https://orcid.org/0000-0002-6148-3064
Abstract
Spontaneous rupture of urinary bladder (SRUB) is a rare condition with no standard of care and significant related morbidity and mortality. Several predisposing factors have been reported, including malignancy, cystitis, binge drinking, bladder outlet obstruction, and connective tissue disorders. We describe the successful minimally invasive surgical management of a SRUB in a 60-years-old woman with known Osler–Weber–Rendu disease. After exclusion of all other recognized causes of spontaneous rupture of urinary bladder, perforation was considered most likely related to chronic inflammatory involvement of the urinary bladder, possibly associated to hereditary haemorrhagic telangiectasia. Although urinary bladder involvement in Osler–Weber–Rendu disease has been documented, typically presenting as hematuria secondary to telangiectatic lesions, to our knowledge this is the first reported case of a spontaneous rupture of urinary bladder associated with hereditary hemorrhagic telangiectasia.
Introduction
Urinary bladder rupture is a clinical entity mainly related to traumatic, possibly iatrogenic, events. Bladder rupture can occur intra-peritoneally (15%–30%), extra-peritoneally (60%–65%), or in both compartments (10%–25%). While extraperitoneal rupture can be managed conservatively in selected cases, intraperitoneal rupture typically requires surgical repair, with excellent postoperative outcome [1, 2].
Spontaneous rupture of urinary bladder (SRUB) has also been described; however, it remains a rare and diagnostically challenging surgical emergency for which no standardized management guidelines exist and which is associated with considerable morbidity and mortality. Reported etiologies include malignancy, cystitis, binge drinking, bladder outlet obstruction, and connective tissue disorders [2, 3].
Urinary bladder involvement in Osler-Rendu-Weber disease, or hereditary haemorrhagic telangiectasia (HHT), has been documented primarily in the form of painless haematuria due to bladder and urethral telangiectasia [4–6]. However, to our knowledge, an association between HHT and SRUB has not previously been reported in the literature.
Case report
A 60-year-old woman with known Osler-Weber-Rendu disease, three previous cesarean sections and a hystero-annexectomy for benign disease 8 years before, presented with a 24-hour history of lower abdominal pain associated with mild dysuria. Inflammatory markers were within the normal range (Table 1). Urinalysis revealed moderate leukocyturia (24 M/l) without hematuria (Table 2). Contrast-enhanced computed tomography (CT) demonstrated a significant amount of free intraperitoneal fluid without any identifiable source. Although delayed-phase imaging did not demonstrate contrast extravasation from the bladder (Fig. 1), the presence of an acute abdomen associated with unexplained intraperitoneal fluid raised concern for an occult surgical emergency. Exploratory laparoscopy was therefore undertaken to establish the diagnosis and exclude gastrointestinal perforation or other intra-abdominal pathology. During laparoscopy, the clear intraperitoneal fluid prompted biochemical analysis, which revealed a markedly elevated creatinine concentration (1101 μmol/L), suggesting urinary ascites. Following minimal adhesiolysis, a 1.5-cm perforation of the bladder dome was identified (Fig. 2). The edges of the defect were excised, and the bladder was closed with interrupted absorbable 3–0 sutures. Histopathological examination of the resected margins demonstrated partially abraded squamous mucosa with fibrous changes of the chorion and underlying musculature, consistent with chronic inflammation. The surgical drain was removed on postoperative day 3, and the patient was discharged the same day. The Foley catheter was removed on Day 12 following normal cystography (Fig. 3). Follow-up cystoscopy at 6 weeks demonstrated an inflammatory lesion at the bladder dome, compatible with healing, along with three additional inflammatory areas (Figs 4 and 5). Urine cytology was negative for high-grade urothelial carcinoma. The chronological sequence of clinical events is summarized in the CARE timeline (Table 3).
Blood tests and complete blood count with differential.
| Test | Unit | Reference range | Result |
|---|---|---|---|
| C-reactive protein | mg/L | 0.00–10.00 | 0.94 |
| Sodium | mmol/L | 136–144 | 141 |
| Potassium | mmol/L | 3.6–4.6 | 4.9 |
| Urea | mmol/L | 3.2–7.5 | 6.1 |
| Creatinine | μmol/L | 44–80 | 106 |
| eGFR (CKD-EPI 2021) | mL/min/1.73 m2 | > 60 | 52 |
| Erythrocytes | T/L | 3.80–5.20 | 4.32 |
| Hemoglobin | g/L | 120–160 | 130 |
| Hematocrit | % | 37.0–47.0 | 39.5 |
| MCV | fL | 82.0–98.0 | 91.4 |
| MCH | pg | 26.0–34.0 | 30.1 |
| MCHC | g/L | 320–360 | 329 |
| Leukocytes | G/L | 4.0–11.0 | 11.1 |
| WBC differential | |||
| Neutrophils | % | 33.0–80.0 | 73 |
| Eosinophils | % | 0.0–5.0 | 0.4 |
| Basophils | % | 0.0–2.0 | 0.5 |
| Monocytes | % | 0.0–9.0 | 5.9 |
| Lymphocytes | % | 15.0–60.0 | 20.2 |
| Platelets | G/L | 150–350 | 362 |
Urinalysis, flow cytometry, and microscopic examination results.
| Test | Unit | Reference range | Result |
|---|---|---|---|
| pH | 5.0–6.5 | 5 | |
| Specific gravity | 1.041 | ||
| Proteins (semi-quantitative) | Negative | Negative | |
| Hemoglobin | ++ | ||
| Glucose (semi-quantitative) | Negative | Negative | |
| Ketone bodies | Negative | + | |
| Bilirubin | Negative | Negative | |
| Urobilinogen | mg/dL | 0.1–2.0 | 0.2 |
| Nitrites | Negative | Negative | |
| Leukocytes (semi-quantitative) | Negative | + | |
| Flow cytometry | |||
| Leukocytes | M/L | 0–16 | 24 |
| Erythrocytes | M/L | 0–26 | 20 |
| Microscopic examination | |||
| Squamous cells | + | ||
| Urothelial cells | + |
Axial delayed-phase contrast-enhanced CT scan showing intravesical contrast without evidence of extravasation.
Laparoscopic view of the bladder dome perforation. Intraoperative inspection reveals a 1.5-cm defect at the bladder dome.
Postoperative cystography showing intact bladder wall without contrast extravasation.
Cystoscopic view at 6 weeks. Endoscopic evaluation at 6 weeks shows mucosal remodeling and inflammatory changes at the previous perforation site, consistent with normal healing.
Cystoscopic view at 6 weeks.
CARE timeline of the patient’s clinical course
| Time | Event |
|---|---|
| Day −1 to 0 | Onset of lower abdominal pain and mild dysuria. |
| Day 0 | Presentation to the Emergency Department. Blood tests normal; urinalysis shows leukocyturia without hematuria. |
| Day 0 | Contrast-enhanced CT reveals free intraperitoneal fluid (no bladder contrast extravasation on delayed sequences). |
| Day 0 | Exploratory laparoscopy performed → detection of intraperitoneal fluid with high creatinine (1101 μmol/L); 1.5-cm bladder dome perforation identified. |
| Day 0 | Laparoscopic repair performed: minimal adhesiolysis, resection of edges, closure with interrupted absorbable 3–0 sutures. Foley catheter + surgical drain placed. |
| Postoperative Day 3 | Surgical drain removed; patient discharged the same day. |
| Postoperative Day 12 | Foley catheter removed following normal cystography. |
| 6-week follow-up | Cystoscopy shows inflammatory lesion at dome consistent with healing + three additional inflammatory areas; urine cytology negative for high-grade carcinoma. |
Discussion
SRUB is a rare clinical entity, with an estimated incidence of 1 per 126 000 individuals and accounting for ~1% of all bladder ruptures [2]. A recent systematic review identified 351 reported cases, with pelvic radiation, bladder outlet obstruction, bladder diverticula, idiopathic etiologies, and neurogenic bladder emerging as the most common predisposing factors in patients over 60 years of age [3].
In the present case, the patient had no history of pelvic radiotherapy or excessive alcohol consumption, and postoperative cystoscopy and cystography demonstrated neither voiding dysfunction nor bladder diverticula. Infectious causes were excluded, as urinalysis and urine culture showed no evidence of urinary tract infection. Malignancy was also ruled out: both the biopsy of the perforation margins and urine cytology were unremarkable, and postoperative evaluations confirmed the absence of neoplastic lesions.
Histopathological examination of the perforation margins instead revealed partially abraded squamous mucosa with fibrous changes in the chorion and underlying musculature—findings compatible with chronic inflammation. A possible association with hereditary haemorrhagic telangiectasia was considered the most plausible explanation after exclusion of all recognized causes of spontaneous bladder rupture.
Bladder involvement in Osler–Weber–Rendu disease has previously been described, typically manifesting as painless hematuria due to bladder or urethral telangiectasia. Interestingly, our patient had never experienced macrohematuria, nor did urinalysis reveal significant microscopic haematuria.
This case highlights two important points. First, SRUB remains diagnostically challenging; in particular, the sudden appearance of unexplained intraperitoneal fluid should raise suspicion for bladder rupture and may warrant prompt laparoscopic exploration. Second, to our knowledge, this represents the first reported association between SRUB and Osler–Weber–Rendu disease. Although the pathological findings do not establish causality, the absence of any recognized predisposing factor raises the possibility of an association, although a causal relationship cannot be established from a single observation.
Given the rarity of both conditions, additional case reports will be necessary to determine whether this coexistence is coincidental or whether a true pathophysiological association exists.
Conflicts of interest
None declared.
Funding
None declared.
