Chapter 10
Disorders of the Female
External Genitalia
Richard S. Hurwitz
Key Points
››Labial adhesions usually resolve spontaneously. Extensive adhesions may be misdiagnosed as vaginal agenesis or as a disorder of sex development. When treatment is required, betamethasone cream 0.05% works faster and with fewer recurrences and side effects than estrogen creams.
››Interlabial masses have a characteristic appearance.
–– Paraurethral cyst – whitish or yellowish mass that displaces the urethral meatus
–– Imperforate hymen/hydrocolpos – bulging vaginal mass covered by thin grayish membrane.Abdominal mass may be present
–– Prolapsed ectopic ureterocele – smooth round mass bulging through the urethral meatus, usually with congested purplish mucosa
–– Urethral prolapse – swollen donut-shaped lesion with congested red to purple mucosa
–– Urethral polyp – single pedunculated structure from urethral meatus
–– Vaginal rhabdomyosarcoma – fleshy polypoid mass with grape-like clusters
P.P. Godbole et al. (eds.), Guide to Pediatric Urology and |
95 |
Surgery in Clinical Practice, DOI: 10.1007/978-1-84996-366-4_10,
© Springer-Verlag London Limited 2011
96R.S. Hurwitz
››Pre-pubertal girls with persistent vaginal discharge and all young girls with vaginal bleeding should be referred immediately for evaluation under anesthesia with cystoscopy and vaginoscopy.
–– Persistent vaginal discharge: Rule out vaginal foreign bodies and sexual abuse
–– Vaginal bleeding: Rule out vaginal malignancy, foreign bodies, benign papillomas, and sexual abuse
10.1 Introduction
In this chapter on the disorders of the female external genitalia, identification and treatment of labial adhesions and recognition of the classic interlabial masses is emphasized. The significance of vaginal discharge and vaginal bleeding in the prepubertal female is discussed.
10.2 Labial Adhesions
Labial adhesions occur when the inner edges of the labia minora, labia majora, or both in continuity somehow become excoriated causing opposing raw surfaces to adhere in the midline. The epithelial breakdown may be due to irritated inner labial skin from chronic wetness, ammoniacal inflammation, or recurrent vaginitis.
Labial adhesions may be asymptomatic and discovered during a diaper change or a routine physical examination
(Figs. 10.1a and b). Patients may also present with symptoms of frequency, UTI, vaginitis, or post void dribbling due to trapping of urine.
Closure of the introitus by labial adhesions may be alarming when first recognized. The degree of introital closure is variable, but in some cases can be nearly complete with only a tiny subclitoral patency remaining. This appearance can
Chapter 10. |
Disorders of the Female External Genitalia |
97 |
a |
b |
|
FIGURE 10.1. (a, b) Labial adhesions: Presumed urogenital sinus with posterior labial fusion in a patient with non-classical CAH.This unsuspected extensive adhesion was opened by the pressure of the cystoscope during endoscopic evaluation.
lead to misdiagnoses such as vagina atresia, vaginal agenesis or urogenital sinus anomaly raising the possibility of CAH or other forms of DSD. There is no associated clitoral enlargement. Sometimes a fine, vertical grayish membrane can be seen representing the thin midline fusion of the labia.
Labial adhesions tend to resolve spontaneously over time. Treatment is indicated for symptomatic and/or more extensive adhesions. Treatment options include topical estrogen cream, betamethasone cream, manual separation, or surgery. In a recent report of 151 patients with labial adhesions, 0.05% betamethasone cream resulted in separating the adhesions quicker (average 1.3 months) than did topical estrogen (premarin) therapy (average 2.2 months). Rates of recurrence were lower for patients treated with betamethasone. Side effect from estrogen treatment included breast budding and vaginal bleeding while side effects from betamethasone were limited to local irritation.1 A success rate of 68% has been reported.2 As more experience with betamethasone has accumulated, it seems to be favored for primary therapy. Surgical
98 R.S. Hurwitz
lysis is occasionally required in cases resistant to medical therapy.
10.3 Interlabial Masses
An interlabial mass is occasionally encountered when inspecting the female genitalia. Some of these may cause discharge and/or bleeding. Differential diagnosis includes paraurethral cysts, imperforate hymen with hydrocolpos, prolapsed ectopic ureterocele, urethral prolapse, urethral polyp, and vaginal malignancy. Understanding the characteristic appearance and typical anatomic relationship to the urethral meatus and vaginal opening of each of these interlabial masses will greatly help in making the correct diagnosis.
10.4 Paraurethral (Skene’s Duct) Cyst
Paraurethral cysts or Skene’s duct cysts typically present as an asymptomatic interlabial mass in newborns. The typical appearance is that of a whitish or yellowish mass that displaces the urethral meatus to an eccentric position. The normal vaginal opening should still be visible.The cyst may cause deviation of the urinary stream or deform the anterior vaginal wall. It is thought that these cysts form because of an obstruction of the paraurethral gland ducts (Fig. 10.2).
Paraurethral cysts in newborns usually resolve by spontaneously rupturing during the first few weeks of life. Incision or needle aspiration may be needed to resolve persistent cysts.
10.5 Imperforate Hymen with Hydrocolpos
Hydrocolpos due to an imperforate hymen presents as a bulging vaginal mass covered by a thin pearly grey (hymeneal) membrane. The urethral meatus should be seen in its normal position just above the mass (Fig. 10.3).
Chapter 10. Disorders of the Female External Genitalia |
99 |
FIGURE 10.2. Paraurethral (Skene’s duct) cyst: Right sided cyst is pushing and flattening urethral meatus to left (red arrow). Vaginal opening visible (blue arrow). (courtesy of Stephens 1983).
FIGURE 10.3. Imperforate hymen with hydrocolpos in double vagina: Urethral meatus splayed above bulging mass (red arrow). Hemostat in second patent hemi-vagina. (courtesy of Cartwright et al. 2002).
The imperforate hymen traps the naturally occurring mucous secretions created by maternal estrogen stimulation during in utero development. In some cases, the build-up of mucous secretions can result in massive vaginal dilation creating a palpable abdominal mass. Compression of the bladder and ureters may lead to difficulty voiding and hydronephrosis. The uterus