Malformación congénita de la vía aérea pulmonar: a propósito de un caso clínico / Congenital pulmonary airway malformation: a clinical case study / Malformação congênita das vias aéreas pulmonares: relato de caso

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.

Malformación adenomatoidea quística congénita asociada a secuestro broncopulmonar en un recién nacido / Congenital cystic adenomatoid malformation presenting with bronchopulmonary sequestration in a newborn

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.

Congenital cystic adenomatoid malformation, antenatal diagnosis: about one case

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.

Cirugía fetal en tumores pulmonares mediante escleroterapia

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.

Manejo prenatal de la malformación adenomatoidea quística pulmonar, variedad macroquística: Reporte de los primeros casos tratados intraútero en el Perú y revisión de la literatura / Prenatal management of congenital cystic adenomatoid lung malformation, macrocystic type: Report of a series of cases treated in utero in Peru and review of the literature

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.

Case of mucinous adenocarcinoma of the lung associated with congenital pulmonary airway malformation in a neonate / 소아과

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.

Progress in diagnosis and treatment of congenital cystic adenomatoid malformation of the lung in children / 国际儿科学杂志

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.

Congenital pulmonary airway malformation with atrial septal defect and pulmonary hypertension for lobectomy‑anesthetic considerations.

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.

Comparative analysis in ultrastructure and histological types for congenital cystic adenomatoid malformation / 中华实用儿科临床杂志

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.

Congenital pulmonary airway malformation / 中华实用儿科临床杂志

Congenital pulmonary airway malformation (CPAM),which was previously known as congenital cystic adenomatoid malformation,is one of the most common congenital lung abnormalities.In recent years,more and more reports about the disease have been published and its classifications also have new changes.This article introduces the concept of CPAM,classification,clinical manifestations,differential diagnosis,and treatment strategies.

Minimally invasive surgery for congenital cystic adenomatoid malformations - early experience

PURPOSE: The aim of this study is to present our experience with minimally invasive surgery (MIS) for congenital cystic adenomatoid malformations (CCAMs). METHODS: The medical records of infants under 2 years of age who underwent operation for a CCAM from 2009 to 2014 were retrospectively reviewed. RESULTS: MIS (9 of thoracoscopy and 1 of laparoscopy) was performed for 10 infants (male:female = 7:3) with CCAM. CCAM were discovered prenatally around gestational age of 24.7 weeks. The median gestational age was 38.6 weeks, and the median body weight was 2,817.5 g. None had respiratory distress after birth. The median age at the time of operation was 0.94 years (range: 8 days-1.66 years). Two underwent the operation during the neonatal period; one because of a coexisting large esophageal duplication cyst and the other due to diagnostic uncertainty. While awaiting operation, 5 of CCAM had grown without respiratory symptoms, and 2 infants had experienced pneumonia. The mean operative time was 98 minutes (range: 70-227 minutes), and there were no conversions or perioperative complications. The infants resumed enteral feeding within 2 days and were discharged within 7 days, except for 1 infant who underwent esophageal duplication cyst excision. During the follow-up period, there were no cases of either remnant lesions or respiratory symptoms. CONCLUSION: MIS for CCAMs is safe and feasible, with excellent cosmesis and short hospital stays. Increasing experience with various MIS procedures will widen the indications for MIS in lung pathology.

Clinical manifestations,imaging findings and pathological features of 15 cases with congenital cystic adenoma-toid malformation in the lung / 中华实用儿科临床杂志

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.

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.

Anesthetic management of infant lung resection of congenital cystic adenomatoid malformation by video-assisted thoracicscopy / 实用医学杂志

Objective To investigate the feasibility and safety of OLV anesthesia about infant lung resection of CCAM by video-assisted thoracicscopy. Methods Endo-tracheal intubation was performed after 43 CCAM infants had undergone rapid intravenous induction. One side of lungs was ventilated by injecting 4 ~ 6 mmHg CO2 for the construction of artificial pneumothorax, and the side lung was compressed forming OLV. SpO2, ECG, MAP, PETCO2, T, PaO2, PaCO2, bleeding volume and urine volume were monitored. The numerical value of SpO2, PaO2, HR, MAP, PETCO2, and PaCO2 were recorded at scheduled intervals. Results Compared with 5min after induction,the PaO2,HR and MAP of the infants significantly reduced; the PETCO2 and PaCO2 significantly increased at OLV at 10 min and 60 min. Compared with OLV at 10 min, the PaO2, PETCO2 significantly increased at OLV 60 min. Conclusion Appropriate respiratory management and drug usage are feasible and safe for infant surgery of CCAM by video-assisted thoracicscopy.

Clinical analysis of neonatal congenital cystic lung lesions in 28 cases / 中国小儿急救医学

Objective To explore the clinical characteristics of neonatal congenital cystic lung lesions. Methods Between January 2008 and June 2014,total 28 cases diagnosed congenital cystic lung lesions in neonatal center of Guangzhou Women and Children's Hospital were collected.The clinical data were analyzed including manifestations,lesion characteristics,imaging,diagnosis,treatment and prognosis,and the related literature were reviewed.Results There were 20 male and 8 female,16 cases of lobar emphysema,7 cases of pulmonary seques-tration,4 cases of congenital cystic adenomatoid malformation and 1 case of bronchogenic cyst.Main symptoms were dyspnea(78.57%),cyanosis (39.29%),wheezing cough (17.86%),feeding difficulties (14.29%),fever (10.71%),asymptomatic(21.43%).Two cases combined with congenital heart disease,4 cases combined with other malformations(such as diaphragmatic hernia,laryngeal stridor,funnel chest,polycystic kidney).There were lesions in chest CT image,4 cases underwent lobectomy,other cases underwent conservative treatment.Seventeen cases got better and discharge,3 cases dead.Conclusion The most common type of congenital cystic lung lesions in neonate is lobar emphysema.Main manifestations are dyspnea,cyanosis.If we suspect congenital cyst-ic lung lesions,we should do chest CT or MRI scan soon ,also do echocardiography and ultrasound to exclude other malformations.The treatment effect is satisfactory,and the outcome need long time to follow up.

Malformación congénita de la vía aérea pulmonar / Congenital pulmonary airway malformation

La malformación congénita de la vía aérea pulmonar, anteriormente llamada malformación adenomatoidea quística pulmonar, es una rara anormalidad en el desarrollo de las vías respiratorias terminales. Las lesiones son de distribución y tamaños variables, usualmente unilaterales. El diagnóstico puede realizarse desde el periodo prenatal mediante ecografía gestacional, encontrándose en ocasiones, graves repercusiones fetales como hidrops fetal, el cual es el mayor predictor de muerte. En los recién nacidos la enfermedad se manifiesta con dificultad respiratoria aguda; en los niños y adultos con infecciones pulmonares recurrentes y complicaciones como absceso pulmonar, hemoptisis, neumotórax y manifestaciones extrapulmonares. 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 de una paciente femenina de cuatro años de edad con infecciones pulmonares recurrentes; las radiografías de tórax y la tomografía pulmonar sugieren malformación adenomatoidea quística, la cual es confirmada posteriormente por estudio patológico.

Congenital pulmonary airway malformation, previously known as congenital cystic adenomatoid malformation, is a rare developmental anomaly of the terminal respiratory structures. Cysts can vary in size, distribution, and are most commonly unilateral. The diagnosis can be made prenatally using ultrasound scanning; the fetal lesions can be associated with hydrops fetalis, which is the major predictor of death. In newborns, it manifests as acute respiratory distress, in children and adults, manifestations include recurrent lung infections, with complications such as lung abscess, hemoptysis, pneumothorax and extrapulmonary anomalies. Surgical treatment is indicated in symptomatic patients to prevent infections and the potential neoplastic transformation, however prophylactic surgery versus expectant treatment remains controversial in asymptomatic patients. We present a 4 year old female patient with recurrent pulmonary infections whose thoracic x-ray and pulmonary computed axial tomography suggests cystic adenomatoid malformation, subsequently confirmed by pathological study.

Malformaciones pulmonares congénitas / Congenital lung malformations

Congenital lung malformations (CLM) comprise a heterogeneous group of lung diseases. They vary widely in their clinical presentation and severity, depending 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. Patients with CLM can present respiratory symptoms at birth or can remain asymptomatic for long periods. There has been an increase in early diagnosis of CLM attributable to the routine use of prenatal ultrasound. Management of these lesions depends on the type of malformation and symptoms. Treatment of asymptomatic patients is controversial, because the prognosis of these diseases is unpredictable. Because of the risk of complications, most authors recommend resection of the lesion at the time of diagnosis. This review describes the principal CLM, their diagnosis, and the controversies regarding treatment.

Las malformaciones pulmonares congénitas (MPC) constituyen un grupo heterogéneo de enfermedades. Varían en su presentación clínica y gravedad en función al grado de afectación pulmonar y a su ubicación en tórax. Se pueden manifestar a cualquier edad y pueden condicionar morbilidad y mortalidad significativa en lactantes y niños. Los pacientes pueden presentar síntomas respiratorios desde el nacimiento o permanecer asintomáticos durante largos períodos. Se ha observado un aumento en el diagnóstico precoz de las MPC atribuible al uso rutinario de la ecografía prenatal. El manejo de estas lesiones depende del tipo de malformación y de los síntomas. El tratamiento de pacientes asintomáticos es controvertido debido a que el pronóstico de estas enfermedades es desconocido. La mayoría de los autores recomiendan la resección de la lesión en el momento del diagnóstico debido al riesgo de complicaciones. Esta revisión describe las principales malformaciones congénitas del pulmón, su diagnóstico y las estrategias de tratamiento.

Diagnóstico antenatal de malformação adenomatoide cística congênita unilateral associada à síndrome de Taussig-Bing:relato de caso / Antenatal diagnosis of unilateral congenital cystic adenomatoid malformationassociated with Taussig-Bing syndrome: case report

Objetivos: Relatar um caso raro de diagnóstico antenatal de malformação adenomatoide cística congênita unilateral associada à síndromede Taussig-Bing.Descrição do caso: Paciente com 40 anos, quatro gestações com três partos vaginais prévios, cor de pele negra, casada, dona de casa, sempatologias prévias, foi encaminhada à Unidade de Medicina Fetal do Hospital Universitário de Santa Maria devido a alterações na regiãotorácica do feto em exame ultrassonográfico obstétrico rotineiro realizado com 30 semanas e três dias de gestação. Ao exame ultrassonográficocom Doppler realizado no serviço de referência, foram constatadas comunicação interventricular perimembranosa e transposição dos grandesvasos da base, caracterizando a síndrome de Taussig-Bing. Adicionalmente, foi constatada a presença na área pulmonar da malformaçãoadenomatoide cística congênita unilateral do tipo 2. Decidiu-se pela realização de parto por via alta em centro de referência de cardiologia, com37 semanas de gestação. O recém-nascido, com peso de 3 kg, apresentava desde a primeira hora de vida disfunção respiratória, com cianoseimportante, sendo submetido à lobectomia. O recém-nascido foi a óbito com duas semanas de vida, por insuficiência ventilatória durante arealização de procedimento cirúrgico para correção da malformação cardíaca.Conclusões: Não há descrição na literatura da associação entre síndrome de Taussig-Bing e malformação adenomatoide cística congênita. Odiagnóstico ultrassonográfico precoce e o acompanhamento em unidade de Medicina Fetal são importantes para o planejamento de intervençõespré e pós-natais e aconselhamento familiar por equipe multiprofissional.

Aims: To report a rare case of antenatal diagnosis of unilateral congenital cystic adenomatoid malformation associated with Taussig-Bingsyndrome.Case description: Patient aged 40 years, four pregnancies with three previous vaginal deliveries, black skin, married, housewife, without priorconditions, was referred to the Fetal Medicine Unit of the University Hospital of Santa Maria due to changes in the thoracic region of the fetusfound in routine obstetric ultrasound examination performed at 30 weeks and three days of gestation. At the Doppler ultrasound assessmentperformed in the reference center, perimembranous ventricular septal defect and transposition of the great vessels were found, characterizingthe Taussig-Bing syndrome. Additionally, a unilateral pulmonary congenital cystic adenomatoid malformation type 2 was found. The medicalstaff decided by a cesarean delivery in a referral center for cardiology, with 37 weeks of gestation. The neonate, weighing 3 kg, presentedsince the first hours acute respiratory distress with severe cyanosis, and underwent lobectomy. The neonate died within two weeks of life dueto respiratory failure during the performance of a surgical procedure to correct the cardiac malformation.Conclusions: There is no description in the literature of association between Taussig-Bing syndrome and congenital cystic adenomatoidmalformation. Early ultrasound diagnosis and monitoring in a Fetal Medicine unit is important for planning pre and post-natal interventionsand family counseling by a multiprofessional team.

Intrauterine therapy for macrocystic congenital cystic adenomatoid malformation of the lung

OBJECTIVE: To report on our experiences with thoracoamniotic shunting and/or the injection of a sclerosing agent (OK-432) to treat fetuses diagnosed with macrocystic congenital cystic adenomatoid malformation (CCAM) of the lung. METHODS: A retrospective study was undertaken in six fetuses with macrocystic CCAM at our institute that had been confirmed by postnatal surgery between August 1999 and January 2012. RESULTS: Six fetuses that had been diagnosed with macrocystic CCAM were analyzed. The median gestational age at diagnosis was 23.5 weeks (range, 19.5-31.0 weeks), and at the time of primary treatment was 24.0 weeks (range, 20.5-31.0 weeks). The mean size of the largest cyst at the initial assessment was 42.5+/-15 mm. Four fetuses were associated with mediastinal shifting, and one also showed fetal hydrops. All fetuses underwent a shunting procedure within the cysts, one case among them was also treated with OK-432. After the completion of all procedures, the mean size of the largest cyst was all decreased (14.2+/-12 mm). The median gestational age at delivery was 38.0 weeks (range, 32.4-40.3 weeks). All of the newborns underwent the surgical resection at a median age of 6 days (range, 1-136 days) and are currently doing well without any complications. CONCLUSION: We suggest that intrauterine decompression therapy to manage fetal macrocystic CCAM is recommendable treatment for good perinatal outcome.