Double-layered manubrium sterni in young children with diastrophic dysplasia

G Currarino1

  • 1Department of Radiology, Texas Scottish Rite Hospital for Children, Dallas 75219, USA.

Pediatric Radiology
|July 6, 2000
PubMed

Insights

Diastrophic dysplasia can cause a rare manubrium anomaly in children, presenting as a double-layered sternum in infants and fusion in older children. This may stem from persistent embryonic midline structures.

Area of Science:

  • Pediatric Orthopedics
  • Skeletal Dysplasias
  • Embryology

Background:

  • Diastrophic dysplasia is a rare skeletal disorder affecting bone and cartilage development.
  • The sternum, particularly the manubrium, has a complex ossification process.
  • Anomalies of the manubrium are infrequently reported in pediatric populations.

Purpose of the Study:

  • To describe a specific sternal anomaly observed in children with diastrophic dysplasia.
  • To investigate the potential embryological origins of this manubrium abnormality.

Main Methods:

  • Retrospective review of lateral sternum roentgenograms.
  • Analysis of 12 pediatric patients diagnosed with diastrophic dysplasia.
  • Correlation of radiographic findings with patient age.

Main Results:

  • Four children (2 newborns, 2 aged 1.5 years) exhibited a double-layered manubrium with ventral accessory ossification.
  • Two older children (aged 5 and 11.5 years) showed manubrium deformity suggesting prior fusion of a ventral ossification center.
  • The anomaly appears to be age-dependent, with fusion occurring over time.

Conclusions:

  • A double-layered manubrium may be an unrecognized feature of diastrophic dysplasia.
  • The findings suggest a possible persistence and ossification of a primitive embryonic midline structure.
  • Further research into sternal embryology may clarify the etiology of this anomaly.

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