By Bryan Wilson, MS3; Larry B. Mellick, MS, MD, FACEP
This 5-year-old African American female presented for evaluation of vaginal bleeding which was first noticed by her mother on the day of arrival. On physical exam there was significant bleeding with clotted blood sitting in the introitus as seen in the picture above. A soft, red mass surrounding the urethral meatus is noted. Urine is found to be normal with no hematuria.
The patient was diagnosed with urethral prolapse, started on Premarin cream twice a day and discharged home with instructions to do sitz baths.
The relative rarity and wide differential diagnosis of urethral prolapse lead to frequent misdiagnosis. In fact, as few as 1 in 5 incidences may be correctly diagnosed at first presentation.1,2 The impact of this delayed diagnosis can range from delayed treatment to mistaken accusations of child abuse.3 This case provides an excellent opportunity to review the following discussion questions:
A urethral prolapse is protrusion of the distal urethra through the external meatus. Prolapse occurs as a result of congenital or acquired weakening of the anatomic support of the urethra. The hallmark defect is the separation of the urethra’s longitudinal and circular-oblique smooth muscle layers.4 The prolapsed mucosa impinges on venous outflow resulting in vascular congestion, strangulation, and eventually necrosis. While a complete prolapse forming a full mucosal ring around the meatus is most common, partial prolapse is also possible.5
Urethral prolapse is most common in pre-pubescent black females and post-menopausal white females. For children, the age of diagnosis has been found to range from 5 days to 11 years with a mean of about 5 years.1,6-11 Predisposing factors include increased weight, chronic cough, asthma, UTI, constipation, and trauma.1,10,11 Documented presentation of urethral prolapse in twins supports the involvement of genetic factors in the pathophysiology of urethral prolapse.12
While nearly 10% of patients can be asymptomatic, the majority will present with urogenital bleeding.1,7,8, 10, 11, 13 This can be accompanied by voiding complaints in up to 25% of cases including the rare but documented incidence of urinary retention syndrome.1,4,7,8,10,11,14,15 Hematuria is common in post-menopausal patients, but it is an uncommon presentation in pre-pubescent girls.
On physical exam, a soft mucosal mass in the form of a edematous, friable rosette of bright red or cyanotic tissue is present around the external meatus.5 The presence of an opening in the mass should be confirmed and its identity as the external meatus confirmed. This can be done by visualization during catheterization or voiding. A physical exam is generally sufficient to diagnose a prolapsed urethra; however voiding cystourethrogram would demonstrate a non-obstructive mass encircling the external meatus at the narrowed distal urethra.16 Histological examination shows ulcerated polypoid tissue composed of fibrovascular stroma with signs of vascular congestion and inflammation.
The differential diagnosis for a vulval vestibular mass is varied and ranges from congenital anomalies to neoplasms. It includes bladder prolapse, uteroceleprolapse, periurethral cyst, ectopic ureter, polyps, papillomas, rhadbomyosarcoma, hydrometrocolpos, condyloma acuminata, periurethral abscess, and sarcoma botryoides.11,17
First line therapy for non-complicated ureteral prolapsed in pre-pubertal patients is 2 weeks of medical treatment with topical estrogen cream in combination with sitz baths.1,7,8,18,19 Cases refractory to medical treatment are an indication for surgical intervention.8,13,18,20 If the genitourinary mass is exquisitely tender, cyanotic strangulation should be considered and emergent surgical excision may be indicated.
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