Summary
A histological, histochemical and ultrastructural study of a case of the clover-leaf skull syndrome is presented. It involved pronounced trilobed deformation of the skull associated with synostosis of the lambdoidal and sagittal sutures, hydrocephalus, downward displacement of the ears, hypertelorism, exophthalmus, a sunken nasal root and antimongoloid slant of the eyes. Shortening and bending of the limbs, narrowing of the chest, and deformation of the feet completed the picture.
Histologically, evident abnormality of the cartilage was found both in the sphenoidal and vertebral bodies and in the chondro-osseous junctions of the ribs. These zones were not seriated and contained only rounded, seemingly degenerating, disarrayed chondrocytes. The intercellular matrix was strongly PAS-positive. It stained irregularly and in general less deeply than in normal subjects after treatment with Toluidine blue, Alcian blue and colloidal iron. Moreover, both the cartilage and bone matrix contained collagen fibrils of highly variable thickness. In both these matrices the process of calcification was irregular and slightly retarded.
It is suggested that the cloverleaf skull syndrome essentially depends upon a chondrodystrophic condition which involves the base of the skull and may be generalized, as in the present case, or limited to one skeletal area, as sometimes reported in the literature.
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Supported by a grant from the Italian Research Council (CNR).
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Bonucci, E., Nardi, F. The cloverleaf skull syndrome. Virchows Arch. Abt. A Path. Anat. 357, 199–212 (1972). https://doi.org/10.1007/BF00542847
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DOI: https://doi.org/10.1007/BF00542847