Hungarian Radiology

[Cheirospondyloenchondromatosis]

IGNYS Anna, MALGORZATA Krajewska-Walasek, MARIKOVA Olga, IVO Marik, KAZIMIERZ Kozlowski

AUGUST 20, 2003

Hungarian Radiology - 2003;77(04)

[The term of cheirospondyloenchondromatosis (CHE) was coined by Spranger et al. This generalised, distinctive form of enchondromatosis is characterised by mild to moderate dwarfism, short hands and feet with beaded fingers, prominent large joints and frequently mental deficiency. Major radiographic features include generalised mild platyspondyly, generalised enchondromatosis with marked involvement of hands and feet and small ilia with eroded crests and acetabular roofs. We report three patients with this rare, severe form of enchondromatosis and stress some differences between our patients and the classical description of Spranger et al.]

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