Distraction in a case of otopalatodigital syndrome type II.

INTRODUCTION: Otopalatodigital syndrome type II is a rare X-linked recessive disorder with generalized skeletal dysplasia and hearing anomalies. Its features include conductive hearing loss, unusual facies, cleft palate, micrognathia, and overlapping flexed fingers and toes. It is a more lethal variant of otopalatodigital syndrome type I. There are many consistently reported craniofacial and dental findings; however, no case has been published in dental literature. CASE REPORT: We report a case of otopalatodigital syndrome type II with micrognathia, cleft of the soft palate, and partial anodontia, and discuss the combined orthodontic and surgical management. CONCLUSION: We also discuss the differential diagnosis and consider more recent theories on possible aetiology as well as clinical management strategies for such cases.

Long term results of mandibular distraction.

Mandibular distraction osteogenesis has become a popular surgical modality due to its many advantages over conventional orthognathic surgical procedures. However, in spite of the technique having been used for over 15 years, no concrete long term results are available regarding the stability of results. We discuss the various studies which have reported either in favour or against the stablility of results after distraction. We report a series of 6 cases (3 unilateral and 3 bilateral distraction) where distraction was carried out before puberty and followed them up to seven years after removal of distractors. This case series shows that results achieved by distraction osteogenesis are unstable or best unpredictable with respect to producing a permanent size increase in the mandible. The role of the distraction osteogenesis in overcoming the pterygomassetric sling is questionable. We suggest a multicenter study with adequate patient numbers treated with a similar protocol and documented after growth cessation to have meaningful conclusions on the debate of distraction osteogenesis versus orthognathic surgery.

Is hemifacial microsomia linked to multiple maternities?

Hemifacial microsomia describes a congenital orofacial malformation in which there is insufficient or disrupted development of the mandible affecting one side of the face. The aetiology of this condition remains unclear, but it has been postulated that twins (predominantly monozygotic) are more liable to be affected than singletons. This study investigates the incidence of multiple births amongst a large number of affected individuals and their families. Data were collected on 145 individuals with hemifacial microsomia and microtia, using postal questionnaires and interviews in a hospital setting. These data were compared with the mean age-standardised twin maternity prevalence for England and Wales between 1975 and 1995 of 1.06% and the triplet maternity prevalence for England and Wales for 1995 of 0.034% (a multiple maternity being where more than one baby is born, either alive or stillborn). The prevalence of twin maternities amongst the affected individuals was 3.96% (P>0.05) and amongst their siblings it was 4.02% (P<0.02). There was also an excess of twins in the rest of the family groups, predominantly due to a stronger history of twinning on the maternal side. As there were more twins amongst the affected individuals than in the general population, it seems likely that whatever the aetiology of hemifacial microsomia and microtia, the presence of a co-twin (or co-triplets) may make the causal event, or series of causal events, more likely to occur. This study supports the hypothesis that hemifacial microsomia and microtia are in some way linked to multiple births. Analysis of this link may provide new directions for research into the aetiology of a variety of congenital defects.

Solitary maxillary central incisor as a new finding in CHARGE association: a report of two cases.

Two cases are reported of a solitary maxillary central incisor in children with CHARGE association. The implications of the finding are discussed, along with the suggested relationship to other craniofacial disorders in which solitary central incisors have been reported.

Current concepts in the understanding and management of hemifacial microsomia.

Hemifacial microsomia (HFM) is a complex congenital condition. This review discusses recent research findings which affect all aspects of HFM, and addresses some prevailing misconceptions. Firstly, the broad phenotype is outlined, with an emphasis on the facial anomalies which are important for diagnosis, classification and treatment. The range of HFM anomalies and their possible embryology also account for the varied terms used in the literature. In addition, consideration of causation helps to shape our understanding of HFM as a clinical entity. Aetiology is described with particular emphasis on the involvement of genetic factors, although at present this is largely hypothetical. Finally, the principles of HFM management are reviewed. Attention is given to the integrated planning and team approach necessary to treat such patients. In addition, the possible attributes of new treatments, such as distraction osteogenesis, are highlighted.

Mango dermatitis: allergic contact dermatitis to Mangifera indica.

Mango Dermatitis' is the common term given to allergic contact dermatitis to the sap or skin of the fruit of Mangifera indica. Four patients presented with urticaria and eczematous rash following exposure to mangoes or the trees. Patch testing with diluted sap, crushed leaf, crushed stem and fruit skin was strongly positive.