Learning objectives
The purpose of my educational exhibit is:
- to introduce readers to the one of the most common craniosynostosis,
- to describe genetic cause of crouzon syndrome,
- to familiarize radiologists with the characteristics of Crouzon syndrome.
Background
Crouzon syndrome is characterized by pre-mature obliteration and ossification of sutures in an infant skull (craniostenosis) assosciated with midfacial hypoplasia and orbital deformities.
The term cranisostenosis was introduced by the great German pathologist Virchow in 1851.He discovered that the skull growth is arrested in perpendicular direction to the closer suture and compensatory overexpansion takes place in the parallel direction to the closer suture.
He also was the first person to classify the different types of skull deformity seen in persons with craniosynostosis and to introduced...
Findings and procedure details
The most notable characteristic of Crouzon syndrome is craniostenosis,
a deformity of the skull that results from premature closure of the sutures between the cranial bones.
The precise cause of premature sutural closure has not been fully clarified.
In the normal patient,
sutures close when the growth of the brain stops.
Recent molecular biologic studies suggest that interactions of fibroblast growth factors (FGFs) and their receptors (FGFR) influence the balance between the proliferation of osteoprogenitor cells and the differentiation of new bone in the membranous...
Conclusion
The appearance of a patient with Crouzon syndrome can vary in severity from a mid presentation with subtle midface characteristic to a severe form with a multiple cranial suture fusion and marked midface hypoplasia and eye problems.
This craniosynostosis syndrome consist of a spectrum of clinical problems .
Overall knowledge of the natural history,
characteristic findings,
and clinical problems of Crouzon syndrome are mandatory.
These patients should be treated by a multidisciplinary team.
Surveillance for hydrocephalus and other possible complications in those at increased risk...
Personal information
Zuzanna Grodzicka
Wojewódzki Szpital Specjalistyczny in Olsztyn,
Department of Radiology
Olsztyn/Poland
In this poster I used materials from Wojewódzki Specjalistyczny Szpital Dziecięcy in Olsztyn,
Pracownia Rezonansu Magnetycznego Spectro and AffideaOlsztyn.
References
1Virchow R: Uber den Certinismus,
namentlich in Franken,and iuber pathologische Schadelformen.
Verh Phys Med GesellschWurzburg 2:231-284,
1851
2Harold Chen,
Bruce Buehle,
Mary L Windle,
David Flannery,
Paul D Petry,
Genetics of Crouzon Syndrome(Medscape)
3Ahmed I,
Afzal A.
Diagnosis and evaluation of Crouzon syndrome.
J Coll Physicians Surg Pak.
May 2009;19(5):318-20.(Medline)
4Genetic home references gene FGFR2,
crouzon syndrome,
Crouzonodermoskeletal syndrome www.ghr.nlm.nih.gov
5 CohenMM; KreiborgS Birth prevalence studies of the Crouzon syndrome: comparison of direct and indirect methods.
Clin Genet.
1992;41(1):12-5
6Online Mendelian Inheritance in Man (OMIM)...
