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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
Octreotid as a treatment of congenital chylothorax.
Bulbul A, Unsur EK
Pediatr Pulmonol 2010 Jun;45(6):628; author reply 629-30. doi: 10.1002/ppul.21217. PMID: 20503291
Treatment of congenital chylothorax.
Brodman RF, Zavelson TM, Schiebler GL
J Pediatr 1974 Oct;85(4):516-7. doi: 10.1016/s0022-3476(74)80457-9. PMID: 4216624

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
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
Congenital chylothorax.
Al-Tawil K, Ahmed G, Al-Hathal M, Al-Jarallah Y, Campbell N
Am J Perinatol 2000;17(3):121-6. doi: 10.1055/s-2000-9281. PMID: 11012135

Diagnosis

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 pulmonary lymphangiectasis.
Reiterer F, Grossauer K, Morris N, Uhrig S, Resch B
Paediatr Respir Rev 2014 Sep;15(3):275-80. Epub 2014 May 29 doi: 10.1016/j.prrv.2014.05.002. PMID: 24997116
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.
Murali N, Jana AK, Thomas N, Jeyanthi P
Indian Pediatr 1997 Jul;34(7):644-5. PMID: 9401261

Therapy

Propranolol Therapy for Congenital Chylothorax.
Handal-Orefice R, Midura D, Wu JK, Parravicini E, Miller RS, Shawber CJ
Pediatrics 2023 Feb 1;151(2) doi: 10.1542/peds.2022-058555. PMID: 36651059
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 treated with oral sildenafil: a case report and review of the literature.
Malleske DT, Yoder BA
J Perinatol 2015 May;35(5):384-6. doi: 10.1038/jp.2015.10. PMID: 25917021
Letter: A plea for conservative therapy in congenital chylothorax.
Martin GI
J Pediatr 1975 May;86(5):821. doi: 10.1016/s0022-3476(75)80396-9. PMID: 805830

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
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
Congenital chylothorax.
Al-Tawil K, Ahmed G, Al-Hathal M, Al-Jarallah Y, Campbell N
Am J Perinatol 2000;17(3):121-6. doi: 10.1055/s-2000-9281. PMID: 11012135

Clinical prediction guides

Indocyanine green lymphography in the congenital chylothorax and chylous ascites.
Lin T, Shibasaki J, Yamamoto K, Shimokaze T, Toyoshima K
J Neonatal Perinatal Med 2024;17(2):247-254. doi: 10.3233/NPM-230171. PMID: 38640176
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
Propranolol Therapy for Congenital Chylothorax.
Handal-Orefice R, Midura D, Wu JK, Parravicini E, Miller RS, Shawber CJ
Pediatrics 2023 Feb 1;151(2) doi: 10.1542/peds.2022-058555. PMID: 36651059
Clinical features and outcomes of congenital chylothorax: a single tertiary medical center experience in China.
Wang B, Feng Y, Guo Y, Kan Q, Zou Y, Wu Y, Zheng M, Cheng R
J Cardiothorac Surg 2022 Oct 27;17(1):276. doi: 10.1186/s13019-022-02009-z. PMID: 36303149Free 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

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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