[MIM 148 800, 600 775]

(Kleeblattschaedel syndrome)

Very rare. Craniosynostosis involving the coronal and lambdoidal sutures producing a trilobe cloverleaf-shaped skull with:

-         exophthalmos and risk of lagophthalmos

-         a flattened nose

-         midface hypoplasia

-         hydrocephalus by stenosis of aqueduct of Sylvius

-         malformations of the brain or cerebellum

This syndrome can be observed alone or, more often, in association with:

-         the acrocephalosyndactylies: Crouzon (7 %), Pfeiffer or Carpenter syndrome

-         thanatophoric dwarfism type II ,

-   Jeune's syndrome

-         camptomelic dwarfism 

-         the sequence of uterine rupture

-         amniotic bands

Anesthetic implications: 

obstructive apneas; difficult manual  ventilation by mask and difficult intubation; intracranial hypertension

References : 

-        Angle CR, McIntire MS, Moore RC. 
Cloverleaf skull : Kleeblattschädel 
Am J Dis Child 1967 ; 114 : 198-202.

-          Iannaccone G, Gerlini G. 
The so-called”Cloverleaf skull” syndrome. 
Pediatr Radiol 1974; 2: 175-84.

-         Chen CP, Lin SP. 
A cloverleaf skull associated with Crouzon syndrome. 
Arch Dis Child  Foetal Neonatal Ed; 2006: F98

-         Meyer P, Renier D, Blanot S, Orliaguet G, Arnaud E, Lajeunie E. 
Anesthesia and intensive care of craniostenosis and craniofacial dysmorphism in children. 
Ann Fr Anesth Reanim 1997; 16:152-64.

-         Sculerati N, Gottlieb MD, Zimbler MS, Chibbaro PD.McCarthy JG. 
Airway management in children with major craniofacial anomalies. 
Laryngoscope 1998; 108:1806-12.

Updated: October 2019