PubMed Health⌕ Search

PubMed · 6828591

A morphogenetic classification of craniofacial malformations.

Abstract

A new classification of malformations of the face and cranium is proposed, based on embryologic studies and observations concerning a great number of patients seen by the authors. First of all, one should distinguish between cerebral craniofacial (with brain and/or eyes involved) and craniofacial malformations. Craniofacial malformations may be characterized by dysostosis and by synostosis. Malformations with dysostosis may be produced by transformation as well as differentiation defects. Synostosis is always caused by a differentiation defect. A new nomenclature is introduced.

Explore related subjects

Keep this discovery

Explore connections, maps & timelines

BibTeXRIS

J C van der Meulen, R Mazzola, C Vermey-Keers, M Stricker, B Raphael. 1983. A morphogenetic classification of craniofacial malformations.. https://doi.org/10.1097/00006534-198304000-00022

Cite the original work for its findings. Save a collection to share your selection of sources.

KEEP EXPLORING

Related citations

First International Meeting on "Stem Cell Applications in the Craniofacial Region".

During the 1st International Meeting on "Stem Cell Applications in the Craniofacial Region" promoted in Naples (Italy), invited researchers presented theirwork and the most innovative methods regarding stem cells (SCs) and their application to the craniofacial region of the human body. In addition, some researchers showed their case-reports on craniofacial reconstruction using either osteo-distraction or reconstruction surgical methods. The aim of this biannual meeting is to stimulate discussion, improve knowledge and promote scientific collaboration among basic and clinical scientists in the main topics of SC use in therapy. A summary of this meeting is given.

Craniofacial Dysostosis↗

Crouzon syndrome: phenotypic signs and symptoms of the postnatally expressed subtype.

In a retrospective study, the characteristics of a group of patients (n = 9) with a postnatally expressed Crouzon syndrome were described. Although they do not always display the physical signs of craniosynostosis, such patients are highly at risk of developing symptoms secondary to multiple suture synostosis. By reviewing the hospital files, radiographs, and three-dimensional computed tomography scans of these patients, it was possible to describe the pattern of suture obliteration chronologically. Furthermore, certain phenotypic signs and symptoms such as skull shape and development of digital impressions, a bulge at the bregma, and intracranial hypertension were inventoried as well as patients' genotypes. Interestingly, ossification started at the lambdoid sutures in at least four patients and most likely in three additional ones. The coronal sutures were the last to ossify in at least three of the patients. Various skull shapes were encountered. Furthermore, all nine patients developed digital impressions, starting occipitally in eight of them. Seven patients developed a bulge at the bregma, and four of them exhibited intracranial hypertension. The genotype varied in our patients. To recognize patients with postnatal Crouzon syndrome as soon as possible, special attention must be paid to 1) occipital development of digital impressions and/or ossification of sutures, 2) development of a prominent bregma, 3) development of intracranial hypertension, and/or 4) progressive characteristic "crouzonoid" features. Such patients can be considered as representing a subtype of Crouzon syndrome. To prevent or treat intracranial hypertension and/or loss of vision, surgical intervention should be performed at the onset of progressive craniosynostosis between 1 and 2 years of age.

Craniofacial Dysostosis↗