Campbell-Walsh Urology, 11th Edition

PART XV

Pediatric Urology

SECTION E

Genitalia

148

Etiology, Diagnosis, and Management of Undescended Testis

Julia Spencer Barthold; Jennifer A. Hagerty

Questions

1. What is the master gene responsible for male sexual differentiation?
2. RSPO1
3. SOX9
4. WT1
5. SRY
6. WNT4
7. During male reproductive tract development, androgens mediate the differentiation of all of the following structures EXCEPT:
8. seminal vesicles.
9. ureter.
10. epididymis.
11. vas deferens.
12. ejaculatory ducts.
13. Which of the following does NOT play a direct role in testicular descent?
14. Testis
15. Epididymis
16. Genitofemoral nerve
17. Gubernaculum
18. Processus vaginalis
19. Peak levels of testosterone and insulin-like 3 (INSL 3) occur in the male fetus at approximately what gestational week?
20. 5
21. 8
22. 10
23. 15
24. 20
25. Cryptorchidism increases the risk of all of the following EXCEPT:
26. spermatic cord torsion.
27. clinical hernia.
28. reactive hydrocele.
29. infertility.
30. testicular malignancy.
31. The risk of cryptorchidism is higher in all of the following syndromes EXCEPT:
32. cerebral palsy.
33. cystic fibrosis.
34. arthrogryposis.
35. prune-belly syndrome.
36. posterior urethral valves.
37. Abdominal cryptorchidism is associated with all of the following anomalies EXCEPT:
38. transverse testicular ectopia.
39. epididymal anomalies.
40. inguinal hernia.
41. vanishing testis syndrome.
42. polyorchidism.
43. Histologic findings in cryptorchid testes may include all of the following EXCEPT:
44. intratubular germ cell neoplasia, unclassified.
45. absence of Ad spermatogonia.
46. early disappearance of gonocytes.
47. failure of Sertoli cell maturation.
48. reduced germ cell counts.
49. What percentage of undescended testes are nonpalpable at presentation?
50. 1%
51. 3%
52. 10%
53. 20%
54. 30%
55. During laparoscopy, spermatic cord structures exiting an open internal ring ipsilateral to a nonpalpable testis implies:
56. vanishing testis, inguinal exploration unnecessary.
57. vanishing testis, inguinal exploration necessary.
58. intracanalicular atrophic testis, inguinal exploration unnecessary.
59. intracanalicular testis, inguinal exploration necessary.
60. further exploration unnecessary if contralateral testicular hypertrophy is present.
61. Advantages of laparoscopic management of an intra-abdominal testis include all of the following EXCEPT:
62. it more accurately assesses the presence or absence, viability, and anatomy of the testis compared with radiographic imaging.
63. it allows for laparoscopic repair of the ipsilateral inguinal hernia when present.
64. it enhances surgical exposure, lighting, and magnification.
65. it allows a greater degree of proximal dissection of the spermatic vessels.
66. it allows diagnosis of associated Müllerian ductal abnormalities if present.
67. Which statement is FALSE regarding Fowler-Stephens orchidopexy?
68. It is less commonly associated with testicular atrophy than laparoscopic orchidopexy.
69. It has a lower success rate in patients who have undergone previous inguinal surgery.
70. Blood supply is based on the deferential artery and collateral peritoneal vessels.
71. It should be performed at a similar age as a standard inguinal orchidopexy.
72. It should be considered if the testis is not near the internal ring.
73. Which of the following is least consistent with a diagnosis of vanishing testis?
74. Patent processus vaginalis
75. Contralateral testicular hypertrophy
76. Palpable nubbin in scrotum
77. Increased serum follicle-stimulating hormone (FSH)
78. Micropenis
79. Lower than expected testicular volume has been associated with all of the following EXCEPT:
80. cryptorchid testes that have descended spontaneously.
81. solitary testes in boys with a vanishing testis.
82. Fowler-Stephens orchidopexy.
83. increased serum FSH.
84. surgery for congenital cryptorchidism at 3 years compared with 9 months of age.
85. Regarding epididymal anatomy, which of the following is the most common finding in boys undergoing orchidopexy for acquired cryptorchidism?
86. Detachment of the cauda epididymis
87. Detachment of the caput epididymis
88. Looped epididymis
89. Long looping epididymis/vas
90. Normal anatomy
91. All of the following factors may influence the reliability of studies of the efficacy of hormone therapy for cryptorchidism EXCEPT:
92. treatment of boys with retractile testes.
93. initial position of the testis.
94. vanishing testis syndrome.
95. randomization protocol.
96. all of the above.
97. A newborn boy presents with a bilateral nonpalpable testes. Next steps in management should be:
98. karyotype analysis.
99. hormonal studies.
100. circumcision.
101. a and b.
102. a, b and c.
103. Of the following, which is the least reliable test in confirming the diagnosis of bilateral anorchia?
104. No change in serum testosterone following human chorionic gonadotropin (hCG) stimulation
105. FSH level greater than 2 IU/L at 1 year of age
106. Laparoscopy
107. Undetectable serum inhibin B
108. Undetectable serum antimüllerian hormone (AMH)
109. Which of the following is least useful to the provider in determining the diagnosis of retractile versus undescended testes?
110. Observation of testicular position with abduction of the patient's legs.
111. History of prior testicular position provided by the patient's family.
112. Failure of the testis to remain stable in the scrotum with sustained traction on the cord.
113. Warm room and hands.
114. Small ipsilateral testis.
115. Which of the following is TRUE regarding spontaneous descent of cryptorchid testes?
116. Spontaneous descent is independent of testicular position.
117. Reascent occurs in 40% of patients.
118. Early descent is more likely in premature boys.
119. Spontaneous descent is unlikely if the scrotum is small.
120. The majority of testes that descend spontaneously do so in the first few months of life.
121. The risk of developing testicular germ cell tumor (TGCT) in males with a history of cryptorchidism is:
122. 2 to 5 times the risk in normal boys.
123. minimal in boys who undergo orchidopexy in infancy.
124. determined by placental alkaline phosphatase (PLAP) staining in prepubertal testes.
125. similar in the contralateral descended testis.
126. increasing geographically with time.
127. All of the following is more common in association with cryptorchid testes, EXCEPT:
128. atrophy.
129. microlithiasis.
130. mature teratoma.
131. ectasia of the rete testis.
132. intratesticular varicocele.
133. The following are possible locations of an ectopic testis, EXCEPT:
134. peripenile.
135. perirenal.
136. perivesical.
137. perianal.
138. femoral.
139. Levels of all the following hormones peak after birth and fall to lower levels during childhood EXCEPT:
140. luteinizing hormone (LH).
141. FSH.
142. AMH.
143. inhibin B.
144. testosterone.
145. When in fetal development does the testicle pass into the inguinal canal?
146. 5 to 7 weeks’ gestation.
147. 10 to 14 weeks’ gestation.
148. 20 to 28 weeks’ gestation.
149. 30 to 34 weeks’ gestation.
150. None of the above.
151. How commonly does cryptorchidism occur in full-term males?
152. Less than 1%
153. 1% to 4%
154. 5% to 10%
155. 15%
156. None of the above
157. A 6-month-old full-term male presents with a unilateral nonpalpable testis. The next step after a confirmatory exam is:
158. ultrasound to identify the position of the testis.
159. hormonal therapy.
160. surgical intervention.
161. observation for spontaneous descent until 1 year of age.
162. a and d.

Answers

1. d. SRY.The SRY gene appears to be primarily responsible for male sexual differentiation through complex interactions involving both activation and repression of other male-specific genes.
2. b. Ureter.Androgens (testosterone, dihydrotestosterone) mediate the differentiation of the paired wolffian ducts into the seminal vesicles, epididymis, vas deferens, and ejaculatory ducts.
3. b. Epididymis.Changes in the gubernaculum and processus vaginalis and their innervation by the genitofemoral nerve, as well as hormone secretion by the testis, are all important in the process of testicular descent.
4. d. 15.Testosterone production peaks at 14 to 16 weeks and INSL3 peaks at 15-17 weeks.
5. c. Reactive hydrocele.All of the others are possible complications of cryptorchidism.
6. b. Cystic fibrosis.All the other syndromes are associated with a higher risk of cryptorchidism; mutations of the cystic fibrosis gene are associated with congenital absence of the vas deferens.
7. d. Vanishing testis syndrome.All the other entities are more frequently associated with cryptorchid testes located in the abdomen.
8. c. Early disappearance of gonocytes. Histological abnormalities that may be present in cryptorchid testes include delayed disappearance of gonocytes, reduced numbers of adult dark (Ad) spermatogonia, reduced number of germ cells per testicular tubule, and carcinoma in situ (CIS).
9. d. 20%.Approximately 20% of undescended testes are nonpalpable at presentation.
10. d. Intracanalicular testis, inguinal exploration necessary.Although atretic spermatic vessels seen exiting the internal ring may be associated with a distal vanishing testis, the appearance of the spermatic vessels during laparoscopy is subjective, and therefore exploration (inguinal or laparoscopic) is needed to rule out an intracanalicular viable or atrophic testis. Further exploration is unnecessary if blind-ending intra-abdominal spermatic vessels are found. Hypertrophy of a normally descended contralateral testis is suggestive of monorchism.
11. b. It allows for laparoscopic repair of the ipsilateral inguinal hernia when present.An inguinal hernia or patent processus vaginalis does not require formal repair at the time of laparoscopic orchidopexy.
12. a. It is less commonly associated with testicular atrophy than laparoscopic orchidopexy. Fowler-Stephens orchidopexy, either 1- or 2-stage, has a higher reported testicular atrophy rate compared with laparoscopic orchidopexy. The other statements are true.
13. a. Patent processus vaginalis. Contralateral testicular hypertrophy and a palpable scrotal nubbin may present in boys with unilateral vanishing testis and increase serum FSH and micropenis may be seen in boys with bilateral vanishing testes. The processus vaginalis is closed in most cases of vanishing testis.
14. b. Solitary testes in boys with a vanishing testis.In vanishing testis syndrome, the contralateral testis may be larger than expected for age.
15. e. Normal anatomy. An abnormal epididymis was reported ipsilateral to 11% to 31% of acquired undescended testes at surgery.
16. e. All of the above.All are confounding factors that may affect the reliability of studies of the efficacy of hormone therapy for cryptorchidism.
17. c. a and b.Circumcision should be avoided in the initial management of phenotypic boys with bilateral nonpalpable testes, pending an evaluation for congenital adrenal hyperplasia.
18. a. No change in serum testosterone following human chorionic gonadotropin (hCG) stimulation. Serum testosterone may not increase significantly in response to hCG stimulation in individuals with abnormal testes.
19. b. History of prior testicular position provided by the patient’s family.Testicular position can change with time and/or be difficult to ascertain in boys with retractile testes; therefore, a careful examination is necessary to differentiate between retractile and undescended testes.
20. e. The majority of testes that descend spontaneously do so in the first few months of life. For full-term boys of normal weight, spontaneous testicular descent typically occurs in the first months after birth and is rare after 6 months of age.
21. a. 2 to 5 times the risk in normal boys. Surgery may reduce but does not eliminate the risk of TGCT in boys with cryptorchidism, and the risk also exists in the contralateral testis, albeit lower. PLAP + germ cells can be found in normal testes after birth.
22. c. Mature teratoma.The risk of benign testicular tumors is not increased in cryptorchidism.
23. d. Perianal.All the other answers reflect possible positions for ectopic testes that are possible although rare.
24. c. AMH.AMH levels remain high during childhood and are downregulated at puberty.
25. a. 20 to 28 weeks’ gestation.The testis passes into the inguinal canal at 20 to 28 weeks’ gestation during the fifth phase of testicular descent.
26. b. 1% to 4%.Cryptorchidism is one of the most common congenital anomalies, occurring in 1% to 4% of full-term male infants.
27. c. Surgical intervention. If descent has not occurred by 6 months of age, surgical treatment should be performed.Diagnostic imaging has not been shown to change the need for surgery. Hormonal therapy is no longer supported, given the lack of evidence to support its use.

Chapter review

1. The Leydig cell hormones insulin-like 3 and testosterone are required for testicular descent.
2. Three fourths of undescended testes are palpable; two thirds are unilateral.
3. The etiology of vanishing testis is most likely due to either in utero torsion or a vascular accident.
4. Confirming that the patient has an absent testis requires identifying a blind-ending spermatic artery.
5. The use of human chorionic gonadotropin for the diagnosis and treatment of undescended testis is not recommended.
6. Laparoscopic identification and mobilization of an abdominal testis is preferred. If the testis can be brought to the opposite internal ring, it can usually be placed in the ipsilateral scrotum.
7. Oligospermia or azoospermia occurs in approximately 75% of patients with bilateral cryptorchidism and in 40% of patients with unilateral cryptorchidism. Paternity in patients with unilateral cryptorchidism is similar to the general population despite the semen abnormalities.
8. Patients with nonsyndromic cryptorchidism have a high incidence of epididymal abnormalities and accompanying inguinal hernias.
9. During fetal development, swelling of the gubernaculum is important to allow for enlargement of the inguinal canal to facilitate testicular passage.
10. Prematurity and low birth weight are risk factors for cryptorchidism; other risk factors include maternal smoking, family history, and maternal exposure to diethylstilbestrol.
11. A karyotype should be obtained if neither gonad is palpable or if there is an undescended testis associated with hypospadias.
12. A two-stage Fowler-Stephens repair is successful approximately 60% of the time.
13. There is a twofold to fivefold increase in the risk of testicular cancer in a cryptorchid testis.
14. Histologic abnormalities that may be present in cryptorchid testes include delayed disappearance of gonocytes, reduced numbers of adult dark (Ad) spermatogonia, reduced number of germ cells per testicular tubule, and carcinoma in situ (CIS).
15. If testicular descent has not occurred by 6 months of age, surgical treatment should be performed.