A radiological study on intra- and extra-cranial calcifications in adults with X-linked hypophosphatemia and associations with other mineralizing enthesopathies and childhood medical treatment.

OBJECTIVES: 1) The objective of this study was to explore radiological signs of intracranial and nuchal ligament calcifications in adult patients with X-linked hypophosphatemia (XLH) compared with controls and 2) to correlate signs of cranial calcifications in XLH patients with the presence of other extra-cranial enthesopathies, with the severity of skeletal XLH impact and with medical treatment during childhood. SETTING AND SAMPLE POPULATION: Lateral and postero-anterior cephalograms from 36 adult XLH patients and 49 adult controls and X-rays from spine, pelvis, knees and ankles from 31 of the 36 XLH patients. METHODS: Radiological signs of intracranial and nuchal ligament calcifications in XLH patients were compared with controls by Fischer's exact test. In XLH patients, the presence of cranial calcifications was correlated with the presence of other enthesopathies, with the severity of skeletal XLH impact and with medical treatment by Fischer's exact or chi-squared test. RESULTS: Six (17%) XLH patients revealed major signs of intracranial calcifications. Nuchal ligament calcifications were common in XLH patients compared with controls (p = 0.018). Enthesopathy was present at 0-24 sites per XLH patient (median 2). Intracranial calcifications trended to correlate positively with vertebral enthesopathies (p = 0.059). Nuchal calcifications correlated positively with the severity of skeletal XLH impact (p = 0.040). Vertebral enthesopathies correlated negatively with medical treatment (p = 0.008). CONCLUSION: More XLH patients than controls showed nuchal ligament calcifications, and some XLH patients showed intracranial calcifications. Severely affected XLH patients often had nuchal ligament calcifications. Medically treated XLH patients had few vertebral enthesopathies.

The ectopic mandibular canines can start tooth formation in three different locations: a case series study based on single orthopantomograms from 47 individuals.

INTRODUCTION: A former study on orthopantomograms from young children with abnormal dental development (not canine ectopia) demonstrated that the tooth bud of the mandibular canine, compared to a stable longitudinal canine axis, could be located normally, anteriorly or posteriorly, with close relation to the first premolar. AIM: The aim of the present study is to analyse on orthopantomograms if the canine axis can demonstrate where the ectopic mandibular canine started tooth formation. MATERIALS: The material consists of orthopantomograms with ectopic mandibular canines and presence of primary mandibular canines from 47 cases (29 cases 9-21 years old and 18 cases with unknown ages). The primary canines demonstrated from minor apical resorption to more severe apical resorption. METHODS: Based on canine maturity, location of the canine axes and the interrelationships between the roots of the permanent canine and first premolar, the location from where the canine started tooth formation was determined. Canine maturity. Maturity stage below half root length and maturity stage above half root length revealed that 11 ectopic canines had less than half root length and 36 cases more than half root length. Canine axes. The canine axis, through the length of the primary canines Ax, is inserted on drawings of the orthopantomograms using the tracing programme Inkscape®. Interrelationship between roots. By visual inspection, the distance between the canine and first premolar was designated close distance, normal distance and extended distance. RESULTS: The results are divided into 3 groups. Group 1: The initial site of the permanent ectopic canine is located within the canine axis (6 cases). Group 2: The initial site of the permanent ectopic canine is located posterior to the canine axis (36 cases). Group 3: The initial site of the permanent ectopic canine is located anterior to the canine axis (5 cases). CONCLUSION: The study explained that the canine axis could divide cases of ectopic canines into three groups according to the location from where tooth formation starts. For getting closer to the pattern of the ectopic canine eruption, it is necessary to analyse series of orthopantomograms taken from the same individual over several years.

Morphological characteristics of frontal sinus and nasal bone focusing on bone resorption and apposition in hypophosphatemic rickets.

OBJECTIVES: To characterize the size and the morphology of the frontal sinus (i.e., structure evolved by bone resorption) and the nasal bone (i.e., structure evolved by bone formation) in adults with hypophosphatemic rickets (HR) compared with controls. SETTING AND SAMPLE POPULATION: Thirty-six patients with HR (12 males and 24 females) aged 21-74 years were included. The control group comprised 49 healthy individuals (23 males and 26 females) aged 20-79 years. MATERIAL AND METHODS: Profile cephalograms were obtained and the following measurements were included: height and width of the frontal sinus; length, width, and area of the nasal bone. The morphology of the nasal bone was assessed. Linear regression analyses were used to compare HR patients with controls. RESULTS: In HR patients, the size of the frontal sinus was unaffected (p = 0.406 to p = 0.862). The proximal width of the nasal bone, and the ratio between the proximal width and the axial length of the nasal bone were increased in HR patients (p < 0.05). CONCLUSIONS: The size of the frontal sinus was unaffected, indicating a normal ability of bone resorption within the bone. The morphology of the nasal bone was abnormal indicating a disturbance in bone formation during growth. The disturbances in nasal bone modeling were mainly expressed in the proximal part supported by structures of cartilaginous origin.

The location of the permanent mandibular canine as identified in orthopantomograms from children younger than 5 years of age: a case series study.

AIM: The aim of this case series study is to describe where the crowns of the permanent mandibular canines are located in early childhood in relation to the preceding primary canines. MATERIALS AND METHODS: From a sample of 31 orthopantomograms from children, younger than 5 years of age, the location of the mandibular canines was analysed by visual inspection. The radiographs were taken due to different deviations in the dentition and forwarded during a period of 28 years for elucidating different diagnostic questions. From an embryological point of view, the primary canine was considered as a stable structure in the jawbone. A longitudinal axis through the primary canine was named the canine axis. The initial site of the permanent crown was the site, where the permanent lamina "placed" the tooth bud for final development. A normal site was determined when the canine axis passed though the permanent crown and a pathological site was determined when this did not happen. RESULTS: Normal sites for the permanent crowns were observed in 19 cases and abnormal sites in 8 cases. In four cases, the interrelationship between the canine axis and the permanent crown could not be decided. The study demonstrated that the tooth germ for the permanent canine can start initial formation misplaced distally or mesially to the preceding primary tooth. CONCLUSION: The study demonstrates different malpositions of the initial crowns of the permanent mandibular canines in children younger than 5 years of age. The results are considered of importance for future understanding of the aetiology behind ectopic mandibular canines.

The primary Maxillary Central Incisor in the Solitary Median Maxillary Central Incisor syndrome.

AIM: Solitary Median Maxillary Central Incisor (SMMCI) is a developmental anomaly in the permanent dentition with one single central incisor in the maxilla, positioned exactly in the midline. This condition has been associated with extra- and intraoral malformations in the frontonasal segment of the cranium and face. It is not known whether the centrally located permanent incisor is always preceded by a centrally located primary incisor. The aim was to analyse whether a permanent single central incisor in SMMCI is always preceded by a primary single central incisor and to study extra- and intraoral phenotypic traits of the condition. STUDY DESIGN: cross-sectional radiographic study of 11 children, visual analysis of photos and dental and panoramic radiographs. RESULTS: Nine of the 11 cases exhibited a primary SMMCI with one symmetrical crown and root. Two cases exhibited two separate primary central incisor crowns with fused roots. The phenotypical traits (indistinct philtrum, lack of normal upper lip contour, missing superior labial frenulum and distinct mid-palatal ridge) were findings observed in young children with a primary SMMCI. CONCLUSION: The present study concludes and stresses the necessity of diagnosing of the SMMCI condition early in life. Furthermore, paediatric dentists are recommended to be aware of the condition and to refer these patients to interdisciplinary diagnostics and treatment.

Abnormal premolar eruption: classification, aetiology, and treatment based on a case series study.

AIM: The aim of this case series study is to classify deviations in mandibular and maxillary premolar eruption according to aetiology, with a focus on the resorption pattern in the preceding primary molars. The purpose is also to give treatment guidance based on aetiology. MATERIALS AND METHODS: Radiographic material from 64 cases with abnormal premolar eruptions were grouped into three eruptions phases: Phase 1, from tooth bud to early root formation, sub-grouped according to "ankylosis" or "not ankylosis" of the primary molars; Phase 2, from start of eruption to the penetration of gingiva, sub-grouped according to normal or abnormal resorption of the primary molars and Phase 3, eruption after penetration of gingiva. RESULTS: Phase 1: early ankylosis of primary molars, ectopic locations of the premolar crown, including occlusally displacement in relation to the primary molar, are demonstrated. Not ankylosed primary molar: different positions, even an upside-down position of the premolar, are demonstrated. The conditions are explained in relation to the early migration pattern of the premolar tooth bud. Regarding treatment, in cases with ankylosed primary molars these should be extracted as soon as diagnosed and in cases with not ankylosed primary molars these should be extracted when root formation of the premolars has started. The premolars should be observed and saved if possible. Phase 2: non-exfoliation of primary molar, aetiology and treatment of premolars depend on tissue types involved. In bone dysplasia, the eruption of premolars is delayed. In these cases, the primary molars should be extracted when eruptive movements of the premolars have started. In cases with ectoderm deviation, the crown follicle does not function normally during the resorption of the primary molars and the recommended treatment is extraction of primary molars before root closure of premolars. In cases in Phase 2 where the premolars were ankylosed these should be surgical removed. Phase 3: different aetiologies are highlighted, with focus on abnormal innervation and enzyme defects. The premolars are seemingly ankylosed, and surgery might be the only treatment. CONCLUSION: The case series presented demonstrate how ectopic and arrested premolars have different aetiologies and as a consequence, different treatments. The study highlights several aspects in pathological eruption, which still need to be elucidated.

How does occipitalization influence the dimensions of the cranium?

OBJECTIVES: To describe occipitalization on human dry skulls and to compare craniofacial morphology including the posterior cranial fossa in skulls with occipitalization and in skulls without occipitalization and with normal craniofacial morphology (controls). SETTING AND SAMPLE POPULATION: A total of 29 skulls were selected from the Björk collection. Nine had occipitalization of atlas. Twenty had no dentoalveolar or craniofacial anomalies, and no occipitalization was visible on the skulls (controls). The ages of the skulls were between adolescence and adulthood. METHODS: Visual assessments to describe the occipitalization pattern, direct measurements to measure the foramen magnum dimensions and cephalometric radiographic analyses to describe the craniofacial morphology. New variables were determined to describe the posterior cranial fossa. RESULTS: Of the skulls with occipitalization, 67% had complete and 33% had partial occipitalization. In the posterior part of the neural arch, 44% had a cleft. Occipitalization was significantly associated with a small foramen magnum (p < 0.01; p < 0.001) and deviant shape of the posterior cranial fossa (p < 0.05). The craniofacial morphology in the vertical and sagittal dimensions including the cranial base angle was normal in skulls with occipitalization, which indicates that occipitalization is associated with deviant morphology of the surrounding bony structures but not with deviations in the craniofacial morphology in general. The results of this study are important for the diagnosis of occipitalization on 2D radiographs.

Skull thickness in patients with clefts.

OBJECTIVES: The purpose was to analyze skull thickness in incomplete cleft lip (CL), cleft palate (CP), and combined cleft lip and palate (UCLP). SETTING AND SAMPLE POPULATION: Copenhagen School of Dentistry and Copenhagen Cleft Lip and Palate Centre. Patients with cleft lip, cleft palate, and combined cleft lip and palate and normal adult men. MATERIAL AND METHODS: Four groups of patients comprised the study. One group of patients with CL (24 patients; 7 women, mean age 6; 17 men, mean age 7.1), one group of patients with UCLP (28 patients; 11 women, mean age 6.6; 17 men, mean age 6.7), one group of patients with CP (57 male patients aged 18-33), and one normal adult male control group. The CL and UCLP groups were compared. The CP group was compared with the normal adult male control group. RESULTS: CL women had a significantly thinner occipital bone compared with CL men (p = 0.027). Women with UCLP had significantly thicker occipital bone than the control women (incomplete CL) (p = 0.014). The study showed gender differences in skull thickness in different cleft types. It also demonstrated that particularly the occipital bone deviated in patients with UCLP, which may explain the considerable deviations in jaw shape and position, previously registered in patients with UCLP.

Delayed dental maturity in dentitions with agenesis of mandibular second premolars.

OBJECTIVES: To evaluate dental maturity in the mandibular canine/premolar and molar innervation fields in children with agenesis of the 2nd mandibular premolar and to associate these findings with normal control material. SETTING AND SAMPLE POPULATION: Department of Orthodontics, Institute of Odontology, University of Copenhagen. Eighty-three panoramic radiographs (27 girls and 31 boys with agenesis of one mandibular 2nd premolar and 17 girls and eight boys with agenesis of both mandibular 2nd premolars) represented all mandibular second premolar agenesis cases from a material of 2847 radiographs. MATERIAL AND METHODS: On each radiograph, dental maturity of all available mandibular premolars, canines and 2nd molars was evaluated and categorized in maturity stages according to Haavikko whose material served as control material. Descriptive statistics given by sample mean, standard deviation and range for each tooth stratified by gender and agenesis. Ninety-five percentage confidence limits and T-statistics were used. p-values <5% were considered significant. RESULTS: In unilateral agenesis, the canines are specifically delayed in both girls and boys, with a larger delay in girls (p=0.009). The second molar is not delayed in boys (p=0.98) but is in girls (p=0.04). The differences in delay for the canine compared to the second molar are significant for both girls and boys. The results show a considerable delay in tooth maturation within the canine/premolar innervation field predominantly in girls. The 2nd molar is delayed in girls but not in boys.

Sagittal, vertical and transversal dimensions of the maxillary complex in patients with ectopic maxillary canines.

OBJECTIVES: To analyse the craniofacial maxillary complex in cases with labially and palatally located ectopic canines, subgrouped accordingly: Group I: no deviations in the dentition; Group IIa: deviations in the maxillary incisors only; Group IIb: deviations in the dentition in general. SETTING AND SAMPLE POPULATION: Sixty nine patients (mean age 13 years 6 months) with palatally or labially located ectopic canines. MATERIAL AND METHODS: Profile radiographs and dental casts were analysed. The patients were subgrouped according to a previous registration of dental deviations registered radiographically. Maxillary cross-arch transversal width was analysed on dental casts. Sagittal and vertical dimensions were registered cephalometrically on profile radiographs. RESULTS: In the patient sample the maxillary cross-arch transversal width (from first maxillary molar left to first maxillary molar right), was significantly larger than the normal mean (0.65 mm, 95% Cl: 0.02-1.28, p = 0.043). The sagittal length N-S was significantly shorter (-0.97, 95% Cl:-1.72-(-)0.22, p = 0.002). The vertical length ANS-N length was also significantly shorter (-0.79, 95% Cl:-1.65-(-)0.02, p = 0.047). The remaining variables were non-significant. Tests for interaction between groups (I, IIa and IIb) and palatal/labial ectopic location did not demonstrate significance. CONCLUSION: In patients with ectopic maxillary canines, the maxillary complex is shorter sagittally as well as vertically, while it is wider transversally.

Differences between dentitions with palatally and labially located maxillary canines observed in incisor width, dental morphology and space conditions.

AIM: To analyze the interrelationship between incisor width, deviations in the dentition and available space in the dental arch in palatally and labially located maxillary ectopic canine cases. MATERIALS AND METHODS: Size: On dental casts from 69 patients (mean age 13 years 6 months) the mesiodistal widths of each premolar, canine and incisor were measured and compared with normal standards. Dental deviations: Based on panoramic radiographs from the same patients the dentitions were grouped accordingly: Group I: normal morphology; Group IIa: deviations in the dentition within the maxillary incisors only; Group IIb: deviations in the dentition in general. Descriptive statistics for the tooth sizes and dental deviations were presented by the mean and 95% confidence limits for the mean and the p-value for the T-statistic. Space: Space was expresses by subtracting the total tooth sizes of incisors, canines and premolars from the length of the arch segments. RESULTS: Size of lateral maxillary incisor: The widths of the lateral incisors were significantly different in groups I, IIa and IIb (p=0.016) and in cases with labially located ectopic canines on average 0.65 (95% CI:0.25-1.05, p=0.0019) broader than lateral incisors in cases with palatally located ectopic canines. Space: Least available space was observed in cases with labially located canines. The linear model did show a difference between palatally and labially located ectopic canines (p=0.03). Space related to deviations in the dentition: When space in the dental arch was related to dental deviations (groups I, IIa and IIb), the cases in group IIb with palatally located canines had significantly more space compared with I and IIa. CONCLUSION: Two subgroups of palatally located ectopic maxillary canine cases based on registration of space, incisor width and deviations in the morphology of the dentition were identified.

Brain malformation in single median maxillary central incisor.

Clinical and radiographic examinations and MR scan of a 12-year-old girl with SMMCI (single median maxillary central incisor) showed impaired growth and a midline defect involving the central incisor, cranium and the midline structures in the brain, falx cerebri and pituitary gland. She had a severe growth hormone deficiency but no other pituitary hormone deficiencies. She was treated with growth hormone and followed during a four-year period with successful gain in body height and sexual maturation. This study focuses on the developmental association between the involved structures and provides guidelines for early diagnostics.

Morphology of the mandibular canal and the angulation between the mandibular and mental canals in dry skulls.

The aim of this study was to analyse the correlation between external and internal mandibular morphology in adult or adolescent normal anthropological mandibles. Lateral radiographs of 31 symmetrical mandibles were analysed. The external morphology was defined by the gonial and beta-angles. In order to analyse internal morphology, a metallic pin was placed in the mental canal on the left side before radiography. The angle between the mental and the mandibular canals was termed the 'mental angle' and that expressing the curvature of the mandibular canal, the 'mandibular angle'. Spearman correlation analysis was used to investigate the relationships between the angles. Internal morphology: a statistically significant correlation was found between the mental and mandibular angles (correlation coefficient: -0.60, P=0.0004). When the mental angle was narrow, the mandibular angle tended to be wide, while a wide mental angle was interrelated with a narrow mandibular angle. External morphology: a statistically significant correlation was found between the mandibular and gonial angles (correlation coefficient: 0.57, P=0.0009). A weaker correlation was also found between the mandibular and beta-angles. The findings show that the internal courses of the mandibular and mental canals are interrelated. They also indicated that the course and morphology of the mandibular canal are interrelated with external mandibular morphology.

Localised scleroderma en coup de sabre affecting the skin, dentition and bone tissue within craniofacial neural crest fields. Clinical and radiographic study of six patients.

PURPOSE: When localised scleroderma occurs in the face, neck and scalp area, it is called scleroderma en coup de sabre (SCS) for its resemblance to the stroke of a sabre. Most observed characteristics: abnormal skin and dental development, facial atrophy and neurological complications. The aim was to evaluate the extent of SCS in the underlying subcutis, including teeth/bone tissues. The goal was to solve, how far the external visual skin abnormality extends in depth, and if the condition appears within and limited to craniofacial neural crest fields. METHODS: Photographic and radiographic materials from six patients (one male, five females, aged 5-39 years) were included. The cases were divided in three groups, two in each, according to similarity in location of SCS in the skin. Dentition and gingiva were analysed clinically and from intra-oral photos, dental radiographs and orthopantomograms. Agenesis, dental maturity stage (root length), deviation in crown and root morphology (size and shape), and eruption disturbances were registered. Profile and frontal radiographs were analysed cephalometrically for jaw relationships and bone structures. RESULTS: In SCS, skin affection corresponds to the neural crest regions/fields. A close spatial association between skin, teeth and bone affections within neural crest fields was found. No common traits in profile analyses were observed. Asymmetry from minor to severe appears in the frontal analyses. A malformation in planum sphenoidale was observed in two individuals with the same location of skin affections. CONCLUSION: SCS conditions seem to extend from the skin in the depth to the sella turcica area within neural crest fields.

A possible association between early apical resorption of primary teeth and ectodermal characteristics of the permanent dentition.

The hypothesis of this study is that children with unexpected early apical resorption of the primary teeth are also predisposed to resorption in the permanent dentition. Accordingly, the aim was to perform a longitudinal study focussing on the permanent teeth in children with unexpected early apical resorption in the primary dentition. Panoramic radiographs of 12 children (7 boys and 5 girls) aged 6 years 4 months to 8 years 9 months with unexpected early apical resorption of primary teeth were identified from a dental archive of 588 patients. After written request, follow-up radiographs were obtained (2-15 year interval between early and follow-up radiographs). The radiographs were examined in order to verify the abnormal resorption pattern of the primary teeth and dental deviations in the permanent teeth, known to predispose for root resorption (i.e. invaginations, narrow crowns, abrupt root deflections, slender roots, short roots, taurodontia, agenesis, deviant pattern of eruption). Primary dentition: Two phenotypically different resorption groups were identified: group I, eight patients (resorption of the roots only), and group II, four patients (resorption of root and crown). Permanent dentition: In all 12 children, dental deviations in the permanent dentition were observed. Additionally, idiopathic external apical resorption of the permanent teeth was seen in three children, two of whom had received orthodontic treatment.

Dental and craniofacial findings in 91 individuals with agenesis of permanent maxillary canines.

AIM: Agenesis of maxillary permanent canines is a rare form of agenesis (prevalence 0.07-0.13%). The aetiology is still unknown. The purpose was to focus on dentitions and craniofacial profiles in individuals with maxillary canine agenesis. METHOD: From 91 individuals (10-18 years of age) 91 Orthopantomograms and 77 profile radiographs were divided accordingly: Group I: agenesis of 1 or 2 maxillary canines. Group II: several ageneses including the maxillary canines. Dentition analysis included crown/root shape, invaginations, eruption disturbances and resorption patterns. Cephalometric angular measurements and mutual comparisons between the groups were performed. Agenesis pattern in Group II was compared to agenesis pattern in a population of children and young adults not characterized by maxillary canine agenesis. Cephalometric values from both groups were compared to normal values from individuals without agenesis. Findings were statistically evaluated. RESULTS: Dentition: Group I: only the missing maxillary canines were deemed to be in an abnormal dental deviation. Group II: abnormal dental development regarding morphology, resorption of primary teeth and also agenesis of the first maxillary premolar. Comparison of the groups showed a difference in the pattern of malformation. Profile: Group I: anterior inclination of the maxilla and increased cranial base angle were significant findings. Group II: diminished incisor slope in the maxilla and enlarged cranial base angle were significant findings. A comparison showed a difference in maxillary inclinations of the teeth. CONCLUSION: Group I dentitions are normally developed with only minor dental malformations. Group II dentitions had several dental malformations and often agenesis of the first maxillary premolar. Significant findings: first maxillary premolar agenesis in Group II and enlarged cranial base angle in both groups.

Unilateral and bilateral dental transpositions in the maxilla-dental and skeletal findings in 63 individuals.

AIM: This was to elucidate dental and skeletal findings in individuals with unilateral and bilateral maxillary dental transpositions. MATERIAL AND METHODS: The sample comprised of radiographic materials from 63 individuals with maxillary dental transpositions from the Departments of Odontology at the Universities of Copenhagen and Aarhus and by the Danish municipal orthodontic service. The cases were divided into three groups: unilateral transposition of the canine and first premolar (Type 1U), bilateral transposition of canine and first premolar (Type 1B), and unilateral transposition of canine and lateral incisor (Type 2). The dentitions were analysed regarding agenesis and dental morphological anomalies on panoramic radiographs, and craniofacial aspects were cephalometrically analysed on profile images The results were statistically evaluated. RESULTS: All groups demonstrated increased occurrences of agenesis (Type 1U and Type 1B: 31 agenesis in 15 patients; and Type 2 three agenesis in three patients). Taurodontic root morphology was most dominant in Type 1U. Peg-shaped lateral incisors showed an increased occurrence, though not in Type 1U. Skeletally, Type 1B and Type 1U demonstrated maxillary retrognathia (more pronounced in Type 1B). Type 2 showed a significant posterior inclination of the maxilla. CONCLUSION: Transpositions of maxillary canines involve dental and skeletal deviations. Dental deviations were predominantly taurodontic root morphology and agenesis. Regarding skeletal deviations, bilateral transpositions of the canines and the first premolars are associated with skeletal changes. Unilateral transpositions are possibly a localised deviation with minor or no skeletal involvements. The results indicate a possible difference in the aetiologies of unilateral and bilateral transpositions.

Linear scleroderma en coup de sabre including abnormal dental development.

BACKGROUND: Linear scleroderma en coup de sabre (SCS) is a rare skin condition, where dense collagen is deposited in a localised groove of the head and neck area resembling the stroke of a sabre. The SCS may involve the oral cavity, but the severity and relation to this skin abnormality is unknown. A paediatric dentist may be the first medical person to identify SCS by its involvement in dentition. It is assumed that the malformation of a dentition could be associated with the severity of the skin deviation. CASE REPORT: A 6-year and 10-month-old Turkish girl with a history of SCS was referred for dental diagnostics and treatment. The SCS skin lesion affected the left side of her hairline over the forehead and nose, involving the left orbit proceeding towards the left oral region. Dental clinical/radiographic examination revealed malformed left maxillary incisors with short roots and lack of eruption. FOLLOW-UP: The patient has been regularly controlled and treated since she was first diagnosed. A surgical and orthodontic treatment was performed to ensure optimal occlusion, space and alveolar bone development. The present age of the patient is 14 years and 10 months. CONCLUSION: This case demonstrated a patient with a left-sided skin defect (SCS) and a left-sided local malformation in her dentition. It is possible that there is a developmental connection between these two left-sided defects, both with an ectodermal origin.

Obesity prevalence in Norwegian adults assessed by body mass index, waist circumference and fat mass percentage.

The prevalence of obesity presents serious public health challenges worldwide and is most commonly estimated by the categorization of body mass index (BMI). The purpose of this study was (i) to describe the sensitivity of BMI, waist circumference (WC) and fat mass percentage in the classification of overweight and obesity and (ii) to describe the prevalence of overweight and obesity using different measurement methods in a national sample of Norwegians. A total of 904 Norwegians (20-85 years) were randomly enrolled. Body weight, height, WC and skinfold thickness were measured; BMI and fat mass percentage (FP(skf) ) were calculated. The sensitivity and specificity varied from 77.0% to 86.9% and from 60.6% to 82.3%, respectively. Area under the curve was 0.837 (95% confidence interval [CI]: 0.801-0.872) for the ability of BMI to predict obesity by FP(skf) and 0.811 (95% CI: 0.770-0.852) for WC. A total of 12.7% (male: 12.1%, female: 13.4%) were obese based on BMI classifications, 29.1% (male: 36.5%, female: 20.0%) of the sample were obese based on FP(skf) and 24.3% of men and 37.8% of women were abdominally obese by WC. The agreement between selected measuring methods was fair to good, although the variation in obesity prevalence rates between the different assessments methods varied markedly.

Studies of the cranial base in 23 patients with cri-du-chat syndrome suggest a cranial developmental field involved in the condition.

The purpose of the present study was to investigate the cranial base on profile radiographs of patients with cri-du-chat syndrome and to relate the findings to current knowledge of brain malformation in an attempt to localize the developmental field affected in cri-du-chat syndrome. The material of profile radiographs of 23 patients was collected in Denmark in the 1970s. Twenty-two patients had terminal deletions of chromosome 5 (5p13.3, 5p14.1, 5p14.2, and 5p14.3), and one patient had an interstitial deletion. The cranial base angle (n-s-ba) was in most cases reduced and in no cases increased compared to age-related standards for normal individuals. Malformations in the bony contours of the sella turcica and the clivus occurred in cri-du-chat patients with terminal deletions. This specific cranial base region develops around the notochord at the location from where the rhombencephalic-derived brainstem, pons, and cerebellum have developed dorsally, and from where the neurons to the larynx have migrated ventrally. As the cranial base, the cerebellum and the larynx are involved in cri-du-chat syndrome, and attention is drawn to a new developmental field which comprises the dorsum sellae, clivus, cerebellum, and larynx. This field seemingly originates from the same notochordal location. The study has demonstrated a cranial base malformation in cri-du-chat patients, which ought to be elucidated in future research and combined with neurological and chromosomal investigations.