Campbell-Walsh Urology, 11th Edition

PART XV

Pediatric Urology

SECTION E

Genitalia

146

Management of Abnormalities of the External Genitalia in Boys

Lane S. Palmer; Jeffrey S. Palmer

Questions

1. In the male, which of the following stimulates the development of the external genitalia?
2. Testosterone
3. Human chorionic gonadotropin
4. Dihydrotestosterone
5. Luteinizing hormone and follicle-stimulating hormone
6. Maternal progesterone
7. What percentage of uncircumcised boys will have persistent phimosis by 17 years of age?
8. Less than 1%
9. 5%
10. 10%
11. 15%
12. 20%
13. Circumcision should not be performed in neonates with which condition of the genitalia?
14. Phimosis
15. Undescended testis
16. Inguinal hernia
17. Penile curvature
18. Testicular atrophy
19. What is the most common complication associated with circumcision?
20. Trauma to the glans
21. Bleeding
22. Meatal stenosis
23. Skin bridges
24. Balanitis xerotica obliterans (BXO)
25. A 4-year-old boy presents with phimosis and BXO of the prepuce. What is the preferred treatment?
26. Observation
27. Topical corticosteroids
28. Excision of BXO skin without circumcision
29. Warm baths
30. Circumcision
31. Penile agenesis is associated with all of the following malformations EXCEPT:
32. cryptorchidism.
33. vesicoureteral reflux.
34. horseshoe kidney.
35. ureteropelvic junction obstruction.
36. renal agenesis.
37. The etiology of the buried penis includes all of the following EXCEPT:
38. suprapubic fat pad.
39. small penis.
40. poor penopubic fixation of the penis.
41. obesity.
42. cicatricial scarring after surgery.
43. A 9-month-old boy who was previously circumcised presents with a buried penis resulting from cicatricial scarring. What is the most appropriate initial treatment?
44. Topical betamethasone and manual retraction
45. Revision of circumcision
46. Penopubic fixation of the penis
47. Liposuction of the suprapubic fat pad
48. Observation
49. What is the minimal normal stretched penile length of a full-term neonate?
50. 1.2 cm
51. 1.9 cm
52. 2.5 cm
53. 3.2 cm
54. 4.5 cm
55. Which of the following statements is TRUE regarding a micropenis in a term male neonate?
56. The testes are usually normal in size and not cryptorchid.
57. It is best managed by gender reassignment.
58. It has an abnormal ratio of the length of the penile shaft to circumference.
59. It is unlikely to respond to testosterone stimulation until puberty.
60. It is less than 1.9 cm in stretched length.
61. What is the most common cause of micropenis?
62. Hypergonadotropic hypogonadism
63. Hypogonadotropic hypogonadism
64. Idiopathic
65. Growth hormone deficiency
66. Androgen insensitivity syndrome
67. Which of the following statements regarding penile masses is FALSE?
68. The treatment of parameatal urethral cysts is complete excision of the cyst.
69. The most common acquired cystic lesion of the penis is smegma under the unretractable prepuce.
70. Congenital penile nevi tend to be malignant.
71. The initial management of juvenile xanthogranulomas is expectant monitoring.
72. Epidermal inclusion cysts may form after penile surgery.
73. A 13-year-old African-American boy with sickle cell disease has a 6-hour painful erection. The initial management should include all of the following EXCEPT:
74. alkalization.
75. hydration.
76. intracavernous injections of β-adrenergic sympathomimetic agents.
77. analgesia.
78. transfusion to reduce hemoglobin S concentration.
79. Which of the following statements is TRUE regarding high-flow priapism?
80. It is usually a drug-induced etiology.
81. The aspirated blood is similar to venous blood on blood gas analysis.
82. Color Doppler ultrasonography commonly demonstrates the fistula.
83. Surgical intervention is the initial management.
84. Sickle cell disease is the most common cause
85. Penoscrotal transposition is associated with all of the following anomalies EXCEPT:
86. sex chromosome abnormalities.
87. distal shaft hypospadias with chordee.
88. autosomal chromosome abnormalities.
89. Aarskog syndrome.
90. caudal regression.
91. All of the following are associated with patency of the processus vaginalis EXCEPT:
92. Transverse testicular ectopia
93. Epididymal anomalies
94. Cryptorchidism
95. Spermatic cord torsion
96. Polyorchidism
97. Abdominoscrotal hydrocele is reported to be associated with all of the following features EXCEPT:
98. a closed processus vaginalis.
99. epididymal anomalies.
100. testicular dysmorphism.
101. hydronephrosis.
102. increased pressure within the tunica vaginalis.
103. Irreversible ischemic injury of the testicular parenchyma may begin as early as how many hours after torsion of the spermatic cord?
104. 1
105. 2
106. 4
107. 6
108. 8
109. Which of the following is most specific in diagnosing spermatic cord torsion?
110. High-riding testis
111. Absence of the cremasteric reflex
112. Transverse lie of the testis
113. Spermatic cord twist on high-resolution Doppler ultrasonography
114. Acute severe pain
115. After manual detorsion of the spermatic chord, which of the following is appropriate management?
116. Color Doppler ultrasonography
117. Radionuclide scan
118. Doppler examination of the testis and spermatic cord
119. Discharge from the hospital and arrangement for an office reevaluation in 1 week
120. Immediate scrotal exploration
121. An adolescent is evaluated for a history of self-limited, intermittent episodes of severe unilateral scrotal pain. Physical examination findings are normal. What is the most appropriate course of action?
122. Color Doppler ultrasonography
123. Reassessment in 6 months
124. Elective scrotal exploration
125. Radionuclide scrotal imaging
126. Immediate scrotal exploration
127. When the diagnosis of torsion of the appendix epididymis is made, which of the following is optimal management?
128. Observation
129. Color Doppler ultrasonography
130. Radionuclide scrotal imaging
131. Immediate scrotal exploration
132. Cord block and manual detorsion
133. Which of the following is the most likely diagnosis in an infant with sterile urine and epididymitis?
134. Unilateral renal agenesis
135. Large prostatic utricle
136. Urethral stricture disease
137. Persistent vasoureteral fusion
138. Radiographically normal urinary tract
139. What is the most appropriate course of action in an otherwise healthy neonate with perinatal testicular torsion?
140. Surgical exploration of the affected testis
141. Surgical exploration of the affected testis with contralateral scrotal orchidopexy
142. Color Doppler ultrasonography of the scrotum
143. Radionuclide testicular scan
144. Observation
145. Most adolescent varicoceles evaluated by urologists are:
146. painful.
147. of cosmetic concern.
148. asymptomatic.
149. associated with an ipsilateral hydrocele.
150. bilateral.
151. Significant testicular volume differential in cases of varicocele is defined as greater than:
152. 5%.
153. 5% to 10%.
154. 10% to 15%.
155. 15% to 20%.
156. 25%.
157. Hydrocele formation after varicocele ligation is least likely to occur after which of the following procedures?
158. Retroperitoneal ligation
159. Subinguinal ligation
160. Laparoscopic ligation
161. Microscopic inguinal ligation
162. Transvenous embolization
163. Which of the following is NOT a relative indication for elective varicocele repair?
164. Pain
165. Oligospermia
166. Small testes
167. Continuous spermatic venous reflux
168. Testicular size discrepancy of greater than 20%

Answers

1. c. Dihydrotestosterone. Influence of dihydrotestosterone on the androgen receptors results in the differentiation of the genital tubercle, genital (labioscrotal) folds, and genital swelling at between 9 and 13 weeks of gestation into the male structures of the glans penis, penile shaft, and scrotum, respectively.
2. a. Less than 1%.Preputial retractability increases with age with 90% of uncircumcised boys 3 years of age with completely retractable prepuces; less than 1% by 17 years of age have phimosis. Therefore, primary phimosis is almost always resolvable during childhood without intervention.
3. d. Penile curvature. Circumcision should not be performed in neonates with other penile conditions that require surgical correction. These conditions include hypospadias, penile curvature, dorsal hood deformity, buried penis, and webbed penis.
4. b. Bleeding.The risk of complications after circumcision is 0.2% to 5%. The most common complication is bleeding, which occurs in 0.1% and is more common in older children.
5. e. Circumcision.Treatment of BXO includes medical and surgical management. The use of topical corticosteroids has had limited benefit to treat mild BXO of the prepuce with minimal scar formation. Circumcision is the preferred treatment.
6. d. Ureteropelvic junction obstruction.Penile agenesis (aphallia) results from failure of development of the genital tubercle. The disorder is rare and has an estimated incidence of 1 in 10 to 30 million births. The karyotype almost always is 46,XY, and the usual appearance is that of a well-developed scrotum with descended testes and an absent penile shaft. The anus is usually displaced anteriorly. Associated malformations are common and include cryptorchidism, vesicoureteral reflux, horseshoe kidney, renal agenesis, imperforate anus, and musculoskeletal and cardiopulmonary abnormalities.
7. b. Small penis. A buried penis can be classified into three categories based on etiology for the concealment: (1) poor penopubic fixation of the skin at the base of the penis; (2) obesity; and (3) a trapped penis from cicatricial scarring after penile surgery, typically a circumcision.
8. a. Topical betamethasone and manual retraction.Young children with secondary cicatricial scarring after penile surgery can undergo forceful dilation of the cicatrix with a fine hemostat in the office after the application or injection of analgesia. Another option is the combination of topical betamethasone and manual retraction.
9. b. 1.9 cm.Stretched penile length is determined by measuring the penis from its attachment to the pubic symphysis to the tip of the glans. One must be careful to depress the suprapubic fat pad completely to obtain an accurate measurement, especially in an obese infant or child. In general, the penis of a full-term neonate should be at least 1.9 cm long.
10. e. It is less than 1.9 cm in stretched length.Micropenis is a normally formed penis that is at least 2.5 SD below the mean size in stretched length for age. The ratio of the length of the penile shaft to its circumference is usually normal, but occasionally the corpora cavernosa are severely hypoplastic. The testes are usually small and frequently cryptorchid, whereas the scrotum is usually fused and often diminutive. A stretched penile length less than 1.9 cm long is consistent with a micropenis.
11. b. Hypogonadotropic hypogonadism.The most common cause of micropenis is hypogonadotropic hypogonadism, which is the failure of the hypothalamus to produce an adequate amount of gonadotropin-releasing hormone (GnRH). This condition may result from hypothalamic dysfunction, which can occur in Prader-Willi syndrome, Kallmann syndrome (genital-olfactory dysplasia), Laurence-Moon-Biedl syndrome, and the CHARGE association.
12. c. Congenital penile nevi tend to be malignant.Congenital penile nevi tend to be superficial and benign. Congenital penile nevi are pigmented lesions that can form on the glans and penile shaft. They tend to be superficial and benign and should be excised.
13. c. Intracavernous injections of β-adrenergic sympathomimetic agents.The initial treatment of low-flow priapism resulting from sickle cell disease is conservative with hydration, oxygenation, alkalization, analgesia, and transfusion with the goal of reducing hemoglobin S concentration. Evacuation of blood and irrigation of the corpora cavernosa along with intracavernous injections of α-adrenergic sympathomimetic agents, such as phenylephrine or epinephrine solution, can be a concurrent therapy. Surgical intervention to allow corporeal drainage by shunt procedures is indicated if there is a lack of response to medical therapy.
14. c. Color Doppler ultrasonography commonly demonstrates the fistula. High-flow priapism is usually due to perineal trauma, such as a straddle injury. Corporeal irrigation is diagnostic and therapeutic.Typically, the aspirated blood is bright red and the aspirate is similar to arterial blood on blood gas analysis. Color Doppler ultrasonography often will demonstrate the fistula. The initial management is observation because spontaneous resolution may occur.Superselective embolization of cavernous and penile arteries is the next line of therapy. If not, angiographic embolization is indicated.
15. b. Distal shaft hypospadias with chordee.Frequently, penoscrotal transposition occurs in conjunction with perineal, scrotal, or penoscrotal hypospadias with chordee. Penoscrotal transposition has also been associated with caudal regression, sex chromosome abnormalities, and Aarskog syndrome. As many as 75% of patients with complete penoscrotal transposition and a normal scrotum have a significant urinary tract abnormality, including renal agenesis and dysplasia, and other nongenitourinary anomalies.
16. d. Spermatic cord torsion. Risk of torsion is associated with abnormal development of the tunica vaginalis but not patency of the processus vaginalis.
17. b. Epididymal anomalies. The processus vaginalis is closed in cases of abdominoscrotal hydrocele; and an elongated dysmorphic testis, increased pressure within the tunica vaginalis, and hydronephrosis have all been reported.
18. c. 4. Irreversible ischemic injury to the testicular parenchyma may begin as soon as 4 hours after occlusion of the cord.
19. d. Spermatic cord twist on high-resolution Doppler ultrasonography. Spermatic cord twist on high-resolution Doppler imaging is the most specific finding, that is, the least likely to be false positive, in spermatic cord torsion.
20. e. Immediate scrotal exploration. It should be remembered that manual detorsion may not totally correct the rotation that has occurred and that prompt exploration is still indicated.
21. c. Elective scrotal exploration. If the suspicion is strong that episodes of intermittent torsion and spontaneous detorsion have occurred, the author's experience has been that the finding of a bell-clapper deformity at exploration can be expected. Elective scrotal exploration should be performed, with scrotal fixation of both testes.
22. a. Observation. When the diagnosis of a torsed appendage is confirmed clinically or by imaging, nonoperative management will allow most cases to resolve spontaneously.
23. e. Radiographically normal urinary tract. The majority of infants with epididymitis have sterile urine and apparently radiographically normal urinary tracts.
24. b. Surgical exploration of the affected testis with contralateral scrotal orchidopexy. Clearly, if the cause of scrotal swelling appears to be related to an acute postnatal event, all efforts should be made to pursue prompt surgical intervention. If torsion is confirmed, contralateral scrotal exploration with testicular fixation should be performed.
25. c. Asymptomatic. Most adolescent varicoceles are asymptomatic.
26. d. 15% to 20%. In adults and adolescents, testicular size (volume) should be approximately equal bilaterally, with the normal differential not being more than 15% to 20% volume.
27. e. Transvenous embolization. Hydrocele formation is related to failure to preserve lymphatic vessels associated with the spermatic cord and its vessels. Hydrocele formation seems most common after retroperitoneal ligation, especially when a mass ligation technique is used, and is least likely to occur after transvenous embolization.
28. d. Continuous spermatic venous reflux. Significant pain associated with varicocele, bilateral small testes, and oligospermia are reasonable indications to proceed with repair in an adolescent male.The standard indication is ipsilateral testicular volume loss, or hypotrophy, of at least 15% to 20%, although this should be documented on serial yearly testicular examinations, because variable growth of the testes may occur during puberty. Continuous reflux may be documented on color Doppler imaging but is not a specific indication for surgery.

Chapter review

1. The normal penile size of a neonate is 3.5 ± 0.7 cm in stretched length. It should be at least 1.9 cm. If it is below 1.9 cm, it is classified as a micropenis.
2. The potential benefits of circumcision include prevention of penile cancer; urinary tract infections; sexually transmitted diseases, including human immunodeficiency virus infection; and phimosis.
3. Glanular adhesions and skin bridges are not uncommon complications of circumcision.
4. Meatal stenosis is a condition that occurs almost exclusively in children after infant circumcision.
5. If a meatotomy is performed, suturing the urethral mucosa to the glans with fine, resorbable sutures reduces the risk of recurrence.
6. The causes of micropenis include (a) hypogonadotropic hypogonadism, (b) hypergonadotropic hypogonadism (primary testicular failure), and (c) idiopathic causes.
7. Most men born with micropenis have male gender identity and satisfactory sexual function.
8. Priapism can be ischemic (veno-occlusive, low flow), nonischemic (arterial, high flow), and stuttering (intermittent).
9. In the female and the male with abnormal testosterone and/or dihydrotestosterone production, 5α-reductase deficiency or androgen receptor dysfunction, the genital tubercle, genital folds and genital swelling becomes the clitoris, labia minora and labia majora, respectively.
10. True micropenis is often due to a deficiency of gonadotropins.
11. In penile torsion, the glans may be rotated but the corpora cavernosa and corpora spongiosum at the base of the penis are normal.
12. Urethral duplication usually occurs in the sagittal plane.
13. Inguinal hernias are more common in premature infants.
14. There is a familial predisposition to intravaginal testicular torsion.
15. An absent cremasteric reflex is associated with testicular torsion.
16. There is no convincing evidence that testicular torsion results in antisperm antibodies.
17. The influence of dihydrotestosterone on the androgen receptors during development results in the differentiation of the genital tubercle, genital (labioscrotal) folds, and genital swelling into the male structures of the glans penis, penile shaft, and scrotum, respectively.
18. Circumcision should not be performed in neonates with other penile conditions that require surgical correction. These conditions include hypospadias, penile curvature, dorsal hood deformity, buried penis, and webbed penis.
19. A buried penis has three etiologies: (1) poor penopubic fixation of the skin at the base of the penis; (2) obesity; and (3) a trapped penis from cicatricial scarring after penile surgery, typically a circumcision.
20. High-flow priapism is usually due to perineal trauma, such as a straddle injury. Corporeal irrigation is diagnostic and therapeutic.
21. In testicular torsion, irreversible ischemic injury to the testicular parenchyma may begin as soon as 4 hours after occlusion of the cord.
22. Significant pain associated with varicocele, bilateral small testes, and oligospermia are reasonable indications to proceed with repair in an adolescent male.