No abstract available.
Bronchogenic Cyst ; Bronchopulmonary Sequestration ; Cystic Adenomatoid Malformation of Lung, Congenital ; Lung*

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.
Bronchogenic Cyst ; Bronchopulmonary Sequestration ; Cystic Adenomatoid Malformation of Lung, Congenital ; Emphysema ; Heart ; Humans ; Infant, Newborn* ; Lung ; Perioperative Period

No abstract available.
Bronchopulmonary Sequestration* ; Cystic Adenomatoid Malformation of Lung, Congenital* ; Lung*

Bronchopulmonary sequestration(BPS) and congenital cystic adenomatoid malformation (CCAM) are rare, but both should be included in the differential diagnosis of fetal lung mass. We experienced a mixed form of Stocker type-III CCAM and extralo-bar BPS, and present this case, together with a rev i ew of the related literature.
Bronchopulmonary Sequestration* ; Cystic Adenomatoid Malformation of Lung, Congenital* ; Diagnosis, Differential ; Lung

Congenital cystic disease of the lung represents pulmonary sequestration, congenital lobar emphysema, bronchogenic cyst and congenital cystic adenomatoid malformation. It has a variety of clinical manifestations from immediate postnatal respiratory distress to heart failure. Pulmonary sequestration sometimes causes heart failure in neonates through a shunt between an anomalous systemic feeding artery and the pulmonary venous system. We hereby report a case with both pulmonary sequestration and congenital lobar emphysema presenting with congestive heart failure and pulmonary hypertension, which improved after lobectomy.
Arteries ; Bronchogenic Cyst ; Bronchopulmonary Sequestration ; Cystic Adenomatoid Malformation of Lung, Congenital ; Emphysema ; Estrogens, Conjugated (USP) ; Heart Failure ; Humans ; Hypertension, Pulmonary ; Infant, Newborn ; Lung ; Pulmonary Emphysema

Intralobar pulmonary sequestration is a rare congenital lung anomaly. It is defined as a portion of nonfunctioning lung parenchyma that receives its blood supply from an anomalous systemic artery. Patients often present with chronic or recurrent pneumonia. A chest radiograph may show a cystic lesion with air-fluid levels in the lung base. A high index of suspicion is needed for a diagnosis. Surgical removal of a symptomatic intralobar pulmonary sequestration is generally the treatment of choice. Identifying the aberrant artery is a difficult problem when resecting a pulmonary sequestration. The thoracic and abdominal aortas are the most common origins for the abnormal blood supply. However, arterial supply from the celiac artery is quite rare. We present a case of intralobar pulmonary sequestration with the blood supply originating from the celiac artery.
Aorta, Abdominal ; Arteries ; Bronchopulmonary Sequestration ; Celiac Artery ; Humans ; Lung ; Pneumonia ; Respiratory System Abnormalities ; Thorax

Bronchopulmonary Sequestration ; classification ; pathology ; surgery ; Humans ; Infant ; Lung ; diagnostic imaging ; Male ; Radiography ; Respiratory System Abnormalities ; diagnosis ; surgery

BACKGROUND: Congenital cystic diseases of the lung are uncommon, and they share similar embryogenic and clinical characteristics. But they are sometimes vary widely in their presentation and severity. Therefore they are often difficult to make different diagnosis each other, and all require surgical treatment. MATERIAL AND METHOD: From 1993 to 2006, 38 patients underwent surgical procedures under these diagnostic categories in the Depart. of Thoracic and Cardiovascular Surgery, Busan-Paik Hospital, College of Medicine, Inje University. And we retrospectively reviewed these patients' charts for clinical presentations, surgical procedures, pathologic findings and postoperative morbidity and mortality. RESULT: There were 22 males and 16 females, ages ranged from 1 month after birth to 51 years and mean age was 20.8 years. The main symptoms were 19 fever, cough, sputum production due to recurrent infection, 7 dyspnea, 8 chest discomfort, 4 hemoptysis, but eight patients were asymptomatic. Computed tomography was chosen as diagnostic modalities and available for operation plan for all of patients. For all the cases, surgical resection were performed. Lobectomy was performed in 28 patients, simple excision (resection) in 8 patients, segmentectomy or wedge resection in 2 patients. There were 10 pulmonary sequestrations, 15 congenital cystic adenomatoid malformations (CCAM), 11 bronchogenic cysts, and 2 congenital lobar emphysemas. They all were confirmed by pathologic exams. The complications were 6 wound disruption or infection, 2 chylothorax, 1 ulnar neuropathy, but all of them were resolved uneventful. There was no persistent air leakage, respiratory failure, operative mortality and recurrence. CONCLUSION: We performed immediate surgical removal of congenital cystic lung lesions after diagnosis and obtained good results, so reported them with literature review.
Bronchogenic Cyst ; Bronchopulmonary Sequestration ; Chylothorax ; Cough ; Cystic Adenomatoid Malformation of Lung, Congenital ; Dyspnea ; Emphysema ; Female ; Fever ; Hemoptysis ; Humans ; Lung ; Lung Diseases ; Male ; Mastectomy, Segmental ; Parturition ; Recurrence ; Respiratory Insufficiency ; Retrospective Studies ; Sputum ; Thorax ; Ulnar Neuropathies

PURPOSE: Congenital cystic adenomatoid malformation (CCAM) is a rare variant of congenital pulmonary cystic disease, which is characterized by a proliferation of the dilated bronchiolar-like structure of varying size and/or distribution. In this study, we described the clinical manifestations of CCAM in a single institution during a 12-year period. METHODS: Twenty patients diagnosed with CCAM who were admitted at Busan National University Hospital between January 1995 and December 2007, were investigated for their presenting characteristics, age at diagnosis, locations, histopathologic types, combined anomalies, treatments and outcomes. RESULTS: Twenty patients diagnosed with CCAM were reviewed. Twelve males and eight females, the mean age of the patients was 7.5 years. The main symptoms were coughing, sputum, recurrent infection, chest pain, cyanosis and tachypnea. Seven patients were antenatally diagnosed and two patients were incidentally diagnosed. Radiologic findings were multiple cysts in 15 cases, solitary cyst in 2, consolidation in 3, pleural effusion in 4, tracheal deviation in 2, and abscess in 1 case. The most common location of cystic lesion was the right lower lobe, which includes 9 cases (45%). According to Stocker's histologic classification of CCAM, type I was found in 11 cases (73%), type II in 2 cases and type III in 1 case. In 5 cases, there were combined anomalies including ipsilateral pulmonary sequestration, dextrocardia, pectus excavatum, patent ductus arteriosus, accessory bronchus, and bronchogenic cyst. Fifteen patients underwent lobectomy, two patients are going to have operation and other 3 patients were transferred from regional hospital after diagnosis. All patients who underwent surgical resection improved. CONCLUSION: CCAM is a rare but potentially life-threatening pulmonary anomaly, which can be found at different ages and presents with various clinical findings. The possibility of an underlying CCAM should be considered in children with recurrent pulmonary infections or persistent abnormalities on chest X-rays following an acute infection.
Abscess ; Bone Cysts ; Bronchi ; Bronchogenic Cyst ; Bronchopulmonary Sequestration ; Chest Pain ; Child ; Cough ; Cyanosis ; Cystic Adenomatoid Malformation of Lung, Congenital ; Dextrocardia ; Ductus Arteriosus, Patent ; Female ; Funnel Chest ; Humans ; Lung ; Male ; Pleural Effusion ; Sputum ; Tachypnea ; Thorax

With the current widespread use of obstetric ultrasonography, increasing cases of congenital malformations have been demonstrated antenatally. Both pulmonary sequestration and congenital cystic adenomatoid malformation of the lung (CCAM) are two infrequent congenital pulmonary diseases. The combination of these entities is rare. In the review of literature, this abnormality is so rare that only 80 cases were reported worldwide. To our knowledge, there has been no report that this combined malformation is located transdiaphragmatically. We report the first case of transdiaphragmatic extralobar pulmonary sequestration (ELS) associated with CCAM which was diagnosed by prenatal ultrasonography at 24 weeks gestational age.
Bronchopulmonary Sequestration* ; Cystic Adenomatoid Malformation of Lung, Congenital* ; Gestational Age ; Lung Diseases ; Lung* ; Ultrasonography ; Ultrasonography, Prenatal