Congenital lobar emphysema
- Christopher M Oermann, MD
Christopher M Oermann, MD
- Professor, Department of Pediatrics
- University of Missouri-Kansas City School of Medicine
- Section Editors
- Joseph A Garcia-Prats, MD
Joseph A Garcia-Prats, MD
- Section Editor — Neonatology
- Professor of Pediatrics
- Baylor College of Medicine
- Gregory Redding, MD
Gregory Redding, MD
- Section Editor — Pediatric Pulmonology
- Professor of Pediatrics
- University of Washington School of Medicine
Congenital lobar emphysema (CLE), also known as congenital alveolar overdistension, is a developmental anomaly of the lower respiratory tract that is characterized by hyperinflation of one or more of the pulmonary lobes [1,2]. Other terms for CLE include congenital lobar overinflation and infantile lobar emphysema [3-5].
Congenital lobar emphysema (CLE) is a rare congenital malformation with a prevalence of 1 in 20,000 to 1 in 30,000 . CLE was diagnosed in 10 of 70 patients with congenital malformations of the lung seen from 1970 to 1995 at Children's National Medical Center in Washington, DC . Depending upon the pattern of referrals, other tertiary medical centers may treat one or two cases per year [3,8].
Progressive lobar hyperinflation is likely the final common pathway that results from a variety of disruptions in bronchopulmonary development. These result from abnormal interactions between embryonic endodermal and mesodermal components of the lung. Disturbances may lead to changes in the number of airways or alveoli or alveolar size . However, a definitive causative agent cannot be identified in approximately 50 percent of cases .
The most frequently identified cause of congenital lobar emphysema (CLE) is obstruction of the developing airway, which occurs in 25 percent of cases . Airway obstruction can be intrinsic or extrinsic, with the former more common. This leads to the creation of a "ball-valve" mechanism in which a greater volume of air enters the affected lobe during inspiration than leaves during expiration, producing air trapping.To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Kravitz RM. Congenital malformations of the lung. Pediatr Clin North Am 1994; 41:453.
- Stanton M, Davenport M. Management of congenital lung lesions. Early Hum Dev 2006; 82:289.
- Stocker JT, Drake RM, Madewell JE. Cystic and congenital lung disease in the newborn. Perspect Pediatr Pathol 1978; 4:93.
- Landing BH, Dixon LG. Congenital malformations and genetic disorders of the respiratory tract (larynx, trachea, bronchi, and lungs). Am Rev Respir Dis 1979; 120:151.
- Hernanz-Schulman M. Cysts and cystlike lesions of the lung. Radiol Clin North Am 1993; 31:631.
- Thakral CL, Maji DC, Sajwani MJ. Congenital lobar emphysema: experience with 21 cases. Pediatr Surg Int 2001; 17:88.
- Schwartz MZ, Ramachandran P. Congenital malformations of the lung and mediastinum--a quarter century of experience from a single institution. J Pediatr Surg 1997; 32:44.
- Nuchtern JG, Harberg FJ. Congenital lung cysts. Semin Pediatr Surg 1994; 3:233.
- Tander B, Yalçin M, Yilmaz B, et al. Congenital lobar emphysema: a clinicopathologic evaluation of 14 cases. Eur J Pediatr Surg 2003; 13:108.
- Olutoye OO, Coleman BG, Hubbard AM, Adzick NS. Prenatal diagnosis and management of congenital lobar emphysema. J Pediatr Surg 2000; 35:792.
- Riedlinger WF, Vargas SO, Jennings RW, et al. Bronchial atresia is common to extralobar sequestration, intralobar sequestration, congenital cystic adenomatoid malformation, and lobar emphysema. Pediatr Dev Pathol 2006; 9:361.
- Fowler DJ, Gould SJ. The pathology of congenital lung lesions. Semin Pediatr Surg 2015; 24:176.
- DeLuca FG, Wesselhoeft CW. Surgically treatable causes of neonatal respiratory distress. Clin Perinatol 1978; 5:377.
- Shanti CM, Klein MD. Cystic lung disease. Semin Pediatr Surg 2008; 17:2.
- Karnak I, Senocak ME, Ciftci AO, Büyükpamukçu N. Congenital lobar emphysema: diagnostic and therapeutic considerations. J Pediatr Surg 1999; 34:1347.
- Stigers KB, Woodring JH, Kanga JF. The clinical and imaging spectrum of findings in patients with congenital lobar emphysema. Pediatr Pulmonol 1992; 14:160.
- Critchley PS, Forrester-Wood CP, Ridley PD. Adult congenital lobar emphysema in pregnancy. Thorax 1995; 50:909.
- Mei-Zahav M, Konen O, Manson D, Langer JC. Is congenital lobar emphysema a surgical disease? J Pediatr Surg 2006; 41:1058.
- Markowitz RI, Mercurio MR, Vahjen GA, et al. Congenital lobar emphysema. The roles of CT and V/Q scan. Clin Pediatr (Phila) 1989; 28:19.
- Pardes JG, Auh YH, Blomquist K, et al. CT diagnosis of congenital lobar emphysema. J Comput Assist Tomogr 1983; 7:1095.
- Rusakow LS, Khare S. Radiographically occult congenital lobar emphysema presenting as unexplained neonatal tachypnea. Pediatr Pulmonol 2001; 32:246.
- Ankermann T, Oppermann HC, Engler S, et al. Congenital masses of the lung, cystic adenomatoid malformation versus congenital lobar emphysema: prenatal diagnosis and implications for postnatal treatment. J Ultrasound Med 2004; 23:1379.
- Babu R, Kyle P, Spicer RD. Prenatal sonographic features of congenital lobar emphysema. Fetal Diagn Ther 2001; 16:200.
- Liu YP, Shih SL. Congenital lobar emphysema: appearance on fetal MRI. Pediatr Radiol 2008; 38:1264.
- Quinton AE, Smoleniec JS. Congenital lobar emphysema--the disappearing chest mass: antenatal ultrasound appearance. Ultrasound Obstet Gynecol 2001; 17:169.
- Usui N, Kamata S, Sawai T, et al. Outcome predictors for infants with cystic lung disease. J Pediatr Surg 2004; 39:603.
- Dillman JR, Sanchez R, Ladino-Torres MF, et al. Expanding upon the unilateral hyperlucent hemithorax in children. Radiographics 2011; 31:723.
- Wasilewska E, Lee EY, Eisenberg RL. Unilateral hyperlucent lung in children. AJR Am J Roentgenol 2012; 198:W400.
- Powers JE, Counselman FL. Congenital lobar emphysema: tube thoracostomy not the treatment. Pediatr Emerg Care 2005; 21:760.
- Lucaya J, Gartner S, García-Peña P, et al. Spectrum of manifestations of Swyer-James-MacLeod syndrome. J Comput Assist Tomogr 1998; 22:592.
- Trimis G, Theodoridou M, Mostrou G, Kakavakis K. Swyer-James (MacLeod's) syndrome following pertussis infection in an infant. Scand J Infect Dis 2003; 35:197.
- Fregonese L, Girosi D, Battistini E, et al. Clinical, physiologic, and roentgenographic changes after pneumonectomy in a boy with Macleod/Swyer-James syndrome and bronchiectasis. Pediatr Pulmonol 2002; 34:412.
- da Silva PS, Lopes R, Neto HM. Swyer-James-MacLeod syndrome in a surgically treated child: a case report and brief literature review. J Pediatr Surg 2012; 47:e17.
- Chen IC, Chen YW, Lin SH, et al. Usefulness of combination of pulmonary ventilation and perfusion scintigraphy on the diagnosis of children with unilateral hyperlucent lung. Nucl Med Commun 2011; 32:1052.
- Arslan N, Ilgan S, Ozkan M, et al. Utility of ventilation and perfusion scan in the diagnosis of young military recruits with an incidental finding of hyperlucent lung. Nucl Med Commun 2001; 22:525.
- Wang JY, Chen KY, Chang YC, et al. Swyer-James syndrome complicated by lung abscess. J Formos Med Assoc 2000; 99:252.
- Soni R, Barnes D. Macleod's syndrome presenting with spontaneous pneumothorax. Respirology 1999; 4:275.
- Koyama T, Osada H, Kitanaka Y, et al. Surgically treated Swyer-James syndrome. Jpn J Thorac Cardiovasc Surg 2001; 49:671.
- Singh R, Davenport M. The argument for operative approach to asymptomatic lung lesions. Semin Pediatr Surg 2015; 24:187.
- Stanton M. The argument for a non-operative approach to asymptomatic lung lesions. Semin Pediatr Surg 2015; 24:183.
- Duan M, Wang L, Cao Y, et al. Results of surgical treatment of congenital cystic lung disease. Thorac Cardiovasc Surg 2005; 53:61.
- Shanmugam G, MacArthur K, Pollock JC. Congenital lung malformations--antenatal and postnatal evaluation and management. Eur J Cardiothorac Surg 2005; 27:45.
- Ayed AK, Owayed A. Pulmonary resection in infants for congenital pulmonary malformation. Chest 2003; 124:98.
- Choudhury SR, Chadha R, Mishra A, et al. Lung resections in children for congenital and acquired lesions. Pediatr Surg Int 2007; 23:851.
- Truitt AK, Carr SR, Cassese J, et al. Perinatal management of congenital cystic lung lesions in the age of minimally invasive surgery. J Pediatr Surg 2006; 41:893.
- Ozçelik U, Göçmen A, Kiper N, et al. Congenital lobar emphysema: evaluation and long-term follow-up of thirty cases at a single center. Pediatr Pulmonol 2003; 35:384.
- Eigen H, Lemen RJ, Waring WW. Congenital lobar emphysema: long-term evaluation of surgically and conservatively treated children. Am Rev Respir Dis 1976; 113:823.
- Calzolari F, Braguglia A, Valfrè L, et al. Outcome of infants operated on for congenital pulmonary malformations. Pediatr Pulmonol 2016; 51:1367.
- Eber E. Antenatal diagnosis of congenital thoracic malformations: early surgery, late surgery, or no surgery? Semin Respir Crit Care Med 2007; 28:355.