[Antenatal ultrasound diagnosis and neonatal results of the congenital cystic adenomatoid malformation of the lung]. / Diagnóstico ecográfico prenatal y resultados neonatales de la malformación adenomatoidea quística pulmonar.

INTRODUCTION: Congenital cystic adenomatoid malformation (CCAM) is a rare congenital lung di sease, and in the most of cases, prenatal diagnosis is feasible. There are discrepancies regarding pre natal management and postpartum treatment. OBJECTIVE: To analyze prenatally diagnosed CCAM in our hospitals, in order to evaluate ultrasound findings with fetal and postnatal evolution. PATIENTS AND METHOD: Retrospective study of all cases diagnosed prenatally by ultrasound between 2005 and 2016 in two reference hospitals. The ultrasounds were performed using high-resolution ultrasound scanners, Toshiba Xario and Voluson 730 Expert Pro, with follow-up from diagnosis to delivery. The variables analyzed included gestational age at diagnosis, the characteristics of the lung lesion, associated malformations, cytogenetic study, the evolution of pregnancy, type of delivery, presence of respiratory distress, need for complementary imaging tests, pediatric clinical course, and necessary postnatal treatments. It was considered a resolution the total disappearance of the lesion in the pre natal ultrasound or that the postnatal chest X-ray showed no lesion. RESULTS: 17 cases were prenatally diagnosed. The evolution ranges from the prenatal resolution of the lesion to the persistence after bir th. Three patients voluntarily decided to have an abortion due to ultrasound findings of poor progno sis. Of the fourteen remaining cases there were no cases of fetal or neonatal deaths, one case required surgery after birth and four patients had mild symptoms during the first year of life. One case of false negative with neonatal death has been reported which necropsy reported as CCAM type 0. Conclu sions: This pulmonary malformation presents good prognosis, excluding cases with fetal hydrops. Two-dimensional ultrasound is usually enough for diagnosis and follow-up. Computed tomography is the technique of choice to confirm the resolution of lesions after birth. Surgical treatment is pre ferable over conservative management, although it is unknown if the potential complications of this disease, even when asymptomatic, justify surgical morbidity.

Diagnóstico ecográfico prenatal y resultados neonatales de la malformación adenomatoidea quística pulmonar / Antenatal ultrasound diagnosis and neonatal results of the congenital cystic adenomatoid malformation of the lung

INTRODUCCIÓN: La malformación adenomatoidea quística (MAQ) es una patología congénita del pulmón poco frecuente y su diagnóstico prenatal es factible en la mayoría de los casos. Existen discrepancias en cuanto al manejo prenatal y tratamiento postparto. OBJETIVO: Analizar una serie de casos de MAQ diagnosticados prenatalmente, evaluando los hallazgos ecográficos y la evolución fetal y post natal. PACIENTES Y MÉTODO: Estudio retrospectivo de todos los casos diagnosticados prenatalmente por ecografìa entre 2005 y 2016 en dos hospitales de referencia. Los exámenes de ultrasonido fueron realizados utilizando ecógrafos de alta resolución, Toshiba Xario y Voluson 730 Expert Pro, con seguimiento desde el diagnóstico hasta el parto. Las variables analizadas incluyeron la edad gestacional en el momento del diagnóstico, las características de la lesión pulmonar, las malformaciones asocia das, el estudio citogenético, la evolución del embarazo, el tipo de parto, presencia de distrés respira torio, necesidad de pruebas de imagen complementarias, evolución clínica pediátrica y tratamientos postnatales necesarios. Se consideró resolución la desaparición total de la lesión ecográfica prenatal o que la radiografía torácica postnatal no mostrara lesión alguna. RESULTADOS: Se identificaron pre natalmente 17 casos. La evolución varía desde la resolución prenatal de la lesión hasta la persistencia de la misma tras el nacimiento. Tres pacientes decidieron abortar voluntariamente por hallazgos ecográficos de mal pronóstico. De los catorce casos restantes no hubo ningún caso de muerte fetal ni neonatal, un caso requirió cirugía tras el nacimiento y cuatro pacientes presentaron sintomatología leve durante el primer año de vida. Se ha reportado un caso de falso negativo con muerte neonatal, que la necropsia informó como MAQ tipo 0. CONCLUSIONES: Esta malformación pulmonar presenta buen pronóstico, excluyendo los casos con hidrops fetal. La ecografía bidimensional suele ser suficiente para el diagnóstico y el seguimiento. La tomografía computarizada es la técnica de elección para confirmar la resolución de las lesiones tras el nacimiento. El tratamiento quirúrgico es preferible sobre el manejo conservador, aunque se desconoce si las complicaciones potenciales de esta patología, aun siendo asintomática, justifican la morbilidad quirúrgica.

INTRODUCTION: Congenital cystic adenomatoid malformation (CCAM) is a rare congenital lung di sease, and in the most of cases, prenatal diagnosis is feasible. There are discrepancies regarding pre natal management and postpartum treatment. OBJECTIVE: To analyze prenatally diagnosed CCAM in our hospitals, in order to evaluate ultrasound findings with fetal and postnatal evolution. PATIENTS AND METHOD: Retrospective study of all cases diagnosed prenatally by ultrasound between 2005 and 2016 in two reference hospitals. The ultrasounds were performed using high-resolution ultrasound scanners, Toshiba Xario and Voluson 730 Expert Pro, with follow-up from diagnosis to delivery. The variables analyzed included gestational age at diagnosis, the characteristics of the lung lesion, associated malformations, cytogenetic study, the evolution of pregnancy, type of delivery, presence of respiratory distress, need for complementary imaging tests, pediatric clinical course, and necessary postnatal treatments. It was considered a resolution the total disappearance of the lesion in the pre natal ultrasound or that the postnatal chest X-ray showed no lesion. RESULTS: 17 cases were prenatally diagnosed. The evolution ranges from the prenatal resolution of the lesion to the persistence after bir th. Three patients voluntarily decided to have an abortion due to ultrasound findings of poor progno sis. Of the fourteen remaining cases there were no cases of fetal or neonatal deaths, one case required surgery after birth and four patients had mild symptoms during the first year of life. One case of false negative with neonatal death has been reported which necropsy reported as CCAM type 0. CONCLUSIONS: This pulmonary malformation presents good prognosis, excluding cases with fetal hydrops. Two-dimensional ultrasound is usually enough for diagnosis and follow-up. Computed tomography is the technique of choice to confirm the resolution of lesions after birth. Surgical treatment is pre ferable over conservative management, although it is unknown if the potential complications of this disease, even when asymptomatic, justify surgical morbidity.

[Congenital cystic adenomatoid malformation of the lung, intrauterine diagnostic and treatment. A case report and literature review]. / Enfermedad adenomatoidea quística pulmonar, diagnóstico y manejo intrauterino. Reporte de un caso y revisión bibliográfica.

The use of prenatal ultrasonography has improve the detection of fetal abnormalities, which affects the perception about the natural history and evolution of them, changing the management of the fetus and neonate. Today, it is possible to perform an early prenatal diagnosis, and be treated even intrauterine with high rates of success. Such is the case of Congenital Cystic Adenomatoid Malformation (CCAM), characterized by abnormal proliferation and dilatation of the terminal structures of the airway, generating multiple cysts of diferent sizes and locations. The CCAM can be asymptomatic until adulthood, but sometimes it is presented as an adverse perinatal outcome, manifested with ascites, hydrothorax, hydrops and hypoplasia of the affected lung. We report a case of a pregnant women, 32-year-old, diagnosed on the 18 week pregnancy by ultrasound Type 3 Congenital Cystic Adenomatoid Malformation disease. Treated with an intrauterine intervention percutaneous fetal sclerotherapy successfully.

Pre and perinatal aspects of congenital cystic adenomatoid malformation of the lung.

OBJECTIVE: To identify the incidence of congenital cystic adenomatoid malformation of the lung (CCAM) at birth; to evaluate prenatal and perinatal history, association with clinical and sociodemographic variables and concordance between CT scan results and anatomopathology studies. METHOD: Descriptive study based on the registry of malformed newborns, deliveries and patients records between August 1990 and November 2010. Ultrasonic, clinical, imaging and anatomopathologic information were studied. Association studies were made using chi-square test. Kappa was used to correlate CT scan to anatomopathology results. RESULTS: The incidence was 1:1980 (25/49 503). The mean gestational age for detection by ultrasonography was 24 ± 3.7 weeks. There were progression of the lesions in 11 cases (44%), stability in 6 (24%) and regression in 8 (32%). Three cases of CCAM followed due to polyhydramnios/hydrops died. There were neither familial cases nor association with sex, weight, age or maternal parity (p > 0.15). Radiographic abnormalities were found in 22/23 studied patients. The correspondence between CT scan and anatomopathologic was 0.77 (Kappa). CONCLUSIONS: The incidence was higher than the one described in the literature, probably, because it is a reference center in fetal medicine. The prenatal lesion involution rate was 32%, an intermediate proportion. There was good concordance between CT scan and anatomopathologic results. The polyhydramnios/hydrops were predictive of worst prognosis.

To shunt or not to shunt a pulmonary adenomatoid cystic malformation after 33 weeks of gestation: a case report.

CONTEXT: Macrocystic adenomatoid malformation of the lung can cause severe mediastinal shift, hydrops and polyhydramnios, thereby increasing the risk of perinatal deaths. After 33 weeks of gestation, repeated puncturing of the cyst is recommended. We present a case in which a cyst-amniotic shunt was placed instead of performing this procedure. CASE REPORT: A cyst-amniotic shunt was placed at 33 weeks of gestation because of a large macrocystic adenomatoid malformation of the lung associated with severe mediastinal shift and polyhydramnios. Although it was confirmed that the catheter was in the correct place, the cyst increased in size again two weeks later, associated with repetition of polyhydramnios. It was postulated that the catheter was blocked, and we chose to place another catheter instead of performing repeated punctures. The cystic volume, polyhydramnios and mediastinal shift regressed progressively. At 38.5 weeks, a 3,310/g male infant was delivered without presenting any respiratory distress. The infant underwent thoracotomy on the 15th day of life. Thus, in the present study, we discuss the possibility of placing a cyst-amniotic shunt instead of performing repeated cystic punctures, even at a gestational age close to full term.

Percutaneous fetal sclerotherapy for congenital cystic adenomatoid malformation of the lung.

OBJECTIVES: Congenital cystic adenomatoid malformation (CCAM) is a rare lesion of the developing fetal lung consisting of increased cell proliferation in the bronchial structures with lack of differentiation of the alveoli. Pregnancies may be at an increased risk for perinatal loss with type III CCAM. The purpose of this paper is to present our experience with the management of complicated types II and III CCAM with a novel technique: percutaneous ultrasound-guided fetal sclerotherapy (FST). METHODS: Three patients were referred with type II-III CCAM. Poor prognostic factors included hydrops, severe mediastinal shift, polyhydramnios and early gestational age (<26 weeks). Patients signed an informed consent. FST was performed with percutaneous injection of Ethamolin (ethanolamine oleate) or Polidocanol (aethoxysklerol) under ultrasound guidance using a 22-gauge needle. RESULTS: Resolution of hydrops and of the mass effect was observed in all cases without any complications. All patients were delivered at term. CONCLUSIONS: Fetal sclerotherapy is a novel approach to the management of complicated type II-III CCAM. Further studies are indicated to assess the risks and benefits of this innovative technique.

To shunt or not to shunt a pulmonary adenomatoid cystic malformation after 33 weeks of gestation: a case report / Derivar ou não uma malformação adenomatóide cística do pulmão após 33 semanas de gestação: relato de caso

CONTEXT: Macrocystic adenomatoid malformation of the lung can cause severe mediastinal shift, hydrops and polyhydramnios, thereby increasing the risk of perinatal deaths. After 33 weeks of gestation, repeated puncturing of the cyst is recommended. We present a case in which a cyst-amniotic shunt was placed instead of performing this procedure. CASE REPORT: A cyst-amniotic shunt was placed at 33 weeks of gestation because of a large macrocystic adenomatoid malformation of the lung associated with severe mediastinal shift and polyhydramnios. Although it was confirmed that the catheter was in the correct place, the cyst increased in size again two weeks later, associated with repetition of polyhydramnios. It was postulated that the catheter was blocked, and we chose to place another catheter instead of performing repeated punctures. The cystic volume, polyhydramnios and mediastinal shift regressed progressively. At 38.5 weeks, a 3,310/g male infant was delivered without presenting any respiratory distress. The infant underwent thoracotomy on the 15th day of life. Thus, in the present study, we discuss the possibility of placing a cyst-amniotic shunt instead of performing repeated cystic punctures, even at a gestational age close to full term.

CONTEXTO: A malformação adenomatóide do pulmão tipo macrocística pode causar compressão mediastinal grave, hidropisia e polihidrâmnio aumentando a chance de óbito perinatal. Após a 33ª semana de gestação, recomenda-se realizar punções repetidas do cisto. Apresentamos um caso em que um dreno cístico-amniótico foi colocado e as punções foram evitadas. RELATO DE CASO: Um dreno cístico-amniótico foi colocado na 33ª semana de gestação devido a grande malfomação adenomatóide cística do pulmão associada a desvio de mediastino grave e polihidrâmnio. Apesar de o cateter ter sido identificado no local correto, o cisto voltou a crescer duas semanas após, repetindo o polihidrâmnio. Postulou-se que o cateter estava obstruído e optamos por colocar um novo cateter ao invés de realizar punções repetidas desse cisto. Foi observada regressão progressiva do volume do cisto, do polihidrâmnio e do desvio de mediastino. Na 38ª semana e meia, um menino de 3.310 g nasceu sem apresentar dificuldade respiratória, sendo submetido a toracotomia no 15º dia de vida. Portanto, no presente estudo, discute-se sobre a possibilidade de colocação do dreno cístico-amniótico no lugar de realizar punções repetidas do cisto mesmo em idades gestacionais próximas do termo.

Malformaciones adenomatoideas congénita pulmonares: diagnóstico prenatal: reporte de seis casos / Congenital cystic adenomatoid malformations of lung: prenatal diagnosis: report of six cases

Las malformaciones adenomatoideas congénitas pulmonares son una anomalía fetal rara. Presentamos seis casos diagnosticados in útero. Los criterios diagnósticos y pronósticos son claramente tratados.

Malformación adenomatosa quística pulmonar: diagnóstico prenatal y evaluación de un caso / Prenatal diagnosis and evaluation of cystic adenomatoid pulmonary malformation case

Se presenta el caso clínico de una mujer que cursa su segundo embarazo, donde se le diagnostica ecográficamente al feto una malformación adenomatosa quística pulmonar (MAQP), a las 19 semanas. La evolución del embarazo fue sin complicaciones, llegando a término y sin requerir tratamientos intrauterinos ni estudios especiales. El recién nacido fue dado de alta con buena evolución y asintomático. Dado lo infrecuente de la enfermedad, así como su evolución y los pocos casos de diagnóstico prenatal reportados, se decide su presentación. (AU)

Malformación adenomatosa quística pulmonar: diagnóstico prenatal y evaluación de un caso / Prenatal diagnosis and evaluation of cystic adenomatoid pulmonary malformation case

Se presenta el caso clínico de una mujer que cursa su segundo embarazo, donde se le diagnostica ecográficamente al feto una malformación adenomatosa quística pulmonar (MAQP), a las 19 semanas. La evolución del embarazo fue sin complicaciones, llegando a término y sin requerir tratamientos intrauterinos ni estudios especiales. El recién nacido fue dado de alta con buena evolución y asintomático. Dado lo infrecuente de la enfermedad, así como su evolución y los pocos casos de diagnóstico prenatal reportados, se decide su presentación.

Enfermedad adenomatoidea quística pulmonar con hipertensión arterial pulmonar (uso de ventilación de alta frecuencia oscilatoria, óxido nítrico inhalado y cirugía) / Cyst adenomatoid malformation of the lung with pulmonary arterial hypertension (use of oscillatory high frequency ventilation, nitric oxide and surgery

Objetivo. Informar de un caso de enfermedad adenomatoidea quística pulmonar diagnosticada in utero que cursó con insuficiencia respiratoria e hipertensión pulmonar severa, no logrando corregirla con neumonectomía, ventilación de alta frecuencia oscilatoria y óxido nítrico inhalado. Caso clínico. Masculino a término eutrófico complicado con polihidramnios e imágenes quísticas en la cavidad torácica izquierda a las 26 semanas de gestación. Al nacimiento y por la posibilidad de hernia diafragmática congénita se orointuba para ventilación asistida. La radiología simple y contrastada diagnosticó enfermedad adenomatoidea quística del pulmón. Se usó inicialmente la ventilación mecánica convencional sin oxigenarlo ni ventilarlo. El ecocardiograma, oximetrías pre y posductales mostraron hipertensión pulmonar severa y cortocircuito de derecha a izquierda por el conducto arterioso. La ventilación de alta frecuencia oscilatoria fue capaz de corregir la insuficiencia respiratoria. Posteriormente se neumonectomiza, recae en hipoxemia e hipercapnea. Se aplica óxido nítrico inhalado. Las alteraciones no se corrigen y fallece (17 horas de vida). Discusión. La malformación adenomatoidea quística pulmonar acompañada de hipertensión pulmonar severa es rara. El análisis del caso considera usar de inicio la ventilación de alta frecuencia oscilatoria y óxido nítrico inhalado y estabilizado el paciente, efectuar resección quirúrgica del pulmón, como en la hernia diafragmática congénita, en donde el estrés quirúrgico incrementa la hipertensión pulmonar y de no lograrlo, considerar oxigenación extracorpórea. Conclusiones. 1) La enfermedad adenomatoidea quística pulmonar puede cursar con hipertensión pulmonar. 2) Estos pacientes deben estabilizarse con ventilación asistida y óxido nítrico inhalado previo a la resección quirúrgica, de no lograrlo, considerar el uso de la oxigenación extracorpórea