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.

Defining occupation-specific performance components for military selection and classification.

Differentiating performance components across military occupations is critical to developing assessments and other applications for matching people to occupations in the military. However, identifying occupation-specific performance components is challenging and resource intensive. The current paper summarizes different methods the services use to define and identify occupation-specific performance components. The paper concludes with recommendations and future directions for advancing the military's needs for information and data on occupation-specific performance components.

Development of a performance taxonomy for entry-level military occupations.

U.S. Military services use the Armed Services Vocational Aptitude Battery (ASVAB) and other indicators to select and classify about 150,000 enlisted personnel annually. The effectiveness of these assessments can be difficult to gauge due to differences in criteria across services and occupations. We developed a cross-service job performance model to be used in development of measures of first-term enlisted job performance. We synthesized job performance research, starting with the services' Job Performance Measurement (JPM) projects (1980s-1990s) through recent projects, to form performance dimensions. The dimension structure was evaluated in a retranslation exercise with performance measurement experts. The resulting hierarchical taxonomy has three levels: (a) 33 dimensions, (b) 11 categories, and (c) four broad categories. The broad performance categories are: (a) Technical, (b) Organizational Citizenship and Peer Leadership, (c) Psychosocial Well-Being, and (d) Physical. A sample of military experts judged the importance of the 33 performance dimensions. Results showed that the four broad performance categories were highly relevant to each service and generalizable across services. While all dimensions were judged important, Psychosocial Well-Being dimensions were rated as the most important by each service. The dimension structure provides a framework for criterion development and future joint-service research.

Identification and evaluation of criterion measurement methods.

Criterion measures vary greatly in terms of their psychometric quality and ease of use. This paper serves two purposes. First, it provides a general summary of different approaches to criterion measurement in a military context. Second, it provides an extensive review of 16 specific types of criterion measurement methods (e.g., job performance rating scales, self-report questionnaires, job knowledge tests) on nine psychometric and ease-of-use evaluation factors. Eight criterion measurement experts read a summary of extant research and made ratings to evaluate each measurement method on the evaluation factors. Rater intra-class correlations (ICCs) were high, ranging from .75 to .95 across the evaluation dimensions with a median of .91. Data showed a quality-feasibility tradeoff, where criterion data that are easy to obtain often have technical flaws. Recommendations for military services and future directions in criterion measurement (e.g., applications of machine learning) are discussed.

Development of cross-service training and job performance criterion measures for the U.S. Military.

As part of an effort to provide standardized criterion measures across the Armed Services, the current research effort developed a set of service-wide criterion measures for first-term enlisted personnel using a recently developed model of cross-service job performance. Two concurrent work streams developed criterion measures which provide complete construct coverage of the cross-service performance model. Using existing service-specific instruments, methods for development of the Cross-Service Situational judgment Test (CSSJT), the Cross-Service Job Performance Ratings Scales (CSPRS), and two cross-service self-report measures (end of training and in unit surveys) are described and discussed.

Indoleamine 2,3-dioxygenase-1, a Novel Therapeutic Target for Post-Vascular Injury Thrombosis in CKD.

BACKGROUND: CKD, characterized by retained uremic solutes, is a strong and independent risk factor for thrombosis after vascular procedures . Urem ic solutes such as indoxyl sulfate (IS) and kynurenine (Kyn) mediate prothrombotic effect through tissue factor (TF). IS and Kyn biogenesis depends on multiple enzymes, with therapeutic implications unexplored. We examined the role of indoleamine 2,3-dioxygenase-1 (IDO-1), a rate-limiting enzyme of kynurenine biogenesis, in CKD-associated thrombosis after vascular injury. METHODS: IDO-1 expression in mice and human vessels was examined. IDO-1-/- mice, IDO-1 inhibitors, an adenine-induced CKD, and carotid artery injury models were used. RESULTS: Both global IDO-1-/- CKD mice and IDO-1 inhibitor in wild-type CKD mice showed reduced blood Kyn levels, TF expression in their arteries, and thrombogenicity compared with respective controls. Several advanced IDO-1 inhibitors downregulated TF expression in primary human aortic vascular smooth muscle cells specifically in response to uremic serum. Further mechanistic probing of arteries from an IS-specific mouse model, and CKD mice, showed upregulation of IDO-1 protein, which was due to inhibition of its polyubiquitination and degradation by IS in vascular smooth muscle cells. In two cohorts of patients with advanced CKD, blood IDO-1 activity was significantly higher in sera of study participants who subsequently developed thrombosis after endovascular interventions or vascular surgery. CONCLUSION: Leveraging genetic and pharmacologic manipulation in experimental models and data from human studies implicate IS as an inducer of IDO-1 and a perpetuator of the thrombotic milieu and supports IDO-1 as an antithrombotic target in CKD.

The utility of magnetic resonance imaging for noninvasive evaluation of diabetic nephropathy.

BACKGROUND: Noninvasive quantitative measurement of fibrosis in chronic kidney disease (CKD) would be desirable diagnostically and therapeutically but standard radiologic imaging is too variable for clinical usage. By applying a vibratory force, tissue shear wave stiffness can be measured by magnetic resonance elastography (MRE) that may correlate with progression of kidney fibrosis. Since decreased kidney perfusion decreases tissue turgor and stiffness, we combined newly available three-dimensional MRE shear stiffness measurements with MR arterial spin labeling (ASL) kidney blood flow rates to evaluate fibrosis in diabetic nephropathy. METHODS: Thirty individuals with diabetes and Stage 0-5 CKD and 13 control individuals without CKD underwent noncontrast MRE with concurrent ASL blood flow measurements. RESULTS: MRE cortical shear stiffness at 90 Hz was decreased significantly below controls in all CKD stages of diabetic nephropathy. Likewise, ASL blood flow decreased progressively from 480 ± 136 mL/min/100 g of cortical tissue in controls to 302 ± 95, 229 ± 7 and 152 ± 32 mL/min/100 g in Stages 3, 4 and 5 CKD, respectively. A magnetic resonance imaging (MRI) surrogate for the measured glomerular filtration fraction [surrogate filtration fraction = estimated glomerular filtration rate (eGFR)/ASL] decreased progressively from 0.21 ± 0.07 in controls to 0.16 ± 0.04 in Stage 3 and 0.10 ± 0.02 in Stage 4-5 CKD. CONCLUSIONS: In this pilot study, MRI with ASL blood flow rates can noninvasively measure decreasing kidney cortical tissue perfusion and, with eGFR, a decreasing surrogate filtration fraction in worsening diabetic nephropathy that appears to correlate with increasing fibrosis. Differing from the liver, MRE shear stiffness surprisingly decreases with worsening CKD, likely related to decreased tissue turgor from lower blood flow rates.

Characterizing Response to Neoadjuvant Chemotherapy in Invasive Lobular Breast Carcinoma.

BACKGROUND: Pathological complete response (pCR) after neoadjuvant chemotherapy (NCT) for breast cancer is associated with improved survival and facilitates conservative surgical strategies. Invasive lobular carcinoma (ILC) has been observed to have decreased response to NCT compared with invasive ductal carcinoma (IDC). This study seeks to evaluate national trends in the use of NCT for ILC compared with IDC, and determine if there is a subset of ILC patients who demonstrate favorable response rates. METHODS: This is a study using the National Cancer Database. The cohort consisted of patients with stage 1-3 ILC treated between 2010 and 2014, and a reference cohort of patient with IDC. For patients receiving NCT, pCR was assessed and clinically relevant variables were used in multivariable logistic regression models for each histologic subtype, modeling for pCR achievement. Survival analysis was performed for each histologic group to evaluate potential survival benefits of achieving pCR. RESULTS: Our study cohort consisted of 384,887 women, of which 9.7% had ILC. A significantly lower rate of pCR after NCT was found in the cases of ILC compared with those of IDC (8.7% versus 23.2%). Increased response was seen in ILC patients with HER2-positive and TNBC subtypes. A survival benefit was demonstrated in patients with ILC who achieved pCR. CONCLUSIONS: While response to NCT in patients with ILC is uncommon, our findings demonstrate a selective benefit for patients with HER2-positive tumors and TNBC. In addition, pCR is correlated with a clear survival advantage in ILC.

Colonic Resection in an 8-Year-Old Girl with Intractable Functional Constipation and Diffuse Colonic Dysmotility and Failed Antegrade Flushes.

Optimal surgical management of patients with intractable constipation and diffuse colonic motility is not well defined. We present a patient with such a history, who ultimately achieved successful surgical management of constipation through a stepwise approach. An 8-year-old female presents with longstanding constipation and diffuse colonic dysmotility demonstrated with colonic manometry. She initially underwent sigmoid resection and cecostomy which failed and required diverting ileostomy. We initially proceeded with an extended resection, colonic derotation (Deloyers procedure), and neo-appendicostomy (neo-Malone) which resulted in successful spontaneous stooling for 1 year. Her constipation recurred and she subsequently underwent completion colectomy with ileorectal anastomosis given that she previously demonstrated ability to stool independently. Six months from surgery the patient continues to stool daily with assistance of fiber and loperamide. This case highlights a stepwise surgical approach to managing constipation due to diffuse colonic dysmotility and demonstrates that diffuse dysmotility may benefit from an upfront subtotal resection; however, it is crucial to assess a patient's ability to empty their rectum prior.

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.

Ultra-Rapid Droplet Digital PCR Enables Intraoperative Tumor Quantification.

The diagnosis and treatment of tumors often depends on molecular-genetic data. However, rapid and iterative access to molecular data is not currently feasible during surgery, complicating intraoperative diagnosis and precluding measurement of tumor cell burdens at surgical margins to guide resections. To address this gap, we developed Ultra-Rapid droplet digital PCR (UR-ddPCR), which can be completed in 15 minutes from tissue to result with an accuracy comparable to standard ddPCR. We demonstrate UR-ddPCR assays for the IDH1 R132H and BRAF V600E clonal mutations that are present in many low-grade gliomas and melanomas, respectively. We illustrate the clinical feasibility of UR-ddPCR by performing it intraoperatively for 13 glioma cases. We further combine UR-ddPCR measurements with UR-stimulated Raman histology intraoperatively to estimate tumor cell densities in addition to tumor cell percentages. We anticipate that UR-ddPCR, along with future refinements in assay instrumentation, will enable novel point-of-care diagnostics and the development of molecularly-guided surgeries that improve clinical outcomes.

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.

Functional constipation refractory to medical management: The colon is the problem.

BACKGROUND: Management of refractory constipation in children has not been standardized. We propose a protocolized approach which includes a contrast enema, anorectal manometry and exclusion of Hirschsprung disease (HD). For those without HD or with normal sphincters, an assessment of the colonic motility may be needed. The subgroups of dysmotility include (1) slow motility with contractions throughout, (2) segmental dysmotility (usually the sigmoid), or (3) a diffusely inert colon. We offered a Malone appendicostomy in all groups with the hope that this would avoid colonic resection in most cases. METHODS: Patients with medically refractory constipation were reviewed at a single institution (2020 to 2021). For patients without HD or an anal sphincter problem, assessment of colonic motility using colonic manometry was performed followed by a Malone appendicostomy for antegrade flushes. RESULTS: Of 196 patients evaluated for constipation refractory to medical management, 22 were felt to have a colonic motility cause. These patients underwent colonic manometry and Malone appendicostomy. 13 patients (59%) had a slow colon but with HAPCs throughout, 5 (23%) had segmental dysmotility, and 4 (18%) had a diffuse colonic dysmotility. 19 (86%) responded well to antegrade flushes with 17 reporting no soiling and 2 having occasional accidents. 3 patients (14%) failed flushes and underwent a colon resection within 6-month following Malone procedure. CONCLUSION: We propose a protocol for medically refractory constipation which provides a collaborative framework to standardize evaluation and management of these patients with antegrade flushes, which aids in avoidance of colonic resection in most cases. LEVEL OF EVIDENCE: Level IV.

Sparing the Perineal Body: A Modification of the Posterior Sagittal Anorectoplasty for Anorectal Malformations with Rectovestibular Fistulae.

BACKGROUND: The posterior sagittal anorectoplasty (PSARP) used to repair an anorectal malformation (ARM) with a rectovestibular fistula involves incising the perineal body skin and the sphincter muscles and a posterior sagittal incision to the coccyx. Perineal body dehiscence is the most common and morbid complication post-PSARP which can have a negative impact on future bowel control. With consideration of all the other approaches described to repair this anomaly, we developed a perineal body sparing modification of the standard PSARP technique. METHODS: Four patients with ARM with a rectovestibular fistula were repaired with a perineal body sparing modified PSARP at a single institution between 2020 and 2021. The incision used was limited, involving only the length of the anal sphincter, with no incision anterior or posterior to the planned anoplasty. Dissection of the distal rectum and fistula was performed without cutting the perineal body. Once the distal rectum was mobilized off the posterior vaginal wall and out of the vestibule, the perineal body muscles, where the fistula had been, were reinforced and an anoplasty was then performed. RESULTS: Operative time was the same as for a standard PSARP. There were no intraoperative or postoperative complications. No postoperative dilations were performed. All patients healed well with an excellent cosmetic result. All are too young to assess for bowel control. CONCLUSION: We present a new technique, a modification of the traditional PSARP for rectovestibular fistula, which spares the perineal body. This approach could eliminate the potential complication of perineal body dehiscence.

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.

Allowing Surgical Pathology Fellows to Release Preliminary Reports Increases Learner Independence and Satisfaction.

CONTEXT.­: Progressive independence in medicine is critical to building confidence and decisiveness in trainees. However, this can be difficult to accomplish in the strict regulatory environment of pathology. OBJECTIVE.­: To pilot and adopt a process whereby surgical pathology fellows independently manage a subset of cases and release preliminary reports. DESIGN.­: Upon program approval, board-certified surgical pathology fellows were eligible for preliminary report sign-out at their discretion. Eligible cases were sent from outside institutions for confirmatory review. Preliminary reports were viewable in the electronic medical record. Safety measures were used to ensure timely release of final reports by attending pathologists. RESULTS.­: Fellows participating in the pilot (n = 4) released 59 preliminary reports out of 101 cases reviewed (58%), with 1 potentially significant discrepancy between preliminary and final report. Turnaround time was not affected. The process was endorsed by all participants and adopted as standard practice. During the first year, eligible fellows (n = 8) released 123 preliminary reports out of 1260 cases reviewed (9.8%). There were no major diagnostic discrepancies and no effects on turnaround time. The number of preliminary reports released by each fellow was variable (range, 2-48; median, 8), likely a reflection of both external factors (number of trainees on service, volume) and trainee-specific factors (confidence, efficiency). CONCLUSIONS.­: Fellows showed good judgment when independently managing cases, with just 1 potentially significant discrepancy out of 182 cases (<1%). No patients were adversely impacted. Use of this process varied widely among fellows and may require closer monitoring and encouragement for fellows who are tentative about releasing preliminary reports.

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.

Reconstructing the anal sphincters to reverse iatrogenic overstretching following a pull-through for Hirschsprung disease. One-year outcomes.

BACKGROUND: In patients with Hirschsprung disease (HSCR), soiling may be related to anal sphincter damage following the initial pull-through. No optimal treatment has been developed for such patients, although enemas (rectal or antegrade) have been applied with some success. We present the one-year outcomes of a new technique for anal sphincter reconstruction. METHODS: All patients with HSCR referred from other institutions for post pull-through soiling were studied. Seven patients with patulous sphincters underwent sphincter reconstruction. Six had a full preoperative evaluation and were included in the study. Their 12-month outcomes were assessed. RESULTS: All six patients had soiling without voluntary bowel movements (VBMs). One patient was clean on Malone flushes when referred. Three underwent pre- and post-reconstruction non-sedated three-dimensional anorectal manometry, and objectively were able to close their sphincters following the reconstruction. All patients without Down syndrome (4 of 6) showed improvement in the abbreviated Baylor Continence Scale (4.5 vs. 0.75). One patient has achieved total bowel control without antegrade flushes, three now have VBMs which they did not have before but have occasional accidents and use antegrade flushes intermittently. They reported higher productivity, the ability to participate in sports and be away from home with confidence in their regimen. Two of 6 patients have Down syndrome and required a redo pull-through for other indications and underwent empiric sphincter reconstruction. For these two patients we do not have an outcomes assessment. CONCLUSIONS: A new technique for sphincter reconstruction shows promising results in improvement of bowel control at one year. LEVEL OF EVIDENCE: IV.

Effect of Hearing Aid Technology Level on New Hearing Aid Users.

BACKGROUND: Hearing aid manufacturers offer several different levels of technology that vary in sophistication and cost. It would stand to reason that higher technology hearing aids would provide greater benefit in the daily life of the user; however, research does not support this assumption for all users. Recent research in experienced hearing aid users indicated noise acceptance and satisfaction for speech in a large group were significantly improved when using the premium devices and suggested noise acceptance may be useful when comparing hearing aid technology levels. PURPOSE: The purpose of this study was to determine the effect of hearing aid technology level on listener outcome measures for new hearing aid users. RESEARCH DESIGN: A repeated measures, single-blinded research design was utilized. STUDY SAMPLE: Twenty-four adults with no previous hearing aid experience were recruited using flyers posted at the University of Tennessee Audiology Clinic and on social media (19 males, 5 females). The average age of the participants was 61 years (48-81 years). DATA COLLECTION AND ANALYSIS: Participants completed two, 4-week trial periods using Unitron T Moxi Fit FLEX:TRIAL devices programmed with basic or premium technology. Following each trial, laboratory evaluations were conducted using the QuickSIN, Hearing in Noise Test (HINT), and the Acceptable Noise Level (ANL). Subjective data was obtained using the Speech, Spatial, and Qualities of Hearing Short Form, satisfaction ratings, and preference. Data was analyzed using one-way repeated-measures analysis of variance. RESULTS: Speech perception results did not reveal significant differences between technology level on the HINT or QuickSIN; however, ANL was significantly improved for the premium devices. Subjective results revealed significantly improved satisfaction for speech in a large group and overall preference when using premium technology. CONCLUSION: Group data revealed similar outcomes between technology levels on most measures; however, noise acceptance, satisfaction for speech in a large group, and overall preference were significantly improved when using the premium devices. Individual characteristics such as noise acceptance, age, and degree of hearing loss may be useful when comparing hearing aid technology levels for new hearing aid users.

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.