45 results match your criteria cleido-cranial dysplasia


Cleidocranial Dysplasia in a 10-year-old Child: A Case Report.

Int J Clin Pediatr Dent 2019 Jul-Aug;12(4):352-355

Department of Pedodontics and Preventive Dentistry, Sathyabama University Dental College and General Hospital, Chennai, Tamil Nadu, India.

Cleidocranial dysplasia is a rare congenital anomaly characterized by multiple skeletal defects of which partial or complete absence of clavicles, delayed closure of fontanels with presence of open sutures and multiple wornian bones forms a striking feature. The oral manifestations are delayed exfoliation, delayed or failing eruption of the permanent dentition with multiple supernumerary teeth, protruding mandible and mid face retrusion. It is also known as Marie and Sainton's disease, mutational dystosis or cleidocranial dysostosis. Read More

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December 2019

Multiple, supernumerary retained teeth in the course of cleido-cranial dysplasia. A case report.

Dev Period Med 2015 Oct-Dec;19(4):503-7

Clinical Department of Maxillo-Facial Surgery, Provincial Specialist Hospital in Rzeszów, ul. Chopina 2, 35-005 Rzeszów, Phone 17 866 62 60, 605-547-070, e-mail:

Cleido-cranial dysplasia, often referred to as Scheuthauer-Marie-Sainton syndrome, is an autosomal dominant disorder of the musculo-skeletal system. Patients with cleido-cranial dysplasia are characterized by short stature, frequent varus or valgus hip, kyphoscoliosis, underdevelopment of the scapulas and the sternum, incorrect number of ribs. The most characteristic feature is unilateral or bilateral, partial or total underdevelopment of clavicles. Read More

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Oral manifestation of cleido cranial displasia.

Minerva Stomatol 2012 10;61(10):421-9

Department of Dentistry, Vita Salute University, San Raffele Hospital, Milan, Italy.

Cleidocranial dysplasia (CCD) is a congenital hereditary condition caused by a dominant autosomal mutation. The orthodontic management of CCD patients is often complicated by the manifestations associated with the condition. The aim of this study was to evaluate the oral manifestations of patients suffering from CCD. Read More

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October 2012

Control of tooth morphogenesis by Runx2.

Crit Rev Eukaryot Gene Expr 2006 ;16(2):143-54

Department of Cell and Developmental Biology, School of Dentistry and Dental Research Institute, Seoul National University, Seoul, Korea.

Odontogenesis is a complex process in which the interplay of signaling cascades of the epithelium and mesenchyme is critical. Evidence for the involvement of Runx2--a well-known osteogenic master transcription factor--in odontogenesis, has been accumulating. Haploinsufficiency of Runx2 in humans results in cleido-cranial dysplasia (CCD), characterized by supernumerary teeth; in Runx2-/- mice, molar odontogenesis does not proceed beyond the late bud stage. Read More

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Cleido cranial dysplasia: report of a family.

J Oral Sci 2004 Dec;46(4):259-66

Research Center, School of Dentistry, School of Medicine, Autonomous University of Mexico State, México.

A family case of Cleidocranial Dysplasia is presented. A mother and two adolescent girls were examined. In all three cases, a radiological series was performed over the entire body. Read More

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December 2004

[Cleido-cranial dysplasia].

Authors:
M Matsuo

Ryoikibetsu Shokogun Shirizu 2000 (30 Pt 5):279-80

International Center for Medical Research, Kobe University.

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January 2001

[Skull roof reconstruction in infants].

Ned Tijdschr Tandheelkd 1996 Jan;103(1):9-10

Afdeling Mond- en Kaakchirurgie, Academisch Ziekenhuis St. Radboud te Nijmegen, postbus 9101, 6500 HB Nijmegen.

In this article an introduction into craniofacial surgery in children is given based on the case histories of two patients (cleido-cranial dysplasia and cranio-synostosis). Read More

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January 1996

[Maxillofacial-dental anomalies in cleidocranial dysostosis].

Minerva Stomatol 1995 Oct;44(10):493-7

Corso di Laurea in Odontoiatria e Protesi Dentaria, Università degli Studi-Perugia.

The authors evaluated the dento-maxillofacial anomalies in cleido-cranial dysplasia; the use of a removable orthodontic appliance can solve orthodontic problems particularly the deficiency of anchorage. Read More

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October 1995

Cleido-cranial dysostosis--skeletal abnormalities.

Australas Radiol 1992 Aug;36(3):238-42

Department of Diagnostic Radiology, Banaras Hindu University, Varanasi, India.

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[The Pierre Marie-Sainton syndrome].

Rev Stomatol Chir Maxillofac 1985 ;86(2):103-6

Pierre Marie and Sainton syndrome, or cleido-cranial dysostosis is characterised by a triad: clavicular aplasia, delayed ossification of the fontanelles and sutures of the vault of the skull and hereditary transmission. To these may be added multiple dental inclusions - hence its interest in stomatology. The authors described a familial hereditary case affecting mother and son, the former with an incomplete syndrome and the latter with the complete picture of major dysostosis. Read More

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[Cleido-cranial dysostosis with malformation of the cervico-occipital junction].

Rev Neurol (Paris) 1982 ;138(4):327-36

A 66 years old man with cleido-cranial dysostosis suffered from a progressive ataxic gait for 45 years. Physical examination discovered cerebellar and pyramidal signs and altered deep sensibility in lower limbs. Roentgenograms showed skeletal dysostosis and a severe malformation of the cervico-occipital junction. Read More

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December 1982

[The association cleido-cranial dysplasia and a Capdepont dysplasia (author's transl)].

Rev Stomatol Chir Maxillofac 1978 ;79(1):53-9

The authors report a rare association: that of two hereditary dysplasias transmitted in a dominant manner. Cleido-cranial dysplasia or Pierre Marie and Saniton syndrome, a congenital polymorphous disease, affecting the bones during their growth with a Capadepont dysplasia or hereditary opalescent dentine, a condition affecting both dentitons. Read More

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September 1978

[Cleido-cranial dysplasia. Contribution to study of this disease on the basis of four cases (author's transl)].

Rev Stomatol Chir Maxillofac 1978 ;79(1):35-51

First described in 1898 under the name of cleido-cranial dysostosis by Pierre Marie and Sainton, this hereditary disease is highly polymorphous and progressive, hence the multiplicity of its manifestations. These involve not only the skull and clavicle, but also the hands, the spine and the teeth. In fact, it is disturbance of growth rather than a dysostosis in the strict sense of the term. Read More

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September 1978

[Cleido-cranial dysplasia--two observations].

Rofo 1978 Jan;128(1):60-6

The radiological changes of cleido-cranial dysplasia are described on the basis of two cases. Special attention is paid to the dental and facio-maxillary aspects. The main features are: (1) Supernumerary teeth, persistence of the first dentition, retention of the second dentition. Read More

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January 1978

[Cleido-cranial-pubic dysostosis (author's transl)].

An Esp Pediatr 1977 Jan;10(1):65-70

Four cases of cleido-cranial dysostosis asociated to pubic dysostosis are presented in its' two forms; sporadic and familiar. Some chromosomal anomalies which have been described by several authors in the last years are pointed out. Denomination of pubic-cleido-cranial dysostosis as more appropiated for this entity is proposed. Read More

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January 1977

Large electroencephalographic responses and their relationship to cleido-cranial dysplasia.

Can J Neurol Sci 1974 Nov;1(4):242-9

We have reported six individuals (five certain heterozygotes for cleido-cranial-dysostosis and one possible heterozygote) who have unusual EEG findings, consisting of very large responses to photic flash stimulation at very low stimulus rates. Such visual responses are extremely rare and have not been seen before in the experience of an EEG department over 12 years and they were not seen in 98 control subjects. It is likely that these responses are an irregular manifestation of the gene for cleido-cranial-dysplasia, and that the responses are independent of skull deformity. Read More

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November 1974

[Cleido-cranial dysostosis. Clinical aspects].

Actual Odontostomatol (Paris) 1974 Sep(107):413-39

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September 1974