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Eur J Cardiothorac Surg 2005;28:483-489
© 2005 Elsevier Science NL

Review

Adult congenital lung disease

Ganesh Shanmugam *

Department of Cardiac Surgery, Royal Hospital for Sick Children, Dalnair Street, Glasgow G3 8SJ, UK

Received 2 April 2005; received in revised form 8 May 2005; accepted 9 May 2005.

* Tel.: +44 141 201 0264. (Email: sgunpat{at}hotmail.com).


   Abstract
Top
 Abstract
1. Introduction
 2. Patterns of presentation
 3. Conclusion
 References
 
Congenital lung anomalies are increasingly diagnosed in adults, because they are missed in childhood, or as a consequence of incomplete resections at a younger age. They have unique manifestations, often mimic other thoracic pathology, and can present acutely and necessitate emergent evaluation and management. Misdiagnosis is common. This article deals with the patterns of presentation, clinical and radiological diagnosis, and the management of these disorders in adults. The lesions analysed include congenital cystic adenomatoid malformation, sequestration, congenital lobar emphysema and bronchogenic cysts. It is important to include these malformations in the differential diagnosis of adult thoracic pathology and to understand the modes of presentation, potential complications and management strategies. The radiological diagnosis is reliably made by computed tomography and/or magnetic resonance imaging. The potential for malignancy in these lesions is an important consideration. The management of asymptomatic lesions was considered controversial, but more lesions are now resected early, in recognition of the potential for subsequent complications. Complete resection is recommended. In contradistinction to childhood malformations, these lesions are more amenable to thoracoscopic resections, provided they are completely benign. Congenital lung malformations call for well-organized cooperation between paediatric and adult pulmonologists to ensure a smooth medical transition from childhood to adulthood.

Key Words: Congenital cystic adenomatoid malformation • Bronchogenic cyst • Sequestration • Congenital lobar emphysema • Video-assisted thoracoscopic surgery


   1. Introduction
Top
 Abstract
 1. Introduction
2. Patterns of presentation
 3. Conclusion
 References
 
The spectrum of congenital broncho-pulmonary malformations includes congenital cystic adenomatoid malformation [CCAM], sequestration, congenital lobar emphysema and bronchogenic cysts (BC). We have previously described the manifestations of these anomalies [1] during childhood. Although the current emphasis is on the intrauterine management of these lesions, an increasing number are being recognized in adults, reflecting improvements in diagnosis. The recognition of these lesions in adults is important from several perspectives:

1. The adult manifestations are unique and the diagnosis is frequently missed.
2. Congenital lesions may mimic neoplasms in adults.
3. They may manifest as respiratory or circulatory distress and warrant emergent management.

These lesions are sufficiently frequent, have unique manifestations in adults, should be considered in the differential diagnosis of adult lung lesions and merit the term ‘ADULT CONGENITAL LUNG DISEASE’.


   2. Patterns of presentation
Top
 Abstract
 1. Introduction
 2. Patterns of presentation
3. Conclusion
 References
 
Adult physicians may be confronted with congenital lesions, in one of 3 ways—children with congenital lesions that are not diagnosed, that are managed expectantly or are partially resected, and who consequently carry their malformations into adulthood.

2.1 CCAM
Most adult CCAM lesions, have Stocker [2] type 1 features on histopathology, presumably because the severe forms are either stillborn or are operated during childhood.

2.1.1 Diffuse CCAM
CCAM is usually unilobar and involves the posterobasal segments of the lower lobes, but bilateral and multilobar involvement [3,4] have been reported. Bilateral multicystic CCAM, can present with features suggesting, interstitial lung disease [5].

2.1.2 Radiology
Computerized Tomography (CT) features consist of complex cystic masses, pneumonitic changes [6] or multiple cavities with fluid levels [7].

2.1.3 Adult manifestations of CCAM
CCAM is an uncommon cause of adult lung pathology, but should be considered in the presence of recurrent lung infections. Several reports describe patients with long standing infections refractory to antibiotics or patients with known radiological abnormalities who are managed medically. The treatment of CCAM is essentially surgical. Long-term antibiotics may prevent recurrent infections. However, prolonging antibiotic therapy delays and complicates eventual surgery and leads to antibiotic resistance.

2.1.4 Consequences of partial resection
Infants who undergo partial resection of CCAM present in adulthood with residual disease or even worse—malignant transformation in the residual lung [8,9]. Unstable epithelium in unresected malformations may be more susceptible to oncogenesis. Sudou et al. [10] reported a bronchio-alveolar carcinoma in a VATS lobectomy for adult CCAM. This demonstrates the importance of complete resection and of closely following children with incomplete resections.

2.1.5 Pleural problems
Haemopneumothorax [11], pyopneumothorax [12] and recurrent pneumothorax [13] have been described in association with CCAM. Bleeding occurs due to vessel tears in the lesion, leading to haemoptysis and haemothorax. Al-Ghitmi and associates [14] described in a CCAM specimen, the presence of bronchiectasis and multiple neuroendocrine nests, which additionally contributed to the haemoptysis.

2.1.6 Malignancy
Thoracic surgeons have to keep the combination of CCAM and malignancy in mind. Lobectomy is the operative procedure, so as not to leave behind malignant tissue existing among the cystic lesions. Mechanisms of carcinogenesis in CCAM include increased cell proliferation, decreased apoptosis [15], and malignant transformation of the glandular component [16]. The unstable mesenchymal cells in CCAM can give rise to blastoma and sarcoma [17]. Premalignant changes [18] and bronchioloalveolar carcinoma [19] have been reported in association with CCAM.

2.2 Congenital lobar emphysema
Critchley et al. [20] reported perhaps the only case of adult congenital lobar emphysema in a pregnant woman. A left upper lobectomy was performed during pregnancy with good results.

2.3 Sequestration
15% of sequestrations remain asymptomatic [21,22]. Sequestrations are unrecognized causes of recurrent pneumonia and haemoptysis. Many patients are repeatedly treated with antibiotics, with diagnostic delays varying between 3 months and 7 years [23]. Misdiagnosis has been attributed to a lack of recognition of radiologic findings [24].

2.3.1 Radiology
Intralobar sequestrations (ILS) manifest as areas of increased opacity, as masses with or without air-fluid levels, or as cysts in the posterobasal segment of the lower lobes. Other findings include pneumonitis and focal bronchiectasis [25]. Extralobar sequestrations (ELS) manifest as well-defined masses at the base of the left hemithorax. CT features include well-defined cysts or abnormal masses with air fluid levels. CT shows the anomalous systemic artery in about 2/3rds of the patients [26]. Helical CT [27] and 3-dimensional CT angiography [28] facilitate the display of the aberrant artery.

2.3.2 MRI and MRA
Breath-hold contrast-enhanced MRA displays the aberrant artery without flow or respiratory artefact. Multiphase scanning after gadolinium administration allows demonstration of arteries and veins separately and improved visualization of the venous drainage.

Two dimensional MRI has limitations: (1) the necessity of breath holding to maximize flow-related enhancement and to eliminate respiratory artefact, (2) the need to correctly orient the plane of acquisition, and (3) flow-dependent signal that may fail to detect small vessels with low flow or inadequately delineate large vessels with turbulent flow. Three-dimensional contrast-enhanced MRI has been used to characterize sequestration [25]. The radiographic images are consistent with surgical findings in most reported cases. Contrast-enhanced 3-dimensional MRA does not require orientation of the plane of acquisition. Imaging is not flow-dependent, thus allowing improved visualization of smaller vessels.

2.3.3 Presentations
2.3.3.1 Haemorrhage
Bleeding can complicate sequestrations in one of 3 forms—intracystic haemorrhage, haemoptysis and haemothorax. Bleeding occurs since the pressure in the sequestral artery is higher than the surrounding pulmonary pressure, the thin abnormal arteries lacking the usual musculature of systemic vessels [29], and aneurysmal changes in the aberrant vessels [30].

2.3.3.2 Intracystic haemorrhage
Intracytic haemorrhage is infrequent but potentially fatal. Dallalana et al. [31] reported profound haemorrhage into an ILS, 2 days following cardiac surgery. Heparinization for bypass and the initiation of oral anticoagulation for the aortic prosthesis were attributed as contributing factors.

2.3.3.3 Haemoptysis
Intracytic haemorrhage is associated with haemoptysis when an airway communication exists. Exsanguinating haemoptyis has been reported [32,33] with both ILS and ELS [34]. Dewan et al. [35] reported the combination of ILS, bronchiectasis and carcinoid tumourlets in a patient with haemoptysis. The bronchiectasis and the carcinoids could have been additional sources for haemoptysis.

2.3.3.4 Haemothorax
Spontaneous haemothorax has been reported in association with ILS [36].

2.3.3.5 Sequestrations mimicking other thoracic pathology
Avishai et al. [37] and Petersen et al. [24] reported cases of sequestrations presenting as a massive haemothorax. The initial diagnoses in these cases were aortic dissection and saccular thoracic aneurysm with dissection into the lung parenchyma. MRA showed a mass in the left lower lobe with a feeding vessel off the descending aorta. At thoracotomy, the left lower lobe was adherent to the descending aorta, a 6-cm portion of which was resected en bloc and replaced with a homograft.

2.3.3.6 Management of acute bleeds
CT shows the sequestration. Angiogram reveals the aberrant artery, its site of origin and contrast leak from the vessel. Catheter embolization of the bleeding vessel with multiple coils or pellets is an important option [38]. This should be followed by vessel ligation and resection of the sequestration. Maull et al. [39] reported a patient with symptoms suggesting empyema, who was found at thoracotomy to have infarcted ELS due to twisting of the vascular pedicle.

2.3.3.7 Coronary origin
Knowledge of uncommon vascular origins, particularly from the coronaries is important to avoid injury and possible ischemia, infarction, exsanguinations or death. Authors have described sequestrations supplied by the circumflex coronary artery [40–42], the right coronary artery [43,44] or both [45]. Patients present with progressive angina caused by myocardial ischemia, vasospastic angina due to coronary steal [42] or with pulmonary symptoms due to the sequestration.

2.3.3.8 Management
Coronary angiography confirms the diagnosis, excludes stenotic lesions requiring simultaneously correction and demonstrates the aberrant vessel and its course. The natural history of coronary sequestration is unknown. There is no consensus regarding the management of these lesions. In the absence of complications observation may be an option [40]. Indications for surgery include pulmonary infections or coronary steal. The surgical approach is dictated by the pulmonary lobe and the coronary vessel involved. Surgical options include coronary artery grafting without resection of the sequestration [41], pulmonary resection, ligation of the aberrant vessel [42] and pharmacological treatment [42].

2.3.3.9 Mediastinal sequestration
ELSs occur between the lower lobe and diaphragm but intradiaphragmatic, pericardial, and retroperitoneal locations have been reported. Sequestrations can mimic mediastinal masses [46–48].

2.3.3.10 Mycobacterial infections
Infections are generally pyogenic, although mycobacterial [49,50] and atypical mycobacterial [51] infections have been reported. Patients presented with features suggesting tuberculosis. Surgical resection establishes the diagnosis, removes the infectious focus and prevents complications related to the infection and the sequestration. Postoperative chemotherapy is required.

2.3.3.11 Aspergillosis
Berna et al. [52] in a review of the literature discovered 14 cases of aspergillus colonization of a sequestration. Aspergillosis manifests as a pulmonary aspergilloma, invasive aspergillosis, or as an allergic broncho-pulmonary aspergillosis. The airways are thought to be the source of infection. Predisposing factors include bullae, abscess and cavities [53], especially in immunocompromised patients, diabetics, and those with malnutrition and malignancy.

Radiological signs of an aspergilloma are large intrapulmonary cavities containing a solid mass. Most lesions occur in the posterior basal segment of the lower lobes. Patients present with pneumonias refractory to conventional antibiotic therapy, long-standing allergic rhinitis or haemoptysis.

Most cases have complicated ILS. The occurrence of a fungal mycetoma within an ILS indicates that the sequestration must have an airway communication as documented by bronchography in some cases [23]. Histopathology confirms the diagnosis, and Grocott Staining demonstrates Aspergillus hyphae within the cysts. It may also be diagnosed by preoperative precipitin tests, sputum culture or CT guided biopsy. Surgical resection is the treatment of choice, and involves a lobectomy [53,54] or a segmentectomy for localized lesions.

2.3.3.12 Nocardiosis
Pulmonary nocardiosis in patients with sequestration may present as an acute or chronic respiratory illness, with clinical and radiographic findings consistent with pneumonia or abscess [55,56]. Nocardia are important pathogens in immunocompromised patients, transplant recipients, patients with malignancies and HIV, and those on corticosteroids or cytotoxic agents.

Sputum culture sometimes establishes the diagnosis [55]. Kilpatrick et al. [56] used percutaneous needle aspiration to establish the diagnosis. Subsequently the patient developed an empyema and required a lobectomy, which was complicated by adhesions. Conservative management usually results in a partial response followed by recurrence [56]. Surgical resection is the treatment of choice. Antibiotics of choice include sulfonamides, amikacin, imipenem, minocycline and dapsone.

2.3.3.13 Malignancy
Malignancies that complicate sequestrations include squamous cell carcinoma [57], lymphoepithelioma-like carcinoma [58] and localized fibrous mesothelioma [59]. Close follow-up is required in these patients due to the propensity for tumour recurrence [59].

2.3.4 Bronchogenic cyst
Congenital foregut cysts are classified as bronchogenic cysts (BC), which can be mediastinal or intrapulmonary, enteric cysts related to the esophagus and neuroenteric cysts related to spinal anomalies.

2.3.4.1 Radiology
Intrapulmonary BC appear as sharply defined, solitary, densities presenting as cysts with a homogeneous water density. They show a predilection for the lower lobes [60]. The CT density of bronchogenic and esophageal cysts can vary from water density (0–20 Hounsefield units) to high-density (80–90 Hounsefield units). The high attenuations are caused by haemorrhage, proteinaceous mucus or calcium oxalate. Areas of low attenuation in the surrounding parenchyma and bandlike linear attenuation correspond histopathologically to areas of emphysema and fibrosis [60].

The MRI appearance depends on the presence of proteinaceous material. Serous cyst fluid shows low signal intensities on T1-weighted images and high signal intensities on T2-weighted images. Most BCs contain proteinaceous material and have high signal intensity on T1-weighted images.

Enteric cysts occur in the distal thirds of the esophagus. Endoscopic ultrasound appears to be the best method to diagnose esophageal cysts [61].

2.3.4.2 Cervical bronchogenic cysts
CT reveals a neck mass extending downwards to displace the trachea and esophagus. Computed three-dimensional modelling has been used to demonstrate cyst relationship to the trachea and lung apices [62]. Virtual bronchoscopy can recreate a 3-dimensional view of the tracheobronchial luminal surface and demonstrate airway connections [62].

2.3.4.3 Perfusion scans
Sub-carinal BCs can cause unilateral absent lung perfusion on an isotope scan, due to pulmonary artery obstruction [63].

2.3.4.4 Infections and haemoptysis
Cyst infection is usually caused by communication with the tracheobronchial tree. Intraparenchymal cysts are more prone to infection. Infected mediastinal cysts are usually adherent and create operative difficulties resulting in tracheobronchial or esophageal injury. Pyogenic infections are common although salmonella [64] has also been reported. Few authors [65,66] have reported haemoptysis in patients with a long standing BC.

2.3.4.5 Mimicking lesions
BCs have been known to clinically and radiologically mimic aortic aneurysms [67,68], mitral stenosis [69], neurogenic tumours of the posterior mediastimum [70], hydropneumothorax [71] and bronchogenic carcinoma [72].

2.3.4.6 Cardiac cysts
BCs have been reported in the interatrial septum [73,74]. Surgical resection involves tumour enucleation and patch closure of the resultant septal defect. MRI shows an isointense tumour on the T 1-weighted images and a high-intensity tumour on the T 2-weighted images [73].

2.3.4.7 Acute presentations
Adult BCs can have catastrophic presentations. Kenebeck [75] reported a patient with a myocardial infarction and cardiac arrest resulting from compression of the left main coronary artery by a large BC. Thoracotomy revealed a sub-carinal cyst between the aortic root and the left main coronary artery. Others have reported cardiac BCs causing ischemic changes [76], haemodynamic compromise [77] from aortic compression, and pulmonary edema due to pulmonary venous [78] compression. BCs can cause extrinsic left atrial compression [79] and mimic a cardiac tumour on echocardiograms, or mitral stenosis [69] but are clearly demonstrated on MRI.

2.3.4.8 Malignancy
Bronchioalveolar [80,81] and undifferentiated [82] carcinomas have been reported to complicate long-standing BCs.

2.3.4.9 Management—asymptomatic lesions
The management of asymptomatic BCs is controversial. Bolton et al. [83] recommended observation for small, asymptomatic cysts and suggested aspiration for decompression. In a review by St Georges and colleagues [84], 82% of patients had cysts that were either symptomatic or complicated. Complications include recurrent respiratory obstruction, intracystic haemorrhage, pulmonary artery obstruction, cyst rupture, cyst recurrence following incomplete excision and malignant transformation.

Major operative difficulties were encountered in 35 symptomatic patients [84]. Kirby et al. [85] reported increased postoperative complications in symptomatic patients (27 vs. 14%).

There is a general consensus that all adult BCs should be resected even if asymptomatic, since the majority will ultimately become symptomatic or complicated [84]. Early intervention prevents future complications.

2.3.4.10 Treatment options
Treatment options include evacuation through mediastinal, percutaneous, bronchoscopic or thoracoscopic approaches, as well as open resection.

2.3.4.11 Aspiration
Percutaneous [86] or transbronchial [87] needle aspiration have been advocated but do not provide useful information, have the potential for infecting the mass and cause recurrence in virtually all patients [86]. In such cases, the cyst becomes densely adherent to and can erode into the airway thus complicating subsequent surgical excision. Tripp et al. [86] reported such an experience where they had to resect the right bronchus to achieve complete cyst clearance.

2.3.4.12 Mediastinoscopy
Mediastinoscopic drainage and chemical sclerosis has been recommended for sub-carinal cysts[88,89]. But these reports have been few and this is not considered the standard of treatment.

2.3.4.13 Iatrogenic injuries—bronchus rupture
Close adherence to vital structures makes surgical excision difficult. Authors [72,86] have reported injury to the bronchus intermedius requiring repair or flap reconstruction, as well as pulmonary artery injuries [90].

2.3.4.14 Partial resection
Complete surgical excision by thoracotomy or VATS is the goal. Partial resection may occasionally be necessary because of cyst adherence to vital structures. Obliteration of the mucosal lining by electrocautery is necessary to prevent recurrence and malignant degeneration. Gharagozloo et al. [91] reported a patient who presented with a pseudocyst 24 years after incomplete excision of a sub-carinal BC. Recurrent cysts lack a true capsule. Attempts at ‘shelling out’ the pseudocyst result in mediastinal spillage of infected cyst content. Sharp dissection of the pseudocapsule is required to separate the residual cyst wall which can be adherent to the bronchus, the left atrium or esophagus. Following complete excision any tracheobronchial defects should be closed. The greater technical difficulties and inevitable formation of a pseudocyst after partial excision present a convincing argument for complete resection.

2.3.4.15 Cervical and esophageal cysts
Cervical BCs [62,92] can communicate with the trachea [62] and require meticulous dissection to separate them from the trachea and esophagus.

Esophageal cysts cause dysphagia. Endoscopic ultrasound is considered valuable for the diagnosis of such cysts. VATS resection has been reported.

Esophageal cysts should be excised by carefully preserving the muscle layer. Both vagi should be identified and preserved. Mucosal integrity should be checked intraoperatively [93]. Sahiyama et al. [94] reported endoscopic mucosal resection of esophageal cysts.

2.3.5 Differential diagnosis of congenital lesions
The differential diagnosis of congenital lesions in adults includes bullous disease, cavitating tumours, bronchogenic cysts, cystic bronchiectasis, sequestrations, lung abscess, malignancy, empyema, fungal diseases, tuberculosis, nocardiosis and vascular malformations.

2.3.6 Video-assisted thoracoscopic surgery
Video-assisted Thoracoscopy (VATS) has been predominantly used in the management of sequestrations and BCs.

2.3.6.1 Sequestration
VATS lobectomy has been successfully performed to excise sequestrations [95]. VATS wedge resection is an important option for localized pulmonary sequestrations [28] and preserves lung volume and function. Potential problems with VATS wedge resection include bleeding and air leaks. Additional suture ligatures, electrocautery, clips or the argon beam coagulator can be helpful. In case of major difficulties a thoracotomy should be considered. It is crucial to secure the aberrant vessels prior to any form of resection to avoid catastrophic haemorrhage [28].

2.3.6.2 Bronchogenic cysts
Both bronchogenic [96,97] and esophageal cysts [98] have been resected by VATS. Martinod and colleagues [97] reported a 35% thoracotomy conversion rate due to bleeding or adhesions to vital organs. Sato et al. [34] reported excision of a mediastinal BC by VATS by an extrapleural approach in a patient with dense pleural adhesions and suggested that a VATS approach is easier and safer even with dense adhesions. Extrapleural dissection, however, has risks for potential injuries to vessels or organs and may cause major bleeding from the extrapleural space.

BCs can be found in close proximity to lung parenchyma, the esophagus and the bronchus intermedius. Cho et al. [99] reported delayed bronchial rupture following VATS excision of a mediastinal BC with pericystic adhesions to the bronchus. For esophageal cysts, Cioffi et al. [93] advocate esophageal transillumination to control mucosal integrity and prevent damage.

2.3.6.3 CCAM
Huang et al. [100] have reported VATS wedge resection for CCAM. It is debatable, however, if this is the appropriate mode of management for CCAM given the propensity for associated malignancy. This was further illustrated by Sudou et al. [10] reported a bronchioloalveolar carcinoma in a VATS lobectomy specimen for CCAM. An open thoracotomy may be the ideal approach for CCAM unless it is possible to achieve complete oncological clearance using VATS.


   3. Conclusion
Top
 Abstract
 1. Introduction
 2. Patterns of presentation
 3. Conclusion
References
 
Congenital lung malformations are increasingly diagnosed in adults. They have unique manifestations, often mimic other thoracic pathology and can present acutely and necessitate emergent evaluation and management. The reports discussed in this article illustrate that several patients have had partial resections as infants, or have recurrent infections for years that are managed medically. They eventually develop complications, malignancies or present acutely and require emergency surgery. These scenarios can be prevented by early diagnosis and surgery and more importantly by recognizing the existence of these lesions as a separate entity. The adult manifestations are sufficiently unique to merit the term ‘ADULT CONGENITAL LUNG DISEASE’. Patients in whom a malformation has been managed expectantly and those who have had partial resections need a well-organized transition from paediatric into adult care.


   References
Top
 Abstract
 1. Introduction
 2. Patterns of presentation
 3. Conclusion
 References
 

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