Monozygotic twins discordant for Ullrich-Turner syndrome.

Ullrich-Turner syndrome occurred in one of a pair of female twins. The chromosome constitution of the affected twin was 45,X/46,XX and that of the normal twin 46,XX. Investigation of banded chromosomes, red cell antigens, HLA types, red cell enzymes, and serum proteins indicates monozygosity. The twins are discordant for height, pterygium colli, ovarian function, strabismus, dental eruption, external ear formation, hearing loss, and performance scores on the Wechsler Intelligence Test. All of these differences can be attributed to X monosomy in one cell line in the affected twin, presumably resulting from mitotic nondisjunction or anaphase lag early during embryonic development. Ten other pairs of apparently monozygotic twins discordant for the Ullrich-Turner syndrome have been reported previously, and the findings in these cases are reviewed.

CT in the evaluation of the orbit and the bony interorbital distance.

The distance between the orbits and their individual dimensions are important in the diagnosis of craniofacial anomalies. Most observers rely on standard radiographs for measuring the bony interorbital distance. Tomography of the skull base and orbital computed tomography (CT) can also be used. This article describes the normal range of the bony interorbital distance and other useful orbital linear and angular measurements as determined from a series of CT scans of the orbits in 400 adults who had CT for other purposes. The normal interorbital distance measured at the posterior border of the frontal processes of the maxilla on nonrotated scans, in the plane of the optic nerve, ranges from 2.29 to 3.21 cm (average, 2.67 cm) in men and 2.29 to 3.20 cm (average, 2.56 cm) in women. The widest interorbital distance lies behind the posterior poles of the globes. This ranges from 3.16 to 4.10 cm (average, 3.37 cm) in men and 2.93 to 3.67 cm (average, 3.20 cm) in women.

Radiographic features of the ear-related developmental anomalies in patients with mandibulofacial dysostosis.

Among the conditions that arise from disturbances in development of the first two branchial arches, the symmetric syndrome of mandibulofacial dysostosis and the asymmetric anomalies of the hemicraniofacial microsomia represent a characteristic pattern of craniofacial malformation distinct from other first and second arch syndromes. Since the usual embryonic aberration in both of these anomalies involves the first and second branchial arch derivatives, malformations of the external and middle ear are common. The external auditory canal is absent in the vast majority of the cases. Incudomallear deformities are usually present. In this paper, radiographic analysis of the ear-related developmental anomalies of 17 patients with mandibulofacial dysostosis is described.

Training program in maxillofacial prosthetics for medical artists.

A program in maxillofacial prosthetics for medical artists was initiated at the University of Illinois Medical Center in 1966 as a joint enterprise of the Center for Craniofacial Anomalies of the Abraham Lincoln School of Medicine and the Department of Medical Art of the School of Associated Medical Sciences. In the intervening years, 25 medical artists have been graduated from the program. The general background of the trainees, the scope of their training, and their clinical contribution were discussed and illustrated.