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Year : 2018  |  Volume : 20  |  Issue : 3  |  Page : 174-176

Presentation and surgical treatment of congenital pulmonary airway malformation in an adult

Department of Thoracic Surgery, Yedikule Chest Diseases and Thoracic Surgery Education and Training Hospital, Istanbul, Turkey

Date of Submission26-Jul-2018
Date of Decision29-Sep-2018
Date of Acceptance11-Nov-2018
Date of Web Publication31-Dec-2018

Correspondence Address:
Cemal Aker
Yedikule Chest Diseases and Thoracic Surgery Education and Research Hospital, Kazliçesme Mh, Belgrade Kapi Yolu Cad No: 1, Zeytinburnu, 34020, Istanbul
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ejop.ejop_39_18

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Congenital pulmonary airway malformation (CPAM) is a congenital lung disease that is usually diagnosed in the prenatal or neonatal period but is rarely seen in adults as well. In this report, we present a 26-year-old male patient who presented to our clinic with respiratory difficulty due to a CPAM that affected the entire right lung, caused mediastinal and diaphragmatic compression, and led to extensive diffuse hemorrhage from the adjacent structures that required massive transfusion.

Keywords: Adult patient, congenital airway malformation, surgical resection

How to cite this article:
Aker C, Kalafat CE, Bilen S, Kocatürk C&. Presentation and surgical treatment of congenital pulmonary airway malformation in an adult. Eurasian J Pulmonol 2018;20:174-6

How to cite this URL:
Aker C, Kalafat CE, Bilen S, Kocatürk C&. Presentation and surgical treatment of congenital pulmonary airway malformation in an adult. Eurasian J Pulmonol [serial online] 2018 [cited 2021 Jan 20];20:174-6. Available from: https://www.eurasianjpulmonol.com/text.asp?2018/20/3/174/245977

  Introduction Top

Congenital pulmonary airway malformation (CPAM) is a congenital disorder of the lungs with an incidence of 1 in 8300–35,000 live births.[1] First described by Stocker and Dehner in 1897 as “congenital cystic adenomatoid malformation,” it was renamed CPAM in 2001.[2] The condition presents with respiratory distress and is very rarely encountered in adults. CPAM is characterized by the formation of hamartomatous lesions originating from the tracheal, bronchial, bronchiolar, or alveolar tissue and containing cystic and adenomatous components. It may lead to compression of adjacent normal tissues and impaired alveolar growth and development. CPAMs affect both lungs equally and can form in all lobes. Although the lesions are usually limited to a single lobe, in rare cases, they may involve the entire lung.[3],[4],[5]

  Case Report Top

A 26-year-old male patient was referred to our hospital from abroad for testing and treatment. He had no significant childhood history but reported respiratory problems starting in adulthood. Chest X-ray showed parenchymal loss and a bullous lung appearance encompassing the entire right hemithorax, displacing the mediastinum to the left [Figure 1].
Figure 1: Posterior-anterior chest X-ray taken at presentation

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He had no history of previous illness, trauma, or smoking. On physical examination, breath sounds from the right hemithorax were decreased while other systemic examination findings were normal. Thoracic computed tomography showed massive bullous structures filling the right hemithorax and causing mediastinal displacement, flat diaphragm, and parenchymal loss [Figure 2].
Figure 2: Computed tomography of the chest taken at presentation

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In pulmonary function test, the patient's forced expiratory volume in 1 s was 1.45 L (36%). Ventilation-perfusion scintigraphy revealed that the right lung was not involved in respiration. Bronchoscopy showed the leftward deviation of the trachea and carina and near-complete closure of the right upper and middle lobe bronchi due to mass effect. No endobronchial lesions were detected. The patient underwent right thoracotomy and right pneumonectomy as advised by the surgical consultation council [Figure 3].
Figure 3: Intraoperative appearance and resected right lung

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A right thoracotomy was made through the fifth intercostal space, but sufficient exploration was not possible due to the mass effect exerted by the enlarged bullous areas. There were tight adhesions between the lung and the chest wall, mediastinal surface, and diaphragmatic space. During pneumolysis, diffuse bleeding from the chest wall and parenchyma occurred. We decided to achieve hemostasis by quickly completing the pneumonectomy. Due to coagulation issues, a total of 12 units of erythrocyte suspension, 6 of fresh frozen plasma, 6000 cc crystalloids, and 4000 cc colloids were infused during the 4.5-h operation. After removing the lung tissue, the hemorrhage continued in the right thoracic cavity despite hemostasis. The thoracic cavity was packed with 20 abdominal pads and closed. The patient was moved to the intensive care unit while intubated. His vital signs and biochemical parameters were within normal limits on postoperative day 1, and he underwent reoperation for decompression. Following rethoracotomy, the pads were removed from the abdomen, and hemostasis was achieved. He was extubated and returned to the intensive care unit. The patient exhibited no further problems during 3 days of follow-up in intensive care and 4 days in the inpatient ward, and he was discharged on postoperative day 7. The pathology results indicated CPAM. The patient remains symptom free under outpatient follow-up [Figure 4].
Figure 4: Follow-up chest X-ray taken on postoperative day 20

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  Discussion Top

CPAM has been classified into four clinically and pathologically distinct types. The classification is based on the size, number, and location of cysts and whether they affect the entire lobe or lung or not. Type 4 is associated with malignancy.[3],[6]

Approximately 80% of CPAMs are diagnosed in the prenatal and neonatal period. The majority of cases that are not diagnosed during this period are identified in infants and school-age children. CPAM is very rarely seen in adults.[7] In 2018, Hamanaka et al. stated that a total of 61 adult CPAM patients are reported in the literature.[7]

CPAM can present with respiratory distress in the neonatal period or, as in our patient, remain asymptomatic for many years. The severity of respiratory difficulty is proportional to the size of the lesion. Typical findings include increased respiratory effort with tachypnea, grunting, and use of accessory muscles, and cyanosis. Bronchopulmonary sequestration (BPS) should be included in the differential diagnosis. BPS appears as a homogenous mass in prenatal ultrasonography and has a systemic arterial supply. Adult patients present with a cough, sputum, respiratory distress, and frequent and recurrent infections.[7]

The treatment of CPAM varies depending on whether patients are symptomatic or not. Surgical treatment, preferably lobectomy, is performed on symptomatic patients. Wedge resection is not recommended because the boundaries of the lesion are not clearly distinguishable. Surgery is generally performed in neonates with severe respiratory distress. Surgical resection is recommended for asymptomatic patients with bilateral or multifocal cysts.

The timing of surgery is a matter of debate. It has been argued that 3–6 months is the ideal time for the operation.[8] Successful outcomes with no complications have been reported with minimally invasive surgery for asymptomatic patients in infancy.[8],[9] In patients with mild symptoms, elective surgery may be performed in later childhood. In older children, resection is usually performed to prevent recurring infections and eliminate concerns of malignancy.[3]

The most common complication in adult patients is recurrent pulmonary infections. The possibility of malignancy (albeit unlikely) in previously undiagnosed adult patients is a valid reason for resection.[3] It should be kept in mind that, as in our patient, surgical resection may be complicated due to the size of the lesion. Surgical resection was planned for our patient to resolve symptoms associated with mediastinal shift. During surgery, we found that due to its delayed diagnosis and slow growth, the lesion had gradually filled the right hemithorax completely and had established vascular connections with the parietal pleura. It was perfused through enhanced vascularization of the mediastinal, parietal, and diaphragmatic surfaces, which resulted in significant bleeding during dissection. Fievet et al. also reported a significant increase in mortality and morbidity in adults undergoing surgical treatment due to delayed diagnosis.[10]

  Conclusion Top

CPAM usually manifests symptomatically and requires treatment when patients are neonates or children but can rarely present in adulthood, as in our case. Surgery is the primary treatment for eligible patients. The increased risk of morbidity and intraoperative hemorrhage in adult patients should be kept in mind, and preoperative preparations should be made accordingly.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Shanti CM, Klein MD. Cystic lung disease. In: Seminars in Pediatric Surgery. Detroit, Michigan: Elsevier; 2008. p. 2-8.  Back to cited text no. 1
Stocker JT, Dehner LP. Pediatric Pathology. Vol. 1. Philedelphia: Lippincott Williams & Wilkins; 2001.  Back to cited text no. 2
Shields TW, LoCicero J, Reed CE, Feins RH. General Thoracic Surgery. Vol. 1. Philadelphia: Lippincott Williams & Wilkins; 2009.  Back to cited text no. 3
Pinter A, Kalman A, Karsza L, Verebely T, Szemledy F. Long-term outcome of congenital cystic adenomatoid malformation. Pediatr Surg Int 1999;15:332-5.  Back to cited text no. 4
Evrard V, Ceulemans J, Coosemans W, De Baere T, De Leyn P, Deneffe G, et al. Congenital parenchymatous malformations of the lung. World J Surg 1999;23:1123-32.  Back to cited text no. 5
Salzberg AM, Krummel TM. Congenital malformations of the lower respiratory tract. In: Chernick V, Kendig EL, editors. Kending's Disorders Of The Respiratory Tract In Children, 5th ed., Philadelphia, W.B. Saunders Company; 1990. p. 227-67.  Back to cited text no. 6
Hamanaka R, Yagasaki H, Kohno M, Masuda R, Iwazaki M. Congenital cystic adenomatoid malformation in adults: Report of a case presenting with a recurrent pneumothorax and a literature review of 60 cases. Respir Med Case Rep 2018. [In press].[doi.org/10.1016/j.rmcr. 2018.02.002].  Back to cited text no. 7
Truitt AK, Carr SR, Cassese J, Kurkchubasche AG, Tracy TF Jr., Luks FI, et al. Perinatal management of congenital cystic lung lesions in the age of minimally invasive surgery. J Pediatr Surg 2006;41:893-6.  Back to cited text no. 8
Vu LT, Farmer DL, Nobuhara KK, Miniati D, Lee H. Thoracoscopic versus open resection for congenital cystic adenomatoid malformations of the lung. J Pediatr Surg 2008;43:35-9.  Back to cited text no. 9
Fievet L, Natale C, D'Journo XB, Coze S, Dubus JC, Guys JM, et al. Congenital pulmonary airway malformation and sequestration: Two standpoints for a single condition. J Minim Access Surg 2015;11:129-33.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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