• Korean Journal of Radiology
• /
• v.22 no.10
• /
• pp.1690-1696
• /
• 2021

Objective: To describe the anatomic locations and imaging features of posterior lung herniation in unilateral pulmonary agenesis and aplasia, focusing on radiograph-CT/MRI correlation. Materials and Methods: A total of 10 patients (seven with pulmonary agenesis and three with pulmonary aplasia, male: female = 1:9, mean age 7.3 years, age range from 1 month to 20 years) were included. Chest radiographs (n = 9), CT (n = 9), and MRI (n = 1) were reviewed to assess the type of lung underdevelopment, presence of anterior and posterior lung herniation, bronchus origin, supplying artery, and draining vein of the herniated lung. Results: Pulmonary agenesis/aplasia more commonly affected the left lung (n = 7) than the right lung (n = 3). Anterior lung herniation was observed in nine of the 10 patients. Posterior lung herniation was observed in seven patients with left pulmonary agenesis/aplasia. Two patients showed posterior lung herniation crossing the midline but not beyond the aorta, and five patients showed the posteriorly herniated right lower lobe crossing the midline to extend into the left hemithorax farther beyond the descending thoracic aorta through the space between the esophagus and the aorta. This anatomical configuration resulted in a characteristic radiographic finding of a radiolucent area with a convex lateral border and a vertical medial border in the left lower lung zone, revealing a tongue-like projection on CT and MRI. Conclusion: Posterior lung herniation occurs in unilateral left lung agenesis/aplasia. Approximately 70% of the cases of posterior lung herniation reveal a unique radiolucent tongue-like projection in the left lower lung zone on imaging studies, which is caused by the extension of the posteriorly herniated right lung farther beyond the descending aorta.

• Journal of Chest Surgery
• /
• v.49 no.1
• /
• pp.59-62
• /
• 2016

A 12-month-old boy was diagnosed with agenesis of the right lung. Mediastinal deviation progressed to the diseased side as the patient matured; therefore, tracheal distortion developed. As a result, tracheal compression developed between the vertebral body and aorta. The patient was repeatedly admitted to the hospital because of recurrent pulmonary infection and combined severe respiratory distress. Diaphragm translocation was performed to treat the patient. The postoperative course was favorable, and computed tomography scan findings and symptoms had improved at 1 year after surgery.

• Journal of Chest Surgery
• /
• v.24 no.1
• /
• pp.83-87
• /
• 1991

A unilateral pulmonary artery agenesis, which develops occasionally as one of the associated anomalies in congenital cardiovascular defect like as tetralogy of Fallot, is very rare anomaly as an isolated congenital defect. The diagnostic approach for the pulmonary artery agenesis is first suggested by the unique appearance of the involved lung on a routinely checked chest roentgenogram, because most patients are asymptomatic unless pulmonary infection had been superimposed. We have recently experienced a case of left pulmonary artery agenesis, which was diagnosed by perfusion scan, digital subtraction angiogram and then treated by left pneumonectomy in a 9 year-old boy, and presented hereby with the review of relevant literature.

• Journal of Chest Surgery
• /
• v.24 no.12
• /
• pp.1238-1241
• /
• 1991

Agenesis of the hemidiaphragm is unusual congenital anomaly associated with a high mortality. A case of congenital agenesis of left diaphragm was experienced in 22-day old male patient who was dyspneic and cyanotic on admission. Emergency exploration through the left eight interspace thoracotomy showed complete agenesis of the left diaphragm. The stomach and transverse colon covered with peritoneal sac was partially herniated into left hemithorax. The left lung was slightly hypoplastic. This neonate had no intestinal malrotation. The defect was reconstructed using Dacron graft. Dacron patch was sutured with interrupted Ethibond to chest wall anteriorly, esophagus aorta and costomediastinal sinus medially, and the tenth rib posterolaterally. Postoperatively, Extubation was performed at 1st day, but some respiratory difficulty was noted. Severe dyspnea was occurred at postoperative 11th day and so reintubation was done. Intermittently ventilatory support and intravenous alimentation were continued for 9 days after that. Thereafter he had no respiratory problems at discharge.

• Tuberculosis and Respiratory Diseases
• /
• v.52 no.3
• /
• pp.288-293
• /
• 2002

A 32-year-old woman presented with cough and hemoptysis. The radiologic findings showed increased interstitial markings in the right lung, a slightly decreased lung volume in the RLL and a hypoplastic right pulmonary artery with collaterals in the mediastinum and subpleural area. The pulmonary angiography showed an abrupt occlusion of the right lower pulmonary artery. The echocardiographic findings indicated pulmonary hypertension. A doppler leg ultrasonograph disclosed that the left popliteal vein was occluded with collateral veins, not filling the defect in the venous lumen. The D-dimer increased 1.0 ug/ml. This condition was initially misdiagnosed as a congenital pulmonary artery agenesis. Finally, a chronic pulmonary thromboembolism with a deep vein thrombosis was confirmed.

• Journal of Chest Surgery
• /
• v.40 no.1 s.270
• /
• pp.60-62
• /
• 2007

Pulmonary hypoplasia is an entity of pulmonary agenesis. Pulmonary agenesis is a rare congenital anomaly, usually diagnosed soon after birth. It is commonly associated with other anomalies, mainly of the cardiovascular systems. Although it may hasten the death of a child, sometimes it is compatible with normal growth. We report a right lateral thoracotomy approach for mitral valve repair in a young woman with pulmonary hypoplasia, in whom preoperative computed tomography showed severe right side shifting of the mediastinum and total collapse of the right lung.

• Journal of Chest Surgery
• /
• v.8 no.1
• /
• pp.13-18
• /
• 1975

Developmental pulmonary abnormalities are known as rare condition. diagnosis was made at autopsy in the early cases reported, however, as diagnostic aids such as X-ray, bronchography, bronchoscopy and exploratory thoracotomy have come into use, the condition is being discovered more often recently in living persons, and it appears to occur with sufficient frequency to merit consideration in the differential diagnosis of certain chest conditions. According to Schneider and Boyden there are three main types of this abnormality: [1] Agenesis, in which there is complete absence of one or both lungs; there is no trace of bronchial or vascular supply or of parenchymal tissue. [2] Aplasia, in which there is suppression of all but a rudimentary bronchus which ends in a blind pouch; there are no vessels or parenchyma. [3] Hypoplasia, in which the bronchus is fully formed but is reduced in size and ends in a _ flesh structure which usually lies within the mediastinum. Rudimentary pulmonary parenchyma may be present around the bronchial stump and often is the site of cystic malformation. We experienced one case of hypoplastic lung with cystic malformation which was originated from a small aberrant rudimentary bronchus, and the rudimentary bronchus was branched from the right side of tracheal end. The diagnosis was finally confirmed by the histopathological finding. Now, we report this case with a brief review of literatures.

• Journal of Chest Surgery
• /
• v.15 no.1
• /
• pp.21-26
• /
• 1982

Fourteen cases of funnel deformity, 11 were male and the others female, treated over a eleven-year period, are presented. The overall results with the methods described by the authors, Wada, Shannon, Adkins and Ravitch appear to be excellent. The symmetric depression was more common In children under the age of 12 years and asymmetric one Increased after the age of 12. Six cases of abnormal cardiac auscultatory findings revealed no consistent hemodynamic abnormalities. An 21-year-old female was associated with left lung agenesis and dextroversion of the heart, and a 6-year-old boy with congenital bronchogenic cyst who underwent right upper lobectomy. EKG changes associated with the anomaly were observed in all. Pulmonary function test showed the range from normal to moderate restriction which did not Improve in only one patient after operation, but the patient did not complain any restriction In activity. Some transient complications were developed which resulted in improvement.

• Journal of Chest Surgery
• /
• v.7 no.2
• /
• pp.153-162
• /
• 1974

Four patients with funnel chest deformity corrected in the Department of Thoracic Surgery, Seoul National University Hospital are presented. The first case was a 21-year old female with cyanosis, clubbed fingers and systolic murmur on the left infrascapular region on physical examination associated with agenesis of the right lung. The deformity was of asymmetrical funnel chest, in which the left hemithorax was more sunken. She was corrected by the method of Funnel Costoplasty of Wada. The second case was a three years old boy whose anterior chest wall was symmetrically deformed, and he was corrected by the method of Ravitch using Adkins strut under the sternum. The third was a 22-year old man with symmetrical deformity, and was corrected by the method described by Shannon in 1973. The last patient was a 22-year old man and he had dyspnea on exertion, palpitation and apical systolic murmur with symmetrical funnel chest deformity. He was also corrected by Ravitch operation, All of them has excellent result.

• Tuberculosis and Respiratory Diseases
• /
• v.65 no.5
• /
• pp.430-434
• /
• 2008

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital anomaly that occurs in association with other cardiovascular anomalies, such as tetralogy of Fallot or ventricular septal defects. On the other hand, it is less commonly found as an isolated finding without accompanying diseases. Isolated UAPA is a rare cause of hemoptysis, and massive hemoptysis has been reported to occur in approximately 18~20% of UAPA patients during their clinical course. Even if a lung resection is considered a treatment option to control life-threatening hemoptysis, the procedure is more difficult than an ordinary lung resection because of the excessive collateral vessels from the systemic circulation. We encountered an isolated UAPA occurring in a young male patient suffering from intermittent blood tinged sputum. To our knowledge, only a few cases of isolated UAPA have been reported in Korea. This case is expected to be a good example to help clinicians better understand isolated UAPA as an unusual cause of hemoptysis.