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Congenital lobar emphysema(CLE)

MedGen UID:
120557
•Concept ID:
C0265797
•
Congenital Abnormality
Synonyms: CLE; Congenital emphysema
SNOMED CT: Congenital lobar emphysema (66987001); CLE - Congenital lobar emphysema (66987001); Congenital emphysema (47895001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
HPO: HP:0033255
Monarch Initiative: MONDO:0007536
OMIM®: 130710
Orphanet: ORPHA1928

Definition

A congenital malformation characterized by an overdistended segment of lung, affecting an party of a lobe or the entire one. It results in progressive overinflation of one or more lobes. [from HPO]

Clinical features

From HPO
Respiratory distress
MedGen UID:
96907
•Concept ID:
C0476273
•
Sign or Symptom
Respiratory distress is objectively observable as the physical or emotional consequences from the experience of dyspnea. The physical presentation of respiratory distress is generally referred to as labored breathing, while the sensation of respiratory distress is called shortness of breath or dyspnea.
See: Feature record | Search on this feature
Bronchial cartilage hypoplasia
MedGen UID:
870575
•Concept ID:
C4025024
•
Finding
See: Feature record | Search on this feature

Conditions with this feature

Cardiac malformation, cleft lip/palate, microcephaly, and digital anomalies
MedGen UID:
318752
•Concept ID:
C1832950
•
Disease or Syndrome
See: Condition Record
Cardiac malformation, cleft lip/palate, microcephaly, and digital anomalies

Professional guidelines

PubMed

A clinical guideline for structured assessment of CT-imaging in congenital lung abnormalities.
Hermelijn SM, Elders BBLJ, Ciet P, Wijnen RMH, Tiddens HAWM, Schnater JM
Paediatr Respir Rev 2021 Mar;37:80-88. Epub 2020 Feb 20 doi: 10.1016/j.prrv.2019.12.004. PMID: 32178987
Congenital lobar emphysema: diagnosis and treatment options.
Demir OF, Hangul M, Kose M
Int J Chron Obstruct Pulmon Dis 2019;14:921-928. Epub 2019 May 1 doi: 10.2147/COPD.S170581. PMID: 31118601Free PMC Article
Prenatal presentation and postnatal management of congenital thoracic malformations.
Bush A
Early Hum Dev 2009 Nov;85(11):679-84. Epub 2009 Sep 16 doi: 10.1016/j.earlhumdev.2009.08.056. PMID: 19758773

Recent clinical studies

Etiology

High comorbidity rates in congenital lobar emphysema and the effect on clinical presentation.
Gatt D, Lapidus-Krol E, Chiu PPL
Eur J Pediatr 2024 Oct;183(10):4573-4577. Epub 2024 Jul 13 doi: 10.1007/s00431-024-05684-3. PMID: 39001901
Congenital Lobar Emphysema: Perioperative Evaluation and Management.
Elsawy Abd Elaziz M, Gaber Elsayed M, Ahmed El-Hag Aly M
Heart Surg Forum 2021 Jun 11;24(3):E517-E521. doi: 10.1532/hsf.3823. PMID: 34173742
Congenital lobar emphysema: diagnosis and treatment options.
Demir OF, Hangul M, Kose M
Int J Chron Obstruct Pulmon Dis 2019;14:921-928. Epub 2019 May 1 doi: 10.2147/COPD.S170581. PMID: 31118601Free PMC Article
Long-term outcomes of congenital lung malformations.
Hall NJ, Stanton MP
Semin Pediatr Surg 2017 Oct;26(5):311-316. Epub 2017 Sep 9 doi: 10.1053/j.sempedsurg.2017.09.001. PMID: 29110827
Congenital lobar emphysema.
Keith HH
Pediatr Ann 1977 Jul;6(7):34, 36-41. doi: 10.3928/0090-4481-19770701-07. PMID: 882286

Diagnosis

Congenital Pulmonary Airway Malformations With a Reconsideration and Current Perspective on the Stocker Classification.
Dehner LP, Schultz KAP, Hill DA
Pediatr Dev Pathol 2023 May-Jun;26(3):241-249. Epub 2023 Feb 21 doi: 10.1177/10935266221146823. PMID: 37334833
Management of Congenital Lung Malformations.
Hegde BN, Tsao K, Hirose S
Clin Perinatol 2022 Dec;49(4):907-926. doi: 10.1016/j.clp.2022.08.003. PMID: 36328607
Congenital lung lesions.
Zobel M, Gologorsky R, Lee H, Vu L
Semin Pediatr Surg 2019 Aug;28(4):150821. Epub 2019 Jul 18 doi: 10.1053/j.sempedsurg.2019.07.004. PMID: 31451177
Congenital lobar emphysema: diagnosis and treatment options.
Demir OF, Hangul M, Kose M
Int J Chron Obstruct Pulmon Dis 2019;14:921-928. Epub 2019 May 1 doi: 10.2147/COPD.S170581. PMID: 31118601Free PMC Article
Congenital Lung Malformations.
Palla J, Sockrider MM
Pediatr Ann 2019 Apr 1;48(4):e169-e174. doi: 10.3928/19382359-20190326-02. PMID: 30986318

Therapy

Congenital lobar emphysema: diagnosis and treatment options.
Demir OF, Hangul M, Kose M
Int J Chron Obstruct Pulmon Dis 2019;14:921-928. Epub 2019 May 1 doi: 10.2147/COPD.S170581. PMID: 31118601Free PMC Article
Gene expression profiling reveals differential patterns between microcystic congenital cystic adenomatoid malformation and congenital lobar emphysema.
Hong C, Deng H, Li M, Zhou WP, Tang J, Xia B, Yu G, Zhang L
Early Hum Dev 2019 Jan;128:77-80. Epub 2018 Dec 22 doi: 10.1016/j.earlhumdev.2018.12.014. PMID: 30583279
Perioperative Complications of Video-Assisted Thoracoscopic Pulmonary Procedures in Neonates and Infants.
Zoeller C, Ure BM, Dingemann J
Eur J Pediatr Surg 2018 Apr;28(2):163-170. Epub 2018 Mar 6 doi: 10.1055/s-0038-1636917. PMID: 29510429
Congenital lobar emphysema: diagnostic and therapeutic challenges.
Mulvany JJ, Weatherall A, Charlton A, Selvadurai H
BMJ Case Rep 2016 Jun 22;2016 doi: 10.1136/bcr-2016-214994. PMID: 27335360Free PMC Article
Anesthetic Management in Children with Congenital Lobar Emphysema.
Celik M, Dostbil A, Aksoy M, Alici HA, Dogan N, Ince I, Aydin Y, Ahiskalioglu A
Acta Chir Belg 2015 Jul-Aug;115(4):279-83. doi: 10.1080/00015458.2015.11681112. PMID: 26324029

Prognosis

Congenital Lobar Emphysema: Perioperative Evaluation and Management.
Elsawy Abd Elaziz M, Gaber Elsayed M, Ahmed El-Hag Aly M
Heart Surg Forum 2021 Jun 11;24(3):E517-E521. doi: 10.1532/hsf.3823. PMID: 34173742
Congenital lung lesions.
Zobel M, Gologorsky R, Lee H, Vu L
Semin Pediatr Surg 2019 Aug;28(4):150821. Epub 2019 Jul 18 doi: 10.1053/j.sempedsurg.2019.07.004. PMID: 31451177
Congenital lobar emphysema: diagnosis and treatment options.
Demir OF, Hangul M, Kose M
Int J Chron Obstruct Pulmon Dis 2019;14:921-928. Epub 2019 May 1 doi: 10.2147/COPD.S170581. PMID: 31118601Free PMC Article
Asymptomatic congenital lung malformations.
Laberge JM, Puligandla P, Flageole H
Semin Pediatr Surg 2005 Feb;14(1):16-33. doi: 10.1053/j.sempedsurg.2004.10.022. PMID: 15770585
Congenital lobar emphysema.
Keith HH
Pediatr Ann 1977 Jul;6(7):34, 36-41. doi: 10.3928/0090-4481-19770701-07. PMID: 882286

Clinical prediction guides

Congenital Pulmonary Airway Malformations With a Reconsideration and Current Perspective on the Stocker Classification.
Dehner LP, Schultz KAP, Hill DA
Pediatr Dev Pathol 2023 May-Jun;26(3):241-249. Epub 2023 Feb 21 doi: 10.1177/10935266221146823. PMID: 37334833
Congenital Lobar Emphysema: Perioperative Evaluation and Management.
Elsawy Abd Elaziz M, Gaber Elsayed M, Ahmed El-Hag Aly M
Heart Surg Forum 2021 Jun 11;24(3):E517-E521. doi: 10.1532/hsf.3823. PMID: 34173742
Congenital lobar emphysema: diagnosis and treatment options.
Demir OF, Hangul M, Kose M
Int J Chron Obstruct Pulmon Dis 2019;14:921-928. Epub 2019 May 1 doi: 10.2147/COPD.S170581. PMID: 31118601Free PMC Article
Long-term outcomes of congenital lung malformations.
Hall NJ, Stanton MP
Semin Pediatr Surg 2017 Oct;26(5):311-316. Epub 2017 Sep 9 doi: 10.1053/j.sempedsurg.2017.09.001. PMID: 29110827
Asymptomatic congenital lung malformations.
Laberge JM, Puligandla P, Flageole H
Semin Pediatr Surg 2005 Feb;14(1):16-33. doi: 10.1053/j.sempedsurg.2004.10.022. PMID: 15770585

Recent systematic reviews

Perioperative Complications of Video-Assisted Thoracoscopic Pulmonary Procedures in Neonates and Infants.
Zoeller C, Ure BM, Dingemann J
Eur J Pediatr Surg 2018 Apr;28(2):163-170. Epub 2018 Mar 6 doi: 10.1055/s-0038-1636917. PMID: 29510429

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