Understanding Congenital Lobar Emphysema (CLE)
At a Glance
Congenital lobar emphysema (CLE) is a rare infant lung condition where weak airways trap air in a single lung lobe. The primary treatment is a highly successful surgery to remove the overinflated lobe, allowing the remaining healthy lung tissue to expand and function normally as the baby grows.
Receiving a diagnosis for your newborn or infant can be overwhelming, especially when it involves something as vital as their breathing. Congenital Lobar Emphysema (CLE) is a rare condition, but it is one that pediatric specialists understand well and can treat effectively [1][2].
While the name sounds complex, the most important thing to know is that your child’s lung tissue is healthy; the issue is simply that air is getting “stuck” in one part of the lung [3].
Understanding the Name: CLE vs. CLO
You may hear your doctors use two different names for this condition: Congenital Lobar Emphysema (CLE) or Congenital Lobar Overinflation (CLO).
- Lobar refers to a “lobe” of the lung.
- Emphysema or Overinflation describes the way the lung becomes too full of air.
Medical experts currently view these as two names for the exact same condition [4][1]. Some doctors prefer “overinflation” because it more accurately describes the air trapping without the associations of adult emphysema (which is caused by smoking or environmental factors) [1][5].
How It Happens: The “One-Way Valve”
In most cases of CLE, the problem isn’t the lung itself, but the “pipe” (the bronchus) leading to it.
- Weak Cartilage: Some babies are born with bronchial cartilaginous hypoplasia, which is a way of saying the rings of cartilage that keep the airway open are too weak or soft [3][6].
- The One-Way Valve: When your baby breathes in, the airway stays open enough to let air into the lung lobe. However, when they breathe out, the weak airway collapses or “kinks” [6].
- Air Trapping: This creates a “one-way valve” effect—air goes in, but it can’t easily get back out [3]. This causes that specific lobe of the lung to overinflate like a balloon that is being pumped up but never deflated.
Finding Stability in the Facts
It is natural to feel anxious when your child has a lung malformation. These facts can help ground your perspective:
- It is usually a spontaneous event: One of the first questions parents ask is if they passed this on. CLE is typically a sporadic, developmental anomaly that occurs by chance during pregnancy, and is not strongly hereditary [7].
- It is rare but recognized: CLE is a rare condition, representing about 10% to 11% of all congenital lung malformations [7][8]. Because it is a known entity, pediatric hospitals have established protocols for managing it.
- The treatment is highly successful: For infants who need surgery, a lobectomy (removing the overinflated lobe) is the standard treatment [2]. This procedure is generally associated with excellent outcomes [9][10].
- The rest of the lung adapts: The human lung is remarkably resilient in infancy. When one lobe is removed, the remaining healthy lobes often expand to fill the space, allowing for normal lung function as the child grows [9].
What to Expect Next
Diagnosis is usually confirmed using a CT scan, which gives doctors a detailed “map” of the lungs [11][12]. If your baby has severe symptoms, such as fast breathing or difficulty feeding, surgery is often performed soon after diagnosis to prevent complications [2][13]. If your baby has no symptoms or very mild ones, your doctor might suggest “conservative management,” which means watching the baby closely and delaying surgery unless it becomes necessary [13][14].
Common questions in this guide
What is the difference between CLE and CLO?
What causes the air to get trapped in my baby's lung?
Is congenital lobar emphysema hereditary?
How is congenital lobar emphysema treated?
Will removing a lung lobe affect my baby's breathing long-term?
Questions to Ask Your Doctor
Curated prompts to bring to your next appointment.
- 1.Which lobe of my baby's lung is affected, and is there any evidence of other issues like heart malformations?
- 2.Is my baby's CLE caused by weak cartilage or is the cause unknown?
- 3.Based on the current size of the overinflation, do you recommend surgery now or a period of 'watchful waiting'?
- 4.If we monitor the condition, what specific red flags should I look for at home?
- 5.How many surgeries for rare lung malformations has this team performed in the last year?
Questions For You
Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.
References
References (14)
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Abdel-Bary M, Abdel-Naser M, Okasha A, et al.
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PMID: 32429981 - 2
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Benbouziane N, Larda L, Pongo C, et al.
Cureus 2023; (15(11)):e49416 doi:10.7759/cureus.49416.
PMID: 38149169 - 3
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Choudhury S, Dubey S, Kumar R
Cureus 2023; (15(6)):e40545 doi:10.7759/cureus.40545.
PMID: 37465796 - 4
Congenital Lobar Hyperinflation - A rare anomaly misdiagnosed as bronchiolitis: A case report.
Mehdi SM, Baig U, Huma S
JPMA. The Journal of the Pakistan Medical Association 2020; (70(1)):197-199 doi:10.5455/JPMA.292068.
PMID: 31954057 - 5
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Oliver ER, DeBari SE, Horii SC, et al.
Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine 2019; (38(5)):1229-1239 doi:10.1002/jum.14801.
PMID: 30208226 - 6
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Current problems in pediatric and adolescent health care 2018; (48(4)):113-118 doi:10.1016/j.cppeds.2018.03.002.
PMID: 29622320 - 7
Current operative management of congenital lobar emphysema in children: A report from the Midwest Pediatric Surgery Consortium.
Kunisaki SM, Saito JM, Fallat ME, et al.
Journal of pediatric surgery 2019; (54(6)):1138-1142 doi:10.1016/j.jpedsurg.2019.02.043.
PMID: 30898401 - 8
Congenital Lung Malformations: Clinical and Functional Respiratory Outcomes after Surgery.
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Children (Basel, Switzerland) 2022; (9(12)) doi:10.3390/children9121881.
PMID: 36553325 - 9
Congenital lobar emphysema: Thoracotomy versus minimally invasive surgery.
Bawazir OA
Annals of thoracic medicine 2020; (15(1)):21-25 doi:10.4103/atm.ATM_203_19.
PMID: 32002043 - 10
Persistent respiratory distress in a neonate: a diagnostic dilemma.
Shukla A, Pandita A, Gupta G, Mishra N
BMJ case reports 2018; (2018()) doi:10.1136/bcr-2017-222290.
PMID: 29666078 - 11
A three month old infant with severe respiratory distress.
Ahmed Z, Sadiq S, Quraishi S, Mirza S
JPMA. The Journal of the Pakistan Medical Association 2024; (74(4)):797-799 doi:10.47391/JPMA.9553.
PMID: 38751283 - 12
Congenital Lobar Emphysema: Perioperative Evaluation and Management.
Elsawy Abd Elaziz M, Gaber Elsayed M, Ahmed El-Hag Aly M
The heart surgery forum 2021; (24(3)):E517-E521 doi:10.1532/hsf.3823.
PMID: 34173742 - 13
Surgery versus conservative management in congenital lobar emphysema: follow up and indicators for surgery.
Tuğcu GD, Polat SE, Soydaş SSA, et al.
Pediatric surgery international 2022; (38(4)):559-568 doi:10.1007/s00383-022-05086-2.
PMID: 35212777 - 14
A rare case of congenital lobar emphysema diagnosed in a 13-year-old boy.
Matuszczak E, Dębek W
Kardiochirurgia i torakochirurgia polska = Polish journal of cardio-thoracic surgery 2020; (17(4)):205-207 doi:10.5114/kitp.2020.102331.
PMID: 33552186
This page is for educational purposes only and does not replace professional medical advice. Always consult your pediatric pulmonologist or surgeon regarding your child's specific diagnosis and treatment plan.
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