Rosemary Grant

Comparative assessment of pediatric testicular volume: orchidometer versus ultrasound.

PURPOSE Testicular volume measurements obtained with the Prader and Rochester orchidometers were compared to those obtained using scrotal ultrasound. The ability of each orchidometer versus ultrasound in detecting volume differential between 2 testes and the accuracy of orchidometer measurement by a less experienced examiner to that of a urologist were compared. MATERIALS AND METHODS A total of 65 males were examined by the attending urologist and urology nurse using the Prader and Rochester orchidometers, and scrotal ultrasound was subsequently performed by an attending radiologist. Statistical analysis of the results was performed to determine the correlation of orchidometer measurements between examiners, as well as with ultrasound, and sensitivity and specificity of orchidometer and ultrasound in detecting defined volume differentials between testes of 10%, 15%, 20% and 25%. RESULTS There was a strong linear relationship between testicular volume measurements using either orchidometer and ultrasound. To detect a defined volume differential as determined by ultrasound orchidometer sensitivity was weak, whereas orchidometer specificity was better. There was a strong correlation between orchidometer measurements of the urology nurse and attending urologist. CONCLUSIONS Although the orchidometer remains valuable in assessing size of the individual testis, it is too insensitive to volume differentials relative to ultrasound to be used routinely to determine growth impairment. For this reason observation of an adolescent with varicocele should include an annual ultrasound assessment of testicular volume.

The outcome of voiding dysfunction managed with clean intermittent catheterization in neurologically and anatomically normal children

Objective To describe the tolerability and efficacy of clean intermittent catheterization (CIC) in the management of dysfunctional voiding in patients who are neurologically and anatomically normal.

Prospective followup in patients after complete primary repair of bladder exstrophy.

PURPOSE The new technique of complete primary repair of bladder exstrophy has offered the promise of improved bladder functional outcomes and yet longitudinal followup in patients with exstrophy who have undergone this form of closure is sparse. We present our median 5-year data on patients who have undergone complete primary repair of bladder exstrophy. MATERIALS AND METHODS The records of patients who underwent complete primary repair of bladder exstrophy were analyzed retrospectively. Patients were followed prospectively using case report forms, radiological and clinical data, and semistructured parental and patient interviews. We reviewed parameters including clinical events, scarring on renal scan, vesicoureteral reflux, surgical procedures, urodynamic studies, urinary and fecal continence status, and episodes of urinary tract infection. RESULTS From 1994 to 2007 complete primary repair of bladder exstrophy was performed in 32 patients. Of these patients 28 underwent closure within 72 hours of life, including 20 males and 8 females, and 4 underwent it after 72 hours at ages 7 days to 12 months. One patient underwent complete primary repair of bladder exstrophy elsewhere. Median followup was 5 years (range 1 to 13). In 32 patients a total of 193 surgical procedures were done, of which 40% were minor endoscopic cases. Six of 23 males (26%) underwent bladder neck reconstruction at a median age of 4.5 years (range 4 to 10). Two boys and 1 girl received Deflux injection to the bladder neck at ages 3 to 5 years. To date no patient has undergone bladder augmentation. Nine patients (28%) had 1 to 4 episodes of pyelonephritis and 6 had cortical defects on renal scan. A total of 30 patients were voiding or incontinent via the urethra. One patient performed clean intermittent catheterization via the urethra and 1 performed it via appendicovesicostomy. All 4 children who were at least 6 months from bladder neck reconstruction after complete primary repair of bladder exstrophy had urinary continence periods of 2 to 3 hours or greater. Parents did not report any fecal incontinence or soiling in children older than 4 years. Relative to males the females had better urinary continence and a decreased need for bladder neck reconstruction. CONCLUSIONS Complete primary repair of bladder exstrophy has been shown to be safe and efficacious. Prospective followup in this small number of evaluable patients reveals that continence periods of more than 2 hours are possible in patients after bladder neck reconstruction. Long-term followup in patients after complete primary repair of bladder exstrophy continues to be necessary to establish the long-term effects of this procedure.

Stress and Coping of Parents of Young Children Diagnosed With Bladder Exstrophy

PURPOSE Previous studies have examined the psychological impact that living with bladder exstrophy has on patients. However, little is known about how parents of children diagnosed with this condition are affected. We examine how parents caring for children diagnosed with bladder exstrophy are impacted. An increased understanding of the stressors these parents face may lead to the development of appropriate parenting interventions, which may ultimately affect psychosocial and health outcomes in the child. MATERIALS AND METHODS All parents of children 10 years and younger treated for bladder exstrophy at our institution were selected from a centralized database. A total of 20 parents (65% of the eligible population) completed standardized questionnaires assessing pediatric specific parenting stress (Pediatric Inventory for Parents) and coping (Ways of Coping Questionnaire). RESULTS Parents identified several common stressors (eg worrying about the long-term impact of the illness, helping the child with his/her hygiene needs) and overall reported using adaptive ways of coping (ie planful problem solving, seeking social support, positive reappraisal). However, when they experienced increased stress they reported using more nonadaptive ways of coping (ie escape/avoidance and distancing). CONCLUSIONS Overall the findings of our study suggest that parents of children diagnosed with bladder exstrophy experience a significant amount of stress. In fact, parents in our study indicated experiencing similar frequency and difficulty of stress compared to parents of the same aged children diagnosed with type 1 diabetes. Increased stress can have negative consequences for parents and children. Future directions and implications of these findings are discussed.

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.