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Considerations about the cloverleaf skull.

R Dambrain1, M Freund, G Verellen

  • 1Unité d'Anatomie humaine, Université Catholique de Louvain, Bruxelles, Belgique.

Journal of Craniofacial Genetics and Developmental Biology
|January 1, 1987
PubMed
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This study examines cloverleaf skull malformations in Pfeiffer syndrome and thanatophoric dysplasia, revealing premature suture closure and abnormal bone resorption as key factors in skull shape.

Area of Science:

  • Medical Genetics
  • Developmental Biology
  • Skeletal Dysplasias

Background:

  • Cloverleaf skull is a rare, severe congenital anomaly characterized by a trilobular skull shape.
  • It is often associated with craniosynostosis and other skeletal abnormalities.
  • Understanding the underlying mechanisms is crucial for diagnosis and management.

Purpose of the Study:

  • To investigate the morphological and etiological aspects of cloverleaf skull in specific genetic conditions.
  • To correlate clinical and radiological findings with microradiographic analysis.
  • To elucidate the pathogenesis of the trilobular skull appearance.

Main Methods:

  • Clinical and radiological examinations of four cases (two fetuses, one infant, one young adult) with cloverleaf skull.

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  • Analysis included two cases of Pfeiffer syndrome, one of thanatophoric dysplasia, and one isolated case.
  • Microradiographic analysis of nondemineralized bone sections.

    • Main Results:

    • Malformations observed in the calvarium, skull base, and orbits, with associated limb abnormalities in some cases.
    • Premature closure of the temporoparietal sutures restricts lateral skull growth.
    • Extension of synostosis to coronal and frontal sutures, along with occipital bone thickening, inhibits skull lengthening.
    • Compensatory patency of sagittal and lambdoid sutures during the neonatal period.
    • Microradiography suggests a potential vascular origin and abnormal osteoclastic resorption.

    Conclusions:

    • Premature suture closure and abnormal bone remodeling are critical in the development of the cloverleaf skull.
    • The findings suggest a complex interplay of genetic factors, vascular influences, and bone resorption in this condition.
    • Further research into the vascular etiology and osteoclastic activity is warranted.