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Fetal chylothorax

MedGen UID:
1814192
•Concept ID:
C5676614
•
Pathologic Function
HPO: HP:0025677

Definition

The presence of chyle (a type of lipid-rich lymph) in the pleural space (the space surrounding the lung) as observed in a fetus. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFetal chylothorax

Conditions with this feature

Lymphatic malformation 12
MedGen UID:
1823976
•Concept ID:
C5774203
•
Disease or Syndrome
Lymphatic malformation-12 (LMPHM12) is characterized by abnormalities in the development and function of major truncal lymphatic vessels, causing nonimmune hydrops fetalis that results in stillbirth in some cases. Other affected individuals experience postnatal subcutaneous lymphedema and chylothorax, with pleural and pericardial effusions and ascites (Byrne et al., 2022). For a general phenotypic description and discussion of genetic heterogeneity of lymphatic malformation, see LMPHM1 (153100).
See: Condition Record
Lymphatic malformation 12

Professional guidelines

PubMed

OK-432 Treatment of Early Fetal Chylothorax: Pregnancy Outcome and Long-Term Follow-Up of 14 Cases.
Nørgaard LN, Nygaard U, Damm JA, Esbjørn BH, Pedersen MMA, Rottbøll A, Jørgensen C, Sundberg K
Fetal Diagn Ther 2019;46(2):81-87. Epub 2018 Oct 3 doi: 10.1159/000489775. PMID: 30282075
Chylothorax and chylous ascites: management and pitfalls.
Lopez-Gutierrez JC, Tovar JA
Semin Pediatr Surg 2014 Oct;23(5):298-302. Epub 2014 Sep 4 doi: 10.1053/j.sempedsurg.2014.09.011. PMID: 25459015
Experimental treatment of bilateral fetal chylothorax using in-utero pleurodesis.
Yang YS, Ma GC, Shih JC, Chen CP, Chou CH, Yeh KT, Kuo SJ, Chen TH, Hwu WL, Lee TH, Chen M
Ultrasound Obstet Gynecol 2012 Jan;39(1):56-62. Epub 2011 Dec 5 doi: 10.1002/uog.9048. PMID: 21584887

Recent clinical studies

Etiology

OK-432 Treatment of Early Fetal Chylothorax: Pregnancy Outcome and Long-Term Follow-Up of 14 Cases.
Nørgaard LN, Nygaard U, Damm JA, Esbjørn BH, Pedersen MMA, Rottbøll A, Jørgensen C, Sundberg K
Fetal Diagn Ther 2019;46(2):81-87. Epub 2018 Oct 3 doi: 10.1159/000489775. PMID: 30282075
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
Chylothorax and chylous ascites: management and pitfalls.
Lopez-Gutierrez JC, Tovar JA
Semin Pediatr Surg 2014 Oct;23(5):298-302. Epub 2014 Sep 4 doi: 10.1053/j.sempedsurg.2014.09.011. PMID: 25459015
Retrospective review of thoracoamniotic shunting using a double-basket catheter for fetal chylothorax.
Miyoshi T, Katsuragi S, Ikeda T, Horiuchi C, Kawasaki K, Kamiya CA, Sasaki Y, Osato K, Neki R, Yoshimatsu J
Fetal Diagn Ther 2013;34(1):19-25. Epub 2013 Apr 12 doi: 10.1159/000348776. PMID: 23595018

Diagnosis

OK-432 Treatment of Early Fetal Chylothorax: Pregnancy Outcome and Long-Term Follow-Up of 14 Cases.
Nørgaard LN, Nygaard U, Damm JA, Esbjørn BH, Pedersen MMA, Rottbøll A, Jørgensen C, Sundberg K
Fetal Diagn Ther 2019;46(2):81-87. Epub 2018 Oct 3 doi: 10.1159/000489775. PMID: 30282075
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
Chylothorax and chylous ascites: management and pitfalls.
Lopez-Gutierrez JC, Tovar JA
Semin Pediatr Surg 2014 Oct;23(5):298-302. Epub 2014 Sep 4 doi: 10.1053/j.sempedsurg.2014.09.011. PMID: 25459015
Congenital chylothorax with hydrops: postnatal care and outcome following antenatal diagnosis.
Mussat P, Dommergues M, Parat S, Mandelbrot L, de Gamarra E, Dumez Y, Moriette G
Acta Paediatr 1995 Jul;84(7):749-55. doi: 10.1111/j.1651-2227.1995.tb13749.x. PMID: 7549291
Pleuro-amniotic shunting for fetal chylothorax.
Booth P, Nicolaides KH, Greenough A, Gamsu HR
Early Hum Dev 1987 Nov;15(6):365-7. doi: 10.1016/0378-3782(87)90031-4. PMID: 3436279

Therapy

OK-432 Treatment of Early Fetal Chylothorax: Pregnancy Outcome and Long-Term Follow-Up of 14 Cases.
Nørgaard LN, Nygaard U, Damm JA, Esbjørn BH, Pedersen MMA, Rottbøll A, Jørgensen C, Sundberg K
Fetal Diagn Ther 2019;46(2):81-87. Epub 2018 Oct 3 doi: 10.1159/000489775. PMID: 30282075
Intrapleural injection of OK-432 as the primary in-utero treatment for fetal chylothorax.
Leung VK, Suen SS, Ting YH, Law LW, Lau TK, Leung TY
Hong Kong Med J 2012 Apr;18(2):156-9. PMID: 22477741
Experimental treatment of bilateral fetal chylothorax using in-utero pleurodesis.
Yang YS, Ma GC, Shih JC, Chen CP, Chou CH, Yeh KT, Kuo SJ, Chen TH, Hwu WL, Lee TH, Chen M
Ultrasound Obstet Gynecol 2012 Jan;39(1):56-62. Epub 2011 Dec 5 doi: 10.1002/uog.9048. PMID: 21584887
A new fetal therapy for chylothorax: pleurodesis with OK-432.
Okawa T, Takano Y, Fujimori K, Yanagida K, Sato A
Ultrasound Obstet Gynecol 2001 Oct;18(4):376-7. doi: 10.1046/j.0960-7692.2001.00556.x. PMID: 11778999
A case of successful fetal therapy for congenital chylothorax by intrapleural injection of OK-432.
Tanemura M, Nishikawa N, Kojima K, Suzuki Y, Suzumori K
Ultrasound Obstet Gynecol 2001 Oct;18(4):371-5. doi: 10.1046/j.0960-7692.2001.00536.x. PMID: 11778998

Prognosis

OK-432 Treatment of Early Fetal Chylothorax: Pregnancy Outcome and Long-Term Follow-Up of 14 Cases.
Nørgaard LN, Nygaard U, Damm JA, Esbjørn BH, Pedersen MMA, Rottbøll A, Jørgensen C, Sundberg K
Fetal Diagn Ther 2019;46(2):81-87. Epub 2018 Oct 3 doi: 10.1159/000489775. PMID: 30282075
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
Retrospective review of thoracoamniotic shunting using a double-basket catheter for fetal chylothorax.
Miyoshi T, Katsuragi S, Ikeda T, Horiuchi C, Kawasaki K, Kamiya CA, Sasaki Y, Osato K, Neki R, Yoshimatsu J
Fetal Diagn Ther 2013;34(1):19-25. Epub 2013 Apr 12 doi: 10.1159/000348776. PMID: 23595018
Experimental treatment of bilateral fetal chylothorax using in-utero pleurodesis.
Yang YS, Ma GC, Shih JC, Chen CP, Chou CH, Yeh KT, Kuo SJ, Chen TH, Hwu WL, Lee TH, Chen M
Ultrasound Obstet Gynecol 2012 Jan;39(1):56-62. Epub 2011 Dec 5 doi: 10.1002/uog.9048. PMID: 21584887
Congenital chylothorax with hydrops: postnatal care and outcome following antenatal diagnosis.
Mussat P, Dommergues M, Parat S, Mandelbrot L, de Gamarra E, Dumez Y, Moriette G
Acta Paediatr 1995 Jul;84(7):749-55. doi: 10.1111/j.1651-2227.1995.tb13749.x. PMID: 7549291

Clinical prediction guides

Chylothorax and chylous ascites: management and pitfalls.
Lopez-Gutierrez JC, Tovar JA
Semin Pediatr Surg 2014 Oct;23(5):298-302. Epub 2014 Sep 4 doi: 10.1053/j.sempedsurg.2014.09.011. PMID: 25459015
Successful treatment of fetal bilateral primary chylothorax--report of the two cases.
Nowakowska D, Gaj Z, Grzesiak M, Gulczyńska E, Wilczyński J
Ginekol Pol 2014 Sep;85(9):708-12. PMID: 25322545
Retrospective review of thoracoamniotic shunting using a double-basket catheter for fetal chylothorax.
Miyoshi T, Katsuragi S, Ikeda T, Horiuchi C, Kawasaki K, Kamiya CA, Sasaki Y, Osato K, Neki R, Yoshimatsu J
Fetal Diagn Ther 2013;34(1):19-25. Epub 2013 Apr 12 doi: 10.1159/000348776. PMID: 23595018
New treatment of early fetal chylothorax.
Nygaard U, Sundberg K, Nielsen HS, Hertel S, Jørgensen C
Obstet Gynecol 2007 May;109(5):1088-92. doi: 10.1097/01.AOG.0000259907.91973.69. PMID: 17470587
Magnetic resonance imaging of the fetus in congenital intrathoracic disorders: preliminary observations.
Liu X, Ashtari M, Leonidas JC, Chan Y
Pediatr Radiol 2001 Jun;31(6):435-9. doi: 10.1007/s002470100450. PMID: 11436891

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