Congenital pulmonary airway malformation

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.

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.

Malformación congénita de la vía aérea pulmonar o malformación adenomatoidea quística congénita: Serie clínica de 16 pacientes / Congenital pulmonary airway malformation or congenital cystic adenomatoid malformation

Objective: To describe 16 patients treated for a congenital pulmonary anomaly named congenital pulmonary airway malformation (CPAM) or congenital cystic adenomatoid malformation (CCAM) in Roberto del Río children S Hospital. Methods: We retrospectively analyzed the medical records of patienes who had had surgery for CPAM/CCAM from January 2000 to July 2014. Results: We found 16 cases, 9 of them were males. Nine had surgery within the first year of life (5 of them within 3 weeks of life). According to Stocker 's classification CPAM/CCAM was type 1 in 9 patients, and type 2 in 7. Diagnosis was based on antenatal ultrasonography s (n = 10), persistent abnormalities in Chest X-Ray (n = 4), recurrent pneumonia (n = 1) and incidental radiological findings (n = 1). Computed Tomography (CT) was performed in 13 patients, CT angiography in 2 and only chest x-ray in one. The lesions were located in right upper lobe (3), right middle lobe (2), right lower lobe (4), left upper lobe (4) and left lower lobe (3). One patient had pectus excavatum and another one had ventricuar septal defect. They all had open thoracotomy with lobectomy of the affected area. Postoperatively was uneventful except for transient lobar atelectasis in 2 patients. There was no mortality. Conclusions: We emphasize the early diagnosis and good outcome of our patients treated which this disease. Our findings are consistent with those that has been reported in the literature.

Objetivo: Describir los pacientes tratados por malformación congénita de la vía aérea pulmonar (MCVAP) o malformación adenomatoidea quística congénita (MAQC) en el Hospital de niños Roberto del Río desde el año 2000 hasta el segundo semestre de 2014. Métodos: Revisión retrospectiva de fichas clínicas. Resultados: 16pacientes, 9 hombres. Se operaron antes del año de vida 9 pacientes (5 de ellos antes de las 3 semanas de vida). Tipo de MCVAP (según clasificación de Stocker) tipo 1 fue 9/16 y tipo 2 fueron 7/16. La sospecha diagnóstica fue en base a diagnóstico prenatal 10/16, imagen radiológica persistente 4/16, neumonías repetidas 1/16 y hallazgo radiológico 1/16. Estudio imagenológico: Tomografia computada (TC) 13/16, Angio-TC 2/16y radiografía de tórax 1/16. Ubicación: lóbulo superior derecho 3/16, lóbulo medio 2/16, lóbulo inferior derecho 4/16, lóbulo superior izquierdo 4/16, lóbulo inferior izquierdo 3/16. Comorbilidad: ninguna 14/16, pectus excavatum 1/16, comunicación interventricular 1/16. Tratamiento quirúrgico: en todos los pacientes se realizó lobectomía total del lóbulo afectado por vía abierta. Evolución postoperatoria: Buena 14/16, atelectasia 2/16. No se registró mortalidad. Conclusiones: Se destaca el diagnóstico precoz y buena evolución de nuestros pacientes concordantes con la literatura.

The Clinical Courses of Patients with Congenital Cystic Adenomatoid Malformation Complicated by Pneumonia

PURPOSE: We evaluated the clinical characteristics and courses of patients with congenital cystic adenomatoid malformation (CCAM) complicated by pneumonia. MATERIALS AND METHODS: We retrospectively reviewed the records of 19 adult patients with surgically confirmed CCAM between March 2005 and July 2013. RESULTS: Eighteen of nineteen patients presented with acute pneumonia symptoms and signs, and inflammatory markers were elevated. On chest computed tomography, all 18 patients had parenchymal infiltration around cystic lesions, 17 (94%) had an air-fluid level, and 2 (11%) had pleural effusion. After antibiotics treatment for a median of 22 days prior to surgery, all acute pneumonia symptoms and signs disappeared in 17 (94%) patients at a median of 10 days. Improvements and normalization of inflammatory marker levels, occurred in 17 (94%) and 9 (50%) patients at medians of 8 and 17 days, respectively. Radiological improvement was evident in 11 (61%) patients, at a median of 18 days, of these patients, partial radiological improvement occurred in 10 (56%) and complete radiological resolution in only 1 (6%). One patient (6%) did not improve in terms of clinical, laboratory, or radiological findings despite antibiotic treatment for 13 days. Consequently, after 17 (94%) elective and 1 (6%) emergency surgeries, all patients improved without development of complications. CONCLUSION: We described the clinical characteristics and courses of patients with CCAM complicated by pneumonia, and showed that surgery may be performed safely after clinicolaboratory improvement is attained upon antibiotic treatment, even in the absence of complete radiological resolution.

Neumotórax hipertensivo como forma de presentación de una malformación adenomatoidea quística pulmonar / Tension pneumothorax as a presentation of congenital cystic adenomatoid malformation of the lung

El propósito de este trabajo es examinar el caso de un paciente con una forma de presentación no habitual y grave de una patología poco frecuente, como la malformación adenomatoidea quística pulmonar. Se trata de un lactante de 50 días de vida que ingresó en la unidad de cuidados intensivos pediátricos con insuficiencia respiratoria y colapso circulatorio refractario a la expansión de volumen y los inotrópicos. La radiografía de tórax mostró una imagen radiolúcida, homogénea, en la base pulmonar derecha, que parecía corresponder a una lesión bullosa en el lóbulo inferior derecho, asociada a neumotórax homolateral. Se realizó drenaje con colocación de un tubo de avenamiento pleural y se observó una mejoría clínica posterior. Se efectuó la resección del quiste mediante lobectomía inferior derecha; el diagnóstico histopatológico fue malformación adenomatoidea quística de la vía aérea de tipo 4 (clasificación de Stocker). El paciente evolucionó favorablemente.

The purpose of this paper is to examine the case of a 50-day-old patient with an unusual and severe presentation of a rare disease: congenital cystic adenomatoid malformation of the lung. The infant was admitted to the pediatric intensive care unit with respiratory failure and circulatory collapse refractory to intravenous fluids and inotropic drugs. Chest X-ray showed a radiolucent homogeneous image in the right lung base, suggesting bullous lesion of the right lower lobe associated with ipsilateral pneumothorax. Drainage was performed by placing a chest tube with subsequent clinical improvement. Later the cyst was resected by right lower lobectomy; the histopathological diagnosis was congenital cystic adenomatoid malformation type 4 (Stocker classification). The patient evolved favorably.

[Congenital pulmonary malformations: clinical, radiological and treatment features]

Congenital broncho-pulmonary malformations [CBM] are rare, essentially presented by congenital lobar emphysema, bronchogenic cysts, pulmonary sequestrations and cystic adenomatoid malformations. The diagnosis can be in prenatal. In postnatal, symptoms are variable. Radiological investigations lead to diagnosis in all cases. To study the principal clinic, radiologic and therapeutic of the congenital broncho-pulmonary malformations through ten cases. Retrospective study of 10 cases of congenital bronchopulmonary malformations diagnosed between 2003 and 2010 in our institution. The mean ages at the time of diagnosis is 2months [4 days to 16months]. The sex ratio is 1. The symptoms consisted of recurrent pneumonia in 4cases, respiratory distress in 2cases, bronchiolite in 2 cases and 2cases of antenatal diagnosis. All patients have a chest X-ray, night patients have a chest computerized tomography and one patient has a bronchial endoscopy. Ten cases of BPM have been investigated: five congenitals lobar emphysema, tow pulmonary sequestrations, tow cystic ad‚nomatoid malformation and one bronchogenic cyst. Eight patients required surgical treatment involving pneumonectomy [1case], lobectomy [5 cases], segmentectomy [1 case] and in 1 case the pulmonary sequestration was treated by ligature of the anomalous artery with pulmonary resection. The histopathological examination confirmed the diagnosis in all cases. The postoperative period was uneventful in 8 cases with a mean of follow-up of 2 years [5 months to 5years]. Tow patient died after surgical treatment. The diagnosis of BPM malformations can be clinical, confirmed by radiological investigations. The improvement in prenatal ultrasound diagnosis modified the management strategy. The treatment varies frome attitude conservatrice to pneumonectomy

Masa pulmonar ecogenética fetal: presentación de un caso / Echogenic fetal lung mass: case report

Toda masa pulmonar ecogénica se considera patológica, pero la gravedad de la misma varía según la etiología de la imagen, que puede variar desde entidades con mala evolución fetal y neonatal a otras que desaparecen sin dejar secuelas. Se presenta un caso de una masa pulmonar que desaparece al tercer trimestre.

Congenital cystic adenomatoid malformation in an adolescent: An unusual presentation with pleural effusion and pneumatocele.

Congenital cystic adenomatoid malformation (CCAM) encompasses a continuum of hamartomatous cystic lung lesions characterised by the presence of abnormal bronchiolar structures of varying sizes or distribution. The CCAM is a disorder of infancy with majority of the cases being diagnosed within the first two years of life. We describe CCAM in a 13-yearold girl complaining of recurrent lower respiratory tract infections since infancy who presented with post-infectious pneumatocele with loculated pleural effusion, and suspected abscess formation and had undergone resection.

Malformación adenomatoidea quística congénita / Congenital cystic adenomatous malformation

Introduction: Congenital Cystic Adenomatous Malformation (CCAM) is an infrequent entity due to an alteration in alveolar-pulmonary development. Material and Methods: A descriptive, retrospective study of newborns presenting CCAM in a tertiary care hospital in Madrid, Spain. Results: Seven patients were found. All were full term, normal weight births. Two patients showed respiratory distress at birth. Two chest x-rays were normal. CT scans showed three clear CCAM lesions, four suggestive of hybrid lesions. All were referred to medical centers with pediatric surgery for followup. Discussion: At birth, this pathology may be asymptomatic and appear as a chest x-ray finding. Long term management is complicated by infection and malignization. Conservative treatment of asymptomatic patients includes regular follow up of lesions. Surgical treatment is reserved for symptomatic or complicated patients. Conclusions: Prenatal suspicion of CCAM is important since clinical exam and radiology may be normal in neonatal period. Chest CT scans are important in confirming diagnosis and determining future surgery. More studies are necessary for the proper diagnosis and management of this disorder.

Introducción: La malformación adenomatoidea quística (MAQ) es una entidad congénita poco frecuente debida a una alteración en el desarrollo alveolar pulmonar. Pacientes y Método: Se realizó un estudio descriptivo y retrospectivo de los recién nacidos con el diagnóstico prenatal de MAQ durante 6 años en un hospital terciario de Madrid (España). Resultados: Se encontraron un total de siete pacientes. Todos fueron recién nacidos a término de peso adecuado. Dos pacientes presentaron distress respiratorio al nacimiento. Dos radiografías de tórax fueron normales. En la tomografía axial (TAC), tres lesiones fueron MAQ y cuatro fueron su-gerentes de lesión híbrida. Todos se derivaron a centro con cirugía pediátrica para seguimiento. Discusión: Al nacimiento, esta patología puede permanecer asintomática y ser un hallazgo casual en una radiografía torácica. A largo plazo el riesgo de infección y malignización complican el manejo. El tratamiento conservador, que se dirige a pacientes asintomáticos, obliga a realizar controles seriados de las lesiones. El tratamiento quirúrgico se reserva para los pacientes con sintomatología o complicaciones postnatales. Conclusiones: El diagnóstico de sospecha prenatal de MAQ es fundamental dado que la clínica y radiología pueden ser normales en el período neonatal. Se debe realizar TAC torácico para confirmar la lesión y valorar futura cirugía. Se necesitan más estudios sobre el correcto diagnóstico y manejo de esta patología.

Malformación adenomatoidea quística pulmonar: importancia del diagnóstico prenatal / Cystic adenomatoid malformation of the lung: importance of prenatal diagnosis

La malformación adenomatoidea quística pulmonar (MAQP)es una anomalía de la vía aérea pulmonar poco frecuente cuyodiagnóstico suele realizarse en el período prenatal medianteecografía. Durante el embarazo, debe realizarse seguimiento ecográfico para valorar el desarrollo pulmonar. Presentamos el caso clínico de una paciente de 4 años con diagnóstico prenatal de MAQP, no confirmado mediante radiografía de tóraxrealizada al nacimiento, lo cual retrasó el diagnóstico definitivo; fue intervenida con 4 años de edad tras haber presentado varias neumonías a repetición. Una radiografía de tórax normal realizada al nacimiento no descarta la presencia de estamalformación, por lo que es necesario realizar una tomografía computarizada a las 4 semanas del nacimiento para confirmar o descartar la MAQP. Una vez diagnosticada, el tratamiento quirúrgico debe ser precoz para evitar complicaciones.

Cystic adenomatoid malformation of the lung is a rare malformation of the lung airway which often performed diagnosed in the prenatal period by ultrasound. Ultrasound monitoring should be performed during pregnancy to assess lung development. We report the case of a 4-year-old patient with prenatal diagnosis of cystic adenomatoid malformation of the lung, not confirmed by chest radiograph at birth. The patient underwent surgery at 4 years of age after diagnosis was made for presenting recurrent pneumonia. A normal chest radiograph at birth does not exclude this malformation and a computerized tomography at 4 weeks of birth must be done to confirm or rule out this anomaly. Once the diagnosis is made, surgical treatment should be prompted to avoid complications.

Malformações pulmonares congênitas / Congenital lung malformations

As malformações congênitas do pulmão são raras e variam muito na sua forma de apresentação clínica e gravidade, dependendo principalmente do grau de envolvimento pulmonar e de sua localização na cavidade torácica. Elas podem se manifestar em qualquer idade e podem ser fonte de importante morbidade e mortalidade em lactentes e crianças. Os indivíduos com malformações congênitas do pulmão podem apresentar sintomas respiratórios ao nascimento, enquanto outros podem permanecer assintomáticos por longos períodos. Atualmente, com o uso rotineiro da ultrassonografia pré-natal, vem ocorrendo um aumento no diagnóstico mais precoce dessas malformações. A manifestação clínica dessas malformações varia desde uma disfunção respiratória pós-natal imediata a um achado acidental na radiografia de tórax. O diagnóstico precoce e o tratamento imediato oferecem a possibilidade de um desenvolvimento pulmonar absolutamente normal. Quando assintomáticos, a conduta para o tratamento dos pacientes com malformações pulmonares ainda é controversa, uma vez que o prognóstico dessas afecções é imprevisível. O manejo dessas lesões depende do tipo de malformação e de sintomas. Devido ao risco de complicação, a maioria dos autores sugere a ressecção da lesão no momento em que essa é identificada. A lobectomia é o procedimento de escolha, fornecendo excelentes resultados a longo prazo. Este artigo descreve as principais malformações pulmonares congênitas, seu diagnóstico e controvérsias quanto o tratamento.

Congenital lung malformations are rare and vary widely in their clinical presentation and severity, depending mostly on the degree of lung involvement and their location in the thoracic cavity. They can manifest at any age and can be the source of significant morbidity and mortality in infants and children. Individuals with congenital lung malformations can present with respiratory symptoms at birth or can remain asymptomatic for long periods. Recently, there has been an increase in the early diagnosis of these malformations, a change that is attributable to the routine use of prenatal ultrasound. The clinical manifestation of these malformations varies from respiratory distress in the immediate postnatal period to an incidental finding on chest X-rays. Early diagnosis and prompt treatment offer the possibility of absolutely normal lung development. The treatment of asymptomatic patients with lung malformations is controversial, because the prognosis of these diseases is unpredictable. The management of these lesions depends on the type of malformation and symptoms. Because of the risk of complications, most authors recommend resection of the lesion at the time of diagnosis. Lobectomy is the procedure of choice and yields excellent long-term results. This article describes the principal congenital lung malformations, their diagnosis, and the controversies regarding treatment.

Embriología del desarrollo de los bronquios y el parénquima pulmonar / Embryology of development of the bronchi and lung parenchyma

Las malformaciones congénitas de aparato respiratorio son un extenso número de patologías que oscilan en gravedad dependiendo del momento embriológico en que se dio el fallo y cuales mecanismos fueron afectados. En la revisión se profundiza en patologías congénitas como el secuestro broncopulmonar y la malformación adenomatoide quística...

role of multi-detector computed tomography in the assessment of cystic and cavitary pulmonary lesions

Lung cysts and cavities are well defined lesions with definable walls containing air or fluid. The differential diagnosis is broad including congenital, idiopathic, infective or neoplastic lesions. Multidetector row CT is primary non-invasive evaluation of cystic and cavitary lesions. To assess the role of multi-detector computed tomography [MDCT] in evaluation of cystic and cavitary lesions in the lung. The study was conducted on 63 patients with cystic or cavitary pulmonary lesions and subjected to MDCT. The study included 33 patients with infective lesions, 13 patients with idiopathic lesions, eight patients with congenital lesions, seven patients with neoplastic lesions and two patients with pseudocystic lesions proved to be due to diaphragmatic hernias. MDCT is an accurate safe diagnostic modality in assessing cystic and cavitary lung lesions; it can assess wall thickness, size, contents and surrounding parenchyma

Malformación congénita de la vía aérea pulmonar: reporte de un caso adulto / Congenital pulmonary airway malformation: an adult case report

Congenital pulmonary airway malformation or CPAM (congenital cystic adenomatoid malformation) that involve the lung is an embryonic anomaly. CPAM is a rare condition in chilhood, and even more so in adulthood. In this article we report the case of a young adult man with CPAM and present a brief review about this condition. Case study: 23 years old man with several episodes of pneumonia and obstructive bronchial syndrome during his childhood. The patient complained of pleuritic chest pain, malaise and fever. On physical examination lung sound was diminished in the lower right zone of the chest, abundant coarse crackles were detected and there was dullness on percussion. Chest roentgenogram and computed tomography (CT) revealed a complex image of multiple cysts with air and fluid in the right lung. Because of the lack of response to medical treatment a right lower lobectomy was performed. Histopathology revealed a type 1 CPAM. After being discharged from the hospital the patient developed a broncho-pleural fistula, that was succesfully treated by a second surgical intervention. Three years after surgery the patient is free of symptoms. Discussion: CPAM is a rare congenital hamartomatous condition. This malformation in adults it is exceptional. Chest CT is the choice diagnostic technique. Considering the high neoplastic potential of this malformation, its treatment should be the surgical resection, even in asymptomatic patients.

La malformación congénita de la vía aérea pulmonar (MCVAP), llamada también malformación congénita adenomatoidea quística que compromete al pulmón es una anomalía embrionaria. La MCVAP es de rara presentación en la infancia y es excepcional en los adultos. En este artículo comunicamos el caso de un hombre adulto con MCVAP y presentamos una breve revisión sobre esta condición. Caso clínico: Hombre de 23 años de edad con varios episodios de neumonía y de síndrome bronquial obstructivo en su niñez. El paciente se quejaba de dolor torácico pleurítico, malestar general y fiebre. En el examen físico se encontró disminución del murmullo pulmonar en la parte inferior del hemitórax derecho, abundantes crepitaciones y matidez. La radiografía y la tomografia computada de tórax (TC) revelaron múltiples imágenes hidro-aéreas complejas en el pulmón derecho. Debido a la falta de respuesta al tratamiento médico, se practicó una lobectomía inferior derecha. La histopatología demostró una MCVAP tipo 1. Después de ser dado de alta el paciente presentó una fístula broncopleural, la cual fue tratada exitosamente con una segunda intervención quirúrgica. Tres años después de la cirugía el paciente está asintomático. Discusión: La MCVAP es una malformación congénita hamartomatosa muy poco frecuente. En adultos es excepcional. La técnica diagnóstica de elección es la TC de tórax. Considerando el alto potencial neoplásico de esta malformación, su tratamiento debe ser la resección quirúrgica, incluso si el paciente está asintomático.

Malformação adenomatóide cística / Cystic adenomatoid malformation

A patologia é uma malformação pulmo nar rara, acreditando ser uma falha na conexão entre o desenvolvimento do coto brõnquico e os espamos aéreos distais. O presente trabalho relata um caso de malformação adenomatoide cística, diagnosticada intra-útero, durante a realização de ultra-sonografia de rotina, em uma gestante de 18 anos, com vinte e duas semanas de idade gestacional e o acompanhamento pós natal, dando ênfase aos exames com plementares antes e após a intervenção cirúrgica (lobectomia parcial direita).

Perinatal management of common neonatal thoracic lesions.

Esophageal atresia, congenital diaphragmatic hernia, bronchopulmonary malformations and cystic lung diseases are the common neonatal thoracic surgical lesions encountered in practice. The availability of antenatal ultrasonography has lead to these lesions being detected before birth. Antenatal diagnosis can be made with a fair degree of accuracy in tertiary fetal medicine centres. Antenatal intervention is limited in a very few centres in the western world and not being done in India at present. The outcome of these babies with antenatal diagnosis of thoracic lesions has changed in the last decade. Earlier intervention is now possible in cystic lung disease before infectious complication has set in. All these lesions are managed exclusively in well developed neonatal surgery units with excellent outcome in the western world. The present study reviews the antenatal detection, clinical presentation, interventional/surgical procedures [antenatally and postnatally] and outcome of these common neonatal thoracic surgical lesions.

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.