A congenital nonsyndromic abnormality with a
characteristic trilobular aspect of the skull.
Airway management was very difficult in this 14-month-old boy with
Three-dimensional reconstruction of the cranial computed tomography
scan of the same boy gives a better impression of the underlying anomaly in
Craniosynostosis occurs in approximately 1:2,100 live
births in the general population. Cloverleaf skull (CLS) accounts for less
than 1% of cases.
All reported cases have been
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 Syndromes).
Based on the clinical findings of trilobular skull
aspect and craniosynostosis in radiologic examinations (conventional
radiography, computed tomography scanning, and magnetic resonance imaging).
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 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.
The presence of raised intracranial
pressure 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.
Patients may have increased intracranial
pressure. On the one hand, this situation makes a rapid-sequence induction
(increased risk of vomiting) for tracheal intubation with controlled ventilation desirable. On the other
hand, maintenance of spontaneous ventilation may be required until the
airway has been secured if airway management is expected to be difficult.
The final decision depends on the clinical situation. Careful eye protection
is required because of increased risk for corneal ulceration secondary to
proptosis. Careful intraoperative positioning is needed because of
Succinylcholine and ketamine should be
avoided if possible in the presence of already increased intracranial pressure.
Cloverleaf Skull and Bone Dysplasia: CLS in combination with genital
and chest anomalies that could increase respiratory risks.
Kozlowski-Warren-Fisher Syndrome: Lethal form of CLS with genital ...