Melanie Pennison

Prospective Measurement of Patient Exposure to Radiation During Pediatric Ureteroscopy

PURPOSE Few data have been reported regarding radiation exposure during pediatric endourological procedures, including ureteroscopy. We measured radiation exposure during pediatric ureteroscopy and identify opportunities for exposure reduction. MATERIALS AND METHODS We prospectively observed ureteroscopy procedures as part of a quality improvement initiative. Preoperative patient characteristics, operative factors, fluoroscopy settings and radiation exposure were recorded. Our outcomes were entrance skin dose and midline dose (both mGy). Specific modifiable factors were identified as targets for potential quality improvement. RESULTS Direct observation was performed in 54 consecutive ureteroscopy procedures. Mean±SD patient age was 14.8±3.8 years (range 7.4 to 19.2), with 9 children being younger than 12 years. Mean±SD entrance skin dose was 46.4±48 mGy. Mean±SD midline dose was 6.2±5.0 mGy. The most important major determinant of radiation dose was total fluoroscopy time (mean±SD 2.68±1.8 minutes) followed by dose rate setting, child anteroposterior diameter and source to skin distance (all p<0.01). Analysis of factors affecting exposure levels revealed that use of ureteral access sheaths (p=0.01) and retrograde pyelography (p=0.04) were significantly associated with fluoroscopy time. We also found that dose rate settings were higher than recommended in up to 43% of cases and ideal C-arm positioning could have reduced exposure by 14% (up to 49% in some cases). CONCLUSIONS Children receive biologically significant radiation doses during ureteroscopy procedures. Several modifiable factors contribute to dose and could be targeted in efforts to implement dose reduction strategies.

Prospective Systematic Intervention to Reduce Patient Exposure to Radiation During Pediatric Ureteroscopy

PURPOSE After prospective measurement of radiation exposure during pediatric ureteroscopy for urolithiasis, we identified targets for intervention. We sought to systematically reduce radiation exposure during pediatric ureteroscopy. MATERIALS AND METHODS We designed and implemented a pre-fluoroscopy quality checklist for patients undergoing ureteroscopy at our institution as part of a quality improvement initiative. Preoperative patient characteristics, operative factors, fluoroscopy settings and radiation exposure were recorded. Primary outcomes were the entrance skin dose in mGy and midline dose in mGy before and after checklist implementation. RESULTS We directly observed 32 consecutive ureteroscopy procedures using the safety checklist, of which 27 were done in pediatric patients who met study inclusion criteria. Outcomes were compared to those in 37 patients from the pre-checklist phase. Pre-checklist and postchecklist groups were similar in patient age, total operative time or patient thickness. The mean entrance skin dose and midline dose were decreased by 88% and 87%, respectively (p <0.01). Significant improvements were noted among the major radiation dose determinants, total fluoroscopy time (reduced by 67%), dose rate setting (appropriately reduced dose setting in 93% vs 51%) and excess skin-to-intensifier distance (reduced by 78%, each p <0.01). CONCLUSIONS After systematic evaluation of our practices and implementation of a fluoroscopy quality checklist, there were dramatic decreases in radiation doses to children during ureteroscopy.

Bladder Neck Reconstruction is Often Necessary After Complete Primary Repair of Exstrophy

PURPOSE A major goal of bladder exstrophy management is urinary continence, often using bladder neck reconstruction. We report our experience with bladder neck reconstruction after complete primary repair of exstrophy. MATERIALS AND METHODS Patient history, ultrasound, voiding cystourethrogram, examination using anesthesia and urodynamics were performed during a prospective evaluation. Continence was assessed using the International Childrens Continence Society classification and the dry interval. Bladder capacity was measured by examination using anesthesia, voiding cystourethrogram and/or urodynamics. Urodynamics were also done to assess bladder compliance and detrusor muscle function. RESULTS From 1994 to 2010 we treated 31 male and 15 female patients with bladder exstrophy after complete primary repair of exstrophy. Of patients 5 years old or older bladder neck reconstruction was performed after complete primary repair in 9 of 21 males (43%) and in 3 of 11 females (27%) at a mean age of 6.3 and 8.1 years, respectively. By the International Childrens Continence Society classification 6 of 12 patients (50%) were continent less than 1.5 years after bladder neck reconstruction and 2 of 9 (23%) were evaluable 1.5 years or greater after reconstruction. Median bladder capacity was 100 ml before, 50 ml less than 1.5 years after and 123 ml 1.5 years or greater after bladder neck reconstruction. Three males and 2 females emptied via an appendicovesicostomy. Two boys underwent augmentation. CONCLUSIONS In our experience most patients with bladder exstrophy require bladder neck reconstruction after complete primary repair of exstrophy. The need for reconstruction is more common in males. Our rates of bladder neck reconstruction after complete primary repair of exstrophy and of continence after bladder neck reconstruction are similar to those in other reports.

A Survey to Assess Body and Self-Image in Individuals with Bladder Exstrophy: A Call for Psychosocial Support

PURPOSE Although research in patients with urological conditions indicates that functional limitations, pain, limited mobility and social restrictions can impact self-image, to our knowledge the influence of the visibility of the condition has not been examined. We assessed body image and self-image at school age through young adulthood in patients with bladder exstrophy compared to patients with kidney stones. MATERIALS AND METHODS Patients diagnosed with bladder exstrophy and kidney stones completed a 25-item multiple choice Urological Body Image Questionnaire for ages 13 to 25 years, which was created for the current study. It consists of questions on physical appearance, avoidance behaviors, urological condition disclosure, romantic relationships and future expectations. A modified Urological Body Image Questionnaire for patients 8 to 12 years old was created by removing questions on relationships and future expectations. RESULTS A total of 24 patients with bladder exstrophy and 24 with kidney stones 8 to 25 years old completed the questionnaire. Compared to patients with kidney stones, patients with bladder exstrophy were significantly more likely to avoid changing clothes in front of peers and to not tell friends about the condition (each p = 0.0001). Patients with bladder exstrophy were also significantly more likely to think that the condition would prevent having future romantic relationships (p = 0.002) and children (p = 0.003). CONCLUSIONS Differences in the visibility and privacy of the 2 conditions likely explain the discrepancy between the groups. Findings may call for an individual approach to assessing issues related to self-esteem and identity in patients with bladder exstrophy and may highlight the importance of receiving psychosocial support.

Health Related Quality of Life in Patients with Bladder Exstrophy: A Call for Targeted Interventions

PURPOSE Research on health related quality of life in patients with bladder exstrophy has demonstrated mixed results compared to population samples. Few studies have had a comparison group with a urological disorder and none correlated body image to health related quality of life. We compared health related quality of life in patients with bladder exstrophy to that in patients with kidney stones and correlated body image to health related quality of life in the bladder exstrophy population. MATERIALS AND METHODS Participants included 24 patients with kidney stones and 24 with bladder exstrophy. Patient age was 8 to 25 years. Participants completed the PedsQL (Pediatric Quality of Life Inventory) and the 25-item multiple choice UBIQ (Urological Body Image Questionnaire), which was created for the current study. RESULTS Compared to patients with kidney stones those with bladder exstrophy indicated significantly higher scores on physical functioning (p = 0.0005) and overall health related quality of life (p = 0.02) in the 8 to 17-year-old cohort. When health related quality of life was compared to body image in patients with bladder exstrophy, lower social scores were associated with those who worried about friends finding out about the condition (p = 0.01) and about having romantic relationships (p = 0.003). Lower social (p = 0.006) and emotional (p = 0.009) functioning scores were associated with patients who thought that they were less likely to have romantic relationships. CONCLUSIONS The finding that patients with bladder exstrophy had a better physical functioning score than those with kidney stones is not surprising, given the morbidity of active stone disease. The negative correlations with body image in the bladder exstrophy population may be due to the possibility of others discovering the condition and the perceived likelihood of not having romantic relationships.

Sacral agenesis and neurogenic bladder: Long-term outcomes of bladder and kidney function

BACKGROUND Sacral agenesis (SA) is a rare congenital condition that refers to the absence of part or all of two or more lower sacral vertebral bodies. It can be associated with neurogenic bladder dysfunction that does not necessarily correlate with the level of spinal or skeletal defect. Patients with SA should undergo urodynamic studies (UDS) to guide lower urinary tract (LUT) management. OBJECTIVE This review aimed to update the present institutional experience since 1981 of this rare patient population with detailed, long-term follow-up of bladder and kidney function. STUDY DESIGN A single institution, retrospective, IRB-approved review was performed on patients born after January 1, 1981 with an isolated diagnosis of sacral agenesis without spina bifida, and followed with urologic involvement at Boston Childrens Hospital. Records were reviewed for demographics, radiologic imaging, UDS including cystometrogram (CMG) and electromyography (EMG), surgery, and blood chemistries. Comparisons were made between groups of patients based on age at diagnosis, with specific focus on renal function and stability of neurogenic bladder lesion. RESULTS Forty-three patients were identified: 23 female and 20 male. Thirty-seven children (86%) had a known age of diagnosis. Nineteen were diagnosed before 2 months old, including five who were diagnosed prenatally, 11 were diagnosed between 2 and 18 months, and seven were diagnosed after 18 months. All 43 had UDS, with 24 (55.8%) studied at the time of diagnosis (Summary Table). Twenty had serial full UDS, with 30% demonstrating neurourologic instability. None developed end-stage renal disease (ESRD) or required spinal cord detethering. DISCUSSION Many children with SA appeared to be diagnosed prenatally or early in life; SA was mostly identified during evaluation of associated anomalies. Though UDS aid in urologic management, testing was not routinely utilized at the time of diagnosis. CONCLUSIONS This review of long-term follow-up in SA patients showed stable LUT and renal function, with minimal risk of progression to ESRD.

Combined Cystometrography and Electromyography of the External Urethral Sphincter Following Complete Primary Repair of Bladder Exstrophy

PURPOSE Concern in patients with bladder exstrophy after reconstruction regarding potential injury to pelvic neurourological anatomy and a resultant functional deficit prompted combined (simultaneous) cystometrography and electromyography after complete primary repair of bladder exstrophy. We determined whether complete primary repair of bladder exstrophy would adversely affect the innervation controlling bladder and external urethral sphincter function. MATERIALS AND METHODS Complete primary repair of bladder exstrophy was performed via a modified Mitchell technique in newborns without osteotomy. Postoperative evaluation included combined cystometrography and needle electrode electromyography via the perineum, approximating the external urethral sphincter muscle complex. Electromyography was done to evaluate the external urethral sphincter response to sacral reflex stimulation and during voiding. RESULTS Nine boys and 4 girls underwent combined cystometrography/electromyography after complete primary repair of bladder exstrophy. Age at study and time after complete primary repair of bladder exstrophy was 3 months to 10 years (median 11.5 months). Cystometrography revealed absent detrusor overactivity and the presence of a sustained detrusor voiding contraction in all cases. Electromyography showed universally normal individual motor unit action potentials of biphasic pattern, amplitude and duration. The external urethral sphincter sacral reflex response was intact with a normal caliber with respect to Valsalva, Credé, bulbocavernosus and anocutaneous (bilateral) stimulation. Synergy was documented by abrupt silencing of external urethral sphincter electromyography activity during voiding. CONCLUSIONS After complete primary repair of bladder exstrophy combined cystometrography/electromyography in patients with bladder exstrophy showed normal neurourological findings, including sacral reflex responses, sustained detrusor voiding contraction and synergic voiding, in all patients postoperatively. These findings confirm the safety of complete primary repair of bladder exstrophy. Based on our results we have discontinued routine electromyography in these patients.

Racial Variation in Timing of Pyeloplasty: Prenatal Versus Postnatal Diagnosis

PURPOSE We have previously shown that nonwhite patients with ureteropelvic junction obstruction undergo pyeloplasty at a younger age than white patients. The mechanisms behind this finding are unclear, since there is no known racial variation in the natural history of ureteropelvic junction obstruction. We used a detailed clinical database to explain this phenomenon. MATERIALS AND METHODS We retrospectively reviewed all patients undergoing primary pyeloplasty at our institution between 1992 and 2008. More than 360 data points were abstracted for each patient, including self-reported race, socioeconomic status, symptom duration and presentation. RESULTS Of 847 patients undergoing pyeloplasty during the study period 741 met inclusion criteria. Nonwhite patients underwent surgery at a younger age (0.6 years) than whites (2.6 years, p <0.0001). When stratified by timing of clinical presentation (prenatal vs postnatal), there was no significant difference in race among patients presenting prenatally (0.37 vs 0.36 years, p = 0.22). Nonwhite patients presenting postnatally were significantly younger than white patients (6.3 vs 8.2 years, p = 0.03). This finding appeared to be due to differences in age at initial clinical presentation (5.4 vs 7.0 years, p = 0.03) and in time from initial clinical presentation to urological evaluation (0.6 vs 3.2 months, p = 0.03). These differences persisted after correcting for other factors, including markers of socioeconomic status. CONCLUSIONS Consistent with previous studies, we found that nonwhite patients underwent primary pyeloplasty at a younger age than whites. This difference is limited to patients presenting after birth. Prenatally diagnosed patients underwent surgery at similar ages regardless of race.

Anatomic findings associated with epispadias in boys: Implications for surgical management and urinary continence

INTRODUCTION Pre-operative physical examination of male epispadias allows for classification of epispadias level as glanular (GE), penile (PE) or penopubic (PPE), and for delineation of anatomic anomalies. The incidence of associated extragenital abnormalities, such as vesicoureteral reflux (VUR), bladder neck (BN) abnormality and abnormal pubic diastasis (PD), and their impact on urinary continence has not yet been systematically studied. OBJECTIVE The goal of this study was to evaluate whether the more proximal level of epispadias correlated with associated extragenital anatomic anomalies seen on initial imaging or endoscopic evaluation, and whether these pre-operative findings contributed to subsequent surgical management and impacted on achieving urinary continence. It was hypothesized that the more severe forms of epispadias may be associated with a higher frequency of associated anomalies. STUDY DESIGN The study was an IRB-approved, retrospective case study of all male patients treated initially for isolated epispadias at the current institution between 1994 and 2011. Data collection was achieved by chart and radiology review evaluating PD, BN appearance, presence of VUR, surgical treatment, and urinary continence. RESULTS A total of 26 patients were identified and divided into three groups based on appearance at physical examination: four glanular (GE), eight penile (PE), and 14 penopubic (PPE); 17 patients had an abnormal BN. Reflux was noted in nine of 20 patients who had a voiding cystourethrogram (VCUG), two of which had an episode of pyelonephritis. Of the 22 patients past the age of toilet training, 17 were continent (64% (9/14) penopubic, 63% (5/8) penile, and 75% (3/4) glanular). DISCUSSION Anatomic classification for male epispadias did not provide sufficient information regarding extragenital findings. This study provided new information regarding PD, BN appearance, presence of reflux, and ultimate urinary continence. Pubic diastasis and BN abnormalities were more frequently seen in more severe forms of epispadias, whereas VUR seemed more prevalent in less severe forms. A template for pre-operative evaluation was outlined. Limitations of the study were its retrospective design and relatively small cohort of patients, which reflected the rarity of the condition. CONCLUSION Based on the information generated, additional anatomic information was generated regarding boys with epispadias. This information will help guide the evaluation and the management of these patients in the future.