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

Development and Internal Validation of a Novel Nomogram Predicting the Outcome of Salvage Radiation Therapy for Biochemical Recurrence after Radical Prostatectomy in Patients without Metastases on Restaging Prostate-specific Membrane Antigen Positron Emission Tomography/Computed Tomography

BACKGROUND AND OBJECTIVE: Owing to the greater use of prostate-specific membrane antigen (PSMA) positron emission tomography/computed tomography (PET/CT) in patients with biochemical recurrence (BCR) of prostate cancer (PCa) after robot-assisted radical prostatectomy (RARP), patient selection for local salvage radiation therapy (sRT) has changed. Our objective was to determine the short-term efficacy of sRT in patients with BCR after RARP, and to develop a novel nomogram predicting BCR-free survival after sRT in a nationwide contemporary cohort of patients who underwent PSMA PET/CT before sRT for BCR of PCa, without evidence of metastatic disease.METHODS: All 302 eligible patients undergoing PCa sRT in four reference centers between September 2015 and August 2020 were included. We conducted multivariable logistic regression analysis using a backward elimination procedure to develop a nomogram for predicting biochemical progression of PCa, defined as prostate-specific antigen (PSA) ≥0.2 ng/ml above the post-sRT nadir within 1 yr after sRT.KEY FINDINGS AND LIMITATIONS: Biochemical progression of disease within 1 yr after sRT was observed for 56/302 (19%) of the study patients. The final predictive model included PSA at sRT initiation, pathological grade group, surgical margin status, PSA doubling time, presence of local recurrence on PSMA PET/CT, and the presence of biochemical persistence (first PSA result ≥0.1 ng/ml) after RARP. The area under the receiver operating characteristic curve for this model was 0.72 (95% confidence interval 0.64-0.79). Using our nomogram, patients with a predicted risk of &gt;20% had a 30.8% chance of developing biochemical progression within 1 yr after sRT.CONCLUSIONS: Our novel nomogram may facilitate better patient counseling regarding early oncological outcome after sRT. Patients with high risk of biochemical progression may be candidates for more extensive treatment.PATIENT SUMMARY: We developed a new tool for predicting cancer control outcomes of radiotherapy for patients with recurrence of prostate cancer after surgical removal of their prostate. This tool may help in better counseling of these patients with recurrent cancer regarding their early expected outcome after radiotherapy.</p

Development and Internal Validation of a Novel Nomogram Predicting the Outcome of Salvage Radiation Therapy for Biochemical Recurrence after Radical Prostatectomy in Patients without Metastases on Restaging Prostate-specific Membrane Antigen Positron Emission Tomography/Computed Tomography

BACKGROUND AND OBJECTIVE: Owing to the greater use of prostate-specific membrane antigen (PSMA) positron emission tomography/computed tomography (PET/CT) in patients with biochemical recurrence (BCR) of prostate cancer (PCa) after robot-assisted radical prostatectomy (RARP), patient selection for local salvage radiation therapy (sRT) has changed. Our objective was to determine the short-term efficacy of sRT in patients with BCR after RARP, and to develop a novel nomogram predicting BCR-free survival after sRT in a nationwide contemporary cohort of patients who underwent PSMA PET/CT before sRT for BCR of PCa, without evidence of metastatic disease.METHODS: All 302 eligible patients undergoing PCa sRT in four reference centers between September 2015 and August 2020 were included. We conducted multivariable logistic regression analysis using a backward elimination procedure to develop a nomogram for predicting biochemical progression of PCa, defined as prostate-specific antigen (PSA) ≥0.2 ng/ml above the post-sRT nadir within 1 yr after sRT.KEY FINDINGS AND LIMITATIONS: Biochemical progression of disease within 1 yr after sRT was observed for 56/302 (19%) of the study patients. The final predictive model included PSA at sRT initiation, pathological grade group, surgical margin status, PSA doubling time, presence of local recurrence on PSMA PET/CT, and the presence of biochemical persistence (first PSA result ≥0.1 ng/ml) after RARP. The area under the receiver operating characteristic curve for this model was 0.72 (95% confidence interval 0.64-0.79). Using our nomogram, patients with a predicted risk of &gt;20% had a 30.8% chance of developing biochemical progression within 1 yr after sRT.CONCLUSIONS: Our novel nomogram may facilitate better patient counseling regarding early oncological outcome after sRT. Patients with high risk of biochemical progression may be candidates for more extensive treatment.PATIENT SUMMARY: We developed a new tool for predicting cancer control outcomes of radiotherapy for patients with recurrence of prostate cancer after surgical removal of their prostate. This tool may help in better counseling of these patients with recurrent cancer regarding their early expected outcome after radiotherapy.</p

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