Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2019 Nov 15;111(19):1577-1583.
doi: 10.1002/bdr2.1597. Epub 2019 Oct 9.

Diaphragmatic paralysis: Evaluation in infants with congenital Zika syndrome

Vanessa van der Linden  1   2 , Otavio Gomes Lins  3 , Natacha Calheiros de Lima Petribu  2 , Ana Claudia Marques Gouveia de Melo  4 , Jazmyn Moore  5 , Sonja A Rasmussen  6   7 , Cynthia A Moore  5
Affiliations
Case Reports

Diaphragmatic paralysis: Evaluation in infants with congenital Zika syndrome

Vanessa van der Linden et al. Birth Defects Res. .

Abstract

Background: Paralysis of the diaphragm in newborn infants can lead to recurrent infections and life-threatening respiratory insufficiency. The clinical diagnosis of unilateral diaphragmatic paralysis has been reported in infants with laboratory evidence of congenital Zika virus infection and/or the congenital Zika syndrome (CZS) phenotype but no evaluation of phrenic nerve function has been described. All reported infants have had accompanying arthrogryposis. High infant mortality is reported.

Methods: The causal mechanism of congenital diaphragmatic paralysis was evaluated in three infants with arthrogryposis as a manifestation of CZS (two of the three infants had laboratory evidence of ZIKV infection shortly after birth; the remaining infant had negative serology for ZIKV when first tested at 7 months of age). Electromyography and phrenic nerve compound muscle action potential (CMAP) were performed in all infants with diaphragmatic paralysis demonstrated on imaging studies.

Results: All infants had evidence of moderate chronic involvement of peripheral motor neurons. Phrenic nerve CMAP was reduced on the side of the diaphragmatic paralysis in two infants and reduced bilaterally in the remaining infant who had primarily anterior involvement of the diaphragm. All three infants had multiple medical complications and one infant died at 18 months of age.

Conclusion: Evaluation of three infants with CZS and diaphragmatic paralysis demonstrated phrenic nerve dysfunction. In these and other affected infants, arthrogryposis appears to be a constant co-occurring condition and health problems are significant; both conditions are likely due to involvement of the peripheral nervous system in some infants with CZS.

Keywords: Zika virus; arthrogryposis; congenital infection; diaphragmatic paralysis; phrenic nerve.

PubMed Disclaimer

Conflict of interest statement

CONFLICT OF INTEREST

The authors have no potential conflict of interest or financial disclosures. The findings and conclusions in this report are those of the authors and do not necessarily represent the official position of the Centers for Disease Control and Prevention.

Figures

FIGURE 1
FIGURE 1
Chest imaging of three infants with congenital Zika syndrome, arthrogryposis and diaphragmatic paralysis, Brazil. (a) Infant 1 at age 7 months, chest X-ray shows elevation of the right hemidiaphragm. (b) Infant 2 at age 11 months, chest X-ray shows elevation of the right hemidiaphragm. (c) Infant 3 at age 8 months, chest X-ray shows elevation of the right and left hemidiaphragms. (d, e) Infant 3 at 11 months, chest CT shows heterogeneous diaphragmatic paralysis, with greater involvement of the right side and anterior area. CT, computed tomography
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

  • Virus as Teratogenic Agents.
    Calado AM, Seixas F, Dos Anjos Pires M. Calado AM, et al. Methods Mol Biol. 2024;2753:105-142. doi: 10.1007/978-1-0716-3625-1_4. Methods Mol Biol. 2024. PMID: 38285335
  • Neurodevelopment in Children Exposed to Zika in utero: Clinical and Molecular Aspects.
    Schuler-Faccini L, Del Campo M, García-Alix A, Ventura LO, Boquett JA, van der Linden V, Pessoa A, van der Linden Júnior H, Ventura CV, Leal MC, Kowalski TW, Rodrigues Gerzson L, Skilhan de Almeida C, Santi L, Beys-da-Silva WO, Quincozes-Santos A, Guimarães JA, Garcez PP, Gomes JDA, Vianna FSL, Anjos da Silva A, Fraga LR, Vieira Sanseverino MT, Muotri AR, Lopes da Rosa R, Abeche AM, Marcolongo-Pereira C, Souza DO. Schuler-Faccini L, et al. Front Genet. 2022 Mar 8;13:758715. doi: 10.3389/fgene.2022.758715. eCollection 2022. Front Genet. 2022. PMID: 35350244 Free PMC article. Review.
  • Time to Evaluate the Clinical Repercussions of Zika Virus Vertical Transmission? A Systematic Review.
    do Amaral YNDV, Malacarne J, Brandão PG, Brasil P, Nielsen-Saines K, Moreira MEL. do Amaral YNDV, et al. Front Psychiatry. 2021 Aug 30;12:699115. doi: 10.3389/fpsyt.2021.699115. eCollection 2021. Front Psychiatry. 2021. PMID: 34526920 Free PMC article.
  • All-Cause and Cause-Specific Mortality in Children With Congenital Zika Syndrome in Brazil.
    Cardim LL, Costa MDCN, Rodrigues LC, Carvalho-Sauer R, Brickley EB, Ximenes RAA, Pescarini JM, Andrade RFS, Barreto ML, Teixeira MDGLC, Paixao ESD. Cardim LL, et al. JAMA Netw Open. 2025 Jan 2;8(1):e2456042. doi: 10.1001/jamanetworkopen.2024.56042. JAMA Netw Open. 2025. PMID: 39847354 Free PMC article.
  • Causes of death in children with congenital Zika syndrome in Brazil, 2015 to 2018: A nationwide record linkage study.
    Costa MDCN, Cardim LL, Moore CA, de Jesus EDS, Carvalho-Sauer R, Barreto ML, Rodrigues LC, Smeeth L, Schuler-Faccini L, Brickley EB, Oliveira WK, Carmo EH, Pescarini JM, Andrade RFS, Rodrigues MMS, Veiga RV, Costa LC, França GVA, Teixeira MG, Paixão ES. Costa MDCN, et al. PLoS Med. 2023 Feb 24;20(2):e1004181. doi: 10.1371/journal.pmed.1004181. eCollection 2023 Feb. PLoS Med. 2023. PMID: 36827251 Free PMC article.
See all "Cited by" articles

References

    1. Adebanjo T, Godfred-Cato S, Viens L, Fischer M, Staples JE, Kuhnert-Tallman W, … Contributors. (2017). Update: Interim guidance for the diagnosis, evaluation, and management of infants with possible congenital Zika virus infection - United States, October 2017. MMWR. Morbidity and Mortality Weekly Report, 66 (41), 1089–1099. 10.15585/mmwr.mm6641a1 - DOI - PMC - PubMed
    1. Alamo L, Gudinchet F, & Meuli R (2015). Imaging findings in fetal diaphragmatic abnormalities. Pediatric Radiology, 45(13), 1887–1900. 10.1007/s00247-015-3418-5 - DOI - PubMed
    1. Aragao M, Brainer-Lima AM, Holanda AC, van der Linden V, Vasco Aragao L, Silva Junior MLM, … Valenca MM (2017). Spectrum of spinal cord, spinal root, and brain mri abnormalities in congenital Zika syndrome with and without arthrogryposis. AJNR. American Journal of Neuroradiology, 38(5), 1045–1053. 10.3174/ajnr.A5125 - DOI - PMC - PubMed
    1. Arantes de Araujo L, Ferraz de Arruda Musegante A, de Oliveira Damasceno E, Barroso U Jr., & Badaro R. (2013). Investigation into neurogenic bladder in arthrogryposis multiplex congenita. Journal of Pediatric Urology, 9(6 Pt A), 895–899. 10.1016/j.jpurol.2012.12.011 - DOI - PubMed
    1. Becroft DM (1979). Prenatal cytomegalovirus infection and muscular deficiency (eventration) of the diaphragm. The Journal of Pedi-atrics, 94(1), 74–75. - PubMed

Publication types

LinkOut - more resources