Haemopneumothorax from congenital cystic adenomatoid malformation in a cryptorchidism patient.

Congenital cystic adenomatoid malformation (CCAM) of the lung is an uncommon congenital anomaly, especially in young adults. This study reports an 18-yr-old male with CCAM involving the right upper lobe, who presented with a moderate spontaneous haemopneumothorax initially. The patient also had bilateral abdominal cryptorchidism which required surgical treatment earlier in childhood. The chest radiographs and contrast-enhanced computed tomographic scan of the chest showed a multicystic lesion with air-fluid levels in the right upper lung. The right upper lobe was resected through a posterolateral thoracotomy. Histological examination confirmed the diagnosis of CCAM. To the authors' knowledge, congenital cystic adenomatoid malformation presenting with spontaneous haemopneumothorax and haemoptysis has never been described in the literature.     

Congenital lung disease: a plea for clear thinking and clear nomenclature

Antenatal ultrasound has allowed the discovery of abnormalities which in the past often escaped detection. It may be unclear what advice to offer. Many large lung malformations seen at the routine 20-weeks gestation scan have largely disappeared at term. There is now even more confusion in terms of how these malformations should be described. The nomenclature of congenital lung disease was never very clear, with terms such as sequestrated segment, cystic adenomatoid malformation, hypoplastic lung, and malinosculation being used to describe often overlapping abnormalities. However, today these terms are used inconsistently in the ante- and postnatal periods. For example, congenital cystic adenomatoid malformation (CCAM) is used prenatally to describe a lesion which may well disappear before birth, but is used postnatally to describe an abnormality which may require lobectomy. CCAM may have a pulmonary arterial supply, or be supplied like a sequestration from the aorta, and histological features of both lesions may coexist. Thus, a complete reappraisal of the nomenclature of congenital lung disease is timely; this review does not discuss treatment options.     

Intralobar pulmonary sequestration (a nonentity?)

Intralobar pulmonary sequestration has generally been considered a congenital malformation in which an accessory lung bud develops, is enveloped by normal lung, and retains its systemic arterial supply. Also usually included in the "sequestration spectrum" are aberrant arteries to normal lung with either normal or anomalous venous connection. We reviewed all surgical pathology specimens and autopsies performed at Texas Children's Hospital from 1955 through 1984. There were 15 cases with an aberrant systemic artery to normal or abnormal lung. Nine were cases of structurally normal lung with an aberrant systemic artery, and five were cystic adenomatoid malformations with a systemic artery. In one older child some features of intralobar sequestration were present, but a major bronchial connection was retained. We propose that most intralobar sequestrations represent either cystic adenomatoid malformations that clinically are unrecognized until they become secondarily infected or developmentally normal lung supplied by a systemic artery.     

Histopathologic Findings of Pneumatocele in a Patient with Hyper-IgE syndrome, compatible with cystic adenomatoid malformation

Hyper-immunoglobulin E syndrome is a rare primary immunodeficiency disease characterized by recurrent abscess formation, respiratory tract infections and very high titers of serum IgE associated with peculiar face and skeletal features. We report a seven-year old girl presenting with persistent productive cough and history of chronic eczematoid facial lesions since infancy and two episodes of hospitalizations due to pneumonia and perianal abscess. Additionally, in physical examination finger tip clubbing, laxity of joints and crackles in both lungs were detected. Immunologic work up revealed markedly raised IgE level and eosinophilia. The patient was diagnosed as hyper IgE syndrome based on his clinical and laboratory findings. Chest X-ray revealed multiple large cystic lesions in left lung which were confirmed by spiral CT-scan. Pneumonectomy specimen examination showed cystic adenomatoid malformation, characterized by the presence of various cysts lined by epithelium in different sizes. There are few reports of cystic adenomatoid malformation in children. To our best known, this is the first report of cystic adenomatoid malformation in a child with hyper IgE syndrome. Early diagnosis and surgical therapy are helpful in prevention of repeated infections in these patients.     

Late presentation of congenital cystic adenomatoid malformation of lung in ten-year-old girl

Congenital cystic adenomatoid malformation of the lung (CCAML) is uncommon, and usually presents in children with respiratory distress. We describe a girl presenting with pneumonia at age 10 years. She had been previously healthy and active, and with no previous hospitalizations or X-rays. A chest CT scan showed right lower lobe cystic lesions suggestive of CCAML.     

Pulmonary cystic adenomatoid malformation in an adult patient: an underdiagnosed disease

Currently, most congenital lower respiratory tract malformations are detected during pregnancy or at birth, thanks to antenatal imaging. However, a pulmonary congenital cystic adenomatoid disease may be found in adulthood. The diagnosis is difficult, due to its rarity. We present the case of a patient whose diagnosis of pulmonary cystic adenomatoid malformation was confirmed when she had tuberculosis. A lobectomy was performed, which enabled identification of tuberculosis and also multiple cysts of adenomatoid malformation. The risk posed by this malformation, i.e. the risk of developing bronchioloalveolar carcinoma and of infection or pneumothorax, is the incentive for proposing formal surgical removal.     

In-flight spontaneous pneumothorax: congenital cystic adenomatoid malformation of the lung

Congenital cystic adenomatoid malformation (CCAM) is a rare congenital abnormality. Symptomatic presentation in adult life is extremely uncommon. The usual radiological appearance of CCAM is a cystic space-occupying lesion. Patients with underlying cystic lung disease can develop in-flight complications because of pressure-volume changes during ascent. We report the first ever case in which spontaneous pneumothorax during flight was the presenting manifestation of CCAM of the lung in a previously healthy and asymptomatic young adult. We also discuss the physiological changes during air travel which contribute to the pathogenesis of respiratory complications during air travel.     

Spontaneous pneumothorax in cystic adenomatoid malformation. Unusual clinical and histologic features

Pneumothorax is a rare presentation of congenital cystic adenomatoid malformation (CCAM) in the newborn period and is presumed to be due to resuscitative measures. A previously well three-week-old baby presented with spontaneous tension pneumothorax due to CCAM. In the lung resection specimen, a malformation was seen, which in addition to the histologic changes of CCAM, showed diffuse vascular proliferation in the interstitium and lining of air space by type 2 pneumocytes. We propose that this is a new variant of CCAM rather than one of the classic three types. The unusual clinical manifestation may be related to the unusual histologic features.     

Diffuse chondroid malformation of the lung: Cases series of a hitherto undescribed congenital lung disease

Congenital chondroid lesions of the lung are rare pathological findings. They are a constant feature of lung malformations such as giant cystic pulmonary chondroid hamartoma, chondroid cystic malformation, and in the “cartilaginous variant” of congenital adenomatoid malformation. All of these present as a large single thoracic mass. We present the cases of three males and two females with hitherto undescribed diffuse chondroid lung disease, all but one of whom had neonatal respiratory distress syndrome with interstitial syndrome on chest radiograph. The pathological findings were similar in all patients, showing large areas of disorganized lung parenchyma containing diffusely distributed mature cartilage islands. With a mean follow‐up of 6 years, all patients had a favorable outcome. This diffuse chondroid lung disease appears to be a new entity whose initial presentation mimicked interstitial lung disease without the usual clinical, radiological, and histological features. We speculate that it could be part of a clinical spectrum between malformative chondroid lung cyst and congenital pulmonary airway malformation. Pediatr Pulmonol. 2010; 45:667–673. © 2010 Wiley‐Liss, Inc.     

成人先天性肺囊性病变影像学分析

目的:探讨成人先天性肺囊性病变的影像学特点以及与肺癌的关系。方法:对23例经外科手术切除病理证实的成人先天性肺囊性病变,进行影像学分析,并系统复习文献。结果:支气管源性肺囊肿12例(52.2%);先天性囊性腺瘤样畸形5例(21.7%),其中1例伴发黏液性原位腺癌(LASLC/ATS/ERS 2011年版肺癌新分类),1例伴发鳞癌;肺隔离症6例(26.1%)。结论:成人先天性肺囊性病变最常见类型,是支气管源性肺囊肿。先天性囊性腺瘤样畸形少见,诊断过程中如怀疑先天性囊性腺瘤样畸形,应警惕发生肺癌的危险。     

Lung volume reduction surgery in lieu of pneumonectomy in an infant with severe unilateral pulmonary interstitial emphysema

A male infant with a prenatal diagnosis (at 20 weeks' gestation) of cystic adenomatoid malformation was delivered after 38 weeks' gestation (birth weight, 3 kg) and admitted to the neonatal intensive care unit. During the first few days of life, he developed mild respiratory distress; a chest radiograph and computed tomography scan showed multiple cystic areas in the left lower lobe with hyperinflation and herniation of the upper lobe across the midline. At 3 weeks of age, a left lower lobectomy was performed for presumed cystic malformation. To our surprise the pathology reports revealed pulmonary interstitial emphysema. The postoperative chest radiograph was unchanged, and mechanical ventilation was necessary and required progressively increasing ventilatory settings to provide adequate support. High-frequency oscillatory ventilation and selective right bronchus intubation failed to improve lung function. After 3 weeks, a left thoracotomy was repeated and lung volume reduction was performed with removal of 50' of the peripheral hyperinflated parenchyma. Postoperative recovery was rapid; the child was weaned from the ventilator after 3 days and discharged after 3 weeks. Follow-up chest X-rays showed a normally expanded right lung with mediastinal structures back to midline and a small left lung. Favorable results persisted at 3 years of follow-up. This first and successful experience with lung volume reduction in a neonate suggests that infants who need removal of a large portion of lung parenchyma to achieve adequate ventilation and gas exchange, lung volume reduction surgery should be considered as an alternative to pneumonectomy.     

Multiple bilateral pulmonary nodules revealing a congenital cystic adenomatoid malformation

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare congenital developmental abnormality, representing about 25% of all congenital lung lesions. It is very rare that presentation is delayed until adulthood. We report a case of 63-year-old woman without notable pathological antecedents in whom a systematic chest X-ray revealed multiple bilateral pulmonary nodules. The patient was asymptomatic and her physical examination was normal. CT scan showed bilateral liquid rounded thin-walled densities of various size, with a homogeneous non calcified content. The diagnosis was based on radiological findings and surgery. The result of histopathological examination obtained by thoracic surgery confirmed CCAM without malignancy. The postoperative follow up showed an excellent recovery.     

Congenital cystic adenomatoid malformation of the lung resembling bronchiectasis in an adult

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare anomaly which is characterized by a proliferation of dilated bronchiolar-like air spaces. It is generally seen in newborns and infants. When seen in adults, which is more uncommon, it presents itself mostly with recurrent pulmonary infections. In this article, 31-year-old man with cough and purulent expectoration and a history of recurrent pulmonary infections who had cystic changes resembling bronchiectasis at the left lower lobe on the computed tomography of the chest and diagnosed CCAM-type 2 after the histopathological examination of the left lower lobectomy specimen is presented.     

Congenital cystic adenomatoid malformation type 4.

A 9-day-old boy presented in respiratory distress and with failure to thrive. The chest X-ray showed a hyperlucent area of the left lung. A resection of the markedly emphysematous segment 2 of the left upper lobe was performed assuming the emphysematous tissue was due to congenital lobar emphysema (CLE). Histological examination of the lung tissue, however, revealed a pattern consistent with congenital cystic adenomatoid malformation (CCAM) type 4. The therapy for CLE as well as for CCAM is similar, i.e., resection of the emphysematous tissue. As far as the prognosis is concerned, it is important to diagnose the exact type of malformation in order to exclude associated anomalies, as well as the risk of development of malignancies in later life. The frequency of associated malformations of CCAM type 4 is unknown. Although the risk for development of malignancies from CCAM type 4 is not clear at the moment, the possible development of malignancies justifies prompt resection shortly after diagnosis, even in asymptomatic patients. A life-long follow-up in those patients who had a resection of CCAM in early childhood is recommended.     

Congenital cystic adenomatoid malformation complicated by esophageal duplication cyst in a 6-month-old girl

We report a case of congenital cystic adenomatoid malformation (CCAM) complicated by an esophageal duplication cyst in a 6-month-old girl. The patient presented with recurrent pneumonia. Magnetic resonance imaging revealed two cystic lesions in the upper and lower lobes of the right lung. After cystectomy, histopathological investigation revealed that the lower cyst was a CCAM Type I, and the upper cyst was an esophageal duplication cyst. The coexistence of these complex anomalies supports the concept that the esophageal duplication cyst is one entity of a broad spectrum of developmental abnormalities caused by abnormal budding of the primitive foregut.     

Pulmonary resection in infants for congenital pulmonary malformation

STUDY OBJECTIVES: To review our experience with indications, timing, and results of pulmonary resection in infants. DESIGN: Retrospective cohort study. SETTING: Thoracic Surgery Department, Chest Diseases Hospital, Kuwait. Patients and intervention: Forty-seven infants with congenital lung diseases were treated with pulmonary resection from January 1993 to December 2000. RESULTS: The mean age at the time of diagnosis was 90 days (range, 7 days to 11 months). Thirty-four patients were male (72%). Congenital lobar emphysema, congenital cystic adenomatoid malformation, pulmonary sequestration, and atelectasis were seen in 26, 10, 6, and 5 patients, respectively. The indications for surgery were respiratory distress in 32 patients (68%), respiratory tract infections in 12 patients (26%), and the presence of asymptomatic chest radiographic findings in 3 patients (6%). A lobectomy was performed in 42 patients (89%), bilobectomy in 2 patients (4%), left pneumonectomy in 1 patient (2%), and excision of a mass in 2 patients with extralobar sequestration (4%). An emergency lobectomy was performed in seven patients (15%). Only one postoperative death occurred following a left pneumonectomy for extensive congenital adenomatoid malformation due to pulmonary hypertension. Four patients (9%) had postoperative complications: atelectasis (n = 2), prolonged air leak (n = 1), and pneumothorax (n = 1). Mean follow-up was 4 years (range, 1 to 5 years) for all patients. None of the patients had any physical limitations. CONCLUSION: Pulmonary resection is indicated for the majority of patients with congenital lung malformations. In case of severe respiratory distress, an emergency lobectomy can be performed safely.     

Congenital cystic adenomatoid malformation in an adolescent: an unusual presentation with pleural effusion and pneumatocele

Congenital cystic adenomatoid malformation (CCAM) encompasses a continuum of hamartomatous cystic lung lesions characterised by the presence of abnormal bronchiolar structures of varying sizes or distribution. The CCAM is a disorder of infancy with majority of the cases being diagnosed within the first two years of life. We describe CCAM in a 13-year-old girl complaining of recurrent lower respiratory tract infections since infancy who presented with post-infectious pneumatocele with loculated pleural effusion, and suspected abscess formation and had undergone resection.     

Congenital cystic adenomatoid malformation of the lung. Report of 3 cases with late presentation

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare congenital developmental abnormality, representing about 25% of all congenital lung lesions. In many cases, respiratory distress occurs during the neonatal period, and in about 80- 85% of patients, CCAM is diagnosed before the age of two years due to respiratory infection. It is very rare that presentation is delayed until adulthood. We report three cases of CCAM presenting in adults. The diagnosis was based on clinical and radiological findings in one case and two patients were not diagnosed until surgery. The lesion was present in the right lung in two and in left lung in one patient. All patients underwent surgical resection. The result of histopathological examination confirmed CCAM Stocker type 1, without malignancy. The post operative follow up showed an excellent recovery. Clinicians and pathologists need to be aware of the fact that CCAM can be present for the first time in adolescents or in adults. The clinical diagnosis is suggested by radiographic findings and is confirmed at pathology as surgery is generally indicated.     

Congenital masses of the lung: prenatal and postnatal imaging evaluation.

Congenital masses of the lung are a spectrum of interrelated abnormalities that includes congenital lobar overinflation, bronchogenic cyst, congenital cystic adenomatoid malformation (CCAM) and sequestration. The prenatal and postnatal imaging features of these lesions are reviewed, emphasizing the importance of serial prenatal sonograms and postnatal imaging studies, including radiography and computed tomography. Masses that become inconspicuous, or disappear on serial prenatal sonograms are discussed, as well as the importance of postnatal imaging studies in the evaluation of these lesions. Finally, the management of congenital masses of the lung is reviewed, emphasizing the importance of imaging studies in the preoperative evaluation.     

Video-assisted thoracoscopic surgery in 3 cases of adult cystic adenomatoid malformation

Congenital cystic adenomatoid malformation involving the lung is a rare hamartomatous condition that is usually diagnosed in the neonatal period. The presentation of this malformation in older patients is exceptional and usually manifests in a series of recurrent lung infections affecting a single lobe or segment. The treatment of choice is complete surgical exeresis. This report of 3 cases of late presentation focuses on the surgical approach used and the unusual manifestation of recurrent spontaneous pneumothoraces in 1 patient. The patients were females aged 15, 16, and 25 years with histories of various respiratory diseases (extrinsic asthma, recurrent pneumonias, and pneumothoraces). The patients were referred to us for surgery with suspected diagnoses that were different from the final diagnoses in all cases. All underwent diagnostic video-assisted thoracoscopy to explore the affected hemothorax, and definitive treatment was possible during the procedure for 2 patients (a lobectomy and an atypical segmentectomy) by video-assisted surgery. The third patient underwent lobectomy by lateral thoracotomy after exploratory video-assisted thoracoscopy. Short- and long-term outcomes were excellent for all 3 patients.