RESUMEN
PURPOSE: We aimed to identify risk factors for neonatal surgical airway intervention among fetuses with prenatally diagnosed cervical masses. METHODS: An 8-year retrospective review identified 23 consecutive patients with a prenatal diagnosis of a neck mass, managed at a single tertiary center. Variables analyzed included anticipated diagnosis, extent of the mass, need for any surgical airway intervention in the neonatal period, final histopathology data, and survival. Statistical analysis was based on the Fisher and Fisher-Freeman-Halton exact tests (significance set at P < or = .05) and exact 95% confidence intervals for risk differences. RESULTS: Eight patients underwent termination of pregnancy or were lost to follow-up. The imaging-based prenatal diagnosis was confirmed postnatally in 93% (14/15) of the remaining patients. Final diagnoses included lymphatic malformation (8), teratoma (6), and esophageal duplication (1). Teratomas were associated with a significantly higher risk for neonatal airway intervention than lymphatic malformations (67% vs 11%, P = .02). The majority of such procedures were performed under ex utero intrapartum treatment. Survival was 93% (14/15). CONCLUSIONS: Cervical teratomas are significantly more likely to demand surgical airway intervention in the neonate, typically under ex utero intrapartum treatment, than cervical lymphatic malformations. These findings should be considered in the prenatal counseling for fetal cervical masses.
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Neoplasias de Cabeza y Cuello/congénito , Neoplasias de Cabeza y Cuello/diagnóstico por imagen , Sistema Linfático/anomalías , Teratoma/congénito , Teratoma/diagnóstico por imagen , Intervalos de Confianza , Femenino , Humanos , Recién Nacido , Embarazo , Estudios Retrospectivos , Factores de Riesgo , Tasa de Supervivencia , Resultado del Tratamiento , Ultrasonografía PrenatalRESUMEN
OBJECTIVE: The first objective of our study was to describe the prevalence and spectrum of posterior fossa anomalies over 5 years in a major fetal care center where the referral diagnosis (by fetal sonography) was investigated by fetal MRI and, if confirmed, by postnatal MRI if possible. The second objective was to assess the accuracy with which fetal MRI predicts postnatal MRI findings in this population. MATERIALS AND METHODS: We retrospectively identified all cases of suspected fetal posterior fossa anomalies referred to our center from 2002 through 2006. We reviewed maternal, fetal, neonatal, and follow-up records of all cases and fetal and early postnatal imaging studies. RESULTS: Of the 90 cases of suspected fetal posterior fossa anomalies (by fetal sonography) referred over the study period, 60 (67%) were confirmed by fetal MRI. Of 42 live-born infants, 39 (93%) underwent postnatal MRI. There was complete agreement in fetal and postnatal MRI diagnoses in 23 infants (59%). In 16 cases (41%), fetal and postnatal MRI diagnoses disagreed; postnatal MRI excluded fetal MRI diagnoses in six cases (15%) and revealed additional anomalies in 10 cases (26%). CONCLUSION: Although a valuable adjunct to fetal sonography in cases of suspected posterior fossa anomaly, current fetal MRI, particularly in early gestation, has limitations in accurately predicting postnatal MRI abnormalities. Advancing the accuracy of MRI for the diagnosis of posterior fossa anomalies will require greater understanding of normal brain development and improved tissue resolution of fetal MRI. During the interim, our findings strongly support the need for postnatal MRI follow-up in cases with suspected posterior fossa anomalies by fetal MRI.
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Fosa Craneal Posterior/anomalías , Fosa Craneal Posterior/patología , Anomalías Craneofaciales/diagnóstico , Anomalías Craneofaciales/epidemiología , Imagen por Resonancia Magnética/estadística & datos numéricos , Medición de Riesgo/métodos , Ultrasonografía Prenatal/estadística & datos numéricos , Boston/epidemiología , Fosa Craneal Posterior/diagnóstico por imagen , Femenino , Humanos , Incidencia , Recién Nacido , Masculino , Pronóstico , Reproducibilidad de los Resultados , Factores de Riesgo , Sensibilidad y EspecificidadRESUMEN
OBJECTIVE: To determine whether any common maternal-fetal variable has prenatal predictive value of prosthetic repair in congenital diaphragmatic hernia. METHODS: This was a 5-year single-center retrospective review of fetal congenital diaphragmatic hernia referrals. Multiple prenatal variables were correlated with the need for a prosthetic repair. Statistical analyses were by Fisher's exact and Mann-Whitney U-tests, as appropriate (p < 0.05). RESULTS: Fetal liver position was a predictor of prosthetic repair. The presence or absence of liver herniation was correlated with prosthetic repair rates of 83.3 and 23.1%, respectively (p < 0.001). All patients with moderate/severe liver herniation required a prosthetic patch. CONCLUSION: Liver herniation has prenatal predictive value for the need for prosthetic repair in congenital diaphragmatic hernia. This finding should be valuable during prenatal counseling for clinical trials of engineered diaphragmatic repair.
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Hernia Diafragmática/cirugía , Hernias Diafragmáticas Congénitas , Hígado/patología , Materiales Biocompatibles , Femenino , Hernia Diafragmática/diagnóstico , Humanos , Recién Nacido , Masculino , Politetrafluoroetileno , Embarazo , Diagnóstico Prenatal , Pronóstico , Estudios Retrospectivos , Mallas Quirúrgicas , Ingeniería de TejidosRESUMEN
PURPOSE: The prognosis for fetuses with large congenital cystic adenomatoid malformations (CCAMs) remains uncertain. This study examined the natural history of large fetal CCAMs managed expectantly at a major referral center. METHODS: A 5-year retrospective review was conducted on fetuses diagnosed with a thoracic lesion (n = 59). Large CCAMs were identified on prenatal imaging and followed longitudinally. Perinatal outcomes were assessed. RESULTS: Twelve (20.3%) fetuses had large CCAMs in the absence of other congenital anomalies. Peak CCAM size occurred at 25.3 +/- 3.6 weeks' gestation. Serial magnetic resonance volumetry demonstrated a trend toward decreasing CCAM mass volume relative to thoracic cavity volume over time. Overall, 6 patients, including 3 with signs of early hydrops, showed a marked regression of their lesions relative to estimated fetal weight. Five fetuses required an emergent intervention postnatally, including extracorporeal membrane oxygenation support (n = 1), cyst aspiration (n = 1), and lung resection (n = 5). Overall survival was 75%, with severe hydrops before 30 weeks seen in all 3 deaths. CONCLUSION: Large fetal CCAMs tend to peak in size at 25 weeks' gestation and are characterized by in utero diminution relative to overall fetal growth. The prognosis for most fetuses with large CCAMs remains quite favorable under careful perinatal management.
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Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Muerte Fetal/epidemiología , Desarrollo Fetal/fisiología , Resultado del Embarazo , Estudios de Cohortes , Malformación Adenomatoide Quística Congénita del Pulmón/mortalidad , Femenino , Monitoreo Fetal , Edad Gestacional , Humanos , Hidropesía Fetal/diagnóstico , Hidropesía Fetal/mortalidad , Recién Nacido , Imagen por Resonancia Magnética , Masculino , Embarazo , Diagnóstico Prenatal/métodos , Estudios Retrospectivos , Medición de Riesgo , Índice de Severidad de la Enfermedad , Tasa de Supervivencia , Tomografía Computarizada por Rayos X , Ultrasonografía PrenatalRESUMEN
PURPOSE: The purpose of this study was to determine whether ex utero intrapartum treatment with extracorporeal membrane oxygenation (EXIT to ECMO) is a reasonable approach for managing patients antenatally diagnosed with severe congenital diaphragmatic hernia (CDH). METHODS: A 6-year retrospective review was performed on fetuses with severe CDH (liver herniation and a lung/head ratio <1.4, percentage of predicted lung volume <15, and/or congenital heart disease). Fourteen of the patients underwent EXIT with a trial of ventilation. Fetuses with poor preductal oxygen saturations despite mechanical ventilation received ECMO before their delivery. Maternal-fetal outcomes were analyzed. RESULTS: There were no maternal-reported complications. Three babies passed the ventilation trial and survived, but 2 of them required ECMO within 48 hours. The remaining 11 fetuses received ECMO before their delivery. Overall survival after EXIT-to-ECMO was 64%. At 1-year follow-up, all survivors had weaned off supplemental oxygen, but 57% required diuretics and/or bronchodilators. CONCLUSION: This is the largest reported experience using EXIT to ECMO in the management of severe CDH. The EXIT-to-ECMO procedure is associated with favorable survival rates and acceptable pulmonary morbidity in fetuses expected to have a poor prognosis under conventional management.
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Oxigenación por Membrana Extracorpórea , Terapias Fetales/métodos , Hernia Diafragmática/cirugía , Hernias Diafragmáticas Congénitas , Respiración Artificial , Adulto , Femenino , Hernia Diafragmática/diagnóstico , Humanos , Masculino , Embarazo , Resultado del Embarazo , Diagnóstico Prenatal , Estudios Retrospectivos , Análisis de Supervivencia , Resultado del TratamientoRESUMEN
PURPOSE: This study was aimed at determining whether a new method of analyzing lung volumes on fetal magnetic resonance (MR) imaging could be used to predict the degree of pulmonary compromise in congenital diaphragmatic hernia (CDH). METHODS: Seventeen fetuses with CDH were prospectively evaluated by MR. Lung volumes were measured using an established technique and expressed as a percentage of the predicted lung volume (PPLV). Predicted lung volume was determined by subtracting measured mediastinal volume from total measured thoracic volume. The PPLV was correlated with postnatal outcomes. Statistical analyses were performed using the Mann-Whitney, Spearman correlation, or Fisher exact tests (P < .05). RESULTS: Of the 14 liveborn patients, the PPLV was 20.3+/-10.4 (gestational age at MR, 22.3 +/- 5.7 weeks). The PPLV was significantly associated with extracorporeal membrane oxygenation (ECMO) use, hospital length of stay, and survival. All patients with a PPLV of less than 15 required prolonged ECMO support and had a 40% survival rate. In contrast, only 11% of patients with a PPLV of greater than 15 required ECMO, and survival was 100%. CONCLUSION: The PPLV as measured by fetal MR imaging can accurately predict disease severity in CDH. A value of less than 15 is associated with a significantly higher risk for prolonged support and/or death, despite aggressive postnatal management.
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Hernias Diafragmáticas Congénitas , Enfermedades Pulmonares/diagnóstico , Mediciones del Volumen Pulmonar , Femenino , Hernia Diafragmática/complicaciones , Humanos , Enfermedades Pulmonares/etiología , Imagen por Resonancia Magnética , Valor Predictivo de las Pruebas , Embarazo , Estudios Prospectivos , Medición de RiesgoRESUMEN
PURPOSE: This study was aimed at determining whether different congenital lung masses represent diverse manifestations of a single developmental abnormality associated with fetal airway obstruction. METHODS: We conducted a 3-year retrospective review of patients who underwent surgical resection of a prenatally diagnosed lung mass. Prenatal imaging was used to define mass position and its effect on adjacent organs. Lung specimens were examined through careful full-specimen microdissections, as well as by plain and contrast roentgenograms. RESULTS: Twenty-five patients underwent lung resection during this study period. Based on the final pathology reports, 56% were congenital cystic adenomatoid malformations, 12% were congenital lobar emphysemas, 8% were bronchopulmonary sequestrations, and 24% had features of both cystic adenomatoid malformation and bronchopulmonary sequestrations. No bronchogenic cysts were present in this series. Overall, bronchial atresia was identified in 77% of the examined specimens (n = 22) and was associated with all types of lung malformations. CONCLUSIONS: Bronchial atresia is a common, unrecognized component of prenatally diagnosed congenital cystic adenomatoid malformations, bronchopulmonary sequestrations, congenital lobar emphysemas, and lesions of mixed pathology. Most congenital lung masses may be part of a spectrum of anomalies linked to obstruction of the developing fetal airway as an underlying component in their pathogenesis.
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Enfermedades Fetales/etiología , Enfermedades Fetales/patología , Enfermedades Pulmonares/complicaciones , Diagnóstico Prenatal , Atresia Pulmonar/etiología , Atresia Pulmonar/patología , Obstrucción de las Vías Aéreas/etiología , Humanos , Recién Nacido , Atresia Pulmonar/diagnóstico por imagen , Radiografía Torácica , Estudios RetrospectivosRESUMEN
BACKGROUND/PURPOSE: Congenital diaphragmatic hernia (CDH) is a condition associated with significant mortality. This study examines the survival rate of neonates with CDH treated by a multidisciplinary team in a single pediatric hospital. Actual survival rate is compared with predicted outcome based on severity of illness. METHODS: A consecutive series of neonates with CDH was evaluated beginning January 1, 2000. There were no excluded patients. A treatment protocol emphasizing minimal barotrauma and control of pulmonary hypertension was used. Predicted survival rate was estimated using birth weight and Apgar score at 5 minutes per the logistic regression equation published by the CDH Study Group. Actual survival rates were calculated and compared with predicted values using a one sample binomial test with significance deemed to be present at P less than.05. RESULTS: Thirty-six of 39 (93%) neonates with CDH survived, whereas the predicted survival rate for this cohort was 68% (P <.001). Twelve of 14 (86%) neonates requiring extracorporeal membrane oxygenation (ECMO) survived, despite a predicted survival rate of 52% for this subset (P <.01). Twenty-five percent of survivors required supplemental oxygen at home. CONCLUSIONS: Using a simple analysis, centers may now objectively compare their CDH survival rates with predicted outcomes adjusted for disease severity. This technique confirmed that a consecutive series of neonates with CDH, treated in one hospital, had a significantly higher than predicted survival rate. Thus, it may be possible to identify other centers with higher than expected survival rates and to define common therapeutic strategies associated with decreased mortality.