Cloverleaf Skull

Kleeblattschadel is the German term used, by Karl Holtermiiller and Hans Wiedemann’ in 1960, to describe a cloverleaf-shaped deformity of the skull. They described one case of their own and reviewed the findings in 12 other cases. These authors used the term kleeblattschadel (cloverleaf skull) to describe a well-defined syndrome. The main features were: ‘(1) cloverleaf deformity, the cardinal symptom; (2) facial malformations involving the orbits, nose and upper maxilla; (3) occasionally, chondrodysplastic changes affecting the limbs and the spine; (4) severe hydrocephalus; and (5) poor prognosis due to progressive intracranial hypertension.’ ² A cloverleaf-shape of the skull (Figure 1) can be seen in several other conditions, especially thanatophoric dysplasia and the craniostenoses ² . ‘The deformity is due to severe alterations in skull development with premature synostosis of some cranial sutures associated with marked hydrocephalus, resulting in a striking bulging of the head upward in the region of the anterior fontanel and laterally in the temporal regions, the latter forming the lateral lobes of the cloverleaf.’ ² Figure 1 Skull radiograph showing cloverleaf deformity. Case courtesy of Dr A. Campbell, University of Maryland https://s3-euw1-ap-pe-df-pch-content-public-p.s3.eu-west-1.amazonaws.com/9781003076568/34c29fd5-5662-4e8f-b58c-badd7fa5d724/content/fig15_1_B.jpg"/>