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Scintigraphic manifestations of "sternal cupping"
B J Barron1, L Robinson, H D Tran
1Department of Radiology, University of Texas Health Science Center at Houston.
Insights
Sternal abnormalities like "sternal cupping" and hypo-ossification are rare in sickle-thalassemia. This study highlights two infants with these findings, one also having Wolf-Hirschhorn syndrome.
Area of Science:
- Pediatric Hematology
- Medical Imaging
- Genetics
Background:
- Sternal abnormalities are documented in sickle-cell anemia but not typically in sickle-thalassemia.
- Sickle-cell disease encompasses various genetic forms, including sickle-cell anemia and sickle-thalassemia.
Observation:
- Two infants with complicated sickle-cell disease presented with unusual sternal abnormalities.
- One infant had sickle-thalassemia with "sternal cupping" and transient hypo-ossification.
- The second infant exhibited persistent sternal hypo-ossification and "sternal cupping," alongside a 4P-chromosomal defect (Wolf-Hirschhorn syndrome).
Findings:
- Radiographic evidence of "sternal cupping" and hypo-ossification of the sternum was observed.
- Transient hypo-ossification was noted in the infant with sickle-thalassemia.
- Persistent hypo-ossification was a key feature in the infant with Wolf-Hirschhorn syndrome and sickle-cell disease.
Implications:
- These cases expand the understanding of sternal manifestations in sickle-cell disease, particularly in sickle-thalassemia.
- The findings suggest a potential association between sternal abnormalities, sickle-cell disease, and chromosomal defects.
- Further research is warranted to elucidate the mechanisms and clinical significance of these rare sternal findings.
Abstract:
Sternal abnormalities in sickle-cell disease have been documented by bone scintigraphy and radiography in patients with homozygous sickle-cell anemia, but not in patients with sickle-thalassemia. We present here two unusual cases of sternal abnormalities in complicated sickle-cell disease. One is an infant with radiographic findings of "sternal cupping" and transient hypo-ossification of the sternum and sickle-thalassemia. The other patient is also a male infant with unusual, persistent under-ossification of bone, in association with radiographic findings of "sternal cupping." The second patient also had a 4P-chromosomal defect (Wolf-Hirschhorn syndrome) in which sternal hypo-ossification was described.