RESUMO
INTRODUCTION: Prenatal ultrasounds often yield indeterminate (incomplete or minor abnormality) findings with limited clinical utility. We evaluate impact of indeterminate findings on maternal anxiety. METHODS: A single-U.S.-center prospective cohort study administered the Perinatal Anxiety Screening Scale (PASS; control mean = 13.4; > 20 denotes clinically significant anxiety) before and after prenatal ultrasounds in February-May 2017. Ultrasound reports were coded as: normal; indeterminate; or major abnormality. Primary outcome was anxiety after indeterminate vs. normal ultrasounds. Secondary outcomes included anxiety change from pre-to-post-ultrasound and relative to women's characteristics. Linear regression adjusted for confounders. RESULTS: Of 286 ultrasounds, 51.0% were normal, 40.5% indeterminate (22.0% incomplete; 18.5% minor abnormality), and 8.0% major abnormalities. Indeterminate findings were unrelated to age, race, parity, infertility, or psychiatric history, but associated with gestational age (26.6%/45.0%/52.5% for first/second/third trimesters; p < 0.001), and obesity (48.8 vs. 37.0%; p = 0.031). Pretest anxiety was highest in second/third trimesters (p = 0.029), and in subjects aged age ≤ 24 or younger(p < 0.001), with a history of anxiety (p < 0.001),) or with prior pregnancy loss (p = 0.011). Mean anxiety score decreased pre-to-posttest across all groups. Indeterminate findings were associated with higher PASS scores than normal findings: pretest 20.1 vs. 16.4 (p = 0.026) and posttest 16.9 vs. 12.2 (p = 0.009; adjusted-p = 0.01). Versus normal ultrasounds, incomplete findings were associated with higher post-ultrasound anxiety (p = 0.007; adjusted-p = 0.01) and smaller decreases from pre-to-posttest (adjusted-p = 0.03), whereas minor abnormalities had higher pretest anxiety (p = 0.029) with larger pre-to-posttest decreases (adjusted-p =0.010). DISCUSSION: Indeterminate ultrasounds, especially incomplete findings, are associated with significantly higher anxiety than normal findings, suggesting need for evidence-based counseling, management and strategies for decreasing number of indeterminate results.
Assuntos
Transtornos de Ansiedade , Ultrassonografia Pré-Natal , Idoso , Ansiedade/epidemiologia , Transtornos de Ansiedade/diagnóstico , Transtornos de Ansiedade/epidemiologia , Feminino , Humanos , Gravidez , Terceiro Trimestre da Gravidez , Estudos ProspectivosRESUMO
BACKGROUND: Blunt impact-induced traumatic abdominal wall hernia (TAWH) is an uncommon pediatric surgical problem classically associated with handlebar injury but increasingly seen with seatbelt use in motor vehicle collisions (MVC). Herein we describe the largest case series of pediatric TAWH to date and review the literature to establish the unique syndromic characteristics of MVC-associated TAWH. METHODS: In this single-institution series, we discuss four pediatric patients, all with seatbelt-associated TAWH after high-speed MVC characterized by full-thickness disruption of the lateral abdominal wall. We then performed a review of the literature to identify additional pediatric MVC-associated TAWH and define the characteristics of patients who sustained this unique injury. RESULTS: In addition to the four patients in our case series, five additional pediatric patients presenting with TAWH after restrained MVC were identified in the literature. Of these nine patients, eight (89%) presented with an obvious seatbelt sign (bruising/laceration to the abdominal wall). Six (67%) had associated injuries typical of the seatbelt syndrome, including four spinal flexion injuries (44%) and five bowel injuries requiring repair or resection (56%). Overall, 56% of seatbelt-associated TAWH occurred in children with a BMI percentile > 95%. CONCLUSIONS: In this case series and literature review, we note a high rate of seatbelt syndrome injuries in pediatric patients presenting with TAWH after restrained MVC. Suspicion for TAWH should be high in children presenting with a seatbelt sign and should trigger a low threshold for pursuing additional axial imaging. LEVEL OF EVIDENCE: Level IV; case series.
Assuntos
Hérnia Abdominal/etiologia , Hérnia Ventral/etiologia , Cintos de Segurança/efeitos adversos , Traumatismos Abdominais/cirurgia , Parede Abdominal/cirurgia , Acidentes de Trânsito , Criança , Pré-Escolar , Contusões , Feminino , Hérnia Ventral/cirurgia , Humanos , Masculino , Pediatria , Ferimentos não Penetrantes/etiologiaRESUMO
INTRODUCTION: Prenatal closure of open spina bifida via open fetal surgery improves neurologic outcomes for infants in selected pregnancies. Fetoscopic techniques that are minimally invasive to the uterus aim to provide equivalent fetal benefits while minimizing maternal morbidities, but the optimal technique is undetermined. We describe the development, evolution, and feasibility of the laparotomy-assisted 2-port fetoscopic technique for prenatal closure of fetal spina bifida in a newly established program. METHODS: We conducted a retrospective cohort study of women consented for laparotomy-assisted fetoscopic closure of isolated fetal spina bifida. Inclusion and exclusion criteria followed the Management of Myelomeningocele Study (MOMS). Team preparation involved observation at the originating center, protocol development, ancillary staff training, and surgical rehearsal using patient-matched models through simulation prior to program implementation. The primary outcome was the ability to complete the repair fetoscopically. Secondary maternal and fetal outcomes to assess performance of the technique were collected prospectively. RESULTS: Of 57 women screened, 19 (33%) consented for laparotomy-assisted 2-port fetoscopy between February 2017 and December 2019. Fetoscopic closure was completed in 84% (16/19) cases. Over time, the technique was modified from a single- to a multilayer closure. In utero hindbrain herniation improved in 86% (12/14) of undelivered patients at 6 weeks postoperatively. Spontaneous rupture of membranes occurred in 31% (5/16) of fetoscopic cases. For completed cases, median gestational age at birth was 37 (range 27-39.6) weeks and 50% (8/16) of women delivered at term. Vaginal birth was achieved in 56% (9/16) of patients. One newborn had a cerebrospinal fluid leak that required postnatal surgical repair. CONCLUSION: Implementation of a laparotomy-assisted 2-port fetoscopic spina bifida closure program through rigorous preparation and multispecialty team training may accelerate the learning curve and demonstrates favorable obstetric and perinatal outcomes.
Assuntos
Meningomielocele , Disrafismo Espinal , Feminino , Fetoscopia/efeitos adversos , Humanos , Lactente , Recém-Nascido , Laparotomia , Meningomielocele/cirurgia , Gravidez , Estudos Retrospectivos , Disrafismo Espinal/diagnóstico por imagem , Disrafismo Espinal/cirurgiaRESUMO
Early pregnancy renal anhydramios (EPRA) comprises congenital renal disease that results in fetal anhydramnios by 22 weeks of gestation. It occurs in over 1 in 2000 pregnancies and affects 1500 families in the US annually. EPRA was historically considered universally fatal due to associated pulmonary hypoplasia and neonatal respiratory failure. There are several etiologies of fetal renal failure that result in EPRA including bilateral renal agenesis, cystic kidney disease, and lower urinary tract obstruction. Appropriate sonographic evaluation is required to arrive at the appropriate urogenital diagnosis and to identify additional anomalies that allude to a specific genetic diagnosis. Genetic evaluation variably includes karyotype, microarray, targeted gene testing, panels, or whole exome sequencing depending on presentation. Patients receiving a fetal diagnosis of EPRA should be offered management options of pregnancy termination or perinatal palliative care, with the option of serial amnioinfusion therapy offered on a research basis. Preliminary data from case reports demonstrate an association between serial amnioinfusion therapy and short-term postnatal survival of EPRA, with excellent respiratory function in the neonatal period. A multicenter trial, the renal anhydramnios fetal therapy (RAFT) trial, is underway. We sought to review the initial diagnosis ultrasound findings, genetic etiologies, and current management options for EPRA.
Assuntos
Anormalidades Múltiplas/diagnóstico por imagem , Anormalidades Congênitas/diagnóstico por imagem , Doenças Renais Císticas/diagnóstico por imagem , Nefropatias/congênito , Rim/anormalidades , Pneumopatias/diagnóstico por imagem , Pulmão/anormalidades , Oligo-Hidrâmnio/diagnóstico por imagem , Obstrução Ureteral/diagnóstico por imagem , Obstrução Uretral/diagnóstico por imagem , Anormalidades Múltiplas/etiologia , Aborto Induzido , Líquido Amniótico , Ensaios Clínicos como Assunto , Feminino , Humanos , Infusões Parenterais , Rim/diagnóstico por imagem , Nefropatias/complicações , Nefropatias/diagnóstico por imagem , Doenças Renais Císticas/complicações , Pulmão/diagnóstico por imagem , Pneumopatias/etiologia , Oligo-Hidrâmnio/etiologia , Oligo-Hidrâmnio/terapia , Cuidados Paliativos , Gravidez , Insuficiência Renal , Ultrassonografia Pré-Natal , Obstrução Ureteral/complicações , Obstrução Uretral/complicaçõesRESUMO
OBJECTIVE: To evaluate delivery management and outcomes in fetuses prenatally diagnosed with CHD. STUDY DESIGN: A retrospective cohort study was conducted on 6194 fetuses (born between 2013 and 2016), comparing prenatally diagnosed with CHD (170) to those with non-cardiac (234) and no anomalies (5790). Primary outcomes included the incidence of preterm delivery and mode of delivery. RESULTS: Gestational age at delivery was significantly lower between the CHD and non-anomalous cohorts (38.6 and 39.1 weeks, respectively). Neonates with CHD had a significantly lower birth weights (p < 0.001). There was an approximately 1.5-fold increase in the rate of primary cesarean sections associated with prenatally diagnosed CHD with an odds ratio of 1.49 (95% CI 1.06-2.10). CONCLUSIONS: Our study provides additional evidence that the prenatal diagnosis of CHD is associated with a lower birth weight, preterm delivery, and with an increased risk of delivery by primary cesarean section.
Assuntos
Cesárea/estatística & dados numéricos , Cardiopatias Congênitas/epidemiologia , Recém-Nascido de Baixo Peso , Nascimento Prematuro/epidemiologia , Adulto , Bases de Dados Factuais , Feminino , Idade Gestacional , Humanos , Recém-Nascido , Masculino , Gravidez , Resultado da Gravidez , Diagnóstico Pré-Natal , Estudos Retrospectivos , Estados Unidos/epidemiologia , Adulto JovemRESUMO
BACKGROUND: Opioid misuse continues to be a major source of morbidity and mortality in the US, in both the adult and pediatric populations. Post-operative opioid prescriptions are often the first exposure children have to opioids and increases their risk of chronic use. There is significant variation in the number of opioids following identical procedures and measures have been taken within the adult population to limit this. However, specific post-operative opioid prescription guidelines are not present in the pediatric population. METHODS: Seven common pediatric surgery procedures were selected for inclusion. The recommended number of opioid doses following each procedure was determined by a multi-disciplinary expert panel. All surgery residents were sent an initial survey to determine the number of opioids they would prescribe for each procedure. They were then shown the guidelines and the survey repeated to determine changes in response. RESULTS: 35 and 27 general surgery residents took part in and pre- and post-educational surveys respectively. In all procedures, there was a decrease in the mean number of post-operative opioids prescribed. In addition, there was an increase in the number of residents who prescribed within the guidelines and a decrease in the number who overprescribed post-operative opioids. CONCLUSION: Pediatric postoperative opioid prescribing guidelines derived from expert opinion increase resident compliance with appropriate dosing; this has the potential to decrease the classic problem of general surgery residents accustomed to treating adults overprescribing opioids to children. These results are promising, and we aim to expand on this work and incorporate these guidelines into our clinical practice. LEVEL OF EVIDENCE: III/IV.
Assuntos
Analgésicos Opioides/uso terapêutico , Prescrições de Medicamentos/estatística & dados numéricos , Dor Pós-Operatória/tratamento farmacológico , Cuidados Pós-Operatórios/normas , Guias de Prática Clínica como Assunto , Padrões de Prática Médica/estatística & dados numéricos , Adolescente , Feminino , Humanos , Masculino , Inquéritos e QuestionáriosRESUMO
BACKGROUND: The "double bubble" sign is an ultrasonographic finding that commonly represents duodenal atresia and is associated with trisomy 21. OBJECTIVES: We sought to evaluate the positive predictive value of a prenatally identified double bubble sign for duodenal atresia and the genetic etiologies associated with it. METHODS: We examined a retrospective cohort with prenatal double bubble sign between January 1, 2008, and June 30, 2017. Postnatal diagnoses were determined by review of operative reports and additional postnatal evaluation including cytogenetic analysis, molecular analysis, and/or clinical genetic evaluation. RESULTS: All live births at our institution with a prenatal double bubble sign had confirmed duodenal atresia. Additional anatomic anomalies and/or genetic abnormalities were identified in 62% of cases. Out of 21 cases, 6 had trisomy 21. Of the remaining 15 cases, 8 were nonisolated duodenal atresia, 3 of which had a heterotaxy syndrome. In the 7 isolated cases, 1 likely pathogenic chromosomal microdeletion was identified. CONCLUSIONS: Prenatal double bubble sign is a reliable predictor of duodenal atresia. In addition to trisomy 21, heterotaxy may be encountered. ZIC3 mutations as well as microdeletion of 4q22.3 may be underlying genetic etiologies to be considered in the diagnostic evaluation of a prenatal double bubble sign.
Assuntos
Obstrução Duodenal/diagnóstico por imagem , Atresia Intestinal/diagnóstico por imagem , Ultrassonografia Pré-Natal , Deleção Cromossômica , Cromossomos Humanos Par 4 , Síndrome de Down/genética , Obstrução Duodenal/genética , Predisposição Genética para Doença , Idade Gestacional , Síndrome de Heterotaxia/genética , Humanos , Atresia Intestinal/genética , Valor Preditivo dos Testes , Estudos Retrospectivos , Fatores de RiscoRESUMO
Anhydramnios caused by early anuria is thought to be universally fatal due to pulmonary hypoplasia. Bilateral renal agenesis and early fetal renal failure leading to anhydramnios constitute early pregnancy renal anhydramnios (EPRA). There have been successful reports of amnioinfusions to promote lung growth in the setting of EPRA. Some of these successfully treated EPRA fetuses have survived the neonatal period, undergone successful dialysis, and subsequently received a kidney transplant. Conversely, there are no reports of untreated EPRA survivors. This early success of amnioinfusions to treat EPRA justifies a rigorous prospective trial. The objective of this study is to provide a review of what is known about fetal therapy for EPRA and describe the Renal Anhydramnios Fetal Therapy trial. We review the epidemiology, pathophysiology, and genetics of EPRA. Furthermore, we have performed systematic review of case reports of treated EPRA. We describe the ethical framework, logistical challenges, and rationale for the current single center (NCT03101891) and planned multicenter trial.
Assuntos
Anuria/complicações , Terapias Fetais/métodos , Nefropatias/terapia , Rim/anormalidades , Anuria/epidemiologia , Anuria/terapia , Feminino , Doenças Fetais/terapia , Terapias Fetais/ética , Humanos , Rim/embriologia , Nefropatias/epidemiologia , Nefropatias/genética , Pulmão/embriologia , GravidezRESUMO
PURPOSE: Enhanced recovery after surgery (ERAS) pathways have been used for two decades to improve perioperative recovery in adults. Nevertheless, little is known about their effectiveness in children. The purpose of this review was to consider pediatric ERAS pathways, review the literature concerned with their potential benefit, and compare them with adult ERAS pathways. SOURCE: A PubMed literature search was performed for articles that included the terms enhanced recovery and/or fast track in the pediatric perioperative period. Pediatric patients included those from the neonatal period through teenagers and/or youths. PRINCIPAL FINDINGS: The literature search revealed a paucity of articles about pediatric ERAS. This lack of academic investigation is likely due in part to the delayed acceptance of ERAS in the pediatric surgical arena. Several pediatric studies examined individual components of adult-based ERAS pathways, but the overall study of a comprehensive multidisciplinary ERAS protocol in pediatric patients is lacking. CONCLUSION: Although adult ERAS pathways have been successful at reducing patient morbidity, the translation, creation, and utility of instituting pediatric ERAS pathways have yet to be realized.
Assuntos
Assistência Perioperatória/métodos , Complicações Pós-Operatórias/prevenção & controle , Analgesia , Anestesia , Criança , Hidratação , Humanos , Avaliação de Resultados da Assistência ao Paciente , Assistência Perioperatória/educação , Recuperação de Função Fisiológica , Infecção da Ferida Cirúrgica/prevenção & controleRESUMO
Coordinated arterial-venous differentiation is crucial for vascular development and function. The origin of the cardinal vein (CV) in mammals is unknown, while conflicting theories have been reported in chick and zebrafish. Here, we provide the first molecular characterization of endothelial cells (ECs) expressing venous molecular markers, or venous-fated ECs, within the emergent dorsal aorta (DA). These ECs, expressing the venous molecular markers Coup-TFII and EphB4, cohabited the early DA with ECs expressing the arterial molecular markers ephrin B2, Notch and connexin 40. These mixed ECs in the early DA expressed either the arterial or venous molecular marker, but rarely both. Subsequently, the DA exhibited uniform arterial markers. Real-time imaging of mouse embryos revealed EC movement from the DA to the CV during the stage when venous-fated ECs occupied the DA. We analyzed mutants for EphB4, which encodes a receptor tyrosine kinase for the ephrin B2 ligand, as we hypothesized that ephrin B2/EphB4 signaling may mediate the repulsion of venous-fated ECs from the DA to the CV. Using an EC quantification approach, we discovered that venous-fated ECs increased in the DA and decreased in the CV in the mutants, whereas the rest of the ECs in each vessel were unaffected. This result suggests that the venous-fated ECs were retained in the DA and missing in the CV in the EphB4 mutant, and thus that ephrin B2/EphB4 signaling normally functions to clear venous-fated ECs from the DA to the CV by cell repulsion. Therefore, our cellular and molecular evidence suggests that the DA harbors venous progenitors that move to participate in CV formation, and that ephrin B2/EphB4 signaling regulates this aortic contribution to the mammalian CV.
Assuntos
Aorta/citologia , Células-Tronco/citologia , Veias/citologia , Animais , Células Endoteliais/citologia , Células Endoteliais/metabolismo , Camundongos , Camundongos Transgênicos , Neovascularização Fisiológica/fisiologia , Transdução de Sinais/fisiologia , Células-Tronco/metabolismoRESUMO
Fetoscopic endotracheal occlusion (FETO) is a prenatal treatment that may increase survival in severe congenital diaphragmatic hernia (CDH). In the USA, FETO is offered for isolated severe left-sided CDH in the context of an FDA-approved feasibility study. FETO in non-isolated cases of severe CDH is only performed with a compassionate use exemption from US regulatory bodies. Anomalies frequently associated with CDH include congenital cystic lesions of the lung and cardiac defects. We describe two cases of non-isolated severe left-sided CDH that underwent prenatal FETO, survived after birth and underwent postnatal surgical repair. The potential benefit of FETO in this setting is discussed. © 2017 John Wiley & Sons, Ltd.
Assuntos
Doenças Fetais/terapia , Fetoscopia , Hérnias Diafragmáticas Congênitas/terapia , Traqueia , Adulto , Oclusão com Balão , Feminino , Idade Gestacional , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Hérnias Diafragmáticas Congênitas/cirurgia , Humanos , Recém-Nascido , Masculino , Gravidez , Resultado do Tratamento , Ultrassonografia Pré-NatalRESUMO
Introduction In the past two decades, Enhanced Recovery After Surgery (ERAS) pathways for adults have improved efficiency of care and decreased length of stay (LOS) without increasing postoperative complications. The effects of enhanced recovery pathways for children are less well known. In this retrospective cohort study, we evaluated the effects of an enhanced recovery protocol (ERP) implementation in children undergoing colorectal surgery. Methods We introduced a colorectal ERP in 2017. Children and young adults (ages 2-22 years) were divided into pre-intervention (2014-2016) and post-intervention groups (2017-2019) for analysis. We abstracted data, including demographics, primary surgery, LOS, postoperative pain scores, and postoperative complications. Results A total of 432 patients were included. Of those,148 (34%) were pre-ERP implementation and 284 (66%) were post-ERP implementation. Post-ERP patients experienced significantly shorter LOS (5.7 vs. 8.3 days, p<0.01); required less intraoperative local anesthetic (9.5% vs. 38.5%, p<0.01) because 55% of patients received an epidural and 18% received an abdominal plane block; and used less postoperative opioid (62.5% vs. 98.7%, p<0.01) than did pre-ERAS patients. After protocol implementation, average pain scores were lower on postoperative day 1 (3.6 vs. 4.5, p<0.05) and across the hospitalization (3.0 vs. 4.0, p<0.01). Conclusion Enhanced recovery pathways decrease LOS, opioid use, and postoperative pain scores for children undergoing colorectal surgery and should be considered for this patient population.
Assuntos
Cirurgia Colorretal , Adolescente , Adulto , Analgésicos Opioides/uso terapêutico , Criança , Pré-Escolar , Humanos , Tempo de Internação , Dor Pós-Operatória/tratamento farmacológico , Dor Pós-Operatória/epidemiologia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/prevenção & controle , Estudos Retrospectivos , Adulto JovemRESUMO
PURPOSE: Anhydramnios secondary to anuria before 22 weeks of gestational age and congenital bilateral renal agenesis before 26 weeks of gestational age are collectively referred to as early-pregnancy renal anhydramnios. Early-pregnancy renal anhydramnios occurs in at least 1 in 2000 pregnancies and is considered universally fatal when left untreated because of severe pulmonary hypoplasia precluding ex utero survival The Renal Anhydramnios Fetal Therapy (RAFT) trial is a nonrandomized, nonblinded, multicenter clinical trial designed to assess the efficacy, safety, and feasibility of amnioinfusions for patients with pregnancies complicated by early-pregnancy renal anhydramnios. The primary objective of this study is to determine the proportion of neonates surviving to successful dialysis, defined as use of a dialysis catheter for ≥14 days. METHODS: A consortium of 9 North American Fetal Therapy Network (NAFTNet) centers was formed, and the RAFT protocol was refined in collaboration with the NAFTNet Scientific Committee. Enrollment in the trial began in April 2020. Participants may elect to receive amnioinfusions or to join the nonintervention observational expectant management group. Eligible pregnant women must be at least 18 years of age with a fetal diagnosis of isolated early-pregnancy renal anhydramnios. FINDINGS: In addition to the primary study objective stated above, secondary objectives include (1) to assess maternal safety and feasibility of the serial amnioinfusion intervention (2) to perform an exploratory study of the natural history of untreated early pregnancy renal anhydramnios (3) to examine correlations between prenatal imaging and lung specific factors in amniotic fluid as predictive of the efficacy of serial percutaneous amnioinfusions and (4) to determine short- and long-term outcomes and quality of life in surviving neonates and families enrolled in RAFT IMPLICATIONS: The RAFT trial is the first clinical trial to investigate the efficacy, safety, and feasibility of amnioinfusions to treat the survival-limiting pulmonary hypoplasia associated with anhydramnios. Although the intervention offers an opportunity to treat a condition known to be almost universally fatal in affected neonates, the potential burdens associated with end-stage kidney disease from birth must be acknowledged. CLINICALTRIALS: gov identifier: NCT03101891.
Assuntos
Terapias Fetais , Oligo-Hidrâmnio , Líquido Amniótico , Feminino , Idade Gestacional , Humanos , Recém-Nascido , Estudos Multicêntricos como Assunto , Oligo-Hidrâmnio/terapia , Gravidez , Qualidade de VidaRESUMO
Congenital abnormalities of the kidney and urinary tract (CAKUT) represent 20% of prenatally diagnosed congenital abnormalities. Although the majority of these abnormalities do not require intervention either pre or postnatally, there is a subset of patients whose disease is so severe that it may warrant intervention prior to delivery to prevent morbidity and mortality. These cases consist of patients with moderate lower urinary tract obstruction (LUTO) in which vesicocentesis, shunting or cystoscopy are options and patients with early pregnancy renal anhydramnios (EPRA) in whom amnioinfusion therapy may be an option. The main causes of EPRA are congenital bilateral renal agenesis (CoBRA), cystic kidney disease (CKD) and severe LUTO. Untreated, EPRA is universally fatal secondary to anhydramnios induced pulmonary hypoplasia. The evidence regarding therapy for LUTO is limited and the stopped early PLUTO (Percutaneous Shunting in Lower Urinary Tract Obstruction) trial was unable to provide definitive answers about patient selection. Evidence for EPRA therapy is also scant. Serial amnioinfusions have shown promise in cases of EPRA due to CoBRA or renal failure and this treatment modality forms the basis of the ongoing NIH funded RAFT (Renal Anhydramnios Fetal Therapy) trial. At present, there is consensus that treatment for EPRA should only occur in the setting of a clinical trial.
RESUMO
Congenital diaphragmatic hernias rarely present after 2 months of age and are typically diagnosed in the perinatal period. Moderate to severe diaphragmatic hernias present with respiratory symptoms, while late-onset hernias have a more varied presentation, depending on the age and content of the hernia. Very rarely, such hernias are found on incidental imaging, in which surgical repair is frequently recommended. A young girl with Loeys-Dietz syndrome and prior abdominal surgeries presents with 1-year history of increasingly severe, intermittent, abdominal and left shoulder pain. Prior imaging incidentally revealed a left diaphragmatic hernia with omentum protruding into the thoracic cavity. This was managed expectantly due to her other medical and surgical issues. Serial imaging revealed that the herniated omentum was increasing in size and symptoms began to develop. An uncomplicated primary thoracoscopic repair was performed. We report the first case of a congenital diaphragmatic hernia in a patient with Loeys-Dietz syndrome.
RESUMO
OBJECTIVE: Congenital diaphragmatic hernia (CDH) is a congenital defect associated with significant mortality and morbidity. We sought to assess the health-related quality of life (HRQoL) for caregivers of infants/children with CDH and determine risk factors for poorer HRQoL. METHODS: Families were recruited from a CDH-specific outpatient clinic and HRQoL was assessed by a validated HRQoL instrument (PedsQLTM Family Impact Module) at several time points. Mixed models were used to identify demographic and clinical factors associated with worse HRQoL for caregivers. RESULTS: A total of 29 subjects were recruited at a mean age of 2.4 ± 2.3 years. In terms of defect size, 6.9% had a Type A, 37.9% a Type B, 31.0% a Type C, and 24.1% a Type D. The mean HRQoL score at the first encounter was 67.6 ± 18.3; scores are reported from 0 to 100 with higher scores representing the higher reported quality of life. Lower median household incomes (p = .021) and use of extracorporeal membrane oxygenation (p = .013) were associated with poorer HRQoL scores. The presence of respiratory symptoms decreased HRQoL for caregivers, including daytime symptoms (p < .001) and nighttime symptoms (p < .001). While emergency department visits were not associated with a decrease in HRQoL, hospital admissions (p = .002), and reoperations for CDH (p < .001) were. CONCLUSION: Our study found a reduced quality of life associated with socioeconomic factors and severity of ongoing disease. Further study is needed to confirm these findings and identify strategies for aiding families cope with the chronicity of this congenital disease.
Assuntos
Oxigenação por Membrana Extracorpórea , Hérnias Diafragmáticas Congênitas , Cuidadores , Criança , Pré-Escolar , Humanos , Lactente , Qualidade de Vida , Fatores SocioeconômicosRESUMO
We present the case of a 16-year-old female with systemic lupus erythematosus who presented with shock of unclear etiology, refractory to fluid resuscitation and triple vasopressors. She suffered pulseless electrical activity and underwent cannulation onto veno-arterial extracorporeal membrane oxygenation (ECMO). After cannulation, it was discovered she had intentionally overdosed on her home medication, amlodipine, a calcium channel blocker (CCB). She was supported on ECMO, treated with IV calcium and insulin, and was able to be weaned off ECMO after 4 days. She developed oligoanuric acute kidney injury, treated with continuous renal replacement therapy followed by intermittent hemodialysis. At discharge, she was neurologically intact and did not require dialysis. Herein, we review the treatment of CCB overdose, review the literature on the use of ECMO in refractory shock due to cardiovascular medication overdose, and highlight the utility of ECMO in pediatric refractory shock and/or cardiac arrest of unclear etiology.
RESUMO
BACKGROUND: The opioid crisis has led to increasing numbers of overdose fatalities in teens and young adults. Surgery, as a common cause of acute pain in children, drives much of the opioid prescribing in pediatrics. Therefore, we sought to characterize opioid prescribing practices of pediatric surgeons by surveying members of the American Pediatric Surgery Association (APSA). STUDY DESIGN: After receiving approval from our institutional review board, we sent an online survey to the entire APSA membership. The survey included four vignettes of common pediatric surgical procedures with questions regarding analgesic prescribing practices, the rationale for these practices, and knowledge about opioid risk mitigation. RESULTS: Of 1127 APSA members contacted, 327 (29%) provided survey responses. For all vignettes, opioid prescribing was within standard ranges for 83% of respondents. Eighty-eight percent of respondents prescribed nonopioid pain medicine. Additionally, 25% reported routinely utilizing a prescription drug monitoring program, 64% did not tell patients how to dispose of opioids, and 37% did not know themselves how to dispose of leftover opioids. CONCLUSIONS: Prescribing by APSA surgeons is largely within standard ranges, but improvement is needed, particularly regarding opioid disposal. Procedure-specific consensus guidelines for opioid prescribing and opioid risk mitigation strategies are warranted. LEVEL OF EVIDENCE: Observational study, level III.
Assuntos
Analgésicos Opioides , Cirurgiões , Adolescente , Analgésicos , Analgésicos Opioides/uso terapêutico , Criança , Humanos , Dor Pós-Operatória/tratamento farmacológico , Padrões de Prática Médica , Inquéritos e Questionários , Estados UnidosRESUMO
BACKGROUND: Congenital pulmonary airway malformation (CPAM) is the most common prenatally-diagnosed lung malformation. This lesion, classified as macrocystic or microcystic, can lead to significant fetal compromise. Management options include observation, maternal antenatal steroid administration, and fetal surgical intervention. Current evidence suggests that microcystic (but not macrocystic) lesions and those with a cyst volume ratio (CVR) >1.6 are responsive to steroid therapy. The objective of this study was to identify patterns of prenatal steroid administration for the management of CPAMs and to identify characteristics of CPAMs prompting steroid administration. METHODS: An 18-question survey was distributed to obstetricians from the Pregnancy-Related Care Research Network (PRCRN) and the North American Fetal Therapy Network (NAFTNet), from January to April 2019, to capture antenatal steroid prescribing patterns. RESULTS: Response rates were 28.3% (138/487) for PRCRN and 63.3% (19/30) for NAFTNet. Among PRCRN members, 16.8% administered prenatal steroids, with most (77.2%) doing so for both microcystic and macrocystic CPAMs; corresponding percentages for NAFTNet members were 90.9% and 52.6%. Two thirds (65.6%) of obstetricians who administer steroids do so for a CVRâ>â1.6, without evidence of mediastinal shift or hydrops fetalis. CONCLUSIONS: There is a lack of consensus among obstetricians as to the CPAM characteristics that should prompt administration of prenatal steroids. Many surveyed obstetricians do not use cyst type or CVR to guide decision-making regarding steroid therapy.