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Congenital chylothorax

MedGen UID:
87398
•Concept ID:
C0340014
•
Congenital Abnormality
Synonym: HYDROTHORAX, CONGENITAL
SNOMED CT: Congenital chylothorax (233646003)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
•Concept ID:
CN307042
•
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
See: This record
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Unknown inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0011331
OMIM®: 603523
Orphanet: ORPHA264688

Definition

Congenital chylothorax is a rare, potentially life-threatening neonatal condition characterized by the accumulation of chyle within the pleural space leading to respiratory distress, malnutrition and immunological compromise, either immediately after birth or within the first few weeks of life. Congenital chylothorax is the most common cause of pleural effusion in neonates; it can occur primarily due to developmental anomalies of the lymphatic duct or can be associated with chromosomal anomalies (e.g. Noonan syndrome, Turner syndrome and Down syndrome), hydrops fetalis, mediastinal neuroblastoma and other congenital malformations. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital chylothorax
Follow this link to review classifications for Congenital chylothorax in Orphanet.

Professional guidelines

PubMed

Management of Congenital Chylothorax of the Newborn.
Resch B
Respiration 2022;101(8):795-796. Epub 2022 Jun 20 doi: 10.1159/000525377. PMID: 35724638Free PMC Article
"Open Questions" and Role of Lung Ultrasound in the Management of Congenital Chylothorax.
Perri A, Tana M, Sbordone A, Patti ML, Sattin G, Vento G
Respiration 2022;101(1):16-17. Epub 2021 Nov 23 doi: 10.1159/000520313. PMID: 34814155
Potential role of high-frequency ventilation in the treatment of severe congenital pleural effusion.
Kugelman A, Gonen R, Bader D
Pediatr Pulmonol 2000 May;29(5):404-8. doi: 10.1002/(sici)1099-0496(200005)29:5<404::aid-ppul11>3.0.co;2-4. PMID: 10790254

Recent clinical studies

Etiology

Congenital Chylothorax of the Newborn: A Systematic Analysis of Published Cases between 1990 and 2018.
Resch B, Sever Yildiz G, Reiterer F
Respiration 2022;101(1):84-96. Epub 2021 Sep 1 doi: 10.1159/000518217. PMID: 34515211Free PMC Article
Congenital chylothorax.
Attar MA, Donn SM
Semin Fetal Neonatal Med 2017 Aug;22(4):234-239. Epub 2017 Mar 27 doi: 10.1016/j.siny.2017.03.005. PMID: 28351595
Congenital chylothorax: a prospective nationwide epidemiological study in Germany.
Bialkowski A, Poets CF, Franz AR; Erhebungseinheit für seltene pädiatrische Erkrankungen in Deutschland Study Group
Arch Dis Child Fetal Neonatal Ed 2015 Mar;100(2):F169-72. Epub 2014 Dec 5 doi: 10.1136/archdischild-2014-307274. PMID: 25480460
Outcomes of fetal intervention for primary hydrothorax.
Derderian SC, Trivedi S, Farrell J, Keller RL, Rand L, Goldstein R, Feldstein VA, Hirose S, MacKenzie TC
J Pediatr Surg 2014 Jun;49(6):900-3; discussion 903-4. Epub 2014 Jan 31 doi: 10.1016/j.jpedsurg.2014.01.020. PMID: 24888831
Long-term follow-up of children with congenital chylothorax.
Resch B, Halmer M, Müller WD, Eber E
Eur Respir J 2012 Oct;40(4):1060-2. doi: 10.1183/09031936.00016812. PMID: 23024332

Diagnosis

Pulmonary lymphangiectasia in myotubular myopathy: a novel unrecognized association?
de Carvalho Nunes G, Grenier K, Maedler Kron C, Kitzler T, Helou JE, Rosenblatt DS, Olivier F
Neuromuscul Disord 2022 Jun;32(6):512-515. Epub 2022 May 2 doi: 10.1016/j.nmd.2022.04.010. PMID: 35584999
Congenital chylothorax.
Attar MA, Donn SM
Semin Fetal Neonatal Med 2017 Aug;22(4):234-239. Epub 2017 Mar 27 doi: 10.1016/j.siny.2017.03.005. PMID: 28351595
Outcomes of fetal intervention for primary hydrothorax.
Derderian SC, Trivedi S, Farrell J, Keller RL, Rand L, Goldstein R, Feldstein VA, Hirose S, MacKenzie TC
J Pediatr Surg 2014 Jun;49(6):900-3; discussion 903-4. Epub 2014 Jan 31 doi: 10.1016/j.jpedsurg.2014.01.020. PMID: 24888831
Congenital chylothorax.
Lahmiti S, Elhoudzi J, Aboussad A
ScientificWorldJournal 2009 Jun 12;9:431-4. doi: 10.1100/tsw.2009.62. PMID: 19526181Free PMC Article
Congenital chylothorax: a case study.
Gaede C
Neonatal Netw 2006 Sep-Oct;25(5):371-81. doi: 10.1891/0730-0832.25.5.371. PMID: 16989135

Therapy

Congenital Chylothorax of the Newborn: A Systematic Analysis of Published Cases between 1990 and 2018.
Resch B, Sever Yildiz G, Reiterer F
Respiration 2022;101(1):84-96. Epub 2021 Sep 1 doi: 10.1159/000518217. PMID: 34515211Free PMC Article
Chemical Pleurodesis with Oxytetracycline in Congenital Chylothorax.
Utture A, Kodur V, Mondkar J
Indian Pediatr 2016 Dec 15;53(12):1105-1106. PMID: 28064267
Congenital chylothorax: a prospective nationwide epidemiological study in Germany.
Bialkowski A, Poets CF, Franz AR; Erhebungseinheit für seltene pädiatrische Erkrankungen in Deutschland Study Group
Arch Dis Child Fetal Neonatal Ed 2015 Mar;100(2):F169-72. Epub 2014 Dec 5 doi: 10.1136/archdischild-2014-307274. PMID: 25480460
An idiopathic congenital chylothorax: surgery or conservative management?
Perisson C, Nathan N, Larroquet M, Corvol H
BMJ Case Rep 2014 Apr 30;2014 doi: 10.1136/bcr-2014-204147. PMID: 24788632Free PMC Article
Octreotide in the treatment of congenital chylothorax.
Rasiah SV, Oei J, Lui K
J Paediatr Child Health 2004 Sep-Oct;40(9-10):585-8. doi: 10.1111/j.1440-1754.2004.00471.x. PMID: 15367160

Prognosis

Congenital chylothorax.
Attar MA, Donn SM
Semin Fetal Neonatal Med 2017 Aug;22(4):234-239. Epub 2017 Mar 27 doi: 10.1016/j.siny.2017.03.005. PMID: 28351595
Congenital chylothorax: a prospective nationwide epidemiological study in Germany.
Bialkowski A, Poets CF, Franz AR; Erhebungseinheit für seltene pädiatrische Erkrankungen in Deutschland Study Group
Arch Dis Child Fetal Neonatal Ed 2015 Mar;100(2):F169-72. Epub 2014 Dec 5 doi: 10.1136/archdischild-2014-307274. PMID: 25480460
Outcomes of fetal intervention for primary hydrothorax.
Derderian SC, Trivedi S, Farrell J, Keller RL, Rand L, Goldstein R, Feldstein VA, Hirose S, MacKenzie TC
J Pediatr Surg 2014 Jun;49(6):900-3; discussion 903-4. Epub 2014 Jan 31 doi: 10.1016/j.jpedsurg.2014.01.020. PMID: 24888831
Congenital chylothorax.
Lahmiti S, Elhoudzi J, Aboussad A
ScientificWorldJournal 2009 Jun 12;9:431-4. doi: 10.1100/tsw.2009.62. PMID: 19526181Free PMC Article
Congenital chylothorax.
Dubin PJ, King IN, Gallagher PG
Curr Opin Pediatr 2000 Oct;12(5):505-9. doi: 10.1097/00008480-200010000-00017. PMID: 11021419

Clinical prediction guides

Neonatal Lymphatic Flow Disorder.
Toptan HH, Ozalkaya E, Karadag N, Topcuoglu S, Dincer E, Karatekin G
Indian J Pediatr 2024 Mar;91(3):248-253. Epub 2023 Apr 11 doi: 10.1007/s12098-023-04531-x. PMID: 37040015
Congenital chylothorax: a prospective nationwide epidemiological study in Germany.
Bialkowski A, Poets CF, Franz AR; Erhebungseinheit für seltene pädiatrische Erkrankungen in Deutschland Study Group
Arch Dis Child Fetal Neonatal Ed 2015 Mar;100(2):F169-72. Epub 2014 Dec 5 doi: 10.1136/archdischild-2014-307274. PMID: 25480460
Congenital idiopathic chylothorax in neonates: chemical pleurodesis with povidone-iodine (Betadine).
Brissaud O, Desfrere L, Mohsen R, Fayon M, Demarquez JL
Arch Dis Child Fetal Neonatal Ed 2003 Nov;88(6):F531-3. doi: 10.1136/fn.88.6.f531. PMID: 14602705Free PMC Article
Congenital pulmonary lymphangiectasis with chylothorax: a heterogeneous lymphatic vessel abnormality.
Moerman P, Vandenberghe K, Devlieger H, Van Hole C, Fryns JP, Lauweryns JM
Am J Med Genet 1993 Aug 1;47(1):54-8. doi: 10.1002/ajmg.1320470112. PMID: 8368253
Congenital chylothorax: management by ligature of the thoracic duct.
Andersen EA, Hertel J, Pedersen SA, Sørensen HR
Scand J Thorac Cardiovasc Surg 1984;18(3):193-4. doi: 10.3109/14017438409109890. PMID: 6528265

Recent systematic reviews

Congenital Chylothorax of the Newborn: A Systematic Analysis of Published Cases between 1990 and 2018.
Resch B, Sever Yildiz G, Reiterer F
Respiration 2022;101(1):84-96. Epub 2021 Sep 1 doi: 10.1159/000518217. PMID: 34515211Free PMC Article
The Practicality of Feeding Defatted Human Milk in the Treatment of Congenital Chylothorax.
Concheiro-Guisan A, Alonso-Clemente S, Suarez-Albo M, Duran-Fernandez Feijoo C, Fiel-Ozores A, Fernandez-Lorenzo JR
Breastfeed Med 2019 Nov;14(9):648-653. Epub 2019 Aug 12 doi: 10.1089/bfm.2019.0100. PMID: 31403320

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