osteogenesis imperfecta type II (perinatal-lethal): spinal column ICD-10: Q78.0
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Neugeborenes Mädchen. Autopsiepräparat
Wirbelsäule:
- Verschmälerter Wirbelkörper mit massiv reduzierter Compacta
und rarefizierter, verschmälerter Spongiosa
- Spongiosabälkchen unregelmässig geformt und schlecht verkalkt
- Frakturen in der Kortikalis
- Knochenmark fibrosiert
New born girl (specimen from autopsy).
- A section of the spinal column is shown. Individual vertebral bodies are narrowed and show a markedly thinned and discontinuous corticalis.
Trabeculae are reduced in number, irregular and abnormally thin.
- Bone lamellation is increased with individual lamellae being thinner than normal.
- Osteocytes are crowded.
- Multiple organized fractures (Frakturen) with fibrosis (fibrosiert) of the bone marrow are observed.
Clinical findings: Osteogenesis imperfecta type II is uniformly fatal in utero or during the perinatal period. Multiple fractures are acquired
while the fetus is still in the womb.