Getting to the bottom of why children with spina bifida use the emergency department: A qualitative analysis of parent and stakeholder perspectives.

BACKGROUND: Children with Spina Bifida (SB) have considerable healthcare utilization, including Emergency Department use (EDU). We aimed to elicit reasons for EDU using qualitative analysis of interviews with both patient-caregiver dyads and stakeholders. METHODS: A cohort of children with SB followed at our institution between 2016 and 2020 was identified and patient and clinical characteristics abstracted. Purposeful sampling by age and degree of past EDU was performed. Semi-structured interviews of dyads were performed using iteratively revised interview guides. Spanish-language interviews were conducted by a native Spanish speaker and transcripts professionally translated. Supplemental interviews with stakeholders, namely knowledgeable healthcare professionals, were also conducted. A qualitative framework approach was used for analysis, including open followed by closed independent coding with calculation of inter-rater reliability. A final interpretation of coding reports assessing convergence, divergence, and variation in themes across participant characteristics. RESULTS: 116 families (4 Spanish-speaking) and 7 stakeholders were interviewed. Sampling yielded a heterogenous cohort for EDU (56% with 0-10, 44% with >10 visits) and age (25% 0-4, 44% 5-11, 31% > 11 years). IRR was optimal (κ = 0.9). Themes in perceived reasons for EDU were 1) desire for "one-stop-shop" care, 2) an emergent medical problem, 3) providers' instructions, 4) negative past healthcare experience, 5) intrinsic caregiver moderators, and 6) temporospatial influences. Themes 1, 2, and 5 predominated in dyads, whereas themes 6, 3, and 5 were most common in stakeholders. Stakeholders focused largely on negative institutional and patient characteristics. Among dyads only, theme #1 was disproportionately emphasized by Spanish-speaking patients. DISCUSSION: Families desired access to coordinated expert care, testing and imaging. The ED offers this for children with SB, regardless of clinical acuity. This may be especially valued by families with inherent challenges to navigating the healthcare system. Negative experiences in community clinical settings, healthcare provider recommendations and intrinsic parental factors were themes that seemed to contribute to seeking this "one-stop-shop" type of care. Care coordination may reduce ED reliance, but themes for the interviews suggest a systems-based efforts should weave in the community care setting. CONCLUSIONS: For both stakeholders and caregivers, the ED represented a valued form of immediate access to multispecialty, expert care and testing in the context of perceived lack of timely, coordinated outpatient care. This may be moderated by intrinsic caregiver factors and negative past experiences. Although stakeholders discussed ideas that fit into patient-caregiver themes, the also uniquely focused on systems-based and patient-caregiver limitations.

Is Collaborative Care the Future of Medicine? Lessons Learned from the Care of Children with Colorectal Conditions.

The treatment of patients with colorectal disorders requires care from a wide variety of medical and surgical specialties over the course of their lifetime. This is ideally handled by a collaborative center which facilitates the assessment and development of patient care among multiple specialties which can enhance the quality and implementation of treatment plans, improve communication among different specialties, decrease morbidity, and improve patient satisfaction and outcomes. This collaborative approach can serve as a model for other parts of medicine requiring a similar multi-disciplinary and integrated method of care delivery. We describe the process, as well as the lessons learned in developing such a program.

Antibiotic Overtreatment of Presumed Urinary Tract Infection Among Children with Spina Bifida.

OBJECTIVE: To identify factors associated with overtreatment of presumed urinary tract infection (UTI) among children with spina bifida using such criteria. STUDY DESIGN: A retrospective review of children with spina bifida (age <21 years) evaluated in the Emergency Department (ED) at a single institution was performed. Patients with a urinalysis (UA) performed who were reliant on assisted bladder emptying were included. The primary outcome was overtreatment, defined as receiving antibiotics for presumed UTI but ultimately not meeting spina bifida UTI criteria (≥2 urologic symptoms plus pyuria and urine culture growing >100k CFU/mL). The primary exposure was whether the components of the criteria available at the time of the ED visit (≥2 urologic symptoms plus pyuria) were met when antibiotics were initiated. RESULTS: Among 236 ED encounters, overtreatment occurred in 80% of cases in which antibiotics were initiated (47% of the entire cohort). Pyuria with <2 urologic symptoms was the most important factor associated with overtreatment (OR 9.6). Non-Hispanic White race was associated with decreased odds of overtreatment (OR 0.3). CONCLUSIONS: Overtreatment of presumed UTI among patients with spina bifida was common. Pyuria, which is not specific to UTI in this population, was the main driver of overtreatment. Symptoms are a cornerstone of UTI diagnosis among children with spina bifida, should be collected in a standardized manner, and considered in a decision to treat.

How we select our surgical approach for cloacal reconstruction.

During cloacal reconstruction, we consider both common channel (CC) and urethral length when deciding between total urogenital mobilization (TUM) and a urogenital separation (UGS). Our concern about TUM in the case of a short urethra is resultant bladder neck incompetence, while conversion to UGS after completing the TUM dissection may result in urethral injury. Use of an interposition flap harvested from the ischiorectal fossa is another measure we use to prevent potential complications.

Posterior Sagittal Approach Provides Optimal Exposure for Urethral Reconstruction in Children With a History of Anorectal Malformations.

OBJECTIVE: To present a unique series of children with previously repaired anorectal malformations (ARM) with subsequent urethral pathology repaired via a posterior sagittal exposure and highlight the associated technical advantages. METHODS: Using a retrospective review of all procedures performed in our pediatric colorectal and pelvic reconstruction program from January 2020 through December 2022, we compiled a case series of patients with a history of ARM and prior posterior sagittal anorectoplasty (PSARP) who had urethral pathology and concurrent indication for redo-PSARP. Clinical features, operative details, and postoperative outcomes were collected. RESULTS: Six male patients presented at a median age of 4.3 years, all born with an ARM of recto-urinary fistula type, of which 3 were recto-prostatic, 1 recto-bladder-neck, and 2 unknown type. In addition to redo-PSARP, 2 underwent remnant of the original fistula excision and 4 had urethral stricture repair. One required post-operative Heineke-Mikulicz anoplasty. Patients underwent cystoscopy 4-6 weeks post-reconstruction, and none showed urethral stricture requiring treatment. Post-procedurally, 5 patients were able to void urethrally and 1 required additional bladder augmentation/Mitrofanoff. CONCLUSION: Redo-PSARP completely mobilizes the rectum, thereby providing excellent exposure to the posterior urethra for repair. This approach also allows the option of a rectal flap for augmented urethroplasty as well as harvest of an ischiorectal fat pad for interposition.

A prospective cohort study of assisted bladder emptying following primary cloacal repair: The Children's National experience.

INTRODUCTION/BACKGROUND: Although the combination of bladder dysfunction and upper tract anomalies puts patient with cloaca at risk for renal disease, the rarity of this condition makes it difficult to study empirically. As a high-volume center, we uniquely capture bladder function outcomes following our growing number of cloacal repairs. OBJECTIVE: 1) Describe the rates of incomplete bladder emptying following primary cloacal repair (at 2-3 months after repair and last follow up), and 2) identify clinical factors associated with assisted bladder emptying. STUDY DESIGN: We performed a prospective cohort study of patients undergoing primary cloaca repair by our Children's National Colorectal Center team between 2020 and 2021. The primary outcome was assisted bladder emptying at 2-3 months postoperatively and last visit. Covariables included preoperative characteristics (cloacagram measurements), ARM complexity (moderate = common channel [CC] <3-cm, severe = CC ≥ 3-cm), vesicoureteral reflux (VUR) status, sacral ratio (good ≥0.7, intermediate 0.7-0.4, poor ≤0.4), spinal cord status, means of preoperative bladder emptying, and operative details (age at repair, repair type, & concomitant laparotomy). RESULTS: Eighteen participants were eligible. A majority had moderate cloaca (78%), VUR (67%), spinal cord abnormalities (89%), and good sacral ratios (56%). Preoperatively, 10 patients were diapered for urine and 8 had assisted bladder emptying. Surgical repairs were performed at a median age of 8 months (range 4-46). Nine (50%) patients underwent urogenital separation (UGS), eight (44%) total urogenital mobilization, and 1 (6%) perineal sparing posterior sagittal anorectoplasty with introitoplasty. Exploratory laparotomy was performed in 7 (39%) patients. At 2-3 months, 7 patients were voiding and 11 required assisted bladder emptying. Median length of long-term follow up was 12 months (range 5-25), and 8 patients were voiding and 10 required assisted bladder emptying. Postoperative need for assisted bladder emptying was significantly associated with assisted bladder emptying preoperatively, a shorter urethra and increasing common channel length, UGS and exploratory laparotomy. Spinal cord imaging findings were not associated. DISCUSSION: Bladder emptying following cloaca repair is likely a result of congenital function and surgical effects. Indeed, increasingly cloaca complexity requiring UGS and laparotomy was associated with both pre- and post-operative assisted bladder emptying. The lack of association with spinal cord imaging may reflect a divergence between anatomy and function. CONCLUSION: Approximately half of patients required assisted bladder emptying in this study. Associated factors included urethral and common channel length, the need for assisted bladder emptying preoperatively, the type of surgical approach and additional laparotomy. Being diapered with seemingly normal voiding prior to surgery did not guarantee normal bladder function postoperatively.

Cloacal Malformation with Associated Urethral Atresia.

Introduction Cloacal malformations comprise a heterogeneous group of anomalies that are considered the most complex anorectal malformations (ARMs) in females. Precise evaluation to identify the unique anatomy prior to reconstruction with collaboration between colorectal surgeons, urologists, and gynecologists is vital. Here, we present a rare anatomical variation in a patient with a cloacal malformation which affected operative and postoperative management. Case description A 6-year-old female with cloaca who underwent colostomy, vaginostomy, and vesicostomy as a newborn presented for reconstruction. Her VACTERL workup was negative except for an atretic right kidney. Her ARM index included the cloaca, a normal spine, and sacrum with a lateral sacral ratio of 0.7, predicting good potential for bowel continence. Cystoscopy through the vesicostomy showed a small bladder with normal ureteral orifices, and a closed bladder neck, with no identifiable urethra. A cloacagram showed an atretic common channel, a single small vagina, and a rectum below the pubococcygeal line. The patient underwent a posterior sagittal anorectovaginourethroplasty, vaginal patch using rectum, rectoplasty, and perineal body reconstruction. The urethra was not amenable to reconstruction, so the vesicostomy was preserved and a future Mitrofanoff was planned. Conclusion Urethral atresia is a rare and challenging finding in cloaca patients, and a vesicostomy is needed to drain urine in the newborn period. Preoperative examination under anesthesia, cystoscopy, vaginoscopy, and cloacagram are crucial to identify the precise anatomy and to plan accordingly.

Where Is the Vagina? A Rectal Stricture after a Presumed Cloacal Repair Turns Out to be the Mobilized Vagina and a Missed High Rectovaginal Fistula.

We present a case of a rare complication in a 10-month-old female referred to our institution for an anal stricture after primary cloacal repair as an infant. Multimodal imaging, careful physical exam, and endoscopic evaluation revealed her vagina had been pulled through to the location of her anal sphincter muscle complex. We describe the correction of this problem, including identification of her rectum.

Fetal and Newborn Management of Cloacal Malformations.

Cloaca is a rare, complex malformation encompassing the genitourinary and anorectal tract of the female in which these tracts fail to separate in utero, resulting in a single perineal orifice. Prenatal sonography detects a few cases with findings such as renal and urinary tract malformations, intraluminal calcifications, dilated bowel, ambiguous genitalia, a cystic pelvic mass, or identification of other associated anomalies prompting further imaging. Multi-disciplinary collaboration between neonatology, pediatric surgery, urology, and gynecology is paramount to achieving safe outcomes. Perinatal evaluation and management may include treatment of cardiopulmonary and renal anomalies, administration of prophylactic antibiotics, ensuring egress of urine and evaluation of hydronephrosis, drainage of a hydrocolpos, and creation of a colostomy for stool diversion. Additional imaging of the spinal cord and sacrum are obtained to plan possible neurosurgical intervention as well as prognostication of future bladder and bowel control. Endoscopic evaluation and cloacagram, followed by primary reconstruction, are performed by a multidisciplinary team outside of the neonatal period. Long-term multidisciplinary follow-up is essential given the increased rates of renal disease, neuropathic bladder, tethered cord syndrome, and stooling issues. Patients and families will also require support through the functional and psychosocial changes in puberty, adolescence, and young adulthood.

Predicting the likelihood of prolongation of half-time among infants with initially indeterminate drainage values: A single-institution retrospective study of 535 patients with ureteropelvic junction obstruction.

INTRODUCTION AND OBJECTIVE: Prior studies have shown a broad half time (T1/2) interval on MAG3 diuresis renography (DR) that is indeterminate for obstruction. We aimed to refine and sub-divide the indeterminate range and associate it with clinically meaningful outcomes: pyeloplasty and pyeloplasty-free survival. METHODS: We identified patients <1.5 years-old at presentation with unilateral, isolated moderate to severe hydronephrosis who underwent DR from 2000 to 2016. A logistic regression model was created using T1/2 to predict surgery. An indeterminate range was defined based on patients with <90% probability of pyeloplasty or resolution. This group was sub-divided into three T1/2 intervals: 5-20, 21-40, and 41-60 min. Endpoints were pyeloplasty and pyeloplasty free survival. Indications for surgery were loss of differential renal function (DRF), worsening T1/2, family preference, and/or pain. RESULTS: Among 2025 patients with DR, 704 met criteria (169 were lost to follow up). Of the remaining 535, 218 had pyeloplasties and 317 did not. The Pyeloplasty group had significantly worse DRF, T1/2 at initial DR, and exited the study earlier, at a median age 1.1years vs 2.3 years (p < 0.001). For all patients with antenatally detected unilateral UPJ obstruction, the odds of undergoing pyeloplasty at any time increased by 1.8 times (p < 0.001 [95% CI: 1.04, 1.08]) per 10 unit increase in T1/2 until T1/2 = 60. However, in patients with intermediate drainage, five year surgery-free survival probability for patients with T1/2 5-20, 21-40, and 41-60 min were 79.7%, 46.7% and 33.3% respectively (χ2 = 41.2, P = <0.001). DISCUSSION: Previous efforts to define indeterminate drainage resulted in ranges for T1/2 that were too broad to be clinically useful. Within our endpoint-defined indeterminate range, our data show that there are significant step offs in 5-year surgery-free survival for patients with T1/2 < 20 min, 21-40 min, and 41-60 min. Although there is a steady decrease in surgery-free survival among patients with a T1/2 of 21-40 min over the first 5 years of life, half can be managed nonoperatively. These patients likely represent the true intermediate risk group and closer follow up is justified. CONCLUSIONS: Initial T1/2 on DR is predictive of future surgery. When drainage is "indeterminate" for obstruction, sub-stratification allows for more accurate prognostication.

Changes in Clinical Presentation and Renal Outcomes among Children with Febrile Urinary Tract Infection: 2005 vs 2015.

PURPOSE: Recent studies have demonstrated trends of decreasing voiding cystourethrogram utilization rates and delayed vesicoureteral reflux diagnosis in some children. It is possible that such delays could lead to more children sustaining repeated episodes of febrile urinary tract infection, and potential kidney injury, prior to diagnosis and treatment. MATERIALS AND METHODS: Using single institutional, cross-sectional cohorts of patients in 2 time periods (2005 and 2015), we compared clinical presentation and renal outcomes among patients 13 years and younger with history of febrile urinary tract infection presenting for initial voiding cystourethrogram. Outcomes included 1) recurrent urinary tract infection, 2) presence of vesicoureteral reflux, 3) grade of vesicoureteral reflux, and 4) renal scarring. Associations between year of presentation and outcomes of recurrent urinary tract infection and vesicoureteral reflux diagnosis were evaluated using multivariable logistic regression models. For the outcome of renal scarring, a logistic regression model was fitted for propensity score matched cohorts. RESULTS: Compared to children presenting in 2005, those in 2015 had 3 times the odds of recurrent urinary tract infection (OR 3.01, 95% CI 2.18-4.16, p <0.0001). Time period was not associated with the odds of vesicoureteral reflux (OR 0.98, 95% CI 0.77-1.23, p=0.85). Those in 2015 were more likely to present with vesicoureteral reflux grade >3 (OR 2.22, 95% CI 1.13-4.34, p=0.02) but not vesicoureteral reflux grade >2 (OR 1.11, 95% CI 0.74-1.67, p=0.60). Renal scarring was more common among children presenting in 2015 (OR 2.9, 95% CI 1.03-8.20, p=0.04). CONCLUSIONS: Compared to 2005, children presenting in 2015 for post-urinary tract infection voiding cystourethrogram have increased likelihood of recurrent urinary tract infection and renal scarring, despite similar likelihood of vesicoureteral reflux diagnosis.

The Use of Telemedicine for the Postoperative Urological Care of Children: Results of a Pilot Program.

PURPOSE: For postoperative visits, which are often brief interactions between family and clinician, patients may prefer the convenience of receiving postoperative care from home. We evaluated the feasibility of telemedicine for postoperative encounters in pediatric urology. MATERIALS AND METHODS: We performed a prospective telemedicine pilot study during an implementation period from November 10, 2017 to March 22, 2018. All postoperative patients deemed eligible by 1 of 4 urologists were offered enrollment in the telemedicine program. Enrollees underwent at least 1 virtual visit within 6 weeks of surgery. Technical difficulties and the number of unscheduled visits and readmissions were noted. After each virtual evaluation the family and clinician were prompted to complete a survey pertaining to perceptions of the telemedicine experience, including how effective the virtual visit was in delivering care. For each virtual visit with a urologist we estimated roundtrip travel cost and time. RESULTS: There was 96% technical success when using the software. A total of 125 postoperative virtual visits were completed in 83 patients. Median age of the children was 3.4 years and 87% were boys. Clinicians found that the virtual visit was "very effective" in 86% of cases, delivering the same care that they would have provided during a visit in person. Families were estimated to have saved a mean $150 travel cost and a median of 113 minutes of travel time per visit. No adverse postoperative outcomes were observed. CONCLUSIONS: This pilot study demonstrates that telemedicine can be successfully implemented in the postoperative care of pediatric urology patients.

Delayed Return of Ejaculatory Function in Adolescent Males Treated With Retroperitoneal Lymph Node Dissection and Adjuvant Therapy for Paratesticular Rhabdomyosarcoma.

Due to the rarity of the disease, adverse events related to ejaculatory function following the management of paratesticular rhabdomyosarcoma with multimodal therapy in adolescents are rarely discussed. Two patients, age 14 and 15 at time of diagnosis were treated with multimodal therapy with nerve-sparing retroperitoneal lymph node dissection, chemotherapy, and radiotherapy. Each developed ejaculatory dysfunction during the treatment period, which resolved 1 year after completion of all therapies. We sought to assess the role of each component of multimodal therapy on the observed side effect and the potential for delayed recovery of function after cessation of all therapies.

Has the robot caught up? National trends in utilization, perioperative outcomes, and cost for open, laparoscopic, and robotic pediatric pyeloplasty in the United States from 2003 to 2015.

INTRODUCTION: Since 2010, there have been few new data comparing perioperative outcomes and cost between open (OP) and robotic pyeloplasty (RP). In a post-adoption era, the value of RP may be converging with that of OP. OBJECTIVE: To 1) characterize national trends in pyeloplasty utilization through 2015, 2) compare adjusted outcomes and median costs between OP and RP, and 3) determine the primary cost drivers for each procedure. STUDY DESIGN: We performed a retrospective cohort study using the Premier database, which provides a nationally representative sample of U.S. hospitalizations between 2003 and 2015. ICD9 codes and itemized billing were used to abstract our cohorts. Trends in utilization and cost were calculated and then stratified by age. We used propensity scores to weight our cohorts and then applied regression models to measure differences in the probability of prolonged operative time (pOT), prolonged length of stay (pLOS), complications, and cost. RESULTS: During the study period 11,899 pyeloplasties were performed: 75% open, 10% laparoscopic, and 15% robotic. The total number of pyeloplasty cases decreased by 7% annually; OP decreased by a rate of 10% while RP grew by 29% annually. In 2015, RP accounted for 40% of cases. The largest growth in RPs was among children and adolescents. The average annual rate of change in cost for RP and OP was near stagnant: -0.5% for open and -0.2% for robotic. The summary table provides results from our regression analyses. RP conferred an increased likelihood of pOT, but a reduced likelihood of pLOS. The odds of complications were equivalent. RP was associated with a significantly higher median cost, but the absolute difference per case was $1060. DISCUSSION: Despite advantages in room and board costs for RP, we found that the cost of equipment and OR time continue to make it more expensive. Although the absolute difference may be nominal, we likely underestimate the true cost because we did not capture amortization, hidden or down-stream costs. In addition, we did not measure patient satisfaction and pain control, which may provide the non-monetary data needed for comparative value. CONCLUSION: Despite an overall decline in pyeloplasties, RP utilization continues to increase. There has been little change in cost over time, and RP remains more expensive because of equipment and OR costs. The robotic approach confers a reduced likelihood of pLOS, but an increased likelihood of pOT. Complication rates are low and similar in each cohort.

Collaborating with our adult colleagues: A case series of robotic surgery for suspicious and cancerous lesions in children and young adults performed in a free-standing children's hospital.

BACKGROUND: In adult urologic oncology the use of robotics has become commonplace; in pediatric urology it is rare. Herein, we describe a collaboration between an adult and a pediatric urologist performing robotic surgery for children and young adults with suspicious or cancerous genitourinary (GU) lesions. OBJECTIVES: To evaluate clinical and oncologic outcomes in children and young adults undergoing robotic surgery for suspicious or cancerous lesions of the GU tract; to describe our collaborative model between an adult and pediatric surgeon at a free-standing children's hospital. DESIGN: We retrospectively reviewed all robotic cases performed at our institution from 2014 to 2016 for patients with a GU malignancy or a suspicious mass. The surgeries were performed by a pediatric urologist with robotic experience and a fellowship-trained MIS adult urologist specializing in oncology. Perioperative and oncologic outcomes were recorded. RESULTS: A total of eight robotic cases were performed: four partial nephrectomies (PN) with retroperitoneal lymph node dissection (LND) (OT 269-338 min, EBL 5-300 mL, LOS 3-6 days), one adrenalectomy with LND (6.4 cm mass; OT 172 min, EBL 5 mL, LOS 3 days), one nephrectomy with pericaval LND (9.8 cm mass; 234 min, EBL 25 mL, LOS 3 days), and two retroperitoneal LNDs (OT 572 and 508 min, EBL 250 and 100, LOS 3 and 4 days). Patient weights ranged from 14 to 79 kg (mean 53.4 kg). There were no major complications (Clavien 3-5). Pathology results for PN included papillary RCC (AJCC pT1aNx) and two cases of segmental cystic renal dysplasia with nephrogenic rests. Bilateral template RPLNDs yielded paratesticular rhabdomyosarcoma (43 nodes; COG low risk group II stage I) and mixed non-seminomatous germ cell tumor (74 nodes; COG stage III). The nephrectomy yielded an undifferentiated sarcoma, low grade; the adrenalectomy favorable-type ganglioneuroma. DISCUSSION: In pediatrics, urologic oncology cases are often managed with open surgery. Our series demonstrates the feasibility of using the robotic approach in carefully selected cases. In doing so, the patient benefits from a minimally invasive surgery, while the surgeon benefits from robotic surgical dexterity. We seamlessly advanced these new techniques through a step-wise collaboration between an adult urologist who routinely performs robotic oncology procedures and a pediatric urologist experienced in robotics for benign conditions. CONCLUSION: In this small series, we safely and effectively adapted adult robotic techniques for genitourinary oncology cases in children and young adults.