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  • Perinatal/Neonatal Case Presentation
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Perinatal/Neonatal Case Presentation

Congenital chylothorax treated with oral sildenafil: a case report and review of the literature

Abstract

Congenital chylothorax (CC) can result from a congenital malformation or an acquired obstruction or disruption of the thoracic duct. Recently, oral administration of the phosphodiesterase-5 inhibitor, sildenafil, was reported to be effective in resolving non-pulmonary lymphatic malformations in infants and young children. We report a case of CC in a late preterm infant with congenital pulmonary lymphangiectasia where octreotide was not effective, but management with oral sildenafil was successful.

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References

  1. Rocha G, Fernandes P, Rocha P, Quintas C, Martins T, Proenca E . Pleural effusions in the neonate. Acta Paediatr 2006; 95: 791–798.

    Article  Google Scholar 

  2. Downie L, Sasi A, Malhotra A . Congenital chylothorax: associations and neonatal outcomes. Journal of paediatrics and child health 2014; 50: 234–238.

    Article  Google Scholar 

  3. Danial C, Tichy AL, Tariq U, Swetman GL, Khuu P, Leung TH et al. An open- label study to evaluate sildenafil for the treatment of lymphatic malformations. J Am Acad Dermatol 2014; 70: 1050–1057.

    Article  CAS  Google Scholar 

  4. Swetman GL, Berk DR, Vasanawala SS, Feinstein JA, Lane AT, Bruckner AL . Sildenafil for severe lymphatic malformations. N Engl J Med 2012; 366: 384–386.

    Article  CAS  Google Scholar 

  5. Iacovidou N, Syggelou A, Fanos V, Xanthos T . The use of sildenafil in the treatment of persistent pulmonary hypertension of the newborn: a review of the literature. Curr Pharm Des 2012; 18: 3034–3045.

    Article  CAS  Google Scholar 

  6. Reiterer F, Grossauer K, Morris N, Uhrig S, Resch B . Congenital pulmonary lymphangiectasis. Paediatr Respir Rev 2014; 15: 275–280.

    PubMed  Google Scholar 

  7. Battaglia A . The inv dup (15) or idic (15) syndrome (Tetrasomy 15q). Orphanet J Rare Dis 2008; 3: 30.

    Article  Google Scholar 

  8. Bellini C, Mazzella M, Campisi C, Taddei G, Mosca F, Toma P et al. Multimodal imaging in the congenital pulmonary lymphangiectasia-congenital chylothorax- hydrops fetalis continuum. Lymphology 2004; 37: 22–30.

    CAS  PubMed  Google Scholar 

  9. Ozeki M, Kanda K, Kawamoto N, Ohnishi H, Fujino A, Hirayama M et al. Propranolol as an alternative treatment option for pediatric lymphatic malformation. Tohoku J Expe Med 2013; 229: 61–66.

    Article  CAS  Google Scholar 

  10. Poralla C, Specht S, Born M, Muller A, Bartmann P, Muller A . Treatment of congenital generalized lymphangiectasia with propranolol in a preterm infant. Pediatrics 2014; 133: e439–e442.

    Article  Google Scholar 

  11. Reinglas J, Ramphal R, Bromwich M . The successful management of diffuse lymphangiomatosis using sirolimus: a case report. Laryngoscope 2011; 121: 1851–1854.

    PubMed  Google Scholar 

  12. Huggenberger R, Siddiqui SS, Brander D, Ullmann S, Zimmermann K, Antsiferova M et al. An important role of lymphatic vessel activation in limiting acute inflammation. Blood 2011; 117: 4667–4678.

    Article  CAS  Google Scholar 

  13. Zhou Q, Guo R, Wood R, Boyce BF, Liang Q, Wang YJ et al. Vascular endothelial growth factor C attenuates joint damage in chronic inflammatory arthritis by accelerating local lymphatic drainage in mice. Arthritis Rheum 2011; 63: 2318–2328.

    Article  CAS  Google Scholar 

  14. Kajiya K, Huggenberger R, Drinnenberg I, Ma B, Detmar M . Nitric oxide mediates lymphatic vessel activation via soluble guanylate cyclase alpha1beta1-impact on inflammation. FASEB J 2008; 22: 530–537.

    Article  CAS  Google Scholar 

  15. Jeon YH, Heo YS, Kim CM, Hyun YL, Lee TG, Ro S et al. Phosphodiesterase: overview of protein structures, potential therapeutic applications and recent progress in drug development. Cell Mol Life Sci 2005; 62: 1198–1220.

    Article  CAS  Google Scholar 

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Correspondence to D T Malleske.

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Malleske, D., Yoder, B. Congenital chylothorax treated with oral sildenafil: a case report and review of the literature. J Perinatol 35, 384–386 (2015). https://doi.org/10.1038/jp.2015.10

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