頭顔部骨形成不全症一例

A 18-month boy of Crouzon's disease was reported here. He was deliverd via Caesarian section with body weight 3100 Gm., and had uneventful neontal life. He showed delay in development: head control at age 4-5 moth, sitting alone at 11-month, walking at 17-month, simple words talking at 16-month, and dentition at 7-month. At age of 3(1/2) month, proptosis(ou) specially left eye was nonted. At 9-month the proptosis became more evident with exophthalmometer 14mm. (OD), 16mm. (OS)' eye ground revealed papilledema (ou); and the skull x-ray showed increase in digital marking and hypoplasia of maxillary bone. At 18-month, the facial and skull deformity and the ophthalmological anomaly became more obvious as following. Ⅰ. Ophthalmologically: V.A.: S-L (+) (ou). Eye position: exophthalmos, 20mm (OD), 21mm (OS). Interpupillary distance: 64mm. Strabismus divergens about 15°, Horizontal nystagmus(+). Eye ball movement: full & free. Lid fissure: no antimongoloid fissure. Pupil: round, isocoric, L-R: prompt. Eye ground: disc atrophic change with residual edematous picture (OU). Ⅱ. Face and Skull: Prominent skull with ”parrot-beak” nose, broad and depressed face and prognathia. The ears seem pulled downward and backward. Ⅲ. X-ray of the Skull: Dome-shaped skull with obvious increase in digital marking and thin in cranium. Relative retardation of facial bone (maxillary bone), and arched palatine bone were seen. Nasal bone was short, with paranasal sinus obliteration. (frontal and maxillary sinus). Orbit was wide, but shallow, and optic canal seems normal, without definite distortion of the lesser wing of sphenoid but deepening in anterior and middle cranial fossa. Sella turcica seems normal. Synostosis of sagital and coronary sutures was seen. Ⅳ. Mental condition: dull. The patient was operated on at age of 20-month by Ingeraham's decompression operation. The result was fair, and vision was slightly improved.

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頭顔部骨形成不全症一例

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