There are many methods for achieving one-lung ventilation (OLV) during thoracic surgery in neonates and the accuracy of OLV may affect postoperative outcome. The authors have performed OLV using a 5 Fr Arndt endobronchial blocker (AEB, Cook Inc., Bloomington, IN, USA) on a neonate diagnosed with congenital cystic adenomatoid malformation and respiratory distress syndrome (RDS) associated with marked mediastinal shift. In spite of sufficient preoxygenation, sudden and severe fall in oxygen saturation had occurred. Since neonates with RDS may develop sudden and severe desaturation, rapid intubation with anticipation of potential difficulty is necessary as well as sufficient preoxygenation.
Anoxia ; Cystic Adenomatoid Malformation of Lung, Congenital ; Humans ; Infant, Newborn ; Intubation ; One-Lung Ventilation ; Oxygen ; Thoracic Surgery

Minimally invasive thoracic surgery has been one of the most important surgical advances recently. Congenital cystic adenomatoid malformation of the lung is a relatively rare anomaly and is clearly associated with various congenital anomalies such as pectus excavatum, cardiac and pulmonary vascular lesions. We have experienced a case that was treated with minimal invasive methods for congenital cystic adenomatoid malformation involving in the right lower lobe and pectus excavatum in a 5-year-old boy. We simultaneously performed thoracoscopic right lower lobectomy and Nuss procedure of pectus excavatum using a substernal steel bar. Therefore, a minimally invasive surgical treatment for this diseases is feasible and cosmetically excellent.
Child, Preschool ; Cystic Adenomatoid Malformation of Lung, Congenital* ; Funnel Chest* ; Humans ; Lung ; Male ; Steel ; Surgical Procedures, Minimally Invasive ; Thoracic Surgery ; Thoracoscopy

BACKGROUND: Congenital cystic adenomatoid malformation of the lung (CCAM) is a rare congenital developmental anomaly of the lower respiratory tract. Most cases are diagnosed within the first 2 years of life, so adult presentation of CCAM is rare. We describe here six adult cases of CCAM and the patients underwent surgical resection, and all these patients were seen during a five and a half year period. The purpose of this study was to analyze the clinical, radiological and histological characteristics of adult patients with CCAM. METHODS: Through medical records analysis, we retrospectively reviewed the clinical characteristics, the chest pictures (X-ray and CT) and the histological characteristics. RESULTS: Four patients were women and the mean age at diagnosis was 23.5 years (range: 18~39 years). The major clinical presentations were lower respiratory tract infection, hemoptysis and pneumothorax. According to the chest CT scan, 5 patients had multiseptated cystic lesions with air fluid levels and one patient had multiple cavitary lesions with air fluid levels, and these lesions were surrounded by poorly defined opacities at the right upper lobe. All the patients were treated with surgical resection. 5 patients underwent open lobectomy and one patient underwent VATS lobectomy. On the pathological examination, 3 were found to be CCAM type I and 3 patients were CCAM type II, according to Stocker's classification. There was no associated malignancy on the histological studies of the surgical specimens. CONCLUSION: As CCAM can cause various respiratory complications and malignant changes, and the risks associated with surgery are extremely low, those patients who are suspected of having or who are diagnosed with CCAM should go through surgical treatment for making the correct diagnosis and administering appropriate treatment.
Adult ; Cystic Adenomatoid Malformation of Lung, Congenital ; Female ; Hemoptysis ; Humans ; Lung ; Medical Records ; Pneumothorax ; Respiratory System ; Respiratory Tract Infections ; Retrospective Studies ; Thoracic Surgery, Video-Assisted ; Thorax

Cystic Adenomatoid Malformation of Lung, Congenital ; diagnostic imaging ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Infant, Newborn ; Lung ; diagnostic imaging ; pathology ; Male ; Pneumonectomy ; Tomography, X-Ray Computed

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.
Adult ; Cystic Adenomatoid Malformation of Lung, Congenital/*diagnosis/*surgery/therapy ; Female ; Humans ; Male ; Physical Examination ; Pleural Effusion ; Pneumonia/complications/*radiography ; Retrospective Studies ; Tomography, X-Ray Computed ; Treatment Outcome

We present a case of congenital cystic adenomatoid malformation (CCAM) in a 25-year-old male who was presented with chronic cough. Chest radiography revealed an abnormal mass-like shadow in the right lower pulmonary zone. A contrast enhanced computed tomography showed an 11 cm solid, cystic mixed mass on the right lower lobe. A right lower lobectomy was performed by video-assisted thoracoscopic surgery without complications. The gross specimen showed a massive cavitation with multiloculated cysts of varying size, consistent with CCAM, along with noticeable granulomatous inflammation. Non-tuberculosis mycobacteria were isolated from a bronchial wash specimen, and the resected tissue homogenates were positive for Mycobacterium avium-intracellulare complex by polymerase chain reaction.
Cough ; Cystic Adenomatoid Malformation of Lung, Congenital ; Humans ; Inflammation ; Male ; Mycobacterium ; Mycobacterium avium Complex ; Nontuberculous Mycobacteria ; Polymerase Chain Reaction ; Thoracic Surgery, Video-Assisted ; Thorax

The development and evolution of fetal surgery and the recognition of the fetus as a patient came from two sources. First, were those obstetricians and perinatologists who detected life threatening anomalies before birth, and re-described a hidden mortality arising from death in utero. Ultrasonography, color Doppler ultrasound and ultrafast fetal magnetic resonance imaging have since enhanced the accuracy of prenatal evaluation. Second, were those pediatricians responsible for treating newborn infants with extremely serious problems, and that appeared untreatable, although, it was believed that they could have been treated at an earlier stage of development. After the natural history of several correctable lesions had been determined and the selection criteria for intervention developed, fetal surgery emerged as a means of improving the overall morbidity and mortality rates.
Cystic Adenomatoid Malformation of Lung, Congenital/surgery ; Fetal Diseases/*surgery ; Fetus/*surgery ; Hernia, Diaphragmatic/congenital/surgery ; Human ; Postoperative Care ; Preoperative Care ; Sacrococcygeal Region ; Spinal Neoplasms/embryology/surgery ; Teratoma/embryology/surgery

PURPOSE: Congenital pulmonary airway malformation (CPAM)—a rare developmental anomaly—affects the lower respiratory tract in newborns. By comparing the reliability of diagnostic tools and identifying predictive factors for symptoms, we provide comprehensive clinical data for the proper management of CPAM. METHODS: We reviewed the medical records of 66 patients with prenatally diagnosed CPAM delivered at Severance Children's Hospital between January 2005 and July 2017. RESULTS: We enrolled 33 boys and 33 girls. Their mean gestational age and birth weight were 38.8 weeks and 3,050 g, respectively. Prenatal ultrasonography and postnatal radiography, lung ultrasonography, and chest computed tomography (CT) showed inconsistent findings. Chest CT showed superior sensitivity (100%) and positive predictive value (90%). Among the 66 patients, 59 had postnatally confirmed CPAM, three had pulmonary sequestration, one had cystic teratoma, and one had a normal lung. Of the 59 patients with CPAM, 21 (35%; mean age, 23.4 months) underwent surgery, including 15 who underwent video-assisted thoracoscopy. Twenty-five and 12 patients exhibited respiratory symptoms at birth and during infancy, respectively. Apgar scores and mediastinal shift on radiography were significantly associated with respiratory symptoms at birth. However, none of the factors could predict respiratory symptoms during infancy. CONCLUSION: Radiography or ultrasonography combined with chest CT can confirm an unclear or inconsistent lesion. Apgar scores and mediastinal shift on radiography can predict respiratory symptoms at birth. However, symptoms during infancy are not associated with prenatal and postnatal factors. Chest CT combined with periodic symptom monitoring is important for diagnosing and managing patients with prenatally diagnosed CPAM and to guide appropriate timing of surgery.
Birth Weight ; Bronchopulmonary Sequestration ; Cystic Adenomatoid Malformation of Lung, Congenital ; Female ; Gestational Age ; Humans ; Infant, Newborn ; Lung ; Medical Records ; Parturition ; Radiography ; Respiratory System ; Teratoma ; Thoracic Surgery, Video-Assisted ; Thoracoscopy ; Thorax ; Tomography, X-Ray Computed ; Ultrasonography ; Ultrasonography, Prenatal

Adolescent ; Biopsy ; Cystic Adenomatoid Malformation of Lung, Congenital ; diagnosis ; diagnostic imaging ; therapy ; Diagnosis, Differential ; Diagnostic Errors ; adverse effects ; prevention & control ; Humans ; Infant ; Lung Diseases ; diagnosis ; diagnostic imaging ; therapy ; Male ; Middle Lobe Syndrome ; diagnosis ; diagnostic imaging ; surgery ; Tomography, X-Ray Computed ; Treatment Outcome