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Arvid Heiberg

Publications and source records attributed to Arvid Heiberg.

4 recordsLinked to original sources
PubMed2005

Neurocranial morphology and growth in Williams syndrome.

Williams syndrome (WS) is a rare congenital neurodevelopmental disorder with distinctive facial features, cardiovascular abnormalities, short stature, mental retardation, and behaviour and cognitive characteristics. The aim of this study was to describe the neurocranial morphology and growth in a group of 62 individuals with WS. The neurocranium was analysed on lateral cephalograms and comparisons were made with neurocranial standards from longitudinal data derived from the Oslo University Craniofacial Growth Archive. The size and morphology of the neurocranium in WS subjects differed from controls. Females as a group showed greater differences than males. The posterior cranial base length was shorter in both WS males and females, and the anterior cranial base length was shorter in WS females whereas it was close to normal in the WS male group. The cranial base angle was, however, not different from the control groups. A flattening was seen in the superior aspect of the parietal bone in both WS males and females. In the posterior part of the neurocranium, the prominence of the occipital bone was larger than in the control groups, which was also reflected in a larger total length of the neurocranium. The thickness of the frontal and occipital bones was considerably greater than in the control group. The deviant size and morphology of the neurocranium in WS subjects was already established in the youngest age group and maintained throughout the observation period. The growth pattern of the neurocranium in WS subjects seemed to be similar to that of the control groups, except in a few individuals.

Adolescent↗
PubMed2004

[Complex craniofacial synostoses].

BACKGROUND: Complex craniofacial synostosis is a group of rare genetic disorders characterized by premature closure of the sutures in the craniofacial skeleton and which to varying degrees affects the extremities. MATERIAL AND METHODS: On the basis of relevant literature, we present a review of syndromal craniofacial synostosis. RESULTS: Phenotypically, the complex craniofacial syndromes have many similarities. Synostosis of the sutures of the cranial vault can result in a variety of skull deformations, depending on the sutures involved, the sequence of premature closure, and the time of closure. Synostosis of the sutures in the skull base and facial skeleton leads to shallow orbits, exophthalmus, hypertelorism, midface retrusion, and prognathia. INTERPRETATION: Precise diagnosis of complex craniofacial syndromes may be difficult solely on the basis of a clinical examination. However, several of the most common syndromes are caused by mutations in genes that code for fibroblast growth-factor receptors. Children with a suspected complex craniofacial syndrome should be referred to genetic testing.

Acrocephalosyndactylia↗
PubMed2003

Dental characteristics in Williams syndrome: a clinical and radiographic evaluation.

Williams syndrome is a rare congenital syndrome with distinctive craniofacial features, cardiovascular abnormalities, and behavior characteristics including mental retardation. The dental abnormalities have received scant attention in previous literature. The aim of this study was to describe dental characteristics in individuals with Williams syndrome. In a group of 41 individuals more than 10 years of age, 40.5% had agenesis of one or more permanent teeth and 11.9% had agenesis of 6 permanent teeth or more. The mesio-distal and labio-lingual dimensions of permanent tooth crowns were measured on 31 dental study casts from individuals older than 12 years. The mesio-distal and labio-lingual dimensions were significantly smaller compared with a reference sample. An analysis of tooth morphology was performed on the same dental study casts revealing altered tooth morphology. A high proportion of maxillary and mandibular incisors was tapered or screwdriver shaped. An evaluation of taurodontism on mandibular permanent molars was performed using a metric crown-body/root ratio. However, most of the molars rated as being taurodontic had short or extremely short total tooth lengths and could thus be rated taurodontic without meeting the classical definition. The results of this study indicate that although there is variation in dental development in individuals with Williams syndrome, agenesis of permanent teeth in combination with aberrations in tooth size and morphology may affect dental esthetics and complicate orthodontic and prosthodontic treatment.

Adolescent↗