9: Conditions of the external genitalia Flashcards
What are labial adhesions in females?
Labial adhesions are a condition in which the labia minora, the inner folds of skin around the vaginal opening, are fused together.
What is the epidemiology of labial adhesions in females?
Labial adhesions occur in 0.6% to 1.8% of females, usually in those under the age of 2 years.
How do labial adhesions typically present?
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.
What is the treatment for labial adhesions in females?
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.
What is the success rate of topical conjugated estrogen for treating labial adhesions in females?
Topical conjugated estrogen (0.625 mg/g) is successful in 90% of cases of labial adhesions in females.
What are the potential side effects of using topical conjugated estrogen for treating labial adhesions in females?
Potential side effects of using topical conjugated estrogen for treating labial adhesions in females include breast budding and skin hyperpigmentation.
What is manual separation with a lubricated probe after lidocaine cream?
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.
Is recurrence common after treating labial adhesions in females?
Yes, recurrence is common after treating labial adhesions in females, and continued application of moisturizing ointment may be necessary to prevent recurrence.
What is urethral prolapse in females?
Answer: Urethral prolapse in females is the circumferential eversion of the urethral mucosa.
Who is more prone to urethral prolapse in females?
Answer: Prepubertal black girls and postmenopausal women are more prone to urethral prolapse in females.
What are the symptoms of urethral prolapse in females?
Answer: Bleeding from the mucosa causes blood spotting in females with urethral prolapse.
What are the treatment options for urethral prolapse in females?
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.
Image
FIG. 9.1 (A and B) Urethral prolapse.
What is a paraurethral cyst in females?
A paraurethral cyst in females is a dilation of periurethral glands (Skene’s glands) just inside the urethral meatus.
How does a paraurethral cyst form in neonates?
In neonates, the glands respond to maternal estrogen and secrete mucoid material resulting in cyst formation.
What are the symptoms of a paraurethral cyst in females?
A paraurethral cyst displaces the urethral meatus and produces a deviated urinary stream.
How is a paraurethral cyst treated?
A paraurethral cyst frequently ruptures spontaneously, but if it persists, it can be drained by needle puncture.
What is Gartner’s duct cyst?
Gartner’s duct cyst is a cystic structure that represents incomplete regression of the wolffian duct along the anteromedial wall of the vagina.
What causes Gartner’s duct cyst?
Gartner’s duct cyst occurs when the wolffian duct fails to regress completely during embryonic development.
What are the symptoms of Gartner’s duct cyst?
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.
How is Gartner’s duct cyst treated?
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.
What are the potential complications of Gartner’s duct cyst?
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.
What is imperforate hymen?
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.
What is the epidemiology of imperforate hymen?
It is the most common congenital obstructive anomaly of the female reproductive tract.
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.
What is the treatment for imperforate hymen in newborns?
In newborns, it may be incised transversely at the bedside.
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.
How is imperforate hymen treated in older children?
In older children, incision under anesthesia is performed.
Image
FIG. 9.2 Imperforate hymen. Note the distention from vaginal secretions.
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.
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.
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.
What is the epidemiology of vaginal septum?
Answer: Vaginal septum is a rare condition, affecting about 1 in 70,000 females.
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.
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).
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.
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.
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.
How is vaginal atresia diagnosed?
Answer: Vaginal atresia can be diagnosed by a distended vagina that can be palpable on rectal exam.
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.
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.
Are Müllerian structures affected in vaginal atresia?
Answer: No, Müllerian structures are not affected in vaginal atresia.
What is vaginal agenesis?
Vaginal agenesis is a congenital absence of the proximal vagina in an otherwise intact female.
What is the epidemiology of vaginal agenesis?
It affects 1 in 5000 live female births.
What are the two forms of vaginal agenesis?
The two forms of vaginal agenesis are type A (typical) and type B (atypical).
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.
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.
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.
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.
What are the complications of vaginal agenesis treatment?
Vaginal stenosis is a common complication that occurs in skin > ileum neovagina > sigmoid neovagina.
Image
FIG. 9.3 Vaginal rhabdomyosarcoma.
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.
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.
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.
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.
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.
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.
What is clitoral hypertrophy?
Clitoral hypertrophy is a condition where the clitoral tissue is enlarged, usually associated with common urogenital sinus.
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.
How is clitoral hypertrophy evaluated?
Clitoral hypertrophy is evaluated through serum electrolytes, 17-hydroxyprogesterone level, and karyotype.
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.
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.
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.
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.
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.
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.
Image
FIG. 9.4 (A and B) Prolapsed Ureterocele.
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.
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.
What is the most common association of urogenital sinus/cloacal anomaly?
Urogenital sinus/cloacal anomaly is usually associated with congenital adrenal hyperplasia (CAH).
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.
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.
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.
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.
Is inguinal hernia more common in boys or girls?
Answer: Inguinal hernia is rarer in girls than boys.
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).
What are the symptoms of inguinal hernia in females?
Answer: The symptoms of inguinal hernia in females include inguinal bulge and occasionally pain.
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.
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.
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.
What are the symptoms of phimosis?
Symptoms of phimosis can include difficulty urinating, pain during sexual activity, and swelling of the foreskin.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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).
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.
What is the cause of meatal stenosis?
Meatal stenosis can occur after circumcision, when the foreskin is removed and the remaining tissue heals improperly.
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.
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.
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.
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.
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.
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.
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.
What is the recommended treatment for a parameatal cyst?
Answer: The recommended treatment for a parameatal cyst is complete excision.
Is a parameatal cyst a common condition in males?
Answer: Parameatal cyst is a relatively uncommon condition in males.
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.
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.
What are the symptoms of BXO?
A: The symptoms of BXO include a tight, scarred foreskin, foreskin irritation, discomfort, bleeding, and acute urinary retention.
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.
What are the risks associated with BXO?
A: BXO recurs in 20%-40% of cases.
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.
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.
How is Lichen sclerosus et atrophicus treated?
A: The treatment of Lichen sclerosus et atrophicus includes topical corticosteroids and immunosuppressive agents.
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.
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.
What are the different types of inconspicuous penis?
There are three types of inconspicuous penis: buried/hidden penis, trapped penis, and webbed penis.
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.
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.
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.
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).
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.
Table 9.1
Hypospadias Complications
Image
FIG. 9.8 Effman classification.
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.)
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.)
Box 9.1
Differential Diagnosis of Pediatric Adolescent Acute Scrotal Pain
Table 9.2
Treatment Options for Varicocele
