Malformación congénita de la vía aérea pulmonar. Reporte de un caso / Congenital malformation of the pulmonary airway. Report of a case

RESUMEN El objetivo de este estudio es realizar una revisión de la Malformación Congénita Pulmonar a propósito del caso de un feto de una paciente de 34 años que cursaba su segunda gesta, cuyo diagnóstico se realizó a la semana 30 de gestación. Además se realizó una revisión bibliográfica de la patología y de casos similares.

ABSTRACT The objective of this study is to perform a review of Congenital Pulmonary Malformation regarding a case of a fetus in a 34-year-old woman who was in her second pregnancy, whose diagnosis was made at week 30 of gestation. In addition, a bibliographic review of the pathology and similar cases was carried out

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.

Comparison of accuracy of prenatal ultrasonography and MRI in the diagnosis of congenital cystic aden-matiod malformation of the lung / 实用医学杂志

Objective To compare prenatal ultrasonography and MRI in view of the accuracy in the diag-nosis of congenital cystic adenmatiod malformation(CAMM)of the lung. Methods From January 2014 to Octo-ber 2015,68 fetus who were examined with both prenatal ultrasonography and MRI and diagnosed as CAMM by pathological findings after operation or autopsy in our study. Taking the final pathological diagnosis of fetal CCAM genotyping as the gold standard,the accuracy of prenatal ultrasonography and MRI were compared in the diagnosis of CAMM of the lung.P<0.05 was considered as statistically significant. Results The alignment degree of prena-tal ultrasound in the diagnosis of CAMM was significantly larger than that of MRI examination to the pathological di-agnosis.Conclusion Prenatal ultrasound is an important method for diagnosing CCAM before pregnancy with high accuracy and affordability.

A Case of Congenital Cystic Adenomatoid Malformation in a 10 Years Old Boy.

Congenital Cystic Adenomatoid Malformation (CCAM) is a relatively rare congenital pulmonary cystic disease. By ultrasonography prenatal diagnosis can be made and most baby born without complication. Some babies need surgery at the first year of life for recurrent respiratory tract infection. Here a case of CCAM in a 10 year old boy is reported who presented with recurrent respiratory tract infection and underwent surgical resection successfully.

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 de presentación tardía: revisión del tema y presentación de un caso / Review and case report: late-onset congenital cystic adenomatoid malformation of the lung

The congenital cystic adenomatoid malformation (CCAM) of the lungs is a rare congenital abnormality that it is usually discovered during childhood. Late-onset CCAM is extremely uncommon, usually detected as a radiographic finding in adult patients. Timely diagnosis of this condition is important mainly because of the risk of recurrent infections and mainly forits neoplastic potential. The case ofan adult patient is presented.

La malformación adenomatoídea quística pulmonar es una extraña anomalía congénita que frecuentemente se detecta durante la infancia. La presentación tardía es poco frecuente y más comúnmente se detecta como un hallazgo radiológico en adultos. El diagnóstico de esta patología es importante, principalmente por el riesgo de infecciones recurrentes y transformación neoplásica.

A Case of Congenital Cystic Adenomatoid Malformation of the Lung in the Patient with Recurrent Pneumonia (Mixed type I and II) / 소아알레르기및호흡기학회지

Congenital cystic adenomatoid malformation (CCAM) of the lung is an uncommon embryonic developmental anomaly, characterized by the presence of one or multiple interconnecting cysts. This disease may present various clinical aspects, stillborn or perinatal death, respiratory distress in newborns, and acute and chronic pulmonary infections in older infant and children. We experienced a case of CCAM in a 3-years, 8-month-old male who had frequent upper respiratory tract infection and recurrent pneumonia. Chest X-ray and chest CT showed a cavitary lesion in the right middle lobe. Histologic examination revealed numerous small and large sized cysts in the parenchyma. The large cysts were mainly lined by pseudostratified ciliated cells and the small cysts are lined by simple cuboidal cells and subclassified Stocker type mixed I and II. Wedge resection was done with satisfactory postoperative courses clinically and radiologically. We report a case of CCAM (I and II mixed type) with a brief review.

A Case of Prenatally Diagnosed Congenital Cystic Adenomatoid Malformation of the Lung / 대한산부인과학회잡지

Congenital cystic adenomatoid malformation of the lung (CCAM) is a rare benign pulmonary lesion characterized by abnormal overgrowth of the fetal bronchiole. The prenatal diagnosis of CCAM is important because the prognosis of the CCAM is depend on the type, bilaterality, and other associated abnormalities, which can be detected by prenatal ultrasonography. We experienced a case of CCAM that was detected by prenatal ultrasonography and report the case with a review of several related literatures.

A Case of Congenital Cystic Adenomatoid Malformation of the Lung Containing Fungal ball-like movable Blood clots in Healthy Young Woman / 결핵

We describe unusual manifestations of congenital cystic adenomatoid malformation ( C.C.A.M.) of the lung, such as movable fungal ball-like intracystic blood clots and hemoptysis, which were detected in previously healthy 23 years-old woman. We identified blood clots only after left upper lobectomy and could not distinguish from fungal ball with radiographic methods. CCAM of the lung, rare and lethal form of congenital pulmonary cystic disease, was initially introduced by Ch'in and Tang in 1949. The histogenesis of this lesion is characterized by polypoid glandular tissue proliferation and overgrowth of mesenchymal elements due to cessation of bronchiolar maturation which occured in after 16weeks intrauterine period. In 80-95% of reported cases, the lesion was confined to a single lobe and there was no lobe and right and left lung predilection. The clinical presentation may be widely variable, ranging from intrauterine fetal death to late discovery in childhood with recurrent pulmonary infection. But there's no reports which were misdiagnosed with intracystic fungal ball. The treatment choice is lobectomy of affected lobe. There's a few case reports with rhabdomyosarcoma, bronchiolar cell carcinoma and myxosarcoma arising in CCAM patients. Therefore, early resection is recommended even if asymtomatic cases. We experienced a rare case of CCAM of the lung in 23 years old female, and there were intracystic fungal ball-like movable blood clots in lower portion of left lung. After left upper lobectomy was performed, now she is discharged and followed up without any complications.

A Case of Late Presentation of Congenital Cystic Adenomatoid Malformation of the Lung / 결핵

Congenital cystic adenomatoid malformation of the Lung(CCAM) is characterized by anomalous fetal development of terminal respiratory structures, resulting in an adenomatoid proliferation of bronchiolar elements and cystic formation. CCAM was first described and differentiated from other cystic lung disease in the English literature by Ch'in and Tang in 1949. CCAM is a rare, potentially lethal form of congenital pulmonary cystic disease and the salient features of lesion are an irregular network of terminal respiratory bronchiole-like structures and macrocysts variably lined by pseudostratified ciliated columnar epithelium and simple cuboidal epithelium. Adult presentation of CCAM of the lung is so tare that only 9 cases have been reported in the literature of date. The pathogenesis of CCAM remains disputed and reseachers have variously proposed that the lesion represents a developmental anomaly, hamartoma, or a form of pulmonary dysplasia. Van Dijk and Wagenvoort divided CCAM into three subtypes: cystic, intermediated, and solid. These correspond to types I, II, and III of Stocker. In adults, the evaluation of cystic or multicystic lung disease requires consideration of a differential diagnosis including the acquired lesions of lung abscess, cavitary neoplasm or inflammatory mass, bullous disease, bronchiectasis, and postinflammatory pneumatocele. Congenital lesions such as sequestration, bronchopulmonary-foregut anomalies, and bronchogenic cyst are also encounted. The definitive treatment for CCAM is complete removal of the involved lobe. Partial lobectomy leads to multiple complications, including severe post-operative infection. We report a case of CCAM in a 14-year-old female presentated with a pneumothorax and large bullae, who was treated by surgical remove of the involved lobe.

Anesthetic Management of A Neonate with Congenital Cystic Adenomatoid Malformation(C.C.A.M) / 대한마취과학회지

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare pulmonary malformation, associated with local aberrations of parenchymal tissue development, which have been classified lung bud anomalies. Lung bud anomalies include infantile lobar emphysema, congenital cyst of the lung, congenital cystic adenomatoid malformation, pulmonary sequestration and bronchogenic cyst. CCAM can enlarge rapidly by ball-valve air entrapment by cysts, lead to mediastinal shift and compression of the heart. compression of opposite lung may result in pulmonary hypoplasia and CCAM causes acute respiratory distress in newborn. We report a neonate with CCAM who were successfuly managed during perioperative period.