Theresa Meyer

Robot-Assisted Laparoscopic Reoperative Repair for Failed Pyeloplasty in Children: A Safe and Highly Effective Treatment Option

PURPOSE Failed pyeloplasty represents a management dilemma, with treatment options including balloon dilation, endopyelotomy and reoperative pyeloplasty. We review our experience with robot-assisted laparoscopic reoperative repair of recurrent/persistent ureteropelvic junction obstruction in children and compare this method to other approaches. MATERIALS AND METHODS We reviewed in detail all cases of failed prior ureteropelvic junction procedures, either open or laparoscopic, managed by robot-assisted laparoscopic reoperative repair between 2006 and July 2011. RESULTS Robot-assisted laparoscopic repair was performed in 16 cases for persistent or recurrent ureteropelvic junction obstruction following a prior procedure involving the ureteropelvic junction (12 open pyeloplasties, 4 robot-assisted laparoscopic repairs). Additional interventions had been performed in 12 patients. Reoperative robot-assisted laparoscopic pyeloplasty was performed in 13 patients and reoperative robot-assisted laparoscopic ureterocalycostomy in 3. Patient age ranged from 12 months to 15.3 years (mean 6.1 years). Mean operative time and length of stay were 303 minutes and 1.6 days, respectively. Mean followup was 14.9 months. All symptomatic patients had resolution of symptoms postoperatively. A total of 14 patients (88%) had improved radiological findings. One patient underwent transfusion and conversion to an open procedure due to bleeding. CONCLUSIONS Robot-assisted laparoscopic reoperative repair of persistent/recurrent ureteropelvic junction obstruction is a safe, highly effective procedure even in the setting of multiple prior procedures. In our series all patients improved symptomatically, 88% improved radiographically and none have required further surgical intervention. Success is greater than with endopyelotomy and comparable to open reoperative repair for this challenging condition during short-term and intermediate followup.

A new computer enhanced visual learning method to train urology residents in pediatric orchiopexy: a prototype for Accreditation Council for Graduate Medical Education documentation.

PURPOSE Residency programs must continue to restructure teaching and assessment of surgical skills to improve the documentation of Accreditation Council for Graduate Medical Education competencies. To improve teaching and documenting resident performance we developed a computer enhanced visual learning method that includes a curriculum and administrative reports. The curriculum consists of 1) study of a step-by-step surgical tutorial of computer enhanced visuals that show specific surgical skills, 2) a checklist tool to objectively assess resident performance and 3) a log of postoperative feedback that is used to structure deliberate practice. All elements of the method are repeated with each case performed. We used the Accreditation Council for Graduate Medical Education index case of orchiopexy to pilot this project. MATERIALS AND METHODS All urology residents who trained at our institution from January 2006 to October 2007 performed orchiopexy using the computer enhanced visual learning method. The computer enhanced visual learning tutorial for orchiopexy consisted of customized computer visuals that demonstrate 11 steps or skills involved in routine inguinal orchiopexy, eg ligate hernia. The attending urologist rated resident competence with each skill using a 5-point Likert scale and provided specific feedback to the resident suggesting ways to improve performance. These ratings were weighted by case difficulty. The computer enhanced visual learning weighted score at entry into the clinical rotation was compared to the best performance during the rotation in each resident. RESULTS Seven attending surgeons and 24 urology residents (resident training postgraduate years 1 to 8) performed a total of 166 orchiopexies. Overall the residents at each postgraduate year performed an average of 7 cases each with complexity ratings that were not significantly different among postgraduate year groups (average 2.4, 1-way ANOVA p not significant). The 7 attending surgeons did not differ significantly in assessment of skill performance or case difficulty (1-way ANOVA p not significant). Of the 24 residents 23 (96%) showed improvement in computer enhanced visual learning score/skill performance. In the entire group the average computer enhanced visual learning weighted score increased more than 50% from entry to best performance (137 to 234 orchiopexy units, paired t test p <0.0001). CONCLUSIONS Computer enhanced visual learning is a novel method that enhances resident learning by breaking a core procedure into discrete steps and providing a platform for constructive feedback. Computer enhanced visual learning, which is a checklist tool, complies with Accreditation Council for Graduate Medical Education documentation requirements. Computer enhanced visual learning has wide applicability among surgical specialties.

Characterizing Early Psychosocial Functioning of Parents of Children with Moderate to Severe Genital Ambiguity due to Disorders of Sex Development

PURPOSE We examined the psychosocial characteristics of parents of children with disorders of sex development at early presentation to a disorders of sex development clinic. Parental anxiety, depression, quality of life, illness uncertainty and posttraumatic stress symptoms were assessed. Additionally we evaluated the relationship of assigned child gender to parental outcomes. MATERIALS AND METHODS A total of 51 parents of children with ambiguous or atypical genitalia were recruited from 7 centers specializing in treatment of disorders of sex development. At initial assessment no child had undergone genitoplasty. Parents completed the Cosmetic Appearance Rating Scale, Beck Anxiety Inventory, Beck Depression Inventory, SF-36, Parent Perception of Uncertainty Scale and Impact of Event Scale-Revised. RESULTS A large percentage of parents (54.5%) were dissatisfied with the genital appearance of their child, and a small but significant percentage reported symptoms of anxiety, depression, diminished quality of life, uncertainty and posttraumatic stress. Few gender differences emerged. CONCLUSIONS Although many parents function well, a subset experience significant psychological distress around the time of diagnosis of a disorder of sex development in their child. Early screening to assess the need for psychosocial interventions is warranted.

L Stent for Stomal Stenosis in Catheterizable Channels

PURPOSE Stomal stenosis in patients with catheterizable channels can be a difficult problem that is managed by surgical revision or dilation. The L stent is a short, knotted catheter that lies flush with skin. The stent is used for any stomal narrowing, typically overnight for several days. The stent bridges the area of stenosis without passing into bowel or bladder lumen. We assessed whether the L stent is effective for preventing and managing stomal stenosis. MATERIALS AND METHODS We retrospectively reviewed the records of patients with catheterizable channels. A telephone survey and chart review were done to identify patients who required an L stent and those with stomal stenosis. Patient satisfaction was evaluated with Likert scale questions. RESULTS We identified 50 patients with a total of 66 catheterizable urinary and enteric channels. Eight patients with a total of 11 (17%) affected stomas had stomal stenosis. Seven of 8 patients used the L stent for management and 100% reported improvement in stenosis. Six of 7 patients used the stent or catheterization with topical betamethasone cream. Four of 7 patients used the L stent greater than 6 months postoperatively and 3 reported that stenosis occurred immediately postoperatively. All patients who used the L stent reported intermittent self-directed stent use as a prophylactic measure to prevent recurrence. CONCLUSIONS Conservative management for stomal stenosis with an L stent is a simple, effective and well tolerated technique. This patient centered management significantly decreases the risk of surgical revision.

Predictors of urinary continence following tethered cord release in children with occult spinal dysraphism

OBJECTIVE Children with occult spinal dysraphism represent a wide spectrum of patients. Previous studies assessing urologic outcomes have in part been deficient due to the inability to appropriately categorize these patients and gather long-term follow-up data. In this study, a uniform set of patients that had occult spinal dysraphism with magnetic resonance imaging findings of a fatty filum terminale (FF) and/or low-lying cord (LLC) was identified. Utilizing long-term follow-up data, predictors for achieving urinary continence following tethered cord release (TCR) were determined. METHODS A retrospective chart review of pediatric patients with a diagnosis of tethered cord who underwent TCR from 1995 to 2005 was performed. Analysis was limited to patients who had primary TCR by one of two neurosurgeons within our multidisciplinary spina bifida clinic, who had greater than 1-year follow-up, and who were old enough to have continence status assessed (age > 6 years unless definitively toilet trained earlier). Patients with other associated forms of spinal dysraphism (lipomyelomeningeocele, spinal lipomas, sacral agenesis), anorectal malformations, and genitourinary anomalies were excluded. Pre- and post-TCR urodynamics, radiographic studies, functional orthopedic status, and urologic outcomes were assessed. Urodynamic results were categorized by three blinded urologists into one of three urodynamic patterns: (1) normal, (2) indeterminate, and (3) high risk. RESULTS A total of 147 patients with FF and/or LLC that underwent TCR were reviewed. 51 patients were excluded because of another associated spinal dysraphism (15/51 patients) or an anorectal/genitourinary anomaly (36/51 patients). Fifty-nine of the remaining 96 patients had adequate long-term follow-up data to be included in the study. 20 patients were asymptomatic at the time of TCR while 39 presented with orthopedic and/or urologic symptoms. The average age at surgery was 59.3 months (range 2-277 months) with an average follow-up of 7.0 years (range 1-16 years). At latest follow-up, 47 (80%) patients were continent while 12 (20%) were either incontinent or utilizing clean intermittent catheterization (CIC). Statistical analysis revealed that age of untethering, type of cutaneous lesion, level of conus, presence of hydronephrosis, and high-grade vesicoureteral reflux (VUR) were not independent predictors of continence. In patients with a cutaneous lesion who were asymptomatic, 19/20 obtained continence post-TCR (*p = 0.036). In patients who were old enough to assess continence pre-TCR, 14/25 patients were continent pre-TCR and 11/25 were incontinent. Of the 14 who were continent pre-TCR, all remained continent post-TCR (*p = 0.002). Of the 11 who were incontinent pre-TCR, five (45%) eventually became continent post-TCR. Assessment of urodynamic data revealed that neither pre- nor post-TCR urodynamics predicted continence status. CONCLUSION Isolated cutaneous lesions and preoperative continence status are positive predictors for post-TCR continence. While pre- and post-TCR urodynamics do not predict continence status, their utility in preoperative work-up, monitoring for retethering, and long-term urologic follow-up requires further examination.

Early administration of oxybutynin improves bladder function and clinical outcomes in newborns with posterior urethral valves.

PURPOSE Abnormal bladder function following posterior urethral valve ablation can lead to deleterious effects on renal function and urinary continence. We performed a pilot study to determine if bladder dysfunction could be ameliorated by the early administration of oxybutynin. MATERIALS AND METHODS We enrolled infants who underwent primary posterior urethral valve ablation by the age of 12 months. On initial urodynamics patients demonstrating high voiding pressures (greater than 60 cm H(2)O) and/or small bladder capacity (less than 70% expected) were started on oxybutynin. Urodynamics and ultrasound were performed every 6 months until completion of toilet training, at which time oxybutynin was discontinued. RESULTS Oxybutynin was started in 18 patients at a mean age of 3.4 months and was continued for a mean of 2.2 years. Urodynamics revealed that initial high voiding pressures improved from a mean of 148.5 to 49.9 cm H(2)O in 15 of 17 patients. All 8 patients with initially poor bladder compliance demonstrated improvement on oxybutynin. All 7 patients with initially low bladder capacity (mean 47.7% expected bladder capacity) demonstrated improvement while on oxybutynin (mean 216% expected bladder capacity). CONCLUSIONS This pilot study demonstrates that early use of anticholinergic therapy in infants with high voiding pressures and/or small bladder capacity after primary posterior urethral valve ablation has beneficial effects on bladder function.

Treatment of Occult Reflux Lowers the Incidence Rate of Pediatric Febrile Urinary Tract Infection

OBJECTIVES To examine whether vesicourethral reflux diagnosed by positioned instillation of contrast (PIC-VUR) shows clinical importance by comparing the incidence rates of febrile urinary tract infection (FUTI) before and after treatment of PIC-VUR. METHODS Beginning in 2001 we used a multi-institutional registry to prospectively enroll consecutive pediatric patients with a history of FUTI without VUR according to voiding cystourethrogram (VCUG) and yet who show PIC-VUR. Treatment of PIC-VUR was with prophylactic antimicrobials or antireflux surgery. The post-treatment occurrence of FUTI was tracked. RESULTS A total of 14 centers enrolled 118 patients (mean age, 7.2 years; range, 0.5 to 20 years). Parents self-selected the treatment of PIC-VUR as endoscopic injection (104), ureteral reimplantation (3), or antimicrobial prophylaxis (11). Study intervals surveying for FUTI before PIC (mean, 12 months; range, 1 to 17 years) and after PIC treatment (mean, 11 months; range, 0 to 3 years) were not significantly different. Overall the incidence rate for FUTI decreased significantly from 0.16 per patient per month before PIC-VUR treatment to 0.008 per patient per month after treatment (rate ratio 20; 95% confidence interval 11 to 36). The post-treatment rate of FUTI in patients treated with antibiotics versus surgery was not significantly different (rate ratio 2.5; 95% confidence interval 0.33 to 27). CONCLUSIONS The diagnosis of PIC-VUR is clinically important because children treated for PIC-VUR with either antimicrobial prophylaxis or surgery show a significant reduction in the incidence rate of FUTI. This is the basis for a current prospective study randomizing patients with PIC-VUR to treatment or observation.

Changes in levels of parental distress after their child with atypical genitalia undergoes genitoplasty

BACKGROUND The birth of a child with a disorder of sex development (DSD) and atypical genitalia can be traumatizing and isolating for families. Parents of children with DSD are at risk for increased levels of psychological distress, including depression, anxiety, illness uncertainty (IU), post-traumatic stress symptoms (PTSS), and impairments in quality of life (QOL). Our previous report indicated that although the majority of parents of children with atypical genitalia were coping well prior to the childs genitoplasty, approximately 25% of them reported experiencing some type of psychological distress. OBJECTIVE The current study sought to examine the trajectory of parental psychological distress prior to, and 6 months after their child underwent genitoplasty. METHODS Parents were recruited as part of an ongoing, prospective, multi-site study involving 10 pediatric hospitals with specialized care for children with atypical genitalia. Results from 49 parents (27 mothers, 22 fathers) of 28 children (17 female sex of rearing, 11 male sex of rearing) born with atypical genitalia (Prader rating of 3-5 in 46,XX DSD or by a Quigley rating of 3-6 in 46,XY DSD or 45,XO/46,XY) were included in the study. RESULTS There were no significant changes in level of depressive or anxious symptoms or quality of life between baseline and 6-month post-operative follow-up, although mothers continued to report significantly higher levels of depressive symptoms than fathers, and as a group, these parents reported lower QOL than published norms. The level of PTSS significantly decreased for all parents, suggesting that parents may have come through the acute stress phase associated with their childs diagnosis. Finally, while there were no significant changes in IU over the time period, the level of IU for parents of boys actually increased, while parents of girls reported no change (Figure). CONCLUSION Six months after their child has undergone genitoplasty, the majority of parents report minimal levels of psychological distress. However, a subset of these parents continue to experience significant distress related to their childs diagnosis. Specifically, parents of boys appear to be at increased risk for difficulties, which may be related to either the lack of clinical diagnosis for almost half of these children or the necessity of two-stage surgeries for the majority of them. We will continue collecting data on these families to better understand the trajectory of these adjustment variables.

Psychological Adjustment of Parents of Children Born with Atypical Genitalia 1 Year after Genitoplasty

Purpose: We examined the psychological adjustment of parents of children born with moderate to severe genital atypia 12 months after their child underwent genitoplasty. Materials and Methods: Parents were recruited longitudinally from a multicenter collaboration of 10 pediatric hospitals with specialty care for children with disorders/differences of sex development and/or congenital adrenal hyperplasia. Parents completed measures of depressive and anxious symptoms, illness uncertainty, quality of life, posttraumatic stress and decisional regret. Results: Compared to levels of distress at baseline (before genitoplasty) and 6 months after genitoplasty, data from 25 mothers and 20 fathers indicated significant improvements in all psychological distress variables. However, a subset of parents continued endorsing clinically relevant distress. Some level of decisional regret was endorsed by 28% of parents, although the specific decision that caused regret was not specified. Conclusions: Overall the majority of parents were coping well 1 year after their child underwent genitoplasty. Level of decisional regret was related to having a bachelors level of education, increased levels of illness uncertainty preoperatively and persistent illness uncertainty at 12 months after genitoplasty but was unrelated to postoperative complications.

Preliminary report: Surgical outcomes following genitoplasty in children with moderate to severe genital atypia

INTRODUCTION Prior studies of outcomes following genitoplasty have reported high rates of surgical complications among children with atypical genitalia. Few studies have prospectively assessed outcomes after contemporary surgical approaches. OBJECTIVE The current study reported the occurrence of early postoperative complications and of cosmetic outcomes (as rated by surgeons and parents) at 12 months following contemporary genitoplasty procedures in children born with atypical genitalia. STUDY DESIGN This 11-site, prospective study included children aged ≤2 years, with Prader 3-5 or Quigley 3-6 external genitalia, with no prior genitoplasty and non-urogenital malformations at the time of enrollment. Genital appearance was rated on a 4-point Likert scale. Paired t-tests evaluated differences in cosmesis ratings. RESULTS Out of 27 children, 10 were 46,XY patients with the following diagnoses: gonadal dysgenesis, PAIS or testosterone biosynthetic defect, severe hypospadias and microphallus, who were reared male. Sixteen 46,XX congenital adrenal hyperplasia patients were reared female and one child with sex chromosome mosaicism was reared male. Eleven children had masculinizing genitoplasty for penoscrotal or perineal hypospadias (one-stage, three; two-stage, eight). Among one-stage surgeries, one child had meatal stenosis (minor) and one developed both urinary retention (minor) and urethrocutaneous fistula (major) (Summary Figure). Among two-stage surgeries, three children developed a major complication: penoscrotal fistula, glans dehiscence or urethral dehiscence. Among 16 children who had feminizing genitoplasty, vaginoplasty was performed in all, clitoroplasty in nine, external genitoplasty in 13, urethroplasty in four, perineoplasty in five, and total urogenital sinus mobilization in two. Two children had minor complications: one had a UTI, and one had both a mucosal skin tag and vaginal mucosal polyp. Two additional children developed a major complication: vaginal stenosis. Cosmesis scores revealed sustained improvements from 6 months post-genitoplasty, as previously reported, with all scores reported as good or satisfied. DISCUSSION In these preliminary data from a multi-site, observational study, parents and surgeons were equally satisfied with the cosmetic outcomes 12 months after genitoplasty. A small number of patients had major complications in both feminizing and masculinizing surgeries; two-stage hypospadias repair had the most major complications. Long-term follow-up of patients at post-puberty will provide a better assessment of outcomes in this population. CONCLUSION In this cohort of children with moderate to severe atypical genitalia, preliminary data on both surgical and cosmetic outcomes were presented. Findings from this study, and from following these children in long-term studies, will help guide practitioners in their discussions with families about surgical management.