A 7-year-old well and healthy boy was hospitalized for the first time for severe respiratory distress. He was diagnosed with congenital cystic adenomatoid malformation (CCAM) and bronchopulmonary sequestration (BPS). CCAM and BPS are rare congenital pulmonary diseases and their presentation concurrently as a hybrid lesion is even rarer. CCAM is usually diagnosed prenatally however it may regress postnatally and the patient may be asymptomatic. BPS usually presents after birth or later in childhood with recurrent lung infections. We highlight this case of hybrid lesion of CCAM and BPS with an atypical clinical presentation which was lifesaving surgically with diagnosis made via radiographic imaging.
Bronchopulmonary Sequestration

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

Communicating bronchopulmonary foregut malformation (CBPFM) is a communication between the respiratory and gastrointestinal tracts that can be difficult to differentiate from pulmonary sequestration or H-type tracheoesophageal fistula (TEF) because of the similarities in clinical features. A female neonate born at full term had been experiencing respiratory difficulty during feeding from the third day of life. The esophagography performed to rule out H-type TEF revealed that the esophageal bronchus directly communicated with the left lower lobe (LLL) of the lung. Lobectomy of the LLL, fistulectomy of the esophagobronchial fistula, and primary repair of the esophagus were performed. Finally, CBPFM type III with pulmonary sequestration was confirmed on the basis of the postoperative histopathological finding. We report the first newborn case of CBPFM type III with pulmonary sequestration in Korea.
Bronchi ; Bronchial Fistula ; Bronchopulmonary Sequestration ; Esophagus ; Female ; Fistula ; Gastrointestinal Tract ; Humans ; Infant, Newborn ; Korea ; Lung ; Tracheoesophageal Fistula

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.
Body Weight ; Bronchopulmonary Sequestration ; Cystic Adenomatoid Malformation of Lung, Congenital ; Enteral Nutrition ; Follow-Up Studies ; Gestational Age ; Humans ; Infant ; Length of Stay ; Lung ; Medical Records ; Operative Time ; Parturition ; Pathology ; Pneumonia ; Retrospective Studies ; Surgical Procedures, Minimally Invasive* ; Thoracoscopy ; Uncertainty

Elevated carbohydrate antigen (CA) 19-9 can indicate malignancies of the gastrointestinal, pancreatic, and biliary tracts, and be found in a pulmonary sequestration. A 30-year-old man visited Seoul National University Bundang Hospital due to elevated CA 19-9 levels, representing pulmonary sequestration of the bilateral lower lobes, which were connected with each other. We performed left lower lobectomy and division of the systemic arteries. After operation, CA 19-9 levels decreased to normal range, even though a small amount of sequestrated lung remained in the right lower lobe. It is not uncommon that presence of pulmonary sequestration might elevate serum CA 19-9 levels; however, horseshoe type bilateral pulmonary sequestration is very rare.
Adult ; Arteries ; Biliary Tract ; Bronchopulmonary Sequestration* ; Humans ; Lung ; Reference Values ; Seoul

We presented a rare case of a 64-year-old man with a combined anomaly of the bronchus and pulmonary artery that was detected incidentally. Computed tomography showed a hyperlucent, aerated sequestered segment of the right lower lung with an independent ectopic bronchus, which had no connection to the other airway. The affected segment was supplied by its own aberrant pulmonary artery branch from the right pulmonary trunk. This anomaly cannot be classified with any of the previously reported anomalies.
Bronchi/pathology ; Bronchopulmonary Sequestration/*radiography ; Humans ; Lung/*radiography ; Male ; Middle Aged ; Pulmonary Artery/*radiography ; Tomography, X-Ray Computed

Systemic arterial supply from the descending thoracic aorta to the basal segment of the left lower lobe without a pulmonary arterial supply is a rare congenital anomaly within the spectrum of sequestration lung disease. The most common pattern of anomalous systemic artery to the lung arises from the descending thoracic aorta and feeds the basal segments of the left lower lobe. We report an extremely rare case of a 29-year-old woman who underwent a successful left upper lobectomy for the treatment of recurrent massive hemoptysis from anomalous bronchial arterial supply to the lingular segment of left upper lobe.
Adult ; Aorta, Thoracic ; Arteries ; Bronchial Arteries* ; Bronchopulmonary Sequestration ; Female ; Hemoptysis* ; Humans ; Lung ; Lung Diseases

OBJECTIVETo investigate clinical and pathological features of lung lesions in children.

METHODSClinical manifestations, radiologic imaging, histopathological features and immunohistochemical results were analyzed in 215 cases of lung lesions in children.

RESULTSA total of 215 cases of lung lesions in children aged 0 day to 13 years (average age of 27.2 months and the median age of 18.0 months) were selected, including 137 male and 78 female patients with a male to female ratio of 1.76:1.00. The incidence of congenital lung disease was higher in patients of less than 1 year old than those of over 1 year old age, and the difference of the two groups was statistically significant (P = 0.004). 142 cases had acquired lung diseases, and 73 cases had congenital bronchopulmonary dysplasia. Lung abscess was the most common lesion seen in 86 cases (40.0%), including 1 case of fungal abscess. Congenital pulmonary airway malformation (CPAM) was the second most common, seen in 44 patients (20.5%), including 20 cases of type 1, 18 cases of type 2 and 6 cases of type 4 CPAM. Pulmonary sequestration was found in 25 cases (11.6%) including 14 cases of intralobar type and 11 cases of extralobar type. Two cases of extralobar pulmonary sequestration showed simultaneous CPAM2 type 2 lesion. Other lesions included tuberculosis (13 cases, 6.0%), emphysema (12 cases, 5.6%), interstitial pneumonia (7 cases, 3.2%), pulmonary hemorrhage (6 cases, 2.8%), bronchogenic cyst (4 cases, 1.9%), bronchiolitis obliterans (2 cases, 0.9%), idiopathic pulmonary hemosiderin deposition disease (2 cases, 0.9%) and 1 cases of lung non-specific changes. 13 cases of neoplastic lesions (6.0%) were found, of which 11 cases were primary tumors (5.1%), including inflammatory myofibroblastic tumor in 5 patients (2.3%), pleuropulmonary blastoma in 5 cases (1 case of type I, 2 type II and 2 type III) and 1 case of mucoepidermoid carcinoma (0.5%) and 2 cases of metastatic tumors (hepatoblastoma and Wilm's tumor, 0.9%).

CONCLUSIONSInfectious diseases are the most common lung diseases in children. Congenital bronchopulmonary dysplasia is the most common in children of less than 1 year old. Malignant lesions are rare.

Abscess ; pathology ; Adolescent ; Bronchopulmonary Sequestration ; pathology ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Infant, Newborn ; Lung ; pathology ; Lung Diseases ; pathology ; Male ; Pulmonary Blastoma ; pathology

We describe a rare case of extralobar pulmonary sequestration with hemorrhagic infarction in a 10-year-old boy who presented with acute abdominal pain and fever. In our case, internal branching linear architecture, lack of enhancement in the peripheral portion of the lesion with internal hemorrhage, and vascular pedicle were well visualized on preoperative magnetic resonance imaging that led to successful preoperative diagnosis of extralobar pulmonary sequestration with hemorrhagic infarction probably due to torsion.
Abdominal Pain/etiology ; Bronchopulmonary Sequestration/*diagnosis/surgery ; Child ; Fever/etiology ; Humans ; Magnetic Resonance Imaging/*methods ; Male ; Pulmonary Infarction/*diagnosis/surgery ; Torsion Abnormality/complications

OBJECTIVE: This study reviewed clinical characteristics of fetal intra-abdominal umbilical vein (FIUV) varices that were detected during antenatal ultrasound examinations. METHODS: Between January 2006 and January 2012, 121 cases of FIUV varices were detected and 7 cases were lost to follow-up. We retrospectively reviewed the medical records of 114 patients and neonates. RESULTS: From a total 96,553 ultrasound examinations in 43,995 pregnancies, 121 cases of FIUV varices were identified (2.8 per 1,000 pregnancies). Gestational age at diagnosis was 32.0 +/- 2.9 weeks (range, 20.1-36.3 weeks), the mean diameter of the FIUV varix was 12.6 +/- 2.1 mm (range, 8.0-21.0 mm) at initial diagnosis and the mean maximal diameter was 13.1 +/- 2.3 mm (range, 8.0-21.0 mm) during follow-up. The most severe pregnancy complications included one case of intrauterine fetal death and another case of fetal hydrops. Associated fetal anomalies (n = 11, 9.6%) detected by ultrasonography included bilateral renal pelvis dilatation, ventriculomegaly, cryptorchidism, incomplete renal duplication and pulmonary sequestration. A total of 104 cases (91.2%) were delivered at term and 10 cases (8.8%) were preterm deliveries before 37 weeks of gestation. CONCLUSION: FIUV varices that are not associated with fetal anomalies based on ultrasound examination during prenatal care have favorable pregnancy outcomes. Nevertheless, close fetal monitoring is recommended to decrease perinatal complications.
Bronchopulmonary Sequestration ; Cryptorchidism ; Diagnosis ; Dilatation ; Female ; Fetal Death ; Fetal Monitoring ; Follow-Up Studies ; Gestational Age ; Humans ; Hydrops Fetalis ; Infant, Newborn ; Kidney Pelvis ; Lost to Follow-Up ; Male ; Medical Records ; Pregnancy ; Pregnancy Complications ; Pregnancy Outcome ; Prenatal Care ; Retrospective Studies ; Ultrasonography ; Umbilical Veins* ; Varicose Veins*