A case of osteopathia striata.

A girl aged three years was first seen on account of nasal obstruction and recurring bronchitis. Birth had been difficult because of a large head, and delivery was completed with the aid of forceps. The neonatal period was stormy ; she had pneumonia and feeding was difficult because of nasal obstruction. Some improvement took place after the first month, but the feeding difficulty persisted and she was referred, at the age of five months, to a London children’s hospital. There it was noted that there was failure to gain weight and this was attributed to the considerable mechanical difficulty in feeding due to nasal obstruction. Thereafter gradual improvement took place, but she was late in passing all the milestones of infancy. Thyroid (half a grain twice a day) was given for a period of six months with further improvement in her general condition. She had pneumonia at the age of two years. The parents were healthy and unrelated. There were two other children, girls aged five and a half and two years, who were normal in every way. Examination-The weight and height were within the normal range for her age. The skull was large and brachycephalic ; the circumference was 21 inches. There was no bossing and the fontanelles were closed. The eyes were widely set with a low forehead and the epicanthic folds were marked. The nose itself was narrow and there was marked obstruction with a purulent discharge. The mouth was constantly open. The tongue showed a marked median groove, the palate was highly arched and there was a bifid uvula. The teeth were all present except the left lower second molar. The hearing was normal to simple tests and the eye grounds were normal. There were several palpable cervical glands. The thyroid gland was normal. The chest was somewhat barrel-shaped with a prominent sternum. The heart was normal. Both sides of the chest moved equally but the excursion was poor. There were signs of generalised bronchitis. The abdomen was distended and resonant to percussion. There was an umbilical hernia. The liver and spleen were not palpable. The vulva was normal. The stools were normal. There was full mobility of all the joints and there was no deformity of the limbs or spine. The gait was normal. The muscles were normally developed and of normal tone but the power was weak in the limbs. The skin was normal. The child was placid, hut played and behaved normally. Speech was indistinct and had a marked nasal character. She could name familiar objects and all the letters of the alphabet. Radiographic changes-Radiographs showed a fine striation of the long bones (Figs. 3, 4 and 5) which was more marked at the ends. The striation was linear, regular and vertical and was well shown in the lower femora and upper tibiae. The ilia (Fig. 2) showed a fan-like striation. The bones of the hands and feet were all within normal limits with the exception of the calcanea which showed a faint striation. The ribs, clavicles and vertebrae were normal. The skull was large and showed increased density of the base and the frontal bone (Fig. 1). Both antra and the right and left ethmoidal cells were obscured. The frontal sinuses had