100 R.S. Hurwitz
may also become distended (hydrometrocolpos), and urinary retention, constipation, and edema or cyanosis of the lower extremities have been known to occur in extreme cases.3
Simple incision of the imperforate hymen drains the distended vagina and relieves the compressive effects on adjacent organs. Renal and bladder ultrasound should be performed to evaluate the urinary tract status after vaginal drainage has been accomplished.
10.6 Prolapsed Ectopic Ureterocele
A prolapsed ureterocele presents as a smooth, round interlabial mass. If the prolapse is recent, the mucosa may still be pink. Spontaneous reduction of the prolapse may occur in the early pre-congestion stage. The mass will be large and purplish if the prolapse is seen after significant congestion and strangulation have occurred. Since the ureterocele has prolapsed through the urethra, a distinct urethral meatus may be difficult to visualize. The normal vaginal opening may be obscured, but should be detectable posteriorly (Fig. 10.4a).
a |
b |
Ureterocele
Dilated upper pole ureter
FIGURE 10.4. (a) Prolapsed strangulated ectopic ureterocele. (b) Ultrasound showing ectopic ureterocele in bladder and dilated upper pole ureter. (courtesy of Thomas et al. 2002).
Chapter 10. Disorders of the Female External Genitalia 101
Ectopic ureteroceles are associated with and cause obstruction of the upper pole system of a duplex kidney.
The diagnosis of an ectopic ureterocele can be confirmed by a renal and bladder ultrasound that will show a dilated upper pole collecting system and an intravesical cystic lesion in the area of the bladder neck (Fig. 10.4b). Urgent urological consultation is indicated when a prolapsed ureterocele is identified.
10.7 Urethral Prolapse
Urethral prolapse has a very characteristic donut-shaped appearance with a congested, red to dark purple discoloration of the mucosa. The urethral meatus is in the middle of the swollen, prolapsed mucosa. The mass created by the prolapsed tissue can be quite large and occupy most of the introitus in younger girls, obscuring the normally positioned vaginal opening (Fig. 10.5).
The typical presentation is with spotting of blood in the underwear. Sometimes this is accompanied by dysuria, perineal discomfort, and rarely urinary retention. The combination of the dark, swollen appearance and bleeding has sometimes led to the erroneous diagnosis of sexual abuse.
FIGURE 10.5. Urethral prolapse: Note the congested donut appearance around the centrally located urethral meatus.
102 R.S. Hurwitz
Urethral prolapsed is more common in black girls 4–5 years of age and is often associated with coughing or constipation.
Treatment is usually conservative with sitz baths, elimination of constipation, and a short course of topical estrogen cream. Rarely, surgical excision is required.
10.8 Urethral Polyp
A urethral polyp is a smooth, mucosa – covered mass that protrudes from the urethral meatus. They are usually single pedunculated structures, either wide based or on a thin stalk averaging 1–3 cm in length. Although rare, large fleshy urethral polyps up to 6 cm in size have been reported in newborns.4 The vaginal opening should be normal (Fig. 10.6).
Urethral polyps usually arise from the posterior wall of the urethra. They are not site-specific and can arise from the proximal, mid, or distal urethra. They commonly present with “vaginal” bleeding and some have associated symptoms of vulvitis, frequency, dysuria, or UTI.5
FIGURE 10.6. Urethral polyp. (courtesy of Stephens 1983).
Chapter 10. Disorders of the Female External Genitalia 103
Urethral polyps are benign lesions. Most are fibroepithelial polyps. Inverted papilloma and hamartomatous variations have been reported. The etiology thought to be related to prolapsing urothelium that undergoes an inflammatory response and then evolves into a polyp.5
Treatment is by simple excision and fulguration or suturing of the base.
10.9 Vaginal Rhabdomyosarcoma
or Endodermal Sinus Tumor
Rhabdomyosacroma or sarcoma botryoides is the most common primary malignant tumor of the vagina in children and usually occurs in the first 5 years with a peak incidence before age 2. It may present as vaginal bleeding, passage of tissue fragments, or as an interlabial polypoid mass protruding from the vagina. The urethral meatus is usually normal. The polypoid masses typically appear as grape-like clusters. The rare endodermal sinus tumor, which carries a worse prognosis, may have a similar presentation (Figs. 10.7a–b).
Urgent urological referral is indicated. Biopsy will confirm the diagnosis. Treatment usually involves a combination of surgical excision, chemotherapy, and radiation therapy.
10.10 Vaginal Discharge and Vaginal Bleeding
Vaginal discharge and vaginal bleeding in prepubertal girls are indicators of a potentially serious social or medical problem. Vaginal discharge is most commonly due to a benign infectious process. It is rarely, if ever associated with malignancy. Development of new vaginal discharge in a child should suggest the possibility of sexual abuse. Initial evaluation should include an external genital exam and vaginal cultures that include analysis for chlamydia and gonorrhea. Persistent discharge after antibiotic treatment requires further investigation. In a referral population of 24 girls less than
104 R.S. Hurwitz
a
b
c
FIGURE 10.7. (a) Vaginal rhabdomyosarcoma: Fleshy grape-like clusters of tissue. (b, c): US and MRI of vaginal rhabdomyosarcoma.