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Venolymphatic malformations: prenatal diagnosis using magnetic resonance imaging, perinatal outcomes and long-term follow-up

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Abstract

Background

Venolymphatic malformations are benign. Fetal MRI can more precisely demonstrate an infiltrative pattern of malformations than US.

Objective

To evaluate perinatal outcomes and long-term follow-up of fetal venolymphatic malformations treated in different medical facilities using fetal MRI.

Materials and methods

This retrospective cohort study evaluated 20 pregnant women between 22 weeks and 37 weeks of gestation who were referred from different institutions. They presented with fetuses with various diagnoses of cystic masses on routine US. The cases were studied using MRI. We analyzed prenatal data, perinatal outcomes and long-term follow-up.

Results

We reviewed the MRI scans of 20 patients with venolymphatic malformation. Referral diagnosis was changed in 40% (8/20) of cases, with postnatal concordance of 100% (20/20). Moreover, 65% (13/20) presented with venolymphatic malformation in more than one body segment. The neck was affected in 70% (14/20) of fetuses, while the head and thorax were affected in 30% (6/20) and 45% (9/20), respectively. There were intrathoracic lesions in 35% (7/20), lesions in the abdomen in 30% (6/20), and lesions in the perineum and extremities in 10% (2/20) each. Tracheal displacement, neck deflection and anatomical displacement caused by tumoral compression were present in 15% (3/20) of cases. Moreover, 25% (5/20) of newborns required neonatal intensive care unit admission, and all presented with cervical or thoracic venolymphatic malformation. Furthermore, 50% (10/20) of cases presented with complete resolution after medical therapy. The intrathoracic and cervical residuals (35%, 7/20) were monitored and treated.

Conclusion

MRI showed good correlation with postnatal examination of venolymphatic malformation, was useful in the differential diagnosis of fetal cysts on US, and presented a significant postnatal correlation with thoracic infiltration. The outcomes of prenatally diagnosed venolymphatic malformations are good despite the varying protocols among medical facilities.

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Authors and Affiliations

  1. Department of Radiology, Clínica de Diagnóstico por Imagem (CPDI), Rio de Janeiro, RJ, Brazil

    Ana Paula Pinho Matos, Heron Werner, Pedro Teixeira Castro, Tatiana Mendonça Fazecas e Costa & Renata do Amaral Nogueira

  2. Department of Radiology, Federal University of Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brazil

    Heron Werner, Pedro Teixeira Castro & Edson Marchiori

  3. Fetal Medicine Unit, Fernandes Figueiras Institute, Rio de Janeiro, RJ, Brazil

    Fernando Maia Peixoto-Filho

  4. Department of Obstetrics, Paulista School of Medicine – Federal University of São Paulo (EPM-UNIFESP), Rua Belchior de Azevedo, 156 apto. 111 Torre Vitoria, São Paulo, SP, CEP 05089-030, Brazil

    Edward Araujo Júnior

  5. Municipal University of São Caetano do Sul (USCS), Bela Vista Campus, São Paulo, SP, Brazil

    Edward Araujo Júnior

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  1. Ana Paula Pinho Matos
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  2. Heron Werner
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Correspondence to Edward Araujo Júnior.

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Matos, A.P.P., Werner, H., Castro, P.T. et al. Venolymphatic malformations: prenatal diagnosis using magnetic resonance imaging, perinatal outcomes and long-term follow-up. Pediatr Radiol 51, 1243–1252 (2021). https://doi.org/10.1007/s00247-020-04939-z

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  • DOI: https://doi.org/10.1007/s00247-020-04939-z

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