification of UDT •Abdominal – Impalpable by definition – Usually “peeping” • Canalicular – Or “peeping” at external ring • Ectopic – 5 major sites corresponding to gubernacular branches – Superficial inguinal is most common • Rectractile – Cremaster reflex – Most common in 5-6 year olds
- 31. Whitaker/Kaplan Classification for UDT •Palpable – Normal – Retractile – Ectopic – Undescended • Unpalpable – Canalicular – Intra-abdominal – Emergent – Absent • Agenesis • atrophy
- 32. Work Up ofUDT • Must determine if testes present at birth • Physical exam is very important to evaluate retractile testes – Non-palpable testis is intraabdominal, intracanalicular, absent • Bilateral UDT requires hormonal evaluation and challenge – Elevated gonadotropins (FSH) suggest bilateral anorchia – Normal serum gonadotropins=>hCG challenge (2000 IU x 3days) – No testosterone response indicates bilateral anorchia
- 33. Imaging of UDT •Herniography-poor sensitivity and specificity • U/S-good for inguinal testes, not reliable if higher • CT-may be helpful for bilateral impalpable testes – Difficult to perform in young children • MRI-least invasive, most expensive – Difficult to perform in young children • Venography-invasive, pampiniform plexus present=>testis present – Non-visualized plexus or blindending does not eliminate testis • Angiography-difficult to perform, high complications • Overall accuracy of radiologic imaging for UDT = 44% – PE is 53% accurate by PMD, 84% accuracy by peds GU
- 34. Laparoscopy • Blind-ending vesselsindicate absent testis • Vessels into the internal ring=>inguinal exploration, laparoscopic orchiopexy/orchiectomy • Intra-abdominal testis – Laparoscopic orchiopexy – 2 stage orchiopexy (Fowler- Stephens) – Abdominal exploration
- 35. Histology • UDT containatrophied structures with thickened basement membrane • Epithelium contains atypical germ cells • Smaller seminiferous tubules with fewer spermatogonia • Higher level of UDT correlates with severity of histologic abnormalities • Some of the changes can be seen in the contralateral normally descended testis UDT histology Normal testis histology
- 36. Neoplasm and UDT •10% of testis CA are in UDT • UDT is 35-48x more likely to have malignancy • Incidence of CA in UDT is 1:2550 testis tumors • Abdominal UDT is 4x more likely than inguinal testis to develop CA • UDT tumors typically occur around puberty • CIS occurs in 1.7% of UTD • Orchiopexy should be performed between 1-1.5 years old • 1/5 of testis CA in patients w/ hx of UDT occurs in contralateral testis
- 37. Neoplasm and UDT •For bilateral UDT, when CA is detected in one testis there is a 15% chance of developing tumor in the contralateral testis – 30% if both were intra-abdominal UDT • Seminoma is most common CA in UDT – Embryonal is second most common • Gonadoblastoma is most common CA in some intersex disorders which present w/ UDT – Often coexists with germ cell tumors – Develop due to deletion on long arm of Y chromosome
- 38. Torsion and UDT •Increased risk for torsion in UDT due to anatomic abnormality between testis and mesentery • Incidence is greatest after puberty with increased testis size • Tumor that increases size also increases risk of torsion • 64% of adult torsion in UDT patients had germ cell tumor • Be aware of abdominal pain and empty hemiscrotum=> torsed intra-abdominal UDT
- 39. Hernia and UDT •Processus vaginalis should obliterate between the 8th month of gestation and 1st month of life • UDT results in patent processus vaginalis • Hernias are found in 90% of patients w/ UDT
- 40. Infertility and UDT •Spermatogenesis is retarded by maldescent • Bilateral UDT => poor fertility • Higher UDT => more damage to seminiferous tubules • Earlier orchiopexy may improve chances for recovery of spermatogenesis • Sperm counts in unilateral UDT are much lower than normal – Contralateral testis may also be defective
- 41. Associated Anomalies withUDT • Abnormalities with vas and epididymis are associated with UDT – Extended length of epididymis, partial atresia, total atresia, dissociation from testis • Cystic Fibrosis is associated with vasal agenesis, UDT is common • DES exposure during pregnancy results in epdidymal defects and UDT • Klinefelter’s syndrome – Sterility the rule although mosaicism may result in fertility • Noonan’s syndrome – Male Turner’s syndrome – Occasional fertility but not passed to offspring • Prader-Willi Syndrome – Hypothalamic defect with obesity, MR, hypotonia, short stature
- 42. Hormonal Treatment • hCGis given to stimulate Leydig cells to produce testosterone=>descent of testes – Success rate is 14-50% with doses ranging from 3K to 40K IU for daily or weekly injections – A dose of at least 10K is needed to have an effect, doses > 15K have significant side effects – No change in testis histology or bone growth • GnRH is given if basal LH is low and abnormality in GnRH secretion is suspected – Perinasal spray of 1.2mg/day is effective to stimulate LH – 6%-70% success rate
- 43. Surgical Treatment • Primarygoal of pexation is to facilitate examination • Basic principles of orchipexy are: localization, mobilization, cord dissection, isolation of processus, tension-free relocation to scrotum • Pexation does not reduce risk of cancer • Orchiopexy should be performed before 2 y.o. • Orchiectomy is an option for post-pubertal males and dysgenetic testes
- 44. Orchiopexy • 80% UDTare palpable • Of non-palpable testes, 45% are absent, 30% are abdominal, 25% are lower testes missed on examination • Orchiopexy is 92% successful for testes below external ring and 87% for inguinal, 82% for peeping, 74% for abdominal • Success rates for different techniques are 89% for inguinal exploration, 84% for microvascular repair, 81% for transabdominal, 73% for two-stage, 77% for staged F-S repair, 67% for standard F-S repair
- 45. Standard Orchiopexy • Transverseinguinal incision, watch for testis • Identify testis and divide gubernaculum • Open tunics and evaluate testis • Open external oblique fascia, avoid nerve • Mobilize spermatic cord • Finger dissection to enlarge scrotal cavity medially • Incise scrotal skin, create dartos pouch • Pass clamp through pouch into inguinal area and bring testis into pouch by gubernaculum or tunica • Pex testis with 4-0 vicryl sutures • Complications: atrophy, retraction, torsion, hematoma, nerve or vas injury
- 46. Fowler-Stephens Orchiopexy • Testishas three sources of arterial blood flow • Testicular artery and veins often limit mobilization • < 1/3 of boys with intra-abdominal testes have long vasal loop needed for vasal pedicle orchiopexy • Testes located > 1cm above internal ring do poorly with this technique • Approach requires long inguinal incision and vasal loop must be protected during inguinal dissection • Must open hairpin turn of vas for length without disrupting blood supply after dividing spermatic vessels • Clamp testicular artery before ligation to ensure collateral blood supply, incising tunica may be misleading • Mobilization with wide peritoneal-vasal cuff, may need more direct exit site • Same scrotal fixation techniques
- 47. Two-Stage Fowler-Stephens • 84%success rate with staged repair probably due to improved collateral circulation • Spermatic vessels are clipped > 1cm cranial to testis, large clip facilitates identification • Wait at least 6 months then perform orchiopexy open or laparoscopically or combined • Open: patient may not have external ring on affected side, divide muscles in general area • Divide vessels above clip, create wide peritoneal cuff around testis and vas, straighten vasal loop • Place in dartos pouch through medial incision in abdominal wall
- 48. Laparoscopy • 95% sensitivefor identifying and/or locating NPT • Three likely findings: – blind-ending vessels – cord structures entering the ring – intra-abdominal testis • One-stage lap orchiopexy brings testis medial to inferior epigastric vessels into pouch (Prentiss) • If vessels and vas enter closed internal ring, controversy about groin exploration • If internal ring is open, can complete orchiopexy laparoscopically or open Laparoscopic view of intra-abdominal testis at internal ring
- 49. Vanishing Testis • Laparoscopicview of closed internal inguinal ring. A, vas deferens and atretic spermatic vessels in vanishing testis syndrome. B, ipsilateral normal vas deferens and spermatic vessels in contralateral vanishing testis syndrome.
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