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Cloverleaf skull syndrome

MedGen UID:
98141
Concept ID:
C0432126
Disease or Syndrome
Synonyms: Isolated cloverleaf skull syndrome; KLEEBLATTSCHADEL; Kleeblattschaedel deformity syndrome; Kleeblattschaedel syndrome
SNOMED CT: Clover leaf skull deformity (254022009); Kleeblattschadel deformity (254022009); Cloverleaf skull syndrome (254022009)
 
Monarch Initiative: MONDO:0007861
OMIM®: 148800

Definition

Cloverleaf skull, or Kleeblattschaedel, consists of a trilobular skull with craniosynostosis. The condition shows pathogenetic variability and etiologic heterogeneity. The cause of isolated cloverleaf skull is unknown (Cohen, 2009). Cohen (1975) pointed out that Kleeblattschaedel is a component of many syndromes, e.g., it is found in some cases of Crouzon syndrome (123500), Pfeiffer syndrome (101600), and Carpenter syndrome (201000). Cohen (2009) listed 12 monogenic disorders with cloverleaf skull as a feature, including type II thanatophoric dysplasia (187601), which accounts for 40% of all cloverleaf skull syndromes. Cohen (2009) published photographs of cloverleaf skull in various syndromes. [from OMIM]

Clinical features

From HPO
Hydrocephalus
MedGen UID:
9335
Concept ID:
C0020255
Disease or Syndrome
Hydrocephalus is an active distension of the ventricular system of the brain resulting from inadequate passage of CSF from its point of production within the cerebral ventricles to its point of absorption into the systemic circulation.
Craniosynostosis syndrome
MedGen UID:
1163
Concept ID:
C0010278
Disease or Syndrome
Craniosynostosis refers to the premature closure of the cranial sutures. Primary craniosynostosis refers to the closure of one or more sutures due to abnormalities in skull development, and secondary craniosynostosis results from failure of brain growth.
Elbow ankylosis
MedGen UID:
592439
Concept ID:
C0409477
Disease or Syndrome
Cloverleaf skull
MedGen UID:
348010
Concept ID:
C1860050
Congenital Abnormality
Trilobar skull configuration when viewed from the front or behind.
Proptosis
MedGen UID:
41917
Concept ID:
C0015300
Disease or Syndrome
An eye that is protruding anterior to the plane of the face to a greater extent than is typical.
Recurrent corneal erosions
MedGen UID:
56353
Concept ID:
C0155119
Disease or Syndrome
The presence of recurrent corneal epithelial erosions. Although most corneal epithelial defects heal quickly, some may show recurrent ulcerations.

Professional guidelines

PubMed

Tubbs RS, Sharma A, Griessenauer C, Loukas M, Shoja MM, Watanabe K, Oakes WJ
Childs Nerv Syst 2013 May;29(5):745-8. Epub 2012 Dec 12 doi: 10.1007/s00381-012-1981-8. PMID: 23233211

Recent clinical studies

Etiology

Tcherbbis Testa V, Jaimovich S, Argañaraz R, Mantese B
Acta Neurochir (Wien) 2021 Nov;163(11):3083-3091. Epub 2021 Sep 27 doi: 10.1007/s00701-021-04980-3. PMID: 34570275

Diagnosis

O'Keefe M, Algawi K, Fitzsimmon S, Early M, Kennedy S, Knight-Nanan D
J Pediatr Ophthalmol Strabismus 1998 Sep-Oct;35(5):292-3. doi: 10.3928/0191-3913-19980901-11. PMID: 9782442
Ito S, Matsui K, Ohsaki E, Goto A, Takagi K, Koresawa M, Ito S, Sekido K, Suzuki M, Torikai K, Aida N
Brain Dev 1996 Jul-Aug;18(4):307-11. doi: 10.1016/0387-7604(96)00020-4. PMID: 8879651

Prognosis

Ito S, Matsui K, Ohsaki E, Goto A, Takagi K, Koresawa M, Ito S, Sekido K, Suzuki M, Torikai K, Aida N
Brain Dev 1996 Jul-Aug;18(4):307-11. doi: 10.1016/0387-7604(96)00020-4. PMID: 8879651
Thompson DN, Hayward RD, Harkness WJ, Bingham RM, Jones BM
J Neurosurg 1995 Jun;82(6):1071-4. doi: 10.3171/jns.1995.82.6.1071. PMID: 7760182
Partington MW, Gonzales-Crussi F, Khakee SG, Wollin DG
Arch Dis Child 1971 Oct;46(249):656-64. doi: 10.1136/adc.46.249.656. PMID: 5315768Free PMC Article

Recent systematic reviews

Tcherbbis Testa V, Jaimovich S, Argañaraz R, Mantese B
Acta Neurochir (Wien) 2021 Nov;163(11):3083-3091. Epub 2021 Sep 27 doi: 10.1007/s00701-021-04980-3. PMID: 34570275

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