When Limb Surgery Has Become the Only Life-Saving Therapy in FOP: A Case Report and Systematic Review of the Literature

Fibrodysplasia ossificans progressiva (FOP) is a rare disease in which heterotopic ossification (HO) is formed in muscles, tendons and ligaments. Traumatic events, including surgery, are discouraged as this is known to trigger a flare-up with risk of subsequent HO. Anesthetic management for patients with FOP is challenging. Cervical spine fusion, ankylosis of the temporomandibular joints, thoracic insufficiency syndrome, restrictive chest wall disease, and sensitivity to oral trauma complicate airway management and anesthesia and pose life-threatening risks. We report a patient with FOP suffering from life-threatening antibiotic resistant bacterial infected ulcers of the right lower leg and foot. The anesthetic, surgical and postoperative challenges and considerations are discussed. In addition, the literature on limb surgeries of FOP patients is systemically reviewed. The 44 year-old female patient was scheduled for a through-knee amputation. Airway and pulmonary evaluation elicited severe abnormalities, rendering standard general anesthesia a rather complication-prone approach in this patient. Thus, regional anesthesia, supplemented with intravenous analgosedation and N2O-inhalation were performed in this case. The surgery itself was securely planned to avoid any unnecessary tissue damage. Postoperatively the patient was closely monitored for FOP activity by ultrasound and [18F]PET/CT-scan. One year after surgery, a non-significant amount of HO had formed at the operated site. The systematic review revealed seventeen articles in which thirty-two limb surgeries in FOP patients were described. HO reoccurrence was described in 90% of the cases. Clinical improvement due to improved mobility of the operated joint was noted in 16% of the cases. It should be noted, though, that follow-up time was limited and no or inadequate imaging modalities were used to follow-up in the majority of these cases. To conclude, if medically urgent, limb surgery in FOP is possible even when general anesthesia is not preferred. The procedure should be well-planned, alternative techniques or procedures should be tested prior to surgery and special attention should be paid to the correct positioning of the patient. According to the literature recurrent HO should be expected after surgery of a limb, even though it was limited in the case described

Unusual complication after immediate postoperative intravesical Mitomycin C instillation

Immediate adjuvant Mitomycin C (MMC) instillation is routine practice in the treatment of superficial bladder cancer. Despite relative safety we describe a case of MMC extravasation after intravesical instillation. This resulted in severe continuous pain in the pelvic region without tendency of spontaneous healing, and required surgical debridement. To assess perivesical soft tissue injury prior to surgery MRI imaging turned out to be more accurate than computer tomography. Suggestions about how to avoid, diagnose and treat this symptomatic extravasation are made

Clinical Case Discussion: Kidney Stone in a Patient with an Ileal Conduit

A patient underwent robot-assisted laparoscopic cystectomy with an ileal conduit urinary diversion. Percutaneous nephrolithotomy was performed for treatment of a kidney stone. Stone analysis revealed struvite

Cystectomie en urinedeviatie bij interstitiële blaasafwijkingen

Aim of the study was to evaluate the available literature on the likelihood of success of urinary diversions with or without cystectomy in patients with bladder pain syndrome/interstitial cystitis. Three reviews were found, zero randomized trials. In the 21 selected case series 475 patients were included. The success rate of urinary diversion was 81%. Predictors for success (presence of Hunner’s lesion and small bladder capacity) could not be confirmed due to heterogeneity of the studies. Urinary diversion in combination with removal of the bladder increased the chance of success in 96% of cases if combined with cystectomy, in 75% after partial cystectomy and in 79% after urinary deviation without cystectomy. It can be concluded that hard scientific evidence for or against a urinary diversion in BPS/interstitial cystitis is not available, because the (pretty good) results in the case series are to be be considered in the light of (inclusion) bias. Therefore, therapeutic choices should still be made on an individual bases

Bladder necrosis: 'A man without a bladder'

Since the use of antibiotics, bladder necrosis has become a rare condition. We report a case of bladder necrosis in a 90-year-old man following urinary retention. After insertion of a transurethral catheter (TUC), 2 L of urine was evacuated. In the following days, the TUC became intermittently blocked. Adequate bladder drainage could not be obtained despite intensive rinsing and placement of a suprapubic catheter. On surgical exploration necrosis of almost the entire bladder wall, except for the trigone, was encountered. Surgical debridement of the non-viable bladder wall without opening the abdominal cavity was conducted, and a TUC was placed in the Retzius cavity to ensure evacuation of urine. Since the patient was haemodynamically unstable, construction of a urinary diversion was waived and urinary drainage of the Retzius cavity by the TUC was accepted, resulting in adequate urinary drainage without compromising renal function

Salvage cystectomy after failure of interstitial radiotherapy and external beam radiotherapy for bladder cancer

OBJECTIVE: To evaluate the long-term results of salvage cystectomy after interstitial radiotherapy (IRT) and external beam radiotherapy (EBRT) for transitional cell carcinoma, and to assess the morbidity and functional results of the different urinary diversions used. PATIENTS AND METHODS: The records of 27 patients treated with salvage cystectomy in one institution between 1988 and 2003 were retrospectively analysed. RESULTS: Salvage cystectomy was used after failure of IRT in 14 or EBRT in 13 patients, with a 3-and 5-year survival probability of 46% (95% confidence interval 26-65) and 33 (11-54)%. The 5-year overall survival after cystectomy was 54% after IRT and 14% after EBRT (P = 0.12). Tumour category, response to radiation, American Society of Anesthesiology score, and complete tumour resection had a significant influence on survival. Five of seven patients with incomplete resection died because of local disease, with a median survival of 5 months. There was clinical understaging after radiotherapy in 41% of patients. Nine patients had an orthotopic neobladder, with complete day- and night-time continence in eight and four, respectively. All patients but one had good voiding function. There were early complications in two and late complications in six patients (for Bricker, seven of 14 and none; for Indiana, none of four and two of four). The duration of hospitalization was not influenced by the type of diversion. Erectile function was maintained in four of six patients after a sexuality-preserving cystectomy and neobladder. CONCLUSIONS: Salvage cystectomy can be performed with acceptable morbidity using any type of urinary diversion. Understaging after radiotherapy is common, but preoperative selection needs improving. A very significant factor for an adverse outcome and death from local tumour recurrence was incomplete resection, suggesting that salvage cystectomy should only be attempted if complete resection is probable

Small cell carcinoma of bladder: A single-center prospective study of 25 cases treated in analogy to small cell lung cancer

Objectives. To evaluate the feasibility and efficacy of a therapeutic algorithm for the management of small cell carcinoma of the bladder derived from the treatment of small cell lung cancer. Methods. During a 10-year period, 25 patients (23 men and 2 women; median age 64 years, with 8 [32%] older than 75 years) with small cell carcinoma of the bladder were defined as having limited disease (LD) or extensive disease (ED) in analogy to the classification of small cell lung cancer. Patients with LD were eligible for chemotherapy and sequential radiotherapy. Patients unfit for chemotherapy were offered complete transurethral resection and radiotherapy or cystectomy for large symptomatic tumors. Patients with ED were offered palliative chemotherapy. Results. Of the 25 patients, 17 (68%) had LD and 8 (32%) ED. Without regard to stage, the median survival of those receiving chemotherapy was 15 months versus 4 months for those who did not. The median survival for those with LD was 12 months versus 5 months for those with ED. Nine patients (52.9%) with LD could not undergo chemoradiotherapy because of comorbidity and reduced performance (n = 7), progression (n = 1), or drug-related death (n = 1). Five of those patients underwent TUR and radiotherapy and two cystectomy. Conclusions. The prognosis of small cell carcinoma of the bladder is poor. This treatment algorithm offers bladder sparing for most patients, with few long-term remissions in patients with small, confined tumors. None of the patients died of locoregional tumor progression, supporting that cystectomy is not the treatment of choice for those with LD. With a significant proportion of elderly patients with comorbidities, chemoradiotherapy was not feasible in more than one half of the patients with LD

Trial-based Cost-effectiveness Analysis of an Immediate Postoperative Mitomycin C Instillation in Patients with Non-muscle-invasive Bladder Cancer

BACKGROUND: Bladder cancer imposes a significant public health burden on the European Union. There is a need for cost-effective treatment and follow-up regimens. OBJECTIVE: To assess the cost-effectiveness of immediate mitomycin C (MMC) instillation within 1 d after surgery compared to delayed MMC instillation within 2 wk after surgery with further adjuvant treatment, depending on the patient's risk group. DESIGN SETTING AND PARTICIPANTS: This economic evaluation was based on a randomized controlled trial among 2243 Dutch patients with non-muscle-invasive bladder cancer (NMIBC) patients from a health care perspective over a 3-yr time period. OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS: The treatment effect was measured as time to recurrence and recurrence-free survival. Missing effect data were imputed with multiple imputation. Health care utilization and related costs were estimated on the basis of treatment protocols for NMIBC patients in the Netherlands. Statistical uncertainty was estimated using bootstrapping and is graphically presented using cost-effectiveness planes and cost-effectiveness acceptability curves. RESULTS AND LIMITATIONS: Time to recurrence was significantly longer for immediate MMC instillation (27.31 mo) than for delayed MMC instillation (24.97 mo), with an adjusted mean difference of 2.21 mo (95% confidence interval [CI] 1.58-2.84). The proportion of patients with recurrence-free survival was significantly higher after immediate MMC instillation (0.65) than after delayed MMC instillation (0.56), with an adjusted mean difference of 0.08 (95% CI 0.06-0.11). Total mean health care costs per patient were significantly lower for immediate MMC instillation (€22 959) than for delayed MMC instillation (€24 624), with an adjusted mean difference of -€1350 (95% CI -€1799 to -€900). The study is limited by the retrospective estimation of costs. CONCLUSIONS: This trial-based cost-effectiveness analysis shows that from a health care perspective, immediate MMC instillation is more effective and less expensive compared to delayed MMC instillation. PATIENT SUMMARY: We assessed the cost-effectiveness of immediate bladder instillation of mitomycin C after surgery to reduce the risk of recurrence after removal of the bladder tumor as compared to delayed instillation in a large Dutch population of patients with non-muscle-invasive bladder cancer. We found that immediate instillation was more effective and less expensive than delayed instillation. We conclude that immediate mitomycin C instillation is a cost-effective treatment for non-muscle-invasive bladder cancer