Sage Journals: Discover world-class research

Abstract

A 10 years old girl was referred with the complaints of “per vaginal bleeding and a mass after cycling”. During the first 2 weeks of symptoms, she had visited three different doctors with possible delayed recognition of her diagnosis. Clinical examination revealed an underweight, non-dysmorphic, prepubertal girl with vulvitis and an inflamed interlabial bleeding mass with ulceration. Imaging revealed a subcentimetre arteriovenous fistula and haematoma in the vagina. After one more week (total 3 weeks) of medical therapy, the indication for surgery was worsening of symptoms and failure of medical management. Examination under anaesthesia and cystoscopy revealed a complete urethra prolapse. Dilute adrenaline was injected into the prolapsed mucosa. The prolapse was excised in elliptical segments, and the edges closed with interrupted absorbable sutures. Sub-centimetre segments of mucosa were left between the excised specimens. Bipolar diathermy was used only for hemostasis and circumferential coagulation on the mucosa was avoided. These measures prevent an iatrogenic stenosis. The urethra prolapse was reduced, with no recurrence demonstrated on Valsalva maneuver. Post-operatively, her symptoms resolved. Oral antibiotics, sitz baths, antimicrobial cream and topical estrogen were administered. There was no recurrent urethra prolapse or stricture. The aim of this case report is to increase awareness of the urethral prolapse in the paediatric population and its management.

Keywords

Urethral prolapse vaginal bleeding interlabial bleeding paediatric interlabial mass

Introduction

The complaint of “per vaginal bleeding” is often attributed to a gynaecological origin. However, between the labia minora, the urinary tract, the gynaecological tract or the vulva vestibule can be the source of pathology. Hence, the term “interlabial bleeding” describes the symptoms more accurately in the paediatric population, and invokes thought on the differential diagnoses.

Case report

A 10 years old girl was referred with the complaints of “per vaginal bleeding and a lump at the introitus”. She presented with interlabial bleeding (spotting), swelling, itch, pain, and dysuria for 2 weeks. This was preceded by cycling with no significant history of falls or trauma. There was no history of fever or non-accidental injury. She had visited three different doctors and received medical therapy. She was reviewed with both parents present. She was non-dysmorphic, prepubertal Tanner Stage 1 and thin with a body mass index (BMI) of 13.6 kg/m². There was vulvitis and an inflamed interlabial mass with ulceration and bleeding (Figure 1). No other mucosa or skin lesions were present. Ultrasound and Magnetic Resonance Imaging pelvis were performed to define the lesion. These revealed a “subcentimetre arteriovenous fistula and haematoma at the posterior vaginal wall”. There was no rhabdomyosarcoma or ectopic ureterocele. Despite one more week (total 3 weeks) of conservative therapy with oral antibiotics, topical antimicrobial cream and sitz baths, she experienced worse interlabial swelling, itch, pain, discharge and dysuria (Figure 2).

Figure 1.

Clinical examination reveals vulvitis and a bleeding interlabial mass with ulceration.

Figure 2.

An increasing interlabial mass with pain, dysuria, discharge and itch despite 3 weeks of medical therapy.

The indications for surgery were failure of medical therapy and definitive treatment of her symptoms. Cystoscopy confirmed no urethra polyp or bladder prolapse. A urethra catheter was placed. The reddish mass was a circumferential urethra prolapse (Figure 3). Dilute adrenaline was injected into the prolapsed mucosa. The prolapse was excised in elliptical segments in each of the four quadrants, and the edges closed with interrupted absorbable sutures. The urethra prolapse was reduced, with no recurrence demonstrated on Valsalva maneuver (Figure 4). Vaginoscopy revealed two subcentimetre polyps which were excised, in accordance with the location on MRI. She was discharged the next day with minimal pain and no bleeding.

Figure 3.

Examination under anaesthesia reveals a circumferential urethra prolapse.

Figure 4.

Post excision, urethra prolapse is completely reduced.

Histology confirmed the urethra specimens had areas of denudation, acute on chronic inflammation, increased vascularity and fibrosis. Invaginated urothelium formed scattered gland-like spaces. There were parts of an organizing haematoma and focal squamous metaplasia. No dysplasia or malignancy was identified. The vaginal fibroepithelial polyps showed suggestion of chronic inflammation.

Post operatively, her symptoms resolved. Oral antibiotics, sitz baths, antimicrobial cream and topical estrogen were administered. At 1 year follow up, the wound was well healed (Figure 5). There was no recurrent prolapse or urethra stricture.

Figure 5.

Post operatively, she was active and had returned to sports. At 1 year follow up, there was no recurrent urethra prolapse or stricture.

Discussion

The aim of this report is to increase awareness of the urethral prolapse in children and its management. A urethral prolapse is a circumferential urethral mucosa eversion. It is rare and thought to be due to poor attachments between the inner longitudinal and outer circular-oblique smooth muscle layers of the urethra, as evidenced in cadaveric dissection.¹ It presents in pre-pubertal and post-menopausal females, suggesting an association with estrogen deficiency. Factors associated with urethral prolapse in children include: African, or African-American ethnicity,² history of prematurity, malnutrition or low body mass index,³ increased intra-abdominal pressure from coughing or constipation,⁴ trauma, urinary and vaginal infections.⁵

The association of ethnicity and low BMI with urethral prolapse in children is in contrast to adults. Underweight children may predispose to a lack of cohesiveness between the inner longitudinal and outer circular-oblique smooth muscle layers of the urethra. In adults, pelvic organ prolapse is associated with postmenopausal Caucasian women and high BMI.⁶ Central obesity correlates with higher intra-abdominal pressure readings. However, this observation has not been demonstrated in growing children, possibly due to the difference in fat distribution.⁷

It can be challenging to diagnose urethral prolapse,⁸ with the child’s embarrassment and discomfort contributing to the difficulty. The incidence of urethral prolapse in an Asian country is estimated as one in 73,000 live births.⁴ Due to the aforementioned, the misdiagnosis rate is high. The initial clinical impression was reportedly correct in only 21% of patients.⁹ The time from onset of symptoms to diagnosis has been reported to range from 1 to 80 days, and significantly delayed in an Asian country as compared with non-Asian countries.⁴ Asian patients with urethral prolapse most commonly complain of “vaginal bleeding”, described as spotting or haematuria,⁴ or an interlabial mass.³ The everted mucosa ulcerates and bleeds due to contact abrasions. With increasing eversion, the patient can complain of vulva discharge,¹⁰ itch and pain. Other presenting lower urinary tract symptoms include dysuria,¹¹ frequency, urgency and straining.¹²

Clinical examination needs to exclude signs of condyloma acuminatum, of which the lesion is raised, fleshy and skin colored, and sexual abuse, for e.g. unusual bruises or tears of the labia or vulva due to forceful penetration. Ultrasound and magnetic resonance imaging help with operative planning. Such imaging modalities are used to look for differential diagnoses including rhabdomyosarcoma, presenting as a grapelike vaginal mass; and a prolapsed ureterocele, originating from an orthotopic, or ectopic distal ureter, or a duplex system.¹³

When the diagnosis is still uncertain, general anaesthesia is needed for confirmation and treatment. Urethral prolapse is the only lesion in which the urethra meatus is completely surrounded by a circular mass. It is an anterior vulval mass and can by haemorrhagic or edematous. Urinary catheterisation allows the meatus to be identified clearly in the centre of the mass. The differential diagnosis is a mass protruding through the urethra that is due to a prolapsed bladder, prolapsed ureterocele, urethral polyp or bladder polyp. Cystoscopy determines the origin of such lesions. A mass separate from and displacing the meatus is found in a periurethral cyst or abscess. An interlabial mass arising from the vaginal orifice can be an imperforate bulging hymen or a vaginal polyp.

Although pending consensus, a review of studies with more than 20 patients under 15 years of age, supports excision for urethral prolapse refractory to medical treatment (Table 1). Medical management was started for most patients, and continued for mild cases (where there was a mass without symptoms). This consists of topical estrogen therapy, sitz baths, antibiotics, topical antimicrobials and/or topical steroids. Therapy has ranged from 3 days to 6 weeks, with up to 84.3% of patients progressing to surgery due to persistent or recurrent symptoms.^{2–5,9–12,14–16} Several days to weeks are needed for the prolapsed mucosa to slough away and heal. Patients can experience bleeding, discomfort and anxiety, with loss of school time and parental working hours.¹⁴ In our patient, during the first 2 weeks of symptoms, she had visited three different doctors with possible delayed recognition of her diagnosis. After one more week (total 3 weeks) of medical therapy, the indication for surgery was worsening of symptoms and failure of medical management.

Table 1.

Management of patients with symptomatic urethral prolapse.

Author	Journal	Therapy	N	Median H days (range)	Complication	Recurrence/persistence of symptoms
Jerkins et al.²	The journal of urology	Excision	15	1	0	0
		Reduction and catheter	14	4.7	0	3
		Antibiotics and topical therapy	6	0	0	4 (all underwent excision)
		Ligation over catheter	3	4	Significant pain (n = 3).	0
		Ligation over catheter	3	4	Difficult drainage (n = 2)	0
		Cauterization	1	1	0	0
		Urethrotomy	1	1	0	0
Yi Wei et al.³	Arab journal of urology	Antibiotics and topical therapy	89	—		75 (fifty-five were excised and 20 underwent ligation)
		Excision	55	4.6 (1–7)	—	0
		Ligation over catheter	20	7.8 (5–12)	Significant pain (n = 16)	0
Ninomiya et al.⁴	Pediatrics international	Excision	42	—		5 (two underwent repeat excision)
Ninomiya et al.⁴	Pediatrics international	Antibiotics, topical steroid, sitz bath, topical oestrogen	35	—		25 (twenty-three underwent excision)
Owens et al.⁵	The journal of urology	Ligation over catheter	42	5	Suture loosening (n = 1) underwent repeat ligation	2 (one underwent excision)
		Excision	9	—	Bleeding (n = 1) required blood transfusion	0
		Coagulation	3	—		0
		Sitz bath	2	—		0
Anveden et al.⁹	Pediatric emergency care	Excision	15	2 (0–6)	0	1
		Sitz bath and topical estrogen	8	—	0	5 (all underwent excision)
		Reduction and catheter	1	—	0	0
Venogopal et al.¹⁰	Pediatric surgery international	Antimicrobial ointment	15	—		9 (two underwent excision)
		Estrogen cream	10	—	Theliarchy (n = 2) resolved after stopping medication	5 (one underwent excision)
		Excision	5	—		0
Fernandes et al.¹¹	Urology	Excision	16	2	Mild haematuria (n = 1)	0
		Ligation over catheter	5	(5-6)	Infection (n = 1). Significant pain (n = 5}	2
		Antibiotics and topical therapy	3	0	0	1
Holbrook et al.¹²	BJU international	Sitz bath	13	—		0
		Reduction under GA	7			Incomplete reduction(n = 4). Recurrence (n = 1)
		Diagnosis under GA	1			0
Hillyer et al.¹⁴	Urology	Excision	30	0 to 3	—	0
Hillyer et al.¹⁴	Urology	Antibiotics and topical therapy	6	0	0	2
Trotmanr et al.¹⁵	British journal of urology	Excision with knife	18	—	0	1 (underwent medical therapy and diathermy excision)
		Excision with diathermy	5	—	Meatal stricture (n = 1) required dilatation	0
		Antibiotics and topical therapy	14	—		1 (underwent medical therapy and knife excision)
		Ligation over catheter	5	—		2
Rudin et al.¹⁶	Journal of pediatric surgery	Excision	33	—	Urethral stenosis (n = 1)	0
Rudin et al.¹⁶	Journal of pediatric surgery	Antibiotics and topical therapy	15	—		4

N: Number of patients. H days: Hospitalization days. —: Not reported. GA: General anaesthesia.

Excision of the prolapse provides prompt resolution of the symptoms and minimizes hospitalization stay (Table 1). Most authors described knife excision of the prolapsed mucosa circumferentially, followed by suturing of the proximal urethra to the adjacent vestibule.^{2,9,11,14–16} This is performed with¹⁶ or without¹¹ the aid of a urethral catheter. The majority of patients were discharged after 1 day.^2,3,9,14 Complications included meatal stricture (n = 1) after diathermy excision,¹⁵ urethral stenosis (n = 1),¹⁶ bleeding requiring blood transfusion (n = 1)⁵ and mild hematuria (n = 1).¹¹ Recurrence of urethral prolapse occurred in up to 12% of patients^4,9,15 and was treated by re-excision,^4,15 and medical therapy.¹⁵

Our modifications of the excision technique has not been reported previously. Adrenaline 1 mg was diluted in 100 mils of normal saline and injected into the prolapsed mucosa. This decreases bleeding. Elliptical segments, about 1 cm each, of the prolapsed urethra were excised with knife, and the edges closed with interrupted PDS 5.0 and 6.0 sutures. Sub-centimetre segments of mucosa were left between the excised specimens. Bipolar diathermy was used only for hemostasis and circumferential coagulation on the mucosa was avoided. These measures prevent an iatrogenic stenosis.

Ligation of the prolapsed urethral mucosa over a catheter causes ischemia and sloughing off. This required prolonged hospitalization stay up to 12 days.^2,3,5,11 Significant postoperative pain was experienced in 80–100% of patients.^2,3,11 Recurrence of symptoms occurred in up to 40% of patients.^2,3,5,15 Reduction of the prolapse under general anaesthesia resulted in residual prolapse of varying degrees, and recurrence in up to 57% of patients.^2,12 Due to these complications, these techniques have not been widely adopted.

Conclusion

It is important to consider a urinary tract aetiology for paediatric interlabial bleeding and mass. Increased awareness aids early diagnosis and effective management in children. Careful excision of a urethra prolapse is curative, and preserves function.

Footnotes

Acknowledgements

God for His guidance. Many thanks to the patient and family for consenting to share the images.

Data availability

Data sharing is not applicable to this article as no datasets were generated or analysed during the current study.

Declaration of conflicting interests

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Ethical approval

Ethical approval was not required for reporting because this is a retrospective audit of an individual case.

Funding

The author(s) received no financial support for the research, authorship, and/or publication of this article.

Informed consent

Written informed consent was obtained from the patient and parent for their anonymised information to be published in this article.

ORCID iD

Zeng Hao Joel Wong

References

Lowe

Hill

Jeffs

, et al. Urethral prolapse in children: insights into etiology and management. J Urol 1986; 135(1): 100–103.

Jerkins

Verheeck

Noe

. Treatment of girls with urethral prolapse. J Urol 1984; 132(4): 732–733.

Wei

Lin

, et al. Diagnosis and treatment of urethral prolapse in children: 16 years’ experience with 89 Chinese girls. Arab J Urol 2017; 15(3): 248–253.

Ninomiya

Koga

. Clinical characteristics of urethral prolapse in Japanese children. Pediatr Int 2017; 59(5): 578–582.

Owens

Morse

. Prolapse of the female urethra in children. J Urol 1968; 100: 171–174.

Whitcomb

Rortveit

Brown

, et al. Racial differences in pelvic organ prolapse. Obstet Gynecol 2009; 114(6): 1271–1277.

Ejike

Kadry

Bahjri

, et al. Semi-recumbent position and body mass percentiles: effects on intra-abdominal pressure measurements in critically ill children. Intensive Care Med 2010; 36: 329–335.

Shavit

Solt

. Urethral prolapse misdiagnosed as vaginal bleeding in a premenarchal girl. Eur J Pediatr 2008; 167(5): 597–598.

Anveden-Hertzberg

Gauderer

Elder

. Urethral prolapse: an often misdiagnosed cause of urogenital bleeding in girls. Pediatr Emerg Care 1995; 11: 212–214.

10.

Venugopal

Duncan

Carpenter

. Urethral prolapse in girls. Pediatr Surg Int 1995; 10: 115–117.

11.

Fernandes

Dekermacher

Sabadin

, et al. Urethral prolapse in children. Urology 1993; 41(3): 240–242.

12.

Holbrook

Misra

. Surgical management of urethral prolapse in girls: 13 years’ experience. BJU Int 2012; 110(1): 132–134.

13.

Nussbaum

Lebowitz

. Interlabial masses in little girls: review and imaging recommendations. AJR Am J Roentgenol 1983; 141(1): 65–71.

14.

Hillyer

Mooppan

Kim

, et al. Diagnosis and treatment of urethral prolapse in children: experience with 34 cases. Urology 2009; 73(5): 1008–1011.

15.

Trotman

Brewster

. Prolapse of the urethral mucosa in prepubertal West Indian girls. Br J Urol 1993; 72(4): 503–505.

16.

Rudin

Geldt

Alecseev

. Prolapse of urethral mucosa in white female children: experience with 58 cases. J Pediatr Surg 1997; 32(3): 423–425.