ABSTRACT
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.
ABSTRACT
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.
Subject(s)
Humans , Male , Adolescent , Cystic Adenomatoid Malformation of Lung, Congenital , Cystic Adenomatoid Malformation of Lung, Congenital/classification , Cystic Adenomatoid Malformation of Lung, Congenital/pathology , Prognosis , Radiography, Thoracic , Tomography, X-Ray ComputedABSTRACT
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.
Subject(s)
Child , Female , Humans , Infant , Infant, Newborn , Male , Pregnancy , Cystic Adenomatoid Malformation of Lung, Congenital , Embryonic Development , Lung , Pneumonia , Respiratory Tract Infections , Thorax , Tomography, X-Ray ComputedABSTRACT
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.
Subject(s)
Bronchioles , Cystic Adenomatoid Malformation of Lung, Congenital , Lung , Prenatal Diagnosis , Prognosis , Ultrasonography, PrenatalABSTRACT
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.