Richieri-costa and Pereira syndrome: severe phenotype

Am J Med Genet A. 2013 Aug;161A(8):1999-2003. doi: 10.1002/ajmg.a.35989. Epub 2013 Jun 21.

Abstract

Richieri-Costa and Pereira syndrome, described in 1992, comprises short stature, Robin sequence, cleft mandible, limb malformations, and short larynx, deformed or lack of epiglottis, and abnormal aryepiglottic folds. There are 32 reported cases, only one described outside Brazil. We describe a 4-month-old boy with the most severe phenotype yet reported.

Keywords: Richieri-Costa and Pereira syndrome; oral cleft; severe phenotype.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Brazil
  • Clubfoot / diagnostic imaging
  • Clubfoot / pathology*
  • Female
  • Hand Deformities, Congenital / diagnostic imaging
  • Hand Deformities, Congenital / pathology*
  • Humans
  • Infant
  • Male
  • Phenotype
  • Pierre Robin Syndrome / diagnostic imaging
  • Pierre Robin Syndrome / pathology*
  • Tomography, X-Ray Computed

Supplementary concepts

  • Richieri Costa Pereira syndrome