Large primary vaginal stone in a woman with multiple sclerosis
- 1 Department of Obstetrics and Gynaecology, Onze Lieve Vrouwe Gasthuis, Amsterdam, The Netherlands
- 2 Department of Urology, Onze Lieve Vrouwe Gasthuis, Amsterdam, The Netherlands
- Correspondence to S Lot Aronson; aronson_lot@hotmail.com
Abstract
Vaginal stones are rare and therefore a delay in accurate diagnosis often occurs. We present a 54-year old woman with multiple sclerosis who was diagnosed with a primary vaginal stone. Initially, she presented with recurring urinary tract infections (UTI) and macroscopic haematuria to the urologist. A cystoscopy showed no abnormalities. Because of persistent bleeding, she was referred to the gynaecologist, and on gynaecological examination, a vaginal stone was revealed. Stone formation was likely to be the result of urinary pooling due to incontinence, which was caused by a neurogenic bladder. Other contributing factors were prolonged recumbency, threads of an intrauterine device and a UTI. The presence of a vesicovaginal fistula was excluded by testing with methylene blue. The stone was surgically removed and composed of 70% struvite and 30% apatite. The patient was treated for decubitus ulcerations of the vaginal wall with estriol (Synapause-E3). Follow-up was uneventful.
Background
The occurrence of a primary vaginal stone is extremely rare and, in many cases, initially misdiagnosed. Primary vaginal stones are believed to be formed by stasis of urine in the vagina, leading to the deposition of urinary salts. Even though most primary stones are detected in association with vesicovaginal or urethrovaginal fistulae, various other aetiologic factors are described—for example, neuropathic dysfunction of the bladder, ectopic ureters or anatomical abnormalities causing vaginal outlet obstructions.1 Secondary stones are the result of deposition of urinary salts around foreign bodies such as contraceptive devices, forgotten surgical tools or gauzes or secondary to vaginal mesh exposure.
We present a case of a neurologically disabled woman with a large vaginal stone in absence of a vesicovaginal fistula, representing delay in the accurate diagnosis that allowed the stone to enlarge.
Case presentation
The patient was a 54-year-old woman, known with multiple sclerosis (MS)-related paraparesis and urinary incontinence as a result of neurogenic bladder dysfunction. Her Expanded Disability Status Scale (EDDS) score was 8.5. She presented to the urologist with recurring urinary tract infections (UTI) and suspected macroscopic haematuria, which had been presented for the past 6 months, causing blood loss in her incontinence sanitary pad every 3–5 weeks. Urinalysis showed red blood cells and bacteriuria. A flexible cystoscopy was performed, while the patient was sitting in her wheelchair, showing no abnormalities. The urologist decided to treat the UTI and advised to differentiate between haematuria and vaginal bleeding through the use of a vaginal tampon. Delay occurred due to a no-show and several unsuccessful attempts of the tampon test at home. Five months later, during a telephone consultation, the bleeding turned out to be persistent and the patient was referred to the gynaecologist to determine whether the bleeding had a genital source. Furthermore, her wish was to remove her intrauterine device (IUD), which was placed 9 years ago because of heavy menstrual bleeding. On request of the patient, the IUD had not been removed earlier, since the menstrual bleeding had stopped completely and she had no reports. She kept postponing the removal of her IUD because she dreaded the difficult transfer to a gynaecological examination chair with her physical disability.
In our outpatient clinic, on gynaecological examination, a large, hard and white vaginal stone that could not be removed was discovered (figure 1). The stone filled up ¾ of the vagina, so we were not able to see the cervix and therefore not able to perform cervical cytology or remove the IUD.
Primary vaginal stone on examination.
Investigations
An attempt was made to perform a transvaginal ultrasonography, results of which were inconclusive. The vaginal stone could not be outlined and blocked the image of the uterus, cervix and adnexa. Based on these findings, the patient was listed to undergo operative removal of the vaginal stone and for further investigation to be performed under general anaesthesia. No additional imaging was performed.
Treatment
Under general anaesthesia, the stone was extracted with the use of several sharp instruments. The museux tenaculum forceps (6 mm jaws, 24 cm) provided the best grip on the stone and were strong and sharp enough to crush it into smaller pieces, spreading a foul odour. The instrument features double-pronged jaws that ensure fixation onto tissues. Their principal use is to stabilise and hold the uterus and cervix during hysterectomy, oophorectomy or tubal ligation. The stone was entirely removed without complications in 109 min. A small laceration of the vaginal wall was sutured. The stone measured approximately 10×6×5 cm and the threads of the IUD were trapped inside the stone. It appeared as though the growing stone had pulled the IUD out of the uterus sometime before. Inspection of vulva and vagina showed no anatomical abnormalities. Several decubitus ulcerations were seen, on which estriol (Synapause-E3) was applied. A cervix cytology test was performed. Transvaginal ultrasound showed a normal uterus and adnexa. A methylene blue solution was instilled into the bladder to exclude the presence of a vesicovaginal fistula.
Outcome and follow-up
On chemical analysis, the stone was found to be composed of 70% magnesium phosphate (struvite) and 30% calcium phosphate (apatite). Furthermore, culture of the urine showed a UTI with Proteus mirabilis. The UTI was treated with ciprofloxacin and, on consultation with the urologist, a long-term urinary catheter was left in situ. The patient continued applying topical estriol (Synapause-E3) for 2 weeks. Cervix cytology appeared inconclusive (Pap 0), which led to the advice to repeat the test at a later stage. Follow-up after 4 weeks was uneventful, and the patient was symptom free after this period. The decubitus ulcerations were believed to be the cause of the vaginal bleeding.
Discussion and review of the literature
In the literature, most primary stones are reported in association with vesicovaginal fistulae.1 This case report describes a different mechanism of stone formation, based on multiple contributing factors: incontinence due to a neurogenic bladder, prolonged recumbency, threads of an IUD and a UTI. The incidence of vaginal stones is not known, as this condition is described in the literature only as case reports.
Neurogenic bladder dysfunction is associated with wheelchair-depended MS and, in this case, led to continuous leakage of urine into the vagina. In combination with the prolonged recumbent posture, the urinary pooling was a prerequisite for formation of the vaginal stone. Navani and others also described a case similar to the present one.2 A 72-year-old bedridden woman suffering from MS presented with abdominal pain and fever due to a primary vaginal stone of 5 cm in diameter. Twelve other cases of primary vaginal stones in women and children with physical disabilities were reported between 1970 and 2018 (table 1).2–14
Review of primary vaginal stones in women and children with physical disabilities
Article | Year | Age (years) | Comorbidity | Composition and measurements | Management |
Navani and Tessier2 | 1970 | 72 | Multiple sclerosis, bedridden | Composition unknown. 5 cm in diameter |
Surgical removal using long Kocher forceps, saline irrigation and sponge forceps |
Miller9 | 1973 | 17 | Microcephaly, spastic quadriparesis, recumbent position | Struvite (88%), microcrystalline carbonate apatite (12%) 4×4.1×2.5 cm |
Non-surgical removal |
Petrillo et al 12 | 1981 | 12 | Meningomyelocele, bedridden | Struvite (MgNH4PO4) 4.5×2.5 cm |
Surgical removal using Kocher forceps |
Bissada et al 4 | 1983 | 12 | Meningomyelocele, neurogenic bladder | Struvite (MgNH4PO4) 4×3×3 cm |
Surgical removal |
Sant et al 10 | 1983 | 9 | Myelodysplasia, urinary incontinence,clitoral hypertrophy, and partial fusion of the labia majora | Struvite (MgNH4PO4) (55%) and apatite (45%) |
Extraction through a midline, posterior episiotomy separating the labia majora inferiorly |
Yoshimura et al 11 | 2000 | 11 | Cerebral infarction, completely recumbent, urinary incontinence | Struvite (MgNH4PO4) 4×3×3 cm |
Removal without episiotomy |
Cetinkursun et al 6 | 2001 | 13 | Cerebral palsy, recumbent position, urinary incontinence | Struvite (MgNH4PO4) (85%), microcrystalline carbonate apatite (15%). 4×3×2 cm |
Removal using Kocher forceps from the vagina through hymenal incision and vaginal dilation |
Lin et al 13 | 2005 | 43 | Cerebral palsy, bedridden, urinary incontinence | Composition unknown 10×8×4.5 cm |
Laparotomy, incision in the anterior vaginal cuff |
Jaspers et al 8 | 2010 | 5 | Infantile encephalopathy, spastic tetraplegia, wheelchair user | Struvite (MgNH4PO4) 3×2 cm and 2×1 cm |
Disintegration of the stones using a Calcuson device and a nephroscope |
Ikeda et al 7 | 2013 | 42 | Cerebral palsy, immobile, urinary incontinence | >98% Struvite (MgNH4PO4), calcium phosphate in the crust. 2.5 cm in diameter |
Non-surgical removal using Kocher forceps |
Avsar et al 3 | 2013 | 22 | Congenital neural tube defect paraplegia | Struvite (MgNH4PO4) 9×7×2 cm |
Right-sided episiotomy and ring Kocher forceps |
Castellan et al 5 | 2017 | 34 | West Syndrome, quadriplegia, urinary incontinence | Struvite (MgNH4PO4) 5.1×3.7×3.1 cm |
Surgical removal using surgical pliers and several manoeuvres |
Tokgoz et al 14 | 2018 | 14 | Neurodegenerative disease with kyphoscoliosis and decorticate posture | Struvite (MgNH4PO4) Long-axis 3.8 cm |
No details of removal |
The IUD and UTI were most likely contributing factors to stone formation. The deposition of urinary salts around the threads of the IUD may have been the start of this vaginal stone. The presence of urease-producing bacteria (eg, Escherichia coli, Proteus mirabilis, Klebsiella species) is the precondition for the formation of struvite or apatite stones.15 These bacteria split urea, which is excreted in urine, forming ammonia, which elevates urine pH. This alkaline environment favours precipitations of struvite and apatite crystals, of which the majority of stones reported in the literature are composed.10 To prevent recurrence of stone formation, the UTI was treated after removal of the stone.
Vaginal stones are often mistaken for bladder calculi, because it is difficult to differentiate when using a plain film X-ray. A vaginal examination (vaginal toucher and speculum exam) is sufficient to diagnose a vaginal stone. Cystoscopy, vaginoscopy, CT or MRI are valuable for excluding bladder stones and estimating the size of the stone. In our case, cystoscopy was already performed recently and showed no abnormalities. Additional imaging was not necessary because the stone was easily visible by inspection of the introitus. Additional imaging may have been useful for an accurate estimation of the measurements of the vaginal stone but would not have altered the treatment.
In the literature, different methods are described for removal of vaginal stones, either with simple extraction using surgical instruments or more invasive procedures through episiotomy, hymenal incision or transperitoneal approach (table 1). Decisive factors are the measurements of the stone, presence of anatomical variations and the priority to preserve the integrity of the genital organs. Preoperative treatment with oestrogen to strengthen the vaginal epithelium and prepare the hymen for iatrogenic stretching is described.8 Jaspers and others combined introduction of a nephroscope and a shock wave lithotripsy probe to disintegrate the stone and remove it by using running fluids.8 Lin and others used an incision in the anterior vaginal cuff during explanatory laparotomy to remove a vaginal stone measuring 10 cm.13 Avsar and others performed a right-sided episiotomy to remove a stone measuring 9×7×2 cm.3 There are no guidelines available worldwide for treatment of vaginal stones. In this case report, we present the successful use of the museux tenaculum forceps for safe and effective removal of the vaginal stone. After removal, treatment of the underlying factors is necessary.
This case report gives us the chance to reflect on reasons for delay in reaching the accurate diagnosis. A thorough physical assessment at time of first presentation might have led to an earlier detection of the stone and reflects doctor delay. This case shows that performing a cystoscopy in a wheelchair is second best and impedes optimal examination. In addition, patient delay has contributed and might have allowed the stone to enlarge in the time interval.
Patient’s perspective
In my opinion, my reports were taken seriously and the examinations in the outpatient clinic were thoroughly performed. Diagnosis and the suggested treatment were clear to me. I was satisfied with the result of the operation, since the blood loss has been resolved. I did not have any pain after surgery. I still have a urinary catheter, which is flushed two times a day by home care services. Solutio R is used to prevent crystal formation in the catheter.
Learning points
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Recumbent posture, incontinence, intrauterine device threads, and urinary tract infection are contributing factors in the formation of a vaginal stone.
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A primary vaginal stone should be considered in the differential diagnosis in high-risk patients presenting with abnormal bleeding. Thorough physical assessment should be performed, including digital and speculum examination of the vagina.
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When a vaginal stone is present, examination to exclude the presence of a vesicovaginal or urethrovaginal fistula is essential.
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Vaginal stones are often composed of struvite, which can easily be crushed into smaller pieces with the use of sharp surgical instruments that prevents performing more invasive procedures.
Footnotes
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Contributors SLA prepared the first draft of the manuscript, which was then revised by EWMJ and MCH.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Competing interests None declared.
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Patient consent for publication Obtained.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.
References
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