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Tracheal atresia

MedGen UID:
75584
Concept ID:
C0265766
Congenital Abnormality
Synonyms: Congenital atresia of trachea; Congenital Tracheal Atresia; Tracheal Atresia
SNOMED CT: Congenital atresia of trachea (53189005)
 
HPO: HP:0100682

Definition

A congenital absence or considerable underdevelopment of the trachea such that communication between the larynx proximally and the alveoli of the lungs distally is lacking. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTracheal atresia

Conditions with this feature

Lung agenesis-heart defect-thumb anomalies syndrome
MedGen UID:
477585
Concept ID:
C3275954
Disease or Syndrome
The Mardini-Nyhan association comprises uni- or bilateral lung agenesis, complex cardiac defects, particularly total anomalous pulmonary venous return (TAPVR), and thumb abnormalities (summary by Hastings et al., 2009).
Fraser syndrome 3
MedGen UID:
1621907
Concept ID:
C4540040
Disease or Syndrome
Fraser syndrome is an autosomal recessive malformation disorder characterized by cryptophthalmos, syndactyly, and abnormalities of the respiratory and urogenital tract (summary by van Haelst et al., 2008). For a general phenotypic description and a discussion of genetic heterogeneity of Fraser syndrome, see 219000.

Professional guidelines

PubMed

Smith MM, Huang A, Labbé M, Lubov J, Nguyen LHP
Int J Pediatr Otorhinolaryngol 2017 Oct;101:57-64. Epub 2017 Jul 24 doi: 10.1016/j.ijporl.2017.07.028. PMID: 28964311
Hubbard AM, Adzick NS, Crombleholme TM, Coleman BG, Howell LJ, Haselgrove JC, Mahboubi S
Radiology 1999 Jul;212(1):43-8. doi: 10.1148/radiology.212.1.r99jl3143. PMID: 10405718
Sun CC, Grumbach K, DeCosta DT, Meyers CM, Dungan JS
Pediatr Dev Pathol 1999 Mar-Apr;2(2):131-42. doi: 10.1007/s100249900101. PMID: 9949219

Recent clinical studies

Etiology

Aoki H, Miyazaki O, Irahara S, Okamoto R, Tsutsumi Y, Miyasaka M, Sago H, Kanamori Y, Suzuki Y, Morimoto N, Nosaka S
Pediatr Radiol 2021 Oct;51(11):2027-2037. Epub 2021 May 14 doi: 10.1007/s00247-021-05092-x. PMID: 33988754
Hoeltzenbein M, Lehmann ML, Beck E, Dathe K, Schaefer C
Eur J Clin Pharmacol 2021 Jul;77(7):1029-1037. Epub 2021 Jan 26 doi: 10.1007/s00228-020-03066-w. PMID: 33501507Free PMC Article
Lim FY, Crombleholme TM, Hedrick HL, Flake AW, Johnson MP, Howell LJ, Adzick NS
J Pediatr Surg 2003 Jun;38(6):940-5. doi: 10.1016/s0022-3468(03)00128-3. PMID: 12778398
Sun CC, Grumbach K, DeCosta DT, Meyers CM, Dungan JS
Pediatr Dev Pathol 1999 Mar-Apr;2(2):131-42. doi: 10.1007/s100249900101. PMID: 9949219
King SJ, Pilling DW, Walkinshaw S
Pediatr Radiol 1995;25(3):208-10. doi: 10.1007/BF02021538. PMID: 7644306

Diagnosis

Georgescu T, Radoi V, Radulescu M, Ilian A, Toader OD, Pop LG, Bacalbasa N
Medicina (Kaunas) 2021 Nov 16;57(11) doi: 10.3390/medicina57111253. PMID: 34833471Free PMC Article
Aoki H, Miyazaki O, Irahara S, Okamoto R, Tsutsumi Y, Miyasaka M, Sago H, Kanamori Y, Suzuki Y, Morimoto N, Nosaka S
Pediatr Radiol 2021 Oct;51(11):2027-2037. Epub 2021 May 14 doi: 10.1007/s00247-021-05092-x. PMID: 33988754
Smith MM, Huang A, Labbé M, Lubov J, Nguyen LHP
Int J Pediatr Otorhinolaryngol 2017 Oct;101:57-64. Epub 2017 Jul 24 doi: 10.1016/j.ijporl.2017.07.028. PMID: 28964311
Lupi M, Bonetti LR, Trani N, Maccio L, Maiorana A
Pathologica 2009 Dec;101(6):235-9. PMID: 20387710
Berrocal T, Madrid C, Novo S, Gutiérrez J, Arjonilla A, Gómez-León N
Radiographics 2004 Jan-Feb;24(1):e17. Epub 2003 Nov 10 doi: 10.1148/rg.e17. PMID: 14610245

Therapy

Tankam CS, Padmanaban V, Pazniokas J, Lane J, Clark JB, Rizk EB
Childs Nerv Syst 2023 Jan;39(1):255-259. Epub 2022 Oct 25 doi: 10.1007/s00381-022-05717-y. PMID: 36282305
Hoeltzenbein M, Lehmann ML, Beck E, Dathe K, Schaefer C
Eur J Clin Pharmacol 2021 Jul;77(7):1029-1037. Epub 2021 Jan 26 doi: 10.1007/s00228-020-03066-w. PMID: 33501507Free PMC Article
Merei J, Kotsios C, Hutson JM, Hasthorpe S
J Pediatr Surg 1997 Jan;32(1):12-4. doi: 10.1016/s0022-3468(97)90081-6. PMID: 9021557

Prognosis

Georgescu T, Radoi V, Radulescu M, Ilian A, Toader OD, Pop LG, Bacalbasa N
Medicina (Kaunas) 2021 Nov 16;57(11) doi: 10.3390/medicina57111253. PMID: 34833471Free PMC Article
Krishnamurthy K, Kochiyil J, Poppiti RJ
Fetal Pediatr Pathol 2020 Feb;39(1):45-50. Epub 2019 Jul 17 doi: 10.1080/15513815.2019.1627628. PMID: 31314628
Smith MM, Huang A, Labbé M, Lubov J, Nguyen LHP
Int J Pediatr Otorhinolaryngol 2017 Oct;101:57-64. Epub 2017 Jul 24 doi: 10.1016/j.ijporl.2017.07.028. PMID: 28964311
Hamid-Sowinska A, Ropacka-Lesiak M, Breborowicz GH
Neuro Endocrinol Lett 2011;32(5):623-6. PMID: 22167132
Sun CC, Grumbach K, DeCosta DT, Meyers CM, Dungan JS
Pediatr Dev Pathol 1999 Mar-Apr;2(2):131-42. doi: 10.1007/s100249900101. PMID: 9949219

Clinical prediction guides

Hoeltzenbein M, Lehmann ML, Beck E, Dathe K, Schaefer C
Eur J Clin Pharmacol 2021 Jul;77(7):1029-1037. Epub 2021 Jan 26 doi: 10.1007/s00228-020-03066-w. PMID: 33501507Free PMC Article
Seker IS, Özlü O, Özkan A, Uzun H, Esbah AÜ, Çetin P
Afr J Paediatr Surg 2017 Oct-Dec;14(4):74-78. doi: 10.4103/ajps.AJPS_117_16. PMID: 30688282Free PMC Article
Gaerty K, Thomas JT, Petersen S, Tan E, Kumar S, Gardener G, Armes J
Pediatr Dev Pathol 2016 Mar-Apr;19(2):154-8. Epub 2015 Sep 14 doi: 10.2350/15-08-1685-CR.1. PMID: 26367770
Sun CC, Grumbach K, DeCosta DT, Meyers CM, Dungan JS
Pediatr Dev Pathol 1999 Mar-Apr;2(2):131-42. doi: 10.1007/s100249900101. PMID: 9949219
King SJ, Pilling DW, Walkinshaw S
Pediatr Radiol 1995;25(3):208-10. doi: 10.1007/BF02021538. PMID: 7644306

Recent systematic reviews

Smith MM, Huang A, Labbé M, Lubov J, Nguyen LHP
Int J Pediatr Otorhinolaryngol 2017 Oct;101:57-64. Epub 2017 Jul 24 doi: 10.1016/j.ijporl.2017.07.028. PMID: 28964311

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