Premature obliteration of the sternal sutures and pigeon-breast deformity.

Abstract

The purpose of this paper is to illustrate an uncommon and little known developmental anomaly of the sternum characterized by premature obliteration of its sutures and by a tendency toward forward angulation. Associated with this angulation is an increase in the sagittal diameter of the thorax of the type seen in “arcuate pigeon breast.” Case Reports Case I: A white male child had been under observation since birth for congenital micrognathia of mild degree, bilateral clubfoot, inguinal hernia, and cryptorchidism. A “pigeon-breast” deformity had also been present since early infancy. When last seen at four years, he appeared well developed, both physically and mentally; the deformity of the feet had improved considerably, the micrognathia only slightly. The child's head was elongated, but there was no roentgen evidence of premature craniosynostosis. The thorax showed a midline bulge, anteriorly (Fig. 1). The lateral roentgenograms of the chest taken at one and at four years are reproduced in Figures 2 and 3. The father and mother were twenty-five and thirty years old, respectively, when the patient was born. They are living and well and show no thoracic deformity. Roentgenographically, the sternum of the father has a normal configuration and an open manubriosternal synchondrosis. The chest films of the mother reveal solid bony fusion of the manubriosternal junction. There are no siblings. Case II: A white female child was hospitalized for the first time at the age of six months. The chief complaints at that time were mild difficulty in swallowing, retarded physical development, asymmetry of the head, face, and eyes, and pigmented skin. On physical examination, the child appeared mentally alert but poorly nourished and underdeveloped; all the somatic measurements were below the third percentile. The skin was covered with white and brown blotches and streaks which were interpreted as manifestations of incontinentia pigmenti. There was an obvious flattening of the right occipital and left frontal regions. The left side of the face was smaller than the right. The left eye was small; the left pupil was cloudy and did not react to light. An opthalmological examination showed microphthalmia and cataract on the left. The thorax showed a midline bulge anteriorly (Fig. 4). No gross abnormality could be recognized in the films of the skull and face. A barium swallow revealed a right aberrant subclavian artery crossing behind the esophagus. A lateral roentgenogram of the chest, taken at this time, is reproduced in Figure 5. The swallowing difficulty subsided gradually. When re-examined at the age of one year, the child still appeared underdeveloped. All the other findings were the same as at the first a dmission. The hemogram, urinalysis, and multiple chemical studies of the blood serum showed no abnormality. The father and mother were, respectively, thirty-three and twenty-three years old when the patient was born.