A Rare Case of Congenital Pulmonary Airway Malformation Associated with Intralobar Pulmonary Sequestration in an Adult - Case Report (original) (raw)
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A rare disease; congenital pulmonary airway malformation in an adult
Tuberkuloz ve Toraks
A rare disease; congenital pulmonary airway malformation in an adult Congenital pulmonary airway malformation (CPAM), is a rare disease known as a developmental abnormality of the lower respiratory tract. It may occur in the neonatal period due to respiratory distress and patients usually die in the first few months of life. Rarely, it may remain asymptomatic until adulthood. In this study we describe an adult case and present a review of the literature. A 19-year-old male with no relevant medical history was admitted to our clinic with cough and wheeze. The patient had a history of frequent lower respiratory tract infection during childhood. Chest radiograph revealed a diffuse opacity and volume loss in the right hemithorax. High resolution tomography showed hypoplasia of the right hemithorax, multiple cysts in all of the lobes and segments on the right side, ground glass opacity and interlobular septal thickening of the whole right lung parenchyma. Right pneumonectomy was performed with the pre-diagnosis of congenital pulmonary airway malformation and the pathological examination was compatible with CPAM. CPAM is a rare disease in adulthood. We should consider CPAM in the differential diagnosis of patients with frequent recurrent pulmonary infection and cystic lung lesions. In order to prevent infections and to eliminate the risk of malignancy, surgical treatment should be applied for definite diagnosis and treatment.
2016
Congenital lesions of lung are rare, with overall incidence of 1/10000 to 1/25000 births (2.2 % as compared to acquired lesions). CPAM and BPS are the commoner lesions. Hybrid lesion (coexistent CPAM and BPS) are extremely rare. Diagnosis can be made antenatally in-utero or postnatally due to complications of the lesion, such as recurrent pneumonias, lung abscesses and pneumothorax. We report a case of a 4 year old male child presenting with recurrent pneumonia. Chest radiograph showed dense consolidation in the left lower zone and multiple small air filled cystic spaces in the left mid and lower zones, few showing air-fluid levels. HRCT showed dense consolidation in the left lower lobe with air-bronchogram and small air-filled cavities. Contrast study showed two arterial feeders arising from descending thoracic aorta with venous drainage into left inferior lobe pulmonary vein. A diagnosis of lung sequestration with associated CCAM was made.
Congenital Pulmonary Airway Malformation in an Adult Male: A Case Report with Literature Review
Case Reports in Pulmonology, 2015
Congenital pulmonary airway malformation (CPAM) is a rare cystic lung lesion formed as a result of anomalous development of airways in fetal life. Majority of the cases are recognized in neonates and infants with respiratory distress with very few presenting later in adult life. A 24-year-old male with history of three separate episodes of pneumonia in the last 6 months presented with left sided pleuritic chest pain for 4 days. He was tachycardic and tachypneic at presentation. White blood count was 14 × 109/L. Chest X-ray showed left lower lobe opacity. CT angiogram of thorax showed a well-defined area of low attenuation in the left lower lobe with dedicated pulmonary arterial and venous drainage and resolving infection, suggesting CPAM. He underwent left lower lobe lobectomy. Histopathology confirmed type 2 CPAM. CPAM is a rare congenital anatomic abnormality that can present with recurrent infections in adults. As a number of cases remain asymptomatic and symptomatic cases are of...
Congenital pulmonary airway malformation
Bratislava Medical Journal, 2013
Background: Congenital cystic adenomatoid malformations (CCAMs) are considered rare developmental anomalies of the lower respiratory tract. These are hamartomatous abnormalities of the lung with adenomatoid proliferation of cysts resembling bronchioles and usually occur sporadically and unilaterally with single lobe involvement. Method: A 6-year-old girl was admitted to our center because of prolonged fever and non-productive cough lasting3 months before admission. Results: The only other complaint was night sweating. She did not have dyspnea and did not mention any respiratory symptoms. On examination, coarse crackle and decreased lung sounds in the left side were detected. White blood cell count was 9.100 /μL, hemoglobin was 11.2 g/dL, erythrocyte sedimentation rate was 50 and Creactive protein was 1+. IgA and IgM for hydatid cyst were tested and both were raised (14 and 1.4, respectively). Conclusion: The patient underwent surgery, with the probable diagnosis of hydatid cyst but in operating room diagnosis was changed and it was adenomatoid cystic malformation. In follow-up, she was in good general condition without any post-surgical complaints (Fig. 3, Ref. 11).
The rare congenital anomaly of pulmonary sequestration experience and review of literature
Indian Journal of Thoracic and Cardiovascular Surgery, 2010
Objective Pulmonary sequestration is rare lung anomaly which had very major theories of origin. The aim of this study was to treat this rare congenital anomaly, pulmonary sequestration, which was not diagnosed of preoperatively, in our centre. Methods A retrospective analysis of 10 cases of pulmonary sequestration operated at the centre between 2000 and 2010. A review of the extralobar and intralobar types of sequestration is discussed. All patients were not diagnosed preoperatively. Results Pulmonary sequestration is found more commonly in women (6 patients). In all patients, the diagnosis was made by exploratory thoracotomy. Six patients had intralobar (one was apical) and 4 were extralobar (one apical). Lobectomy for intralobar and sequestrectomy for extralobar sequestration were performed. Conclusions Although rare, some radiological features are sufficiently suggestive of diagnosis of pulmonary sequestration. We believe that pulmonary sequestration should be considered when intrathoracic masses cannot be differentiated through invasive and non-invasive evaluation. In difficult cases methods such as aortagraphy, doppler angiography and Magnetic Resonance (MR) angiography may be used.
Congenital Lung Malformations, Update and Treatment
2009
SUMMARY There is a broad spectrum of bronchopulmonary malformations which present in early infancy and childhood. These include Bronchogenic cysts, Bronchopulmonary sequestrations, congenital cystic adenomatoid malformation (CCAM), and congenital lobar emphysema. These lesions maybe detected by prenatal diagnosis, present as acute respiratory distress in the newborn period, or may remain undiagnosed and asymptomatic until late in life. Pulmonary sequestration (PS) is a rare congenital malformation of the lower respiratory tract. It consists of a non-functioning mass of lung tissue that lacks normal communication with the tracheobronchial tree and receives its’ arterial blood supply from the systemic circulation. CCAMs are extremely rare with the reported incidence being between 1/25,000 and 1/35,000 . The pathogenesis is uncertain but appears to result from a abnormality of the branching morphogenesis of the lung and represents a maturation defect. Congenital Lobar Emphysema (CLE) i...
Congenital Pulmonary Airway Malformation: A Case Report and Review of the Literature
Respiratory Care, 2011
Congenital parenchymal lung malformations have an estimated incidence at 1:25,000-1:35,000 births. We present a case of this rare congenital abnormality in a 38 year-old male, review the current literature with discussion of proposed causes, malignant potential, and management strategies. A 38-year-old white male presented with a 4-day history of chronic stable hemoptysis. Social history was notable for a 50-pack-year active smoking history and remote heavy alcohol consumption. Physical examination was normal. Chest radiograph revealed an ill defined right lower lobe infiltrate. Chest computed tomography demonstrated an irregular, thin-walled, cystic lesion with adjacent nodularity and calcifications. The patient received a right lower lobectomy. Pathologic specimen demonstrated a 10-cm, mostly thin-walled cyst with features suggestive of a congenital cyst adenomatoid malformation and areas of adenocarcinoma (mixed subtype with acinar and bronchioloalveolar patterns). Congenital cyst adenomatoid malformations have recently been renamed as congenital pulmonary airway malformations and are the most common type of congenital parenchymal lung malformations. Individuals typically present with recurrent pulmonary infections, pneumothorax, or hemoptysis. The development is controversial but believed to be a result of arrested development of the fetal bronchial tree during the sixth and seventh week of fetal development. Defects in thyroid transcription factor 1 have also been proposed. With the increasing use and image resolution of ultrasound in modern obstetric practice, congenital pulmonary airway malformations rarely go undetected into adulthood. Management remains controversial; however, most authors agree with early surgical excision.
Congenital Pulmonary Airway Malformation – Case Presentation
Revista română de pediatrie, 2017
Congenital pulmonary airway malformation (CPAM) is a rare cystic lesion, whose prognosis depends on the extent of the lesion, the association of other congenital abnormalities, the associated pulmonary pathology and the possibility of curative surgery. We present the case of a 5-month-old infant diagnosed with bilateral CPAM, based on clinical and imaging investigations, with acute pulmonary manifestations and without other associated congenital anomalies.
The management of asymptomatic congenital lung malformations
Paediatric Respiratory Reviews, 2004
Congenital Pulmonary Airway Malformation (CPAM) and lung sequestration are often discovered on routine prenatal sonography. While some lesions may be large and cause serious complications in the fetus or newborn, many will be asymptomatic at birth. Some authors advocate simple observation because of the lack of data on the incidence of long-term complications. However, there are very few described cases where CPAM and intralobar sequestration (ILS) have remained asymptomatic throughout life; complications eventually develop in virtually all patients. The most common complication is pneumonia, which may respond poorly to medical treatment. Other complications include the development of malignancies (carcinomas and pleuropulmonary blastomas), pneumothorax and hemoptysis or hemothorax.
Medical Bulletin of Haseki, 2019
Congenital bronchopulmonary malformations are uncommon anomalies of infants and children presenting as congenital lobar emphysema, bronchogenic cyst, congenital cystic adenomatoid malformation (CCAM), or pulmonary sequestration (PS). CCAM is the second most common congenital lung malformation in newborns. There are five types of CCAM based on the embryologic and the histologic features. PS is defined as a mass of tissue developing in the lung but having no respiratory function. PS is classified into two types: extralobar and intralobar. Coexistence .of CCAM and PS is a rare occurrence. Hybrid lesions, especially type 2 CCAM and PS combination may have a more severe clinical progress. Herein, we report a male infant with type 2 CCAM in combination with PS who died despite both medical and surgical treatment.