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At a glance

A congenital nonsyndromic abnormality with a characteristic trilobular aspect of the skull.

Synonyms

Kleeblattschädel; Holtermueller-Wiedemann Syndrome; Holtermüller-Wiedemann Syndrome.

Incidence

Craniosynostosis occurs in approximately 1:2,100 live births in the general population. Cloverleaf skull (CLS) accounts for less than 1% of cases.

Genetic inheritance

All nonsyndromic reported cases have been sporadic.

Pathophysiology

The abnormally shaped skull is caused by premature ossification of the coronal, sagittal, and lambdoid sutures. The metopic and squamous sutures do not fuse. It has been hypothesized that abnormal development of the periosteal vascular plexus alters the ossification and development of the skull. CLS may occur as an isolated finding, but is more commonly associated with ☞Thanatophoric Dwarfism, ☞Achondrogenesis, and Camptomelic Dysplasia. It is also seen in ☞Acrocephalopolysyndactyly (Carpenter Syndrome), and ☞Acrocephalosyndactyly Syndromes (Apert, Pfeiffer, and Crouzon Syndrome).

Diagnosis

Based on the clinical findings of trilobular skull aspect and craniosynostosis in radiologic examinations (conventional radiography, CT- or MRI-scanning).

Cloverleaf Skull Syndrome: Airway management was very difficult in this 14-month-old boy with cloverleaf skull.

Clinical aspects

Neonatal death is frequent. Patients may have mental retardation. On examination, the skull has a trilobed appearance with high forehead, beaked nose, and depressed premaxillary region. Hydrocephalus is common. Raised intracranial pressure (ICP) is seen in approximately 45% of patients with complex craniosynostoses. Other features involve the eyes (marked proptosis, exophthalmos, corneal ulcerations) and the limbs (elbow ankylosis, dysostosis multiplex, syndactyly of toes and fingers, restricted joint mobility). Abnormal vertebral size has occasionally been reported.

Precautions before anesthesia

The presence of raised ICP should be determined. If CLS is part of a syndrome or skeletal dysplasia, the patient should be investigated for features of that condition. Evaluate for difficult airway management.

Anesthetic considerations

The anesthetic management should take increased ICP into consideration and aim at maintaining an appropriate cerebral perfusion pressure while at the same time avoiding further increases in ICP, prevent seizures and hyperthermia. On the one hand, this situation makes a rapid-sequence induction for tracheal intubation with controlled ventilation indicated (due to increased risk of vomiting). On the other hand, if difficult airway management is expected, maintenance of spontaneous ventilation is recommended until the airway has been secured. Alternative airway management options should be immediately available (eg, supraglottic airway device, fiberoptic bronchoscope) and adherence to an established difficult airway algorithm is advised. A surgeon familiar with surgical airway management and the necessary equipment should easily be available in the operating room. Alternatively, an awake fiberoptic intubation should be considered. The final decision about which approach is favored depends on the clinical ...

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