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1.
Arch. pediatr. Urug ; 93(2): e309, dic. 2022. ilus, tab
Artigo em Espanhol | LILACS, UY-BNMED, BNUY | ID: biblio-1383655

RESUMO

La malformación congénita de la vía aérea pulmonar (MVAP), antes llamada malformación adenomatoidea quística pulmonar, es una rara anormalidad del desarrollo de las vías respiratorias terminales. Las lesiones son de distribución y tamaños variables, usualmente unilaterales. El diagnóstico puede realizarse desde el período prenatal mediante ecografía gestacional, encontrándose, en ocasiones, graves repercusiones fetales. En los recién nacidos la enfermedad puede manifestarse con dificultad respiratoria aguda. En niños y adultos puede diagnosticarse ante infecciones pulmonares recurrentes u otras complicaciones. En pacientes sintomáticos está indicado el tratamiento quirúrgico para prevenir infecciones y la transformación neoplásica; sin embargo, sigue siendo controversial el tratamiento profiláctico frente al tratamiento expectante en pacientes asintomáticos. Se presenta el caso clínico de una lactante de 2 meses, que en el curso de una bronquiolitis se realizó una radiografía de tórax que evidenció una imagen radiolúcida del lóbulo medio. La tomografía computada visualizó gran imagen quística en pulmón derecho, que podría corresponder a una MVAP. Se decidió tratamiento quirúrgico coordinado. Se realizó una segmentectomía, confirmándose con anatomía patológica una MVAP tipo IV. Evolucionó favorablemente.


Congenital pulmonary airway malformation (CPAM), formerly called pulmonary cystic adenomatoid malformation, is a rare developmental abnormality of the terminal airways. Lesions are of variable size and distribution, usually unilateral. The diagnosis can be made from the prenatal period by means of gestational ultrasound, occasionally causing serious fetal repercussions. In newborns, the disease may manifest itself through acute respiratory distress. In children and adults it can be diagnosed through recurrent lung infections or other complications. In symptomatic patients, surgical treatment is indicated to prevent infections and neoplastic transformation. However, prophylactic versus expectant management in asymptomatic patients remains controversial. We present the clinical case of a 2-month-old infant who, during the course of bronchiolitis underwent a chest X-ray that revealed a radiolucent image of the middle lobe. The computed tomography scan showed a large cystic image in the right lung, which could be linked to an CPAM. The surgical treatment was carried out and the segmentectomy confirmed a pathological type IV CPAM. The patient evolved favorably.


A malformação congênita das vias aéreas pulmonares (MVAP), anteriormente chamada de malformação adenomatoide cística pulmonar, é uma anormalidade rara do desenvolvimento das vias aéreas terminais. As lesões são de tamanho e distribuição variáveis, geralmente unilaterais. O diagnóstico pode ser feito desde o pré-natal por meio da ultrassonografia gestacional, podendo encontrar repercussões fetais graves. Em recém-nascidos, a doença pode se manifestar com dificuldade respiratória aguda. Em crianças e adultos, pode ser diagnosticada com infecções pulmonares recorrentes ou outras complicações. Em pacientes sintomáticos, o tratamento cirúrgico é indicado para prevenir infecções e transformações neoplásicas; no entanto, o tratamento profilático versus expectante em pacientes assintomáticos permanece controverso. Apresentamos o caso clínico de uma criança de 2 meses que foi submetida a radiografia de tórax durante bronquiolite, que revelou imagem radiotransparente do lobo médio. A tomografia computadorizada mostrou grande imagem cística em pulmão direito, que poderia corresponder a uma MVAP. O tratamento cirúrgico foi realizado e a segmentectomia confirmou um tipo IV de MVAP com patologia. A criança evoluiu favoravelmente.


Assuntos
Humanos , Feminino , Lactente , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico por imagem , Malformação Adenomatoide Cística Congênita do Pulmão/cirurgia , Malformação Adenomatoide Cística Congênita do Pulmão/patologia
2.
Rev. Assoc. Med. Bras. (1992, Impr.) ; 68(11): 1582-1586, Nov. 2022. tab, graf
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-1406592

RESUMO

SUMMARY OBJECTIVE: This study aimed to assess the perinatal outcomes of pregnancies with a prenatal diagnosis of congenital cystic adenomatoid malformation. METHODS: We conducted a retrospective cohort study based on information contained in the medical records of pregnant women whose fetuses had been prenatally diagnosed with congenital cystic adenomatoid malformation by ultrasonography. RESULTS: Sample analysis was based on 21 singleton pregnancies with confirmed isolated fetal congenital cystic adenomatoid malformations. The mean maternal±standard deviation age was 28±7.7 years. Types I, II, and III congenital cystic adenomatoid malformation were detected in 19% (4/21), 52.4% (11/21), and 28.6% (6/21), respectively. All fetuses presented with unilateral congenital cystic adenomatoid malformation (21/21) without associated anomalies, and 52.3% (11/21) were in the right lung. In total, 33.3% (7/21) of fetuses presented a "congenital cystic adenomatoid malformation volume ratio" >1.6 and were managed with maternal betamethasone administration. The mean gestational age at the time of steroid administration was 28.5±0.9 weeks, with a reduction in the lesion dimensions of 9.5% (2/21) (Types I and III of congenital cystic adenomatoid malformation). The mean gestational age at delivery was 38.7±2.4 weeks, and a cesarean section was performed in 76.2% (16/21) cases. Postsurgical resection was necessary for 23.8% (5/21) of the patients, and 4.7% (1/21) of them died because of respiratory complications after surgery. Pulmonary hypoplasia occurred in 9.5% (2/21) of the patients, and 4.7% (1/21) of them died because of respiratory insufficiency. The survival rate was 90.5% (19/21), and 57.2% (12/21) remained asymptomatic. CONCLUSION: Despite the isolated prenatal diagnosis of congenital cystic adenomatoid malformation, which showed good survival, congenital cystic adenomatoid malformation is associated with significant perinatal morbidity. Maternal betamethasone administration did not significantly reduce fetal lung lesion dimensions.

3.
Chinese Journal of Perinatal Medicine ; (12): 866-870, 2022.
Artigo em Chinês | WPRIM | ID: wpr-958154

RESUMO

Congenital pulmonary airway malformation-volume ratio (CVR) greater than 1.6 or 2.0 cm2 was considered to be helpful in predicting fetal hydrops and could serve as an indicator for steroid application, forecasting respiratory symptoms, and the timing of surgery after birth. However, the optimal CVR threshold remains controversial. This review focuses on the value of CVR in fetal prognosis, steroid administration, and the prediction of postnatal respiratory symptoms and surgical timing in fetuses with congenital pulmonary airway malformation, aiming to guide the perinatal management.

4.
Perinatol. reprod. hum ; 35(3): 114-117, sep.-dic. 2021. graf
Artigo em Espanhol | LILACS-Express | LILACS | ID: biblio-1406194

RESUMO

Resumen Antecedentes: La malformación adenomatoidea quística y el secuestro broncopulmonar son malformaciones que se pueden diagnosticar prenatalmente. Los adecuados controles prenatales ayudan a un diagnóstico temprano y su manejo. Objetivo: Presentar el caso de un recién nacido con diagnóstico parental de una malformación pulmonar y su desenlace posterior. Reporte de caso: Recién nacido a término con diagnóstico antenatal de malformación pulmonar, el cual se confirmó tras su nacimiento. Discusión: Estas dos malformaciones son entidades cuya incidencia viene aumentando, razón por la cual es importante el conocimiento del curso clínico, ayudas diagnósticas, complicaciones y las posibilidades de manejo con las que se cuenta.


Abstract Background: Cystic adenomatoid malformation and bronchopulmonary sequestration are malformations that can be diagnosed prenatally. Appropriate prenatal controls helps to early diagnosis and management. Objective: To present the case of a newborn with a parental diagnosis of a pulmonary malformation and its subsequent outcome. Case report: Newborn at term with antenatal diagnosis of pulmonary malformation which was confirmed postnatal. Discussion: These two malformations are entities whose incidence is increasing, which is why knowledge of the clinical course, diagnostic aids, complications, and the management possibilities that are available are important.

5.
Neumol. pediátr. (En línea) ; 16(1): 41-47, 2021. tab, ilus
Artigo em Espanhol | LILACS | ID: biblio-1284223

RESUMO

Congenital pulmonary airway malformations (CPAM) are rare and occur due to a failure in lung embryological development. They are classified according to their pathological characteristics and their anatomical origin. They can occur from the antenatal period to adulthood, can be associated with hydrops fetalis, respiratory distress, recurrent infections, or in an otherwise asymptomatic patient. In this article we carry out a scope review of the literature to answer frequent questions of the clinical teams in charge of patients with CPAM, such as the antenatal approach, the different types of surgeries and the management of asymptomatic patients. Although the indication for surgery is clear in symptomatic patients, little is known about its natural history of this condition, including the possibility of spontaneous resolution and the development of complications or neoplasm. So, the treatment of asymptomatic patients continues to be controversial. Therapeutic decisions must be made by multidisciplinary teams with the informed participation of parents and patients. In our opinion, considering the excellent results of minimally invasive surgery, its low incidence of complications, and practically zero mortality when performed by experienced groups, it seems reasonable to consider elective resection of a MCVAP in asymptomatic patients.


Las malformaciones congénitas de la vía aérea pulmonar (MCVAP) son infrecuentes y ocurren debido a una falla en el desarrollo embriológico pulmonar. Se clasifican de acuerdo con sus características patológicas y a su origen anatómico. Se pueden presentar desde el periodo antenatal hasta la adultez, asociarse a cuadros de hidrops fetal, distrés respiratorio, infecciones recurrentes, o como un hallazgo en pacientes asintomáticos. En este artículo realizamos una revisión bibliográfica exploratoria para responder dudas frecuentes de los equipos clínicos a cargo de pacientes con MCVAP, como el enfrentamiento antenatal, los distintos tipos de cirugía y su abordaje, y el manejo de pacientes asintomáticos. Si bien la indicación de cirugía es clara en pacientes sintomáticos, poco se conoce acerca de su historia natural, incluyendo la posibilidad de resolverse de forma espontánea, de complicarse o de evolucionar hacia el desarrollo de una neoplasia, por lo que el tratamiento de pacientes asintomáticos continúa siendo controversial. Las decisiones terapéuticas deben ser tomadas por equipos multidisciplinarios con la participación informada de los padres y de los pacientes. En nuestra opinión, considerando los excelentes resultados de la cirugía mínimamente invasiva, su baja incidencia de complicaciones y prácticamente nula mortalidad, al ser realizada por grupos con experiencia, nos parece razonable plantear la resección electiva de una MCVAP en un paciente asintomático.


Assuntos
Humanos , Criança , Anormalidades do Sistema Respiratório/cirurgia , Pneumonectomia , Cuidado Pré-Natal , Imageamento por Ressonância Magnética , Toracotomia , Radiografia Torácica , Malformação Adenomatoide Cística Congênita do Pulmão/cirurgia , Anormalidades do Sistema Respiratório/classificação , Anormalidades do Sistema Respiratório/embriologia , Anormalidades do Sistema Respiratório/diagnóstico por imagem , Risco , Pulmão/anormalidades
6.
Chinese Journal of Pediatrics ; (12): 19-24, 2020.
Artigo em Chinês | WPRIM | ID: wpr-798572

RESUMO

Objective@#To summarize the diagnosis, clinical manifestations, treatment and prognosis of congenital cystic lung lesions.@*Methods@#A retrospective study described the clinical course of 96 patients (46 female and 50 male) diagnosed with congenital cystic lung lesions treated at the Tianjin Children′s Hospital from January 2010 to March 2019. The clinical findings, imaging examinations, pathological findings, treatment and follow-up were analyzed.@*Results@#Totally 96 patients (aged from 4 days to 13 years) with congenital cystic lung lesions were included in this study. Eighty-six patients (90%) were diagnosed when they had cough and fever symptoms. Forty (42%) patients exhibited congenital cystic adenomatoid malformation, 30 underwent surgical excision, two were at emergency operations and one dead. There were 12 (13%) patients with pulmonary sequestration and four were surgical treated. Twelve (13%) patients with bronchogenic cyst were included and 4 were surgically treated. There were 3 (3%) patients with congenital lobar emphysema and one was surgically treated. Another patient with pneumothorax was operated in other hospital 2 months after discharge. Twenty-nine (30%) patients with unclassified congenital cystic lung lesions could not be definitively diagnosed by CT. Some of them were difficult to be distinguished from necrotizing pneumonia. Finally, 2 patients were diagnosed as necrotizing pneumonia after 6, 10 months follow-up. After operation 37 out of 39 patients recovered well.@*Conclusions@#The diagnosis of congenital pulmonary cystic disease depend on imaging and pathological examination. Most patients are diagnosed when they have respiratory tract infection. The main clinical manifestations are cough and fever. The prognosis of operative management is good.

7.
Artigo | IMSEAR | ID: sea-206887

RESUMO

Antenatal ultrasound screening of fetal malformations requires for management, the immediate nature of which at certain birth, conditions the survival of the newborn. The Objectives Describe a case of pulmonary cystic adenomatoid malformation discover during antenatal ultrasonography. It was a 36 years old primary patient, referred to the maternity of Lafontaine hospital (France) for the presence, on obstetrical ultrasound screening, of suspected lesions on the foetal right lung’s field in a 21 weeks gestation pregnancy. After successive obstetric ultrasound scan, it was discovered that the left lungs were big with some parenchymal cysts of different sizes more than 3 mm in diameter. We made a diagnosis of type I left pulmonary cyst adenoma with excess amniotic fluid, without associated anomalies. The pregnancy progressed to 40 weeks gestation and a caesarean section was done with extraction of a live female new born weighing 2730 gr. The new born had 40 days later a successful surgical lobectomy for a cystic adenomatoid dysplasia. Histological examination revealed type I Stocker congenital pulmonary cystic adenomatoid malformation. During obstetrical ultrasound screening, we paid more attention to the brain, the heart, the face. Through this study we realised that during routine antenatal ultrasound for morphology, we should also pay more attention to the lungs.

8.
Rev. chil. obstet. ginecol. (En línea) ; 84(3): 208-216, jun. 2019. tab, graf, ilus
Artigo em Espanhol | LILACS | ID: biblio-1020638

RESUMO

RESUMEN OBJETIVO: el propósito es describir el efecto de los corticoides prenatales en la malformación congénita de la vía aérea pulmonar (MCVAP) mediante la evaluación secuencial del CVR y mostrar la experiencia en nuestro centro de terapia fetal. MATERIALES Y MÉTODOS: serie de casos en la cual se incluyeron ocho pacientes del Hospital de San José; con diagnóstico de MCVAP; se evaluó edad gestacional, el CVR (longitud x largo x ancho, en cms de la masa) x 0.523/circunferencia cefálica) como factor pronóstico, la desviación mediastínica, la presencia de hidrops, el tipo de MCVAP y la localización. Se aplicó betametasona a dosis de inducción de madurez pulmonar fetal. Se realizó la medición del CVR pre y pos tratamiento. Los datos obtenidos fueron analizados mediante el software estadístico SPSS (Versión 11.0 SPSS Inc. Chicago, IL). Se utilizaron la prueba Wilcoxon y el índice de correlación de Spearman según el caso. Un valor de p<0.05 se consideró significativo. RESULTADOS: ocho pacientes fueron incluidas. La mediana de la edad gestacional fue 30.5 semanas. Cuatro casos fueron tipo I, dos tipo II y dos tipo tres. Cuatro casos presentaron desviación mediastinal. Cinco casos fueron derechos y uno bilateral. Ningún caso presentó hidrops. El CVR pre tratamiento fue 0,93(RIQ:0,17-2,1) y pos tratamiento 0,55(RIQ:0,07-1,39). Se encontró una disminución del CVR pos tratamiento en todos los casos (p 0.0117) e índice de correlación de Spearman 0.9524 CONCLUSIÓN: Después de la aplicación de corticoides encontramos una disminución del CVR en todos los casos descritos. El uso de corticoides podría ser parte de la terapia prenatal con miras a mejorar el pronóstico.


ABSTRACT OBJECTIVE: The purpose of this study is to describe the effect of prenatal corticosteroids in the CPAM by sequential evaluation of the CVR, and to show the experience in our fetal therapy center. MATERIALS AND METHODS: Series of cases in which eight patients from the Hospital of San José were included; with diagnosis of CPAM; gestational age was evaluated, CVR (CVR = length x length x width cm, mass) x 0.523 / head circumference) as a prognostic factor, mediastinal shift, hydrops, type of CPAM, location. Betamethasone was applied to fetal lung maturation dose. In all patients underwent CVR measurement pre and post treatment. The data were analyzed using SPSS statistical software (Version 11.0 SPSS Inc. Chicago, IL). The Wilcoxon test and the Spearman correlation index were used according to the case. A value of p <0.05 was considered significant. RESULTS: eight patients were included. The median of gestational age was 30.5 weeks. Four cases were type I, two type II and two type three. Four cases were mediastinal shift. Five cases were rights and one bilateral. No case presented hydrops. The pretreatment CVR was 0,93(RIQ: 0,17-2,1) and post-treatment 0,55(RIQ: 0,07-1,39). We found a decrease in CVR after treatment in all cases (p 0.0117) and Spearman correlation index 0.9524 CONCLUSION: After application of corticosteroids are a reduction in CVR in all the cases described. The use of corticosteroids may be part of prenatal therapy to improve prognosis.


Assuntos
Humanos , Feminino , Gravidez , Malformação Adenomatoide Cística Congênita do Pulmão/genética , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico por imagem , Corticosteroides/uso terapêutico , Gestantes , Hidropisia Fetal , Ultrassonografia , Idade Gestacional , Doenças Fetais/diagnóstico , Feto
9.
International Journal of Pediatrics ; (6): 790-793, 2019.
Artigo em Chinês | WPRIM | ID: wpr-823442

RESUMO

Congenital cystic adenomatoid malformation (CCAM) is a rare cystic lung disease with an incidence rate ranging from 1/35 000 to 1/25 000 births.The mechanism is still unclear and is regarded a non-hereditary anomaly caused by arrest of lung.In some countries,over 99% of CCAMs were diagnosed by fetal sonography at gestational age of 18 ~ 20 weeks.Chest X-ray and CT are common methods to detect the CCAMs after birth.Newborns with CCAM may present with respiratory distress,but most patients are asymptomatic in life.Although many methods for diagnosis and treatment were suggested,no unified guideline is provided yet.Herein,we reviewed the advances in the mechanism,diagnosis and treatment of CCAM to highlight this rare event.

10.
Neonatal Medicine ; : 138-146, 2019.
Artigo em Inglês | WPRIM | ID: wpr-760588

RESUMO

PURPOSE: Congenital pulmonary airway malformation (CPAM)—a rare developmental anomaly—affects the lower respiratory tract in newborns. By comparing the reliability of diagnostic tools and identifying predictive factors for symptoms, we provide comprehensive clinical data for the proper management of CPAM. METHODS: We reviewed the medical records of 66 patients with prenatally diagnosed CPAM delivered at Severance Children's Hospital between January 2005 and July 2017. RESULTS: We enrolled 33 boys and 33 girls. Their mean gestational age and birth weight were 38.8 weeks and 3,050 g, respectively. Prenatal ultrasonography and postnatal radiography, lung ultrasonography, and chest computed tomography (CT) showed inconsistent findings. Chest CT showed superior sensitivity (100%) and positive predictive value (90%). Among the 66 patients, 59 had postnatally confirmed CPAM, three had pulmonary sequestration, one had cystic teratoma, and one had a normal lung. Of the 59 patients with CPAM, 21 (35%; mean age, 23.4 months) underwent surgery, including 15 who underwent video-assisted thoracoscopy. Twenty-five and 12 patients exhibited respiratory symptoms at birth and during infancy, respectively. Apgar scores and mediastinal shift on radiography were significantly associated with respiratory symptoms at birth. However, none of the factors could predict respiratory symptoms during infancy. CONCLUSION: Radiography or ultrasonography combined with chest CT can confirm an unclear or inconsistent lesion. Apgar scores and mediastinal shift on radiography can predict respiratory symptoms at birth. However, symptoms during infancy are not associated with prenatal and postnatal factors. Chest CT combined with periodic symptom monitoring is important for diagnosing and managing patients with prenatally diagnosed CPAM and to guide appropriate timing of surgery.


Assuntos
Feminino , Humanos , Recém-Nascido , Peso ao Nascer , Sequestro Broncopulmonar , Malformação Adenomatoide Cística Congênita do Pulmão , Idade Gestacional , Pulmão , Prontuários Médicos , Parto , Radiografia , Sistema Respiratório , Teratoma , Cirurgia Torácica Vídeoassistida , Toracoscopia , Tórax , Tomografia Computadorizada por Raios X , Ultrassonografia , Ultrassonografia Pré-Natal
11.
International Journal of Pediatrics ; (6): 790-793, 2019.
Artigo em Chinês | WPRIM | ID: wpr-801554

RESUMO

Congenital cystic adenomatoid malformation(CCAM) is a rare cystic lung disease with an incidence rate ranging from 1/35 000 to 1/25 000 births.The mechanism is still unclear and is regarded a nonhereditary anomaly caused by arrest of lung.In some countries, over 99% of CCAMs were diagnosed by fetal sonography at gestational age of 18~20 weeks.Chest X-ray and CT are common methods to detect the CCAMs after birth.Newborns with CCAM may present with respiratory distress, but most patients are asymptomatic in life.Although many methods for diagnosis and treatment were suggested, no unified guideline is provided yet.Herein, we reviewed the advances in the mechanism, diagnosis and treatment of CCAM to highlight this rare event.

12.
Artigo em Espanhol | LILACS-Express | LILACS | ID: biblio-1508925

RESUMO

Microcystic fetal lung tumors or masses such as the congenital cystic adenomatoid malformation (CCAM) and bronchopulmonary sequestration (SBP) are rare congenital pathologies, diagnosed by ultrasound during prenatal assessment, with a high mortality rate of 95% as a consequence of prenatal complications due to hydrops and postnatal death from pulmonary hypoplasia. Minimally invasive therapies using sclerosing agents are positioned among the low cost and highly effective techniques for such pathologies. Methodology: We conducted a prospective, randomized, blind clinical study in 17 pregnant women with diagnosis of fetal microcystic CCAM or hybrid lesion (CCAM + SBP), who were treated with betamethasone or polidocanol. Alternative therapy was employed in case of failure of the initial procedure. Results: In four (44.4%) of the nine cases treated with betamethasone, the alternative therapy of sclerosis with polidocanol was required for treatment due to fetal compromise. Involution of the lung lesion and the hydropic condition was faster and progressive with percutaneous sclerotherapy compared to the maternal steroid effect. Perinatal results were also more satisfactory in the group with polidocanol (91.7%) than in the group with the steroid (60%). Conclusions: Percutaneous sclerotherapy with polidocanol was found efficacious for CCAM and hybrid lesion treatment, with faster and more efficient resolution of the pathology in cases resistant to previously administered steroid therapy.


Las tumoraciones o masas pulmonares fetales microquísticas, tales como la malformación adenomatoidea quística congénita (CCAM, por sus siglas en inglés) y el secuestro bronco pulmonar (SBP), representan patologías congénitas poco frecuentes, diagnosticadas por ultrasonido durante la evaluación prenatal, con un alto índice de mortalidad de 95% como consecuencia de las complicaciones prenatales por hidropesía y fallecimiento posnatal debido a hipoplasia pulmonar. Las terapias de mínima invasión usando agentes esclerosantes han tomado posicionamiento entre las técnicas de bajo costo y alta efectividad para dichas patologías. Metodología. Se realizó un estudio clínico prospectivo, aleatorio, ciego, en 17 embarazadas con diagnóstico de CCAM microquística o de lesión híbrida (CCAM +SBP) tratadas con betametasona o con polidocanol, optando por la terapia alternativa en caso de fallar la técnica inicial. Resultados. En 4/9 (44,4%) de los 9 casos tratados con betametasona se requirió la terapia alternativa de esclerosis con polidocanol para la resolución del cuadro, que comprometía el estado fetal. La involución de la lesión pulmonar y del cuadro hidrópico fue más rápida y progresiva con la escleroterapia percutánea en comparación con el efecto esteroideo materno. Los resultados perinatales fueron asimismo más satisfactorios en el grupo con polidocanol (91,7%) que en el grupo con el esteroide (60%). Conclusiones. Se comprobó la eficacia de la escleroterapia percutánea con polidocanol para el tratamiento de la CCAM y de lesión híbrida, con resolución más rápida y eficaz de la patología en los casos donde existía resistencia a la terapia esteroidea previamente administrada.

13.
Autops. Case Rep ; 8(2): e2018022, Apr.-May 2018. ilus
Artigo em Inglês | LILACS | ID: biblio-905523

RESUMO

Congenital pulmonary airway malformation (CPAM) is a developmental malformation of the lower respiratory tract. We report the case of a male newborn diagnosed with cystic lung disease during prenatal ultrasound. A cesarean section was performed at the 32nd gestational week because of premature rupture of the membranes, and soon after the delivery the newborn developed respiratory failure and died. The aim of this study is to report an autopsy case because of its rarity, and to briefly discuss the CPAM subtypes and differential diagnosis of cystic lung diseases of childhood.


Assuntos
Humanos , Feminino , Gravidez , Recém-Nascido , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico , Diagnóstico Pré-Natal/estatística & dados numéricos , Aborto , Autopsia , Malformação Adenomatoide Cística Congênita do Pulmão/patologia , Diagnóstico Diferencial , Evolução Fatal , Recém-Nascido Prematuro , Síndrome do Desconforto Respiratório do Recém-Nascido/etiologia
14.
Rev. peru. ginecol. obstet. (En línea) ; 64(1): 91-97, ene.-mar. 2018. ilus, tab
Artigo em Espanhol | LILACS-Express | LILACS | ID: biblio-1014452

RESUMO

Se reporta una serie de 3 casos con diagnóstico prenatal de malformación adenomatoide quística pulmonar variedad macroquística sometida a terapia intraútero con derivación tóraco-amniótica. Se muestra que esta intervención intrauterina altamente especializada puede ser realizada con éxito en nuestro país. Los tres casos sobrevivieron. Se presenta una revisión corta de esta patología y se propone un flujograma de manejo.


We report a series of three cases with prenatal diagnosis of congenital cystic adenomatoid malformation of the lung, macrocystic type, treated in utero with thoraco-amniotic shunting. This highly specialized intervention is feasible in our local setting. The three cases survived. A mini-review of this condition is presented and an algorithm of prenatal management is proposed.

15.
Korean Journal of Pediatrics ; : 30-34, 2018.
Artigo em Inglês | WPRIM | ID: wpr-741350

RESUMO

Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare developmental lung abnormality associated with rhabdomyosarcoma, pleuropulmonary blastoma, and mucinous adenocarcinoma of the lung. We report an unusual case of a 10-day-old male newborn with a left lower lobe pulmonary cyst who underwent lobectomy, which revealed type II CPAM complicated by multifocal mucinous adenocarcinoma. KRAS sequencing revealed a somatic mutation in Codon12 (GGT → GAT), suggesting the development of a mucinous adenocarcinoma in the background of mucinous metaplasia. Mucinous adenocarcinoma is the most common lung tumor associated with CPAM, but it generally occurs in older children and adults. Further, all cases in the literature are of type I CPAM. This case in a neonate indicates that malignant transformation can occur very early in type II CPAM.


Assuntos
Adulto , Criança , Humanos , Recém-Nascido , Masculino , Adenocarcinoma Mucinoso , Malformação Adenomatoide Cística Congênita do Pulmão , Pulmão , Metaplasia , Mucinas , Rabdomiossarcoma
16.
International Journal of Pediatrics ; (6): 505-508, 2018.
Artigo em Chinês | WPRIM | ID: wpr-692534

RESUMO

Congenital cystic adenomatoid malformation of the lung is a rare clinical lung disease.It is the deformity formed by abnormal embryo development of lung tissue.Its pathological morphology is very different from other types of pulmonary cyst.The main pathogenesis,clinical manifestation,diagnosis,differential diagnosis and treatment are described.

17.
Neumol. pediátr. (En línea) ; 12(3): 122-124, jul. 2017. tab
Artigo em Espanhol | LILACS | ID: biblio-999090

RESUMO

Cystic adenomatoid malformation is the most frequent congenital pulmonary malformation. The usual treatment is surgical resection. However there is controversy over management in asymptomatic patients. The possible malignization would justify surgery of cystic lesions. Relation with pleuropulmonary blastoma has been described, however it is not clear whether this is a primary tumor or cyst malignization. Cystic adenomatoid malformation also has association with adenocarcinoma and rhabdomyosarcoma. Currently available evidence suggests surgical resection, despite the natural course of congenital lung cystic lesions is uncertain


La malformación adenomatoidea quística (MAQ) es la anomalía del desarrollo pulmonar más frecuente. El tratamiento habitual es la resección quirúrgica, no obstante existe controversia sobre el manejo en pacientes asintomáticos. La posible malignización de las lesiones quísticas es uno de los argumentos que justifican la cirugía en estos pacientes. Se ha descrito relación con blastoma pleuropulmonar, sin embargo no está claro si se trataría de una lesión quística que se maligniza o es una entidad diferente. También hay asociación con adenocarcinoma y rabdomiosarcoma . Actualmente se sugiere la resección quirúrgica como el tratamiento más adecuado, sin embargo la evolución natural de las lesiones quísticas pulmonares congénitas es incierta


Assuntos
Humanos , Malformação Adenomatoide Cística Congênita do Pulmão/cirurgia , Malformação Adenomatoide Cística Congênita do Pulmão/complicações , Blastoma Pulmonar/etiologia , Neoplasias Pulmonares/etiologia , Rabdomiossarcoma/etiologia , Rabdomiossarcoma/prevenção & controle , Adenocarcinoma/etiologia , Adenocarcinoma/prevenção & controle , Neoplasias Pulmonares/prevenção & controle
18.
Ann Card Anaesth ; 2016 Apr; 19(2): 372-374
Artigo em Inglês | IMSEAR | ID: sea-177415

RESUMO

The association of congenital pulmonary airway malformation (CPAM) with congenital heart disease is rare. We present the case of a 6‑month‑old child with atrial septal defect and pulmonary hypertension (PH) who presented with severe respiratory distress and hypoxia. The patient underwent right lobectomy for CPAM. With timely management, real‑time monitoring, one lung ventilation, and adequate analgesia, we were able to extubate the child in the immediate postoperative period. We conclude that with meticulous planning and multidisciplinary team approach, such complex cases can be managed successfully.

19.
Chinese Journal of Applied Clinical Pediatrics ; (24): 769-772, 2016.
Artigo em Chinês | WPRIM | ID: wpr-497775

RESUMO

Objective To explore relationship between imaging classification and pathological type,and the electron microscopic ultrastructure by observing the ultrastructural features of congenital cystic adenomatoid malformation (CCAM).Methods Children with CCAM in the Guangdong Women and Children Hospital from May 2014 to November 2014,were analyzed,imaging classification was performed according to enhanced Computed Tomography (CT) scanning,and the surgical specimens were stained by HE and scanning electron microscope was used to observe the tissue structures of various of CCAM.Results Eight cases were enrolled in this research (5 boys and 3 girls),and the mean age of surgery was (5.4 ± 1.1) month old.According to the features of enhanced CT scanning,there were 2 cases of the type-Ⅰ,and the other 3 cases of type-Ⅱ,and 3 cases of type-Ⅲ;According to the characteristics of the pathological types,there was 1 case of type-Ⅰ,4 cases of type-Ⅱ,and the other 3 cases of type-Ⅲ.There was 1 case which classified as type-Ⅰ by CT,however classified as type-Ⅱ by pathological typing.The findings of CT features were in complete correspondence with the pathological types in the rest cases.Electron microscopic findings showed the alveolar spaces were enlarged,the alveolar cells were reduced,the lamellar bodies were decreased and immature and microvilli were also decreased.There was no significant difference between Ⅰ and Ⅱ type under the viewpoint of electron microscope.The common features between type Ⅰ,and type Ⅱ included that the alveolar space was enlarged,the alveolar space had been pressed between enlarged alveolar space,the alveolar cell were decreased and immature,and fibrous hypertrophy was also found.Conclusions There are differences between ultrastructural analysis and clinical pathological typing which suggests the clinical importance of the electron microscopy ultrastructural analysis combined with pathology for typing.There need further clinical and laboratory studies,especially the combination of microscopy-electrical control microscope for typing with related predictive models for follow-up of CCAM.

20.
Chinese Journal of Applied Clinical Pediatrics ; (24): 299-301, 2016.
Artigo em Chinês | WPRIM | ID: wpr-488222

RESUMO

Objective To explore the clinical manifestations,imaging findings,pathological classification and treatment of congenital cystic adenomatoid malformation (CCAM)of the lung.Methods The clinical features,imaging findings,pathology information,diagnosis,treatment method and its prognosis of children with CCAMconfirmed by ope-ration and pathology were retrospectively analyzed in Yuying Children′s Hospital Affiliated to Wenzhou Medical Univer-sity from August 2006 to August 201 4.Results Eleven patients were boys and 4 patients were girls.One case had a-symptomatic clinical features,1 2 cases had pulmonary infection,1 case had recurrent chest pain,and 1 case had de-pressed deformity in sternum inferior segment.Chest CT scanning indicated that 9 cases had multiple gas cysts at unila-teral side of lung,among which 1 case was of funnel chest and pulmonary sequestration,1 case of huge cyst containing air and fluid at inferior lobe of left lung,and 4 cases of high density lung shadow;CT examination indicated that 1 case had recurrent chest pain and eventration of diaphragm of the right side combined with pulmonary sequestration.All ca-ses were treated by surgical resection,of whom 1 case was given cystectomy and sequestrectomy,diaphragmatic plication respectively,1 case complicated with funnel chest disease underwent lesion pulmonary lobectomy,sequestrectomy and minimally invasive corrective surgery in pectus excavatum (Nuss surgery),and the remaining 1 2 cases received lesion pulmonary lobectomy.All of 1 5 cases recovered well without complications.Pathological classification type of CCAMin-cluded 1 1 cases of type Ⅰ,3 cases of type Ⅱ and 1 case of type Ⅲ,among which 2 cases had pulmonary sequestration. Conclusions CCAMis a rare disease which can be discovered along with pulmonary infection.Multiple gas cysts are the most common imaging findings and the preoperative diagnosis of CCAM is mostly based on chest CT examination. Type Ⅰ and type Ⅱ are the most common pathological classification.The surgical resection should be given early surgi-cal resection and the prognosis is usually good.

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