Neonatal Marfan Syndrome

Am J Perinatol. 2019 Jul;36(S 02):S74-S76. doi: 10.1055/s-0039-1691770. Epub 2019 Jun 25.

Abstract

Objective: The Marfan syndrome (MFS) is an autosomal dominant disorder of connective tissue resulting from pathogenic variants of the fibrillin-1 gene (FBN1) with skeletal, cardiac, and ocular involvement.

Study design: We report on a full-term male neonate, who showed at birth characteristics and dysmorphisms suggestive of nMFS, combined with the detection of severe cardiovascular disease. A multidisciplinary team made up of neonatologists and pediatricians, cardiologists, geneticists, ophtalmologists, physiatrists and physioterapists was formed to manage this patient.

Results and conclusion: Early diagnosis of this rare condition is critical for adequate treatment and specific follow-up, and impacts significantly on prognosis.

Publication types

  • Case Reports

MeSH terms

  • Electrocardiography
  • Humans
  • Infant, Newborn
  • Infant, Newborn, Diseases*
  • Male
  • Marfan Syndrome* / genetics
  • Marfan Syndrome* / surgery
  • Mitral Valve Prolapse / surgery
  • Mutation
  • Tricuspid Valve Prolapse / surgery