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Pediatrics

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.

  1. 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].
  2. 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].
  3. 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?
Congenital Lobar Emphysema (CLE) and Congenital Lobar Overinflation (CLO) are two names for the exact same condition. Some doctors prefer CLO because it better describes the trapped air without the negative associations of adult emphysema, which is caused by environmental factors.
What causes the air to get trapped in my baby's lung?
In most cases, the airway leading to the lung has weak cartilage. This creates a one-way valve effect where air can enter the lung when the baby breathes in, but the airway collapses when they breathe out, trapping the air inside the lobe.
Is congenital lobar emphysema hereditary?
No, CLE is typically a sporadic, developmental anomaly that happens by chance during pregnancy. It is not considered to be strongly hereditary or passed down from parents.
How is congenital lobar emphysema treated?
The standard treatment for infants with symptoms like fast breathing or feeding difficulties is a lobectomy, a surgical procedure to remove the overinflated lobe. For babies with mild or no symptoms, doctors may recommend a period of careful observation known as watchful waiting.
Will removing a lung lobe affect my baby's breathing long-term?
Infant lungs are remarkably resilient. When an overinflated lobe is removed, the remaining healthy lung tissue typically expands to fill the space. This allows for normal lung function as the child grows.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Which lobe of my baby's lung is affected, and is there any evidence of other issues like heart malformations?
  2. 2.Is my baby's CLE caused by weak cartilage or is the cause unknown?
  3. 3.Based on the current size of the overinflation, do you recommend surgery now or a period of 'watchful waiting'?
  4. 4.If we monitor the condition, what specific red flags should I look for at home?
  5. 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)
  1. 1

    Clinical and surgical aspects of congenital lobar over-inflation: a single center retrospective study.

    Abdel-Bary M, Abdel-Naser M, Okasha A, et al.

    Journal of cardiothoracic surgery 2020; (15(1)):102 doi:10.1186/s13019-020-01145-8.

    PMID: 32429981
  2. 2

    Congenital Lobar Emphysema in Children: Case Series.

    Benbouziane N, Larda L, Pongo C, et al.

    Cureus 2023; (15(11)):e49416 doi:10.7759/cureus.49416.

    PMID: 38149169
  3. 3

    Congenital Lobar Emphysema Presenting as Respiratory Distress in a Newborn.

    Choudhury S, Dubey S, Kumar R

    Cureus 2023; (15(6)):e40545 doi:10.7759/cureus.40545.

    PMID: 37465796
  4. 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. 5

    Congenital Lobar Overinflation: A Rare Enigmatic Lung Lesion on Prenatal Ultrasound and Magnetic Resonance Imaging.

    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. 6

    Laryngomalacia, Tracheomalacia and Bronchomalacia.

    Hysinger EB

    Current problems in pediatric and adolescent health care 2018; (48(4)):113-118 doi:10.1016/j.cppeds.2018.03.002.

    PMID: 29622320
  7. 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. 8

    Congenital Lung Malformations: Clinical and Functional Respiratory Outcomes after Surgery.

    Farolfi A, Ghezzi M, Calcaterra V, et al.

    Children (Basel, Switzerland) 2022; (9(12)) doi:10.3390/children9121881.

    PMID: 36553325
  9. 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. 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. 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. 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. 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. 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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