A 42-year-old man was diagnosed with cancer of the right lower lung lobe with a posteparterial type of tracheal bronchus, in which the posterior segmental bronchus of the right upper lobe arose from the distal bronchus intermedius. A mass involved the distal bronchus intermedius, requiring a right lower bilobectomy with an additional posterior segmental resection of the right upper lung lobe. Thus, we performed a right lower bilobectomy and sleeve anastomosis of the posterior segmental bronchus of the right upper lobe to the proximal bronchus intermedius, sparing the pulmonary parenchyma of the same lobe.
Adult ; Bronchi* ; Humans ; Lung Neoplasms* ; Lung* ; Respiratory System Abnormalities ; Trachea

OBJECTIVETo study the etiology, pathogenesis, clinicopathologic characteristics, prognosis and treatment of congenital pulmonary airway malformation (CPAM).

METHODSEighteen cases of CPAM were enrolled into the study. The clinical history, autopsy findings and immunohistochemical results were evaluated, with review of literature. The pathogenetic mechanism, pathologic features and differential diagnosis of CPAM were studied.

RESULTSHistologic examination showed that 2 cases were classified as Stocker type I, 12 cases as type II, and 4 cases as type III. The lesion was unilateral and involved single lobe in 13 cases. The remaining 5 cases had bilateral diseases. Of the 18 cases studied, 12 cases showed single organ involvement and 6 cases had malformations affecting multiple organs. The associated malformations included cardiac anomalies (4 cases), polycystic kidney with gastrointestinal atresia (1 case) and nuchal cystic hygroma with hydrothorax (1 case).

CONCLUSIONSCPAM is a rare pulmonary disorder. The etiology of this non-neoplastic condition is unknown. Imaging analysis is a valuable tool to suggest CPAM, while definite diagnosis requires pathologic examination. The overall prognosis is determined by the presence of associated malformations, fetal hydrops and pulmonary hypoplasia.

Congenital pulmonary hypoplasia is a rare developmental abnormality of the lung with an incidence rate of around 1/5000 000. As a fatal condition associated with respiratory insufficiency after birth, this disease is rare in adults. We treated a 29-year-old female patient with congenital pulmonary hypoplasia and concurrent of cystic duct calculus in May, 2011 using single-port transumbilical laparoscopic cholecystectomy, which resulted in a good therapeutic effect comparable to that by routine laparoscopic cholecystectomy.
Abnormalities, Multiple ; Adult ; Cholecystectomy, Laparoscopic ; methods ; Female ; Gallstones ; complications ; surgery ; Humans ; Lung ; abnormalities ; Lung Diseases ; complications

Ehlers-Danlos Syndrome ; pathology ; Humans ; Lung ; abnormalities ; pathology ; Male ; Patella ; abnormalities

Genetic Heterogeneity ; Heart Ventricles ; abnormalities ; Humans ; Lung ; abnormalities ; Male ; Middle Aged ; Ventricular Dysfunction, Left ; genetics

Endocardial Cushion Defects ; complications ; Female ; Humans ; Infant, Newborn ; Lung ; abnormalities ; Lung Diseases ; complications ; congenital

We report a case of bilateral pulmonary agenesis (BPA), which was suspected during a prenatal US examination and diagnosed by fetal magnetic resonance imaging (MRI). BPA is an extremely rare congenital anomaly and, although many fetal structural defects can be detected with a high degree of confidence after introducing high-resolution US, the prenatal diagnosis of BPA remains problematic. Other thoracic abnormalities, such as a congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, and pulmonary sequestration, should be excluded from the list of possible diagnoses before coming to the conclusion of BPA, because BPA is absolutely incompatible with extrauterine life, and an accurate internal diagnosis can prevent a futile intervention from being performed.
Abnormalities, Multiple ; Female ; Humans ; Infant, Newborn ; Lung/*abnormalities/ultrasonography ; *Magnetic Resonance Imaging ; Male ; Pregnancy ; *Prenatal Diagnosis ; *Ultrasonography, Prenatal

Lung agensis is a rare developmental anomaly. It can range from total bronchial and parechymal agensis to mild pulmonary parenchymal hypoplasia of one or both lungs. A case of lobar agenesis of the left upper lung in a 15-year-old boy is presented. The patient had mild exertional dyspnea. Pulmonary angiography revealed the absence of the left upper pulmonary artery and vein. Bronchography showed no branching of bronchus to the left upper lobe. Intravenous pyelography revealed incomplete duplication of the right urinary tract.
Abnormalities, Multiple/*radiography ; Adolescent ; Bronchography ; Cardiac Catheterization ; Dyspnea/etiology ; Heart Murmurs/etiology ; Humans ; Lung/*abnormalities ; Male ; Pulmonary Artery/*abnormalities/radiography ; Pulmonary Veins/*abnormalities ; Tomography, X-Ray Computed ; Urinary Tract/*abnormalities ; Urography

PURPOSE: We reviewed the cases of 33 patients from our clinic and 142 patients from the literature with congenital bronchopulmonary vascular malformations (BPVM), systematically analyzed the bronchopulmonary airways, pulmonary arterial supplies, and pulmonary venous drainages, and classified these patients by pulmonary malinosculation (PM). MATERIALS AND METHODS: From January 1990 to January 2007, a total of 33 patients (17 men or boys and 16 women or girls), aged 1 day to 24 years (median, 2.5 months), with congenital BPVM were included in this study. Profiles of clinical manifestations, chest radiographs, echocardiographs, esophagographs, computer tomography (CT), magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), cardiac catheterizations with angiography, contrast bronchographs, bronchoscopies, chromosomal studies, surgeries, and autopsies of these patients were analyzed to confirm the diagnosis of congenital BPVM. A total of 142 cases from the literature were also reviewed and classified similarly. RESULTS: The malformations of our 33 patients can be classified as type A isolated bronchial PM in 13 patients, type B isolated arterial PM in three, type C isolated venous PM in two, type D mixed bronchoarterial PM in five, type F mixed arteriovenous PM in one, and type G mixed bronchoarteriovenous PM in nine. CONCLUSION: Dysmorphogeneses of the primitive foregut system and the primitive aortic arch system may lead to haphazard malinosculations of the airways, arteries, and veins of the lung. A systematic classification of patients with congenital BPVM is clinically feasible by assessing the three basic bronchovascular systems of the lung independently.
Adolescent ; Adult ; Aorta, Thoracic/*abnormalities ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Infant, Newborn ; Intestines/*abnormalities/*blood supply ; Lung/*abnormalities/*blood supply ; Male ; Vascular Malformations/*classification

BACKGROUND: Congenital cystic adenomatoid malformation (CCAM) is a congenital abnormality of branching morphogenesis of the lung. Thyroid transcription factor-1 (TTF-1) is detected in human respiratory epithelial cells from 11 weeks of gestation, and at full term, TTF-1 expression is confined within type II epithelial cells and in some respiratory nonciliated bronchiolar epithelial cells. Immunoexpression of bcl-2 is intimately related to apoptosis during the development. METHODS: To elucidate the nature of the lesion, TTF-1 expression was evaluated in twenty-four cases of CCAM (eight cases of type 1 and sixteen cases of type 2) along with immunostaining for bcl-2. For the control group, four cases of fetal lungs (19 week-, 21 week-, 27 week- and 40 week-gestational age) were also evaluated. In all cases of CCAM, TTF-1 was detected in the nuclei of epithelial cells lining the cysts. RESULTS: TTF-1 was expressed in the majority of the bronchiolar-like epithelial cells of the cysts in CCAM types 1, and 2, where almost 100% of the lining cells of the cysts were TTF-1 positive with variable intensity, while negative TTF-1 expressions were found in the alveolar-like epithelium of the adjacent alveoli or distal nonciliated bronchi. For bcl-2 immunostaining, no lining epithelial cells of the cysts were stained except for the infiltrating lymphocytes. In the control group, strong immunoreactivities found in early fetal stages were absent in the full-term aged lung (40 gestational weeks). CONCLUSION: These results support the hypothesis that CCAM types 1 and 2 reflect the abnormalities in lung morphogenesis and differentiation that are distinct from those for normally developed alveolar epithelium or adjacent bronchial epithelium, thus retaining the abnormal TTF-1 immunoreactions. Though restricted to CCAM types 1 and 2 in this study, CCAM might be related to TTF-1 rather than apoptosis in the morphogenesis of the developing lung.
Apoptosis ; Bronchi ; Congenital Abnormalities ; Cystic Adenomatoid Malformation of Lung, Congenital* ; Epithelial Cells ; Epithelium ; Humans ; Lung ; Lymphocytes ; Morphogenesis ; Pregnancy ; Thyroid Gland*