Sheila L. Ryan

Urologic Outcomes of Children With Hemorrhagic Cystitis After Bone Marrow Transplant at a Single Institution

OBJECTIVE To analyze clinical outcomes and the risk factors associated with genitourinary (GU) morbidity and mortality in children who present with hemorrhagic cystitis (HC) after bone marrow transplant (BMT). METHODS A retrospective chart review of patients with HC who had undergone BMT at a single pediatric hospital from 2008 to 2015 was conducted. Demographic data, severity of hematuria, HC management, and mortality were analyzed. Bivariate analysis and binary logistic regression were performed to identify risk factors. RESULTS Out of 43 patients who met inclusion criteria, 67.4% were male with a median age at BMT of 10.2 years (interquartile range 5.8-14.6). Percutaneous nephrostomy catheters were inserted in 5 patients for urinary diversion. All-cause mortality was 32.6% (N = 14). Intravesical retroviral therapy (P <.001), HC grade (P <.001), total Foley time (P <.001), total gross hematuria time (P <.001), total days hospitalized (P = .012), and days to most improved hematuria (P = .032) were associated with significant GU morbidity on bivariate analysis. On multivariable analysis, days to most improved hematuria was associated with significant GU morbidity odds ratio of 1.177 (1.006-1.376) (P = .042). Status of percutaneous nephrostomy was not associated with increased mortality (P = .472); however, in the multivariate model, BK viremia (P = .023), need for renal dialysis (P = .003), and presence of Foley catheter (P = .005) were associated with increased mortality. CONCLUSION Children with HC after BMT fall in a very high-risk category with high mortality and significant GU morbidity. The presence of a Foley catheter, need for dialysis, and BK viremia are associated with increased mortality.

Interhospital transfer of pediatric neurosurgical patients

OBJECTIVE The purpose of this study was to describe patterns of transfer, resource utilization, and clinical outcomes associated with the interhospital transfer of pediatric neurosurgical patients. METHODS All consecutive, prospectively collected requests for interhospital patient transfer to the pediatric neurosurgical service at Texas Childrens Hospital were retrospectively analyzed from October 2013 to September 2014. Demographic patient information, resource utilization, and outcomes were recorded and compared across predefined strata (low [< 5%], moderate [5%-30%], and high [> 30%]) of predicted probability of mortality using the Pediatric Risk of Mortality score. RESULTS Requests for pediatric neurosurgical care comprised 400 (3.7%) of a total of 10,833 calls. Of 400 transfer admissions, 96.5%, 2.8%, and 0.8% were in the low, moderate, and high mortality risk groups, respectively. The median age was 54 months, and 45% were female. The median transit time was 125 minutes. The majority of transfers were after-hours (69.8%); nearly a third occurred during the weekend (32.3%). The median intensive care unit stay for 103 patients was 3 days (range 1-269 days). Median length of hospital stay was 2 days (range 1-269 days). Ninety patients (22.5%) were discharged from the emergency room after transfer. Seventy-seven patients (19.3%) required neurosurgical intervention after transfer, with the majority requiring a cranial procedure (66.2%); 87.3% of patients were discharged home. CONCLUSIONS This study highlights patient characteristics, resource utilization, and outcomes among pediatric neurosurgical patients. Opportunities for quality improvement were identified in diagnosing and managing isolated skull fractures and neck pain after trauma.

Vesicoamniotic Shunting Improves Outcomes in a Subset of Prune Belly Syndrome Patients at a Single Tertiary Center

Objective: Review outcomes of Prune Belly Syndrome (PBS) with the hypothesis that contemporary management improves mortality. Methods: A retrospective chart review of inpatient and outpatient PBS patients referred between 2000 and 2018 was conducted to assess outcomes at our institution. Data collected included age at diagnosis, concomitant medical conditions, imaging, operative management, length of follow-up, and renal function. Results: Forty-five PBS patients presented during these 18 years. Prenatal diagnoses were made in 17 (39%); 65% of these patients underwent prenatal intervention. The remaining patients were diagnosed in the infant period (20, 44%) or after 1 year of age (8, 18%). Twelve patients died from cardiopulmonary complications in the neonatal period; the neonatal mortality rate was 27%. The mean follow-up among patients surviving the neonatal period was 84 months. Forty-two patients had at least one renal ultrasound (RUS); of the 30 patients with NICU RUSs, 26 (89%) had hydronephrosis and/or ureterectasis. Of the 39 patients who underwent voiding cystourethrogram (VCUG), 28 (62%) demonstrated VUR. Fifty-nine percent had respiratory distress. Nine patients (20%) were oxygen-dependent by completion of follow up. Thirty-eight patients (84%) had other congenital malformations including genitourinary (GU) 67%, gastrointestinal (GI) 52%, and cardiac 48%. Sixteen patients (36%) had chronic kidney disease (CKD) of at least stage 3; three patients (7%) had received renal transplants. Eighty-four percent of patients had at least one surgery (mean 3.4, range 0–6). The most common was orchiopexy (71%). The next most common surgeries were vesicostomy (39%), ureteral reimplants (32%), abdominoplasty (29%), nephrectomy (25%), and appendicovesicostomy (21%). After stratifying patients according to Woodard classification, a trend for 12% improvement in mortality after VAS was noted in the Woodard Classification 1 cohort. Conclusions: PBS patients frequently have multiple congenital anomalies. Pulmonary complications are prevalent in the neonate while CKD (36%) is prevalent during late childhood. The risk of CKD increased significantly with the presence of other congenital anomalies in our cohort. Mortality in childhood is most common in infancy and may be as low as 27%. Contemporary management of PBS, including prenatal interventions, reduced the neonatal mortality rate in a subset of our cohort.