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1.
Pediatr Surg Int ; 39(1): 157, 2023 Mar 23.
Artículo en Inglés | MEDLINE | ID: mdl-36952009

RESUMEN

PURPOSE: Fecal incontinence is a problem for many patients born with an anorectal malformation (ARM) that can impact quality of life. It is unknown if racial, ethnic, and socioeconomic disparities relate to fecal continence in these children. We sought to examine outcomes and potential disparities in care. METHODS: We performed a multicenter retrospective study of children > 3y with ARM evaluated at sites participating in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC). The primary outcome was fecal continence. We evaluated for associations between fecal continence and race, sex, age, and insurance status. RESULTS: 509 patients with ARM from 11 institutions were included. Overall, 24% reported complete fecal continence, and fecal continence was associated with older age (p < .001). For school-aged children, 27% reported complete continence, while 53% reported none. On univariate analysis, patients with combined private and public insurance showed lower rates of continence when compared to those with private insurance (23 vs. 12%; p = 0.02). Age was associated with continence on univariate and multivariable analyses. CONCLUSION: Rates of complete fecal continence in this population are low. Differences based on payor status may exist. There were no observed disparities related to sex and race. Further investigation is warranted to improve care for this patient population. LEVEL OF EVIDENCE: III. TYPE OF STUDY: Multi-institutional retrospective comparative study.


Asunto(s)
Malformaciones Anorrectales , Cirugía Colorrectal , Incontinencia Fecal , Humanos , Niño , Malformaciones Anorrectales/cirugía , Malformaciones Anorrectales/complicaciones , Estudios Retrospectivos , Recto/cirugía , Calidad de Vida , Canal Anal/anomalías , Incontinencia Fecal/epidemiología , Incontinencia Fecal/complicaciones
2.
Cleft Palate Craniofac J ; 60(1): 122-128, 2023 01.
Artículo en Inglés | MEDLINE | ID: mdl-34755554

RESUMEN

INTRODUCTION: Transparent, patient-specific vocal tract replicas are helpful in research and educational endeavors but challenging to procure. An accessible method for fabricating these models, improving on previously suggested processes, would make them more widely available. METHOD: Detailed instructions for fabricating a transparent, patient-specific vocal tract model were addressed. The broad steps were (1) digitally reconstructing (patient-specific) vocal tract geometry, (2) producing a vocal tract mold (using methods such as three-dimensional [3D] printing), and (3) casting transparent material (such as silicone) around the vocal tract mold and removing the mold. The cavities remaining within the cast represented the exact geometry of the vocal tract. DISCUSSION: A combination of 3D printing and silicone casting can produce useful vocal tract replicas. Several simple changes to previous methods can improve consistency and reduce the labor and cost of production. Limitations and potential modifications to expand the applications of this method are discussed.

3.
J Pediatr ; 240: 122-128.e2, 2022 01.
Artículo en Inglés | MEDLINE | ID: mdl-34487770

RESUMEN

OBJECTIVE: To identify cumulative 5-year healthcare costs and healthcare days in children with anorectal malformation (ARM) and to compare the cumulative 5-year healthcare costs and healthcare days in children with ARM with 3 control cohorts: healthy, premature, and congenital heart disease (CHD). STUDY DESIGN: We performed a retrospective case-control study using the Truven MarketScan database of commercial claims encounters between 2008 and 2017. The ARM, CHD, and premature cohorts were identified using a targeted list of International Classification of Diseases 9th or 10th Revision diagnosis and Current Procedural Terminology codes. The healthy cohort included patients without ARM, preterm birth, or CHD. RESULTS: We identified 664 children with ARM, 3356 children with heart disease, 63 190 children who were born preterm, and 2947 healthy patients. At 5 years, the total healthcare costs of children with ARM ($273K, 95% CI $168K-$378K) were similar to the premature cohort ($246K, 95% CI $237K-$255K) and lower than the CHD cohort ($466K, 95% CI $401K-$530K, P < .001). Total healthcare days were similar in children with ARM (158 days, 95% CI 117-198) and prematurity (141 days, 95% CI 137-144) but lower than CHD (223 days, 95% CI 197-250, P = .02). In ARM, outpatient care (126 days, 95% CI 93-159) represented the largest contribution to total healthcare days. CONCLUSIONS: Children with ARM accumulate similar healthcare costs to children with prematurity and comparable healthcare days to children with CHD and prematurity in the first 5 years of life. Outpatient care represents the majority of healthcare days in children with ARM, identifying this as a target for quality improvement and demonstrating the long-term impact of this condition.


Asunto(s)
Malformaciones Anorrectales/economía , Costos de la Atención en Salud/estadística & datos numéricos , Malformaciones Anorrectales/epidemiología , Estudios de Casos y Controles , Preescolar , Humanos , Lactante , Recién Nacido , Recien Nacido Prematuro , Estudios Retrospectivos , Estados Unidos/epidemiología
4.
Cleft Palate Craniofac J ; 57(5): 637-645, 2020 05.
Artículo en Inglés | MEDLINE | ID: mdl-31867995

RESUMEN

OBJECTIVE: The loud and severely distorting form of audible nasal emission (commonly known as nasal turbulence or nasal rustle) typically occurs with a small velopharyngeal opening during production of pressure-sensitive consonants. The purpose of this study was to determine whether bubbling of the secretions, which commonly occurs on the superior aspect of the velopharyngeal port when there is a small opening, is a periodic process that can generate sound in the nasal cavity. PARTICIPANTS: Ten pediatric patients were included in the study. All participants had normal articulation and resonance but exhibited audible nasal emission characterized as nasal rustle. MEASURES: For each participant, high-speed video (HSV) nasopharyngoscopy and acoustic signals were recorded simultaneously. The acoustic recordings were captured in a manner similar to nasometry using nasal and oral microphones connected to a separation plate. Spectral analysis of the audio recordings and the HSV images was used to determine correlation between the acoustic and visual measurements. RESULTS: This study showed that secretion bubbling is a periodic process and its frequency, measured from the HSV data, was also captured by the acoustic measurements. The nasal acoustic signal correlated more strongly with the video of bubbling than the oral acoustic signal in the majority of the cases where bubbling occurred. CONCLUSION: These findings are strong evidence that secretion bubbling plays a significant role in the mechanism that generates undesired sound in the nasal cavity. Further work is needed to determine whether this sound is perceived as nasal rustle.


Asunto(s)
Insuficiencia Velofaríngea , Niño , Endoscopía , Humanos , Cavidad Nasal/diagnóstico por imagen , Nariz , Faringe
5.
J Pediatr Gastroenterol Nutr ; 69(3): 306-309, 2019 09.
Artículo en Inglés | MEDLINE | ID: mdl-31107797

RESUMEN

Hirschsprung disease, like most surgically correctable congenital anomalies, is now survivable well into adulthood. Patients are, therefore, presenting later in life with problems that were previously infrequently encountered by colorectal surgeons or gastroenterologists. The goals of our review are to describe the current state of care transition for this patient population, identify the specific needs for these patients, and propose guidelines which could inform a future model of transition from the pediatric to adult setting. This is a review of the current state of care transition and long-term outcomes for patients with Hirschsprung disease. Although these patients report a generally good quality of life, the majority suffer from some degree of lifelong gastrointestinal complication, regardless of the type of operative repair. A more formalized transition of care will provide a guide for pediatric surgeons and patients, alleviate colorectal surgeon and gastroenterologist concerns, and provide better long-term care for these patients.


Asunto(s)
Enfermedad de Hirschsprung/rehabilitación , Cuidados a Largo Plazo , Transición a la Atención de Adultos , Niño , Femenino , Humanos , Masculino , Adulto Joven
6.
J Acoust Soc Am ; 145(2): 775, 2019 02.
Artículo en Inglés | MEDLINE | ID: mdl-30823814

RESUMEN

Occupational speech users such as schoolteachers develop voice disorders at higher rates than the general population. Previous research has suggested that room acoustics may influence these trends. The research reported in this paper utilized varying acoustical conditions in a reverberant room to assess the effects on vocal parameters of healthy talkers. Thirty-two participants were recorded while completing a battery of speech tasks under eight room conditions. Vocal parameters were derived from the recordings and the statistically significant effects of room acoustics were verified using mixed-model analysis of variance tests. Changes in reverberation time (T20), early decay time (EDT), clarity index (C50), speech transmission index (STI), and room gain (GRG) all showed highly correlated effects on certain vocal parameters, including speaking level standard deviation, speaking rate, and the acoustic vocal quality index. As T20, EDT, and GRG increased, and as C50 and STI decreased, vocal parameters showed tendencies toward dysphonic phonation. Empirically derived equations are proposed that describe the relationships between select room-acoustic parameters and vocal parameters. This study provides an increased understanding of the impact of room acoustics on voice production, which could assist acousticians in improving room designs to help mitigate unhealthy vocal exertion and, by extension, voice problems.


Asunto(s)
Acústica , Acústica del Lenguaje , Voz/fisiología , Absorción de Radiación , Disfonía/fisiopatología , Femenino , Humanos , Masculino , Salud Laboral , Fonación/fisiología , Medición de la Producción del Habla , Calidad de la Voz/fisiología
7.
J Hum Genet ; 63(10): 1029-1034, 2018 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-29980720

RESUMEN

Familial recurrence of anorectal malformations (ARMs) has been reported in single institution case series and in two population-based studies. Here, we investigate the familial aggregation of ARMs using well-established, unbiased methods in a population genealogy of Utah. Study subjects included 255 ARM cases identified from among the two largest healthcare providers in Utah with linked genealogy data using International Classification of Diseases, Ninth Revision (ICD-9) diagnosis codes. The genealogical index of familiality (GIF) statistic, which compares the average pair-wise relatedness of cases to sets of matched controls, was used to test excess familial clustering. We also estimated relative risks (RRs) for ARM and associated phenotypes in relatives of cases adjusting for age-, sex-, and birthplace. Significant excess familial clustering was observed for all ARM subjects (GIF p < 1e-3). Significant RR estimates for ARM (RR = 15.6, p = 3.3e-6), and for almost all co-morbid birth defects previously associated with ARM, were observed among first-degree relatives of ARM case subjects. This genealogically-based population survey of familial aggregation of ARMs confirms the presence of a heritable component to ARMs and provides unbiased risk estimates to relatives of cases, which may have clinical utility.


Asunto(s)
Malformaciones Anorrectales/genética , Linaje , Malformaciones Anorrectales/epidemiología , Femenino , Humanos , Masculino , Factores de Riesgo , Utah/epidemiología
8.
Dis Colon Rectum ; 61(3): 390-399, 2018 03.
Artículo en Inglés | MEDLINE | ID: mdl-29420431

RESUMEN

BACKGROUND: Anorectal malformations are one of the most common congenital intestinal anomalies affecting newborns. Despite advances in neonatal care and surgical techniques, many patients with a history of anorectal malformations are affected by long-term challenges involving bowel and bladder dysfunction, sexual dysfunction, and psychosocial issues. These outcomes or challenges are additionally exacerbated by the lack of a structured transition of care from the pediatric to the adult setting. OBJECTIVE: The purpose of this review is to describe the long-term outcomes affecting patients with a history of anorectal malformations, review the current literature on transition of care, and make recommendations for developing a standardized program for transitioning care for a select group of colorectal surgical patients. DATA SOURCES: An extensive PubMed review of articles in English was performed to evaluate current best practices for chronic illnesses of childhood with residual symptoms or need for medical care into adulthood. STUDY SELECTION: Meta-Analysis of Observational Studies in Epidemiology group guidelines were followed. MAIN OUTCOME MEASURES: The primary outcome for this review was the existence of transitional services for patients with a history of anorectal malformations and evaluations of long-term outcomes affecting patients with a history of anorectal malformations. RESULTS: Systematic review revealed improved results in transition programs as determined by patient follow-up, medication adherence, and patient and family satisfaction through the use of multidisciplinary teams. Standardized tools for assessing all aspects of patient outcomes and quality of life are essential for describing the burden of disease affecting a transitioning population. LIMITATIONS: This is a retrospective review of the current status of a complex and rapidly evolving field of delivery of care. More work is needed to apply uniform approaches and assess the impact, patient outcomes, and quality of life. CONCLUSIONS: Patients who undergo childhood procedures for anorectal malformations often experience chronic symptoms related to the bowel, bladder, and reproductive organs, as well as psychosocial disturbances. This population will benefit from appropriate engagement in transitional care plans. See Video Abstract at http://links.lww.com/DCR/A543.


Asunto(s)
Canal Anal/cirugía , Malformaciones Anorrectales/terapia , Recto/cirugía , Transición a la Atención de Adultos , Femenino , Humanos , Masculino , Complicaciones Posoperatorias/terapia , Resultado del Tratamiento
9.
Ann Surg ; 262(1): 189-93, 2015 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-25185471

RESUMEN

OBJECTIVE: To determine whether charge awareness affects patient decisions. BACKGROUND: Pediatric uncomplicated appendicitis can be treated with open or laparoscopic techniques. These 2 operations are considered to have clinical equipoise. METHODS: In a prospective, randomized clinical trial, nonobese children admitted to a children's hospital with uncomplicated appendicitis were randomized to view 1 of 2 videos discussing open and laparoscopic appendectomy. Videos were identical except that only one presented the difference in surgical materials charges. Patients and parents then choose which operation they desired. Videos were available in English and Spanish. A postoperative survey was conducted to examine factors that influenced choice. The trial was registered at ClinicalTrials.gov (NCT 01738750). RESULTS: Of 275 consecutive cases, 100 met enrollment criteria. In the group exposed to charge data (n = 49), 63% chose open technique versus 35% not presented charge data (P = 0.005). Patients were 1.8 times more likely to choose the less expensive option when charge estimate was given (95% confidence interval, 1.17-2.75). The median total hospital charges were $1554 less for those who had open technique (P < 0.001) and $528 less for the group exposed to charge information (P = 0.033). Survey found that 90% of families valued having input in this decision and 31% of patients exposed to charge listed it as their primary reason for their choice in technique. CONCLUSIONS: Patients and parents tended to choose the less expensive but equally effective technique when given the opportunity. A discussion of treatment options, which includes charge information, may represent an unrealized opportunity to affect change in health care spending.


Asunto(s)
Apendicectomía/métodos , Apendicitis/cirugía , Precios de Hospital , Adolescente , Apendicectomía/economía , Apendicectomía/psicología , Apendicitis/economía , Niño , Preescolar , Conducta de Elección , Femenino , Humanos , Laparoscopía/economía , Laparoscopía/psicología , Masculino , Padres/psicología , Estudios Prospectivos
10.
Pediatr Radiol ; 45(5): 678-85, 2015 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-25416931

RESUMEN

BACKGROUND: The use of ultrasound to diagnose appendicitis in children is well-documented but not universally employed outside of pediatric academic centers, especially in the United States. Various obstacles make it difficult for institutions and radiologists to abandon a successful and accurate CT-based imaging protocol in favor of a US-based protocol. OBJECTIVE: To describe how we overcame barriers to implementing a US-based appendicitis protocol among a large group of nonacademic private-practice pediatric radiologists while maintaining diagnostic accuracy and decreasing medical costs. MATERIALS AND METHODS: A multidisciplinary team of physicians (pediatric surgery, pediatric emergency medicine and pediatric radiology) approved an imaging protocol using US as the primary modality to evaluate suspected appendicitis with CT for equivocal cases. The protocol addressed potential bias against US and accommodated for institutional limitations of radiologist and sonographer experience and availability. Radiologists coded US reports according to the probability of appendicitis. Radiology reports were compared with clinical outcomes to assess diagnostic accuracy. During the study period, physicians from each group were apprised of the interim US protocol accuracy results. Problematic cases were discussed openly. RESULTS: A total of 512 children were enrolled and underwent US for evaluation of appendicitis over a 30-month period. Diagnostic accuracy was comparable to published results for combined US/CT protocols. Comparing the first 12 months to the last 12 months of the study period, the proportion of children achieving an unequivocal US result increased from 30% (51/169) to 53% (149/282) and the proportion of children undergoing surgery based solely on US findings increased from 55% (23/42) to 84% (92/109). Overall, 63% (325/512) of patients in the protocol did not require a CT. Total patient costs were reduced by $30,182 annually. CONCLUSION: We overcame several barriers to implementing a US protocol. During the study period our ability to visualize the appendix with US increased and utilization of CT decreased. Our overall diagnostic accuracy with the US-based protocol was comparable to other published results and remained unchanged throughout the study.


Asunto(s)
Apendicitis/diagnóstico por imagen , Apendicitis/economía , Apéndice/diagnóstico por imagen , Niño , Análisis Costo-Beneficio , Costos de la Atención en Salud , Hospitales Pediátricos , Hospitales Urbanos , Humanos , Guías de Práctica Clínica como Asunto , Reproducibilidad de los Resultados , Estudios Retrospectivos , Sensibilidad y Especificidad , Ultrasonografía , Estados Unidos
11.
Children (Basel) ; 11(6)2024 May 28.
Artículo en Inglés | MEDLINE | ID: mdl-38929233

RESUMEN

BACKGROUND: Pediatric colorectal specialists care for patients with a variety of defecation disorders. Anorectal (AR) manometry testing is a valuable tool in the diagnosis and management of these children. This paper provides a summary of AR manometry techniques and applications as well as a review of AR manometry findings in pediatric patients with severe defecation disorders referred to a pediatric colorectal center. This is the first study describing multi-year experience using a portable AR manometry device in pediatric patients. METHODS: An electronic medical record review was performed (1/2018 to 12/2023) of pediatric patients with defecation disorders who had AR manometry testing. Demographics, diagnostic findings, and outcomes are described. KEY RESULTS: A total of 297 unique patients (56.9% male, n = 169) had AR manometry testing. Of these, 72% (n = 188) had dyssynergic defecation patterns, of which 67.6% (n = 127) had fecal soiling prior to treatment. Pelvic rehabilitation (PR) was administered to 35.4% (n = 105) of all patients. A total of 79.5% (n = 58) of the 73 patients that had fecal soiling at initial presentation and completed PR with physical therapy and a bowel management program were continent after therapy. AR manometry was well tolerated, with no major complications. CONCLUSIONS: AR manometry is a simple test that can help guide the management of pediatric colorectal surgical patients with defecation disorders. As a secondary finding, PR is a useful treatment for patients with dyssynergic stooling.

12.
J Pediatr Surg ; 59(7): 1369-1373, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38614946

RESUMEN

INTRODUCTION: Reducing soybean lipid emulsion (SLE) dose may prevent parenteral nutrition-associated cholestasis (PNAC) but effects on growth and neurodevelopment are unknown. The purpose of this study was to evaluate the effect of reduced dose SLE on growth and neurodevelopment. METHODS: Surgical neonates at 4 centers were randomized to standard SLE (3 g/kg/day) or reduced SLE (1 g/kg/day) over a 12-week period. Bilirubin levels and growth parameters were measured baseline and weekly while on study. The effects of time and group on direct bilirubin and growth were evaluated with a linear mixed effects model. Neurodevelopmental outcomes were assessed at 12- and 24-months corrected gestational age. RESULTS: Twenty-one individuals were randomized (standard dose = 9, reduced dose = 12). Subjects in the reduced dose group had slower rates of direct bilirubin increase and overall levels decreased earlier than those in the standard dose group. There was a trend toward a faster direct bilirubin decrease in the reduced dose group (p = 0.07 at day 84). There were no differences in the rates of change in weight (p = 0.352 at day 84) or height Z-scores (p = 0.11 at day 84) between groups. One subject in the reduced dose group had abnormal neurodevelopmental testing at 24 months. CONCLUSIONS: Surgical neonates randomized to a reduced dose of SLE had improved trends in direct bilirubin levels without clinically significant differences in overall growth and neurodevelopment. TYPE OF STUDY: Randomized Controlled Trial. LEVEL OF EVIDENCE: II.


Asunto(s)
Bilirrubina , Colestasis , Emulsiones Grasas Intravenosas , Nutrición Parenteral , Aceite de Soja , Humanos , Colestasis/etiología , Colestasis/prevención & control , Recién Nacido , Aceite de Soja/administración & dosificación , Aceite de Soja/uso terapéutico , Femenino , Emulsiones Grasas Intravenosas/administración & dosificación , Emulsiones Grasas Intravenosas/uso terapéutico , Nutrición Parenteral/efectos adversos , Nutrición Parenteral/métodos , Masculino , Bilirrubina/sangre , Lactante , Recien Nacido Prematuro , Relación Dosis-Respuesta a Droga
13.
J Pediatr Surg ; 59(2): 225-229, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-37968156

RESUMEN

BACKGROUND: Cloacal exstrophy (CE) represents a rare sub-group of anorectal malformations. Traditionally managed with a permanent colostomy, colonic pull-through (PT) has emerged to allow cleanliness without a life-long stoma. We sought to understand outcomes of PT in a large multi-center CE population. METHODS: We performed a retrospective study involving eleven pediatric hospitals. We gathered data on demographics, outcomes, and anatomical factors including colon length. Continuous variables were analyzed with Wilcoxon rank-sum tests and categorial variables with Fisher's exact tests. RESULTS: There were 98 patients, of which the majority (n = 70, 71.4 %) never underwent PT. There were no differences in exstrophy type, demographics, or associated anomalies. Median age at PT was 1.3 years (IQR 0.3-3.7). Of the cohort that continue to use their PT, the majority (n = 16, 69.6 %) are not clean. In total, 7.1 % (n = 7) of the cohort is clean with a PT, and only one patient is continent. Clean patients have a longer colon length than those who are not clean or opt for re-do ostomy (64.0 cm [IQR 46.0-82.0] vs 26.5 cm [IQR 11.6-41.2], p = 0.005). CONCLUSION: Overall, we demonstrate that most children born with CE will keep their stoma. Only a small percentage who elect to undergo colonic PT are clean for stool. Greater colon length correlates with success. This suggests that multiple factors, including colon length, are important when considering PT in a child with CE. LEVEL OF EVIDENCE: III.


Asunto(s)
Malformaciones Anorrectales , Extrofia de la Vejiga , Preescolar , Humanos , Lactante , Malformaciones Anorrectales/cirugía , Extrofia de la Vejiga/cirugía , Colon/cirugía , Colon/anomalías , Colostomía , Estudios Retrospectivos
14.
Eur J Pediatr Surg ; 2024 Mar 11.
Artículo en Inglés | MEDLINE | ID: mdl-37940124

RESUMEN

PURPOSE: The purpose of the study was to determine if antegrade continence enema (ACE) alone is an effective treatment for patients with severe functional constipation and segmental colonic dysmotility. METHODS: A retrospective study of patients with functional constipation and segmental colonic dysmotility who underwent ACE as their initial means of management. Data was collected from six participating sites in the Pediatric Colorectal and Pelvic Learning Consortium. Patients who had a colonic resection at the same time as an ACE or previously were excluded from analysis. Only patients who were 21 years old or younger and had at least 1-year follow-up after ACE were included. All patients had segmental colonic dysmotility documented by colonic manometry. Patient characteristics including preoperative colonic and anorectal manometry were summarized, and associations with colonic resection following ACE were evaluated using Fisher's exact test and Wilcoxon rank-sum test. p-Values of less than 0.05 were considered significant. Statistical analyses and summaries were performed using SAS version 9.4 (SAS Institute Inc., Cary, North Carolina, United States). RESULTS: A total of 104 patients from 6 institutions were included in the study with an even gender distribution (males n = 50, 48.1%) and a median age of 9.6 years (interquartile range 7.4, 12.8). At 1-year follow-up, 96 patients (92%) were successfully managed with ACE alone and 8 patients (7%) underwent subsequent colonic resection for persistent symptoms. Behavioral disorder, type of bowel management, and the need for botulinum toxin administered to the anal sphincters was not associated with the need for subsequent colonic resection. On anorectal manometry, lack of pelvic floor dyssynergia was significantly associated with the need for subsequent colonic resection; 3/8, 37.5% without pelvic dyssynergia versus 1/8, 12.5% (p = 0.023) with pelvic dyssynergia underwent subsequent colonic resection. CONCLUSION: In patients with severe functional constipation and documented segmental colonic dysmotility, ACE alone is an effective treatment modality at 1-year follow-up. Patients without pelvic floor dyssynergia on anorectal manometry are more likely to receive colonic resection after ACE. The vast majority of such patients can avoid a colonic resection.

15.
J Pediatr Surg ; 59(8): 1652-1656, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38735806

RESUMEN

PURPOSE: Congenital anorectal stenosis is managed by dilations or operative repair. Recent studies now propose use of dilations as the primary treatment modality to potentially defer or eliminate the need for surgical repair. We aim to characterize the management and outcomes of these patients via a multi-institutional review using the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry. METHODS: A retrospective database review was performed using the PCPLC registry. The patients were evaluated for demographics, co-morbidities, diagnostic work-up, surgical intervention, current bowel management, and complications. RESULTS: 64 patients with anal or rectal stenosis were identified (57 anal, 7 rectal) from a total of 14 hospital centers. 59.6% (anal) and 42.9% (rectal) were male. The median age was 3.2 (anal) and 1.9 years (rectal). 11 patients with anal stenosis also had Currarino Syndrome with 10 of the 11 patients diagnosed with a presacral mass compared to only one rectal stenosis with Currarino Syndrome and a presacral mass. 13 patients (22.8%, anal) and one (14.3%, rectal) underwent surgical correction. Nine patients (8 anal, 1 rectal) underwent PSARP. Other procedures performed were cutback anoplasty and anterior anorectoplasty. The median age at repair was 8.4 months (anal) and 10 days old (rectal). One patient had a wound complication in the anal stenosis group. Bowel management at last visit showed little differences between groups or treatment approach. CONCLUSION: The PCPLC registry demonstrated that these patients can often be managed successfully with dilations alone. PSARP is the most common surgical repair chosen for those who undergo surgical repair. LEVEL OF EVIDENCE: III.


Asunto(s)
Dilatación , Humanos , Masculino , Estudios Retrospectivos , Femenino , Lactante , Preescolar , Constricción Patológica/cirugía , Dilatación/métodos , Sistema de Registros , Malformaciones Anorrectales/cirugía , Malformaciones Anorrectales/complicaciones , Niño , Resultado del Tratamiento , Recto/anomalías , Recto/cirugía , Enfermedades del Recto/terapia , Enfermedades del Recto/cirugía , Enfermedades del Recto/congénito , Enfermedades del Recto/diagnóstico , Canal Anal/anomalías , Canal Anal/cirugía , Ano Imperforado/cirugía , Ano Imperforado/complicaciones , Ano Imperforado/diagnóstico
16.
J Pediatr Surg ; 59(7): 1250-1255, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38677965

RESUMEN

BACKGROUND: Hirschsprung Disease (HD) is a rare cause of functional bowel obstruction in children. Patients are typically diagnosed in the neonatal period and undergo pull-through (PT) soon after diagnosis. The optimal management and post-operative outcomes of children who present in a delayed fashion are unknown. METHODS: A multi-center retrospective review of children with HD was performed at participating Pediatric Colorectal and Pelvic Learning Consortium sites. Children were stratified by age at diagnosis (neonates <29 days; infants 29 days-12 months; toddler 1 year-5 years and child >5 years). RESULTS: 679 patients with HD from 14 sites were included; Most (69%) were diagnosed in the neonatal period. Age at diagnosis was not associated with differences in 30-day complication rates or need for PT revision. Older age at diagnosis was associated with a greater likelihood of undergoing fecal diversion after PT (neonate 10%, infant 12%, toddler 26%, child 28%, P < 0.001) and a greater need for intervention for constipation or incontinence postoperatively (neonate 56%, infant 62%, toddler 78%, child 69%, P < 0.001). CONCLUSION: Delayed diagnosis of HD does not impact 30-day post-operative outcomes or need for revision surgery but, delayed diagnosis is associated with increased need for fecal diversion after pull-through. LEVEL OF EVIDENCE: III.


Asunto(s)
Diagnóstico Tardío , Enfermedad de Hirschsprung , Complicaciones Posoperatorias , Humanos , Enfermedad de Hirschsprung/cirugía , Enfermedad de Hirschsprung/diagnóstico , Estudios Retrospectivos , Lactante , Diagnóstico Tardío/estadística & datos numéricos , Preescolar , Femenino , Masculino , Recién Nacido , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/diagnóstico , Resultado del Tratamiento , Niño , Reoperación/estadística & datos numéricos
17.
J Pediatr Surg ; 59(5): 997-1002, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38365475

RESUMEN

BACKGROUND: Anorectal malformations (ARM) are rare and heterogenous which creates a challenge in conducting research and offering recommendations for best practice. The Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) was formed in 2016 to address this challenge and created a shared national data registry to collect information about pediatric colorectal patients. There has been no external validation of the data collected. We sought to evaluate the database by performing a patient matched analysis comparing 30-day outcomes identified in the PCPLC registry with the NSQIP-P database for patients undergoing surgical repair of ARM. METHODS: Patients captured in the PCPLC database from 2016 to 2021 at institutions also participating in NSQIP-P who underwent ARM repair younger than 12 months old were reviewed for 30-day complications. These patients were matched to their NSQIP-P record using their hospital identification number, and records were compared for concordance in identified complications. RESULTS: A total of 591 patient records met inclusion criteria in the PCPLC database. Of these, 180 patients were also reviewed by NSQIP-P. One hundred and fifty-six patient records had no complications recorded. Twenty-four patient records had a complication listed in one or both databases. There was a 91 % concordance rate between databases. When excluding complications not tracked in the PCPLC registry, this agreement improved to 93 %. CONCLUSION: Including all patients evaluated for this subpopulation, a 91 % concordance rate was observed when comparing PCPLC collected complications to NSQIP-P. Future efforts can focus on further validating the data within the PCPLC for other patient populations. LEVEL OF EVIDENCE: V.


Asunto(s)
Malformaciones Anorrectales , Neoplasias Colorrectales , Humanos , Niño , Lactante , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Malformaciones Anorrectales/cirugía , Malformaciones Anorrectales/complicaciones , Sistema de Registros , Bases de Datos Factuales , Neoplasias Colorrectales/complicaciones , Mejoramiento de la Calidad , Estudios Retrospectivos
18.
J Pediatr Surg ; 59(8): 1638-1642, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38760309

RESUMEN

PURPOSE: Antegrade colonic enemas (ACE) can be an effective management option for defecation disorders and improve quality of life. Best practice regarding channel placement is unclear and variation may exist around preferred initial type of channel, age at placement, and underlying diagnoses. We aimed to describe practice patterns and patient characteristics around ACE channel placement. METHODS: We conducted a multicenter retrospective study of children with an ACE channel cared for at sites participating in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) from 2017 to 2022. Kruskal-Wallis test was utilized to test the age at surgery by site with significance level of 0.05. RESULTS: 500 patients with ACE channel were included. 293 (58.6%) patients had their ACE procedure at a PCPLC center. The median age at surgery was 7.6 [IQR 5.3-11.0] years for the overall cohort and 8.1 [IQR 5.3-11.5] years for placement at PCPLC centers. For PCPLC centers, median age at placement varied significantly across centers (p = 0.009). 371 (74.2%) patients received Malone appendicostomy, 116 (23.2%) received cecostomy, and 13 (2.6%) received Neo-Malone appendicostomy. Median age of patients by channel type was 7.7 [IQR 5.3-11.0], 7.5 [IQR 5.7-11.0], and 9.8 [IQR 4.2-11.6] years, respectively. The most common indication for cecostomy was idiopathic/refractory constipation (52.6%), whereas anorectal malformation was the most common indication for Malone (47.2%) and Neo-Malone (61.5%). Among ACE channels placed at PCPLC centers, there was variation across institutions in preferred initial channel type. The 4 highest volume centers favored Malone appendicostomy over cecostomy. CONCLUSION: There is variation in practice of ACE channel placement. At specialty pediatric colorectal centers, age at time of placement and type of channel placed varied across institutions. Further work is needed to better characterize diagnosis- and age-focused patient centered outcomes to clarify recommendations for our patients who benefit from these procedures. TYPE OF STUDY: Retrospective comparative study. LEVEL OF EVIDENCE: Level III.


Asunto(s)
Estreñimiento , Enema , Pautas de la Práctica en Medicina , Humanos , Estudios Retrospectivos , Enema/métodos , Niño , Femenino , Masculino , Preescolar , Estreñimiento/terapia , Pautas de la Práctica en Medicina/estadística & datos numéricos , Cecostomía/métodos
20.
J Voice ; 37(4): 515-521, 2023 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-33775469

RESUMEN

Wearing a face mask has been accepted as one of the most effective ways for slowing the spread of COVID-19. Yet information regarding the degree to which masks affect acoustics and perception associated with voice performers is scarce. This study examines these effects with common face masks, namely a neck gaiter, disposable surgical mask, and N95 mask, as well as a novel material that could be used as a mask (acoustic foam). A recorded excerpt from the "Star-Spangled Banner" was played through a miniature speaker placed inside the mouth of a masked manikin. Experienced listeners were asked to rate perceptual qualities of these singing stimuli by blindly comparing them with the same recording captured without a mask. Acoustic analysis showed that face masks affected the sound by enhancing or suppressing different frequency bands compared to no mask. Acoustic energy around the singer's formant was reduced when using surgical and N95 masks, which matches observations that these masks are more detrimental to the perceptions of singing voice compared with neck gaiter or acoustic foam. It suggests that singers can benefit from masks designed for minimal impact on auditory perception of the singing voice while maintaining reasonable efficacy of filtering efficiency.


Asunto(s)
COVID-19 , Canto , Humanos , Calidad de la Voz , Máscaras , Acústica
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