Outcomes of fetal intervention for primary hydrothorax

Published:February 03, 2014DOI:



      Primary hydrothorax is a rare congenital anomaly with outcomes ranging from spontaneous resolution to fetal demise. We reviewed our experience with fetuses diagnosed with primary hydrothorax to evaluate prenatal management strategies.


      We reviewed the records of patients evaluated for fetal pleural effusions at our Fetal Treatment Center between 1996 and 2013. To define fetuses with primary hydrothorax, we excluded those with structural or genetic anomalies, diffuse lymphangiectasia, immune hydrops, and monochorionic diamniotic twin gestations.


      We identified 31 fetuses with primary hydrothorax, of whom 24 had hydrops. Hydropic fetuses were more likely to present with bilateral effusions. Of all fetuses with primary hydrothorax, 21 had fetal interventions. Survival without hydrops was 7/7 (100%), whereas survival with hydrops depended on whether or not the patient had fetal intervention: 12/19 (63%) with intervention and 1/5 (20%) without intervention. Premature delivery was common (44%) among those who had fetal intervention.


      Fetal intervention for primary hydrothorax may lead to resolution of hydrops, but preterm birth and neonatal demise still occur. Understanding the pathophysiology of hydrops may provide insights into further prenatal management strategies, including targeted therapies to prevent preterm labor.

      Key words

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Journal of Pediatric Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Longaker M.T.
        • Laberge J.M.
        • Dansereau J.
        • et al.
        Primary fetal hydrothorax: natural history and management.
        J Pediatr Surg. 1989; 24: 573-576
        • Rocha G.
        Pleural effusions in the neonate.
        Curr Opin Pulm Med. 2007; 13: 305-311
        • Pisek G.R.
        Report of a case of chylothorax.
        Arch Pediatr. 1917; 34: 929
        • Hematti H.
        • Mehran R.J.
        Anatomy of the thoracic duct.
        Thorac Surg Clin. 2011; 21 ([ix]): 229-238
        • Skandalakis J.E.
        • Skandalakis L.J.
        • Skandalakis P.N.
        Anatomy of the lymphatics.
        Surg Oncol Clin N Am. 2007; 16: 1-16
        • Inselman L.S.
        • Mellins R.B.
        Growth and development of the lung.
        J Pediatr. 1981; 98: 1-15
        • Yinon Y.
        • Kelly E.
        • Ryan G.
        Fetal pleural effusions.
        Best Pract Res Clin Obstet Gynaecol. 2008; 22: 77-96
        • Chen C.P.
        • Chang T.Y.
        • Wang W.
        Resolution of fetal bilateral chylothorax and ascites after two unilateral thoracocenteses.
        Ultrasound Obstet Gynecol. 2001; 18: 401-402
        • Murabayashi N.
        • Sugiyama T.
        • Kusaka H.
        • et al.
        Thoracoamniotic shunting with double-basket catheters for fetal chylothorax in the second trimester.
        Fetal Diagn Ther. 2007; 22: 425-427
        • Picone O.
        • Benachi A.
        • Mandelbrot L.
        • et al.
        Thoracoamniotic shunting for fetal pleural effusions with hydrops.
        Am J Obstet Gynecol. 2004; 191: 2047-2050
        • Smith R.P.
        • Illanes S.
        • Denbow M.L.
        • et al.
        Outcome of fetal pleural effusions treated by thoracoamniotic shunting.
        Ultrasound Obstet Gynecol. 2005; 26: 63-66
        • Yamamoto M.
        • Insunza A.
        • Carrillo J.
        • et al.
        Intrathoracic pressure in congenital chylothorax: keystone for the rationale of thoracoamniotic shunting?.
        Fetal Diagn Ther. 2007; 22: 169-171
        • Nygaard U.
        • Sundberg K.
        • Nielsen H.S.
        • et al.
        New treatment of early fetal chylothorax.
        Obstet Gynecol. 2007; 109: 1088-1092
        • Randenberg A.L.
        Nonimmune hydrops fetalis part II: does etiology influence mortality?.
        Neonatal Netw. 2010; 29: 367-380
        • Rustico M.A.
        • Lanna M.
        • Coviello D.
        • et al.
        Fetal pleural effusion.
        Prenat Diagn. 2007; 27: 793-799
        • Pettersen H.N.
        • Nicolaides K.H.
        Pleural effusions.
        in: Fisk N.M. Moise K.J. Fetal Therapy. Invasive and Transplacental. Cambridge University Press, Cambridge, England1997: 261-272
        • Klam S.
        • Bigras J.L.
        • Hudon L.
        Predicting outcome in primary fetal hydrothorax.
        Fetal Diagn Ther. 2005; 20: 366-370
        • Chen M.
        • Hsieh C.Y.
        • Shih J.C.
        • et al.
        Proinflammatory macrophage migratory inhibition factor and interleukin-6 are concentrated in pleural effusion of human fetuses with prenatal chylothorax.
        Prenat Diagn. 2007; 27: 435-441
        • Tsukimori K.
        • Nakanami N.
        • Fukushima K.
        • et al.
        Pleural fluid/serum immunoglobulin ratio is a diagnostic marker for congenital chylothorax in utero.
        J Perinat Med. 2006; 34: 313-317
        • Yeang C.H.
        • Ma G.C.
        • Shih J.C.
        • et al.
        Genome-wide gene expression analysis implicates the immune response and lymphangiogenesis in the pathogenesis of fetal chylothorax.
        PLoS One. 2012; 7: e34901
        • Chan F.Y.
        • Borzi P.
        • Cincotta R.
        • et al.
        Limb constriction as a complication of intra-uterine vesico-amniotic shunt: fetoscopic release.
        Fetal Diagn Ther. 2002; 17: 315-320
        • Weber A.M.
        • Philipson E.H.
        Fetal pleural effusion: a review and meta-analysis for prognostic indicators.
        Obstet Gynecol. 1992; 79: 281-286
        • Gotsch F.
        • Romero R.
        • Kusanovic J.P.
        • et al.
        The fetal inflammatory response syndrome.
        Clin Obstet Gynecol. 2007; 50: 652-683