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Biomedical subjects

S Kreiborg

Publications and source records attributed to S Kreiborg.

At least 91 records · Page 5Linked to original sources

Craniofacial growth in plagiocephaly and Crouzon syndrome.

Very little information is available in the literature regarding the morphology and growth of the cranial base, the orbits and the jaws, in patients with premature craniosynostosis. The present report deals with the craniofacial morphology and growth in plagiocephaly and Crouzon syndrome. For each anomaly a dry skull was compared with a clinical case followed longitudinally with roentgencephalometry. Metallic implants in the jaws were employed in one case. Cranial base involvement was demonstrated for both types of premature craniosynostosis. In Crouzon syndrome the cartilagenous cranial base was affected. Marked compensatory and dysplastic growth changes were observed in the facial skeleton in both anomalies. The methodological problems related to roentgencephalometric examination of patients with plagiocephaly are discussed, and a roetgencephalometric method for evaluation of exophthalmos in patients with Crouzon syndrome is presented.

Adult↗

Motion of bones and volume changes in the neurocranium after craniectomy in Crouzon's disease. A roentgen stereometric study.

Craniectomy was performed on a boy with Crouzon's disease at 22 months of age. Metallic implants (tantalum balls, 0.5 mm in diameter) were inserted in the calvaria during surgery, and the child was examined postoperatively by roentgen stereometry at intervals of about 100 days (total observation time, 309 days). The thyroid radiation dose was 250 muGy for one examination. The effect of craniectomy was recorded with a high degree of accuracy in terms of motion of bones and volume changes in the calvaria. Expansion occurred almost entirely through motion of free bone flaps in the frontal region, while a linear craniectomy in the region of the sagittal suture caused small changes. The rate of expansion decreased rapidly and stabilization was recorded about 250 days after surgery.

Bone Development↗

Variable expressivity of Crouzon's syndrome within a family.

Three individuals in two generations affected with Crouzon's syndrome are reported. The proband, a 10-year-old boy, had been diagnosed until the age of 4. His 3-year-old sister had not been diagnosed previously and his mother was unaware of having the syndrome and had never received any genetic counseling. The individuals are reported with emphasis on clinical and roentgencephalometric variation. The ultimate goal in teratolgoy is prevention. Currently one of the most important factors related to prevention is genetic counseling in conditions with well-documented modes of inheritance. The present report describes how the variable expressivity of Crouzon's syndrome within a family had caused diagnostic problems and consequently had delayed genetic counseling.

Cephalometry↗

Craniofacial morphology in the nevoid basal cell carcinoma syndrome.

A roentgencephalometric analysis of eight males and four females with nevoid basal cell carcinoma syndrome (NBS) is presented. The patients were 14-54 years of age. Fourteen angular and 26 linear variables were measured and compared with control data. The main roentgencephalometric features were: proportionate increase of the size of the calvarium, protrusion of the frontal and parietal region, low position of the occipital region, increased interorbital distance, increased length of the mandible, mandibular retrognathia due to changes in shape and position of the mandible. The suggestion of a characteristic craniofacial morphology as a part of the syndrome was substantiated. Together with other skeletal aberrations the roentgencephalometric findings seemed to indicate that the syndrome is a dysostosis of generalized type.

Adolescent↗

Clinical conference I. Calvarium and cranial base in Apert's syndrome: an autopsy report.

A post-mortem examination of a 22-month-old boy with Apert's syndrome is reported. A linear coronal craniectomy performed at 11 months of age had fused completely in spite of the insertion of polyethylene film between the bony edges. Furthermore, progressive synostosis of other cranial sutures was observed. Histological examination of the cranial base showed that the sphenofrontal suture was fused and that the entire cerebral surface, including the sella turcica, was characterized by bone resorption. The sphenooccipital synchondrosis was extremely wide and abnormally shaped, and the chondral growth activity seemed diminished. It was concluded that the patient exhibited primary as well as secondary growth distrubances in the calvarium and cranial base. The premature synostosis of the coronal and sphenofrontal sutures and the anomaly of the sphenooccipital synchondrosis were interpreted as primary defects. The altered shape of the calvarium and the marked resorption and shortening of the cranial base were considered secondary changes. Furthermore, the investigation indicated that the conventional linear coronal craniectomy cannot be expected to improve the craniofacial growth pattern in patients with Apert syndrome. Alternative surgical procedures were suggested.

Craniosynostoses↗

Estimates of orthodontic treatment need in Danish schoolchildren.

Orthodontic treatment need has been assessed on the basis of complete orthodontic records in a random sample of 293 Danish children aged 13-17 years by a group of postgraduate orthodontic students and by three orthodontists. The estimates of treatment need ranged from 45% to 61%. Interexaminer agreement in pairs was observed in 77%-92% (mean 83%) of the cases. Uniformity in four individual assessments was reached in 69% of the cases: 38% were considered in need of treatment and 31% were considered not to present such need; conversely, 31% gave rise to disagreement. It is argued that, at present, the orthodontist's subjective estimate of treatment need probably constitutes a more realistic approach to the problem of assigning treatment priority than the various indices of malocclusion.

Adolescent↗

A morphologic description of a dry skull with mandibulofacial dysostosis.

The morphology of an East Indian, dry skull exhibiting the characteristics of mandibulofacial dysostosis (MFD) is described. The skull was small, but the shape of the calvarium was essentially normal. Extensive anomalies were registered in the sphenoid bone, the temporal bone, the zygomatic bone, the maxilla, and the mandible. A lateral cephalometric radiograph of the skull was compared with a mean value diagram of adult Danish males in order to illustrate the morphologic aberrations of the MFD skull. The etiology and pathogenesis of the syndrome are disucssed.

Humans↗

Roentgencephalometric studies of the premature craniofacial synostoses: report of a family with the Saethre-Chotzen syndrome.

Five affected individuals in 4 generations of a family with acrocephalosyndactyly (McKusick ACS Type III; Saethre-Chotzen syndrome) are reported. Serial roentgencephalometric data obtained pre- and postoperatively on the proband were compared with similar measurements on the affected mother and maternal uncle, both of whom have not had operative corrections, and the proband's unaffected older brother. Similarity in skull form among the affected individuals was demonstrated. Head circumference as an index of cranial growth or intracranial capacity was misleading in assessing the intellectual potential of the affected adults. In contrast, cranial capacity, as measured by the modulus, was found to be more reliable.

Acrocephalosyndactylia↗

Cephalometric study of the Apert syndrome in adolescence and adulthood.

This paper reports a cephalometric analysis of the craniofacial morphology in adolescents and adults with Apert syndrome. The sample comprised 26 patients with Apert syndrome (15 males and 11 females). The control group consisted of 153 adults (102 males and 51 females). Both lateral and frontal cephalograms were studied. The data were presented as mean plots of the craniofacial region together with data on some of the most significant findings. Marked differences were found in nearly all craniofacial regions except the mandible. The calvaria was increased in height and width but length was decreased. The cranial base showed marked protrusion of the greater wing of the sphenoid, which contributed to severe reduction of orbital volume and protrusion of the eyeglobe. Orbital volume was further reduced by maxillary hypoplasia in all three planes of space together with retrognathia. Maxillary height was extremely short and so was the nose. The width of the nasal cavity, height and depth of the bony nasopharynx, and the nasopharyngeal airway were all markedly reduced in size. The mandible was of fairly normal size and shape but was posteriorly inclined. Head posture was extended in relation to the cervical column. Total facial height was increased, whereas upper facial height was markedly reduced. Incisor occlusion showed mandibular overjet and open bite. Apert syndrome patients were then compared to a group of Crouzon syndrome patients. Marked and significant differences were found between the two syndromes in nearly all craniofacial regions, and craniofacial dysmorphology was generally more severe in Apert syndrome patients.

Acrocephalosyndactylia↗