Causes of death among people with myelomeningocele: A multi-institutional 47-year retrospective study.

PURPOSE: This study aimed to analyze organ system-based causes and non-organ system-based mechanisms of death (COD, MOD) in people with myelomeningocele (MMC), comparing urological to other COD. METHODS: A retrospective review was performed of 16 institutions in Canada/United States of non-random convenience sample of people with MMC (born > = 1972) using non-parametric statistics. RESULTS: Of 293 deaths (89% shunted hydrocephalus), 12% occurred in infancy, 35% in childhood, and 53% in adulthood (documented COD: 74%). For 261 shunted individuals, leading COD were neurological (21%) and pulmonary (17%), and leading MOD were infections (34%, including shunt infections: 4%) and non-infectious shunt malfunctions (14%). For 32 unshunted individuals, leading COD were pulmonary (34%) and cardiovascular (13%), and leading MOD were infections (38%) and non-infectious pulmonary (16%). COD and MOD varied by shunt status and age (p < = 0.04), not ambulation or birthyear (p > = 0.16). Urology-related deaths (urosepsis, renal failure, hematuria, bladder perforation/cancer: 10%) were more likely in females (p = 0.01), independent of age, shunt, or ambulatory status (p > = 0.40). COD/MOD were independent of bladder augmentation (p = >0.11). Unexplained deaths while asleep (4%) were independent of age, shunt status, and epilepsy (p >= 0.47). CONCLUSION: COD varied by shunt status. Leading MOD were infectious. Urology-related deaths (10%) were independent of shunt status; 26% of COD were unknown. Life-long multidisciplinary care and accurate mortality documentation are needed.

Application of the decisional regret scale (DRS) in men undergoing anti-incontinence procedures for stress urinary incontinence.

Background: Decisional regret (DR) has previously been applied to other urologic issues (hypospadias, prostate cancer). The present study seeks to evaluate certain patient factors that directly correlate to high DR scores in anti-incontinence procedures. Methods: Medical records for 119 patients undergoing anti-incontinence procedures between 2009 and 2020 were retrospectively reviewed. Forty-one patients were accessible for telephone follow-up and provided a Decisional Regret Scale (DRS) questionnaire. If patients had both a sling and an artificial urinary sphincter (AUS) placed, questionnaires were administered for each. DRS score was quantified in accordance with prior literature, with scores ranging 0-100. We subdivided patients based on demographics and surgical data, correlating this with DRS score. Results: In 41 patients, 46 procedures (13 slings, 33 AUS) were performed. Thirty-nine (95.1%) men underwent robotic-assisted laparoscopic prostatectomy, and 11 (26.8%) men reported prior pelvic radiation. Post-procedural continence, irrespective of procedure, yielded an average 2±1.56 pads per day (PPD). Mean DRS score across the cohort was 29.78. DRS score was subdivided into mild, moderate, and severe, with majority noting "none to mild" regret (63%), 15.2% reporting moderate and 21.7% severe. Predictors of higher regret included history of radiation (P=0.056), choice of anti-incontinence procedure (P=0.011), and need for surgical revision (P=0.00042). DR was unrelated to race, complete continence, and time to follow-up. Conclusions: DRS has recently been applied to anti-incontinence procedures for male stress incontinence; our study highlights novel findings not previously assessed. Majority of men had minimal regret with a subset that had significant regret-history of radiation, multiple revisions/explant, and those who elected for sling upfront. These results highlight the importance of patient selection and pre-operative counselling.

9-Year Evaluation of a Transitional Care Program for Congenital Neurogenic Bladder Patients.

OBJECTIVE: To evaluate short and long-term adherence and nonadherence patterns within a staged transition clinic developed in 2014 to assist congenital neurogenic bladder patients transfer from pediatric to adult urologic care. METHODS: We performed a retrospective chart review of all congenital neurogenic bladder patients who had been prospectively identified in the transition clinic and adult urology clinic since 2014. Demographic and clinical data were collected and analyzed to determine patient adherence with transfer of care, to study long-term compliance with follow-up, and to identify factors that positively and negatively influenced transition clinic success. RESULTS: 157 patients participated in the transition clinic. 76/79 (96.2%) patients ready to transition successfully transferred to adult care. 55/76 (72.4%) remain active. Greater number of visits between transition stages was significant among patients who maintained active follow-up after transition (P = .006). 171 patients initiated care in our adult urology clinic without transition. Irrespective of nontransitioned patients longer follow-up, formally transitioned adult patients had higher yearly participation rates, with 55 (72.4%) transitioned patients compared to 63 (36.8%) nontransitioned patients remaining active (P < .001). CONCLUSION: Congenital neurogenic bladder patients who participated in a transition clinic were successful transferring from pediatric to adult care, with improved clinic adherence in adulthood, compared to patients who initiated adult care without structured transition. Early introduction to adult providers and increased number of visits positively impacted adherence upon transition to adult care. Noncompliance with pediatric provider visits negatively impacted transition adherence.

Stromal sarcoma of the prostate.

Primary spindle cell sarcoma of the prostate is exceedingly rare and accounts for 0.1% of all prostatic cancers. Typically, the disease course is rapid and portends a short and dismal prognosis. We report a case of prostatic stromal sarcoma (PSS) which likely lay dormant for several years after a transurethral resection of the prostate. This case is unique in that this cancer did not display the rapid growth of PSS in prior reports. Our patient received a cystoprostatectomy and an ileal conduit. This article describes a rare presentation of a rare tumor and a brief review of the literature.

A cross sectional single institution study of quality of life in adult patients with spina bifida.

OBJECTIVE: To describe and compare differences in perception of independence, urinary continence, and quality of life in an adult spina bifida (SB) population. METHODS: We collected data on adult neurogenic bladder patients which included demographics, relevant procedures, and quality of life (QoL) questionnaires. QoL and functional outcomes were assessed using spinal cord independence measure (SCIM) and SF-8 health questionnaire. International consultation of incontinence questionnaire (ICIQ) was used to assess incontinence. Comparisons were drawn between patients who underwent surgical reconstruction and those who did not. Student t-tests were used for comparisons and a P-value <0.05 was considered statistically significant. RESULTS: Fifty-four patients with SB were included. A total of 43% underwent bladder augmentation (BA) and 30% underwent antegrade continence enema (ACE). Patients with BA scored 49 ± 25 on the SCIM survey while those without had higher scores of 68 ± 19 with a P-value of 0.016. This difference remained evident when patients with ACE were excluded. When comparing ICIQ and SF-8, no statistically significant differences were found between those who underwent surgical procedures and those who did not. CONCLUSIONS: Assessing QoL in congenital NGB patients is a complex task. In our cohort, patients who underwent BA and ACE were shown to have decreased SCIM scores. SCIM scores for BA patients were significantly higher in patients who did not receive a BA independent of ACE status. SF-8 and ICIQ scores did not show any statistically significant difference in quality of life survey scores in those who underwent procedures versus those who did not.

Analysis of Factors Associated with Patient or Caregiver Regret following Surgery for Fecal Incontinence.

PURPOSE: Malone antegrade continence enema has been a successful and widely used procedure for achieving fecal continence in children. We present data on the previously uninvestigated issue of patient and caregiver regret following surgery for intractable constipation and fecal incontinence. MATERIALS AND METHODS: We reviewed all patients undergoing antegrade continence enema or cecostomy creation at a single institution between 2006 and 2016. Patients and caregivers were assessed for decisional regret using the Decisional Regret Scale. Results were correlated with demographics, surgical outcomes and complications. RESULTS: A total of 81 responses (49 caregivers and 32 patients) were obtained. Mean followup was 49 months. Decisional regret was noted in 43 subjects (53%), including mild regret in 38 (47%) and moderate to severe regret in 5 (6%). No statistical difference in regret was noted based on gender, complications or performance of concomitant procedures. On regression analysis incontinence was strongly associated with decisional regret (OR 4.4, 95% CI 1.1-18.1, p <0.001) and regret increased as age at surgery increased, particularly when patients were operated on at age 13 to 15 years (OR 2.6, 95% CI 1.0-6.4 for age 13 years; OR 2.9, 95% CI 1.1-7.8 for age 14 years; OR 3.1, 95% CI 1.1-8.8 for age 15 years). CONCLUSIONS: This is the first known study describing decisional regret following surgery for fecal incontinence. Surgical factors aimed at achieving continence may be effective in decreasing postoperative regret. The finding of increased regret in teenage patients compared to younger children should be shared with families since it may impact the age at which surgery is pursued.

A framework for transitioning patients from pediatric to adult health settings for patients with neurogenic bladder.

BACKGROUND: Adolescents with neurogenic bladder are a vulnerable population that severely lacks consistent transitional care from pediatric to adult urology settings. AIMS: Our practice determined that 100 patients with spina bifida and other neurogenic bladder conditions were not appropriately transferred to the adult setting once reaching adulthood. METHODS: We initiated a transitional program to establish a dedicated and formal process for adolescent patients to transition to adult urology. The REACH clinic implements a formalized staging framework to facilitate migration of adolescents and young adults to the adult health setting. A social worker was incorporated to act as a patient advocate, behavioral health consultant, and resource specialist. RESULTS: To date 45 patients have been enrolled in the transition program. We have identified and categorized according to the appropriate stage. The REACH clinic has appropriately outlined the goals and mission of the program and resources utilized are financially practical and feasible by conducting a monthly combined clinic. The program has been instrumental in improving tracking and monitoring of these patients through their transition period. CONCLUSION: Through the efforts of the pediatric and adult urology teams, the REACH program is a dedicated framework that provides structure for transition of the adolescent patient. The addition of a social worker has resulted in enriched rapport and will likely result in improved compliance. This program allows for surveillance and evaluation of patient outcome indicators in this patient population. We believe that early introduction and frequent encounters with the adult urologic team is crucial to successful transitions. Neurourol. Urodynam. 36:973-978, 2017. © 2016 Wiley Periodicals, Inc.

Contemporary Trends in the Inpatient Management of Fournier's Gangrene: Predictors of Length of Stay and Mortality Based on Population-based Sample.

OBJECTIVE: To describe clinical management of Fournier's gangrene and to characterize predictive factors associated with inpatient mortality and extended hospital stay. MATERIALS AND METHODS: The National Inpatient Sample was queried from 2004 to 2012 based on the International Classification of Diseases, Ninth Revision, Clinical Modification procedural and diagnosis codes. Patients admitted for Fournier's gangrene who underwent debridement were selected. Multivariate logistic regression analysis was performed to identify predictors of inpatient mortality and extended hospital stay. RESULTS: A total of 9249 patients were identified for a weighted estimate of 43,146 cases. Inpatient mortality was 4.7%. The median length of stay was 9 days (interquartile range 5-17 days). The most common procedure in addition to debridement was a complex closure (8.82%), followed by suprapubic tube placement (5.70%) and fecal diversion (4.68%). Increasing age, yearly hospital volume >10 cases, and Medicaid insurance status were associated with increased risk of mortality. Increasing age, teaching hospital status, increasing number of comorbidities, and Medicaid as a payor were predictive of increased hospital stay. Suprapubic tube placement (odds ratio [OR] 2.8 [95% confidence interval {CI} 1.92-4.07], P ≤ .001), fecal diversion (OR 11.1 [95% CI 6.20-19.7], P ≤ .001), and complex wound closure (OR 4.89 [95% CI 3.97-6.89], P ≤ .001) were also predictive of increased length of stay. CONCLUSION: Identifiable patient and hospital characteristics are predictive of both mortality and length of stay in the management of Fournier's gangrene. Overall inpatient mortality appears lower than what has been reported in the majority of prior reports. The strongest predictor for increased length of stay is the need for complex wound closure, and urinary or fecal diversion.

Transition from Pediatric to Adult Health Care in Patients with Chronic Illnesses: An Integrative Review.

A systematic review of the literature was performed to identify barriers, themes, or additional insight specific to the transitional care processes from a pediatric to an adult health care setting for patients with spina bifida.

Primary Yolk Sac Tumor of the Prostate: A Case Report and Review of the Literature.

Endodermal sinus (yolk sac) tumors (ESTs) are rare neoplasms that most commonly arise in the ovaries or testis. Only six cases of prostatic ESTs have been reported in the literature. We report a case of prostatic EST, the first case in which the patient had a history of previous testis cancer. Treatment included cisplatin-based chemotherapy and radical prostatectomy. Previous cases of primary ESTs and use of cisplatin-based therapy as well as metastatic tumors to the prostate are discussed.

Melanotic Xp11 translocation renal cancer: report of a case with a unique intratumoral sarcoid-like reaction.

BACKGROUND: Melanotic Xp11 translocation renal cancer is a rare tumor belonging to the family of microphthalmia-associated transcription factor (MiTF)/transcription factor E (TFE) neoplasms. This tumor family also includes alveolar soft part sarcoma, perivascular epithelioid cell neoplasms, Xp11 translocation renal cell carcinoma, and melanoma. To date, six confirmed melanotic Xp11 translocation cancers (five renal, one ovarian) have been reported in the literature. CASE REPORT: Here, we report the clinical, histologic, immunohistochemical, and molecular features of a unique melanotic Xp11 translocation renal cancer arising in a 34-year-old African-American female. Histologically, the tumor was composed of epithelioid tumor cells arranged in a nested pattern. The cells had clear to eosinophilic granular cytoplasm, vesicular nuclear chromatin, and prominent nucleoli. Multifocal intracytoplasmic deposits of granular brown melanin pigment were identified and confirmed by Fontana-Masson stain. An unusual histologic feature, not previously reported in melanotic Xp11 translocation renal cancer, was a sarcoid-like granulomatous reaction consisting of tight epithelioid granulomas with lymphocytic cuffing, numerous giant cells, and calcifications. Nuclear transcription factor E3 expression was identified by immunohistochemistry and TFE3 rearrangement was confirmed by fluorescence in situ hybridization. Additional immunohistochemical findings included immunoreactivity for HMB45, cathepsin K, and progesterone receptor; negative staining was seen with actin, desmin, cytokeratins, epithelial membrane antigen, CD10, vimentin, and PAX-8. The patient is currently free of disease, two years following initial clinicoradiologic presentation and twenty-two months following partial nephrectomy without additional therapy. CONCLUSION: This report further expands the spectrum of morphologic and clinical findings previously described in melanotic Xp11 translocation renal cancer, a distinctive tumor showing overlapping features between Xp11 translocation renal cell carcinoma, melanoma, and perivascular epithelioid cell neoplasms. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/7225796341180634.

Surgical management of urolithiasis in spinal cord injury patients.

Urolithiasis is a common condition in patients with spinal cord injury (SCI). Surgical management of stones in this population is more challenging and associated with lower clearance rates than the general population. The rate of complications - specifically infectious complications - is also high due to the chronic bacterial colonization. Shock wave lithotripsy (SWL) has a low clearance rate of 44-73 %. Percutaneous nephrolithotripsy is indicated for larger nephrolithiasis, but multiple procedures may be required to clear the stones. Ureteroscopy has been associated with low success rates because of difficulty in obtaining ureteral access. Historically, bladder stones were managed with open surgery or SWL. Recently, good results have been reported with the combination of endoscopic and laparoscopic techniques. Surgical management of urolithiasis in patients with SCI should be performed in high-volume centers in light of the technical challenges and higher rate of perioperative complications.

Chronic Cystitis Associated With Keratinizing Squamous Metaplasia Progressing to Squamous Papilloma.

We present a patient with extensive keratinizing squamous metaplasia that progressed to squamous papilloma over the course of follow-up. To our knowledge, this is the first published case of keratinizing squamous metaplasia with melanotic deposits of an unknown material with synchronous development of squamous papilloma.

Urethral injury by an orthopedic screw: an unusual cause of hematuria.

Pelvic fractures are commonly associated with urethral disruption; however, urethral injury from the repair of these fractures is rarely reported. Thus, the management of these injuries has not been well defined. We report a case of urethral injury from orthopedic screw insertion presenting 3 months postoperatively and effectively managed with removal of the screw and temporary Foley catheterization.

Muscle-invasive bladder cancer in a young adult: a case report and a review of the literature.

The peak incidence of bladder cancer (BC) is in the sixth decade of life. Muscle-invasive bladder cancer (MIBC) in young adults is extremely rare. We report a case of MIBC in a 28-year-old smoking male patient. The patient presented with hematuria and flank pain for which he underwent a computerized tomography (CT) scan of the abdomen and pelvis with and without contrast. The CT scan showed a 6 cm mass on the left side of the trigone extending to the left urteric orifice and left hydronephrosis, but no lymphadenopathy was noted. The patient then underwent a left nephrostomy tube placement followed by trans-urethral resection of bladder tumor (TURBT). The tumor involved both ureteric orifices and extended to the prostatic urethra. Complete resection was not feasible. Pathology showed high-grade T1 urothelial carcinoma. CT scan of the chest showed no distant lung metastasis. The patient then elected to undergo radical cystectomy with ileal conduit urinary diversion. Final pathology revealed T2a N0 urothelial carcinoma of the bladder. Our aim is to present our experience and review the literature for the natural history and oncological and quality of life outcomes of urothelial carcinoma of the bladder in young patients.

Renal abscess yields elusive hypermucoviscous phenotype of, uropathogenic Escherichia coli: a case report.

Uropathogenic E. coli (UPEC) strains are described as extraintestinal pathogenic E. coli with preference for the urinary tract. Bottone et al2 recently described the first documentation of a hypermucoviscous phenotype of a UPEC strain that displays a "stringing" phenomenon analogous to those produced by Klebsiella pneumonia strains known to invade the liver. The occurrence of this hypermucoviscous phenotype of UPEC strains causing urinary tract infection has not been well established. Following, we present a case report of two separate renal isolates from a patient with recurrent renal abscesses yielding the aforementioned hypermucoviscous phenotype of UPEC strains.

Plasmacytoid urothelial carcinoma of the bladder metastatic to the stomach: a case report.

Introduction. Plasmacytoid urothelial carcinoma (PUC) of the bladder is a rare histological variant of urothelial carcinoma that was recently identified. Available data on this histological variant is limited. Case Report. We report the case of a 75-year-old man with presumed history of high-grade urothelial cancer of the bladder, treated with transurethral resection and Bacille Calmette-Guérin (BCG) in 2004. Six years after treatment of the bladder cancer, the patient underwent gastrectomy for an undifferentiated carcinoma of the stomach. On followup, patient developed right ureterohydronephrosis and peritoneal carcinomatosis. Biopsy of the bladder during stent placement revealed a plasmacytoid urothelial carcinoma of the bladder. Rereadings revealed that the initial bladder and gastric malignancies were also plasmacytoid carcinoma, indicating that, the patient had since 2004, a PUC of the bladder that spread to the stomach and peritoneal cavity. Conclusion. Plasmacytoid urothelial carcinoma of the bladder is an aggressive variant of urothelial carcinoma. Based on our case and the literature review, this tumor can be misdiagnosed because of its rarity, leading to treatment delays. Both the urologist and the pathologist need to have a high index of suspicion for PUC whenever they encounter unusual clinical and/or pathological findings.

Percutaneous nephrolithotomy in spinal cord neuropathy patients: a single institution experience.

BACKGROUND AND PURPOSE: Patients with spinal neuropathy are at an increased risk for urolithiasis. Data on percutaneous nephrolithotomy (PCNL) in this population are limited. Our objective is to review our experience in managing stones with PCNL in patients with spinal neuropathy. PATIENTS AND METHODS: Twenty-one patients with spinal neuropathy underwent PCNL at our institution between January 2005 and August 2011. Their medical records were reviewed retrospectively to collect data relating to stone characteristics, treatment outcomes, and complications. RESULTS: Forty-two PCNL were performed on 26 kidneys. Five patients had bilateral stones. They were 14 (66.7%) patients with spinal cord injury, 5 (23.8%) with spina bifida, and 2 (9.5%) with other neurologic abnormalities. There were 90.5% of patients with preoperative bacteriuria and 47.6% with severe scoliosis, making positioning for PCNL challenging. Complete staghorn stones occurred in 46.2% of kidneys, and 50% of stones were struvite. Only 53.8% of kidneys were stone free after the first PCNL. The success rate increased to 80.8% after the second and 88.5% after the third PCNL. Urosepsis developed in three (14.3%) patients, necessitating admission to the intensive care unit postoperatively. Six (28.6%) patients needed blood transfusion. One patient had a pneumothorax and another had a perforation of the collecting system. CONCLUSIONS: Based on our experience, PCNL in patients with spinal neuropathy had a stone clearance rate comparable with that of the general population. These patients, however, needed multiple PCNLs to be stone free and had a higher incidence of complications (especially infectious).

Primary mixed neuroendocrine carcinoma of the bladder with large cell component: a case report and review of the literature.

Primary large cell neuroendocrine carcinomas (NECs) of the bladder are rarely encountered, and only a few reports have been documented. Frequently, they are found to be admixed with other histologies. In this report, we describe such a tumor found in a 65-year-old man who underwent radical cystectomy, after initial transurethral resection discovered a small cell NEC pathology. We also reviewed the limited number of neuroendocrine tumors reported containing a large cell component. Given the paucity of these tumors and the resultant difficulty in developing generalized treatment protocols, we promote the use of gene expression models to tailor chemotherapeutic regimens for individual tumors.

Multiple endocrine neoplasia 2a presenting with pheochromocytoma and pituitary macroadenoma.

Multiple Endocrine Neoplasia type 2A (MEN-2a) is a rare disease associated with tumors of endocrine organs. Presentation most commonly is with medullary thyroid cancer and infrequently with other complaints. Pituitary adenoma has been seen coincidentally with this disease very rarely. Presented is a case of coincident MEN-2a with a symptomatic pituitary adenoma and an asymptomatic pheochromocytoma. A brief review is also provided.