Download PDF
CC BY-NC-ND 4.0 · AJP Rep 2020; 10(04): e403-e407
DOI: 10.1055/s-0040-1715178
Case Report

Chylothorax Associated with Congenital Complete Atrioventricular Block

Shuhei Fujino
1   Division of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, Tokyo, Japan
,
Hidehiko Maruyama
1   Division of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, Tokyo, Japan
,
Keiko Tsukamoto
1   Division of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, Tokyo, Japan
,
Hiroshi Ono
2   Division of Cardiology, National Center for Child Health and Development, Tokyo, Japan
,
Tetsuya Isayama
1   Division of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, Tokyo, Japan
,
Yushi Ito
1   Division of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, Tokyo, Japan
› Author Affiliations
› Further Information
Also available at
   Permissions and Reprints

Abstract

Introduction Congenital complete atrioventricular block (CCAVB) associated with congenital chylothorax is a rare finding that has been reported in only one case in the literature. We report here the case of an infant with CCAVB complicated by congenital chylothorax.

Patient Report We present the case of a male neonate with a birth weight of 2114 g. Fetal bradycardia and right pleural effusion were detected at gestational age of 22 weeks. Maternal serum levels of anti-Sjögren's-syndrome-related antigen A autoantibody were high (4840 U/mL). The neonate was delivered at gestational age of 33 weeks; a temporary external pacemaker was placed immediately after birth that resulted in an improved cardiac output. Milk-colored pleural effusion increased in volume together with the initiation of breast milk feeding. Lymphocytosis and high triglyceride levels in the pleural fluid led to the diagnosis of chylothorax. The pleural effusion resolved in response to prednisolone, octreotide, and total parenteral nutrition.

Discussion The causal relationship between CCAVB and congenital chylothorax can be explained by considering the damage to the lymphatic vessels secondary to inflammation due to maternal autoantibodies and venous congestion due to bradycardia.

Conclusion In any case of CCAVB associated with atypical pleural effusion, one must consider the possibility of congenital chylothorax.

Keywords

congenital complete atrioventricular block - congenital chylothorax - anti-SS-A/Ro antibody - prednisolone - octreotide - coagulation factor XIII


Publication History

Received: 08 March 2020

Accepted: 29 May 2020

Article published online:
03 December 2020

© 2020. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

Thieme Medical Publishers, Inc.
333 Seventh Avenue, 18th Floor, New York, NY 10001, USA

 

  • References

  • 1 Michaëlsson M, Engle MA. Congenital complete heart block: an international study of the natural history. Cardiovasc Clin 1972; 4 (03) 85-101
    PubMedGoogle Scholar
  • 2 Keller BA, Hirose S, Farmer DL. Surgical Disorders of the Chest and Airways. In: Gleason CA, Juul SE. eds. Avery's Diseases of the Newborn. 10th edition. Philadelphia: Elsevier Saunders; 2018: 695-723
    Google Scholar
  • 3 Saito K, Tao K, Mizukaki N. et al. A case of Down syndrome baby, with fetal hydrops and congenital complete AV block. . [Article in Japanese] J Jpn Soc Perin Neon Med 2013; 49: 1073-1077
    PubMedGoogle Scholar
  • 4 Tanaka N, Muro Y, Sugiura K, Tomita Y. Anti-SS-A/Ro antibody determination by indirect immunofluorescence and comparison of different methods of anti-nuclear antibody screening: evaluation of the utility of HEp-2 cells transfected with the 60 kDa SS-A/Ro as a substrate. Mod Rheumatol 2008; 18 (06) 585-592
    CrossrefPubMedGoogle Scholar
  • 5 Boville B, Young LC. Quick Guide to Pediatric Cardiopulmonary Care. Irvine: Edwards Lifesciences; 2015: 3-78
    Google Scholar
  • 6 Lopez-Gutierrez JC, Tovar JA. Chylothorax and chylous ascites: management and pitfalls. Semin Pediatr Surg 2014; 23 (05) 298-302
    CrossrefPubMedGoogle Scholar
  • 7 Tsukimori K, Nakanami N, Fukushima K, Yoshimura T, Hikino S, Nakano H. Pleural fluid/serum immunoglobulin ratio is a diagnostic marker for congenital chylothorax in utero. J Perinat Med 2006; 34 (04) 313-317
    CrossrefPubMedGoogle Scholar
  • 8 van Straaten HL, Gerards LJ, Krediet TG. Chylothorax in the neonatal period. Eur J Pediatr 1993; 152 (01) 2-5
    CrossrefPubMedGoogle Scholar
  • 9 Villena V, de Pablo A, Martín-Escribano P. Chylothorax and chylous ascites due to heart failure. Eur Respir J 1995; 8 (07) 1235-1236
    CrossrefPubMedGoogle Scholar
  • 10 Soysal DE, Hizar Turan S, Ozmen M. et al. A rare case of systemic lupus erythematosus with chylous ascites and chylothorax. Case Rep Rheumatol 2013; 2013: 797696
    PubMedGoogle Scholar
  • 11 Lin YJ, Chen DY, Lan JL, Hsieh TY. Chylothorax as the initial presentation of systemic lupus erythematosus: a case report. Clin Rheumatol 2007; 26 (08) 1373-1374
    CrossrefPubMedGoogle Scholar
  • 12 Lee CK, Han JM, Lee KN. et al. Concurrent occurrence of chylothorax, chylous ascites, and protein-losing enteropathy in systemic lupus erythematosus. J Rheumatol 2002; 29 (06) 1330-1333
    PubMedGoogle Scholar
  • 13 Dohmoto T, Kuranobu Y, Mino Y. et al. Case report of congenital chylothorax effectively treated by prednisolone administration. . [Article in Japanese] Journal of Japan Society for Premature and Newborn Medicine 2009; 21: 279-285
    PubMedGoogle Scholar
  • 14 Schroth M, Meißner U, Cesnjevar R. et al. Plasmatic [corrected] factor XIII reduces severe pleural effusion in children after open-heart surgery. Pediatr Cardiol 2006; 27 (01) 56-60
    CrossrefPubMedGoogle Scholar