Ann Thorac Surg 2002;73:1594-1598
© 2002 The Society of Thoracic Surgeons
Original article: general thoracic
Cystic lung lesions in the pediatric and adult population: surgical experience at the Brompton hospital
Konstantinos Papagiannopoulos, MMED (Thorax)a,
Sean Hughes, MBBSa,
Andrew G. Nicholson, MRCPathb,
Peter Goldstraw, FRCS*a
a Department of Thoracic Surgery, Royal Brompton Hospital, London, United Kingdom
b Department of Histopathology, Royal Brompton Hospital, London, United Kingdom
Accepted for publication January 21, 2002.
* Address reprint requests to Mr Goldstraw, Department of Thoracic Surgery, Royal Brompton Hospital, Sydney St, SW3 6NP, London, UK
e-mail: pgoldstraw{at}rbh.nthames.nhs.uk
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Abstract
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Background. Cystic lung lesions are found in the adult and pediatric populations. We present our 19-year experience with such lesions from a single institution.
Methods. We retrospectively reviewed our experience with such lung pathology, comparing the pediatric and adult populations with regard to their spectrum of pathology, presentation, surgical procedures, and postoperative morbidity and mortality.
Results. Forty-six operations were performed on 44 patients (24 children and 20 adults). Cystic adenomatoid malformation was the most common pathology in the pediatric group (53.9%), followed equally by simple cysts and sequestrations (15.4%). In three of the pediatric patients malignant features were identified after resection; bronchoalveolar carcinoma in 2 patients with cystic adenomatoid malformation and pleuropulmonary blastoma in 1 patient with bilateral cystic disease. In the adult population the most common pathology was simple cysts in 8 (40%), followed by cystic adenomatoid malformation in 5 (25%) and sequestrations in 2 patients (10%). The majority of the children, 16 (61.5%), required operation because of expanding or infected cysts, recurrent cysts, or chest infections; 4 were operated on as an urgent basis. In contrast, the majority of adults 11 (55%) were asymptomatic or had no specific symptoms, and all operations were elective. A larger number of major resections (65.4% versus 55%) were necessary in the pediatric group, possibly due to differences in the pathology. However, morbidity was comparable in the two groups with no mortality.
Conclusions. Operation for cystic lung disease is safe. Asymptomatic cysts in children should be resected to avoid later complications of the cysts, which could make operation more difficult. Patients should be evaluated for associated congenital anomalies. Conservative anatomic resections should be attempted to salvage functional lung tissue. Careful histologic examination of the resection specimen is mandatory to identify occult malignancy.
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Introduction
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Cystic lesions of the lung present an interesting spectrum of pathology. Controversy still exists regarding their classification as well as their management.
The term cyst has been applied to many pulmonary lesions. It is derived from the Greek word kystis that means bladder (a well-defined, closed sac that contains fluid). Few pulmonary cysts adhere to this definition. Many lung cysts contain air, some are solid, and others have ill-defined boundaries [1, 2].
The first recorded description of a pulmonary cyst is credited to Morgagni in 1769. Grawitz in 1880 made the first attempt to classify this group of pathology. Cheney and Garland in 1938 emphasized the separation of cystic lung lesions into congenital and acquired groups [3]. Saxby-Willis in 1943 proposed two different categories: bronchial cysts that are lined by ciliated columnar epithelium and alveolar cysts, which result from the destruction of lung parenchyma. Shamji classified cystic and bullous lung disease based on the pathogenesis of the lesion [1]. Cysts were grouped as developmental, acquired, and pulmonary blebs and emphysematous bullae.
It is often difficult to know whether a cyst is congenital or acquired. Buntain and colleagues proposed a definition stating that a lung cyst is considered congenital if a similar pathologic picture can be found in the prenatal or immediate neonatal period before any postnatal traumatic or inflammatory insult, or if anomalous blood supply is present at the time of diagnosis [4, 5].
This study presents the experience of a single Institution with such lesions in children and adults during a 19-year period. The aim of this study was to assess the timing and role of operation in patients of various age groups with cystic lung disease.
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Material and methods
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Between 1981 and 1999, 44 patients were identified who had been classified as having localized cystic lung lesions. In 3 patients, all of whom were adults, a tumor was identified in association with the cyst (glomus tumor [n = 1], leiomyoma [n = 1], glandular papilloma [n = 1]). In 1 patient, bilateral cystic disease was found later to be associated with a pleuropulmonary blastoma, as previously reported [6]. In the remaining 40 patients, pulmonary lesions were classified according to established criteria for cystic lesions, in particular Stocker’s classification of congenital adenomatoid malformations (CAM) [7]. Clinical data were subsequently recorded with regard to presenting symptoms, operative procedure, postoperative complications, and follow-up.
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Results
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The most common lesions were CAM, of which there were 19 cases, 11 type I, 4 type II, and 4 type IV, followed by simple congenital cysts (n = 12), bronchogenic cysts (n = 2), sequestrations (n = 6), and bronchial atresia (n = 3). Two patients presented on two occasions. Coexistent bronchioloalveolar carcinoma was noted in 2 patients with CAM type I.
In adults simple cysts accounted for the majority (8; 40%), followed by cystic adenomatoid malformation in 5 (25%), and sequestrations in 2 patients (10%). In pediatric patients the most common pathology was CAM in 14 (53.9%), followed equally by sequestrations and simple mesothelial cysts in 4 (15.4%). Bronchogenic cysts were present in 2 patients (7.7%; Table 1).
Presenting symptoms in both groups can be found in Table 2.
In the pediatric group, 16 children (61.5%) underwent operation because of expansion or infection within the cysts, or because of recurrent infections, recurrence of the cyst, or recurrent pneumothorax. Four operations in 3 children were undertaken on an urgent basis due to severe respiratory distress as a result of expanding cysts in 3 and recurrent pneumothorax in 1. Ten patients (38.5%) had nonspecific symptoms or were asymptomatic before operation.
In the adult group no urgent operations were required. Eleven patients (55%) were asymptomatic or had symptoms not related to the cystic pathology. The remainder presented with symptoms secondary to expansion or infection of the cysts, infection sometimes being recurrent (n = 3).
All patients had chest films and computed tomographic scans of the chest. Associated anomalies, namely thyroglossal cyst, ventricular septal defect and pulmonary stenosis, vesicoureteric reflux, congenital diaphragmatic hernia, pulmonary valve stenosis, cystic fibrosis, and anomalous connection of right pulmonary veins, were discovered in 7 patients (15%): 4 children (8.7%) and 1 patient in each of the neonatal, infant, and adult groups (2.2%).
We undertook operation in all patients by a posterolateral thoracotomy. An equal number of right and left thoracotomies were performed. The majority of operations, 28 (61%), involved lobectomy or bilobectomy; simple resection of the cyst proved possible in 12 patients (26%). Wedge resection or anatomic segmental resection was possible in 6 patients (13%) (Table 3).
Lobectomies or bilobectomies were performed in 65.4% of the pediatric group compared with 55% in the adults. Minor resections were 34.6% and 45%, respectively.
Drains were removed at a mean of 4.7 days (range, 1 to 14 days). The postoperative mean hospital stay was 8.5 days (range, 3 to 25 days). One patient developed a persistent air leak. The drain was attached to a Heimlich system; he was discharged and the drain was removed in the first follow-up appointment, 15 days after the operation.
There were no in-hospital deaths. Major complications were encountered in 6 patients (13%) (Table 4).
In the adult group 1 patient had persistent lobar collapse requiring bronchoscopy and bronchial lavage; another patient, described previously, experienced prolonged air leak; one developed deep venous thrombosis and pulmonary embolus and another developed an empyema requiring surgical drainage with rib resection. In the pediatric group one child required reexploration for bleeding and another required a completion lobectomy after lingulectomy for cystic adenomatoid malformation complicated by recurrent pneumothoraces.
Five patients developed insignificant pneumothorax after removal of their intercostal drains requiring no further management. None of the patients required intraoperative or postoperative blood transfusions, even the one reexplored for bleeding. All patients were seen a month after operation and were alive and well.
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Comment
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The prevalence of cystic lung lesions has been difficult to ascertain principally because of differences in classification and variations in nomenclature. An early study by Schenck [8] revealed that 10% of cases are recognized at birth, 14% are discovered during infancy, and the rest present after the age of 15 years. Cooke and Blades [9] reported an overall incidence of 0.04% to 0.06%. Furthermore, controversy still exists as to whether these lesions are all congenital or whether some are acquired. Clearly, those lesions found at prenatal examination or shortly after birth are congenital, but it has been suggested that those with later presentation are secondary to pulmonary insults such as infection. However, this study concentrates on the surgical approach to these lesions independent of etiological complexities, and we chose to apply the most up-to-date classification system of Stocker and colleagues [7], which enabled classification of all cystic lung lesions seen in the context of this study. We are not entirely clear for the small number of bronchogenic cysts. There may have been distorting influences in our referral routes; some patients with asymptomatic bronchogenic cysts have gone elsewhere and only the sick, symptomatic patients came to our institution. It may be also that our incidence is true and other reports inaccurately included other cysts in bronchogenic cysts.
Although all surgeons would recommend resection of lung cysts in symptomatic patients, the situation in asymptomatic patients is less clear. First, routine prenatal ultrasound examination now not infrequently results in the detection of congenital cystic lung lesions. Although this often causes alarm, it is unusual for the cyst to cause difficulties soon after birth and the majority can be observed and dealt with electively [10–12]. However, the timing of operation in asymptomatic patients, especially when after diagnosis in utero, is not well delineated.
In our experience, as the child grows, the technical problems of anesthesia, operation, and postoperative care lessen. This has to be balanced against the ability of infants and very young children to replace lost lung parenchyma, as the potential for alveolar growth recedes with age, and is lost completely by 5 to 7 years. In our practice we have chosen to delay operation until the child is around 2 years of age if there is nothing to indicate that their development is being retarded by the cystic abnormality. Although there is no controlled comparative study, the relative lack of postoperative morbidity indicates that this is a reasonable compromise.
In asymptomatic patients older than 2 years of age, we would argue for the routine excision of all asymptomatic cases on the grounds that cysts rarely involute [10] and complications not infrequently arise, sometimes requiring urgent and more extensive operation. These complications may not arise until adult life and there is no large study showing the incidence of late complications in patients with asymptomatic lung cysts. However, respiratory embarrassment, rupture into pleural space, compression atelectasis of surrounding lung, and infection or hemorrhage into the cyst are all described and can both increase the risk of operation and lead to more extensive resections (Figs 1 and 2)
[12].
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Fig 1. Chest roentgenogram showing an infected cyst with air–fluid level in the left upper lobe. The patient complained of repeated respiratory infections and flulike symptoms.
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Fig 2. Computed tomographic scan of the same patient as in Fig 1. A large cyst was found intraoperatively, which proved to be a cystic adenomatoid malformation.
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It should, however, be noted that neoplastic lesions can also present in relation to pulmonary cysts, either arising in association with indolent tumors, or in a preexistent cyst or being cystic de novo [13–18]. All of these scenarios were found in our cohort, with three examples of indolent tumors causing cystic changes, two CAM containing bronchioloalveolar carcinomas and 1 patient later presenting with a pleuropulmonary blastoma. Elective early resection of cysts at an early stage would therefore reduce the likelihood of tumor dissemination.
Our study also shows that the extent of resection required is primarily influenced by the anatomic extent of the pathology, which is frequently only finalized at thoracotomy when the abnormal anatomy can be directly viewed. We found that simple cysts can usually be resected without loss of any significant functional lung tissue unless long-term compression atelectasis or superimposed infections have caused adjacent parenchymal abnormalities. Patients with CAM might require more extensive resections, but we believe that conservative resection should be undertaken whenever possible.
Although studies have shown high rates of major complications with segmental resections, particularly recurrent air leaks leading to lobectomy [4, 11], we have experienced this in only 1 patient. Segmental resection was accomplished without complication in 4 patients. We believe that the possibility of prolonged, postoperative air leak should not discourage surgeons from attempting conservative resections whenever this proves feasible.
Several studies have reported the presence of associated cardiac or other congenital anomalies emphasizing the importance of systematic preoperative examination [11]. In our pediatric group associated anomalies were found in 30% of patients with half of them being cardiac in nature.
In conclusion, although surgical intervention for pulmonary cysts in infants and young children carries a low risk, elective operation at or after 2 years of age appears to be a reasonable compromise for asymptomatic cases, in the absence of concerns regarding malignancy. After this age, the balance of available evidence supports the conservative surgical excision of symptomatic pulmonary cysts. In asymptomatic cases routine operation might be an overstatement due to lack of substantial data; however, complications may lead to more complex operation. In addition, surgical management will also pick up the rare cases associated with neoplasia that are not identified with preoperative investigations.
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References
|
|---|
-
Shamji F.M. Classification of cystic and bullous lung disease. Chest surgery clinics of North America 1995;5:701-716.[Medline]
-
Hernanz-Schulman M. Cysts, and cystlike lesions of the lung. Radiol Clin N Am 1993;31:631-643.[Medline]
-
Cheney G., Garland L.H. Pulmonary pneumocyst. Report of enormous solitary cyst in healthy adult female. Am J Med Sci 1938;196:699-703.
-
Buntain W.L., Isaacs H., Payne V.C., Lindesmith G.G., Rosenkrantz J.G. Lobar emphysema, cystic adenomatoid malformation, pulmonary sequestration, and bronchogenic cyst in infancy and childhood: a clinical group. J Pediatr Surg 1974;9:85-93.[Medline]
-
Soosay G.N., Baudouin S.V., Hanson P.J.V., et al. Symptomatic cysts in otherwise normal lungs of children and adults. Histopathology 1992;20:517-522.[Medline]
-
Papagiannopoulos K., Sheppard M., Bush A.P., Goldstraw P. Pleuropulmonary blastoma: is prophylactic resection of congenital lung cysts effective?. Ann Thorac Surg 2001;72:604-605.[Abstract/Free Full Text]
-
Stocker J.T., Madewell J.E., Drake R.M. Congenital cystic adenomatoid malformation of the lung. Hum Pathol 1977;8:155-171.[Medline]
-
Schenck S. Congenital cystic disease of the lungs. A clinicopathologic study. AJR 1936;35:604-629.
-
Cooke F.N., Blades B. Cystic disease of the lungs. J Thorac Surg 1952;23:546-549.
-
Dell’Agnola C.A., Tadini B., Mosca F., et al. Advantages of prenatal diagnosis and early surgery for congenital cystic disease of the lung. J Perinat Med 1996;24:621-631.[Medline]
-
Bogers A.J.J., Hazebroek F.W., Bos E. surgical treatment of congenital bronchopulmonary disease in children. Eur J Cardio-Thorac Surg 1993;7:117-120.[Abstract]
-
Hutchin P. Congenital cystic disease of the lung. Rev Surg 1971;28:79-87.[Medline]
-
Granata C., Gambini C., Balducci T., et al. Bronchioalveolar carcinoma arising in congenital cystic adenomatoid malformation in a child: a case report and review on malignancies originating in congenital cystic adenomatoid malformation. Pediat Pulm 1998;25:62-66.
-
Ribet M., Copin M.C., Soots J., et al. Bronchioalveolar carcinoma and congenital cystic adenomatoid malformation. Ann Thorac Surg 1995;60:1126-1128.[Abstract/Free Full Text]
-
Endo C., Imai T., Nakagawa H., Ebina A., et al. Bronchioalveolar carcinoma arising in a bronchogenic cyst. Ann Thorac Surg 2000;69:933-935.[Abstract/Free Full Text]
-
Sheffield E.A., Addis B.J., Corrin B., et al. Epithelial hyperplasia and malignant change in congenital lung cysts. J Clin Pathol 1987;40:612-614.[Abstract/Free Full Text]
-
Benjamin D.R., Cahill J.L. Bronchioalveolar carcinoma of the lung and congenital cystic adenomatoid malformation. Am J Clin Pathol 1991;95:889-892.[Medline]
-
Priest J.R. Pleuropulmonary blastoma: a clinicopathologic study of 50 cases. Cancer 1997;80:147-161.[Medline]
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Abstract
Introduction
