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1.
J Ultrasound Med ; 43(9): 1595-1604, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-38752367

RESUMEN

OBJECTIVES: Mercapto acetyl tri-glycine renogram (MAG3) scan has been the gold standard assessment of pediatric ureteropelvic junction obstruction (UPJO) but requires intravenous access and radiation exposure. While Doppler ultrasound measurements of resistive indices (RI) of the arcuate arteries have been proposed as an alternative assessment of obstruction, they have not been widely adopted in the pediatric population. We hypothesized that RI of the main renal artery (RA) is more strongly correlated with MAG3 findings than arcuate RI. METHODS: Pediatric patients with unilateral Society for Fetal Urology grade 3-4 hydronephrosis undergoing concomitant RUS and MAG3 were recruited. Doppler ultrasound peak systolic velocity (PSV); RI of bilateral RA at the origin, middle, and hilum; and RI of the superior, middle, and lower pole arcuate arteries were obtained. MAG3 differential renal function (DRF) and T½ were recorded. Differences in RI measurements (DRI) between the affected and normal kidney were calculated and compared with DRF and T½. RESULTS: 31 patients (median 4.6-month-old) were enrolled. Only RA RI at the origin differed between affected and normal kidneys (p < .001). DRI RA at the origin showed weak evidence for an association with MAG3 DRF < 40% (p .07). DRI was not associated with T½ > 20 minutes. CONCLUSION: There was weak evidence for an association between RA DRI at the origin and DRF but not with T½. These findings suggest that RA DRI may provide additional data in the evaluation of patients with UPJO to tailor the use of MAG3 and associated risk of radiation exposure to those patients most at risk for concomitant renal function impairment.


Asunto(s)
Ultrasonografía Doppler , Obstrucción Ureteral , Humanos , Obstrucción Ureteral/diagnóstico por imagen , Obstrucción Ureteral/fisiopatología , Femenino , Masculino , Lactante , Ultrasonografía Doppler/métodos , Arteria Renal/diagnóstico por imagen , Arteria Renal/fisiopatología , Pelvis Renal/diagnóstico por imagen , Pelvis Renal/fisiopatología , Preescolar , Resistencia Vascular/fisiología , Niño , Estudios Prospectivos , Reproducibilidad de los Resultados , Hidronefrosis/diagnóstico por imagen , Hidronefrosis/fisiopatología , Riñón/diagnóstico por imagen , Riñón/fisiopatología
2.
Pediatr Radiol ; 54(11): 1850-1861, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-39179930

RESUMEN

BACKGROUND: Micrognathia can be diagnosed in utero with ultrasound by measuring the jaw index and/or inferior facial angle, though it can be challenging due to fetal positioning. The jaw index can be measured with magnetic resonance imaging (MRI) using the masseter muscle, but indistinct margins can lead to inaccuracy; the easily visualized posterior teeth buds may be a better landmark. OBJECTIVE: We aimed to evaluate inter-reader variability, agreement with ultrasound, and association with postnatal outcomes using MRI to measure the inferior facial angle, jaw index by masseter muscle, and jaw index by posterior teeth buds. MATERIALS AND METHODS: A single-institution retrospective review was performed of singleton pregnancies with prenatally diagnosed micrognathia by ultrasound or MRI from September 2013-June 2022. Ultrasound measurements were obtained by a maternal-fetal medicine specialist and MRI measurements by two radiologists to evaluate inter-reader variability. Intraclass correlation coefficients (ICC) and Bland-Altman analysis were used to assess agreement between imaging methods and logistic regressions and ROC curves to assess associations with postnatal outcomes. RESULTS: Forty-three fetuses (median gestational age 26 weeks (IQR 22-31); 47% male (20/43)) were included. Ultrasound measurements could not be obtained for jaw index in 15/43 (35%) fetuses and inferior facial angle in 11/43 (26%); MRI measurements were obtained by at least one reader in all cases. Jaw index by teeth buds demonstrated lowest inter-reader variability (ICC = 0.82, P < 0.001) and highest agreement with ultrasound (bias -0.23, 95% CI -2.8-2.2). All MRI measurements, but not ultrasound, predicted need for mandibular distraction (inferior facial angle P = 0.02, jaw index by masseter muscle P = 0.04, jaw index by teeth buds P = 0.01). CONCLUSION: Fetal MRI measurements, particularly jaw index measured by posterior teeth buds, demonstrate low inter-reader variability and high agreement with ultrasound, and may predict need for mandibular distraction postnatally.


Asunto(s)
Imagen por Resonancia Magnética , Micrognatismo , Ultrasonografía Prenatal , Humanos , Femenino , Imagen por Resonancia Magnética/métodos , Ultrasonografía Prenatal/métodos , Estudios Retrospectivos , Embarazo , Masculino , Micrognatismo/diagnóstico por imagen , Puntos Anatómicos de Referencia/diagnóstico por imagen , Maxilares/diagnóstico por imagen , Diagnóstico Prenatal/métodos
3.
J Magn Reson Imaging ; 2023 Oct 19.
Artículo en Inglés | MEDLINE | ID: mdl-37855630

RESUMEN

BACKGROUND: Recent advances in hardware and software permit the use of cardiac MRI of late gestation fetuses, however there is a paucity of MRI-based reference values. PURPOSE: To provide initial data on fetal cardiac MRI-derived cardiac dimensions, volumes, ventricular function, and left ventricular longitudinal strain in healthy developing fetuses >30 weeks gestational age. STUDY TYPE: Prospective. POPULATION: Twenty-five third trimester (34 ± 1 weeks, range of 32-37 weeks gestation) women with healthy developing fetuses. FIELD STRENGTH/SEQUENCE: Studies were performed at 1.5 T and 3 T. Cardiac synchronization was achieved with a Doppler ultrasound device. The protocol included T2 single shot turbo spin echo stacks for fetal weight and ultrasound probe positioning, and multiplanar multi-slice cine balanced steady state free precession gradient echo sequences. ASSESSMENT: Primary analyses were performed by a single observer. Weight indexed right ventricular (RV) and left ventricular (LV) volumes and function were calculated from short axis (SAX) stacks. Cardiac dimensions were calculated from the four-chamber and SAX stacks. Single plane LV longitudinal strain was calculated from the four-chamber stack. Interobserver variability was assessed in 10 participants. Cardiac MRI values were compared against available published normative fetal echocardiogram data using z-scores. STATISTICAL TESTS: Mean and SDs were calculated for baseline maternal/fetal demographics, cardiac dimensions, volumes, ventricular function, and left ventricular longitudinal strain. Bland-Altman and intraclass correlation coefficient analysis was performed to test interobserver variability. RESULTS: The mean gestational age was 34 ± 1.4 weeks. The mean RV and LV end diastolic volumes were 3.1 ± 0.6 mL/kg and 2.4 ± 0.5 mL/kg respectively. The mean RV cardiac output was 198 ± 49 mL/min/kg while the mean LV cardiac output was 173 ± 43 mL/min/kg. DATA CONCLUSION: This paper reports initial reference values obtained by cardiac MRI in healthy developing third trimester fetuses. MRI generally resulted in slightly larger indexed values (by z-score) compared to reports in literature using fetal echocardiography. EVIDENCE LEVEL: 1 TECHNICAL EFFICACY: Stage 2.

4.
Am J Med Genet A ; 191(10): 2571-2577, 2023 10.
Artículo en Inglés | MEDLINE | ID: mdl-37353964

RESUMEN

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.


Asunto(s)
Enanismo , Osteocondrodisplasias , Niño , Humanos , Glipicanos/genética , Hermanos , Proteínas Hedgehog , Osteocondrodisplasias/diagnóstico por imagen , Osteocondrodisplasias/genética , Osteocondrodisplasias/metabolismo , Enanismo/genética
5.
Prenat Diagn ; 43(12): 1514-1519, 2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-37807606

RESUMEN

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.


Asunto(s)
Hernia Umbilical , Hernias Diafragmáticas Congénitas , Lactante , Femenino , Embarazo , Humanos , Hernia Umbilical/complicaciones , Pulmón/diagnóstico por imagen , Mediciones del Volumen Pulmonar , Hernias Diafragmáticas Congénitas/complicaciones , Hernias Diafragmáticas Congénitas/diagnóstico por imagen , Feto , Estudios Retrospectivos , Imagen por Resonancia Magnética , Morbilidad
6.
Pediatr Radiol ; 53(3): 479-486, 2023 03.
Artículo en Inglés | MEDLINE | ID: mdl-36289070

RESUMEN

BACKGROUND: Over the last two decades, fetal imaging has greatly improved, and new prenatal imaging measurements have been developed to characterize congenital diaphragmatic hernia (CDH) severity. OBJECTIVE: To determine the best prenatal imaging predictor of postnatal CDH outcomes, including use of extracorporeal membrane oxygenation (ECMO) and in-hospital mortality, with particular attention to the percentage of liver herniation (%LH) as a predictor. Additionally, we sought to guide best practices across hospital systems including improved models of prenatal risk assessment. MATERIALS AND METHODS: We conducted a retrospective review of infants with left CDH who were prenatally diagnosed. We analyzed prenatal imaging measurements including observed-to-expected (O/E) lung-to-head ratio (LHR) on US, percentage predicted lung volume (PPLV) on MRI, and O/E total fetal lung volume (TFLV) and %LH on MRI. We compared prenatal imaging characteristics for infants with (1) in-hospital postnatal mortality and (2) use of ECMO. Then we performed multivariate logistic regression to determine independent predictors of postnatal outcomes. RESULTS: We included 63 infants with a median gestation of 34 weeks at the time of prenatal MRI. Low O/E LHR (31.2 vs. 50, P < 0.0001), PPLV (14.7 vs. 22.6, P < 0.0001) and O/E TLFV (24.6 vs. 38.3, P < 0.0001) and high %LH (15.1 vs. 2.1, P = 0.0006) were associated with worse postnatal outcomes; however, only PPLV was predictive of survival and need for ECMO on multivariable analysis. PPLV survival to discharge model showed an area under the curve (AUC) of 0.93 (95% confidence interval [CI]: 0.86, 0.99), P < 0.0001; and an odds ratio of 68.7 (95% CI: 6.5-2,302), P = 0.003. PPLV need for ECMO model showed AUC = 0.87 (95% CI: 0.78, 0.96), P < 0.0001; and odds ratio = 20.1 (95% CI: 3.1-226.3), P = 0.011. CONCLUSION: Low O/E LHR, PPLV and O/E TFLV and high %LH in the third trimester are associated with worse postnatal outcomes. PPLV most strongly predicted outcome using a logistic regression model. Percentage of liver herniation was not an independent predictor of outcomes.


Asunto(s)
Hernias Diafragmáticas Congénitas , Embarazo , Femenino , Humanos , Tercer Trimestre del Embarazo , Pronóstico , Pulmón/diagnóstico por imagen , Mediciones del Volumen Pulmonar/métodos , Hígado , Imagen por Resonancia Magnética , Estudios Retrospectivos , Ultrasonografía Prenatal
7.
Fetal Diagn Ther ; 49(7-8): 293-300, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35917796

RESUMEN

INTRODUCTION: The aim of this study was to determine the feasibility of fetal MRI in identifying the normal anal dimple (AD) and compare it with prenatal ultrasound (US). METHODS: Retrospective review of 130 patients with both fetal MRI and US. The gestational age (GA) was stratified into four groups: (1) 16 to 21 weeks-6 days; (2) 22 to 27 weeks-6 days; (3) 28 to 33 weeks-6 days; and (4) 34 weeks and beyond. Steady-state free precession (SSFP) and single shot fast spin echo (SSFSE) axial T2 MRI and transverse US images of the fetal perineum were analyzed, and visualization of the AD was determined. Clinical indication, gender, single versus multiple gestation, best MRI sequence where it was seen, and postnatal AD information were recorded. RESULTS: The AD was visualized in 125/130 fetal MRIs, and visualization was independent of GA (p 0.230). US visualized the AD in 67/130 cases, and the best GA for visualization was in group 3 (p < 0.001). There was no difference in AD visualization between SSFSE and SSFP sequences (p 0.167). CONCLUSION: Prenatal visualization of the AD by MRI is feasible and superior to US, independent of GA. Adding AD visualization to routine screening prenatal US and MRI may increase recognition of anorectal malformation.


Asunto(s)
Imagen por Resonancia Magnética , Diagnóstico Prenatal , Embarazo , Femenino , Humanos , Recién Nacido , Diagnóstico Prenatal/métodos , Imagen por Resonancia Magnética/métodos , Estudios Retrospectivos , Edad Gestacional , Atención Prenatal
8.
Pediatr Surg Int ; 37(11): 1499-1504, 2021 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-34505169

RESUMEN

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.


Asunto(s)
Hernias Diafragmáticas Congénitas , Femenino , Edad Gestacional , Hernias Diafragmáticas Congénitas/diagnóstico por imagen , Hernias Diafragmáticas Congénitas/cirugía , Humanos , Pulmón/diagnóstico por imagen , Imagen por Resonancia Magnética , Embarazo , Pronóstico , Estudios Prospectivos , Estudios Retrospectivos , Ultrasonografía Prenatal
9.
Pediatr Surg Int ; 37(4): 425-430, 2021 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-33399927

RESUMEN

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.


Asunto(s)
Malformaciones Anorrectales , Diagnóstico Prenatal/métodos , Ultrasonografía Prenatal , Adulto , Canal Anal/anomalías , Malformaciones Anorrectales/diagnóstico por imagen , Colorado , Femenino , Edad Gestacional , Humanos , Lactante , Embarazo , Atención Prenatal , Estudios Prospectivos , Ultrasonografía
10.
Pediatr Radiol ; 50(2): 264-274, 2020 02.
Artículo en Inglés | MEDLINE | ID: mdl-31975184

RESUMEN

Mounting evidence suggests that the placenta is involved in nearly all abnormalities of pregnancy and fetal development. Traditional imaging evaluation of the placenta by ultrasound has more recently been complemented by MRI for complex cases requiring additional information, such as in the diagnosis of the placenta accreta spectrum (placenta accreta, increta and percreta). MRI can often help delineate the safest approach to delivery and adds diagnostic certainty to enable prognostication and to avoid potentially lethal complications. Increasingly, prenatal MRI has become the purview of the pediatric imager and is becoming the standard of care for select gestational indications. However, placental MRI might be unfamiliar to the radiologist. Thus, we provide a simple and systematic approach to evaluating the placenta by MRI, to enable delivery planning and family counseling.


Asunto(s)
Imagen por Resonancia Magnética/métodos , Placenta/anatomía & histología , Femenino , Humanos , Embarazo
11.
Pediatr Radiol ; 50(2): 275-284, 2020 02.
Artículo en Inglés | MEDLINE | ID: mdl-31975185

RESUMEN

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.


Asunto(s)
Imagen por Resonancia Magnética/métodos , Placenta Accreta/diagnóstico por imagen , Femenino , Humanos , Placenta/diagnóstico por imagen , Embarazo
12.
Pediatr Radiol ; 50(13): 1921-1933, 2020 12.
Artículo en Inglés | MEDLINE | ID: mdl-33252759

RESUMEN

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.


Asunto(s)
Enfermedades Renales Quísticas , Riñón Displástico Multiquístico , Femenino , Feto , Humanos , Lactante , Riñón/diagnóstico por imagen , Enfermedades Renales Quísticas/diagnóstico por imagen , Riñón Displástico Multiquístico/diagnóstico por imagen , Embarazo , Diagnóstico Prenatal , Ultrasonografía Prenatal
13.
Fetal Diagn Ther ; 47(12): 933-938, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-33105131

RESUMEN

OBJECTIVES: There is a paucity of evidence to guide the perinatal management of difficult airways in fetuses with micrognathia. We aimed to (1) develop a postnatal grading system based on the extent of airway intervention required at birth to assess the severity of micrognathic airways and (2) compare trends in airway management and outcomes by location of birth [nonfetal center (NFC), defined as a hospital with or without an NICU and no fetal team, versus fetal center (FC), defined as a hospital with an NICU and fetal team]. METHODS: We retrospectively reviewed the prenatal and postnatal records of all neonates diagnosed with micrognathia from January 2010 to April 2018 at a quaternary children's hospital. We developed a novel grading scale, the Micrognathia Grading Scale (MGS), to grade the extent of airway intervention at birth from 0 (no airway intervention) to 4 (requirement of EXIT or advanced airway instrumentation for airway securement). RESULTS: We identified 118 patients with micrognathia. Eighty-nine percent (105/118) were eligible for grading using the MGS. When the MGS was applied, the airway grades were as follows: grade 0 (30%), grade 1 (10%), grade 2 (9%), grade 3 (48%), and grade 4 (4%). A quarter of micrognathic patients with grade 0-2 airways had postnatal hospital readmissions for airway obstruction after birth, of which all were born at NFC. Over 40% of patients with grade 3-4 micrognathic airways required airway intervention within 24 h of birth. Overall, NFC patients had a readmission rate of (27%) for airway obstruction after birth compared to FC patients (17%). CONCLUSIONS: Due to the high incidence of grade 3-4 airways on the MGS in micrognathic patients, fetuses with prenatal findings suggestive of micrognathia should be referred to a comprehensive fetal care center capable of handling complex neonatal airways. For grade 0-2 airways, infants frequently had postnatal complications necessitating airway intervention; early referral to a multidisciplinary team for both prenatal and postnatal airway management is recommended.


Asunto(s)
Obstrucción de las Vías Aéreas , Micrognatismo , Retrognatismo , Manejo de la Vía Aérea , Obstrucción de las Vías Aéreas/terapia , Niño , Femenino , Feto , Humanos , Lactante , Recién Nacido , Micrognatismo/diagnóstico por imagen , Micrognatismo/terapia , Embarazo , Estudios Retrospectivos , Ultrasonografía Prenatal
14.
Pediatr Radiol ; 49(6): 839, 2019 May.
Artículo en Inglés | MEDLINE | ID: mdl-30949728

RESUMEN

The published version of this article unfortunately contained a mistake. Author name Mariana Z. Meyers was incorrect. The correct middle initial is given above.

15.
Pediatr Radiol ; 49(3): 387-398, 2019 03.
Artículo en Inglés | MEDLINE | ID: mdl-30547222

RESUMEN

The term cloacal malformation is commonly used to describe the classic cloacal malformation where there is a single common urogenital and intestinal channel located at the expected site of the urethra. There is, however, a spectrum of cloacal abnormalities that differ from this classic type and are less well discussed in the radiologic and surgical literature. The aim of this pictorial essay is to familiarize radiologists with the anatomy, appropriate terminology and key prenatal imaging findings that differentiate the six entities that constitute the spectrum of cloacal abnormalities.


Asunto(s)
Cloaca/anomalías , Cloaca/diagnóstico por imagen , Feto/anomalías , Feto/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Anomalías Urogenitales/diagnóstico por imagen , Diagnóstico Diferencial , Femenino , Humanos , Embarazo
16.
Pediatr Surg Int ; 35(9): 979-983, 2019 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-31256295

RESUMEN

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.


Asunto(s)
Malformaciones Anorrectales/complicaciones , Malformaciones Anorrectales/patología , Neoplasias de los Tejidos Blandos/complicaciones , Neoplasias de los Tejidos Blandos/patología , Teratoma/complicaciones , Teratoma/patología , Malformaciones Anorrectales/diagnóstico , Preescolar , Diagnóstico Diferencial , Femenino , Humanos , Lactante , Masculino , Estudios Retrospectivos , Región Sacrococcígea/cirugía , Neoplasias de los Tejidos Blandos/diagnóstico , Teratoma/diagnóstico
17.
JAMA ; 322(19): 1877-1886, 2019 11 19.
Artículo en Inglés | MEDLINE | ID: mdl-31742630

RESUMEN

Importance: Umbilical cord milking as an alternative to delayed umbilical cord clamping may provide equivalent benefits to preterm infants, but without delaying resuscitation. Objective: To determine whether the rates of death or severe intraventricular hemorrhage differ among preterm infants receiving placental transfusion with umbilical cord milking vs delayed umbilical cord clamping. Design, Setting, and Participants: Noninferiority randomized clinical trial of preterm infants (born at 23-31 weeks' gestation) from 9 university and private medical centers in 4 countries were recruited and enrolled between June 2017 and September 2018. Planned enrollment was 750 per group. However, a safety signal comprising an imbalance in the number of severe intraventricular hemorrhage events by study group was observed at the first interim analysis; enrollment was stopped based on recommendations from the data and safety monitoring board. The planned noninferiority analysis could not be conducted and a post hoc comparison was performed instead. Final date of follow-up was December 2018. Interventions: Participants were randomized to umbilical cord milking (n = 236) or delayed umbilical cord clamping (n = 238). Main Outcomes and Measures: The primary outcome was a composite of death or severe intraventricular hemorrhage to determine noninferiority of umbilical cord milking with a 1% noninferiority margin. Results: Among 540 infants randomized, 474 (88%) were enrolled and completed the trial (mean gestational age of 28 weeks; 46% female). Twelve percent (29/236) of the umbilical cord milking group died or developed severe intraventricular hemorrhage compared with 8% (20/238) of the delayed umbilical cord clamping group (risk difference, 4% [95% CI, -2% to 9%]; P = .16). Although there was no statistically significant difference in death, severe intraventricular hemorrhage was statistically significantly higher in the umbilical cord milking group than in the delayed umbilical cord clamping group (8% [20/236] vs 3% [8/238], respectively; risk difference, 5% [95% CI, 1% to 9%]; P = .02). The test for interaction between gestational age strata and treatment group was significant for severe intraventricular hemorrhage only (P = .003); among infants born at 23 to 27 weeks' gestation, severe intraventricular hemorrhage was statistically significantly higher with umbilical cord milking than with delayed umbilical cord clamping (22% [20/93] vs 6% [5/89], respectively; risk difference, 16% [95% CI, 6% to 26%]; P = .002). Conclusions and Relevance: In this post hoc analysis of a prematurely terminated randomized clinical trial of umbilical cord milking vs delayed umbilical cord clamping among preterm infants born at less than 32 weeks' gestation, there was no statistically significant difference in the rate of a composite outcome of death or severe intraventricular hemorrhage, but there was a statistically significantly higher rate of severe intraventricular hemorrhage in the umbilical cord milking group. The early study termination and resulting post hoc nature of the analyses preclude definitive conclusions. Trial Registration: ClinicalTrials.gov Identifier: NCT03019367.


Asunto(s)
Hemorragia Cerebral Intraventricular/prevención & control , Constricción , Enfermedades del Prematuro/prevención & control , Recien Nacido Prematuro , Cordón Umbilical , Terminación Anticipada de los Ensayos Clínicos , Femenino , Edad Gestacional , Humanos , Lactante , Muerte del Lactante , Recién Nacido , Enfermedades del Prematuro/mortalidad , Masculino , Evaluación de Resultado en la Atención de Salud , Embarazo
18.
J Urol ; 200(2): 434-439, 2018 08.
Artículo en Inglés | MEDLINE | ID: mdl-29572157

RESUMEN

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.


Asunto(s)
Enfermedades Renales Quísticas/diagnóstico , Neoplasias Renales/diagnóstico , Riñón/diagnóstico por imagen , Adolescente , Biopsia , Niño , Preescolar , Estudios de Factibilidad , Humanos , Lactante , Riñón/patología , Riñón/cirugía , Enfermedades Renales Quísticas/patología , Enfermedades Renales Quísticas/cirugía , Neoplasias Renales/patología , Neoplasias Renales/cirugía , Laparoscopía/métodos , Nefrectomía/métodos , Periodo Preoperatorio , Estudios Retrospectivos , Medición de Riesgo/métodos , Tomografía Computarizada por Rayos X , Ultrasonografía
19.
J Urol ; 199(5): 1330-1336, 2018 05.
Artículo en Inglés | MEDLINE | ID: mdl-29180299

RESUMEN

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.


Asunto(s)
Neoplasias Renales/diagnóstico por imagen , Exposición a la Radiación , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Estudios Retrospectivos , Procedimientos Innecesarios
20.
J Pediatr ; 192: 93-98.e1, 2018 01.
Artículo en Inglés | MEDLINE | ID: mdl-29246364

RESUMEN

OBJECTIVE: To determine whether endotracheal tube (ETT) insertion depth should be modified in infants with congenital diaphragmatic hernia (CDH) to reduce the risk of main-stem intubation. STUDY DESIGN: The distance from the thoracic inlet to the carina was measured antenatally by fetal magnetic resonance imaging (MRI) between 20-28 weeks' (early) and 30-34 weeks' (late) gestation in 30 infants with CDH and compared with 12 early and 36 late MRIs in control infants without CDH. Postnatal tube position was assessed by chest radiograph in the same 30 infants with CDH and compared with 20 control infants with postnatal birth depression. RESULTS: The carina position was displaced upward in fetuses and newborns with CDH. Distance from the thoracic inlet to the carina compared with controls was 1.04 ± 0.1 cm vs 1.42 ± 0.07 cm on early MRI (P < .05), 1.43 ± 0.14 cm vs 1.9 ± 0.04 cm on late MRI (P < .01), and 2.36 ± 0.07 cm vs 3.28 ± 0.05 cm on postnatal radiographs (P < .01). Adjusting the ETT depth by 1 cm resulted in a median distance of 1.27 cm from the tip of the ETT to the carina. CONCLUSION: Cephalad displacement of the carina in infants with CDH may predispose them to right main-stem intubation and subsequent development of pneumothorax. We speculate that modifying the ETT insertion depth to 5.5 cm + weight in newborns born at term may prevent pneumothoraces and improve outcomes for infants with CDH.


Asunto(s)
Hernias Diafragmáticas Congénitas/diagnóstico por imagen , Imagen por Resonancia Magnética , Diagnóstico Prenatal , Tráquea/anomalías , Estudios de Casos y Controles , Femenino , Hernias Diafragmáticas Congénitas/embriología , Hernias Diafragmáticas Congénitas/patología , Hernias Diafragmáticas Congénitas/terapia , Humanos , Recién Nacido , Intubación Intratraqueal/métodos , Masculino , Embarazo , Tráquea/diagnóstico por imagen , Tráquea/embriología
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