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1.
J Am Coll Radiol ; 21(6S): S310-S325, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38823953

RESUMO

Soft tissue vascular anomalies may be composed of arterial, venous, and/or lymphatic elements, and diagnosed prenatally or later in childhood or adulthood. They are divided into categories of vascular malformations and vascular tumors. Vascular malformations are further divided into low-flow and fast-flow lesions. A low-flow lesion is most common, with a prevalence of 70%. Vascular tumors may behave in a benign, locally aggressive, borderline, or malignant manner. Infantile hemangioma is a vascular tumor that presents in the neonatal period and then regresses. The presence or multiple skin lesions in an infant can signal underlying visceral vascular anomalies, and complex anomalies may be associated with overgrowth syndromes. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.


Assuntos
Sociedades Médicas , Malformações Vasculares , Humanos , Malformações Vasculares/diagnóstico por imagem , Estados Unidos , Medicina Baseada em Evidências , Lactente , Neoplasias Vasculares/diagnóstico por imagem , Neoplasias de Tecidos Moles/diagnóstico por imagem , Recém-Nascido , Criança , Diagnóstico por Imagem/métodos , Hemangioma/diagnóstico por imagem , Guias de Prática Clínica como Assunto
2.
Prenat Diagn ; 43(12): 1514-1519, 2023 11.
Artigo em Inglês | MEDLINE | ID: mdl-37807606

RESUMO

OBJECTIVE: Giant omphaloceles (GO) have associated pulmonary hypoplasia and respiratory complications. Total lung volumes (TLV) on fetal MRI can prognosticate congenital diaphragmatic hernia outcomes; however, its applicability to GO is unknown. We hypothesize that late gestation TLV and observed-to-expected TLV (O/E TLV) on fetal MRI correlate with postnatal pulmonary morbidity in GO. METHOD: A single-institution retrospective review of GO evaluated between 2012 and 2022 was performed. Fetal MRI TLV between 32 and 36 weeks' gestation and O/E TLV throughout gestation were calculated and correlated with postnatal outcomes. RESULTS: 86 fetuses with omphaloceles were evaluated; however, only 26 met strict inclusion criteria. MRIs occurred between 18 and 36 weeks' gestation. Those requiring delivery room intubation had significantly lower late gestation TLV and O/E TLV. O/E TLV predicted tracheostomy placement and survival. Neither TLV nor O/E TLV predicted the length of hospitalization or supplemental oxygen after discharge. Three fetuses had a TLV less than 35 mL: one died of respiratory failure, and the other two required tracheostomy. CONCLUSIONS: Fetal MRI TLV measured between 32 and 36 weeks' gestation and O/E TLV predict the need for delivery room intubation and tracheostomy. O/E TLV correlated with survival. These data support fetal MRI as a prognostic tool to predict GO associated pulmonary morbidity.


Assuntos
Hérnia Umbilical , Hérnias Diafragmáticas Congênitas , Lactente , Feminino , Gravidez , Humanos , Hérnia Umbilical/complicações , Pulmão/diagnóstico por imagem , Medidas de Volume Pulmonar , Hérnias Diafragmáticas Congênitas/complicações , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Feto , Estudos Retrospectivos , Imageamento por Ressonância Magnética , Morbidade
3.
Am J Med Genet A ; 191(10): 2571-2577, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-37353964

RESUMO

Skeletal dysplasias broadly include disorders of cartilage or bone. Omodysplasia-1 is a type of skeletal dysplasia caused by biallelic loss of function variants in the GPC6 gene. GPC6 codes for the protein glypican 6 (GPC6) (OMIM *604404), which stimulates bone growth. We report a family in which five out of nine children were presented with a skeletal dysplasia characterized phenotypically by mild short stature and rhizomelia. All affected individuals were found to have homozygous missense variants in GPC6: c.511 C>T (p.Arg171Trp). Radiograph findings included rhizomelic foreshortening of all four extremities, coxa breva, and ulna minus deformity. Using a Hedgehog (Hh) reporter assay, we demonstrate that the variant found in this family results in significantly reduced stimulation of Hh activity when compared to the wild-type GPC6 protein, however protein function is still present. Thus, the milder phenotype seen in the family presented is hypothesized due to decreased GPC6 protein activity versus complete loss of function as seen in omodysplasia-1. Given the unique phenotype and molecular mechanism, we propose that this family's findings widen the phenotypic spectrum of GPC6-related skeletal dysplasias.


Assuntos
Nanismo , Osteocondrodisplasias , Criança , Humanos , Glipicanas/genética , Irmãos , Proteínas Hedgehog , Osteocondrodisplasias/diagnóstico por imagem , Osteocondrodisplasias/genética , Osteocondrodisplasias/metabolismo , Nanismo/genética
4.
Urology ; 172: 192-195, 2023 02.
Artigo em Inglês | MEDLINE | ID: mdl-36283505

RESUMO

Pediatric hypertension represents a rare though increasingly common medical problem. When encountered, a workup to determine the etiology should be conducted. In this report, we detail an unusual case in which a teenager presenting with hypertension was found to have multifocal primary paragangliomas. We illustrate important considerations in management which include appropriate preoperative labs and imaging, collaboration with endocrinology for preoperative alpha-blockade, surgical management with close perioperative hemodynamic control, and genetic evaluation for all patients with paragangliomas.


Assuntos
Neoplasias das Glândulas Suprarrenais , Hipertensão , Paraganglioma , Humanos , Adolescente , Criança , Paraganglioma/complicações , Paraganglioma/diagnóstico , Paraganglioma/genética , Diagnóstico por Imagem , Hipertensão/complicações , Neoplasias das Glândulas Suprarrenais/cirurgia
6.
Pediatr Surg Int ; 37(11): 1499-1504, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34505169

RESUMO

PURPOSE: In congenital diaphragmatic hernia (CDH), ultrasound (U/S) measurements of the contralateral lung commonly provide the observed-to-expected lung-to-head ratio (O/E LHR) and are used to determine the severity of pulmonary hypoplasia. Fetal magnetic resonance imaging (MRI) measurement of the observed-to-expected total lung volume (O/E TLV) has been used as an adjunct to O/E LHR in predicting outcomes. Since O/E LHR only measures the contralateral lung, we sought to investigate if MRI measurements of the contralateral lung volume (O/E CLV) can accurately predict outcomes in CDH. We hypothesize that O/E CLV is a better predictor of CDH outcomes than O/E LHR. METHODS: We identified all infants with a prenatal diagnosis of CDH at our fetal center who had both MRI and U/S measurements. Using lung volume ratios of right-left 55:45, we calculated O/E CLV from O/E TLV. We used receiver-operating characteristic (ROC) curves to calculate the area under the curve (AUC) to compare the predictive accuracy of O/E CLV to O/E LHR for ECMO support, as well as survival to both discharge and 1 year. RESULTS: Seventy-four patients had complete prenatal imaging with 39% requiring ECMO support. The median O/E CLV was 48.0% and the median O/E LHR was 42.3%. O/E CLV was a better predictor of the need for ECMO support (AUC 0.81 vs. 0.74). O/E CLV was a better predictor of survival to discharge (AUC 0.84 vs. 0.64) and 1-year survival (AUC 0.83 vs. 0.63) than O/E LHR. CONCLUSION: O/E LHR is a well-validated standard for predicting outcomes and guiding prenatal counseling in CDH. We provide evidence that fetal MRI measurements of the contralateral lung volume corrected for gestational age were more accurate in predicting the need for ECMO and survival. Future prospective studies validating O/E CLV regarding outcomes and ECMO utilization are warranted. LEVEL OF EVIDENCE: Level III, retrospective comparative study.


Assuntos
Hérnias Diafragmáticas Congênitas , Feminino , Idade Gestacional , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Hérnias Diafragmáticas Congênitas/cirurgia , Humanos , Pulmão/diagnóstico por imagem , Imageamento por Ressonância Magnética , Gravidez , Prognóstico , Estudos Prospectivos , Estudos Retrospectivos , Ultrassonografia Pré-Natal
7.
Pediatr Surg Int ; 37(4): 425-430, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33399927

RESUMO

PURPOSE: The goal of this study was to determine the feasibility of identifying the anal dimple (AD) on routine prenatal ultrasound. Using the presence, absence, appearance, and location of the anal dimple as an indirect sign for possible underlying anorectal malformations (ARM), we hypothesize that evaluation of the anal dimple as part of the fetal anatomic survey may increase the sensitivity in detecting less severe ARMs. METHODS: In a prospective longitudinal observational study, pregnant women who underwent prenatal ultrasound (US) at the Colorado Fetal Care Center between January 2019 and 2020 were enrolled. The variables recorded included gestational age, singleton versus multiple pregnancy, gender of the fetus, visualization of the AD, and reason for non-visualization of the AD. RESULTS: A total of 900 ultrasounds were performed, evaluating 1044 fetuses, in 372 different pregnant women. Gestational ages ranged from 16 to 38 weeks. The AD was visualized in 612 fetuses (58.6%) and not seen in 432 (41.4%). The two most common reasons for non-visualization were extremes in gestational age (n = 155; 36%) and fetal position (n = 152; 35.3%). The optimal gestational age range for AD visualization was 28-33 weeks + 6 days, with 78.1% visualization rate. CONCLUSION: Visualization of the anal dimple by ultrasound is feasible and may aid in the detection of less severe ARMs, ultimately impacting pregnancy management and family counseling. The optimal timing for anal dimple visualization is late second and third trimester.


Assuntos
Malformações Anorretais , Diagnóstico Pré-Natal/métodos , Ultrassonografia Pré-Natal , Adulto , Canal Anal/anormalidades , Malformações Anorretais/diagnóstico por imagem , Colorado , Feminino , Idade Gestacional , Humanos , Lactente , Gravidez , Cuidado Pré-Natal , Estudos Prospectivos , Ultrassonografia
8.
Pediatr Radiol ; 50(13): 1921-1933, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-33252759

RESUMO

With the advent of routine prenatal imaging, the number of renal anomalies identified prenatally has significantly increased; however, the underlying etiologies of these anomalies and the clinical significance of these findings remains unclear. This confusion is especially true for the prenatal diagnosis of cystic renal changes. The terms "cystic kidney disease" and "renal cystic dysplasia" encompass myriad renal diseases. Although renal cystic dysplasia in infants shares many similarities with multicystic dysplastic kidney (MCDK), it is important to distinguish MCDK from other etiologies that would lead to renal cysts, to ensure proper patient diagnosis and appropriate counseling regarding risks and to guide clinical management. The purpose of this review is to highlight the multiple etiologies of cystic kidney disease, including genetic associations, associations with underlying syndromes, and associations with underlying anatomical abnormalities. Here we focus on prenatal imaging, associated pathological findings, and clinical significance, with an emphasis on the defining characteristics of MCDK as compared to other forms of cystic renal disease.


Assuntos
Doenças Renais Císticas , Rim Displásico Multicístico , Feminino , Feto , Humanos , Lactente , Rim/diagnóstico por imagem , Doenças Renais Císticas/diagnóstico por imagem , Rim Displásico Multicístico/diagnóstico por imagem , Gravidez , Diagnóstico Pré-Natal , Ultrassonografia Pré-Natal
9.
Pediatr Radiol ; 50(2): 275-284, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31975185

RESUMO

The human placenta remains an enigma to many. Its position as the point of communication between distinct maternal and fetal circulations means that it must act as both source of nourishment and gatekeeper for the developing pregnancy. In vivo assessment of the placenta is perhaps the greatest challenge, yet it is most essential for diagnostic and prognostic purposes. In particular, there is a need for improved diagnostic accuracy in recognizing the invasive forms of the placenta accreta spectrum that require surgical intervention at delivery and often cesarean hysterectomy. The costs of insufficient sensitivity and specificity are high, with well-documented cases of adverse outcomes ranging from unnecessary surgery to maternal hemorrhage and even death. In Part I of this pictorial essay series, we reviewed the appearance of the normal developing placenta across gestation by MRI. With this as a background, we here consider the varied appearances of the placenta accreta spectrum (placenta accreta, increta, percreta), which is a growing challenge given the rapidly expanding number of women worldwide with history of cesarean section delivery. Accurate prenatal imaging is crucial for recognizing cases of the placenta accreta spectrum and for planning the necessary surgery.


Assuntos
Imageamento por Ressonância Magnética/métodos , Placenta Acreta/diagnóstico por imagem , Feminino , Humanos , Placenta/diagnóstico por imagem , Gravidez
10.
Pediatr Surg Int ; 35(9): 979-983, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31256295

RESUMO

PURPOSE: Presacral masses associated with anorectal malformations (ARM) are most frequently dermoid or teratomas. Sacrococcygeal teratoma (SCT), in isolation, is a different condition. There are limited data comparing the two. The aim of this study was to compare presacral masses associated with ARM and isolated SCTs. METHODS: A retrospective review was performed from 1979 to 2018. Patients with presacral masses and associated ARM (n = 39) were identified and compared to patients with isolated SCTs (n = 32). RESULTS: Those with presacral mass and ARM had a lower proportion of immature and mixed teratomas by pathology and a longer time to mass resection. They had higher percentages of sacral anomalies (76% versus 9%), tethered cord (50% versus 6%), and other anomalies (51% versus 9%). For long-term bowel function, patients with presacral mass and ARM were more likely to be fecally incontinent and require enemas (59% versus 11%). Regarding isolated SCTs, 4% recurred with a time to recurrence of 7 months. CONCLUSIONS: Compared to patients with isolated SCT, patients with presacral mass and ARM had lower grade tumors. In addition, they had a higher proportion of sacral defects and other congenital defects, as well as worse bowel function outcomes.


Assuntos
Malformações Anorretais/complicações , Malformações Anorretais/patologia , Neoplasias de Tecidos Moles/complicações , Neoplasias de Tecidos Moles/patologia , Teratoma/complicações , Teratoma/patologia , Malformações Anorretais/diagnóstico , Pré-Escolar , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Região Sacrococcígea/cirurgia , Neoplasias de Tecidos Moles/diagnóstico , Teratoma/diagnóstico
11.
Urology ; 130: 151-154, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30807774

RESUMO

Due to the rarity of Wilms tumor (WT) and the relative urgency with which pediatric renal tumors are treated, there is little reported data on the natural history and growth of WTs. Historical reports of estimated doubling times of WTs were based on time to disease recurrence after initial diagnosis and treatment, and were published before the current advancements in molecular biomarker testing. We compare 2 cases of WT with sequential imaging, and postulate how the growth parameters of these tumors may be associated with differing chromosomal traits.


Assuntos
Neoplasias Renais/patologia , Tumor de Wilms/patologia , Adolescente , Pré-Escolar , Humanos , Neoplasias Renais/diagnóstico por imagem , Neoplasias Renais/cirurgia , Masculino , Fatores de Tempo , Carga Tumoral , Tumor de Wilms/diagnóstico por imagem , Tumor de Wilms/cirurgia
12.
J Urol ; 200(2): 434-439, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29572157

RESUMO

PURPOSE: We characterize and apply the modified Bosniak classification system to a cohort of children with cystic renal lesions and known surgical pathology. MATERIALS AND METHODS: We identified all patients at our institution with cystic renal masses who also underwent surgery for these lesions. Patients without available preoperative imaging or pathology were excluded. All radiological imaging was independently reviewed by a pediatric radiologist blinded to pathological findings. Imaging characteristics (size, border, septations, calcifications, solid components, vascularity) were recorded from the most recent preoperative ultrasounds and computerized tomograms. The modified Bosniak classification system was applied to these scans and then correlated with final pathology. RESULTS: A total of 22 patients met study criteria. Median age at surgery was 6.1 years (range 11 months to 16.8 years). Of the patients 12 (54.5%) underwent open nephrectomy, 6 (27.3%) open partial nephrectomy, 2 (9.1%) laparoscopic cyst decortication, 1 (4.5%) open renal biopsy and 1 (4.5%) laparoscopic partial nephrectomy. Final pathology was benign in 9 cases (41%), intermediate in 6 (27%) and malignant in 7 (32%). All malignant lesions were modified Bosniak class 4, all intermediate lesions were modified class 3 or 4 and 8 of 9 benign lesions (89%) were modified class 1 or 2. CONCLUSIONS: Cystic renal lesions in children with a modified Bosniak class of 1 or 2 were most often benign, while class 3 or 4 lesions warranted surgical excision since more than 90% of masses harbored intermediate or malignant pathology. The modified Bosniak classification system appears to allow for a reasonable clinical risk stratification of pediatric cystic renal masses.


Assuntos
Doenças Renais Císticas/diagnóstico , Neoplasias Renais/diagnóstico , Rim/diagnóstico por imagem , Adolescente , Biópsia , Criança , Pré-Escolar , Estudos de Viabilidade , Humanos , Lactente , Rim/patologia , Rim/cirurgia , Doenças Renais Císticas/patologia , Doenças Renais Císticas/cirurgia , Neoplasias Renais/patologia , Neoplasias Renais/cirurgia , Laparoscopia/métodos , Nefrectomia/métodos , Período Pré-Operatório , Estudos Retrospectivos , Medição de Risco/métodos , Tomografia Computadorizada por Raios X , Ultrassonografia
13.
Congenit Heart Dis ; 13(3): 444-449, 2018 May.
Artigo em Inglês | MEDLINE | ID: mdl-29473337

RESUMO

BACKGROUND: The current management paradigm for children with single ventricle heart disease involves a series of palliative surgeries, culminating in the Fontan operation. This physiology results in a high central venous pressure, and in the setting of single ventricle heart disease, results in hepatic injury and fibrosis over time. Fontan-associated liver disease (FALD) is universally present in this cohort, and the current gold standard for diagnosis remains biopsy. Noninvasive assessments of liver fibrosis, such as ultrasound with elastography or acoustic radiation force impulse (ARFI), has been utilized in this cohort. The effect of poor cardiovascular efficiency, as measured by cardiopulmonary exercise test (CPET), on assessments of liver fibrosis remains poorly understood. METHODS: Retrospective, cross-sectional study. Subjects were evaluated in a multidisciplinary clinic setting for patients who have undergone Fontan operation. CPETs, liver ultrasound with elastography (ARFI), and standard laboratory tests were performed as part of routine clinical care pathway. Statistical analysis included linear correlation. RESULTS: There was a poor correlation between mean ARFI velocity and peak oxygen consumption (VO2max ) in this cohort (r = .20, P = NS). Similarly, there was poor correlation between ARFI and biomarkers of liver injury, time since Fontan operation and Fontan pressure. DISCUSSION: ARFI had poor correlation with functional capacity after Fontan, as measured by VO2max obtained during CPET. While a single measurement of liver elastography was not associated with cardiopulmonary efficiency, longitudinal data may reveal an association.


Assuntos
Técnicas de Imagem por Elasticidade/métodos , Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Cirrose Hepática/diagnóstico , Fígado/diagnóstico por imagem , Complicações Pós-Operatórias/diagnóstico , Criança , Estudos Transversais , Teste de Esforço , Feminino , Humanos , Cirrose Hepática/etiologia , Masculino , Complicações Pós-Operatórias/etiologia , Curva ROC , Reprodutibilidade dos Testes , Estudos Retrospectivos
14.
J Pediatr Orthop ; 38(3): 163-169, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-27261963

RESUMO

BACKGROUND: Radiographic surveillance of the hip is vital in the diagnosis and treatment of developmental dysplasia of the hip (DDH) in children. The acetabular index (AI) and the acetabular depth ratio (ADR) are radiographic parameters for evaluation of acetabular morphology. Normal reference curves for these parameters that allow for serial evaluation of acetabular development in a manner that is independent of age are necessary and clinically useful. The purpose of this study was (1) to establish normal values of AI and ADR in the normally developing pediatric hip up to age 14, (2) to generate percentile reference curves of both parameters, (3) to determine the extent of correlation between AI and ADR, and (4) to assess intrarater and interrater reliability of AI measurement. METHODS: We identified 1734 patients who underwent anterior-posterior pelvic radiography between 2004 and 2014. A total of 1152 patients (age range, 0.15 to 13.97 y; 2304 hips) were identified as radiographically normal in the radiology report, signed by the attending pediatric radiologist on the basis of the absence of structural deformity of the hip and previously established reference values for DDH assessment. A review of the medical records confirmed that patients had no diagnosis of DDH or any other orthopaedic hip pathology. The AI and ADR were measured in all radiographs. Normal values and fully parametric percentile curves were generated from birth to skeletal maturity. Correlation between AI and ADR was assessed using linear regression analysis. RESULTS: Normal AI decreased, and ADR increased, with age. Percentile curves were generated for AI and ADR. Using the provided equations, measured values can be converted to age-appropriate percentile and Z-score. The 2 parameters exhibited strong correlation (Pearson correlation=-0.789, P<0.001). For every unit increase in ADR, AI decreased by 0.94 degrees. CONCLUSIONS: We present updated normative values of AI that expand up to age 14, and novel reference values for ADR. The reference curves allow for the easy conversion of measured values to percentile and Z-score. Using the presented method during surveillance of the pathologic hip, change in acetabular development can now be assessed in a manner that is independent of age and the natural development of the acetabulum. LEVEL OF EVIDENCE: Level IV-case series.


Assuntos
Acetábulo/anatomia & histologia , Acetábulo/diagnóstico por imagem , Adolescente , Criança , Pré-Escolar , Feminino , Luxação Congênita de Quadril/diagnóstico , Humanos , Lactente , Masculino , Radiografia , Valores de Referência , Reprodutibilidade dos Testes , Estudos Retrospectivos
15.
J Urol ; 199(5): 1330-1336, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-29180299

RESUMO

PURPOSE: Current Children's Oncology Group studies on renal malignancy focus on minimizing treatment side effects with a goal of decreasing long-term complications. In this series we evaluate the patterns of initial imaging in children with renal tumors. MATERIALS AND METHODS: We retrospectively reviewed records of 122 patients treated for renal tumors at our institution between 2005 and 2016. Ideal imaging was defined as cross-sectional imaging of the chest, abdomen and pelvis in a single setting without any additional ionizing radiation exposing scans. RESULTS: Median patient age was 33.5 months (range 1 to 195). A total of 101 patients (83%) were initially evaluated elsewhere and subsequently referred to oncology (67.2%) for further evaluation. Before treatment 58 patients (47.5%) underwent imaging that was obtained in an ideal manner. Compared to those undergoing ideal imaging, median additional radiation exposure was 2.31 mSv (range 0.9 to 11.5), 3.08 mSv (0.6 to 11.7) and 5.1 mSv (1.2 to 16) in patients younger than 5 years, 5 to 9 years old and 10 years or older, respectively. Factors associated with undergoing ideal imaging included undergoing abdominal ultrasound as an initial scan (OR 3.637, p = 0.001), while presentation to an emergency department resulted in a reduced likelihood of undergoing ideal imaging (OR 0.351, p = 0.012). Factors associated with a decreased likelihood of undergoing initial screening ultrasound included presenting with vague symptoms (OR 0.072, p = 0.045) and presenting to a tertiary care emergency department (OR 0.228, p = 0.027). CONCLUSIONS: Current patterns of initial imaging for pediatric renal tumors are often associated with unnecessary and avoidable imaging studies, resulting in increased radiation exposure. Presenting to the emergency room as the initial point of contact with vague symptoms is associated with a decreased likelihood of undergoing appropriate or ideal pretherapy imaging, while initial evaluation with ultrasound is associated with a greater likelihood of undergoing ideal imaging, reducing overall radiation exposure. We advocate initial abdominal ultrasound in all pediatric patients suspected of having an abdominal mass. Our data highlight an opportunity for quality improvement across specialties caring for children with renal tumors.


Assuntos
Neoplasias Renais/diagnóstico por imagem , Exposição à Radiação , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Procedimentos Desnecessários
16.
J Pediatr Orthop ; 38(10): 498-502, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-27662383

RESUMO

BACKGROUND: We asked whether patient-specific factors and ultrasound (US) measurements of hip dysplasia severity at initial examination influence short-term residual acetabular dysplasia in patients successfully treated with Pavlik harness for developmental hip dysplasia. METHODS: After IRB approval, 134 hips (84 patients) successfully treated by the Pavlik method between August 2011 and October 2014 with follow-up at 12 months of age were identified. Early successful treatment was defined as normal examination and US after approximately 12 weeks of Pavlik treatment. Multivariate linear and logistic regression models were used to identify factors associated with acetabular index (AI) measurements at 12 months as well as factors associated with an increase in AI between the 6- and 12-month timepoints (dysplastic progression). RESULTS: The study consisted of 134 hips (84 patients). The distribution of dysplastic, Barlow, and Ortolani hips was 44.8% (N=60), 30.6% (N=41), and 24.6% (N=33), respectively. The crude incidence of residual dysplasia at the 6-month visit (AI>30 degrees) was 11.7% (12/102). The incidence of residual dysplasia at the 12-month visit (AI>28 degrees) was 11.8% (13/111). Graf type was the only variable associated with AI values at the 12-month visit (mean difference: Graf type-IV-Other, 2.6; 95% confidence interval, 0.3-4.9; P=0.026). CONCLUSIONS: The risk of residual acetabular dysplasia after normal hip US following Pavlik treatment is not negligible. Radiographic surveillance is warranted to monitor and screen for dysplasia progression. Patients with dislocated Graf type-IV hips at diagnosis are at increased risk of residual acetabular dysplasia at 1 year after successful treatment with the Pavlik method. LEVEL OF EVIDENCE: Level III-therapeutic study.


Assuntos
Acetábulo/diagnóstico por imagem , Acetábulo/patologia , Luxação Congênita de Quadril/diagnóstico por imagem , Luxação Congênita de Quadril/terapia , Articulação do Quadril/diagnóstico por imagem , Pré-Escolar , Progressão da Doença , Feminino , Articulação do Quadril/anormalidades , Humanos , Lactente , Masculino , Aparelhos Ortopédicos , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Ultrassonografia
17.
J Med Case Rep ; 11(1): 64, 2017 Mar 18.
Artigo em Inglês | MEDLINE | ID: mdl-28314387

RESUMO

BACKGROUND: The antenatal diagnosis of a combined esophageal atresia without tracheoesophageal fistula and duodenal atresia with or without gastric perforation is a rare occurrence. These diagnoses are difficult and can be suspected on ultrasound by nonspecific findings including a small stomach and polyhydramnios. Fetal magnetic resonance imaging adds significant anatomical detail and can aid in the diagnosis of these complicated cases. Upon an extensive literature review, there are no reports documenting these combined findings in a twin pregnancy. Therefore we believe this is the first case report of an antenatal diagnosis of combined pure esophageal and duodenal atresia in a twin gestation. CASE PRESENTATION: We present a case of a 30-year-old G1P0 white woman at 22-week gestation with a monochorionic-diamniotic twin pregnancy discordant for esophageal atresia, duodenal atresia with gastric perforation, hypoplastic left heart structures, and significant early gestation maternal polyhydramnios. In this case, fetal magnetic resonance imaging was able to depict additional findings including area of gastric wall rupture, hiatal hernia, dilation of the distal esophagus, and area of duodenal obstruction and thus facilitated the proper diagnosis. After extensive counseling at our multidisciplinary team meeting, the parents elected to proceed with radiofrequency ablation of the anomalous twin to maximize the survival of the normal co-twin. The procedure was performed successfully with complete cessation of flow in the umbilical artery and complete cardiac standstill in the anomalous twin with no detrimental effects on the healthy co-twin. CONCLUSIONS: Prenatal diagnosis of complex anomalies in twin pregnancies constitutes a multitude of ethical, religious, and cultural factors that come into play in the management of these cases. Fetal magnetic resonance imaging provides detailed valuable information that can assist in management options including possible prenatal intervention. The combination of a cystic structure with peristalsis-like movement above the diaphragm (for example, "the upper thoracic pouch sign"), polyhydramnios, and progressive distention of the stomach and duodenum should increase suspicion for a combined pure esophageal and duodenal atresia.


Assuntos
Obstrução Duodenal/embriologia , Atresia Esofágica/embriologia , Complicações na Gravidez/terapia , Redução de Gravidez Multifetal , Gravidez de Gêmeos , Diagnóstico Pré-Natal , Adulto , Obstrução Duodenal/diagnóstico por imagem , Atresia Esofágica/diagnóstico por imagem , Feminino , Aconselhamento Genético , Humanos , Recém-Nascido , Atresia Intestinal , Poli-Hidrâmnios , Gravidez , Complicações na Gravidez/diagnóstico por imagem , Complicações na Gravidez/psicologia , Resultado da Gravidez , Redução de Gravidez Multifetal/psicologia , Gravidez de Gêmeos/psicologia , Gêmeos
18.
Clin Orthop Relat Res ; 474(8): 1847-54, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-26975383

RESUMO

BACKGROUND: Patients with developmental dysplasia of the hip (DDH) whose hips are dislocated but reducible (Ortolani positive) are more likely to experience Pavlik harness treatment failure than are patients with dysplastic and reduced but dislocatable (Barlow positive) hips. However, data regarding factors associated with failure are limited and conflicting. QUESTIONS/PURPOSES: We asked: (1) What is the frequency of Pavlik harness treatment failure among Ortolani-positive hips, Barlow-positive hips, and dysplastic hips? (2) What are the factors predictive of failure of Pavlik harness treatment for Ortolani-positive hips? METHODS: In this retrospective study we identified 150 patients who underwent the Pavlik harness method for treatment of DDH between August 2011 and July 2015. Six patients initially treated at an outside facility, four patients with associated conditions, and three who pursued treatment elsewhere were excluded. A total of 137 patients (215 hips) with a median age at the time of Pavlik placement of 30 days (range, 4-155 days) were included. Of the 215 hips, 78 (36.3%) were Ortolani positive, 60 (27.9%) were Barlow positive, and 77 (35.8%) were stable, with the diagnosis of dysplasia made on ultrasound. All patients were treated with the Pavlik harness method. The primary outcome was failure of the Pavlik harness to achieve and maintain concentric hip reduction assessed by examination and ultrasound. All patients were followed after completion of Pavlik treatment for a minimum of 2 months (mean, 3 months; range, 2-4 months). In addition, 90% (122 of 137) of the patients were followed for a minimum of 6 months. Patient-specific data including family history, breech versus cephalic presentation at birth, age, sex, laterality, and hip abduction were recorded. Ultrasound data at the time of diagnosis included Graf classification, alpha angle, and percentage of femoral head coverage. RESULTS: The Pavlik harness method failed in 27% (21 of 78) of hips that were Ortolani positive, 8% (six of 77) with dysplasia, and 5% (three of 60) that were Barlow positive. After controlling for potential confounding variables, such as range of hip abduction, male sex (adjusted odds ratio [OR], 6.9; 95% CI, 2.0-24.2; p = 0.002) and Graf Type IV ultrasound classification (dislocated hip with alpha angle less than 43° and labrum displaced downward) (OR, 4.4; 95% CI, 1.3-15.4; p = 0.019) were identified as independent predictors of failure of Pavlik treatment among Ortolani-positive hips. CONCLUSIONS: Ultrasound imaging of the hip should be part of the initial assessment for Ortolani-positive hips, as the ultrasound classification was found to have prognostic implications. Parents of male infants with Graf Type IV hips should be counseled regarding the higher risk of Pavlik failure. Future well-designed prospective controlled studies are necessary to establish whether alternative strategies to the Pavlik harness might improve the early outcomes of DDH in males with Graf Type IV hips. LEVEL OF EVIDENCE: Level III, therapeutic study.


Assuntos
Luxação Congênita de Quadril/terapia , Articulação do Quadril/fisiopatologia , Procedimentos Ortopédicos/instrumentação , Aparelhos Ortopédicos , Fenômenos Biomecânicos , Desenho de Equipamento , Feminino , Luxação Congênita de Quadril/classificação , Luxação Congênita de Quadril/diagnóstico por imagem , Luxação Congênita de Quadril/fisiopatologia , Articulação do Quadril/anormalidades , Articulação do Quadril/diagnóstico por imagem , Humanos , Lactente , Recém-Nascido , Masculino , Razão de Chances , Procedimentos Ortopédicos/efeitos adversos , Valor Preditivo dos Testes , Recuperação de Função Fisiológica , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores Sexuais , Fatores de Tempo , Falha de Tratamento , Ultrassonografia/métodos
19.
AJR Am J Roentgenol ; 189(5): 1096-103, 2007 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-17954646

RESUMO

OBJECTIVE: The objective of our study was to evaluate intraoperative low-field MRI for the frequency and duration of imaging sessions needed during surgery, the direct additional procedure time attributable to imaging, and the proportion of cases in which information provided by intraoperative MRI led to a change in the procedure or otherwise was deemed valuable by operating surgeons. MATERIALS AND METHODS: One hundred twenty-two patients (65 males, 57 females; age range, 6-77 years; mean age, 43.8 years) underwent 130 neurosurgical and ENT procedures (106 craniotomies, 17 transsphenoidal pituitary resections, three biopsies, three intracranial cyst aspirations or injections, and one skull base resection) in a specially designed surgical MRI suite equipped with a 0.2-T imager and a prototype rotating, tiltable surgical table. The intraoperative MR sequences included free induction with steady-state precession (fast imaging with steady-state precession [FISP]), steady-state free precession T2-weighted, reverse fast imaging with steady-state free precession (PSIF), FLASH, spin-echo T1-weighted, turbo spin-echo (TSE) T2-weighted, and TSE FLAIR. Each case was analyzed for the number of imaging sessions, duration of each session, total imaging time during surgery, and impact of imaging information on procedure. RESULTS: Each patient underwent between one and five intraor postoperative imaging sessions. Imaging times were 1.7 seconds-8 minutes 31 seconds per sequence. The mean total imaging time was 35 minutes 17 seconds per surgical procedure. Imaging was continuous during biopsy and cyst aspiration procedures and averaged 200.67 and 54.66 minutes, respectively. Additional surgical resection based on intraoperative imaging findings was performed in 72.8% of the cases. CONCLUSION: Intraoperative low-field MRI provides valuable information for surgical decision making that is predominantly related to detection of residual tumor and the exclusion of complications. The benefits of this technology surpass the time cost associated with its implementation when using proper imaging strategies.


Assuntos
Leitos , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/cirurgia , Aumento da Imagem/instrumentação , Imagem por Ressonância Magnética Intervencionista/instrumentação , Procedimentos Neurocirúrgicos/instrumentação , Adolescente , Adulto , Idoso , Criança , Feminino , Humanos , Aumento da Imagem/métodos , Imagem por Ressonância Magnética Intervencionista/métodos , Masculino , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos/métodos , Rotação , Fatores de Tempo , Resultado do Tratamento
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