9: Conditions of the external genitalia Flashcards
1
Q
What are labial adhesions in females?
A
Labial adhesions are a condition in which the labia minora, the inner folds of skin around the vaginal opening, are fused together.
2
Q
What is the epidemiology of labial adhesions in females?
A
Labial adhesions occur in 0.6% to 1.8% of females, usually in those under the age of 2 years.
3
Q
How do labial adhesions typically present?
A
Labial adhesions are usually asymptomatic, but may cause postvoid dribbling, a deviated urinary stream, or local irritation. They are usually seen on exam or noticed by a parent.
4
Q
What is the treatment for labial adhesions in females?
A
Treatment is only necessary if the adhesions are symptomatic. Most labial adhesions will resolve spontaneously. Topical conjugated estrogen (0.625 mg/g) is successful in 90% of cases, but can have side effects such as breast budding and skin hyperpigmentation. Manual separation with a lubricated probe after lidocaine cream can also be used. Recurrence is common without continued application of moisturizing ointment.
5
Q
What is the success rate of topical conjugated estrogen for treating labial adhesions in females?
A
Topical conjugated estrogen (0.625 mg/g) is successful in 90% of cases of labial adhesions in females.
6
Q
What are the potential side effects of using topical conjugated estrogen for treating labial adhesions in females?
A
Potential side effects of using topical conjugated estrogen for treating labial adhesions in females include breast budding and skin hyperpigmentation.
7
Q
What is manual separation with a lubricated probe after lidocaine cream?
A
Manual separation with a lubricated probe after lidocaine cream is a procedure that involves using a lubricated probe to manually separate the fused labia minora after applying lidocaine cream to numb the area.
8
Q
Is recurrence common after treating labial adhesions in females?
A
Yes, recurrence is common after treating labial adhesions in females, and continued application of moisturizing ointment may be necessary to prevent recurrence.
9
Q
What is urethral prolapse in females?
A
Answer: Urethral prolapse in females is the circumferential eversion of the urethral mucosa.
10
Q
Who is more prone to urethral prolapse in females?
A
Answer: Prepubertal black girls and postmenopausal women are more prone to urethral prolapse in females.
11
Q
What are the symptoms of urethral prolapse in females?
A
Answer: Bleeding from the mucosa causes blood spotting in females with urethral prolapse.
12
Q
What are the treatment options for urethral prolapse in females?
A
Answer: The treatment options for urethral prolapse in females include observation, sitz baths, topical corticosteroids, and conjugated estrogens. Surgical excision of redundant mucosa is necessary for recurrence.
13
Q
Image
FIG. 9.1 (A and B) Urethral prolapse.
A
14
Q
What is a paraurethral cyst in females?
A
A paraurethral cyst in females is a dilation of periurethral glands (Skene’s glands) just inside the urethral meatus.
15
Q
How does a paraurethral cyst form in neonates?
A
In neonates, the glands respond to maternal estrogen and secrete mucoid material resulting in cyst formation.
16
Q
What are the symptoms of a paraurethral cyst in females?
A
A paraurethral cyst displaces the urethral meatus and produces a deviated urinary stream.
17
Q
How is a paraurethral cyst treated?
A
A paraurethral cyst frequently ruptures spontaneously, but if it persists, it can be drained by needle puncture.
18
Q
What is Gartner’s duct cyst?
A
Gartner’s duct cyst is a cystic structure that represents incomplete regression of the wolffian duct along the anteromedial wall of the vagina.
19
Q
What causes Gartner’s duct cyst?
A
Gartner’s duct cyst occurs when the wolffian duct fails to regress completely during embryonic development.
20
Q
What are the symptoms of Gartner’s duct cyst?
A
In many cases, Gartner’s duct cyst is asymptomatic and is only discovered incidentally during a routine gynecological exam. However, in some cases, it can cause pain, discomfort, and swelling in the vulvar area.
21
Q
How is Gartner’s duct cyst treated?
A
Gartner’s duct cyst is usually treated by incising it to relieve obstruction. It can also be injected with contrast to delineate the anatomy. If an ectopic ureter is present and draining a dysplastic kidney or upper pole segment, surgical intervention may be necessary to correct the incontinence.
22
Q
What are the potential complications of Gartner’s duct cyst?
A
If left untreated, Gartner’s duct cyst can lead to chronic pain and discomfort in the vulvar area. In rare cases, it can become infected or develop into a malignancy.
23
Q
What is imperforate hymen?
A
Imperforate hymen is a condition where the hymenal membrane lacks an opening, which can cause the retention of vaginal secretions due to maternal estradiol stimulation.
24
Q
What is the epidemiology of imperforate hymen?
A
It is the most common congenital obstructive anomaly of the female reproductive tract.
25
How is imperforate hymen diagnosed?
It is usually diagnosed at birth when a whitish bulge is seen, but it can also be identified on examination or in an adolescent with amenorrhea.
26
What is the treatment for imperforate hymen in newborns?
In newborns, it may be incised transversely at the bedside.
27
Why should needle drainage not be performed for imperforate hymen?
Needle drainage should not be performed because it may cause damage to the surrounding tissues and worsen the condition.
28
How is imperforate hymen treated in older children?
In older children, incision under anesthesia is performed.
29
Image
FIG. 9.2 Imperforate hymen. Note the distention from vaginal secretions.
30
What are hymenal skin tags and what causes them?
Answer: Hymenal skin tags are small excess hymenal tissue found in the female external genitalia. They are often a normal finding and are not necessarily caused by any specific factors.
31
What is the vaginal septum and where is it usually located?
Answer: The vaginal septum is a thin wall or membrane that can be found at various levels within the vagina, most commonly in the middle and upper third of the vagina.
32
What are the typical characteristics of the vaginal septum?
Answer: The vaginal septum is usually less than 1 cm thick and may have small perforations.
33
What is the epidemiology of vaginal septum?
Answer: Vaginal septum is a rare condition, affecting about 1 in 70,000 females.
34
What are the common symptoms of vaginal septum?
Answer: Women with vaginal septum may present with amenorrhea (absence of menstruation) and a distended upper vagina.
35
What are the imaging methods used to diagnose vaginal septum?
Answer: Diagnostic imaging methods used to identify vaginal septum include transperineal, transrectal, or abdominal ultrasound, and magnetic resonance imaging (MRI).
36
How can a high transverse septum be distinguished from congenital absence of a cervix?
Answer: A high transverse septum can be distinguished from congenital absence of a cervix through imaging studies such as MRI, which can provide a more detailed view of the anatomy.
37
What is the treatment for vaginal septum?
Answer: The treatment for vaginal septum may involve delaying surgery with hormonal suppression, followed by incision or complete excision of the septum with Z-plasties or a vaginal mold to prevent vaginal stenosis.
38
What is vaginal atresia?
Answer: Vaginal atresia is a condition where the distal vagina fails to form from the urogenital sinus, while Müllerian structures remain unaffected.
39
How is vaginal atresia diagnosed?
Answer: Vaginal atresia can be diagnosed by a distended vagina that can be palpable on rectal exam.
40
What imaging techniques are used to define Müllerian structures in vaginal atresia?
Answer: Ultrasound and/or MRI imaging techniques are used to define Müllerian structures in vaginal atresia.
41
What is the treatment for vaginal atresia?
Answer: The treatment for vaginal atresia involves a transverse incision at the hymenal ring with dissection proximally to the upper vagina. A pull-through procedure is performed to bring the vagina to the introitus, sometimes with skin flaps.
42
Are Müllerian structures affected in vaginal atresia?
Answer: No, Müllerian structures are not affected in vaginal atresia.
43
What is vaginal agenesis?
Vaginal agenesis is a congenital absence of the proximal vagina in an otherwise intact female.
44
What is the epidemiology of vaginal agenesis?
It affects 1 in 5000 live female births.
45
What are the two forms of vaginal agenesis?
The two forms of vaginal agenesis are type A (typical) and type B (atypical).
46
What is the difference between type A and type B vaginal agenesis?
Type A vaginal agenesis is characterized by symmetrical uterine remnants and normal fallopian tubes. Type B vaginal agenesis is characterized by asymmetrical uterine buds and abnormally developed fallopian tubes, with abnormalities in other organ systems.
47
How is vaginal agenesis diagnosed?
Vaginal agenesis is diagnosed when a female presents with amenorrhea. The hymenal ring and small distal vaginal pouch are present, as these derive from the urogenital sinus.
48
What imaging techniques are used to diagnose vaginal agenesis?
Ultrasound and MRI are used to delineate remnant Müllerian structures, identify if the cervix is present, and identify associated renal or skeletal anomalies.
49
What are the treatment options for vaginal agenesis?
Nonoperative treatment involves gradual pressure on the perineum with dilators to create progressive invagination of the vagina. Operative treatment involves skin or intestinal neovagina or buccal mucosal vaginoplasty. Neovagina is connected to the uterus only if the cervix is present.
50
What are the complications of vaginal agenesis treatment?
Vaginal stenosis is a common complication that occurs in skin > ileum neovagina > sigmoid neovagina.
51
Image
FIG. 9.3 Vaginal rhabdomyosarcoma.
52
What is vaginal rhabdomyosarcoma?
Vaginal rhabdomyosarcoma is a type of cancer that occurs in the vagina, specifically in the muscles of the vaginal wall. It is primarily an embryonal cell type.
53
What is the best prognosis of female genital tract tumors?
According to the description, vaginal rhabdomyosarcoma has the best prognosis of female genital tract tumors.
54
What is the average age of patients diagnosed with vaginal rhabdomyosarcoma?
The epidemiology section states that the mean age of patients diagnosed with vaginal rhabdomyosarcoma is under 2 years.
55
What are the common symptoms of vaginal rhabdomyosarcoma?
The diagnosis section mentions that common symptoms of vaginal rhabdomyosarcoma include bleeding or a visible mass bulging from the introitus, which may sometimes be "grapelike" (botryoid) in appearance.
56
How is vaginal rhabdomyosarcoma diagnosed?
Vaginal rhabdomyosarcoma is diagnosed through tissue biopsy. Imaging, such as abdominal/pelvis/chest CT and bone marrow biopsy, may also be used for staging.
57
What is the treatment for vaginal rhabdomyosarcoma?
The recommended first-line treatment for vaginal rhabdomyosarcoma is chemotherapy. Surgery may follow for local resection or restaging, as outlined in Chapter 11.
58
What is clitoral hypertrophy?
Clitoral hypertrophy is a condition where the clitoral tissue is enlarged, usually associated with common urogenital sinus.
59
What is the etiology of clitoral hypertrophy?
Clitoral hypertrophy is usually caused by an enzymatic defect in adrenal steroid synthesis producing excess androgen metabolites. The most common cause is a deficiency of 21-hydroxylase or 11-hydroxylase. It can also be caused by androgen-producing maternal tumors or local growth factor from neurofibromas.
60
How is clitoral hypertrophy evaluated?
Clitoral hypertrophy is evaluated through serum electrolytes, 17-hydroxyprogesterone level, and karyotype.
61
What is the treatment for clitoral hypertrophy?
If the cause of clitoral hypertrophy is congenital adrenal hyperplasia (CAH), the treatment is replacement of glucocorticoids and mineralocorticoids to prevent the production of androgens and further stimulation of the external genitalia.
62
What is a prolapsed ureterocele in female external genitalia?
Answer: A prolapsed ureterocele is a condition where a large ureterocele protrudes through the urethra in the female external genitalia.
63
What are the symptoms of prolapsed ureterocele?
Answer: The symptoms of prolapsed ureterocele include a pink or dark purple bulge from the urethra, which can cause urinary retention.
64
How is prolapsed ureterocele diagnosed?
Answer: Prolapsed ureterocele is diagnosed by observing a bulge from the urethra. Additionally, renal and bladder ultrasound (RBUS) may be used to confirm the diagnosis.
65
What is the treatment for prolapsed ureterocele?
Answer: The treatment for prolapsed ureterocele may involve needle decompression or incision and reduction. Placement of a urethral catheter may also be necessary for treatment.
66
What are the possible complications of untreated prolapsed ureterocele?
Answer: Untreated prolapsed ureterocele can lead to urinary tract infections, kidney damage, and sepsis. It is essential to seek medical attention for any unusual symptoms in the external genitalia.
67
Image
FIG. 9.4 (A and B) Prolapsed Ureterocele.
68
What is a urogenital sinus/cloacal anomaly?
A urogenital sinus/cloacal anomaly is a condition in which the vagina and the urinary tract merge, typically occurring from the bladder to the urethral meatus. In cases of persistent cloaca, the rectum also joins the vagina posteriorly, resulting in a single perineal opening.
69
What is the incidence of urogenital sinus/cloacal anomaly in females?
The incidence of urogenital sinus/cloacal anomaly in females is approximately 1 in 500.
70
What is the most common association of urogenital sinus/cloacal anomaly?
Urogenital sinus/cloacal anomaly is usually associated with congenital adrenal hyperplasia (CAH).
71
What are the diagnostic features of urogenital sinus/cloacal anomaly?
The diagnostic feature of urogenital sinus/cloacal anomaly is a single perineal opening. In addition, a suprapubic mass may be present due to a distended bladder or hydrometrocolpos.
72
What imaging modalities are used in the diagnosis of urogenital sinus/cloacal anomaly?
RBUS (real-time bladder ultrasound) and pelvic ultrasound are used in the diagnosis of urogenital sinus/cloacal anomaly. MRI may also be considered in some cases.
73
What is the most critical factor in the surgical management of urogenital sinus/cloacal anomaly?
The level of confluence in relation to the bladder neck is the most critical factor in the surgical management of urogenital sinus/cloacal anomaly. Urogenital mobilization is typically performed to bring the urethra and vagina to the perineum, followed by catheterization.
74
What is an inguinal hernia in females?
Answer: An inguinal hernia in females is a condition where the patent processus vaginalis extends beyond the internal inguinal ring and contains abdominal contents such as peritoneal fluid, bowel, omentum, gonads.
75
Is inguinal hernia more common in boys or girls?
Answer: Inguinal hernia is rarer in girls than boys.
76
What is the association of inguinal hernia with complete androgen insensitivity syndrome (CAIS)?
Answer: Inguinal hernia may be associated with complete androgen insensitivity syndrome (CAIS).
77
What are the symptoms of inguinal hernia in females?
Answer: The symptoms of inguinal hernia in females include inguinal bulge and occasionally pain.
78
What is the treatment for inguinal hernia in females?
Answer: The treatment for inguinal hernia in females includes open or laparoscopic closure of patent processus. It is important to ensure phenotypic females are not genetically male by performing a pelvic ultrasound, vaginoscopy to see the cervix and identify the ovary and fallopian tube through the hernia sac.
79
What is phimosis?
Phimosis is a condition where the foreskin of the penis is too tight and cannot be pulled back over the head of the penis.
80
What causes phimosis?
Phimosis can be a normal condition in newborns, but it can become pathologic later on due to chronic irritation from urine or balanitis.
81
What are the symptoms of phimosis?
Symptoms of phimosis can include difficulty urinating, pain during sexual activity, and swelling of the foreskin.
82
How is phimosis treated?
In physiologic cases (due to smegma and erections), phimosis can resolve spontaneously by 3-4 years. If the condition is pathologic, first-line treatment is with steroid cream. In severe cases, circumcision may be necessary.
83
What is the difference between physiologic and pathologic phimosis?
Physiologic phimosis is a normal condition in newborns where the foreskin is tight and cannot be retracted. This condition usually resolves on its own by 3-4 years of age. Pathologic phimosis occurs later on due to chronic irritation and can cause pain and difficulty with urination and sexual activity.
84
Image
FIG. 9.5 Conditions associated with the uncircumcised penis. (A) Phimosis caused by a preputial ring. (B) Paraphimosis with associated entrapped prepuce behind the glans penis.
85
What is paraphimosis?
Paraphimosis is a medical condition that occurs when the foreskin of the penis gets trapped behind the glans and cannot be pulled back to its normal position.
86
What are the consequences of paraphimosis?
If left untreated, paraphimosis can cause severe edema (swelling) of the foreskin within hours, and can lead to ischemia (loss of blood supply) of the glans.
87
How is paraphimosis treated?
Paraphimosis can be treated by manually reducing the foreskin after pressure/icing to reduce edema. If this is unsuccessful, a dorsal slit procedure may be necessary.
88
What is a dorsal slit procedure?
A dorsal slit procedure is a minor surgical procedure that involves making a small incision on the foreskin to relieve pressure and allow it to be retracted to its normal position.
89
What should be done in case of severe edema of the foreskin?
In case of severe edema of the foreskin, pressure/icing should be applied to reduce the swelling, and manual reduction or a dorsal slit procedure should be performed to relieve the trapped foreskin. It is important to seek medical attention immediately to prevent complications.
90
Smegma
Description – Entrapped sloughed skin cells under unretractable prepuce or under glanular adhesions after circumcision.
Diagnosis – White, round, smooth-walled lesion under skin.
Treatment – Resolution as adhesions open.
Answer: Smegma refers to the collection of dead skin cells, oils, and other bodily fluids that can accumulate under the foreskin of an uncircumcised penis or in the folds of skin around the clitoral hood in females.
91
What are the benefits of circumcision?
Circumcision can help prevent penile cancer, decrease urinary tract infections (UTI), and decrease the risk of sexually transmitted diseases.
92
What are the complications of circumcision?
Answer: The complications of circumcision range from 0.2%–5% of boys and can include bleeding (0.1%), wound infection, skin separation, cicatricial scar or secondary phimosis.
93
What is skin separation after circumcision?
Answer: Skin separation after circumcision appears as a loss of penile shaft skin. It can be treated with topical petroleum-based ointment, which usually leads to a normal outcome.
94
How is cicatricial scar or secondary phimosis after circumcision treated?
Answer: Cicatricial scar or secondary phimosis after circumcision is treated with betamethasone 0.05% or 0.1% (2–3x daily for 21–30 days).
95
What is meatal stenosis?
Meatal stenosis is the narrowing of the urethral meatus, which is the opening at the end of the penis where urine exits.
96
What is the cause of meatal stenosis?
Meatal stenosis can occur after circumcision, when the foreskin is removed and the remaining tissue heals improperly.
97
What are the symptoms of meatal stenosis?
The main symptoms of meatal stenosis include a dorsally deflected (upward-pointing) stream of urine and a narrow urinary stream.
98
How is meatal stenosis treated?
Meatal stenosis is typically treated with a meatotomy, which is a midline ventral incision to enlarge the meatus. This can be done in a clinic or in an operating room (OR). In some cases, a meatoplasty may be necessary, which involves excision of a wedge of ventral glans and suturing the edges of the urethral mucosa to the glans.
99
What is a meatotomy?
A meatotomy is a procedure in which a midline ventral incision is made to enlarge the meatus, which is the opening at the end of the penis.
100
What is a meatoplasty?
A meatoplasty is a more extensive surgical procedure than a meatotomy, which involves excision of a wedge of ventral glans and suturing the edges of the urethral mucosa to the glans.
101
Can meatal stenosis be prevented?
Meatal stenosis can be prevented by leaving the foreskin intact and avoiding circumcision, which is not medically necessary in most cases.
102
What is a parameatal cyst in male external genitalia?
Answer: Parameatal cyst is a small blister or cyst that appears near the urethral meatus in the male external genitalia.
103
What is the composition of the cyst wall in parameatal cyst?
Answer: The cyst wall of parameatal cyst is made up of transitional and squamous or columnar epithelium.
104
What is the recommended treatment for a parameatal cyst?
Answer: The recommended treatment for a parameatal cyst is complete excision.
105
Is a parameatal cyst a common condition in males?
Answer: Parameatal cyst is a relatively uncommon condition in males.
106
Can a parameatal cyst cause any complications?
Answer: Generally, parameatal cysts do not cause any complications, but they can sometimes become infected or inflamed, leading to discomfort or pain. Therefore, it is recommended to get them treated as soon as possible.
107
What is balanitis xerotica obliterans (BXO)?
A: BXO is a chronic inflammatory and infiltrative dermatosis that affects the glans, meatus, and urethra of the penis. It can cause phimosis, which is a tightening of the foreskin.
108
What are the symptoms of BXO?
A: The symptoms of BXO include a tight, scarred foreskin, foreskin irritation, discomfort, bleeding, and acute urinary retention.
109
How is BXO treated?
A: Topical steroids such as betamethasone or clobetasol or systemic tacrolimus can be used to treat the meatus. Circumcision is recommended for the prepuce. After hypospadias repair, excising and replacing the tissue may be necessary.
110
What are the risks associated with BXO?
A: BXO recurs in 20%-40% of cases.
111
What is Lichen sclerosus et atrophicus?
A: Lichen sclerosus et atrophicus is a chronic inflammatory disease that affects the skin of the genital area, including the external genitalia in men.
112
What are the symptoms of Lichen sclerosus et atrophicus?
A: The symptoms of Lichen sclerosus et atrophicus include thinning and whitening of the skin, itching, and pain.
113
How is Lichen sclerosus et atrophicus treated?
A: The treatment of Lichen sclerosus et atrophicus includes topical corticosteroids and immunosuppressive agents.
114
What are the potential complications of Lichen sclerosus et atrophicus?
A: The complications of Lichen sclerosus et atrophicus include scarring, phimosis, and squamous cell carcinoma.
115
What is an inconspicuous penis?
An inconspicuous penis is a condition where the penis is enclosed in skin, making it appear small despite having a normal stretched penile length.
116
What are the different types of inconspicuous penis?
There are three types of inconspicuous penis: buried/hidden penis, trapped penis, and webbed penis.
117
What is a buried/hidden penis?
A buried/hidden penis is a condition where the penis is hidden or buried under the skin, making it difficult to see or access.
118
What is a trapped penis?
A trapped penis is a condition where the penis is trapped by fibrous bands or scar tissue, resulting in its enclosure by skin.
119
What is a webbed penis?
A webbed penis is a condition where the skin of the scrotum extends up onto the base of the penis, making it appear smaller.
120
What are the causes of an inconspicuous penis?
The causes of an inconspicuous penis can be congenital (poor penopubic/penoscrotal fixation of skin) or acquired (obesity or trapping from cicatricial scarring).
121
What is the surgical correction for an inconspicuous penis?
The surgical correction for an inconspicuous penis involves degloving with removal of fibrous bands and fixing subcutaneous tissue to Bucks fascia at penopubic and penoscrotal junctions. Preputial skin is used for ventral skin coverage, and an alternative for webbing is a transverse incision of the scrotal web and vertical closure.
122
Image
FIG. 9.6 Buried penis (A and C), which may be visualized by retraction of the skin lateral to the penile shaft (B and D).
123
What is penile curvature?
Answer: Penile curvature, also known as chordee, is an abnormal bending or curvature of the penis, usually in a ventral direction but can also occur dorsally or laterally.
124
What is the usual cause of penile curvature?
Answer: Penile curvature is usually associated with hypospadias, a congenital condition in which the urethral opening is located on the underside of the penis, but it can also be caused by overgrowth or hypoplasia of the corporal body.
125
How is penile curvature surgically corrected?
Answer: The surgical correction of penile curvature involves degloving the penis, excising fibrous tissue superficial to Buck fascia, and if resolved, skin coverage. If the curvature persists, dorsal plication, urethral division, and/or corporal grafting may be necessary. Flaps are also used for skin coverage.
126
What is the role of Buck fascia in penile curvature surgery?
Answer: Buck fascia is a layer of connective tissue that covers the corpora cavernosa of the penis. In penile curvature surgery, the fibrous tissue superficial to Buck fascia is excised to correct the curvature.
127
Can penile curvature be corrected without surgery?
Answer: Penile curvature cannot be corrected without surgery. However, in mild cases, the use of vacuum erection devices, penile traction therapy, and intralesional injection of collagenase may be beneficial in reducing the curvature.
128
What is penile torsion?
Penile torsion is a rotational deformity of the penile shaft, usually counterclockwise. The median raphe spirals around the shaft.
129
How is penile torsion surgically corrected?
Answer: The surgical correction of penile torsion involves degloving the penis, rotating the glans opposite to the defect, and suturing the skin and dartos to the coronal collar. In case of severe torsion, proximal anchoring sutures or rotational dartos flap may also be used.
130
What is the median raphe?
Answer: The median raphe is a seam-like structure that runs down the midline of the penis and scrotum, representing the fusion of the two halves during fetal development.
131
Is penile torsion a common condition?
Answer: Penile torsion is a relatively rare condition and can occur either at birth or later in life due to trauma or scarring.
132
How does penile torsion affect sexual function?
Answer: Penile torsion may affect the cosmetic appearance of the penis but usually does not affect sexual function or performance. However, severe torsion may cause pain or discomfort during erection.
133
What is a congenital hemangioma and what are the types?
A congenital hemangioma is a type of benign tumor that develops in the blood vessels at birth. The most common types are cutaneous or strawberry hemangioma and subcutaneous or cavernous hemangioma.
134
How does a cutaneous hemangioma typically grow and what happens over time?
Cutaneous hemangiomas grow rapidly for 3-6 months and then typically begin to involute or shrink.
135
How does a subcutaneous hemangioma differ from a cutaneous hemangioma?
A subcutaneous hemangioma is a vascular malformation that tends to enlarge over time, while a cutaneous hemangioma is a benign tumor that tends to shrink over time.
136
What diagnostic tests are used to evaluate the size and depth of a congenital hemangioma?
Color Doppler ultrasound (CDUS), computed tomography (CT), or MRI may be used to delineate the size and depth of a congenital hemangioma.
137
What are the treatment options for cutaneous and subcutaneous hemangiomas?
For cutaneous hemangiomas, short-term oral corticosteroids or propranolol may be used. For subcutaneous hemangiomas, en bloc resection may be necessary, and preoperative angioembolization may be performed to reduce the size and risk of bleeding.
138
What diagnostic tests are used to evaluate the size and depth of a congenital hemangioma?
Color Doppler ultrasound (CDUS), computed tomography (CT), or MRI may be used to delineate the size and depth of a congenital hemangioma.
139
What are the treatment options for cutaneous and subcutaneous hemangiomas?
For cutaneous hemangiomas, short-term oral corticosteroids or propranolol may be used. For subcutaneous hemangiomas, en bloc resection may be necessary, and preoperative angioembolization may be performed to reduce the size and risk of bleeding.
140
What is genital lymphedema?
Genital lymphedema is a condition that causes swelling in the penile and scrotal area due to impaired lymphatic drainage.
141
What are the causes of genital lymphedema?
Genital lymphedema can be caused by congenital factors, such as an inherited genetic mutation or sporadic development, or it can be acquired.
142
What percentage of people with genital lymphedema present at puberty?
Approximately 80% of people with genital lymphedema present at puberty.
143
Is genital lymphedema associated with any other diseases?
Genital lymphedema is associated with Crohn's disease, specifically anogenital granulomatomosis.
144
What is the initial treatment for genital lymphedema?
The initial treatment for genital lymphedema is observation, where the condition is monitored to see if it progresses.
145
What medication has been used to treat genital lymphedema?
Azathioprine has been used to treat genital lymphedema.
146
When is surgery recommended for genital lymphedema?
Surgery is recommended if the condition is significant or if it progresses. The surgery involves removing all involved tissue.
147
What is azathioprine?
Azathioprine is an immunosuppressive medication that is commonly used to prevent the rejection of transplanted organs or to treat autoimmune disorders. It works by suppressing the immune system, which can help control the symptoms of diseases like genital lymphedema. However, it also carries potential side effects and requires close monitoring by a healthcare provider.
148
What is hypospadias?
Hypospadias is a condition where the urethral opening in males is located on the underside of the penis instead of at the tip.
149
What is the classic triad associated with hypospadias?
What is hypospadias?
The classic triad includes an ectopic, ventrally located urethral meatus, ventral penile curvature, and incomplete, dorsally hooded foreskin.
150
What is the epidemiology of hypospadias?
Hypospadias affects 1 in 150 to 300 live births, and there is a wide spectrum of severity ranging from minimal defects to severe proximal hypospadias.
151
What is the recommended workup for hypospadias?
In addition to evaluating for hypospadias, it is recommended to evaluate for inguinal hernia/hydrocele and cryptorchidism. If associated with undescended testes, testing for disorder of sexual differentiation (DSD) may be necessary. Routine imaging is not recommended.
152
What are the goals of surgical correction for hypospadias?
The goals of surgical correction are to correct penile curvature and advance the urethra. Surgery is ideally performed between 6-12 months of age.
153
Is preoperative androgen stimulation recommended for hypospadias surgery?
Preoperative androgen stimulation is controversial. While it may lead to a larger glans for tubularization, it can also impair wound healing and lead to the development of secondary male characteristics.
154
What is the perioperative consideration for urinary diversion during hypospadias surgery?
A soft urethral stent is often used to facilitate healing and may decrease the risk of meatal stenosis and urethrocutaneous fistula.
155
Are antibiotics necessary for hypospadias surgery?
Perioperative antibiotics are recommended, and prophylaxis for the stent may also be necessary.
156
What is the recommended wound care for hypospadias surgery?
Petroleum-based ointment is recommended for wound care.
157
How is postoperative pain controlled after hypospadias surgery?
A combination of acetaminophen and nonsteroidal anti-inflammatory drugs (NSAIDs) is recommended, and ibuprofen is considered safe even in the immediate postoperative period. Narcotics should be minimized.
158
What is the basic procedure for treating hypospadias in males?
Answer: The basic procedure for treating hypospadias in males involves assessing the location of the urethral meatus and the degree of curvature. A circumcising incision is made, followed by degloving proximal to the urethral meatus. The curvature is then corrected, and a urethroplasty is performed if it is a single-stage repair. If a staged repair is necessary, the skin is transferred to the ventrum for the second stage.
159
What do you need to assess in the basic procedure for treating hypospadias?
Answer: The basic procedure for treating hypospadias involves assessing the location of the urethral meatus and the degree of curvature.
160
What is the purpose of the circumcising incision in the basic procedure for treating hypospadias?
Answer: The circumcising incision is made to allow access to the urethral meatus and the proximal urethra.
161
When is a urethroplasty performed in the basic procedure for treating hypospadias?
Answer: A urethroplasty is performed if the repair is a single-stage repair.
162
What is the difference between a single-stage and staged repair for hypospadias?
Answer: In a single-stage repair, the curvature is corrected, and a urethroplasty is performed. In a staged repair, the skin is transferred to the ventrum for the second stage.
163
Why is it important to correct the curvature in hypospadias?
Answer: It is important to correct the curvature in hypospadias to allow for normal urination and sexual function.
164
What is degloving in the basic procedure for treating hypospadias?
Answer: Degloving is the process of separating the skin from the underlying tissue. In the basic procedure for treating hypospadias, degloving is done proximal to the urethral meatus to allow for access to the urethra.
165
Image
FIG. 9.7 Algorithm for management of penile curvature. Once the penis is degloved, artificial erection is performed. If the curvature is resolved, the urethroplasty can be completed. If there is persistent penile curvature, we use a measurement of 30 degrees as the defining measurement for performing a dorsal plication or a corporal lengthening procedure. If the surgeon is concerned about the quality of the ventral shaft skin in spite of curvature of less than 30 degrees, a corporal lengthening procedure can be considered.
166
What are specific procedures used for repairing hypospadias?
Urethromeatoplasty, Meatal advancement glanuloplasty (MAGPI), and M inverted V glansplasty (MIV) are specific procedures used for repairing hypospadias.
167
What is urethromeatoplasty?
Urethromeatoplasty is a surgical procedure that involves making a single vertical incision between the ectopic meatus (abnormal opening) and the distal pit (normal urethral opening) and closing it horizontally.
168
What is Meatal advancement glanuloplasty (MAGPI) and M inverted V glansplasty (MIV)?
MAGPI and MIV are surgical procedures used to repair proximal glanular meatus (abnormal urethral opening on the glans). The procedures involve advancing the meatus distally by closing the glans underneath, without tubularizing the urethra.
169
What are advancement procedures used for in the repair of hypospadias?
Advancement procedures such as MAGPI and MIV are used to advance the location of the meatus (urethral opening) in hypospadias repair. These procedures help to achieve a functional and cosmetically acceptable outcome.
170
What are specific procedures for repairing hypospadias?
There are several procedures for repairing hypospadias, including Thiersch-Duplay (TD), Tubularized incised plate (TIP), Onlay island flap (OIF), and Transverse preputial island flap (TPIF).
171
What is the Thiersch-Duplay (TD) procedure for repairing hypospadias?
The TD procedure involves using local tissue distal to the meatus to create a neourethra over a catheter. The neourethra is then covered with a Dartos flap.
172
What is the Tubularized incised plate (TIP) procedure for repairing hypospadias?
The TIP procedure is a modification of the TD repair. It involves making a deep incision into the urethral plate to ease the tubularization of the neourethra. However, there is a risk of contraction and delayed stricture, so a dorsal inlay graft (DIG) of inner preputial skin can be placed to limit scarring.
173
What is the Onlay island flap (OIF) procedure for repairing hypospadias?
The OIF procedure is used for narrow urethral plates. It involves creating an island flap of dorsal inner preputial skin and transposing it ventrally to be sutured onto the urethral plate. However, there are risks associated with this procedure, including glans dehiscence and urethral diverticulum.
174
What is the Transverse preputial island flap (TPIF) procedure for repairing hypospadias?
The TPIF procedure involves using an island flap of inner preputial skin to create the entire neourethra in proximal hypospadias if no curvature is present after degloving.
175
Table 9.1
Hypospadias Complications
176
What is a two-staged repair for hypospadias?
A two-staged repair for hypospadias involves correcting severe penile curvature (plication, division of urethral plate, corporotomy), and tissue transfer (preputial flap or free graft of prepuce or buccal mucosa) in the first stage; and tubularization of neourethra with U-shaped incision and vascular coverage in the second stage.
177
What is the purpose of correcting severe penile curvature in the first stage of a two-staged repair for hypospadias?
Correcting severe penile curvature in the first stage of a two-staged repair for hypospadias helps to prepare the penis for the tubularization procedure by creating a straighter and more functional structure.
178
What is the purpose of tubularization of neourethra in the second stage of a two-staged repair for hypospadias?
Tubularization of neourethra in the second stage of a two-staged repair for hypospadias involves creating a functional and aesthetically pleasing structure for urination and sexual function. The U-shaped incision and vascular coverage help to ensure proper blood flow and healing.
179
What is Epispadias?
Epispadias is a rare condition where the urethra opens on the top of the penis rather than at the tip.
180
What are the symptoms of Epispadias?
The symptoms of Epispadias include a shortened and widened phallus, dorsal chordee, and abnormal bladder neck which can lead to incontinence.
181
What is the epidemiology of Epispadias?
Epispadias affects 1 in 117,000 males, and the majority of cases are complete epispadias.
182
What diagnostic tests are used to evaluate Epispadias?
RBUS (Renal and Bladder Ultrasound), pelvic x-ray, and voiding cystourethrogram (VCUG) are used to evaluate the bladder neck and the extent of the defect.
183
At what age is surgical repair of Epispadias usually done?
Surgical repair of Epispadias is usually done between 6-12 months of age.
184
What is the surgical repair for Epispadias?
The surgical repair for Epispadias involves an isolated repair to separate the urethra off the corpora and transfer it ventrally or combined with bladder neck reconstruction for a proximal defect.
185
What is diphallia?
Answer: Diphallia is a rare condition where there is a duplication of the penis, usually adjacent.
186
What is the epidemiology of diphallia?
Answer: Diphallia occurs in approximately 1 in 5 million people.
187
Are there any associated anomalies commonly seen with diphallia?
Answer: Yes, anal and cardiac anomalies are common in people with diphallia.
188
What is aphallia?
Answer: Aphallia is a medical condition characterized by the absence of a phallus.
189
What are the typical physical characteristics of someone with aphallia?
Answer: A person with aphallia will have a normally developed scrotum and descended testes, but their anus will be anteriorly displaced, and their urethra will open at the anal verge or in the rectum.
190
What is the typical karyotype of someone with aphallia, and is fertility possible?
Answer: The typical karyotype of someone with aphallia is 46 XY, and fertility is possible.
191
How common is aphallia?
Answer: Aphallia is a rare condition, with an incidence of 1 in 10-30 million.
192
What diagnostic tests may be used to identify aphallia?
Answer: Karyotyping, renal and bladder ultrasound (RBUS), and magnetic resonance imaging (MRI) may be used to diagnose aphallia.
193
What is the typical treatment for aphallia?
Answer: The surgical treatment for aphallia is neophallus reconstruction using complex local or distant flaps.
194
Image
FIG. 9.8 Effman classification.
195
What is urethral duplication?
Urethral duplication is a condition where there are two urethras instead of one.
196
How is urethral duplication usually seen in the body?
Urethral duplication is usually seen in the sagittal plane, with the ventral urethra being more normal and the dorsal urethra considered accessory.
197
How many types of urethral duplication are there?
There are three types of urethral duplication.
198
What are the common presentations of urethral duplication?
The common presentations of urethral duplication are a double meatus and a double urinary stream, incontinence, and recurrent UTIs.
199
What are the diagnostic tests used for urethral duplication?
The diagnostic tests used for urethral duplication are VCUG and direct vision with cystoscopy.
200
When is surgical treatment required for urethral duplication?
Surgical treatment for urethral duplication is only required if the condition is symptomatic.
201
What is the recommended surgical treatment for urethral duplication?
The recommended surgical treatment for urethral duplication depends on the anatomy. The accessory urethra should not be used as primary, but it can be joined to the primary urethra, excised, or fulgurated using cautery or sclerosing agents.
202
What is a micropenis?
A micropenis is a penis that is 2.5 standard deviations below the mean stretched penile length for age (1.9 cm for newborns).
203
What causes micropenis?
Micropenis can be caused by hypogonadotropic hypogonadism, hypergonadotropic hypogonadism (primary testicular failure), or idiopathic (unknown cause).
204
What is hypogonadotropic hypogonadism?
Hypogonadotropic hypogonadism is a condition in which the pituitary gland or hypothalamus doesn't produce enough hormones to stimulate the testes to produce enough testosterone. This can result in micropenis.
205
What is hypergonadotropic hypogonadism?
Hypergonadotropic hypogonadism is a condition in which the testes fail to produce enough testosterone due to a problem with the testes themselves. This can also result in micropenis.
206
Why are overweight preteen boys sometimes referred for micropenis evaluation?
Overweight preteen boys may have a normal-sized penis, but it may appear small due to concealment by the prepubic fat pad. Therefore, they are sometimes referred for micropenis evaluation.
207
What is priapism?
Priapism is a medical condition where the penis remains erect for more than four hours without physical or psychological stimulation.
208
What are the four types of priapism?
The four types of priapism are ischemic, nonischemic, stuttering, and priapism of neonates.
209
What is ischemic priapism?
Ischemic priapism is also called venoocclusive or low-flow priapism. It occurs when there is no cavernous blood flow, and the cavernous blood gas is hypoxic, hypercapnic, and acidotic.
210
What are the symptoms of ischemic priapism?
The corpora, or the spongy tissue of the penis, are rigid and painful during ischemic priapism.
211
What is the usual cause of ischemic priapism?
Ischemic priapism is usually caused by homozygous sickle cell disease, where there is a predominance of hemoglobin S. Other unusual causes include leukemia, other hemoglobinopathies, and local malignancy.
212
What is the pathophysiology of ischemic priapism in sickle cell disease?
Sickling of red blood cells within sinusoids of corpora during erection leads to venous stasis, decreased pH, and local oxygen tension. This, in turn, causes more sickling and stasis.
213
What is nonischemic priapism?
Nonischemic priapism is also called arterial or high-flow priapism. It occurs when there is unregulated cavernous arterial inflow, usually from a vascular fistula. The corpora are neither fully rigid nor painful during nonischemic priapism.
214
What are the usual causes of nonischemic priapism?
Nonischemic priapism is usually caused by a history of perineal trauma, such as a straddle injury. Other causes include Fabry disease and sickle cell anemia.
215
What is stuttering priapism?
Stuttering priapism is also called intermittent priapism. It is a recurrent ischemic priapism with short duration and intervening detumescence.
216
What is priapism of neonates?
Priapism of neonates spontaneously resolves. It is caused by idiopathic reasons, birth trauma, polycythemia, or nitric oxide use.
217
How is ischemic priapism treated?
The treatment for ischemic priapism includes hydration, oxygenation, alkalinization, and analgesia. Aspiration/irrigation of corpora with α-adrenergic sympathomimetic agent (phenylephrine typically diluted in normal saline to a concentration of 100–200 ug/mL or epinephrine 1:100,000 solution) can follow. Exchange transfusion is recommended to reduce HbS concentration. To prevent recurrent priapism, pseudoephedrine (α-adrenergic) at bedtime promotes muscle contraction within erectile tissue. A phosphodiesterase type 5 (PDE5) inhibitor can also be prescribed since PDE5 dysregulation may be involved in priapism.
218
What is the description of penoscrotal transposition and bifid scrotum?
Penoscrotal transposition and bifid scrotum are conditions in which the scrotum extends superiorly to the penis or labioscrotal folds are completely separated, usually with proximal hypospadias.
219
What is the etiology of penoscrotal transposition and bifid scrotum?
The etiology of penoscrotal transposition and bifid scrotum is incomplete or failed inferomedial migration of the labioscrotal swellings.
220
What is the workup for penoscrotal transposition and bifid scrotum?
If complete transposition, RBUS and consider voiding cystourethrography are the workups for penoscrotal transposition and bifid scrotum.
221
What is the surgical correction for penoscrotal transposition and bifid scrotum?
The surgical correction for penoscrotal transposition and bifid scrotum is usually done during hypospadias repair. Superior aspects of each half circumscribed and closed ventral to the penis.
222
What are the risks associated with the surgical correction of penoscrotal transposition and bifid scrotum?
The risks associated with the surgical correction of penoscrotal transposition and bifid scrotum are injury to tunica vaginalis and spermatic cord during deeper dissection.
223
What is an inguinal hernia?
An inguinal hernia is when the abdominal contents, such as peritoneal fluid, bowel, omentum, and gonads, protrude through a patent processus vaginalis that extends beyond the internal inguinal ring, resulting in an inguinal bulge.
224
What is the epidemiology of inguinal hernia?
Inguinal hernia affects 1%-5% of children, with boys being 5-10 times more likely to develop it. It is more common on the right side, especially in premature and low birthweight infants.
225
Is imaging required for inguinal hernia diagnosis?
Imaging is not required for the diagnosis of inguinal hernia.
226
How is inguinal hernia treated?
The recommended treatment for inguinal hernia is surgical repair with high ligation of the hernia sac at the internal ring. Early repair is crucial to prevent incarceration. If incarceration occurs, urgent exploration is necessary. Laparoscopic repair is also possible.
227
What are the complications associated with inguinal hernia repair?
Recurrence of inguinal hernia occurs in 0.5%-1% of cases, with a higher rate of up to 2% in premature infants. Secondary cryptorchidism, testicular atrophy, and vasal injury are rare complications.
228
Image
FIG. 9.9 Anatomy of the processus vaginalis in hydrocele. (A) Normal closure of the processus vaginalis; straight arrows indicate the funicular process; curved arrow is the tunica vaginalis. (B) Communicating hydrocele with complete patency of the processus vaginalis. (C) Funicular hydrocele with distal closure of the processus vaginalis; communication with the peritoneal cavity may also result in hernia. (D) Encysted hydrocele of the spermatic cord. Source: (From Martin LC, Share JC, Peters C, et al. Hydrocele of the spermatic cord: embryology and ultrasonographic appearance. Pediatr Radiol 1996;26:528-530.)
229
What is a communicating hydrocele?
A communicating hydrocele is a medical condition where the peritoneal fluid is contained in the patent processus vaginalis and may extend to the tunica vaginalis around the testis. It is a condition where fluid flows freely between the scrotum and abdomen.
230
How does a communicating hydrocele resolve?
Answer: A communicating hydrocele may resolve by the age of one year.
231
What is the etiology of a communicating hydrocele?
Answer: The increased incidence of abdominal ascites, peritoneal dialysis, or ventriculoperitoneal shunt are some of the etiologies of a communicating hydrocele.
232
How is a communicating hydrocele treated?
Answer: The treatment of a communicating hydrocele is similar to inguinal hernia.
233
What is a hydrocele of the spermatic cord?
A hydrocele of the spermatic cord is the presence of fluid within a segment of the patent processus vaginalis with obliterated processus distally and proximally.
234
What is a scrotal hydrocele?
A scrotal hydrocele is the presence of fluid within the tunica vaginalis surrounding the testis without communication proximally.
235
What is the etiology of scrotal hydrocele?
Approximately 5% of male infants have scrotal hydrocele, and most resolve spontaneously.
236
Is imaging necessary for the diagnosis of hydrocele?
Imaging is not required for the diagnosis of hydrocele. However, it may be helpful to evaluate the testis if it is not palpable through a tense hydrocele.
237
What is an abdominoscrotal hydrocele?
An abdominoscrotal hydrocele is a type of hydrocele that extends from the scrotum into the abdomen, without communication with the peritoneum.
238
What is the epidemiology of abdominoscrotal hydrocele?
Abdominoscrotal hydrocele is present in approximately 1.25% of all hydroceles, and about 30% of cases are bilateral. It is typically present in infancy and tends to enlarge over time.
239
How is abdominoscrotal hydrocele diagnosed?
Ultrasound imaging may be used to visualize the extent of the hydrocele, particularly its proximal extent.
240
Can abdominoscrotal hydrocele improve or resolve spontaneously?
Yes, it is possible for an abdominoscrotal hydrocele to improve or resolve on its own without intervention.
241
What is the recommended treatment for abdominoscrotal hydrocele?
If treatment is needed, a primary scrotal approach may be used to drain and excise the enlarged tunica vaginalis with limited dissection to decrease inflammation and morbidity from an inguinal dissection. Additionally, if the testes are undescended, subsequent orchiopexy may be necessary.
242
What is cryptorchidism?
Cryptorchidism is a condition in which one or both testes are not located in the scrotum, and they can be palpable or nonpalpable.
243
What are the causes of absent testes in cryptorchidism?
Absent testes in cryptorchidism can be lost due to vascular accident or torsion (vanishing), or they might have never formed (agenesis).
244
What is the association between Müllerian duct persistence and cryptorchidism?
When testes are never formed in cryptorchidism, they are associated with ipsilateral Müllerian duct persistence.
245
What is the risk of testicular cancer in cryptorchidism?
Cryptorchidism is associated with a 2–5x increased risk of testicular cancer.
246
What are retractile testes?
Retractile testes are scrotal testes that retract out of the scrotum, but they can be manually pulled to the scrotum where they remain temporarily after fatiguing the cremasteric reflex.
247
What is the workup for bilateral nonpalpable testes associated with abnormal penile development?
In case of bilateral nonpalpable testes associated with abnormal penile development, karyotype and hormonal analysis are required to rule out congenital adrenal hyperplasia.
248
What is the recommended treatment for cryptorchidism?
Hormonal therapy is not recommended for cryptorchidism. Surgery is the preferred treatment based on testis location preoperatively. If the testis is palpable, an inguinal approach is used. If nonpalpable, a diagnostic laparoscopy is done to define the presence and location of the testis in the abdomen, or to confirm if it is absent.
249
What are the potential complications of cryptorchidism?
Testicular retraction and atrophy are potential complications of cryptorchidism.
250
What is the recommended follow-up period for cryptorchidism after treatment?
A follow-up of at least 6 months is required to confirm the position of the testis. Longer follow-up is optional.
251
Image
FIG. 9.10 Algorithm for management of the undescended testis. The American Urological Association guideline algorithm for diagnosis and treatment of palpable and nonpalpable testes in patients confirmed to have undescended testis by an experienced examiner. DSD, Disorder of sexual differentiation. Source: (From American Urological Association. Evaluation and treatment of cryptorchidism. 2014. http://www.auanet.org/education/guidelines/cryptorchidism.cfm.)
252
What is the most common cause of scrotal pain in prepubertal boys?
Answer: The most common cause of scrotal pain in prepubertal boys is torsion of appendix testis.
253
What is torsion of appendix testis?
Torsion of appendix testis occurs when the appendix testis twists, causing pain and swelling.
254
What are the symptoms of torsion of appendix testis?
The symptoms of torsion of appendix testis include blue dot sign, focal tenderness at superior pole, preserved symmetric cremasteric reflux, swelling, tenderness, and edema.
255
How is torsion of appendix testis diagnosed?
Torsion of appendix testis is diagnosed through physical examination and imaging. The blue dot sign, focal tenderness at superior pole, preserved symmetric cremasteric reflux, swelling, tenderness, and edema are the physical examination findings. Color Doppler ultrasonography (CDUS) may show abnormal appendage, hyperperfusion of epididymis.
256
What is the management for torsion of appendix testis?
: The management for torsion of appendix testis is conservative, which includes using ice, oral anti-inflammatory agents, and limiting physical activity. Surgery may be required in rare cases.
257
What is the blue dot sign?
The blue dot sign is a physical examination finding that can indicate torsion of the appendix testis. It refers to a small blue or black dot that appears on the scrotum, near the head of the epididymis, due to the engorgement of the affected appendix. The blue dot sign is a useful diagnostic feature to differentiate torsion of the appendix testis from other causes of scrotal pain, such as testicular torsion or epididymitis.
258
What is the appendix testis?
The appendix testis is a small, pedunculated structure that is located on the upper pole of the testis in males. It is a vestigial remnant of the Müllerian duct, which is a structure that develops in the female fetus and contributes to the formation of the female reproductive system. In males, the appendix testis has no known function and is considered to be a benign anatomic variant. However, in some cases, the appendix testis can become twisted, leading to acute scrotal pain and swelling. This condition is known as torsion of the appendix testis.
259
What is the appendix epididymis?
The appendix epididymis is a small, tubular structure that is located on the surface of the epididymis, which is a coiled tube located on the upper and back part of the testis in males. The appendix epididymis is a vestigial remnant of the Wolffian duct, which is a structure that develops in the male fetus and contributes to the formation of the male reproductive system. The appendix epididymis has no known function and is considered to be a benign anatomic variant. However, in some cases, the appendix epididymis can become twisted, leading to acute scrotal pain and swelling. This condition is known as torsion of the appendix epididymis.
260
Box 9.1
Differential Diagnosis of Pediatric Adolescent Acute Scrotal Pain
261
What is a paradidymis?
The paradidymis is a small, vestigial structure that is sometimes present in the male reproductive tract. It is located near the head of the epididymis and is considered to be a normal anatomical variant. The function of the paradidymis is not well understood, but it is thought to be a remnant of the mesonephric duct, which is a structure that is involved in the development of the male urinary and reproductive systems. The presence of the paradidymis is generally considered to be of no clinical significance, and it does not typically cause any symptoms or require any specific treatment.
262
What is a vas aberrans?
A vas aberrans is an anatomical variant of the male reproductive tract in which an extra vas deferens (the tube that carries sperm from the testes to the urethra) is present. This extra tube is usually small and has no known function. Vas aberrans can be unilateral (affecting one side) or bilateral (affecting both sides), and they are often discovered incidentally during a physical examination or imaging studies of the reproductive tract. Vas aberrans is generally considered to be of no clinical significance, and it does not typically cause any symptoms or require any specific treatment. However, in some cases, it may be associated with other reproductive tract abnormalities.
263
What is testicular torsion?
Testicular torsion is a medical condition in which the spermatic cord that supplies blood to the testicles twists, cutting off blood flow to the affected testicle.
264
What is the difference between extravaginal and intravaginal testicular torsion?
Extravaginal torsion occurs before the tunica vaginalis is firmly attached to the dartos muscle and can happen prenatally, during delivery, or postpartum. Intravaginal torsion, on the other hand, happens within the tunica vaginalis, and it's often related to anatomical deformities that predispose to torsion.
265
What is the epidemiology of testicular torsion?
Testicular torsion occurs in 6.1 out of 100,000 births and is more common in infants with high birth weight or difficult delivery. Intravaginal torsion affects about 1 in 4000 males, with a left-side prevalence and peak incidence between 12-16 years of age.
266
What are the typical clinical findings in a patient with intravaginal testicular torsion?
Patients with intravaginal torsion typically experience sudden, severe scrotal pain that starts at rest or wakes them up from sleep. They may also have nausea and vomiting. On exam, the affected testicle is usually tender, firm, and high riding, with absent cremasteric reflex, edema, and erythema.
267
How is testicular torsion diagnosed?
Clinical suspicion is the key to the diagnosis of testicular torsion. Imaging with color Doppler ultrasound (CDUS) can confirm reduced or absent blood flow to the affected testicle and may show a twist in the spermatic cord or heterogeneity of the testicular parenchyma. In intermittent torsion, imaging may show residual edema and hyperemia.
268
What is the treatment for testicular torsion?
Testicular torsion is a surgical emergency that requires immediate exploration to salvage the affected testis and prevent contralateral torsion. The optimal time window for successful detorsion is within 6 hours of onset of symptoms. Manual detorsion may be attempted before surgery, but exploration is still required. The surgical approach may involve a hemiscrotal or midline incision, untwisting the testis, performing a fasciotomy in the tunica albuginea to enhance perfusion, and fixing the contralateral testis with non-absorbable sutures. If the affected testis is viable, it may be fixed to the scrotum (pexed), but if not, orchiectomy may be necessary. In intermittent torsion, elective bilateral septopexy may be considered.
269
Why is the treatment of extravaginal torsion controversial?
The treatment of extravaginal torsion is controversial because it is often diagnosed in newborns with non-specific symptoms, and the salvageability of the affected testis is uncertain. Some experts recommend immediate exploration to prevent contralateral torsion and future fertility problems, while others advocate for elective exploration due to the low incidence of metachronous torsion and concerns about anesthetic risk in newborns.
270
What is epididymitis?
Answer: Epididymitis is a medical condition that is characterized by inflammation or infection of the epididymis, a coiled tube located at the back of the testicles that stores and transports sperm.
271
What are the symptoms of epididymitis?
Answer: The common symptoms of epididymitis include insidious onset of pain, scrotal swelling, fever, dysuria, and rarely, nausea.
272
How is epididymitis diagnosed?
Answer: Epididymitis is diagnosed based on a physical exam, which typically involves assessing diffuse tenderness and scrotal swelling, and a workup that may involve testing for pyuria and bacteriuria. The patient's history of UTIs, sexual activity, dysfunctional voiding, intermittent catheterization, or urethral anomalies is also evaluated.
273
What is the imaging modality of choice for diagnosing epididymitis?
Answer: Color Doppler ultrasound (CDUS) is the preferred imaging modality for diagnosing epididymitis. It can detect an increase in epididymal size and blood flow.
274
How is epididymitis managed?
Answer: The management of epididymitis aims to reduce inflammation and relieve symptoms. This typically involves using ice packs, NSAIDs, and scrotal elevation. If an infection is suspected, antibiotics are prescribed. Adolescents with epididymitis are also tested and treated for sexually transmitted infections (STIs).
275
What is a varicocele?
A varicocele is an abnormal dilation of the internal spermatic veins within the pampiniform plexus, often associated with subfertility.
276
Who is most likely to develop a varicocele?
Varicoceles can occur in 8% to 16% of adolescents and often present after age 10.
277
What causes a varicocele?
A varicocele is caused by increased venous pressure in the left renal vein, collateral venous anastomoses, or valvular incompetence of the left internal spermatic vein at the junction with the left renal vein.
278
How does a varicocele present?
A varicocele usually presents as a left-sided swelling above the testis, which can be found by the patient or clinician. It can sometimes be bilateral, and rarely, it may cause mild pain.
279
How is a varicocele diagnosed?
A varicocele can be diagnosed through physical examination, which grades it based on its visibility and palpability. Testicular size and consistency should also be assessed, and abdominal imaging may be done to rule out an abdominal or pelvic mass.
280
What is the controversy surrounding semen analysis in relation to varicoceles?
There is no reliable standard for adolescents based on Tanner stage, and there is some controversy regarding the correlation between unilateral or total testicular volume and semen parameters. Additionally, varicocele grade and postoperative catch-up growth do not predict ultimate semen quality.
281
What is the standard treatment for a varicocele?
Observation is the standard treatment for most cases. However, surgery may be indicated if there is size discrepancy (differential or total testicular volume hypotrophy >20%), abnormal semen analysis, pain (rare), or an intratesticular component of the varicocele.
282
What surgical approaches can be used to treat a varicocele?
Surgical approaches for treating a varicocele include inguinal, subinguinal, laparoscopic, or venographic methods. The choice of approach will depend on the specific case.
283
What is an epididymal cyst?
An epididymal cyst is a cystic structure that is found on the epididymis. It appears similar to a spermatocele and may contain fluid, but not sperm.
284
What is a spermatocele?
A spermatocele is a cyst that develops on the epididymis and contains sperm. They typically occur post-puberty.
285
What is the epidemiology of epididymal cysts?
Epididymal cysts occur in approximately 14% of men, with increasing incidence with age. They are found in 35% of boys over the age of 15.
286
How is an epididymal cyst diagnosed?
An epididymal cyst is typically palpable by either the patient or physician. It may cause transient pain and can also be an incidental finding on an ultrasound.
287
What is the management of epididymal cysts?
Epididymal cysts may spontaneously resolve, and surgical intervention is rarely indicated.
288
What are the symptoms of an epididymal cyst?
Epididymal cysts usually do not cause any symptoms. However, they may cause pain, swelling, or discomfort if they grow in size.
289
Can epididymal cysts cause infertility?
Epididymal cysts do not usually cause infertility. However, if they are large enough to obstruct the flow of sperm, they may interfere with fertility.
290
Are epididymal cysts cancerous?
Epididymal cysts are generally benign and do not have the potential to become cancerous. However, in rare cases, a tumor may develop in the epididymis, and it may be mistaken for an epididymal cyst.
291
Can epididymal cysts recur after treatment?
Epididymal cysts may recur after treatment. Surgical removal of the cyst is the most effective way to prevent recurrence. However, the recurrence rate is low, and the cysts usually do not require further intervention.
292
How can epididymal cysts be prevented?
There is no specific way to prevent epididymal cysts from developing. However, maintaining good genital hygiene and avoiding injuries to the scrotum may help reduce the risk of developing an epididymal cyst.
293
What is Congenital absence of the vas deferens?
Congenital absence of the vas deferens is a condition that results in the absence or complete blockage of the vas deferens, which is a tube that carries sperm from the testes to the urethra for ejaculation.
294
Is this condition unilateral or bilateral?
Answer: Congenital absence of the vas deferens can be unilateral or bilateral, meaning it can affect one or both sides of the body.
295
What is the typical fertility outcome for someone with this condition?
Answer: Individuals with Congenital absence of the vas deferens are typically infertile due to the lack of sperm transport.
296
What is the association between Congenital absence of the vas deferens and cystic fibrosis gene CFTR?
Answer: Congenital absence of the vas deferens is associated with less severe mutations of the cystic fibrosis gene CFTR. Mutations in this gene can affect the function of sweat glands, lungs, pancreas, and reproductive organs.
297
What other anomalies may be associated with Congenital absence of the vas deferens?
Answer: Congenital absence of the vas deferens is associated with persistent mesonephric duct anomaly with renal agenesis or ectopia and partial or complete agenesis of the epididymis and seminal vesicles. These anomalies affect the development of the urinary and reproductive systems.
298
Table 9.2
Treatment Options for Varicocele
