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1.
J Pediatr ; 271: 114053, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38615944

RESUMEN

The Renal Anhydramnios Fetal Therapy (RAFT) trial is a study of serial amnioinfusions to prevent lethal neonatal pulmonary hypoplasia from early renal anhydramnios. Infant neurologic outcomes were not originally evaluated. We describe the high incidence of stroke observed among infants in the treatment arm of the trial at our center.


Asunto(s)
Accidente Cerebrovascular , Humanos , Femenino , Incidencia , Accidente Cerebrovascular/epidemiología , Embarazo , Recién Nacido , Masculino , Terapias Fetales/métodos , Oligohidramnios/epidemiología , Enfermedades Pulmonares/epidemiología , Enfermedades Pulmonares/etiología , Pulmón/diagnóstico por imagen , Pulmón/anomalías , Anomalías Múltiples
2.
Prenat Diagn ; 44(6-7): 739-746, 2024 06.
Artículo en Inglés | MEDLINE | ID: mdl-38666896

RESUMEN

BACKGROUND: Fetal aortic valvuloplasty (FAV) is proposed to prevent hypoplastic left heart syndrome due to fetal critical aortic stenosis. OBJECTIVE: to report our experience on FAV as the first step in a complex therapeutic strategy. METHOD: Series of patients with FAV over an 18-year period. RESULTS: 27 FAVs were performed in 26 fetuses, with technical success in 82% (22/27) and periprocedural fetal demise in 22% (6/27), decreasing to 15% in the second half-cohort. Loss to follow-up was due to birth or postnatal therapy in other centers (5) and termination of pregnancy (1), A normal-sized LV at birth was observed in 46% (6/13), 4 neonates underwent aortic valvuloplasty and 2 cardiac surgeries, with 5/6 achieving biventricular circulation at 28 days, and 3 transplant-free survival at mid-term follow-up. The 7/13 born with a borderline LV underwent LV rehabilitation strategy, with survival at 28 days in 4/7 and at mid-term in 3: one with biventricular circulation, one with a ventricle-and-a-half repair, and one lost to follow-up. CONCLUSION: FAV was feasible in most cases, with no maternal complications, and biventricular circulation at 28 days in ∼40% of survivors. After FAV, a diverse range of postnatal cardiac interventions are performed, reflecting the challenging innovation in current cardiovascular therapy.


Asunto(s)
Estenosis de la Válvula Aórtica , Humanos , Femenino , Embarazo , Estenosis de la Válvula Aórtica/cirugía , Estenosis de la Válvula Aórtica/terapia , Síndrome del Corazón Izquierdo Hipoplásico/terapia , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Valvuloplastia con Balón/métodos , Ultrasonografía Prenatal , Recién Nacido , Estudios Retrospectivos , Enfermedades Fetales/terapia , Adulto , Terapias Fetales/métodos
3.
Pediatr Neurosurg ; 59(2-3): 87-93, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38342093

RESUMEN

INTRODUCTION: Open spina bifida (OSB) is the most common congenital anomaly of the central nervous system. It is associated with severe neurodevelopmental delay, motor impairment, hydrocephalus, and bowel and bladder dysfunction. In selected cases, intrauterine spina bifida repair has been shown to improve neonatal outcomes. Rarely, the spine can have a double defect compromising two different segments and there is a lack of evidence on the feasibility and benefits of intrauterine repair in these cases. CASE PRESENTATION: We present a case with both cervicothoracic and lumbosacral myelomeningocele, Arnold-Chiari malformation type II and bilateral ventriculomegaly, that was treated successfully at 25 weeks with open micro-neurosurgery. Double myelomeningocele was successfully treated through a single 2-cm micro-hysterotomy, by performing external versions to sequentially expose and repair both defects. Weekly postoperative follow-up showed no progression of ventriculomegaly or complications attributable to the procedure. Preterm rupture of membranes prompted a conventional cesarean delivery at 32 weeks of gestation. Neurodevelopmental outcome at 20 months was within normal ranges, having achieved ambulation without orthopedic support and with no need for ventriculoperitoneal shunting. CONCLUSION: This report demonstrates for the first time the feasibility of double OSB repair through a single 2-cm micro-hysterotomy, suggesting that selected isolated cases of double myelomeningocele could be candidates for fetal intervention. Further prospective studies should be carried out to assess the potential benefit of double OSB intrauterine open repair.


Asunto(s)
Histerotomía , Meningomielocele , Humanos , Meningomielocele/cirugía , Meningomielocele/diagnóstico por imagen , Femenino , Histerotomía/métodos , Embarazo , Recién Nacido , Malformación de Arnold-Chiari/cirugía , Malformación de Arnold-Chiari/diagnóstico por imagen , Adulto , Terapias Fetales/métodos
4.
Prenat Diagn ; 41(13): 1615-1623, 2021 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-34676573

RESUMEN

BACKGROUND/OBJECTIVES: Prenatal myelomeningocele (MMC) repair has been shown to improve neurological outcomes. It has been suggested that decreases in the hysterotomy diameter during surgery can improve perinatal outcomes without altering neurologic outcomes. The objective of this study is to describe and compare the main maternal and fetal outcomes of fetuses undergoing open surgery for MMC repair, through the different modifications (standard-classical, mini-hysterotomy, and microneurosurgery). DATA SOURCE: MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, Ovid, SciELO, LILACS, PROSPERO. RESULTS: From a total of 112 studies, seven case series were selected including 399 fetuses with open fetal surgery, five studies using the classical technique (n = 181), one with mini-hysterotomy (n = 176), and one with the microneurosurgery technique (n = 42). The mini-hysterotomy and microneurosurgery techniques presented a lower risk of preterm delivery (21.4% and 30%, respectively) compared to the classic technique (47.3%), premature rupture of membranes (78%, 62%, and 72.5 %, respectively), oligohydramnios (0% and 72.5%, respectively), dehiscence of hysterotomy, maintaining the same frequency of Chiari reversion (78%, 62%, and 72.5%, respectively), postnatal correction requirement (0%, 4.8%, and 5.8%, respectively), and lower frequency of requirement for a ventriculoperitoneal shunt placement (13.0%, 7.5%, and 29.1%, respectively). CONCLUSION: The least invasive techniques (minihysterotomy-microneurosurgery) are possible and reproduceable, as they are associated with better maternal and perinatal outcomes.


Asunto(s)
Terapias Fetales/normas , Edad Gestacional , Disrafia Espinal/cirugía , Derivación Ventriculoperitoneal/métodos , Adulto , Femenino , Terapias Fetales/métodos , Fetoscopía/métodos , Humanos , Embarazo , Derivación Ventriculoperitoneal/tendencias
5.
Prenat Diagn ; 41(13): 1634-1642, 2021 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-34583428

RESUMEN

OBJECTIVE: To analyze the impact of gestational age (GA) at the time of fetal open spinal dysraphism (OSD) repair through a mini-hysterotomy on the ability of children to walk. METHODS: Children who underwent in utero repair of OSD and had formal neurological assessment after 2.5 years of age were compared regarding their ability to walk in relation to pre-surgical predictors. RESULTS: Sixty-nine children fulfilled the inclusion criteria. Among them, 63.7% (44/69) were able to walk with or without orthesis. Fetal OSD correction performed earlier in gestation (from 19.7 to 26.9 weeks) was associated with a higher probability of walking with or without orthesis (p = 0.033). The median GA at delivery was 35.3 weeks. Multivariate binary logistic regression showed that the upper anatomical level of the OSD ( L5) (p < 0.004; OR: 10.31 [95% CI: 2.07-51.28]) and GA at the time of fetal surgery (p = 0.026; OR = 0.68 [95% CI: 0.48-0.95]) were independent predictors of the postnatal ability to walk with or without orthesis. CONCLUSION: Fetuses with OSD who were operated on earlier in pregnancy (range: 19.7-26.9 weeks), were more likely to walk with or without orthesis.


Asunto(s)
Edad Gestacional , Histerectomía/normas , Disrafia Espinal/cirugía , Caminata/estadística & datos numéricos , Adulto , Niño , Femenino , Terapias Fetales/métodos , Terapias Fetales/normas , Terapias Fetales/estadística & datos numéricos , Humanos , Histerectomía/métodos , Histerectomía/estadística & datos numéricos , Embarazo , Estudios Retrospectivos , Disrafia Espinal/complicaciones
7.
Arch. argent. pediatr ; 118(2): e149-e161, abr. 2020. tab, ilus
Artículo en Inglés, Español | LILACS, BINACIS | ID: biblio-1100327

RESUMEN

Dentro de las malformaciones congénitas, las cardiopatías son las anomalías más frecuentes y se asocian a una elevada morbimortalidad perinatal y a largo plazo. El objetivo de esta actualización es revisar la tasa de detección prenatal, las características del tamizaje a lo largo del embarazo, tanto en el primero como en el segundo trimestre, las indicaciones de ecocardiografía avanzada, y establecer un algoritmo de manejo ante el diagnóstico prenatal de una cardiopatía congénita. Se discutirán los estudios invasivos y no invasivos que pueden realizarse y el seguimiento obstétrico. Finalmente, se revisarán las características principales de la terapia fetal en anomalías cardíacas, tanto intervencionismo cardíaco como el tratamiento intrauterino de las arritmias.


Among congenital malformations, heart defects are the most common type of anomaly, and these are associated with a high perinatal, long-term morbidity and mortality. The objective of this update was to review the rate of prenatal detection, screening characteristics throughout the pregnancy, in both the first and second trimesters, indications for advanced echocardiography, and to establish a management algorithm in case of prenatal diagnosis of a congenital heart disease. Potential invasive and non-invasive tests and obstetric follow-up will be discussed here. Finally, the main characteristics of fetal therapy in heart anomalies will be reviewed, both cardiac interventions and intrauterine treatment of arrhythmias.


Asunto(s)
Humanos , Masculino , Femenino , Embarazo , Cardiopatías Congénitas/diagnóstico por imagen , Diagnóstico Prenatal , Tamizaje Masivo , Estudios de Seguimiento , Terapias Fetales/métodos , Corazón Fetal/anomalías
8.
Arch Argent Pediatr ; 118(2): e149-e161, 2020 04.
Artículo en Inglés, Español | MEDLINE | ID: mdl-32199055

RESUMEN

Among congenital malformations, heart defects are the most common type of anomaly, and these are associated with a high perinatal, longterm morbidity and mortality. The objective of this update was to review the rate of prenatal detection, screening characteristics throughout the pregnancy, in both the first and second trimesters, indications for advanced echocardiography, and to establish a management algorithm in case of prenatal diagnosis of a congenital heart disease. Potential invasive and non-invasive tests and obstetric follow-up will be discussed here. Finally, the main characteristics of fetal therapy in heart anomalies will be reviewed, both cardiac interventions and intrauterine treatment of arrhythmias.


Dentro de las malformaciones congénitas, las cardiopatías son las anomalías más frecuentes y se asocian a una elevada morbimortalidad perinatal y a largo plazo. El objetivo de esta actualización es revisar la tasa de detección prenatal, las características del tamizaje a lo largo del embarazo, tanto en el primero como en el segundo trimestre, las indicaciones de ecocardiografía avanzada, y establecer un algoritmo de manejo ante el diagnóstico prenatal de una cardiopatía congénita. Se discutirán los estudios invasivos y no invasivos que pueden realizarse y el seguimiento obstétrico. Finalmente, se revisarán las características principales de la terapia fetal en anomalías cardíacas, tanto intervencionismo cardíaco como el tratamiento intrauterino de las arritmias.


Asunto(s)
Terapias Fetales/métodos , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/terapia , Diagnóstico Prenatal/métodos , Femenino , Humanos , Embarazo
9.
Prenat Diagn ; 40(6): 689-697, 2020 05.
Artículo en Inglés | MEDLINE | ID: mdl-32112579

RESUMEN

OBJECTIVE: To analyze the impact of gestational age (GA) at the time of fetal open spinal dysraphism (OSD) repair through a mini-hysterotomy on the perinatal outcomes and the infants' ventriculoperitoneal shunt rates. METHODS: Retrospective study of cases of fetal OSD correction performed from 2014 and 2019. RESULTS: One hundred and ninety women underwent fetal surgery for OSD through a mini-hysterotomy, and 176 (176/190:92.6%) have since delivered. Fetal OSD correction performed earlier in the gestational period, ranging from 19.7 to 26.9 weeks, was associated with lower rates of postnatal ventriculoperitoneal shunting (P: .049). Earlier fetal surgeries were associated with shorter surgical times (P: .01), smaller hysterotomy lengths (P < .001), higher frequencies of hindbrain herniation reversal (P: .003), and longer latencies from surgery to delivery (P < .001). Median GA at delivery was 35.3 weeks. Multivariate binary logistic regression showed that both fetal lateral ventricle-to-hemisphere ratio (%; P < .001; OR: 1.14 [95% CI: 1.09-1.21]) and GA at the time of fetal surgery (P: .016; OR: 1.37 [95% CI: 1.07-1.77]) were independent predictors of postnatal ventriculoperitoneal shunting. CONCLUSION: Fetuses with OSD who were operated on earlier in the gestational interval, which ranged from 19.7 to 26.9 weeks, were less prone to receiving postnatal ventriculoperitoneal shunts.


Asunto(s)
Terapias Fetales/métodos , Edad Gestacional , Procedimientos Neuroquirúrgicos/métodos , Espina Bífida Quística/cirugía , Derivación Ventriculoperitoneal/estadística & datos numéricos , Adolescente , Adulto , Malformación de Arnold-Chiari/complicaciones , Malformación de Arnold-Chiari/diagnóstico por imagen , Femenino , Humanos , Histerotomía/métodos , Recién Nacido , Masculino , Persona de Mediana Edad , Embarazo , Espina Bífida Quística/complicaciones , Espina Bífida Quística/diagnóstico por imagen , Resultado del Tratamiento , Adulto Joven
10.
Prenat Diagn ; 40(1): 58-65, 2020 01.
Artículo en Inglés | MEDLINE | ID: mdl-31306500

RESUMEN

Fetal severe central nervous system ventriculomegaly is associated with poor neurologic outcomes, usually driven by a primary malformation, deformation, or disruption of brain parenchyma. In utero shunting of excess cerebrospinal fluid (CSF) in hopes of improving neurologic outcomes was attempted in the 1980s but was abandoned due to perceived lack of effect, likely due to technological limitations of the time that precluded proper patient selection. Little progress on the antenatal management of severe ventriculomegaly has been made in the intervening decades. A multidisciplinary, evidence-based reassessment of ventriculoamniotic shunting for isolated fetal aqueductal stenosis (FAS), a unique form of severe ventriculomegaly (supratentorial intracranial hypertension), is currently underway. An accurate diagnosis of FAS must precede in utero intervention. Magnetic resonance imaging (MRI) will be an excellent adjunct to high-resolution prenatal ultrasound and next-generation genetic testing to correctly diagnose FAS in a timely fashion while excluding other intracranial and extracranial anomalies. This manuscript will briefly discuss the history, current management, and future directions of the prenatal diagnosis and potential intervention for FAS.


Asunto(s)
Derivaciones del Líquido Cefalorraquídeo/métodos , Enfermedades Fetales/diagnóstico por imagen , Terapias Fetales/métodos , Hidrocefalia/diagnóstico por imagen , Femenino , Enfermedades Fetales/cirugía , Humanos , Hidrocefalia/cirugía , Imagen por Resonancia Magnética , Embarazo , Diagnóstico Prenatal , Ultrasonografía Prenatal
11.
Fetal Diagn Ther ; 46(5): 319-322, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-30884481

RESUMEN

INTRODUCTION: After the successful results of in utero myelomeningocele (MMC) repair presented by the Management of Myelomeningocele Study, the concept of fetal surgery was introduced in our institution in 2011. Since then, we have been able to follow prospectively a group of patients with attention to urological care. In the present study, we were interested in estimating urinary and fecal continence in this new subset of patients. MATERIAL AND METHODS: We selected from our database patients aged 5 years or older for evaluation of urinary and fecal continence. We reviewed all charts and completed a questionnaire to study aspects of urinary and fecal continence. RESULTS: We identified 14 patients, i.e., 4 (28.6%) males and 10 (71.4%) females. The mean age at MMC surgery was 25.6 gestational weeks. The uro-dynamic class was high-risk in 6 (42.9%), incontinent in 4 (28.6%), hypocontractile in 1 (7.1%), and normal in 3 (21.4%) patients. Three patients had undergone surgery (2 augmentations, i.e., 1 in association with a left colon ACE Macedo-Malone procedure and 1 mini-sling urethroplasty). Twelve patients underwent clean intermittent catheterization (CIC) (85.7%). Only 3 (21.4%) patients had no urinary leakage. Eleven patients (78.6%) used diapers. Eight patients (57.2%) underwent retrograde rectal irrigation and 11 (78.6%) complained of fecal loss. Eleven patients (78.6%) did not report an impact on their self-esteem. CONCLUSION: Despite the use of CIC in 85.7% of the cases, the continence rate in MMC patients operated on in utero was low and 78.6% of the patients used diapers. This data can be used to educate parents about future conditions of their 5-year-old children and may stimulate the debate regarding further attempts (surgical or not) to improve fecal and urinary continence.


Asunto(s)
Incontinencia Fecal/etiología , Terapias Fetales/métodos , Meningomielocele/cirugía , Incontinencia Urinaria/etiología , Procedimientos Quirúrgicos Urogenitales , Factores de Edad , Preescolar , Bases de Datos Factuales , Defecación , Incontinencia Fecal/fisiopatología , Incontinencia Fecal/terapia , Femenino , Terapias Fetales/efectos adversos , Edad Gestacional , Humanos , Masculino , Meningomielocele/complicaciones , Meningomielocele/diagnóstico por imagen , Meningomielocele/fisiopatología , Estudios Prospectivos , Factores de Riesgo , Resultado del Tratamiento , Ultrasonografía Prenatal , Incontinencia Urinaria/fisiopatología , Incontinencia Urinaria/terapia , Urodinámica , Procedimientos Quirúrgicos Urogenitales/efectos adversos
12.
BJU Int ; 123(4): 676-681, 2019 04.
Artículo en Inglés | MEDLINE | ID: mdl-30548158

RESUMEN

OBJECTIVES: To evaluate the first 100 cases of in utero myelomeningocoele (MMC) repair and urological outcomes in a prospective analysis aiming to define possible improvement in bladder function. PATIENTS AND METHODS: We used a protocol consisting of a detailed medical history, urinary tract ultrasonography, voiding cystourethrography, and urodynamic evaluation. Patients were categorised into four groups: normal, high risk (overactive bladder with a detrusor leak-point pressure >40 cm H2 O and high filling pressures also >40 cm H2 O), incontinent, and underactivity (underactive bladder with post-void residual urine), and patients were treated accordingly. RESULTS: We evaluated 100 patients, at a mean age of 5.8 months (median 4 months), classified as high risk in 52.6%, incontinent in 27.4%, with underactive bladder in 4.2%, and only 14.7% had a normal bladder profile. Clean intermittent catheterisation was initiated in 57.3% of the patients and anticholinergics in 52.6%. Antibiotic prophylaxis was initiated in 19.1% of the patients presenting with vesico-ureteric reflux. CONCLUSION: The high incidence of abnormal bladder patterns suggests little benefit of in utero MMC surgery concerning the urinary tract.


Asunto(s)
Terapias Fetales/métodos , Meningomielocele/fisiopatología , Ultrasonografía Prenatal , Urodinámica/fisiología , Reflujo Vesicoureteral/fisiopatología , Femenino , Humanos , Lactante , Recién Nacido , Meningomielocele/diagnóstico por imagen , Meningomielocele/embriología , Meningomielocele/cirugía , Estudios Prospectivos , Reflujo Vesicoureteral/diagnóstico por imagen , Reflujo Vesicoureteral/embriología
13.
Bol Med Hosp Infant Mex ; 75(2): 119-126, 2018.
Artículo en Español | MEDLINE | ID: mdl-29658947

RESUMEN

Introduction: Congenital pulmonary malformations are a rare cause of neonatal morbidity. Some of them have a common origin, which allows the identification of combined lesions. Its diagnosis can be made prenatally by ultrasound, with the limitation that this study is performed in specialized centers and depends on the expertise of the operator. The association of pulmonary sequestration and congenital malformation of the airway has been described in approximately 40-60 cases since its first description in 1949. Many lesions are not perceptible in intrauterine life and in the neonatal period there are recurrent respiratory symptoms that in some cases are associated with a congenital pulmonary malformation. Case report: We report the case of a young infant, who was diagnosed with pulmonary sequestration at 24 weeks of gestational age, undergoing intrauterine surgical treatment with a report of complete resolution of the malformation in posterior ultrasounds. She was valued by pediatric pneumology at 4 months of age, where angiotomography was performed and the presence of pulmonary sequestration was confirmed by lobectomy. The histopathological study reported extralobar pulmonary sequestration with congenital malformation of the pulmonary airway type 2. These combined lesions were identified by histopathological study. The treatment of choice was surgical. Conclusions: Upon the confirmation of a malformation, we emphasize the importance of performing a screening in order to search for other that could be associated.


Introducción: Las malformaciones pulmonares congénitas son una causa poco frecuente de morbilidad neonatal. Algunas de ellas tienen un origen común, lo que permite identificar lesiones combinadas. Su diagnóstico puede realizarse de forma prenatal mediante ultrasonido, con las limitaciones de que solo se realiza en centros especializados y que depende de la pericia del operador. La asociación entre el secuestro pulmonar y la malformación congénita de la vía aérea se ha descrito aproximadamente en 40-60 casos desde 1949, cuando se observó por primera vez. Muchas lesiones no son perceptibles en la vida intrauterina. Sin embargo, en el periodo neonatal se presentan síntomas respiratorios recurrentes que en algunos casos están asociados con una malformación pulmonar. Caso clínico: Se presenta el caso de una lactante diagnosticada con secuestro pulmonar a las 24 semanas de edad gestacional. Recibió tratamiento quirúrgico intrauterino con reporte de resolución completa de la malformación en ultrasonidos posteriores. Fue valorada por neumología pediátrica a los 4 meses de edad. Se realizó una angiotomografía y se confirmó la presencia de secuestro pulmonar, por lo que se realizó una lobectomía. El estudio histopatológico reportó secuestro pulmonar extralobar con malformación congénita de la vía aérea pulmonar tipo 2. Estas lesiones combinadas se identificaron mediante un estudio histopatológico. El tratamiento de elección fue quirúrgico. Conclusiones: Ante la confirmación de una malformación, destaca la importancia de realizar la búsqueda de otras malformaciones que pudieran estar asociadas.


Asunto(s)
Secuestro Broncopulmonar/diagnóstico , Diagnóstico Prenatal/métodos , Anomalías del Sistema Respiratorio/diagnóstico , Secuestro Broncopulmonar/cirugía , Angiografía por Tomografía Computarizada/métodos , Femenino , Terapias Fetales/métodos , Edad Gestacional , Humanos , Lactante , Neumonectomía/métodos , Anomalías del Sistema Respiratorio/cirugía
14.
Bol. méd. Hosp. Infant. Méx ; 75(2): 119-126, mar.-abr. 2018. graf
Artículo en Español | LILACS | ID: biblio-951299

RESUMEN

Resumen Introducción: Las malformaciones pulmonares congénitas son una causa poco frecuente de morbilidad neonatal. Algunas de ellas tienen un origen común, lo que permite identificar lesiones combinadas. Su diagnóstico puede realizarse de forma prenatal mediante ultrasonido, con las limitaciones de que solo se realiza en centros especializados y que depende de la pericia del operador. La asociación entre el secuestro pulmonar y la malformación congénita de la vía aérea se ha descrito aproximadamente en 40-60 casos desde 1949, cuando se observó por primera vez. Muchas lesiones no son perceptibles en la vida intrauterina. Sin embargo, en el periodo neonatal se presentan síntomas respiratorios recurrentes que en algunos casos están asociados con una malformación pulmonar. Caso clínico: Se presenta el caso de una lactante diagnosticada con secuestro pulmonar a las 24 semanas de edad gestacional. Recibió tratamiento quirúrgico intrauterino con reporte de resolución completa de la malformación en ultrasonidos posteriores. Fue valorada por neumología pediátrica a los 4 meses de edad. Se realizó una angiotomografía y se confirmó la presencia de secuestro pulmonar, por lo que se realizó una lobectomía. El estudio histopatológico reportó secuestro pulmonar extralobar con malformación congénita de la vía aérea pulmonar tipo 2. Estas lesiones combinadas se identificaron mediante un estudio histopatológico. El tratamiento de elección fue quirúrgico. Conclusiones: Ante la confirmación de una malformación, destaca la importancia de realizar la búsqueda de otras malformaciones que pudieran estar asociadas.


Abstract Introduction: Congenital pulmonary malformations are a rare cause of neonatal morbidity. Some of them have a common origin, which allows the identification of combined lesions. Its diagnosis can be made prenatally by ultrasound, with the limitation that this study is performed in specialized centers and depends on the expertise of the operator. The association of pulmonary sequestration and congenital malformation of the airway has been described in approximately 40-60 cases since its first description in 1949. Many lesions are not perceptible in intrauterine life and in the neonatal period there are recurrent respiratory symptoms that in some cases are associated with a congenital pulmonary malformation. Case report: We report the case of a young infant, who was diagnosed with pulmonary sequestration at 24 weeks of gestational age, undergoing intrauterine surgical treatment with a report of complete resolution of the malformation in posterior ultrasounds. She was valued by pediatric pneumology at 4 months of age, where angiotomography was performed and the presence of pulmonary sequestration was confirmed by lobectomy. The histopathological study reported extralobar pulmonary sequestration with congenital malformation of the pulmonary airway type 2. These combined lesions were identified by histopathological study. The treatment of choice was surgical. Conclusions: Upon the confirmation of a malformation, we emphasize the importance of performing a screening in order to search for other that could be associated.


Asunto(s)
Femenino , Humanos , Lactante , Diagnóstico Prenatal/métodos , Anomalías del Sistema Respiratorio/diagnóstico , Secuestro Broncopulmonar/diagnóstico , Neumonectomía/métodos , Anomalías del Sistema Respiratorio/cirugía , Secuestro Broncopulmonar/cirugía , Edad Gestacional , Terapias Fetales/métodos , Angiografía por Tomografía Computarizada/métodos
15.
Einstein (Säo Paulo) ; 15(4): 395-402, Oct.-Dec. 2017. tab, graf
Artículo en Inglés | LILACS | ID: biblio-891439

RESUMEN

ABSTRACT Objective: To describe our initial experience with a novel approach to follow-up and treat gastroschisis in "zero minute" using the EXITlike procedure. Methods: Eleven fetuses with prenatal diagnosis of gastroschisis were evaluated. The Svetliza Reductibility Index was used to prospectively evaluate five cases, and six cases were used as historical controls. The Svetliza Reductibility Index consisted in dividing the real abdominal wall defect diameter by the larger intestinal loop to be fitted in such space. The EXIT-like procedure consists in planned cesarean section, fetal analgesia and return of the herniated viscera to the abdominal cavity before the baby can fill the intestines with air. No general anesthesia or uterine relaxation is needed. Exteriorized viscera reduction is performed while umbilical cord circulation is maintained. Results: Four of the five cases were performed with the EXIT-like procedure. Successful complete closure was achieved in three infants. The other cases were planned deliveries at term and treated by construction of a Silo. The average time to return the viscera in EXIT-like Group was 5.0 minutes, and, in all cases, oximetry was maintained within normal ranges. In the perinatal period, there were significant statistical differences in ventilation days required (p = 0.0169), duration of parenteral nutrition (p=0.0104) and duration of enteral feed (p=0.0294). Conclusion: The Svetliza Reductibility Index and EXIT-like procedure could be new options to follow and treat gastroschisis, with significantly improved neonatal outcome in our unit. Further randomized studies are needed to evaluate this novel approach.


RESUMO Objetivo: Descrever a experiência inicial com a nova técnica cirúrgica EXIT-like para acompanhamento e correção de gastrosquise no "minuto zero". Métodos: Foram avaliados onze fetos com diagnóstico pré-natal de gastrosquise. O Svetliza Reducibility Index foi usado prospectivamente para avaliar cinco casos, e seis foram utilizados como controles. O índice foi calculado dividindo-se o diâmetro do defeito da parede abdominal pela maior alça intestinal que coubesse neste espaço. O procedimento EXIT-like compreendeu cesárea programada, analgesia fetal e redução da víscera herniada para a cavidade abdominal, antes da deglutição de ar pelo recém- nascido. Não são necessários anestesia geral e nem relaxamento uterino. A redução da víscera exteriorizada é realizada enquanto se mantém a circulação do cordão umbilical. Resultados: Quatro casos, dos cinco, foram submetidos ao EXIT-like. A correção foi completa em três casos. Os demais casos foram partos a termo planejados, e a correção do defeito foi feita com Silo. O tempo médio de redução da víscera foi de 5,0 minutos no grupo submetido ao procedimento EXITlike, e a oximetria foi mantida dentro dos valores de variação normal em todos os casos. No período perinatal, foram observadas diferenças estatisticamente significativas no tempo de ventilação mecânica (p=0,0169), duração da nutrição parenteral (p = 0,0104) e da nutrição enteral (p=0,0294). Conclusão: O Svetliza Reducibility Index e o procedimento EXIT-like podem ser novas opções para acompanhar e tratar gastroquise, com desfecho neonatal significativamente melhor em nossa unidade. Novos estudos randomizados são necessários para avaliar esta nova abordagem.


Asunto(s)
Humanos , Masculino , Femenino , Embarazo , Recién Nacido , Cesárea/métodos , Gastrosquisis/cirugía , Terapias Fetales/métodos , Enfermedades Fetales/cirugía , Grupo de Atención al Paciente , Cuidado Intensivo Neonatal , Estudios Prospectivos , Estudios Retrospectivos , Ultrasonografía Prenatal , Edad Gestacional , Edad Materna , Resultado del Tratamiento , Cuidados Posteriores , Gastrosquisis/embriología , Gastrosquisis/diagnóstico por imagen , Dilatación Patológica , Feto/patología
18.
Einstein (Sao Paulo) ; 15(4): 395-402, 2017.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-29364360

RESUMEN

OBJECTIVE: To describe our initial experience with a novel approach to follow-up and treat gastroschisis in "zero minute" using the EXITlike procedure. METHODS: Eleven fetuses with prenatal diagnosis of gastroschisis were evaluated. The Svetliza Reductibility Index was used to prospectively evaluate five cases, and six cases were used as historical controls. The Svetliza Reductibility Index consisted in dividing the real abdominal wall defect diameter by the larger intestinal loop to be fitted in such space. The EXIT-like procedure consists in planned cesarean section, fetal analgesia and return of the herniated viscera to the abdominal cavity before the baby can fill the intestines with air. No general anesthesia or uterine relaxation is needed. Exteriorized viscera reduction is performed while umbilical cord circulation is maintained. RESULTS: Four of the five cases were performed with the EXIT-like procedure. Successful complete closure was achieved in three infants. The other cases were planned deliveries at term and treated by construction of a Silo. The average time to return the viscera in EXIT-like Group was 5.0 minutes, and, in all cases, oximetry was maintained within normal ranges. In the perinatal period, there were significant statistical differences in ventilation days required (p = 0.0169), duration of parenteral nutrition (p=0.0104) and duration of enteral feed (p=0.0294). CONCLUSION: The Svetliza Reductibility Index and EXIT-like procedure could be new options to follow and treat gastroschisis, with significantly improved neonatal outcome in our unit. Further randomized studies are needed to evaluate this novel approach.


Asunto(s)
Cesárea/métodos , Enfermedades Fetales/cirugía , Terapias Fetales/métodos , Gastrosquisis/cirugía , Cuidados Posteriores , Dilatación Patológica , Femenino , Feto/patología , Gastrosquisis/diagnóstico por imagen , Gastrosquisis/embriología , Edad Gestacional , Humanos , Recién Nacido , Cuidado Intensivo Neonatal , Masculino , Edad Materna , Grupo de Atención al Paciente , Embarazo , Estudios Prospectivos , Estudios Retrospectivos , Resultado del Tratamiento , Ultrasonografía Prenatal
19.
J Surg Res ; 203(2): 466-75, 2016 06 15.
Artículo en Inglés | MEDLINE | ID: mdl-27363657

RESUMEN

BACKGROUND: Congenital diaphragmatic hernia (CDH) is associated with lung hypoplasia and pulmonary hypertension. Tracheal occlusion (TO) stimulates fetal lung growth and maturation and reverse vascular changes responsible for pulmonary hypertension, which are related to mechanisms involving nitric oxide (NO) in CDH. We aim to evaluate the effect of TO and ventilation on NO pathways. METHODS: Eight groups were created: (1) control; (2) control ventilated (CV); (3) CDH (CDH); (4) CDH ventilated (CDHV); (5) TO control; (6) TO ventilated; (7) TO + CDH; and (8) TO + CDH ventilated (CDHTOV). Fetuses were weighed, and volume ventilated for 30 min after harvested. Total lung weight and the ratio of total lung weight to body weight, thickness of the middle layer of the pulmonary arteriole, and the air space diameter were measured. The NO synthase inducible and NO synthase inducible were performed by immunohistochemistry and Western blotting. RESULTS: The total lung weight and the ratio of total lung weight to body weight decreased in animals with nitrofen and also after ventilation for all groups (P < 0.05). The thickness of the middle layer of the pulmonary arteriole decreased in all groups with TO when compared with controls (P < 0.001). The air space diameter decreased after ventilation in the CDHTOV compared to the TO + nitrofen-induced CDH (P < 0.001). Compared to nonventilated cohorts, NO synthase inducible increased in CV and TO ventilated (P < 0.001) and decreased in CDHV and CDHTOV (P < 0.001). NO synthase inducible increased in CV and CDHV (P < 0.001) and decreased in the TO control and CDHTOV (P < 0.001). CONCLUSIONS: TO and ventilation alter the NO pathway with possible implications in reducing the pulmonary hypertension in CDH.


Asunto(s)
Terapias Fetales , Hernias Diafragmáticas Congénitas/terapia , Óxido Nítrico Sintasa de Tipo III/metabolismo , Óxido Nítrico Sintasa de Tipo II/metabolismo , Óxido Nítrico/metabolismo , Respiración Artificial , Oclusión Terapéutica , Animales , Biomarcadores/metabolismo , Western Blotting , Femenino , Terapias Fetales/métodos , Hernias Diafragmáticas Congénitas/metabolismo , Hernias Diafragmáticas Congénitas/fisiopatología , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/metabolismo , Hipertensión Pulmonar/prevención & control , Inmunohistoquímica , Pulmón/embriología , Pulmón/metabolismo , Óxido Nítrico Sintasa/metabolismo , Tamaño de los Órganos , Ratas , Ratas Sprague-Dawley
20.
Rev. pediatr. electrón ; 12(3): 24-32, oct. 2015. ilus, tab
Artículo en Español | LILACS | ID: lil-776773

RESUMEN

El teratoma sacrococcígeo representa la lesión tumoral más frecuente durante la gestación. Si bien su frecuencia es baja, en asociación con hidrops puede determinar la aparición de insuficiencia cardiaca fetal y posterior muerte fetal in útero. La evaluación prenatal mediante ultrasonografía, debe orientarse hacia la selección de aquellos fetos candidatos a intervención prenatal para mejorar el resultado perinatal de fetos con compromiso hemodinámico que se encuentran bajo la viabilidad. El objetivo de la presente actualización es exponer las herramientas actualmente en uso para realizar dicha evaluación y los resultados de la cirugía prenatal. La puntuación del perfil cardiovascular fetal mediante la ecografía, se propone como una herramienta sensible y útil en la selección de fetos candidatos a intervención prenatal. Tanto la cirugía abierta como el abordaje mínimamente invasivo se exponen como alternativas en el manejo prenatal de fetos candidatos a intervención con sobrevida reportada en torno al 50 y 44 por ciento respectivamente.


The sacrococcygeal teratoma is the most common tumor during prenatal period. Although its frequency is low, together with hydrops could determine the occurrence of fetal heart failure, and subsequent fetal death in utero. Prenatal ultrasonographic evaluation, should be directed toward selection of those fetuses as candidates for prenatal therapy, to improve their perinatal outcome, when they present hemodynamic compromise while they are under fetal viability. The purpose of this update is to expose the tools currently used to conduct the assessment and the results of prenatal surgery. The fetal cardiovascular profile score by ultrasound is proposed as a sensible and useful tool in the selection of candidates for prenatal intervention. Both open surgery as minimally invasive approach are presented as alternatives in the prenatal management of fetus as candidates to intervention, has been reported a survival about 50 and 44 percent respectively.


Asunto(s)
Humanos , Femenino , Embarazo , Enfermedades Fetales/cirugía , Enfermedades Fetales/diagnóstico , Neoplasias de la Columna Vertebral/cirugía , Neoplasias de la Columna Vertebral/diagnóstico , Teratoma/cirugía , Teratoma/diagnóstico , Región Sacrococcígea , Terapias Fetales/métodos
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