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Hemifacial microsomia and hypodontia: a case report.

A 6-year-old girl with hemifacial microsomia was examined. Abnormalities found were: severely malformed pinna of the right ear, right malar hypoplasia and unilateral mandibular hypoplasia. Dental examination revealed normal intraoral soft tissues with all deciduous teeth present. Radiographic examination disclosed that the maxillary and mandibular right and left second premolars were not developing. The mandibular ramus was short in length and the mandibular condyle had not developed on the right. The patient had no renal, cardiac or skeletal anomalies and her hearing was normal. No previous publications have been located which report the association of hypodontia and hemifacial microsomia.

Anodontia↗

CREST syndrome: clinical manifestations and dental management.

Scleroderma is a multisystem connective tissue disease of unknown etiology, which is characterized by inflammation, vascular, and fibrotic changes of the skin as well as various internal organs. This article will review the systemic form of scleroderma known as CREST syndrome, its clinical manifestations including the dentofacial complications, as well as provide information to assist the prosthodontist in managing the dental treatment for patients with this disease.

CREST Syndrome↗

Clinical evaluation of patients with epidermolysis bullosa: review of the literature and case reports.

Epidermolysis bullosa (EB) is a relatively rare inherited disorder, which includes blister and vesicle formation on the skin and mucous membranes as a result of trauma or heat. There are different forms of this disorder. Mild manifestations are relatively uncomfortable, usually involving the knees, elbows, and fingers. Severe forms of this disease compromise normal functioning of multiple organs, which may result in premature death. The lack of a specific treatment to cure EB makes genetic counseling and prenatal diagnosis of primary importance to control this disorder. Three case histories of persons with dystrophic recessive epidermolysis bullosa are reported, focusing on appropriate dental care for patients with EB.

Anemia↗

Oral rehabilitation of a patient with scleroderma associated with systematic lupus erythematosus: a case report.

This case report presents a patient who had a limited oral opening due to systematic lupus erythematosus (SLE) and describes how oral rehabilitation was achieved with a removable partial denture. Various techniques for the delivery of oral health care have been reported for patients who have limited mouth openings as a result of SLE and scleroderma. After a review of the literature, a new approach was required for this patient with SLE who had a very limited mouth opening. The retention for the removable partial denture (RPD) used the undercut in the abutment teeth and a magnet-retained sectional RPD. A set of tweezers was modified to help with the insertion and removal of the sectional RPD by the patient. This paper reports a new clinical and laboratory technique of fabricating a sectional RPD using magnets and customized tweezers for this patient.

Adult↗

Freeman-Sheldon syndrome: a disorder of congenital myopathic origin?

Freeman-Sheldon syndrome was diagnosed in an unrelated adult man and woman, with severe abnormalities of the extremities but only slight anomalies of the face. Electromyography and muscle biopsy showed a myopathy which was classified as a congenital disproportion of fibre type and seemed to be the primary cause of the deformities. This allowed classification of the syndrome as a separate type of myopathic arthrogryposis.

Adult↗

The Ohdo blepharophimosis syndrome: a third case.

A patient with a syndrome consisting of blepharophimosis, simple ears, hypoplastic teeth, developmental delay, and hypotonia is described. Previous case reports are reviewed and a differential diagnosis is described. Many of the features in the subject are similar to those described in two previous reports and they constitute a distinct syndrome.

Abnormalities, Multiple↗

Radiological assessment of hemifacial microsomia by three-dimensional computed tomography.

Hemifacial microsomia (HFM) is the commonest asymmetrical craniofacial deformity leading to underdevelopment of the craniofacial skeleton and soft tissues on the affected side. Timing of reconstructive surgery is based on a comprehensive analysis and classification of the deformity. Lateral cephalometric radiographs are of limited value because of superimposition of normal and abnormal bony structures. The skeletal and soft-tissue findings of six patients with HFM who underwent three-dimensional (3D) CT are presented. This method allows a comprehensive assessment of the deformity at a low radiation dose, 22% of that of a conventional CT scan, by the use of a low dose technique.

Adolescent↗

Transmigration of a maxillary canine. A case report.

In the oral cavity, transmigration is defined as a tooth that crosses the mid-line by more than half its length. Following extensive literature review, it was found that, although well documented with respect to mandibular canines, there were only two publications detailing maxillary cuspid transmigration. This report presents a case of transmigration where the left maxillary canine gradually crossed the mid-line and migrated to the right side in a patient with hemifacial microsomia and cleft palate. The Mupparapu classification of the migratory pattern of the mandibular canines is discussed. Various clinical considerations, as well as guidelines for general practitioners to diagnose and manage such a condition, are also discussed.

Child↗

Agnathia and associated malformations: a case report.

A case of a rare first branchial arch anomaly with severe hypoplasia of the mandible and fusion between the mandible and adjacent bones is presented. The patient also had intracranial, cardiac, and acral deformities. The craniofacial malformations may reflect incomplete separation of the first branchial arch into its maxillary and mandibular processes. The association between the craniofacial and other corporal anomalies is unknown.

Branchial Region↗

Measurement of facial soft tissue mobility in man.

OBJECTIVE: The assessment of facial mobility is a key element in the treatment of patients with facial motor deficits. In this study, we explored the utility of a three-dimensional tracking system in the measurement of facial movements. METHODS AND RESULTS: First, the three-dimensional movement of potentially stable facial soft-tissue, headcap, and dental landmarks was measured with respect to a fixed space frame. Based on the assumption that the dental landmarks are stable, their motion during a series of standardized facial animations was subtracted from that of the facial and headcap landmarks to estimate their movement within the face. This residual movement was used to determine which points are relatively stable (< or = 1.5 mm of movement) and which are not (> or = 1.5 mm of movement). Headcap landmarks were found to be suitable as references during smile, cheek puff, and lip purse animations, and during talking. In contrast, skin-based landmarks were unsuitable as references because of their considerable and highly variable movement during facial animation. Second, the facial movements of patients with obvious facial deformities were compared with those of matched controls to characterize the face validity of three-dimensional tracking. In all instances, pictures that appear to be characteristic of the various functional deficits emerged. CONCLUSIONS: Our results argue that tracking instrumentation is a potentially useful tool in the measurement of facial mobility.

Adolescent↗

Euclidean distance matrix analysis of surgical changes in prepubertal craniofacial microsomia patients treated with an inverted L osteotomy.

OBJECTIVES: Correction of craniofacial microsomia (CFM) presents several challenges concerning the modality of surgical intervention. The aim of this study was to assess early and late surgical outcome, by undertaking Euclidean distance matrix analysis (EDMA) of CFM patients exhibiting an unilateral mandibular deformity that was surgically corrected by an inverted L osteotomy and autogenous bone graft. DESIGN: Longitudinal study. Preoperative, approximately =1-year postoperative and approximately 3-year postoperative assessments of 14 consecutive children (mean age 9 years) with CFM. Posteroanterior cephalographs were scanned and five homologous mandibular landmarks were digitized in triplicate (< 1% digitization error). Average mandibular geometries, scaled to an equivalent size, were generated using a generalized rotational fit program (Procrustes superimposition) and subjected to EDMA. RESULTS: The mean pre- and both postoperative mandibular configurations differed statistically (p < .01). Early postoperative improvements in mandibular form were noted; increases in length arising in the treated mandibular body (approximately =19%) and ramus (approximately =13%). Comparing early and late postoperative configurations, a decrease of approximately =22% in the late postoperative mandibular body length was evident, but the ramus maintained steady vertical growth (approximately =7%). Comparing the preoperative and late postoperative configurations, the decrease observed in the mandibular body on the treated side was reduced to approximately =8% while the ramus maintained good growth (approximately =20%) on that side. CONCLUSION: Mandibular morphology is improved significantly in CFM patients surgically treated by an inverted L osteotomy, but relapse in the mandibular body is evident after approximately =3 years. Nevertheless, ramus growth proceeds well after the surgical reconstruction.

Adolescent↗

Freeman-Sheldon syndrome: dental and orthodontic implications.

The manifestations of the Freeman-Sheldon syndrome (FSS) in four members of a South African family of Xhosa stock have been documented. Orofacial manifestations are a major syndromic component and warrant early, specialized orthodontic intervention. Our protocol for dental management is outlined and suggestions for holistic oro-dental care are provided.

Adult↗