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Ann Thorac Surg 2004;78:337-339
© 2004 The Society of Thoracic Surgeons

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Case report

Infected cystic adenomatoid malformation in an adult

^a Department of Surgery, Changhua Christian Hospital, Changhua, Taiwan
^b Department of Critical Care Medicine, Changhua Christian Hospital, Changhua, Taiwan
^c Department of Internal Medicine, Chung Shan Medical University, Taichung, Taiwan

Accepted for publication June 13, 2003.

^* Address reprint requests to Dr Fang, Department of Surgery, Changhua Christian Hospital, 135 Nanhsiao St, Changhua, 500 Taiwan
e-mail: 51302{at}cch.org.tw

	Abstract

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Congenital cystic adenomatoid malformation of the lung is rare in adults. We present a 51-year-old man with recurrent lower respiratory tract infections and intermittent febrile episodes for more than 10 years. Multiple cavitary lesions with fluid accumulation were seen in the right lower lobe of the lung on radiography and computed tomography of the chest. Wedge resection of the right lower lobe of the lung was performed through a mini-thoracotomy using video-assisted thoracoscopy. Multiple infected cysts were found in a 10 x 8 x 6 cm area of lung. The histologic diagnosis was infected type I congenital cystic adenomatoid malformation. The patient remains well 1 year after the operation.

	Introduction

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Congenital cystic adenomatoid malformation (CCAM) of the lung is detected most commonly before birth or immediately afterwards in the newborn period [1]; however, diagnosis may be delayed until adulthood. To date, more than 30 adult cases have been reported in the literature, mostly in two studies [1, 2]. Usually the right lower lobe is involved, but the left lower lobe [3], bilateral lobe involvement [4], and even the entire lung [5] have been affected.

Recurrent lower respiratory tract infections are the chief presentation in adults, although recurrent pneumothorax has also been reported [1, 2]. We present here an adult patient with CCAM and a history of recurrent lower respiratory tract infections who was treated by surgical resection of the affected area using a video-assisted thoracoscopic surgical (VATS) approach through a mini-thoracotomy. There has only been one other report of the use of this technique in the treatment of CCAM [4].

The patient was a 51-year-old man with a long history of episodes of fever and dry cough treated by his local doctor as lower respiratory tract infections. Over the past 10 years, these episodes had increased in frequency, and 3 months before the index admission the patient presented to our clinic complaining of increasing weakness, difficulty performing daily tasks, intermittent fever, sore throat, and a persistent dry cough that caused pain on the right side of his chest extending through to his back.

A chest radiograph showed a right lower lobe lesion with consolidation and multiple fluid-containing cavities. Chest computed tomography (CT) confirmed multiple cystic cavities in the posterior basal segment of the right lung, associated with ground-glass opacities and collections of fluid consistent with secondary infection (Fig 1). Pulmonary emphysema was also present and a pattern resembling pulmonary sequestration. Magnetic resonance imaging and angiography (MRA) identified multiple cystic lesions without associated vascular abnormalities.

View larger version (97K): [in this window] [in a new window]   Fig 1. (Left) Chest radiograph showing multiple cavitary lesions and consolidation of the right lower lobe of the lung. (Right, top and bottom) Chest computerized tomography scans showing multiple cystic cavities in the posterior basal segment of the right lower lobe of the lung.

We collected about 100 mL of yellowish turbid pleural fluid. A 10 x 8 x 6 cm portion of lung with multiple purulent cysts was removed and sent for culture and histology. At histology, the excised specimen showed neutrophil aggregation, with macrophages filling the alveolar spaces. Scattered lymph follicle formation and slight interstitial thickening were present throughout the section. The bronchus and respiratory alveolar ducts were markedly dilated and plugged by mucus and exudates (Fig 2). Culture of the excised specimen resulted in no bacterial growth on either anaerobic or TB culture medium. This was probably due to the prolonged preoperative oral antibiotic treatment.

View larger version (149K): [in this window] [in a new window]   Fig 2. The bronchus and respiratory alveolar ducts are dilated and filled with neutrophil and macrophages. (Hematoxylin & eosin; x100, x200 [inset].)

	Comment

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Congenital cystic adenomatoid malformation accounts for about 95% of congenital cystic lung disease [2] and is a cause of respiratory distress in infants due to obstruction from its bulk. The condition is rare in older children and adults, but when CCAM occurs, it most commonly presents as recurrent pulmonary infection [1, 2]. On occasion, recurrent pneumothorax may be a problem [1, 2].

The typical finding from chest CT is a localized, thin-walled, multiple cystic abnormality [2]. In adult cases, no other associated congenital abnormality is found. In our patient, a chest radiograph 1 year earlier showed right lower lobe consolidation of the lung but, before this sign, recurrent respiratory tract infections had been diagnosed from symptoms.

Several pulmonary diseases should be included in the differential diagnosis; these include intralobular pulmonary sequestration, intrapulmonary bronchogenic cyst, congenital lobar emphysema, pneumothorax, pyopneumothorax, cystic bronchiectasis, and prior pneumatocele formation [2]. In our patient, chest radiography did not provide a definite diagnosis, but chest CT confirmed the diagnosis and ruled out bilateral involvement. We excluded vascular lesions by chest MRA.

Criteria used to distinguish CCAM from other cystic lung disease include the absence of cartilage, the absence of bronchial gland, foci of tall columnar mucinous epithelium, overproduction of terminal bronchiolar structure without alveolar differentiation, and enlargement of the affected lobe [6]. Congenital cystic adenomatoid malformation affects all lobes with the same frequency, with no right or left predominance [2].

Congenital cystic adenomatoid malformation is classified into types I to III, depending on cyst diameter and the dominant cells histologically (bronchial, bronchiolar, or bronchiolar/alveolar dust) [7]. Type I is the most common variety and consists of single or multiple large cysts lined by ciliated, pseudostratified columnar epithelium with elastic tissue and smooth muscle in the walls of the cysts. Type II consists of multiple small cysts lined by cuboidal to columnar epithelium and no cartilage or mucous cells. Type III is a large, bulky, noncystic lesion producing mediastinal shift and containing bronchiole-like structures lined by cuboidal epithelium [8]. Our case was type I consisting of multiple large cysts.

Congenital cystic adenomatoid malformation is an uncommon cause of pulmonary pathology in adults, but in the presence of recurrent lower respiratory tract infections or recurrent pneumonia, a diagnosis of this malformation should be considered. Radiologic studies can be useful in the differential diagnosis, but a definitive diagnosis cannot be made without histologic confirmation of the embryonic abnormalities.

Oral antibiotic therapy was ineffective in our patient. Long-term oral antibiotics may be of some benefit in preventing recurrences; however, prolonging antibiotic therapy probably delays the eventual surgical procedure and increases the risk of antibiotic resistance developing. The treatment of CCAM is surgical and should be performed as soon as the diagnosis is made [1]. The association of bronchoalveolar carcinoma or rhabdomyosarcoma with CCAM suggested in the reports of neonatal cases is not supported in adult case series [1, 2].

The VATS approach through a mini-thoracotomy was a technique that facilitated comprehensive inspection of the relevant lung areas during operation, while leaving the patient with only a small operation incision that led to rapid postoperative recovery.

	References

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1. Lujan M., Bosque M., Mirapeix R.M., et al. Late-onset congenital cystic adenomatoid malformation of the lung. Embryology, clinical symptomatology, diagnostic procedures, therapeutic approach and clinical follow-up. Respiration 2002;69:148-154.[Medline]
2. Patz E.F., Jr, Muller N.L., Swensen S.J., Dodd L.G. Congenital cystic adenomatoid malformation in adults: CT findings. J Comput Assist Tomogr 1995;19:361-364.[Medline]
3. Han Y.M., Lee D.K., Lee S.Y., et al. Adult presentation of congenital cystic adenomatoid malformation of the lung: a case report. J Korean Med Sci 1994;9:86-91.[Medline]
4. Yim A.P. Video-assisted thoracoscopic resection of type I cystic adenomatoid malformation in a 3-month old girl. Surg Endosc 1995;9:1292-1294.[Medline]
5. Lackner R.P., Thompson A.B., 3rd, Rikkers L.F., Galbraith T.A. Cystic adenomatoid malformation involving an entire lung in a 22-year-old woman. Ann Thorac Surg 1996;61:1827-1829.[Abstract/Free Full Text]
6. Cass D.L., Quinn T.M., Yang E.Y., et al. Increased cell proliferation and decreased apoptosis characterize congenital cystic adenomatoid malformation of the lung. J Pediatr Surg 1998;33:1043-1047.[Medline]
7. Rosado-de-Christenson M.L., Stocker J.T. Congenital cystic adenomatoid malformation. Radiographics 1991;11:865-886.[Abstract]
8. Cloutier M.M., Schaeffer D.A., Hight D. Congenital cystic adenomatoid malformation. Chest 1993;103:761-764.[Abstract/Free Full Text]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Huang, H.-J. Articles by Fang, H.-Y. Search for Related Content PubMed PubMed Citation Articles by Huang, H.-J. Articles by Fang, H.-Y. Related Collections Lung - other