Scheuthauer-Marie-Sainton syndrome: a case report

The term Scheuthauer-Marie-Sainton syndrome is also known as cleidocranial dysplasia or cleidocranial dystosis and is derived from ancient greek words cleido (collar bone), knanion (head) and dysplasia (abnormal formation). It is an uncommon but well known genetic skeletal condition and an autosomal dominant malformation affecting bones and teeth. The most common skeletal and dental abnormalities in affected individuals are hypoplastic/ aplastic clavicles, open frontanelles, short stature, retention of primary teeth, delayed eruption of permanent teeth, supernumerary teeth and multiple impacted teeth. Affected person have a characteristic facial appearance with a bulky forehead, hypertelorism and midfacial hypoplasia. General health is usually good and the intellect is unimpaired. This article describes clinical and radiographic features of Scheuthauer-Marie-Sainton syndrome in a 29 years old male patient

Cleıdocranıal Dysplasıa: A Rare Skeletal Dysplasıa

ABSTRACT Cleidocranial dysplasia (CCD) is a rare autosomal dominant skeletal dysplasia. Hypoplastic clavicles, delayed closure of the cranial sutures, dental abnormalities, and short stature are main features in this syndrome. RUNX2 is the responsible gene for CCD. Here we reported two cases with CCD; they are admitted to clinic for short stature and dental abnormalities. We report these cases to increase the awareness of clinicians

The Radiologic Comparison of Operative Treatment Using a Hook Plate versus a Distal Clavicle Locking Plate of Distal Clavicle Fracture

BACKGROUND: The purpose of this study was to compare the radiologic results of patients who underwent surgery with a hook plate and a locking plate in distal clavicle fractures. METHODS: Sixty patients underwent surgical treatment for Neer type IIa, IIb, III, and V distal clavicle fracture. Twenty-eight patients underwent fracture fixation with a hook plate and 32 with a locking plate. Coracoclavicular distance was measured on standard anteroposterior radiographs before and after the surgery, and union was confirmed by radiograph or computed tomography taken at 6 months postoperatively. Other radiologic complications like osteolysis was also checked. RESULTS: Bony union was confirmed in 59 patients out of 60 patients, and 1 patient in the hook plate group showed delayed union. Coracoclavicular distance was decreased more in the hook plate group after surgery (p < 0.01). After 6 weeks of the hook plate removal, the coracoclavicular distance was increased a little compared to before metal removal, but there was no difference compared to the contralateral shoulder. Eleven out of 28 patients (39.3%) showed osteolysis on the acromial undersurface in the hook plate group. CONCLUSIONS: Both the hook plate group and the locking plate group showed satisfactory radiologic results in distal clavicle fractures. Both hook plate and locking plate could be a good treatment option if it is used in proper indication in distal clavicle fracture with acromioclavicular subluxation or dislocation.

Operative versus nonoperative treatment for displaced midshaft clavicle fractures: a meta-analysis based on current evidence

Literature searches of the Cochrane Library, PubMed, EMBASE, Web of Science, LILACS, China National Knowledge Infrastructure, and Wanfang Data databases were performed from 1966 to September 2014. Only randomized and quasi-randomized controlled clinical trials comparing operative and nonoperative treatments for displaced midshaft clavicle fractures were included. Data collection and extraction, quality assessment, and data analyses were performed according to the Cochrane standards. Thirteen studies were considered in the meta-analysis. Constant scores and the Disabilities of the Arm, Shoulder and Hand scores were improved in the operative fixation group at a follow up of one year or more. The nonunion and symptomatic malunion rates were significantly lower in the operative group. Additionally, the nonoperative group had a higher likelihood of neurological symptoms compared with the operative group. A significantly higher risk of complications was found in patients treated conservatively than in those who underwent operative fixation. However, when patients with nonunion and symptomatic malunion were excluded from the analysis, no significant differences in the complication rate were found. We concluded that based on the current clinical reports, operative treatment is superior to nonoperative treatment in the management of displaced midshaft clavicle fractures. However, we do not support the routine use of primary operative fixation for all displaced midshaft clavicle fractures in adults.

Cleidocranial dysostosis: a report on two familial cases / Disostose cleidocraniana: relato de dois casos familiares

Cleidocranial dysostosis is a rare genetic syndrome with an autosomal dominant inheritance pattern. The most common manifestations include clavicular aplasia or hypoplasia, open fontanelles and abnormal dentition. The present report describes two familial cases whose late diagnosis was made by means of clinical and radiographic findings. The treatment was radical, with complete surgical teeth extraction and making of total dental prosthesis.

Cleidocranial dysplasia with spina bifida: a case report / Displasia cleido-craniana com espinha bífida: relato de caso

OBJECTIVE: To present and discuss a case of a rare disease in a 35 year old otherwise healthy maleIndian in origin reported to the Department of Oral Medicine and Radiology of the Dental College andResearch Institute, Bangalore, India. DISCUSSION: The cleidocranial dysplasia is a rare disease whichcan occur either spontaneously (40%) or by an autosomal dominant inheritance. The dentists are, mostof the times, the first professionals who patients look for to solve their problem, since there is a delayin the eruption and /or absence of permanent teeth. In the present case multiple missing teeth was thereason for patient’s visit to odontologist. CONCLUSION: An early diagnosis allows proper orientationfor the treatment, offering a better life quality for the patient.

OBJETIVO: Apresentar e discutir um caso de doença rara em paciente masculino, de 35 anos de idade, sadio, demodo geral, de origem indiana, que foi encaminhado ao Departamento de Medicina Bucal e Radiologia da Escola deOdontologia e Instituto de Pesquisa, Bangalore, Índia. DISCUSSÃO: A displasia cleidocraniana é uma doença raraque pode ocorrer espontaneamente (40%) ou por herança autossômica dominante. O cirurgião-dentista é, na maioriadas vezes, o primeiro profissional que o paciente procura para solução de seu problema, uma vez que há demora na erupção e/ou ausências de dentes permanentes. No presente caso, múltiplos dentes ausentes foram a razão para opaciente visitar o dentista. CONCLUSÃO: O diagnóstico precoce permite a orientação adequada para o tratamento,visando melhor qualidade de vida ao paciente.

Cleidocranial dysplasia: a dilemma in diagnosis?

Cleidocranial dysplasia is a developmental anomaly and is characterized by craniofacial and skeletal malformations as well as the presence of numerous supernumerary and unerupted teeth. A 16-year-old patient presented with an unaesthetic facial appearance due to unerupted front teeth. General examination showed the absence of the clavicles, a brachiocephalic skull with frontal bossing and a depressed nasal bridge with concave facial profile. Radiographic examination showed multiple supernumerary teeth, rudimentary clavicles with a bell shaped rib cage and an open sagittal suture. A final diagnosis of cleidocranial dysplasia was made and comprehensive management was planned which involved multi disciplinary approach. The role of dentist is vital in the early diagnosis and management of such cases which require interdisciplinary cooperation for better results.