Cloverleaf Skull Syndrome (Kleeblattschädel Deformity): Findings on Cone Beam Computed Tomography

Background: Cloverleaf skull syndrome or Kleeblattschädel Abnormality involves an abnormal configuration of the calvarium, a rare presentation of craniosynostosis consisting of premature ossification of cranial sutures. Craniofacial dysostosis is encountered in different congenital malformations as well. Clinically and radiographically enlarged and trilobed cranium resulting from cranial and basal synostosis associated with hydrocephalus is the hallmark feature. A slew of other clinical and radiographic features is noted including upper airway compromise. Clinical and Radiographic Findings: The present report is aimed at describing radiographic features of this syndrome carried by a 12 year-old female, as visualized on maxillofacial cone-beam computed tomography (CBCT) study. In addition to the patient's clinical presentation of dysmorphic facial features, multiple cranial deformities status post craniotomies are reported. Conclusion: The relevance of imaging diagnosis and characterization of this syndrome must be emphasized in order to allow for proper planning and implementation of treatment as early as possible. The need for repeated surgical corrective maxillofacial procedures in a pediatric patient is to be noted as well. Statement of Clinical Relevance: Cloverleaf skull syndrome, the most severe form of craniosynostosis often associated with other abnormalities in the calvaria as well as in the skull base and face, is requiring the most complex multidisciplinary approach. A thorough radiographical evaluation is required to characterize the craniofacial bone deformities and guide the corrective treatment planning surgical procedures in a timely fashion in order to facilitate improvement in the prognosis of the affected children.