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Ann Thorac Surg 1996;61:1827-1829
© 1996 The Society of Thoracic Surgeons

Case Report

Cystic Adenomatoid Malformation Involving an Entire Lung in a 22-Year-Old Woman

Rudy P. Lackner, MD, Austin B. Thompson, III, MD, Layton F. Rikkers, MD, Timothy A. Galbraith, MD

Division of Thoracic Surgery, Department of Surgery, and Division of Pulmonary Medicine, University of Nebraska Medical Center, Omaha, Nebraska

Accepted for publication November 30, 1995.


   Abstract
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Congenital cystic adenomatoid malformation is an uncommon cause of respiratory distress in infants and is a rare entity in adults. Presentation in older patients is that of recurrent pulmonary infections. Usually a single lobe is involved. This report describes congenital cystic adenomatoid malformation involving the entire right lung in a 22-year-old woman presenting with gastrointestinal bleeding due to cavernous transformation of the portal and splenic veins.


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Congenital cystic adenomatoid malformation (CCAM) of the lung is an uncommon cause of respiratory distress in neonates and infants. Diagnosis of this entity in adults has occasionally been reported, with most cases involving a single lobe [1–5]. This report describes CCAM that involved the entire lung in a 22-year-old woman.

The patient was a 22-year-old woman with a history of gastrointestinal bleeding due to cavernous transformation of the portal and splenic veins. Administration of propranolol, prescribed to control gastrointestinal bleeding, was discontinued by the patient 1 year before admission. Massive esophageal bleeding developed requiring placement of a Minnesota tube and transfusion of multiple units of blood. Sclerotherapy was performed with control of the bleeding, and a gastric devascularization procedure was planned as definitive therapy. Chest radiographs demonstrated hyperinflation of the right lung with mediastinal shift and compression of the left lung (Fig 1Go).



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  		Fig 1. . Chest roentgenogram showing marked hyperinflation of the right lung with mediastinal shift.

 
The pulmonary abnormality had been diagnosed 3 years earlier, when the patient presented with her first episode of gastrointestinal bleeding. At that time there were no pulmonary symptoms and she was actively involved in aerobic exercise. Over the past 3 years, worsening dyspnea and occasional episodes of hemoptysis developed.

After cessation of the bleeding, a computed tomographic scan revealed diffuse cystic changes in the right lung, with some normal parenchyma in the upper lobe. An area of consolidation was present in the right lower lobe (Fig 2Go). Other radiographic abnormalities included an osseous vertebral malformation and a presacral mass. Pulmonary function tests demonstrated forced vital capacity of 1.33 L, a forced expiratory volume in 1 second of 1.08 L, and a carbon monoxide diffusing capacity of 18.5 mL•min-1•mm Hg-1 (50%). The split function perfusion scan showed 82% of the pulmonary blood flow directed to the left lung. A right middle and lower lobectomy was planned.



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  		Fig 2. . Computed tomographic scan of the chest demonstrating cystic changes and an area of consolidation in the right lower lobe.

 
Examination of the right lung revealed extensive disease throughout all lobes, with minimal normal parenchyma in the apical segment of the right upper lobe. The normal architecture of the lung was distorted, without clearly identifiable interlobar fissures, and clot-filled cysts were present posteriorly (Fig 3Go). Pneumonectomy was facilitated by shifting the mediastinum to the right by adding 10 cm H2O positive end-expiratory pressure to the left lung.



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  		Fig 3. . Right pneumonectomy specimen with multiple various-sized cysts and evidence of recent bleeding.

 
The postoperative film showed a well-expanded left lung, without evidence of cystic disease. Pathologic analysis was significant for congenital cystic adenomatoid malformation (type I) involving the entire right lung. Two additional sclerotherapy procedures were performed on the remaining esophageal varices.

She was discharged home on the eleventh postoperative day. Six months postoperatively there have been no further bleeding episodes and she has resumed her normal activities.


   Comment
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References
 
Cystic pulmonary diseases can be a cause of respiratory distress in infants, and considerable attention has been directed toward antenatal detection and intrauterine treatment of these lesions. In a review of more than 150 patients, only 17% of the patients with CCAM were more than 6 months of age [6]. The presentation in older patients is usually that of recurrent pulmonary infections. In addition to the typical respiratory lining cells and mucin-secreting cells, the cysts in older patients demonstrate an inflammatory component not found in infants.

The characteristic cystic changes found in CCAM usually involve a single lobe. Of the 153 cases collected by Cloutier and associates [6], there were only 27 cases of multilobar involvement including a single report of bilateral disease. All three lobes in our patient had cystic changes, with the most severe cystic disease located in the middle and lower lobes. Due to the associated pathologic findings, every attempt was made to preserve any normal right lung. Browdie and colleagues [7] have successfully performed nonanatomic resections of CCAM in infants, with long-term follow-up showing increased function in the remaining lung. Certainly in infants, the potential for lung growth exists, but whether significant growth could occur in the adult to offset the immediate space problems created is unclear.

The constellation of cavernous transformation of the portal and splenic veins, vertebral anomalies, sacral mass, and cystic adenomatoid malformation of the lung is unusual. Other anomalies associated with CCAM have been described in up to 18% of patients [6]. These have most commonly been renal agenesis and cardiac anomalies.

Congenital cystic adenomatoid malformation is an uncommon cause of pulmonary pathology in the adult. Resection usually results in improved respiratory function. In this case, the split function perfusion study was very helpful in determining operability. Associated anomalies are common and may influence perioperative management.


   Footnotes
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Address reprint requests to Dr Lackner, Department of Cardiothoracic Surgery, University of Nebraska Medical Center, 600 S 42nd St, Omaha, NE 68198-2315.


   References
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 References
 

  1. Han YM, Lee DK, Lee SY, et al. Adult presentation of congenital cystic adenomatoid malformation of the lung: a case report. J Korean Med Sci 1994;9:86–91.
  2. Akiba T, Yamazaki Y, Yasukawa S, Yuno S, Yoshida T, Sakurai K. A case of congenital cystic adenomatoid malformation of the lung in an 18 year old male. Nippon Kyobu Geka Gakkai Zasshi 1992;40:161–4.
  3. Pulpeiro JR, Lopez I, Sotelo T, Ruiz JC, Garcia-Hidalgo E. Congenital cystic adenomatoid malformation of the lung in a young adult. Br J Radiol 1987;60:1128–30.
  4. Sagawa H, Ebihara Y, Kuwabara T, Sasaki T, Yanagisawa M, Kidokoro T. Two adult cases of pulmonary congenital cystic adenomatoid malformation. Nippon Kyobo Shikkan Gakkai Zasshi 1985;23:593–8.
  5. Hlnick DH, Naidich DP, McCauley DI, et al. Late presentation of congenital cystic adenomatoid malformation of the lung. Radiology 1984;151:569–73.
  6. Cloutier MM, Schaeffer DA, Hight D. Congenital cystic adenomatoid malformation. Chest 1993;103:761–4.
  7. Browdie D, Todd D, Agnew R, Rosen W, Beardmore H. The use of nonanatomic resection in infants with extensive congenital adenomatoid malformation of the lung. J Thorac Cardiovasc Surg 1993;105:732–6.



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This Article
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Right arrow Author home page(s):
Rudy P. Lackner
Timothy A. Galbraith
Right arrow Permission Requests
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Right arrow Articles by Lackner, R. P.
Right arrow Articles by Galbraith, T. A.
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Right arrow Articles by Lackner, R. P.
Right arrow Articles by Galbraith, T. A.

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